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Strategies to treat food allergy with oral immunotherapy
according to a new review.
In OIT, a patient who is allergic to a specific food consumes increasing amounts of the allergen over time to reduce their risk for allergic reaction.
“OIT is an elective, usually noncurative procedure with inherent risks that require families to function as amateur medical professionals. Preparing them for this role is essential to protect patients and ensure the long-term success of this life-changing procedure,” lead author Douglas P. Mack, MD, MSc, a pediatric allergy, asthma, and immunology specialist at McMaster University in Hamilton, Ont., and colleagues write in Clinical & Experimental Allergy.
From strict avoidance to desensitization
Food allergy treatment has traditionally involved avoiding accidental exposure that may lead to anaphylaxis and providing rescue medication. In recent years, OIT “has been recommended by several guidelines as a primary option,” Dr. Mack and coauthors write. And with the “approval by European and USA regulators of [peanut allergen powder] Palforzia [Aimmune Therapeutics], there are now commercial and noncommercial forms of OIT available for use in several countries.”
They advise physicians to take a proactive, educational, supportive approach to patients and their families throughout the therapy.
“Ultimately, the decision to pursue OIT or continue avoidance strategies remains the responsibility of the family and the patients,” they write. “Some families may not be prepared for the role that they have to play in actively managing their child’s food allergy treatment.”
Strategies to overcome OIT challenges
Reviewing the literature about OIT for food allergy, the authors suggest various strategies physicians can use to help OIT patients and their families prepare for and overcome common treatment-related challenges.
Two experts welcome the report
Rita Kachru, MD, a specialist in allergy and immunology and a codirector of the food allergy program at UCLA Health in Los Angeles, called this “an excellent report about a wonderful, individualized option in food allergy management.
“The authors did an excellent job delineating OIT terminology, outlining the goals, risks, and benefits of OIT, noting that it’s not a cure, and emphasizing the crucial importance of discussions with each family throughout the process,” Dr. Kachru, who was not involved in developing the report, told this news organization.
“I thoroughly agree with their assessment,” she added. “The more you do OIT research and clinical care, the more you realize the pitfalls, the benefits, and the importance of patient goals and family dynamics. Discussing the goals, risks, benefits, and alternatives to OIT in detail with the family is crucial so they fully understand the process.”
Basil M. Kahwash, MD, an allergy, pulmonary, and critical care medicine specialist at Vanderbilt University Medical Center in Nashville, Tenn., said that providers in the immunology community have been discussing OIT for years and that he welcomes the well-written report that summarizes the evidence.
“It’s important to periodically summarize the evidence, as well as the consensus expert opinion about the evidence, so we may better inform our colleagues and patients,” said Dr. Kahwash, who was not involved in developing the report. “The authors are well-known experts in our field who have experience with OIT and with reviewing the evidence of food allergy.
“OIT can be a fantastic option for some patients, especially those who are very highly motivated and understand the process from start to finish. But OIT is not the best option for every child, and it’s not much of an option for adults,” he explained. “Patients need to be chosen carefully and understand the level of motivation required to safely follow through with the treatment.
“The report will hopefully affect patient care positively and allow patients to understand the limitations around OIT when they consider their candidacy for it,” he added. “In most cases, OIT patients will still need to avoid the allergen, but if a small amount accidentally gets into their food, they probably won’t have a very severe reaction to it.”
Dr. Kahwash would like to see data on patients who have seen long-term remission with OIT.
“Clearly, some patients benefit from OIT. What differentiates patients who benefit from OIT from those who do not?” he asked. “In the future, we need to consider possible biomarkers of patients who are and who aren’t good candidates for OIT.
“Regardless of OIT’s limitations, the potential for desensitization rather than strict avoidance represents a big step in the evolution of food allergy treatment,” Dr. Kahwash noted.
No funding details were provided. Dr. Mack and three coauthors report financial relationships with Aimmune. Aimmune Therapeutics is the manufacturer of Palforzia OIT (AR101 powder provided in capsules and sachets). Most coauthors also report financial relationships with other pharmaceutical companies. The full list can be found with the original article. Dr. Kachru was an investigator in the PALISADES clinical trial of AR101. Dr. Kahwash reports no relevant financial relationships.
A version of this article first appeared on Medscape.com.
according to a new review.
In OIT, a patient who is allergic to a specific food consumes increasing amounts of the allergen over time to reduce their risk for allergic reaction.
“OIT is an elective, usually noncurative procedure with inherent risks that require families to function as amateur medical professionals. Preparing them for this role is essential to protect patients and ensure the long-term success of this life-changing procedure,” lead author Douglas P. Mack, MD, MSc, a pediatric allergy, asthma, and immunology specialist at McMaster University in Hamilton, Ont., and colleagues write in Clinical & Experimental Allergy.
From strict avoidance to desensitization
Food allergy treatment has traditionally involved avoiding accidental exposure that may lead to anaphylaxis and providing rescue medication. In recent years, OIT “has been recommended by several guidelines as a primary option,” Dr. Mack and coauthors write. And with the “approval by European and USA regulators of [peanut allergen powder] Palforzia [Aimmune Therapeutics], there are now commercial and noncommercial forms of OIT available for use in several countries.”
They advise physicians to take a proactive, educational, supportive approach to patients and their families throughout the therapy.
“Ultimately, the decision to pursue OIT or continue avoidance strategies remains the responsibility of the family and the patients,” they write. “Some families may not be prepared for the role that they have to play in actively managing their child’s food allergy treatment.”
Strategies to overcome OIT challenges
Reviewing the literature about OIT for food allergy, the authors suggest various strategies physicians can use to help OIT patients and their families prepare for and overcome common treatment-related challenges.
Two experts welcome the report
Rita Kachru, MD, a specialist in allergy and immunology and a codirector of the food allergy program at UCLA Health in Los Angeles, called this “an excellent report about a wonderful, individualized option in food allergy management.
“The authors did an excellent job delineating OIT terminology, outlining the goals, risks, and benefits of OIT, noting that it’s not a cure, and emphasizing the crucial importance of discussions with each family throughout the process,” Dr. Kachru, who was not involved in developing the report, told this news organization.
“I thoroughly agree with their assessment,” she added. “The more you do OIT research and clinical care, the more you realize the pitfalls, the benefits, and the importance of patient goals and family dynamics. Discussing the goals, risks, benefits, and alternatives to OIT in detail with the family is crucial so they fully understand the process.”
Basil M. Kahwash, MD, an allergy, pulmonary, and critical care medicine specialist at Vanderbilt University Medical Center in Nashville, Tenn., said that providers in the immunology community have been discussing OIT for years and that he welcomes the well-written report that summarizes the evidence.
“It’s important to periodically summarize the evidence, as well as the consensus expert opinion about the evidence, so we may better inform our colleagues and patients,” said Dr. Kahwash, who was not involved in developing the report. “The authors are well-known experts in our field who have experience with OIT and with reviewing the evidence of food allergy.
“OIT can be a fantastic option for some patients, especially those who are very highly motivated and understand the process from start to finish. But OIT is not the best option for every child, and it’s not much of an option for adults,” he explained. “Patients need to be chosen carefully and understand the level of motivation required to safely follow through with the treatment.
“The report will hopefully affect patient care positively and allow patients to understand the limitations around OIT when they consider their candidacy for it,” he added. “In most cases, OIT patients will still need to avoid the allergen, but if a small amount accidentally gets into their food, they probably won’t have a very severe reaction to it.”
Dr. Kahwash would like to see data on patients who have seen long-term remission with OIT.
“Clearly, some patients benefit from OIT. What differentiates patients who benefit from OIT from those who do not?” he asked. “In the future, we need to consider possible biomarkers of patients who are and who aren’t good candidates for OIT.
“Regardless of OIT’s limitations, the potential for desensitization rather than strict avoidance represents a big step in the evolution of food allergy treatment,” Dr. Kahwash noted.
No funding details were provided. Dr. Mack and three coauthors report financial relationships with Aimmune. Aimmune Therapeutics is the manufacturer of Palforzia OIT (AR101 powder provided in capsules and sachets). Most coauthors also report financial relationships with other pharmaceutical companies. The full list can be found with the original article. Dr. Kachru was an investigator in the PALISADES clinical trial of AR101. Dr. Kahwash reports no relevant financial relationships.
A version of this article first appeared on Medscape.com.
according to a new review.
In OIT, a patient who is allergic to a specific food consumes increasing amounts of the allergen over time to reduce their risk for allergic reaction.
“OIT is an elective, usually noncurative procedure with inherent risks that require families to function as amateur medical professionals. Preparing them for this role is essential to protect patients and ensure the long-term success of this life-changing procedure,” lead author Douglas P. Mack, MD, MSc, a pediatric allergy, asthma, and immunology specialist at McMaster University in Hamilton, Ont., and colleagues write in Clinical & Experimental Allergy.
From strict avoidance to desensitization
Food allergy treatment has traditionally involved avoiding accidental exposure that may lead to anaphylaxis and providing rescue medication. In recent years, OIT “has been recommended by several guidelines as a primary option,” Dr. Mack and coauthors write. And with the “approval by European and USA regulators of [peanut allergen powder] Palforzia [Aimmune Therapeutics], there are now commercial and noncommercial forms of OIT available for use in several countries.”
They advise physicians to take a proactive, educational, supportive approach to patients and their families throughout the therapy.
“Ultimately, the decision to pursue OIT or continue avoidance strategies remains the responsibility of the family and the patients,” they write. “Some families may not be prepared for the role that they have to play in actively managing their child’s food allergy treatment.”
Strategies to overcome OIT challenges
Reviewing the literature about OIT for food allergy, the authors suggest various strategies physicians can use to help OIT patients and their families prepare for and overcome common treatment-related challenges.
Two experts welcome the report
Rita Kachru, MD, a specialist in allergy and immunology and a codirector of the food allergy program at UCLA Health in Los Angeles, called this “an excellent report about a wonderful, individualized option in food allergy management.
“The authors did an excellent job delineating OIT terminology, outlining the goals, risks, and benefits of OIT, noting that it’s not a cure, and emphasizing the crucial importance of discussions with each family throughout the process,” Dr. Kachru, who was not involved in developing the report, told this news organization.
“I thoroughly agree with their assessment,” she added. “The more you do OIT research and clinical care, the more you realize the pitfalls, the benefits, and the importance of patient goals and family dynamics. Discussing the goals, risks, benefits, and alternatives to OIT in detail with the family is crucial so they fully understand the process.”
Basil M. Kahwash, MD, an allergy, pulmonary, and critical care medicine specialist at Vanderbilt University Medical Center in Nashville, Tenn., said that providers in the immunology community have been discussing OIT for years and that he welcomes the well-written report that summarizes the evidence.
“It’s important to periodically summarize the evidence, as well as the consensus expert opinion about the evidence, so we may better inform our colleagues and patients,” said Dr. Kahwash, who was not involved in developing the report. “The authors are well-known experts in our field who have experience with OIT and with reviewing the evidence of food allergy.
“OIT can be a fantastic option for some patients, especially those who are very highly motivated and understand the process from start to finish. But OIT is not the best option for every child, and it’s not much of an option for adults,” he explained. “Patients need to be chosen carefully and understand the level of motivation required to safely follow through with the treatment.
“The report will hopefully affect patient care positively and allow patients to understand the limitations around OIT when they consider their candidacy for it,” he added. “In most cases, OIT patients will still need to avoid the allergen, but if a small amount accidentally gets into their food, they probably won’t have a very severe reaction to it.”
Dr. Kahwash would like to see data on patients who have seen long-term remission with OIT.
“Clearly, some patients benefit from OIT. What differentiates patients who benefit from OIT from those who do not?” he asked. “In the future, we need to consider possible biomarkers of patients who are and who aren’t good candidates for OIT.
“Regardless of OIT’s limitations, the potential for desensitization rather than strict avoidance represents a big step in the evolution of food allergy treatment,” Dr. Kahwash noted.
No funding details were provided. Dr. Mack and three coauthors report financial relationships with Aimmune. Aimmune Therapeutics is the manufacturer of Palforzia OIT (AR101 powder provided in capsules and sachets). Most coauthors also report financial relationships with other pharmaceutical companies. The full list can be found with the original article. Dr. Kachru was an investigator in the PALISADES clinical trial of AR101. Dr. Kahwash reports no relevant financial relationships.
A version of this article first appeared on Medscape.com.
FROM CLINICAL & EXPERIMENTAL ALLERGY
Younger doctors call for more attention to patients with disabilities
As an undergraduate student at Northeastern University in Boston, Meghan Chin spent her summers working for a day program in Rhode Island. Her charges were adults with various forms of intellectual and developmental disabilities (IDD).
“I was very much a caretaker,” Ms. Chin, now 29, said. “It was everything from helping them get dressed in the morning to getting them to medical appointments.”
During one such visit Ms. Chin got a lesson about how health care looks from the viewpoint of someone with an IDD.
The patient was a woman in her 60s and she was having gastrointestinal issues; symptoms she could have articulated, if asked. “She was perfectly capable of telling a clinician where it hurt, how long she had experienced the problem, and what she had done or not done to alleviate it,” Ms. Chin said.
And of comprehending a response. But she was not given the opportunity.
“She would explain what was going on to the clinician,” Ms. Chin recalled. “And the clinician would turn to me and answer. It was this weird three-way conversation – as if she wasn’t even there in the room with us.”
Ms. Chin was incensed at the rude and disrespectful way the patient had been treated. But her charge didn’t seem upset or surprised. Just resigned. “Sadly, she had become used to this,” Ms. Chin said.
For the young aide, however, the experience was searing. “It didn’t seem right to me,” Ms. Chin said. “That’s why, when I went to medical school, I knew I wanted to do better for this population.”
Serendipity led her to Georgetown University, Washington, where she met Kim Bullock, MD, one of the country’s leading advocates for improved health care delivery to those with IDDs.
Dr. Bullock, an associate professor of family medicine, seeks to create better training and educational opportunities for medical students who will likely encounter patients with these disabilities in their practices.
When Dr. Bullock heard Ms. Chin’s story about the patient being ignored, she was not surprised.
“This is not an unusual or unique situation,” said Dr. Bullock, who is also director of Georgetown’s community health division and a faculty member of the university’s Center for Excellence for Developmental Disabilities. “In fact, it’s quite common and is part of what spurred my own interest in educating pre-med and medical students about effective communication techniques, particularly when addressing neurodiverse patients.”
More than 13% of Americans, or roughly 44 million people, have some form of disability, according to the National Institute on Disability at the University of New Hampshire, a figure that does not include those who are institutionalized. The Centers for Disease Control and Prevention estimates that 17% of children aged 3-17 years have a developmental disability.
Even so, many physicians feel ill-prepared to care for disabled patients. A survey of physicians, published in the journal Health Affairs, found that some lacked the resources and training to properly care for patients with disabilities, or that they struggled to coordinate care for such individuals. Some said they did not know which types of accessible equipment, like adjustable tables and chair scales, were needed or how to use them. And some said they actively try to avoid treating patients with disabilities.
Don’t assume
The first step at correcting the problem, Dr. Bullock said, is to not assume that all IDD patients are incapable of communicating. By talking not to the patient but to their caregiver or spouse or child, as the clinician did with Ms. Chin years ago, “we are taking away their agency, their autonomy to speak for and about themselves.”
Change involves altering physicians’ attitudes and assumptions toward this population, through education. But how?
“The medical school curriculum is tight as it is,” Dr. Bullock acknowledged. “There’s a lot of things students have to learn. People wonder: where we will add this?”
Her suggestion: Incorporate IDD all along the way, through programs or experiences that will enable medical students to see such patients “not as something separate, but as people that have special needs just as other populations have.”
Case in point: Operation House Call, a program in Massachusetts designed to support young health care professionals, by building “confidence, interest, and sensitivity” toward individuals with IDD.
Eight medical and allied health schools, including those at Harvard Medical School and Yale School of Nursing, participate in the program, the centerpiece of which is time spent by teams of medical students in the homes of families with neurodiverse members. “It’s transformational,” said Susan Feeney, DNP, NP-C, director of adult gerontology and family nurse practitioner programs at the graduate school of nursing at the University of Massachusetts, Worcester. “They spend a few hours at the homes of these families, have this interaction with them, and journal about their experiences.”
Dr. Feeney described as “transformational” the experience of the students after getting to know these families. “They all come back profoundly changed,” she told this news organization. “As a medical or health care professional, you meet people in an artificial environment of the clinic and hospital. Here, they become human, like you. It takes the stigma away.”
One area of medicine in which this is an exception is pediatrics, where interaction with children with IDD and their families is common – and close. “They’re going to be much more attuned to this,” Dr. Feeney said. “The problem is primary care or internal medicine. Once these children get into their mid and later 20s, and they need a practitioner to talk to about adult concerns.”
And with adulthood come other medical needs, as the physical demands of age fall no less heavily on individuals with IDDs than those without. For example: “Neurodiverse people get pregnant,” Dr. Bullock said. They also can get heart disease as they age; or require the care of a rheumatologist, a neurologist, an orthopedic surgeon, or any other medical specialty.
Generation gap
Fortunately, the next generation of physicians may be more open to this more inclusionary approach toward a widely misunderstood population.
Like Ms. Chin, Sarah Bdeir had experience with this population prior to beginning her training in medicine. She had volunteered at a school for people with IDD.
“It was one of the best experiences I’ve ever had,” Ms. Bdeir, now 23 and a first-year medical student at Wayne State University, Detroit, said. She found that the neurodiverse individuals she worked with had as many abilities as disabilities. “They are capable of learning, but they do it differently,” she said. “You have to adjust to the way they learn. And you have to step out of your own box.”
Ms. Bdeir also heard about Dr. Bullock’s work and is assisting her in a research project on how to better improve nutritional education for people with IDDs. And although she said it may take time for curriculum boards at medical schools to integrate this kind of training into their programs, she believes they will, in part because the rising cohort of medical students today have an eagerness to engage with and learn more about IDD patients.
As does Ms. Chin.
“When I talk to my peers about this, they’re very receptive,” Ms. Chin said. “They want to learn how to better support the IDD population. And they will learn. I believe in my generation of future doctors.”
A version of this article first appeared on Medscape.com.
As an undergraduate student at Northeastern University in Boston, Meghan Chin spent her summers working for a day program in Rhode Island. Her charges were adults with various forms of intellectual and developmental disabilities (IDD).
“I was very much a caretaker,” Ms. Chin, now 29, said. “It was everything from helping them get dressed in the morning to getting them to medical appointments.”
During one such visit Ms. Chin got a lesson about how health care looks from the viewpoint of someone with an IDD.
The patient was a woman in her 60s and she was having gastrointestinal issues; symptoms she could have articulated, if asked. “She was perfectly capable of telling a clinician where it hurt, how long she had experienced the problem, and what she had done or not done to alleviate it,” Ms. Chin said.
And of comprehending a response. But she was not given the opportunity.
“She would explain what was going on to the clinician,” Ms. Chin recalled. “And the clinician would turn to me and answer. It was this weird three-way conversation – as if she wasn’t even there in the room with us.”
Ms. Chin was incensed at the rude and disrespectful way the patient had been treated. But her charge didn’t seem upset or surprised. Just resigned. “Sadly, she had become used to this,” Ms. Chin said.
For the young aide, however, the experience was searing. “It didn’t seem right to me,” Ms. Chin said. “That’s why, when I went to medical school, I knew I wanted to do better for this population.”
Serendipity led her to Georgetown University, Washington, where she met Kim Bullock, MD, one of the country’s leading advocates for improved health care delivery to those with IDDs.
Dr. Bullock, an associate professor of family medicine, seeks to create better training and educational opportunities for medical students who will likely encounter patients with these disabilities in their practices.
When Dr. Bullock heard Ms. Chin’s story about the patient being ignored, she was not surprised.
“This is not an unusual or unique situation,” said Dr. Bullock, who is also director of Georgetown’s community health division and a faculty member of the university’s Center for Excellence for Developmental Disabilities. “In fact, it’s quite common and is part of what spurred my own interest in educating pre-med and medical students about effective communication techniques, particularly when addressing neurodiverse patients.”
More than 13% of Americans, or roughly 44 million people, have some form of disability, according to the National Institute on Disability at the University of New Hampshire, a figure that does not include those who are institutionalized. The Centers for Disease Control and Prevention estimates that 17% of children aged 3-17 years have a developmental disability.
Even so, many physicians feel ill-prepared to care for disabled patients. A survey of physicians, published in the journal Health Affairs, found that some lacked the resources and training to properly care for patients with disabilities, or that they struggled to coordinate care for such individuals. Some said they did not know which types of accessible equipment, like adjustable tables and chair scales, were needed or how to use them. And some said they actively try to avoid treating patients with disabilities.
Don’t assume
The first step at correcting the problem, Dr. Bullock said, is to not assume that all IDD patients are incapable of communicating. By talking not to the patient but to their caregiver or spouse or child, as the clinician did with Ms. Chin years ago, “we are taking away their agency, their autonomy to speak for and about themselves.”
Change involves altering physicians’ attitudes and assumptions toward this population, through education. But how?
“The medical school curriculum is tight as it is,” Dr. Bullock acknowledged. “There’s a lot of things students have to learn. People wonder: where we will add this?”
Her suggestion: Incorporate IDD all along the way, through programs or experiences that will enable medical students to see such patients “not as something separate, but as people that have special needs just as other populations have.”
Case in point: Operation House Call, a program in Massachusetts designed to support young health care professionals, by building “confidence, interest, and sensitivity” toward individuals with IDD.
Eight medical and allied health schools, including those at Harvard Medical School and Yale School of Nursing, participate in the program, the centerpiece of which is time spent by teams of medical students in the homes of families with neurodiverse members. “It’s transformational,” said Susan Feeney, DNP, NP-C, director of adult gerontology and family nurse practitioner programs at the graduate school of nursing at the University of Massachusetts, Worcester. “They spend a few hours at the homes of these families, have this interaction with them, and journal about their experiences.”
Dr. Feeney described as “transformational” the experience of the students after getting to know these families. “They all come back profoundly changed,” she told this news organization. “As a medical or health care professional, you meet people in an artificial environment of the clinic and hospital. Here, they become human, like you. It takes the stigma away.”
One area of medicine in which this is an exception is pediatrics, where interaction with children with IDD and their families is common – and close. “They’re going to be much more attuned to this,” Dr. Feeney said. “The problem is primary care or internal medicine. Once these children get into their mid and later 20s, and they need a practitioner to talk to about adult concerns.”
And with adulthood come other medical needs, as the physical demands of age fall no less heavily on individuals with IDDs than those without. For example: “Neurodiverse people get pregnant,” Dr. Bullock said. They also can get heart disease as they age; or require the care of a rheumatologist, a neurologist, an orthopedic surgeon, or any other medical specialty.
Generation gap
Fortunately, the next generation of physicians may be more open to this more inclusionary approach toward a widely misunderstood population.
Like Ms. Chin, Sarah Bdeir had experience with this population prior to beginning her training in medicine. She had volunteered at a school for people with IDD.
“It was one of the best experiences I’ve ever had,” Ms. Bdeir, now 23 and a first-year medical student at Wayne State University, Detroit, said. She found that the neurodiverse individuals she worked with had as many abilities as disabilities. “They are capable of learning, but they do it differently,” she said. “You have to adjust to the way they learn. And you have to step out of your own box.”
Ms. Bdeir also heard about Dr. Bullock’s work and is assisting her in a research project on how to better improve nutritional education for people with IDDs. And although she said it may take time for curriculum boards at medical schools to integrate this kind of training into their programs, she believes they will, in part because the rising cohort of medical students today have an eagerness to engage with and learn more about IDD patients.
As does Ms. Chin.
“When I talk to my peers about this, they’re very receptive,” Ms. Chin said. “They want to learn how to better support the IDD population. And they will learn. I believe in my generation of future doctors.”
A version of this article first appeared on Medscape.com.
As an undergraduate student at Northeastern University in Boston, Meghan Chin spent her summers working for a day program in Rhode Island. Her charges were adults with various forms of intellectual and developmental disabilities (IDD).
“I was very much a caretaker,” Ms. Chin, now 29, said. “It was everything from helping them get dressed in the morning to getting them to medical appointments.”
During one such visit Ms. Chin got a lesson about how health care looks from the viewpoint of someone with an IDD.
The patient was a woman in her 60s and she was having gastrointestinal issues; symptoms she could have articulated, if asked. “She was perfectly capable of telling a clinician where it hurt, how long she had experienced the problem, and what she had done or not done to alleviate it,” Ms. Chin said.
And of comprehending a response. But she was not given the opportunity.
“She would explain what was going on to the clinician,” Ms. Chin recalled. “And the clinician would turn to me and answer. It was this weird three-way conversation – as if she wasn’t even there in the room with us.”
Ms. Chin was incensed at the rude and disrespectful way the patient had been treated. But her charge didn’t seem upset or surprised. Just resigned. “Sadly, she had become used to this,” Ms. Chin said.
For the young aide, however, the experience was searing. “It didn’t seem right to me,” Ms. Chin said. “That’s why, when I went to medical school, I knew I wanted to do better for this population.”
Serendipity led her to Georgetown University, Washington, where she met Kim Bullock, MD, one of the country’s leading advocates for improved health care delivery to those with IDDs.
Dr. Bullock, an associate professor of family medicine, seeks to create better training and educational opportunities for medical students who will likely encounter patients with these disabilities in their practices.
When Dr. Bullock heard Ms. Chin’s story about the patient being ignored, she was not surprised.
“This is not an unusual or unique situation,” said Dr. Bullock, who is also director of Georgetown’s community health division and a faculty member of the university’s Center for Excellence for Developmental Disabilities. “In fact, it’s quite common and is part of what spurred my own interest in educating pre-med and medical students about effective communication techniques, particularly when addressing neurodiverse patients.”
More than 13% of Americans, or roughly 44 million people, have some form of disability, according to the National Institute on Disability at the University of New Hampshire, a figure that does not include those who are institutionalized. The Centers for Disease Control and Prevention estimates that 17% of children aged 3-17 years have a developmental disability.
Even so, many physicians feel ill-prepared to care for disabled patients. A survey of physicians, published in the journal Health Affairs, found that some lacked the resources and training to properly care for patients with disabilities, or that they struggled to coordinate care for such individuals. Some said they did not know which types of accessible equipment, like adjustable tables and chair scales, were needed or how to use them. And some said they actively try to avoid treating patients with disabilities.
Don’t assume
The first step at correcting the problem, Dr. Bullock said, is to not assume that all IDD patients are incapable of communicating. By talking not to the patient but to their caregiver or spouse or child, as the clinician did with Ms. Chin years ago, “we are taking away their agency, their autonomy to speak for and about themselves.”
Change involves altering physicians’ attitudes and assumptions toward this population, through education. But how?
“The medical school curriculum is tight as it is,” Dr. Bullock acknowledged. “There’s a lot of things students have to learn. People wonder: where we will add this?”
Her suggestion: Incorporate IDD all along the way, through programs or experiences that will enable medical students to see such patients “not as something separate, but as people that have special needs just as other populations have.”
Case in point: Operation House Call, a program in Massachusetts designed to support young health care professionals, by building “confidence, interest, and sensitivity” toward individuals with IDD.
Eight medical and allied health schools, including those at Harvard Medical School and Yale School of Nursing, participate in the program, the centerpiece of which is time spent by teams of medical students in the homes of families with neurodiverse members. “It’s transformational,” said Susan Feeney, DNP, NP-C, director of adult gerontology and family nurse practitioner programs at the graduate school of nursing at the University of Massachusetts, Worcester. “They spend a few hours at the homes of these families, have this interaction with them, and journal about their experiences.”
Dr. Feeney described as “transformational” the experience of the students after getting to know these families. “They all come back profoundly changed,” she told this news organization. “As a medical or health care professional, you meet people in an artificial environment of the clinic and hospital. Here, they become human, like you. It takes the stigma away.”
One area of medicine in which this is an exception is pediatrics, where interaction with children with IDD and their families is common – and close. “They’re going to be much more attuned to this,” Dr. Feeney said. “The problem is primary care or internal medicine. Once these children get into their mid and later 20s, and they need a practitioner to talk to about adult concerns.”
And with adulthood come other medical needs, as the physical demands of age fall no less heavily on individuals with IDDs than those without. For example: “Neurodiverse people get pregnant,” Dr. Bullock said. They also can get heart disease as they age; or require the care of a rheumatologist, a neurologist, an orthopedic surgeon, or any other medical specialty.
Generation gap
Fortunately, the next generation of physicians may be more open to this more inclusionary approach toward a widely misunderstood population.
Like Ms. Chin, Sarah Bdeir had experience with this population prior to beginning her training in medicine. She had volunteered at a school for people with IDD.
“It was one of the best experiences I’ve ever had,” Ms. Bdeir, now 23 and a first-year medical student at Wayne State University, Detroit, said. She found that the neurodiverse individuals she worked with had as many abilities as disabilities. “They are capable of learning, but they do it differently,” she said. “You have to adjust to the way they learn. And you have to step out of your own box.”
Ms. Bdeir also heard about Dr. Bullock’s work and is assisting her in a research project on how to better improve nutritional education for people with IDDs. And although she said it may take time for curriculum boards at medical schools to integrate this kind of training into their programs, she believes they will, in part because the rising cohort of medical students today have an eagerness to engage with and learn more about IDD patients.
As does Ms. Chin.
“When I talk to my peers about this, they’re very receptive,” Ms. Chin said. “They want to learn how to better support the IDD population. And they will learn. I believe in my generation of future doctors.”
A version of this article first appeared on Medscape.com.
Understanding of capillary malformation characteristics continue to evolve
INDIANAPOLIS – The way Maria C. Garzon, MD, sees it,
“The challenge is, we also use that term to describe a diagnosis,” Dr. Garzon, professor of dermatology and pediatrics at Columbia University, New York, said at the annual meeting of the Society for Pediatric Dermatology. “We have imperfect terminology. We use many different terms like capillary nevi and vascular stain. Instead of port wine stain, we now use the term port wine birthmark, and old terms like nevus flammeus are still used. This leads to diagnostic confusion, and it’s a barrier to developing care guidelines.”
Some capillary malformations, she noted, are benign and fade away while others can cause disfigurement or herald significant medical issues.
Histologically, she continued, not all capillary malformations are composed of capillaries. “Some are composed of postcapillary venules,” she said. “There are also mixed type capillary malformations that include lymphatic tissue, and the capillary malformation of capillary malformation-arteriovenous malformation (CM-AVM) syndrome shares histologic features of evolving AVMs as opposed to classic port wine birthmarks.”
The most recent International Society for the Study of Vascular Anomalies Classification of Vascular Anomalies was published in 2018 and is currently being updated. Other proposed clinical classifications have been published, including one that is diagnosis-specific and includes 20 different types of capillary malformations (J Eur Acad Dermatol Venereol. 2015 29[12]:2295-305, Pediatr Dermatol. 2016;33[6]:570-84).
“There are also syndromic classifications. Another question relates to the role of genomics: Are we ready for a classification that’s based purely on genetic variants, or do we need to incorporate it into existing classifications?” Dr. Garzon said. “Novel testing technologies using cell-free DNA and digital droplet PCR may be used in the future to establish diagnoses.” Genetic variants are found within capillary malformations, and they tend to be associated with three major pathways: the RAS-MAPK/ERK pathway, the PI3K/Akt/mTOR pathway, and the G protein pathway.
The type of capillary malformation that dermatologists and pediatricians most commonly see is nevus simplex, which occurs in 20%-82% of neonates. Other terms used include angel’s kiss, stork bite, salmon patch, nevus flammeus simplex, fading vascular stain, medial telangiectatic nevus, and butterfly mark. “It’s important to differentiate this from a port wine birthmark,” Dr. Garzon said. “This can be challenging when the birthmark is a darker red color. I have cared for patients who were initially thought to have nevus simplex and later found to have Sturge-Weber syndrome.”
Typical locations of nevus simplex include the central forehead/glabella, eyelids, the nape of the neck, scalp (parietal and occipital), nose, lip area (including philtrum), and the back (lumbosacral area and upper back). Most lesions fade/disappear without treatment (J Am Acad Dermatol. 2020;63[5]:805-14). Rare genetic syndromes associated with exaggerated nevus simplex complex include macrocephaly-capillary malformation syndrome and Beckwith-Wiedemann syndrome, “which tells us that this is a heterogeneous group of patients,” she said.
Dr. Garzon added that it’s “incredibly common” to see an eczema flare occurring within a nevus simplex on the nape of the neck. These patients will have a patch of atopic dermatitis that doesn’t get better. “Beneath it is their nevus simplex,” she said. “Remind parents that even after treating the eczema, the pink patch is not going to go away” (Pediatr Rep. 2021;13[1]:131-4).
Meanwhile, the classic port wine birthmark is usually congenital, uniform, and darker red in color. It darkens with maturity and the pattern will correlate with embryonic vasculature. “I am very wary of acquired port wine lesions,” she added. “It’s been described with trauma-related lesions, but early morphea can also mimic a port wine birthmark. You will see this if you’re practicing pediatric dermatology.”
Nearly a decade ago researchers established a link between port wine birthmarks and genetic variants in the GNAQ gene. “We see this in GNA11 as well,” Dr. Garzon said. “These changes are found in isolated port wine stains, and in Sturge-Weber syndrome. We now know that GNAQ drives the formation of large blood vessels through angiopoietin-2,” she noted (Arterioscler Throm Vasc Biol. 2022;42[1]:e27-43).
In general, studies that have examined genotype-phenotype correlations have demonstrated that the classic port wine birthmark is associated with GNAQ while GNA11 variants can be associated with a more reticulated pattern. “But this is not as clearcut as it seems,” she said. Investigators of a recent study showed an association between hypertension and renal anomalies in patients with skin capillary malformations and mosaic GNAQ or GNA11 variants. “This is a new finding,” she said. “Investigators are working to understand this association.”
Port wine birthmarks with the highest risk of Sturge-Weber syndrome include those that involve the forehead, upper eyelid, the midline frontonasal area, the hemifacial area, and median sites. “Patients who have this should be evaluated at birth,” Dr. Garzon said. “You should not delay for 2 months. They should be evaluated by ophthalmology and neurology early.”
The other morphologies commonly seen are “geographic” well-demarcated capillary malformations, which are dark in color. These lesions can be seen in conditions that are associated with genetic variants in PIK3CA (PROS) and include classic Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal) syndrome, and CLAPO (capillary malformation of the lower lip, lymphatic malformation of the face and neck, asymmetry of the face and limbs, and partial or generalized overgrowth) syndrome.
“Reticulated stains are much more heterogeneous,” Dr. Garzon said. “They can be localized or widespread. When you see a patient with a widespread reticulated capillary malformation, think about diffuse capillary malformation with overgrowth (DCMO). This condition is clinically and genetically heterogenous with the affected tissue of some patients showing variants in GNA11 while others have variants in PIK3CA. Therefore, a thorough examination at presentation and long-term follow-up is very important.”
Dr. Garzon disclosed that she is a member of the executive board for the International Society for the Study of Vascular Anomalies.
INDIANAPOLIS – The way Maria C. Garzon, MD, sees it,
“The challenge is, we also use that term to describe a diagnosis,” Dr. Garzon, professor of dermatology and pediatrics at Columbia University, New York, said at the annual meeting of the Society for Pediatric Dermatology. “We have imperfect terminology. We use many different terms like capillary nevi and vascular stain. Instead of port wine stain, we now use the term port wine birthmark, and old terms like nevus flammeus are still used. This leads to diagnostic confusion, and it’s a barrier to developing care guidelines.”
Some capillary malformations, she noted, are benign and fade away while others can cause disfigurement or herald significant medical issues.
Histologically, she continued, not all capillary malformations are composed of capillaries. “Some are composed of postcapillary venules,” she said. “There are also mixed type capillary malformations that include lymphatic tissue, and the capillary malformation of capillary malformation-arteriovenous malformation (CM-AVM) syndrome shares histologic features of evolving AVMs as opposed to classic port wine birthmarks.”
The most recent International Society for the Study of Vascular Anomalies Classification of Vascular Anomalies was published in 2018 and is currently being updated. Other proposed clinical classifications have been published, including one that is diagnosis-specific and includes 20 different types of capillary malformations (J Eur Acad Dermatol Venereol. 2015 29[12]:2295-305, Pediatr Dermatol. 2016;33[6]:570-84).
“There are also syndromic classifications. Another question relates to the role of genomics: Are we ready for a classification that’s based purely on genetic variants, or do we need to incorporate it into existing classifications?” Dr. Garzon said. “Novel testing technologies using cell-free DNA and digital droplet PCR may be used in the future to establish diagnoses.” Genetic variants are found within capillary malformations, and they tend to be associated with three major pathways: the RAS-MAPK/ERK pathway, the PI3K/Akt/mTOR pathway, and the G protein pathway.
The type of capillary malformation that dermatologists and pediatricians most commonly see is nevus simplex, which occurs in 20%-82% of neonates. Other terms used include angel’s kiss, stork bite, salmon patch, nevus flammeus simplex, fading vascular stain, medial telangiectatic nevus, and butterfly mark. “It’s important to differentiate this from a port wine birthmark,” Dr. Garzon said. “This can be challenging when the birthmark is a darker red color. I have cared for patients who were initially thought to have nevus simplex and later found to have Sturge-Weber syndrome.”
Typical locations of nevus simplex include the central forehead/glabella, eyelids, the nape of the neck, scalp (parietal and occipital), nose, lip area (including philtrum), and the back (lumbosacral area and upper back). Most lesions fade/disappear without treatment (J Am Acad Dermatol. 2020;63[5]:805-14). Rare genetic syndromes associated with exaggerated nevus simplex complex include macrocephaly-capillary malformation syndrome and Beckwith-Wiedemann syndrome, “which tells us that this is a heterogeneous group of patients,” she said.
Dr. Garzon added that it’s “incredibly common” to see an eczema flare occurring within a nevus simplex on the nape of the neck. These patients will have a patch of atopic dermatitis that doesn’t get better. “Beneath it is their nevus simplex,” she said. “Remind parents that even after treating the eczema, the pink patch is not going to go away” (Pediatr Rep. 2021;13[1]:131-4).
Meanwhile, the classic port wine birthmark is usually congenital, uniform, and darker red in color. It darkens with maturity and the pattern will correlate with embryonic vasculature. “I am very wary of acquired port wine lesions,” she added. “It’s been described with trauma-related lesions, but early morphea can also mimic a port wine birthmark. You will see this if you’re practicing pediatric dermatology.”
Nearly a decade ago researchers established a link between port wine birthmarks and genetic variants in the GNAQ gene. “We see this in GNA11 as well,” Dr. Garzon said. “These changes are found in isolated port wine stains, and in Sturge-Weber syndrome. We now know that GNAQ drives the formation of large blood vessels through angiopoietin-2,” she noted (Arterioscler Throm Vasc Biol. 2022;42[1]:e27-43).
In general, studies that have examined genotype-phenotype correlations have demonstrated that the classic port wine birthmark is associated with GNAQ while GNA11 variants can be associated with a more reticulated pattern. “But this is not as clearcut as it seems,” she said. Investigators of a recent study showed an association between hypertension and renal anomalies in patients with skin capillary malformations and mosaic GNAQ or GNA11 variants. “This is a new finding,” she said. “Investigators are working to understand this association.”
Port wine birthmarks with the highest risk of Sturge-Weber syndrome include those that involve the forehead, upper eyelid, the midline frontonasal area, the hemifacial area, and median sites. “Patients who have this should be evaluated at birth,” Dr. Garzon said. “You should not delay for 2 months. They should be evaluated by ophthalmology and neurology early.”
The other morphologies commonly seen are “geographic” well-demarcated capillary malformations, which are dark in color. These lesions can be seen in conditions that are associated with genetic variants in PIK3CA (PROS) and include classic Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal) syndrome, and CLAPO (capillary malformation of the lower lip, lymphatic malformation of the face and neck, asymmetry of the face and limbs, and partial or generalized overgrowth) syndrome.
“Reticulated stains are much more heterogeneous,” Dr. Garzon said. “They can be localized or widespread. When you see a patient with a widespread reticulated capillary malformation, think about diffuse capillary malformation with overgrowth (DCMO). This condition is clinically and genetically heterogenous with the affected tissue of some patients showing variants in GNA11 while others have variants in PIK3CA. Therefore, a thorough examination at presentation and long-term follow-up is very important.”
Dr. Garzon disclosed that she is a member of the executive board for the International Society for the Study of Vascular Anomalies.
INDIANAPOLIS – The way Maria C. Garzon, MD, sees it,
“The challenge is, we also use that term to describe a diagnosis,” Dr. Garzon, professor of dermatology and pediatrics at Columbia University, New York, said at the annual meeting of the Society for Pediatric Dermatology. “We have imperfect terminology. We use many different terms like capillary nevi and vascular stain. Instead of port wine stain, we now use the term port wine birthmark, and old terms like nevus flammeus are still used. This leads to diagnostic confusion, and it’s a barrier to developing care guidelines.”
Some capillary malformations, she noted, are benign and fade away while others can cause disfigurement or herald significant medical issues.
Histologically, she continued, not all capillary malformations are composed of capillaries. “Some are composed of postcapillary venules,” she said. “There are also mixed type capillary malformations that include lymphatic tissue, and the capillary malformation of capillary malformation-arteriovenous malformation (CM-AVM) syndrome shares histologic features of evolving AVMs as opposed to classic port wine birthmarks.”
The most recent International Society for the Study of Vascular Anomalies Classification of Vascular Anomalies was published in 2018 and is currently being updated. Other proposed clinical classifications have been published, including one that is diagnosis-specific and includes 20 different types of capillary malformations (J Eur Acad Dermatol Venereol. 2015 29[12]:2295-305, Pediatr Dermatol. 2016;33[6]:570-84).
“There are also syndromic classifications. Another question relates to the role of genomics: Are we ready for a classification that’s based purely on genetic variants, or do we need to incorporate it into existing classifications?” Dr. Garzon said. “Novel testing technologies using cell-free DNA and digital droplet PCR may be used in the future to establish diagnoses.” Genetic variants are found within capillary malformations, and they tend to be associated with three major pathways: the RAS-MAPK/ERK pathway, the PI3K/Akt/mTOR pathway, and the G protein pathway.
The type of capillary malformation that dermatologists and pediatricians most commonly see is nevus simplex, which occurs in 20%-82% of neonates. Other terms used include angel’s kiss, stork bite, salmon patch, nevus flammeus simplex, fading vascular stain, medial telangiectatic nevus, and butterfly mark. “It’s important to differentiate this from a port wine birthmark,” Dr. Garzon said. “This can be challenging when the birthmark is a darker red color. I have cared for patients who were initially thought to have nevus simplex and later found to have Sturge-Weber syndrome.”
Typical locations of nevus simplex include the central forehead/glabella, eyelids, the nape of the neck, scalp (parietal and occipital), nose, lip area (including philtrum), and the back (lumbosacral area and upper back). Most lesions fade/disappear without treatment (J Am Acad Dermatol. 2020;63[5]:805-14). Rare genetic syndromes associated with exaggerated nevus simplex complex include macrocephaly-capillary malformation syndrome and Beckwith-Wiedemann syndrome, “which tells us that this is a heterogeneous group of patients,” she said.
Dr. Garzon added that it’s “incredibly common” to see an eczema flare occurring within a nevus simplex on the nape of the neck. These patients will have a patch of atopic dermatitis that doesn’t get better. “Beneath it is their nevus simplex,” she said. “Remind parents that even after treating the eczema, the pink patch is not going to go away” (Pediatr Rep. 2021;13[1]:131-4).
Meanwhile, the classic port wine birthmark is usually congenital, uniform, and darker red in color. It darkens with maturity and the pattern will correlate with embryonic vasculature. “I am very wary of acquired port wine lesions,” she added. “It’s been described with trauma-related lesions, but early morphea can also mimic a port wine birthmark. You will see this if you’re practicing pediatric dermatology.”
Nearly a decade ago researchers established a link between port wine birthmarks and genetic variants in the GNAQ gene. “We see this in GNA11 as well,” Dr. Garzon said. “These changes are found in isolated port wine stains, and in Sturge-Weber syndrome. We now know that GNAQ drives the formation of large blood vessels through angiopoietin-2,” she noted (Arterioscler Throm Vasc Biol. 2022;42[1]:e27-43).
In general, studies that have examined genotype-phenotype correlations have demonstrated that the classic port wine birthmark is associated with GNAQ while GNA11 variants can be associated with a more reticulated pattern. “But this is not as clearcut as it seems,” she said. Investigators of a recent study showed an association between hypertension and renal anomalies in patients with skin capillary malformations and mosaic GNAQ or GNA11 variants. “This is a new finding,” she said. “Investigators are working to understand this association.”
Port wine birthmarks with the highest risk of Sturge-Weber syndrome include those that involve the forehead, upper eyelid, the midline frontonasal area, the hemifacial area, and median sites. “Patients who have this should be evaluated at birth,” Dr. Garzon said. “You should not delay for 2 months. They should be evaluated by ophthalmology and neurology early.”
The other morphologies commonly seen are “geographic” well-demarcated capillary malformations, which are dark in color. These lesions can be seen in conditions that are associated with genetic variants in PIK3CA (PROS) and include classic Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal) syndrome, and CLAPO (capillary malformation of the lower lip, lymphatic malformation of the face and neck, asymmetry of the face and limbs, and partial or generalized overgrowth) syndrome.
“Reticulated stains are much more heterogeneous,” Dr. Garzon said. “They can be localized or widespread. When you see a patient with a widespread reticulated capillary malformation, think about diffuse capillary malformation with overgrowth (DCMO). This condition is clinically and genetically heterogenous with the affected tissue of some patients showing variants in GNA11 while others have variants in PIK3CA. Therefore, a thorough examination at presentation and long-term follow-up is very important.”
Dr. Garzon disclosed that she is a member of the executive board for the International Society for the Study of Vascular Anomalies.
AT SPD 2022
New EU guidelines: Individualize care for thyroid cancer in kids
Comprehensive evaluation, multidisciplinary care, individualized treatment, and ongoing follow-up are all key to the management of pediatric thyroid nodules and differentiated thyroid carcinoma (DTC), according to the first European guidelines for this rare disease.
The guidelines were recently published in the European Thyroid Journal.
Lead author Chantal A. Lebbink told this news organization one of the key takeaways for clinicians is that management of pediatric thyroid nodules and DTC is «challenging and cannot be captured in a one-size-fits-all model.”
She also underlined the need for a “multidisciplinary approach in pediatric thyroid cancer expertise centers.”
Above all, Ms. Lebbink, who is a PhD student in the department of pediatric endocrinology, Wilhelmina Children’s Hospital, Utrecht, the Netherlands, said that pediatric DTC “is not adult DTC in a small person; it has different genetics and a different clinical behavior.”
The authors noted that DTC may be a rare disease, but its worldwide incidence is rising. It has several histologic subtypes, although the “vast majority” of cases are papillary thyroid carcinoma.
Crucially, there are “important differences” between adult and pediatric DTC in terms of their clinical, molecular, and pathologic characteristics, with pediatric patients commonly presenting with more advanced disease with greater lymph node involvement, distant metastases, and multifocal disease.
“However, despite the aggressive presentation, the overall survival rates are excellent,” Ms. Lebbink said.
There are also differences in genetic alterations between adult and pediatric patients. RET-PTC and NTRK fusions are more common in pediatric patients, while mutations in BRAF V600E and RAS point mutations are less frequent.
First European guidelines on thyroid cancer, thyroid nodules in children
Despite these differences, and the existence of U.S. guidelines, until now there have been no European recommendations on the management of pediatric thyroid nodules and DTC.
The European Thyroid Association therefore convened a panel of experts in pediatric and adult endocrinology, pathology, endocrine surgery, nuclear medicine, clinical genetics, and oncology, and tasked them with looking at diagnostics and staging, treatment, and follow-up.
The 2015 American Thyroid Association pediatric guideline was used as framework for the European guideline, with the expert panel identifying areas of discordance and outstanding clinical questions (Thyroid. 2015 Jul;25[7]:716-59).
To answer these questions, they searched PubMed and identified 3,251 studies, of which 45 studies met the inclusion criteria. From this they developed a comprehensive set of recommendations. These include that a child with suspected or proven cancer be referred to an experienced multidisciplinary team and their likely benefit from higher- versus lower-intensity treatment be established.
In addition, children should undergo a preoperative evaluation, with neck palpation, comprehensive neck ultrasonography, and laboratory work-up as a minimum, with further testing suggested in case of a family history or extensive disease.
Total thyroidectomy is the recommended treatment, although the authors call for further studies to assess the impact of limited surgery, and they suggest that prophylactic central lymph node dissection be reserved for advanced cases.
Crucially, all children “should be operated on by high-volume pediatric thyroid cancer surgeons with experience in pediatric thyroid cancer and who are embedded in a center with expertise in the management of DTC,” they wrote.
RAI therapy recommended for all children, in contrast to ATA guidelines
Radioactive iodine (I-131) therapy is recommended for all children following total thyroidectomy, with treatment following an individual patient-based approach.
This differs slightly from the ATA guidelines, which recommend against radioactive iodine (RAI) therapy for children with low-risk differentiated thyroid cancer that is mostly confined to the thyroid (N0 or minimal N1a disease). A study presented at the recent 2022 annual meeting of the ATA found that such children who were spared RAI showed no increases in risk of remission compared with those who did receive it.
The ETA guidelines then go on to recommend that children should be followed up with thyroid-stimulating hormone monitoring and suppression to low-normal levels, as well as serum thyroglobulin measurement and neck ultrasound, although other imaging modalities are not recommended.
In children with persistent or recurrent cervical disease, “surgery or I-131 therapy are indicated depending on the size, tumor load, and degree of progression,” and the authors said that cases of radioactive refractory disease should be “thoroughly investigated.”
Patients should also be counseled on the risk of the late effects of treatment for DTC and undergo monitoring, with follow-up continued for at least 10 years. Any subsequent follow-up should be “the result of shared decision-making between the physician and the patient.”
Evidence for molecular testing is scarce
Ms. Lebbink said that developing the guidelines nevertheless revealed a series of gaps in current knowledge, notably that the evidence for molecular testing “and the clinical implications in the preoperative stage are scarce.”
Specifically, the “positive and negative predictive value of molecular testing in fine needle biopsy specimen for the presence of DTC in a thyroid nodule must be further investigated.»
She also said that there has been a shift towards less aggressive treatment, due to a reluctance to performed prophylactic central neck dissection, and to offer I-131 therapy after surgery.
“However, before less aggressive treatment could be recommended,” Ms. Lebbink said, “it first must be investigated if there are differences in outcomes,” such as recurrence rates, disease-free survival rates, and survival rates between patients who do and do not receive the treatments.
No funding was declared. One author has reported relationships with Sanofi, AstraZeneca, Bayer, and GE. No other relevant financial relationships were reported.
A version of this article first appeared on Medscape.com.
Comprehensive evaluation, multidisciplinary care, individualized treatment, and ongoing follow-up are all key to the management of pediatric thyroid nodules and differentiated thyroid carcinoma (DTC), according to the first European guidelines for this rare disease.
The guidelines were recently published in the European Thyroid Journal.
Lead author Chantal A. Lebbink told this news organization one of the key takeaways for clinicians is that management of pediatric thyroid nodules and DTC is «challenging and cannot be captured in a one-size-fits-all model.”
She also underlined the need for a “multidisciplinary approach in pediatric thyroid cancer expertise centers.”
Above all, Ms. Lebbink, who is a PhD student in the department of pediatric endocrinology, Wilhelmina Children’s Hospital, Utrecht, the Netherlands, said that pediatric DTC “is not adult DTC in a small person; it has different genetics and a different clinical behavior.”
The authors noted that DTC may be a rare disease, but its worldwide incidence is rising. It has several histologic subtypes, although the “vast majority” of cases are papillary thyroid carcinoma.
Crucially, there are “important differences” between adult and pediatric DTC in terms of their clinical, molecular, and pathologic characteristics, with pediatric patients commonly presenting with more advanced disease with greater lymph node involvement, distant metastases, and multifocal disease.
“However, despite the aggressive presentation, the overall survival rates are excellent,” Ms. Lebbink said.
There are also differences in genetic alterations between adult and pediatric patients. RET-PTC and NTRK fusions are more common in pediatric patients, while mutations in BRAF V600E and RAS point mutations are less frequent.
First European guidelines on thyroid cancer, thyroid nodules in children
Despite these differences, and the existence of U.S. guidelines, until now there have been no European recommendations on the management of pediatric thyroid nodules and DTC.
The European Thyroid Association therefore convened a panel of experts in pediatric and adult endocrinology, pathology, endocrine surgery, nuclear medicine, clinical genetics, and oncology, and tasked them with looking at diagnostics and staging, treatment, and follow-up.
The 2015 American Thyroid Association pediatric guideline was used as framework for the European guideline, with the expert panel identifying areas of discordance and outstanding clinical questions (Thyroid. 2015 Jul;25[7]:716-59).
To answer these questions, they searched PubMed and identified 3,251 studies, of which 45 studies met the inclusion criteria. From this they developed a comprehensive set of recommendations. These include that a child with suspected or proven cancer be referred to an experienced multidisciplinary team and their likely benefit from higher- versus lower-intensity treatment be established.
In addition, children should undergo a preoperative evaluation, with neck palpation, comprehensive neck ultrasonography, and laboratory work-up as a minimum, with further testing suggested in case of a family history or extensive disease.
Total thyroidectomy is the recommended treatment, although the authors call for further studies to assess the impact of limited surgery, and they suggest that prophylactic central lymph node dissection be reserved for advanced cases.
Crucially, all children “should be operated on by high-volume pediatric thyroid cancer surgeons with experience in pediatric thyroid cancer and who are embedded in a center with expertise in the management of DTC,” they wrote.
RAI therapy recommended for all children, in contrast to ATA guidelines
Radioactive iodine (I-131) therapy is recommended for all children following total thyroidectomy, with treatment following an individual patient-based approach.
This differs slightly from the ATA guidelines, which recommend against radioactive iodine (RAI) therapy for children with low-risk differentiated thyroid cancer that is mostly confined to the thyroid (N0 or minimal N1a disease). A study presented at the recent 2022 annual meeting of the ATA found that such children who were spared RAI showed no increases in risk of remission compared with those who did receive it.
The ETA guidelines then go on to recommend that children should be followed up with thyroid-stimulating hormone monitoring and suppression to low-normal levels, as well as serum thyroglobulin measurement and neck ultrasound, although other imaging modalities are not recommended.
In children with persistent or recurrent cervical disease, “surgery or I-131 therapy are indicated depending on the size, tumor load, and degree of progression,” and the authors said that cases of radioactive refractory disease should be “thoroughly investigated.”
Patients should also be counseled on the risk of the late effects of treatment for DTC and undergo monitoring, with follow-up continued for at least 10 years. Any subsequent follow-up should be “the result of shared decision-making between the physician and the patient.”
Evidence for molecular testing is scarce
Ms. Lebbink said that developing the guidelines nevertheless revealed a series of gaps in current knowledge, notably that the evidence for molecular testing “and the clinical implications in the preoperative stage are scarce.”
Specifically, the “positive and negative predictive value of molecular testing in fine needle biopsy specimen for the presence of DTC in a thyroid nodule must be further investigated.»
She also said that there has been a shift towards less aggressive treatment, due to a reluctance to performed prophylactic central neck dissection, and to offer I-131 therapy after surgery.
“However, before less aggressive treatment could be recommended,” Ms. Lebbink said, “it first must be investigated if there are differences in outcomes,” such as recurrence rates, disease-free survival rates, and survival rates between patients who do and do not receive the treatments.
No funding was declared. One author has reported relationships with Sanofi, AstraZeneca, Bayer, and GE. No other relevant financial relationships were reported.
A version of this article first appeared on Medscape.com.
Comprehensive evaluation, multidisciplinary care, individualized treatment, and ongoing follow-up are all key to the management of pediatric thyroid nodules and differentiated thyroid carcinoma (DTC), according to the first European guidelines for this rare disease.
The guidelines were recently published in the European Thyroid Journal.
Lead author Chantal A. Lebbink told this news organization one of the key takeaways for clinicians is that management of pediatric thyroid nodules and DTC is «challenging and cannot be captured in a one-size-fits-all model.”
She also underlined the need for a “multidisciplinary approach in pediatric thyroid cancer expertise centers.”
Above all, Ms. Lebbink, who is a PhD student in the department of pediatric endocrinology, Wilhelmina Children’s Hospital, Utrecht, the Netherlands, said that pediatric DTC “is not adult DTC in a small person; it has different genetics and a different clinical behavior.”
The authors noted that DTC may be a rare disease, but its worldwide incidence is rising. It has several histologic subtypes, although the “vast majority” of cases are papillary thyroid carcinoma.
Crucially, there are “important differences” between adult and pediatric DTC in terms of their clinical, molecular, and pathologic characteristics, with pediatric patients commonly presenting with more advanced disease with greater lymph node involvement, distant metastases, and multifocal disease.
“However, despite the aggressive presentation, the overall survival rates are excellent,” Ms. Lebbink said.
There are also differences in genetic alterations between adult and pediatric patients. RET-PTC and NTRK fusions are more common in pediatric patients, while mutations in BRAF V600E and RAS point mutations are less frequent.
First European guidelines on thyroid cancer, thyroid nodules in children
Despite these differences, and the existence of U.S. guidelines, until now there have been no European recommendations on the management of pediatric thyroid nodules and DTC.
The European Thyroid Association therefore convened a panel of experts in pediatric and adult endocrinology, pathology, endocrine surgery, nuclear medicine, clinical genetics, and oncology, and tasked them with looking at diagnostics and staging, treatment, and follow-up.
The 2015 American Thyroid Association pediatric guideline was used as framework for the European guideline, with the expert panel identifying areas of discordance and outstanding clinical questions (Thyroid. 2015 Jul;25[7]:716-59).
To answer these questions, they searched PubMed and identified 3,251 studies, of which 45 studies met the inclusion criteria. From this they developed a comprehensive set of recommendations. These include that a child with suspected or proven cancer be referred to an experienced multidisciplinary team and their likely benefit from higher- versus lower-intensity treatment be established.
In addition, children should undergo a preoperative evaluation, with neck palpation, comprehensive neck ultrasonography, and laboratory work-up as a minimum, with further testing suggested in case of a family history or extensive disease.
Total thyroidectomy is the recommended treatment, although the authors call for further studies to assess the impact of limited surgery, and they suggest that prophylactic central lymph node dissection be reserved for advanced cases.
Crucially, all children “should be operated on by high-volume pediatric thyroid cancer surgeons with experience in pediatric thyroid cancer and who are embedded in a center with expertise in the management of DTC,” they wrote.
RAI therapy recommended for all children, in contrast to ATA guidelines
Radioactive iodine (I-131) therapy is recommended for all children following total thyroidectomy, with treatment following an individual patient-based approach.
This differs slightly from the ATA guidelines, which recommend against radioactive iodine (RAI) therapy for children with low-risk differentiated thyroid cancer that is mostly confined to the thyroid (N0 or minimal N1a disease). A study presented at the recent 2022 annual meeting of the ATA found that such children who were spared RAI showed no increases in risk of remission compared with those who did receive it.
The ETA guidelines then go on to recommend that children should be followed up with thyroid-stimulating hormone monitoring and suppression to low-normal levels, as well as serum thyroglobulin measurement and neck ultrasound, although other imaging modalities are not recommended.
In children with persistent or recurrent cervical disease, “surgery or I-131 therapy are indicated depending on the size, tumor load, and degree of progression,” and the authors said that cases of radioactive refractory disease should be “thoroughly investigated.”
Patients should also be counseled on the risk of the late effects of treatment for DTC and undergo monitoring, with follow-up continued for at least 10 years. Any subsequent follow-up should be “the result of shared decision-making between the physician and the patient.”
Evidence for molecular testing is scarce
Ms. Lebbink said that developing the guidelines nevertheless revealed a series of gaps in current knowledge, notably that the evidence for molecular testing “and the clinical implications in the preoperative stage are scarce.”
Specifically, the “positive and negative predictive value of molecular testing in fine needle biopsy specimen for the presence of DTC in a thyroid nodule must be further investigated.»
She also said that there has been a shift towards less aggressive treatment, due to a reluctance to performed prophylactic central neck dissection, and to offer I-131 therapy after surgery.
“However, before less aggressive treatment could be recommended,” Ms. Lebbink said, “it first must be investigated if there are differences in outcomes,” such as recurrence rates, disease-free survival rates, and survival rates between patients who do and do not receive the treatments.
No funding was declared. One author has reported relationships with Sanofi, AstraZeneca, Bayer, and GE. No other relevant financial relationships were reported.
A version of this article first appeared on Medscape.com.
FROM THE EUROPEAN THYROID JOURNAL
Has the pandemic affected babies’ brain development?
There’s some good overall news in a large analysis that looked at whether a mother’s COVID-19 infection or birth during the pandemic could affect a baby’s brain development.
Researchers studied 21,419 infants who had neurodevelopmental screening during the pandemic (from January 2020 to January 2021) and compared them with babies born before the pandemic (2015-2019).
They found in an analysis of eight studies that, generally, brain development in infants ages 6-12 months old was not changed by COVID-19.
Communication skill scores lower than prepandemic
However, one area did see a significant difference when they looked at answers to the Ages and Stages Questionnaire, 3rd edition (ASQ-3): Scores were lower in communication skills.
Compared with the prepandemic babies, the pandemic group of babies was more likely to have communication impairment (odds were 1.7 times higher).
Additionally, mothers’ SARS-CoV-2 infection was not associated with significant differences in any neurodevelopment sector in offspring, with one exception: Odds were 3.5 times higher for fine motor impairment in the pandemic baby group.
The babies in this study were either exposed in the womb to the SARS-CoV-2 infection or screened during the pandemic regardless of whether they were exposed to the virus.
The study, led by Kamran Hessami, MD, with the Maternal Fetal Care Center at Boston Children’s Hospital and Harvard Medical School in Boston, was published in JAMA Network Open.
Potential reasons for lower communication skills
The study points to some factors of the pandemic that may be tied to impaired communication skills.
“Higher levels of COVID-19–related stress were reported for both mothers and fathers of infants aged 0-6 months and were associated with insensitive parenting practices, including decreased emotional responsiveness in only mothers, which could lessen the reciprocal exchanges that support language development in early childhood,” they write. “Additionally, opportunities to promote language and social development through new experiences outside the home, including visits with extended family and friends or attendance at a child care center, were lessened for many during the pandemic.”
Viviana M. Fajardo Martinez, MD, with neonatal/perinatal medicine at University of California, Los Angeles, Health, told this publication her team is also studying child development before and after the pandemic over a 3-year period, and delayed communication skills is something she is seeing in clinic there.
She says some parents have been concerned, saying their babies aren’t talking enough or are behind in vocabulary.
Babies can catch up after 12 months
One thing she tells parents is that babies who are a bit delayed at 12 months can catch up.
Up to 18 months, they can catch up, she said, adding that they can be reevaluated then for improvement. If, at that point, the baby is not catching up, “that’s when we refer for early intervention,” she said.
Dr. Martinez also tells parents concerned about their infant’s communication skills that it’s important to talk, read, and sing to their child. She said amid pandemic stress, corners may have been cut in asking children to use language skills.
For instance, if a child points to an apple, a stressed parent may just give the child the apple instead of asking the child to request it by name and repeat the word several times.
She also said a limitation of this study is the use of the ASQ-3 questionnaire, which is filled out by parents. Answers are subjective, she notes, and sometimes differ between one child’s two parents. The questionnaire was commonly used during the pandemic because a more objective, professional evaluation has been more difficult.
However, a measure like the Bayley Scales of Infant and Toddler Development Screening Test adds objectivity and will likely give a better picture as research progresses, Dr. Martinez said.
Some information missing
Andréane Lavallée, PhD, and Dani Dumitriu, MD, PhD, both with the department of pediatrics at Columbia University, New York, write in an invited commentary that the overall positive message of the study “should not make researchers complacent” and results should be viewed with caution.
They point out that the precise effects of this novel virus are still unclear and the age group and variables studied may not tell the whole story.
“It should be noted that this systematic review did not consider timing of exposure during pregnancy, maternal infection severity, or exposure to various SARS-CoV-2 variants – all factors that could eventually be proven to contribute to subtle adverse neurodevelopmental outcomes,” they write.
Additionally, past pandemics “such as the 1918 Spanish flu, 1964 rubella, and 2009 H1N1” have taught researchers to watch for increases in diagnoses such as autism spectrum disorder (ASD) and schizophrenia in subsequent years.
“ASD is generally diagnosed at age 3-5 years (and often not until early teens), while schizophrenia is generally diagnosed in mid-to-late 20s,” the editorialists point out. The authors agree and emphasize the need for long-term studies.
Authors report no relevant financial relationships. Editorialist Dr. Dumitriu reports grants from National Institute of Mental Health, the U.S. Centers for Disease Control and Prevention, and the W. K. Kellogg Foundation; and has received gift funds from Einhorn Collaborative during the conduct of the study to the Nurture Science Program, for which Dr Dumitriu serves as director. Dr. Dumitriu received personal fees from Medela outside the submitted work; and is the corresponding author for one of the studies (Shuffrey et al., 2022) included in the systematic review conducted by Dr. Hessami et al. Dr. Lavallée reports grants from the Canadian Institutes of Health Research. Dr. Martinez reports no relevant financial relationships.
There’s some good overall news in a large analysis that looked at whether a mother’s COVID-19 infection or birth during the pandemic could affect a baby’s brain development.
Researchers studied 21,419 infants who had neurodevelopmental screening during the pandemic (from January 2020 to January 2021) and compared them with babies born before the pandemic (2015-2019).
They found in an analysis of eight studies that, generally, brain development in infants ages 6-12 months old was not changed by COVID-19.
Communication skill scores lower than prepandemic
However, one area did see a significant difference when they looked at answers to the Ages and Stages Questionnaire, 3rd edition (ASQ-3): Scores were lower in communication skills.
Compared with the prepandemic babies, the pandemic group of babies was more likely to have communication impairment (odds were 1.7 times higher).
Additionally, mothers’ SARS-CoV-2 infection was not associated with significant differences in any neurodevelopment sector in offspring, with one exception: Odds were 3.5 times higher for fine motor impairment in the pandemic baby group.
The babies in this study were either exposed in the womb to the SARS-CoV-2 infection or screened during the pandemic regardless of whether they were exposed to the virus.
The study, led by Kamran Hessami, MD, with the Maternal Fetal Care Center at Boston Children’s Hospital and Harvard Medical School in Boston, was published in JAMA Network Open.
Potential reasons for lower communication skills
The study points to some factors of the pandemic that may be tied to impaired communication skills.
“Higher levels of COVID-19–related stress were reported for both mothers and fathers of infants aged 0-6 months and were associated with insensitive parenting practices, including decreased emotional responsiveness in only mothers, which could lessen the reciprocal exchanges that support language development in early childhood,” they write. “Additionally, opportunities to promote language and social development through new experiences outside the home, including visits with extended family and friends or attendance at a child care center, were lessened for many during the pandemic.”
Viviana M. Fajardo Martinez, MD, with neonatal/perinatal medicine at University of California, Los Angeles, Health, told this publication her team is also studying child development before and after the pandemic over a 3-year period, and delayed communication skills is something she is seeing in clinic there.
She says some parents have been concerned, saying their babies aren’t talking enough or are behind in vocabulary.
Babies can catch up after 12 months
One thing she tells parents is that babies who are a bit delayed at 12 months can catch up.
Up to 18 months, they can catch up, she said, adding that they can be reevaluated then for improvement. If, at that point, the baby is not catching up, “that’s when we refer for early intervention,” she said.
Dr. Martinez also tells parents concerned about their infant’s communication skills that it’s important to talk, read, and sing to their child. She said amid pandemic stress, corners may have been cut in asking children to use language skills.
For instance, if a child points to an apple, a stressed parent may just give the child the apple instead of asking the child to request it by name and repeat the word several times.
She also said a limitation of this study is the use of the ASQ-3 questionnaire, which is filled out by parents. Answers are subjective, she notes, and sometimes differ between one child’s two parents. The questionnaire was commonly used during the pandemic because a more objective, professional evaluation has been more difficult.
However, a measure like the Bayley Scales of Infant and Toddler Development Screening Test adds objectivity and will likely give a better picture as research progresses, Dr. Martinez said.
Some information missing
Andréane Lavallée, PhD, and Dani Dumitriu, MD, PhD, both with the department of pediatrics at Columbia University, New York, write in an invited commentary that the overall positive message of the study “should not make researchers complacent” and results should be viewed with caution.
They point out that the precise effects of this novel virus are still unclear and the age group and variables studied may not tell the whole story.
“It should be noted that this systematic review did not consider timing of exposure during pregnancy, maternal infection severity, or exposure to various SARS-CoV-2 variants – all factors that could eventually be proven to contribute to subtle adverse neurodevelopmental outcomes,” they write.
Additionally, past pandemics “such as the 1918 Spanish flu, 1964 rubella, and 2009 H1N1” have taught researchers to watch for increases in diagnoses such as autism spectrum disorder (ASD) and schizophrenia in subsequent years.
“ASD is generally diagnosed at age 3-5 years (and often not until early teens), while schizophrenia is generally diagnosed in mid-to-late 20s,” the editorialists point out. The authors agree and emphasize the need for long-term studies.
Authors report no relevant financial relationships. Editorialist Dr. Dumitriu reports grants from National Institute of Mental Health, the U.S. Centers for Disease Control and Prevention, and the W. K. Kellogg Foundation; and has received gift funds from Einhorn Collaborative during the conduct of the study to the Nurture Science Program, for which Dr Dumitriu serves as director. Dr. Dumitriu received personal fees from Medela outside the submitted work; and is the corresponding author for one of the studies (Shuffrey et al., 2022) included in the systematic review conducted by Dr. Hessami et al. Dr. Lavallée reports grants from the Canadian Institutes of Health Research. Dr. Martinez reports no relevant financial relationships.
There’s some good overall news in a large analysis that looked at whether a mother’s COVID-19 infection or birth during the pandemic could affect a baby’s brain development.
Researchers studied 21,419 infants who had neurodevelopmental screening during the pandemic (from January 2020 to January 2021) and compared them with babies born before the pandemic (2015-2019).
They found in an analysis of eight studies that, generally, brain development in infants ages 6-12 months old was not changed by COVID-19.
Communication skill scores lower than prepandemic
However, one area did see a significant difference when they looked at answers to the Ages and Stages Questionnaire, 3rd edition (ASQ-3): Scores were lower in communication skills.
Compared with the prepandemic babies, the pandemic group of babies was more likely to have communication impairment (odds were 1.7 times higher).
Additionally, mothers’ SARS-CoV-2 infection was not associated with significant differences in any neurodevelopment sector in offspring, with one exception: Odds were 3.5 times higher for fine motor impairment in the pandemic baby group.
The babies in this study were either exposed in the womb to the SARS-CoV-2 infection or screened during the pandemic regardless of whether they were exposed to the virus.
The study, led by Kamran Hessami, MD, with the Maternal Fetal Care Center at Boston Children’s Hospital and Harvard Medical School in Boston, was published in JAMA Network Open.
Potential reasons for lower communication skills
The study points to some factors of the pandemic that may be tied to impaired communication skills.
“Higher levels of COVID-19–related stress were reported for both mothers and fathers of infants aged 0-6 months and were associated with insensitive parenting practices, including decreased emotional responsiveness in only mothers, which could lessen the reciprocal exchanges that support language development in early childhood,” they write. “Additionally, opportunities to promote language and social development through new experiences outside the home, including visits with extended family and friends or attendance at a child care center, were lessened for many during the pandemic.”
Viviana M. Fajardo Martinez, MD, with neonatal/perinatal medicine at University of California, Los Angeles, Health, told this publication her team is also studying child development before and after the pandemic over a 3-year period, and delayed communication skills is something she is seeing in clinic there.
She says some parents have been concerned, saying their babies aren’t talking enough or are behind in vocabulary.
Babies can catch up after 12 months
One thing she tells parents is that babies who are a bit delayed at 12 months can catch up.
Up to 18 months, they can catch up, she said, adding that they can be reevaluated then for improvement. If, at that point, the baby is not catching up, “that’s when we refer for early intervention,” she said.
Dr. Martinez also tells parents concerned about their infant’s communication skills that it’s important to talk, read, and sing to their child. She said amid pandemic stress, corners may have been cut in asking children to use language skills.
For instance, if a child points to an apple, a stressed parent may just give the child the apple instead of asking the child to request it by name and repeat the word several times.
She also said a limitation of this study is the use of the ASQ-3 questionnaire, which is filled out by parents. Answers are subjective, she notes, and sometimes differ between one child’s two parents. The questionnaire was commonly used during the pandemic because a more objective, professional evaluation has been more difficult.
However, a measure like the Bayley Scales of Infant and Toddler Development Screening Test adds objectivity and will likely give a better picture as research progresses, Dr. Martinez said.
Some information missing
Andréane Lavallée, PhD, and Dani Dumitriu, MD, PhD, both with the department of pediatrics at Columbia University, New York, write in an invited commentary that the overall positive message of the study “should not make researchers complacent” and results should be viewed with caution.
They point out that the precise effects of this novel virus are still unclear and the age group and variables studied may not tell the whole story.
“It should be noted that this systematic review did not consider timing of exposure during pregnancy, maternal infection severity, or exposure to various SARS-CoV-2 variants – all factors that could eventually be proven to contribute to subtle adverse neurodevelopmental outcomes,” they write.
Additionally, past pandemics “such as the 1918 Spanish flu, 1964 rubella, and 2009 H1N1” have taught researchers to watch for increases in diagnoses such as autism spectrum disorder (ASD) and schizophrenia in subsequent years.
“ASD is generally diagnosed at age 3-5 years (and often not until early teens), while schizophrenia is generally diagnosed in mid-to-late 20s,” the editorialists point out. The authors agree and emphasize the need for long-term studies.
Authors report no relevant financial relationships. Editorialist Dr. Dumitriu reports grants from National Institute of Mental Health, the U.S. Centers for Disease Control and Prevention, and the W. K. Kellogg Foundation; and has received gift funds from Einhorn Collaborative during the conduct of the study to the Nurture Science Program, for which Dr Dumitriu serves as director. Dr. Dumitriu received personal fees from Medela outside the submitted work; and is the corresponding author for one of the studies (Shuffrey et al., 2022) included in the systematic review conducted by Dr. Hessami et al. Dr. Lavallée reports grants from the Canadian Institutes of Health Research. Dr. Martinez reports no relevant financial relationships.
FROM JAMA NETWORK OPEN
Children with low-risk thyroid cancer can skip radioactive iodine
MONTREAL – Pediatric patients with low-risk differentiated thyroid cancer (DTC) who are spared radioactive iodine (RAI) therapy show no increases in the risk of remission, compared with those who do receive it, supporting guidelines that recommend against use of RAI in such patients.
“In 2015, when the American Thyroid Association [ATA] created their pediatric guidelines [on RAI therapy in DTC], they were taking a leap of faith that these [pediatric DTC] patients would be able to achieve remission without RAI,” said first author Mya Bojarsky, Children’s Hospital of Philadelphia (CHOP), when presenting the findings at the American Thyroid Association annual meeting.
“This is the first study to validate those guidelines and support the sentiment that for ATA low-risk pediatric thyroid cancer patients, withholding RAI therapy is clinically beneficial as it reduces exposure to radiation while having no negative impact on remission,” she said.
Prior to 2015, thyroidectomy in combination with RAI was the standard treatment for DTC in pediatric patients. However, data showing that radiation exposure in children increases the risk of secondary hematologic malignancies by 51% and solid malignancies by 23% over a lifetime raised concerns and led to a push to change the treatment approach.
In response, the 2015 ATA pediatric guidelines recommended that patients not receive RAI for the treatment of DTC that was mostly confined to the thyroid (N0 or minimal N1a disease).
Senior author Andrew J. Bauer, MD, noted that, in addition to being the first study to confirm that withholding RAI in low-risk patients is associated with the same rate of achieving remission as patients treated with RAI, the study also endorses that assessments at 1 year can be reliable predictors of remission.
“For these patients, the 1-year mark post-initial treatment (thyroidectomy) is an early and accurate time point for initial assessment of remission, with increasing rates of remission with continued surveillance (at last clinical follow-up) of approximately 90% 2 years post initial treatment,” said Dr. Bauer, medical director, CHOP, and professor of pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia.
“This approach has recently been validated through a prospective study in adult patients,” he added. A large recent study of 730 patients, published in the New England Journal of Medicine, supported the omission of RAI in low-risk DTC in adults, showing that, compared with those who received RAI, the no-RAI group was noninferior in the occurrence of functional, structural, and biologic events at 3 years.
Safe to eliminate RAI therapy in low-risk DTC in children
With limited data on how or if the change in treatment had an impact on rates of remission in pediatric patients, Ms. Bojarsky and colleagues conducted a retrospective cohort study of patients under the age of 19 years with ATA low-risk DTC who had undergone a total thyroidectomy at CHOP between 2010 and 2020.
Overall, they identified 95 patients, including 50 who had been treated with RAI in addition to thyroidectomy and 45 who did not receive RAI. Among those who did receive RAI, 31 were treated prior to 2015, and 19 were treated after 2019.
For the study, remission was defined as having undetectable thyroglobulin levels as well as no evidence of disease by ultrasound, Ms. Bojarsky said.
“This is important to show, because we want to ensure that as we are reducing our RAI use in the pediatric population, we were not negatively impacting their ability to achieve remission,” she explained.
The percentage of low-risk pediatric patients with DTC treated with RAI had already dropped from 100% in 2010 down to 38% by 2015 when the guidelines were issued, and after a slight rise to 50% by 2018, the practice plummeted to 0% by 2020, the study shows.
In terms of remission, at 1 year post-treatment, 80% of patients who received RAI were in remission, and the rate was even slightly higher, at 84%, among those who did not receive RAI, for a difference that was not significant.
Further looking at disease status as of the last clinical evaluation, 90% in the group treated with RAI had no evidence of disease at a median of 4.9 years of follow-up, and the rate was 87% in the group not receiving RAI, which had a median of 2.7 years of follow-up.
“In ATA low-risk patients, there is no detriment in achieving remission if RAI therapy is withheld,” say investigators.
The median tumor size in the RAI group was larger (19.5 mm vs. 12.0 mm; P < .001), and the primary tumor was T1 in 44% of the RAI group but 82% in the no-RAI group (P < .001).
The lymph node status was N0 in 72% of those receiving RAI and 76% in the no RAI group, which was not significantly different.
The leading risk factors associated with treatment with RAI included larger primary tumor size (OR, 1.07; P = .003), lymph node metastasis (OR, 3.72; P = .036), and surgery pre-2015 (OR, 9.83; P < .001).
RAI administration, N1a disease, and surgery prior to 2015 were not independent risk factors for evidence of persistent disease or indeterminate status.
Ms. Bojarsky has reported no relevant financial relationships.
A version of this article first appeared on Medscape.com.
MONTREAL – Pediatric patients with low-risk differentiated thyroid cancer (DTC) who are spared radioactive iodine (RAI) therapy show no increases in the risk of remission, compared with those who do receive it, supporting guidelines that recommend against use of RAI in such patients.
“In 2015, when the American Thyroid Association [ATA] created their pediatric guidelines [on RAI therapy in DTC], they were taking a leap of faith that these [pediatric DTC] patients would be able to achieve remission without RAI,” said first author Mya Bojarsky, Children’s Hospital of Philadelphia (CHOP), when presenting the findings at the American Thyroid Association annual meeting.
“This is the first study to validate those guidelines and support the sentiment that for ATA low-risk pediatric thyroid cancer patients, withholding RAI therapy is clinically beneficial as it reduces exposure to radiation while having no negative impact on remission,” she said.
Prior to 2015, thyroidectomy in combination with RAI was the standard treatment for DTC in pediatric patients. However, data showing that radiation exposure in children increases the risk of secondary hematologic malignancies by 51% and solid malignancies by 23% over a lifetime raised concerns and led to a push to change the treatment approach.
In response, the 2015 ATA pediatric guidelines recommended that patients not receive RAI for the treatment of DTC that was mostly confined to the thyroid (N0 or minimal N1a disease).
Senior author Andrew J. Bauer, MD, noted that, in addition to being the first study to confirm that withholding RAI in low-risk patients is associated with the same rate of achieving remission as patients treated with RAI, the study also endorses that assessments at 1 year can be reliable predictors of remission.
“For these patients, the 1-year mark post-initial treatment (thyroidectomy) is an early and accurate time point for initial assessment of remission, with increasing rates of remission with continued surveillance (at last clinical follow-up) of approximately 90% 2 years post initial treatment,” said Dr. Bauer, medical director, CHOP, and professor of pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia.
“This approach has recently been validated through a prospective study in adult patients,” he added. A large recent study of 730 patients, published in the New England Journal of Medicine, supported the omission of RAI in low-risk DTC in adults, showing that, compared with those who received RAI, the no-RAI group was noninferior in the occurrence of functional, structural, and biologic events at 3 years.
Safe to eliminate RAI therapy in low-risk DTC in children
With limited data on how or if the change in treatment had an impact on rates of remission in pediatric patients, Ms. Bojarsky and colleagues conducted a retrospective cohort study of patients under the age of 19 years with ATA low-risk DTC who had undergone a total thyroidectomy at CHOP between 2010 and 2020.
Overall, they identified 95 patients, including 50 who had been treated with RAI in addition to thyroidectomy and 45 who did not receive RAI. Among those who did receive RAI, 31 were treated prior to 2015, and 19 were treated after 2019.
For the study, remission was defined as having undetectable thyroglobulin levels as well as no evidence of disease by ultrasound, Ms. Bojarsky said.
“This is important to show, because we want to ensure that as we are reducing our RAI use in the pediatric population, we were not negatively impacting their ability to achieve remission,” she explained.
The percentage of low-risk pediatric patients with DTC treated with RAI had already dropped from 100% in 2010 down to 38% by 2015 when the guidelines were issued, and after a slight rise to 50% by 2018, the practice plummeted to 0% by 2020, the study shows.
In terms of remission, at 1 year post-treatment, 80% of patients who received RAI were in remission, and the rate was even slightly higher, at 84%, among those who did not receive RAI, for a difference that was not significant.
Further looking at disease status as of the last clinical evaluation, 90% in the group treated with RAI had no evidence of disease at a median of 4.9 years of follow-up, and the rate was 87% in the group not receiving RAI, which had a median of 2.7 years of follow-up.
“In ATA low-risk patients, there is no detriment in achieving remission if RAI therapy is withheld,” say investigators.
The median tumor size in the RAI group was larger (19.5 mm vs. 12.0 mm; P < .001), and the primary tumor was T1 in 44% of the RAI group but 82% in the no-RAI group (P < .001).
The lymph node status was N0 in 72% of those receiving RAI and 76% in the no RAI group, which was not significantly different.
The leading risk factors associated with treatment with RAI included larger primary tumor size (OR, 1.07; P = .003), lymph node metastasis (OR, 3.72; P = .036), and surgery pre-2015 (OR, 9.83; P < .001).
RAI administration, N1a disease, and surgery prior to 2015 were not independent risk factors for evidence of persistent disease or indeterminate status.
Ms. Bojarsky has reported no relevant financial relationships.
A version of this article first appeared on Medscape.com.
MONTREAL – Pediatric patients with low-risk differentiated thyroid cancer (DTC) who are spared radioactive iodine (RAI) therapy show no increases in the risk of remission, compared with those who do receive it, supporting guidelines that recommend against use of RAI in such patients.
“In 2015, when the American Thyroid Association [ATA] created their pediatric guidelines [on RAI therapy in DTC], they were taking a leap of faith that these [pediatric DTC] patients would be able to achieve remission without RAI,” said first author Mya Bojarsky, Children’s Hospital of Philadelphia (CHOP), when presenting the findings at the American Thyroid Association annual meeting.
“This is the first study to validate those guidelines and support the sentiment that for ATA low-risk pediatric thyroid cancer patients, withholding RAI therapy is clinically beneficial as it reduces exposure to radiation while having no negative impact on remission,” she said.
Prior to 2015, thyroidectomy in combination with RAI was the standard treatment for DTC in pediatric patients. However, data showing that radiation exposure in children increases the risk of secondary hematologic malignancies by 51% and solid malignancies by 23% over a lifetime raised concerns and led to a push to change the treatment approach.
In response, the 2015 ATA pediatric guidelines recommended that patients not receive RAI for the treatment of DTC that was mostly confined to the thyroid (N0 or minimal N1a disease).
Senior author Andrew J. Bauer, MD, noted that, in addition to being the first study to confirm that withholding RAI in low-risk patients is associated with the same rate of achieving remission as patients treated with RAI, the study also endorses that assessments at 1 year can be reliable predictors of remission.
“For these patients, the 1-year mark post-initial treatment (thyroidectomy) is an early and accurate time point for initial assessment of remission, with increasing rates of remission with continued surveillance (at last clinical follow-up) of approximately 90% 2 years post initial treatment,” said Dr. Bauer, medical director, CHOP, and professor of pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia.
“This approach has recently been validated through a prospective study in adult patients,” he added. A large recent study of 730 patients, published in the New England Journal of Medicine, supported the omission of RAI in low-risk DTC in adults, showing that, compared with those who received RAI, the no-RAI group was noninferior in the occurrence of functional, structural, and biologic events at 3 years.
Safe to eliminate RAI therapy in low-risk DTC in children
With limited data on how or if the change in treatment had an impact on rates of remission in pediatric patients, Ms. Bojarsky and colleagues conducted a retrospective cohort study of patients under the age of 19 years with ATA low-risk DTC who had undergone a total thyroidectomy at CHOP between 2010 and 2020.
Overall, they identified 95 patients, including 50 who had been treated with RAI in addition to thyroidectomy and 45 who did not receive RAI. Among those who did receive RAI, 31 were treated prior to 2015, and 19 were treated after 2019.
For the study, remission was defined as having undetectable thyroglobulin levels as well as no evidence of disease by ultrasound, Ms. Bojarsky said.
“This is important to show, because we want to ensure that as we are reducing our RAI use in the pediatric population, we were not negatively impacting their ability to achieve remission,” she explained.
The percentage of low-risk pediatric patients with DTC treated with RAI had already dropped from 100% in 2010 down to 38% by 2015 when the guidelines were issued, and after a slight rise to 50% by 2018, the practice plummeted to 0% by 2020, the study shows.
In terms of remission, at 1 year post-treatment, 80% of patients who received RAI were in remission, and the rate was even slightly higher, at 84%, among those who did not receive RAI, for a difference that was not significant.
Further looking at disease status as of the last clinical evaluation, 90% in the group treated with RAI had no evidence of disease at a median of 4.9 years of follow-up, and the rate was 87% in the group not receiving RAI, which had a median of 2.7 years of follow-up.
“In ATA low-risk patients, there is no detriment in achieving remission if RAI therapy is withheld,” say investigators.
The median tumor size in the RAI group was larger (19.5 mm vs. 12.0 mm; P < .001), and the primary tumor was T1 in 44% of the RAI group but 82% in the no-RAI group (P < .001).
The lymph node status was N0 in 72% of those receiving RAI and 76% in the no RAI group, which was not significantly different.
The leading risk factors associated with treatment with RAI included larger primary tumor size (OR, 1.07; P = .003), lymph node metastasis (OR, 3.72; P = .036), and surgery pre-2015 (OR, 9.83; P < .001).
RAI administration, N1a disease, and surgery prior to 2015 were not independent risk factors for evidence of persistent disease or indeterminate status.
Ms. Bojarsky has reported no relevant financial relationships.
A version of this article first appeared on Medscape.com.
AT ATA 2022
In childhood sickle cell disease stroke prevention is key
CINCINNATI – Sickle cell disease is well known for its associated anemia, but patients experience a range of other complications as well. These include vision and kidney problems, delayed growth, susceptibility to infection, and pain.
Another issue, not always as well recognized, is a considerably heightened risk for childhood stroke. “, and there’s also an elevated risk of five times the general population in adults with sickle cell disease,” said Lori Jordan, MD, PhD, in an interview.
At the 2022 annual meeting of the Child Neurology Society, Dr. Jordan spoke about stroke as a complication of sickle cell disease, and the role that neurologists can play in preventing primary or secondary strokes. “At least in children, studies have shown that if we screen and identify patients who are at highest risk of stroke, there are primary prevention therapies – usually implemented by hematologists, but that neurologists often are involved with – both monitoring for cognitive effects of silent cerebral infarct and also with treating patients who unfortunately still have an acute stroke,” said Dr. Jordan, who is an associate professor of pediatrics, neurology, and radiology at Vanderbilt University Medical Center, Nashville, Tenn. She also is director of the pediatric stroke program at Vanderbilt.
Time is of the essence
“In general, stroke in children is rare, but it’s more common in sickle cell disease, so it’s really important for providers to know that stroke risk is higher in those patients, particularly in those children, and then identify it and treat it earlier. Time is of the essence, and if we can give them the same therapeutics that we give the general stroke population, then time really becomes a factor, so it’s important that people know that it’s an issue for this population,” said Eboni Lance, MD, PhD, who coordinated the session where Dr. Jordan spoke.
Sickle cell disease is caused by a double mutation in the gene encoding the hemoglobin gene, producing the altered sickle hemoglobin (hemoglobin S). The change causes the hemoglobin proteins to tend to stick to one another, which can lead red blood cells to adopt a sickle-like shape. The sickle-shaped blood cells in turn have a tendency to aggregate and can block blood flow or lead to endothelial injury. Symptoms of stroke in children can include hemiparesis, aphasia, and seizure, but they can also be silent.
If no preventive is employed, one in nine with sickle cell disease will experience a stroke by the age of 19. Cerebrovascular symptoms are the most frequent debilitating complication of the condition. Nearly 40% of patients with sickle cell disease will have a silent cerebral infarct by age 18, as will 50% by age 30. Silent strokes have been associated with worse educational attainment and a greater need for educational special services.
Factors contributing to stroke in children with sickle cell disease include anemia and a low blood oxygen count, reduced oxygen affinity of hemoglobin variant, and cerebral vasculopathy. An estimated 10%-15% of young adults with sickle cell disease have severe intracranial stenosis.
Primary and secondary stroke prevention strategies
The dire consequences of stroke in this patient population underline the importance of primary stroke prevention, which requires the use of transcranial Doppler (TCD) ultrasound. It has been validated as a tool to screen for initial stroke risk in children with no history of stroke. High velocity measured on TCD indicates a narrowed blood vessel or elevated blood that is compensating for anemia. It adds up to a “struggling brain,” said Dr. Jordan, during her talk. If the TCD ultrasound velocity is greater than 200 cm/sec (or 170 cm/sec, depending on nonimaging versus imaging TCD), the TWiTCH trial showed that seven monthly transfusions is the number needed to treat to prevent one stroke. After 1 year, patients can be switched from transfusions to hydroxyurea if the patient has no significant intracranial stenosis. Hydroxyurea boosts both fetal and total hemoglobin, and also counters inflammation.
Following an acute stroke or transient ischemic attack, patients should receive a transfusion within 2 hours of presenting in the health care setting. American Society of Hematology guidelines recommend exchange transfusion rather than a simple transfusion. A simple transfusion can be initiated if an exchange transfusion is not available within 2 hours and hemoglobin values are less than 8.5 g/dL, to be followed by performance of exchange transfusion when available.
For chronic secondary stroke prevention, transfusions should be performed approximately monthly with the goal of maintaining hemoglobin above 9 g/dL at all times, as well as suppressing hemoglobin S levels to 30% or less of total hemoglobin.
Sudden, severe headache is a potential harbinger of complications like aneurysm, which occurs 10-fold more often among patients with sickle cell disease than the general population. It could also indicate increased intracranial pressure or cerebral venous sinus thrombosis.
Treatment of acute headache in sickle cell disease should avoid use of triptans, since vasoconstriction can counter the increased cerebral blood flow that compensates for anemia. Gabapentin and amitriptyline are good treatment choices.
New-onset seizures are a potential sign of stroke or posterior reversible leukoencephalopathy (PRES) in patients with sickle cell disease. Urgent MRI should be considered for all new-onset seizures. If blood pressure is high, PRES may be present. Seizures may also be an indicator of a previous brain injury.
Dr. Jordan has no relevant financial disclosures. Dr. Lance has served on an advisory board for Novartis.
CINCINNATI – Sickle cell disease is well known for its associated anemia, but patients experience a range of other complications as well. These include vision and kidney problems, delayed growth, susceptibility to infection, and pain.
Another issue, not always as well recognized, is a considerably heightened risk for childhood stroke. “, and there’s also an elevated risk of five times the general population in adults with sickle cell disease,” said Lori Jordan, MD, PhD, in an interview.
At the 2022 annual meeting of the Child Neurology Society, Dr. Jordan spoke about stroke as a complication of sickle cell disease, and the role that neurologists can play in preventing primary or secondary strokes. “At least in children, studies have shown that if we screen and identify patients who are at highest risk of stroke, there are primary prevention therapies – usually implemented by hematologists, but that neurologists often are involved with – both monitoring for cognitive effects of silent cerebral infarct and also with treating patients who unfortunately still have an acute stroke,” said Dr. Jordan, who is an associate professor of pediatrics, neurology, and radiology at Vanderbilt University Medical Center, Nashville, Tenn. She also is director of the pediatric stroke program at Vanderbilt.
Time is of the essence
“In general, stroke in children is rare, but it’s more common in sickle cell disease, so it’s really important for providers to know that stroke risk is higher in those patients, particularly in those children, and then identify it and treat it earlier. Time is of the essence, and if we can give them the same therapeutics that we give the general stroke population, then time really becomes a factor, so it’s important that people know that it’s an issue for this population,” said Eboni Lance, MD, PhD, who coordinated the session where Dr. Jordan spoke.
Sickle cell disease is caused by a double mutation in the gene encoding the hemoglobin gene, producing the altered sickle hemoglobin (hemoglobin S). The change causes the hemoglobin proteins to tend to stick to one another, which can lead red blood cells to adopt a sickle-like shape. The sickle-shaped blood cells in turn have a tendency to aggregate and can block blood flow or lead to endothelial injury. Symptoms of stroke in children can include hemiparesis, aphasia, and seizure, but they can also be silent.
If no preventive is employed, one in nine with sickle cell disease will experience a stroke by the age of 19. Cerebrovascular symptoms are the most frequent debilitating complication of the condition. Nearly 40% of patients with sickle cell disease will have a silent cerebral infarct by age 18, as will 50% by age 30. Silent strokes have been associated with worse educational attainment and a greater need for educational special services.
Factors contributing to stroke in children with sickle cell disease include anemia and a low blood oxygen count, reduced oxygen affinity of hemoglobin variant, and cerebral vasculopathy. An estimated 10%-15% of young adults with sickle cell disease have severe intracranial stenosis.
Primary and secondary stroke prevention strategies
The dire consequences of stroke in this patient population underline the importance of primary stroke prevention, which requires the use of transcranial Doppler (TCD) ultrasound. It has been validated as a tool to screen for initial stroke risk in children with no history of stroke. High velocity measured on TCD indicates a narrowed blood vessel or elevated blood that is compensating for anemia. It adds up to a “struggling brain,” said Dr. Jordan, during her talk. If the TCD ultrasound velocity is greater than 200 cm/sec (or 170 cm/sec, depending on nonimaging versus imaging TCD), the TWiTCH trial showed that seven monthly transfusions is the number needed to treat to prevent one stroke. After 1 year, patients can be switched from transfusions to hydroxyurea if the patient has no significant intracranial stenosis. Hydroxyurea boosts both fetal and total hemoglobin, and also counters inflammation.
Following an acute stroke or transient ischemic attack, patients should receive a transfusion within 2 hours of presenting in the health care setting. American Society of Hematology guidelines recommend exchange transfusion rather than a simple transfusion. A simple transfusion can be initiated if an exchange transfusion is not available within 2 hours and hemoglobin values are less than 8.5 g/dL, to be followed by performance of exchange transfusion when available.
For chronic secondary stroke prevention, transfusions should be performed approximately monthly with the goal of maintaining hemoglobin above 9 g/dL at all times, as well as suppressing hemoglobin S levels to 30% or less of total hemoglobin.
Sudden, severe headache is a potential harbinger of complications like aneurysm, which occurs 10-fold more often among patients with sickle cell disease than the general population. It could also indicate increased intracranial pressure or cerebral venous sinus thrombosis.
Treatment of acute headache in sickle cell disease should avoid use of triptans, since vasoconstriction can counter the increased cerebral blood flow that compensates for anemia. Gabapentin and amitriptyline are good treatment choices.
New-onset seizures are a potential sign of stroke or posterior reversible leukoencephalopathy (PRES) in patients with sickle cell disease. Urgent MRI should be considered for all new-onset seizures. If blood pressure is high, PRES may be present. Seizures may also be an indicator of a previous brain injury.
Dr. Jordan has no relevant financial disclosures. Dr. Lance has served on an advisory board for Novartis.
CINCINNATI – Sickle cell disease is well known for its associated anemia, but patients experience a range of other complications as well. These include vision and kidney problems, delayed growth, susceptibility to infection, and pain.
Another issue, not always as well recognized, is a considerably heightened risk for childhood stroke. “, and there’s also an elevated risk of five times the general population in adults with sickle cell disease,” said Lori Jordan, MD, PhD, in an interview.
At the 2022 annual meeting of the Child Neurology Society, Dr. Jordan spoke about stroke as a complication of sickle cell disease, and the role that neurologists can play in preventing primary or secondary strokes. “At least in children, studies have shown that if we screen and identify patients who are at highest risk of stroke, there are primary prevention therapies – usually implemented by hematologists, but that neurologists often are involved with – both monitoring for cognitive effects of silent cerebral infarct and also with treating patients who unfortunately still have an acute stroke,” said Dr. Jordan, who is an associate professor of pediatrics, neurology, and radiology at Vanderbilt University Medical Center, Nashville, Tenn. She also is director of the pediatric stroke program at Vanderbilt.
Time is of the essence
“In general, stroke in children is rare, but it’s more common in sickle cell disease, so it’s really important for providers to know that stroke risk is higher in those patients, particularly in those children, and then identify it and treat it earlier. Time is of the essence, and if we can give them the same therapeutics that we give the general stroke population, then time really becomes a factor, so it’s important that people know that it’s an issue for this population,” said Eboni Lance, MD, PhD, who coordinated the session where Dr. Jordan spoke.
Sickle cell disease is caused by a double mutation in the gene encoding the hemoglobin gene, producing the altered sickle hemoglobin (hemoglobin S). The change causes the hemoglobin proteins to tend to stick to one another, which can lead red blood cells to adopt a sickle-like shape. The sickle-shaped blood cells in turn have a tendency to aggregate and can block blood flow or lead to endothelial injury. Symptoms of stroke in children can include hemiparesis, aphasia, and seizure, but they can also be silent.
If no preventive is employed, one in nine with sickle cell disease will experience a stroke by the age of 19. Cerebrovascular symptoms are the most frequent debilitating complication of the condition. Nearly 40% of patients with sickle cell disease will have a silent cerebral infarct by age 18, as will 50% by age 30. Silent strokes have been associated with worse educational attainment and a greater need for educational special services.
Factors contributing to stroke in children with sickle cell disease include anemia and a low blood oxygen count, reduced oxygen affinity of hemoglobin variant, and cerebral vasculopathy. An estimated 10%-15% of young adults with sickle cell disease have severe intracranial stenosis.
Primary and secondary stroke prevention strategies
The dire consequences of stroke in this patient population underline the importance of primary stroke prevention, which requires the use of transcranial Doppler (TCD) ultrasound. It has been validated as a tool to screen for initial stroke risk in children with no history of stroke. High velocity measured on TCD indicates a narrowed blood vessel or elevated blood that is compensating for anemia. It adds up to a “struggling brain,” said Dr. Jordan, during her talk. If the TCD ultrasound velocity is greater than 200 cm/sec (or 170 cm/sec, depending on nonimaging versus imaging TCD), the TWiTCH trial showed that seven monthly transfusions is the number needed to treat to prevent one stroke. After 1 year, patients can be switched from transfusions to hydroxyurea if the patient has no significant intracranial stenosis. Hydroxyurea boosts both fetal and total hemoglobin, and also counters inflammation.
Following an acute stroke or transient ischemic attack, patients should receive a transfusion within 2 hours of presenting in the health care setting. American Society of Hematology guidelines recommend exchange transfusion rather than a simple transfusion. A simple transfusion can be initiated if an exchange transfusion is not available within 2 hours and hemoglobin values are less than 8.5 g/dL, to be followed by performance of exchange transfusion when available.
For chronic secondary stroke prevention, transfusions should be performed approximately monthly with the goal of maintaining hemoglobin above 9 g/dL at all times, as well as suppressing hemoglobin S levels to 30% or less of total hemoglobin.
Sudden, severe headache is a potential harbinger of complications like aneurysm, which occurs 10-fold more often among patients with sickle cell disease than the general population. It could also indicate increased intracranial pressure or cerebral venous sinus thrombosis.
Treatment of acute headache in sickle cell disease should avoid use of triptans, since vasoconstriction can counter the increased cerebral blood flow that compensates for anemia. Gabapentin and amitriptyline are good treatment choices.
New-onset seizures are a potential sign of stroke or posterior reversible leukoencephalopathy (PRES) in patients with sickle cell disease. Urgent MRI should be considered for all new-onset seizures. If blood pressure is high, PRES may be present. Seizures may also be an indicator of a previous brain injury.
Dr. Jordan has no relevant financial disclosures. Dr. Lance has served on an advisory board for Novartis.
FROM CNS 2022
Remote assessment of atopic dermatitis is feasible with patient-provided images: Study
MONTREAL – , as well as the possibility of conducting remote clinical trials that would be less expensive and less burdensome for participants, according to investigators, who presented the study at the annual meeting of the International Society of Atopic Dermatitis.
Still, practical barriers need to be addressed, particularly the problem of image quality, noted study investigator Aviël Ragamin, MD, from the department of dermatology, Erasmus MC, University Medical Center, Rotterdam, the Netherlands.
“Good-quality images are crucial, [and] in our study, patients didn’t have any incentive to provide images because they had already received their medical consultation,” he explained. He suggested that this problem could be overcome by providing technical support for patients and compensation for trial participants.
The study included 87 children (median age, 7 years), who were assessed for AD severity at an academic outpatient clinic. The in-person visit included assessment with the Eczema Area and Severity Index (EASI) score, as well as the collection of whole-body clinical images. Parents were then asked to return home and to provide their own clinical images and self-administered EASI assessments of their child for comparison. Four raters were asked to rate all images twice and to compare in-clinic and self-administered EASI scores based on the images.
At the in-clinic visit, the median EASI score of the group was 8.8. The majority of patients had moderate (46.6%) or severe (14.8%) AD. Roughly 40% of the patients had darker skin (Fitzpatrick skin types IV–VI).
Using Spearman rank correlation of 1,534 in-clinic and 425 patient-provided images, the study found good inter- and intra-rater reliability for clinical image assessment and strong agreement between images and the in-clinic EASI scores. The top outliers in the assessment were individuals with either darker skin or significant postinflammatory hyperpigmentation, which are “the most difficult cases to rate, based on images,” Dr. Ragamin noted.
There was only moderate correlation between the in-clinic and self-administered EASI scores, with a significant number of patients either underestimating or overestimating their AD severity, he added.
Overall, the main problem with remote assessment seems to be the feasibility of patients providing images, said Dr. Ragamin. Only 36.8% of parents provided any images at all, and of these, 1 of 5 were deemed too blurry, leaving just 13 for final assessment, he explained.
“Pragmatically, it’s tricky,” said Aaron Drucker, MD, a dermatologist at Women’s College Hospital and associate professor at the University of Toronto, who was asked to comment on the study. “It takes long enough to do an EASI score in person, let alone looking through blurry pictures that take too long to load into your electronic medical record. We know it works, but when our hospital went virtual [during the COVID pandemic] ... most of my patients with chronic eczema weren’t even sending me pictures.”
Regarding the utility of remote, full-body photography in clinical practice, he said, “There’s too many feasibility hoops to jump through at this point. The most promise I see is for clinical trials, where it’s hard to get people to come in.”
Dr. Ragamin and Dr. Drucker have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.
MONTREAL – , as well as the possibility of conducting remote clinical trials that would be less expensive and less burdensome for participants, according to investigators, who presented the study at the annual meeting of the International Society of Atopic Dermatitis.
Still, practical barriers need to be addressed, particularly the problem of image quality, noted study investigator Aviël Ragamin, MD, from the department of dermatology, Erasmus MC, University Medical Center, Rotterdam, the Netherlands.
“Good-quality images are crucial, [and] in our study, patients didn’t have any incentive to provide images because they had already received their medical consultation,” he explained. He suggested that this problem could be overcome by providing technical support for patients and compensation for trial participants.
The study included 87 children (median age, 7 years), who were assessed for AD severity at an academic outpatient clinic. The in-person visit included assessment with the Eczema Area and Severity Index (EASI) score, as well as the collection of whole-body clinical images. Parents were then asked to return home and to provide their own clinical images and self-administered EASI assessments of their child for comparison. Four raters were asked to rate all images twice and to compare in-clinic and self-administered EASI scores based on the images.
At the in-clinic visit, the median EASI score of the group was 8.8. The majority of patients had moderate (46.6%) or severe (14.8%) AD. Roughly 40% of the patients had darker skin (Fitzpatrick skin types IV–VI).
Using Spearman rank correlation of 1,534 in-clinic and 425 patient-provided images, the study found good inter- and intra-rater reliability for clinical image assessment and strong agreement between images and the in-clinic EASI scores. The top outliers in the assessment were individuals with either darker skin or significant postinflammatory hyperpigmentation, which are “the most difficult cases to rate, based on images,” Dr. Ragamin noted.
There was only moderate correlation between the in-clinic and self-administered EASI scores, with a significant number of patients either underestimating or overestimating their AD severity, he added.
Overall, the main problem with remote assessment seems to be the feasibility of patients providing images, said Dr. Ragamin. Only 36.8% of parents provided any images at all, and of these, 1 of 5 were deemed too blurry, leaving just 13 for final assessment, he explained.
“Pragmatically, it’s tricky,” said Aaron Drucker, MD, a dermatologist at Women’s College Hospital and associate professor at the University of Toronto, who was asked to comment on the study. “It takes long enough to do an EASI score in person, let alone looking through blurry pictures that take too long to load into your electronic medical record. We know it works, but when our hospital went virtual [during the COVID pandemic] ... most of my patients with chronic eczema weren’t even sending me pictures.”
Regarding the utility of remote, full-body photography in clinical practice, he said, “There’s too many feasibility hoops to jump through at this point. The most promise I see is for clinical trials, where it’s hard to get people to come in.”
Dr. Ragamin and Dr. Drucker have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.
MONTREAL – , as well as the possibility of conducting remote clinical trials that would be less expensive and less burdensome for participants, according to investigators, who presented the study at the annual meeting of the International Society of Atopic Dermatitis.
Still, practical barriers need to be addressed, particularly the problem of image quality, noted study investigator Aviël Ragamin, MD, from the department of dermatology, Erasmus MC, University Medical Center, Rotterdam, the Netherlands.
“Good-quality images are crucial, [and] in our study, patients didn’t have any incentive to provide images because they had already received their medical consultation,” he explained. He suggested that this problem could be overcome by providing technical support for patients and compensation for trial participants.
The study included 87 children (median age, 7 years), who were assessed for AD severity at an academic outpatient clinic. The in-person visit included assessment with the Eczema Area and Severity Index (EASI) score, as well as the collection of whole-body clinical images. Parents were then asked to return home and to provide their own clinical images and self-administered EASI assessments of their child for comparison. Four raters were asked to rate all images twice and to compare in-clinic and self-administered EASI scores based on the images.
At the in-clinic visit, the median EASI score of the group was 8.8. The majority of patients had moderate (46.6%) or severe (14.8%) AD. Roughly 40% of the patients had darker skin (Fitzpatrick skin types IV–VI).
Using Spearman rank correlation of 1,534 in-clinic and 425 patient-provided images, the study found good inter- and intra-rater reliability for clinical image assessment and strong agreement between images and the in-clinic EASI scores. The top outliers in the assessment were individuals with either darker skin or significant postinflammatory hyperpigmentation, which are “the most difficult cases to rate, based on images,” Dr. Ragamin noted.
There was only moderate correlation between the in-clinic and self-administered EASI scores, with a significant number of patients either underestimating or overestimating their AD severity, he added.
Overall, the main problem with remote assessment seems to be the feasibility of patients providing images, said Dr. Ragamin. Only 36.8% of parents provided any images at all, and of these, 1 of 5 were deemed too blurry, leaving just 13 for final assessment, he explained.
“Pragmatically, it’s tricky,” said Aaron Drucker, MD, a dermatologist at Women’s College Hospital and associate professor at the University of Toronto, who was asked to comment on the study. “It takes long enough to do an EASI score in person, let alone looking through blurry pictures that take too long to load into your electronic medical record. We know it works, but when our hospital went virtual [during the COVID pandemic] ... most of my patients with chronic eczema weren’t even sending me pictures.”
Regarding the utility of remote, full-body photography in clinical practice, he said, “There’s too many feasibility hoops to jump through at this point. The most promise I see is for clinical trials, where it’s hard to get people to come in.”
Dr. Ragamin and Dr. Drucker have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.
Children and COVID: Weekly cases fall to lowest level in over a year
With the third autumn of the COVID era now upon us, the discussion has turned again to a possible influenza/COVID twindemic, as well as the new-for-2022 influenza/COVID/respiratory syncytial virus tripledemic. It appears, however, that COVID may have missed the memo.
For the sixth time in the last 7 weeks, the number of new COVID cases in children fell, with just under 23,000 reported during the week of Oct. 14-20, according to the American Academy of Pediatrics and the Children’s Hospital Association. That is the lowest weekly count so far this year, and the lowest since early July of 2021, just as the Delta surge was starting. New pediatric cases had dipped to 8,500, the lowest for any week during the pandemic, a couple of weeks before that, the AAP/CHA data show.
Weekly cases have fallen by almost 75% since over 90,000 were reported for the week of Aug. 26 to Sept. 1, even as children have returned to school and vaccine uptake remains slow in the youngest age groups. Rates of emergency department visits with diagnosed COVID also have continued to drop, as have new admissions, and both are nearing their 2021 lows, according to the Centers for Disease Control and Prevention.
New vaccinations in children under age 5 years were up slightly for the most recent week (Oct. 13-19), but total uptake for that age group is only 7.1% for an initial dose and 2.9% for full vaccination. Among children aged 5-11 years, 38.7% have received at least one dose and 31.6% have completed the primary series, with corresponding figures of 71.2% and 60.9% for those aged 12-17, the CDC said on its COVID Data Tracker.
Despite the low overall numbers, though, the youngest children are, in one respect, punching above their weight when it comes to vaccinations. In the 2 weeks from Oct. 6 to Oct. 19, children under 5 years of age, who represent 5.9% of the U.S. population, received 9.2% of the initial vaccine doses administered. Children aged 5-11 years, who represent 8.7% of the total population, got just 4.2% of all first doses over those same 2 weeks, while 12- to 17-year-olds, who make up 7.6% of the population, got 3.4% of the vaccine doses, the CDC reported.
On the vaccine-approval front, the Food and Drug Administration recently announced that the new bivalent COVID-19 vaccines are now included in the emergency use authorizations for children who have completed primary or booster vaccination. The Moderna vaccine is authorized as a single-dose booster for children as young as 6 years and the Pfizer-BioNTech vaccine can be given as a single booster dose in children as young as 5 years, the FDA said.
“These bivalent COVID-19 vaccines include an mRNA component of the original strain to provide an immune response that is broadly protective against COVID-19 and an mRNA component in common between the omicron variant BA.4 and BA.5 lineages,” the FDA said.
With the third autumn of the COVID era now upon us, the discussion has turned again to a possible influenza/COVID twindemic, as well as the new-for-2022 influenza/COVID/respiratory syncytial virus tripledemic. It appears, however, that COVID may have missed the memo.
For the sixth time in the last 7 weeks, the number of new COVID cases in children fell, with just under 23,000 reported during the week of Oct. 14-20, according to the American Academy of Pediatrics and the Children’s Hospital Association. That is the lowest weekly count so far this year, and the lowest since early July of 2021, just as the Delta surge was starting. New pediatric cases had dipped to 8,500, the lowest for any week during the pandemic, a couple of weeks before that, the AAP/CHA data show.
Weekly cases have fallen by almost 75% since over 90,000 were reported for the week of Aug. 26 to Sept. 1, even as children have returned to school and vaccine uptake remains slow in the youngest age groups. Rates of emergency department visits with diagnosed COVID also have continued to drop, as have new admissions, and both are nearing their 2021 lows, according to the Centers for Disease Control and Prevention.
New vaccinations in children under age 5 years were up slightly for the most recent week (Oct. 13-19), but total uptake for that age group is only 7.1% for an initial dose and 2.9% for full vaccination. Among children aged 5-11 years, 38.7% have received at least one dose and 31.6% have completed the primary series, with corresponding figures of 71.2% and 60.9% for those aged 12-17, the CDC said on its COVID Data Tracker.
Despite the low overall numbers, though, the youngest children are, in one respect, punching above their weight when it comes to vaccinations. In the 2 weeks from Oct. 6 to Oct. 19, children under 5 years of age, who represent 5.9% of the U.S. population, received 9.2% of the initial vaccine doses administered. Children aged 5-11 years, who represent 8.7% of the total population, got just 4.2% of all first doses over those same 2 weeks, while 12- to 17-year-olds, who make up 7.6% of the population, got 3.4% of the vaccine doses, the CDC reported.
On the vaccine-approval front, the Food and Drug Administration recently announced that the new bivalent COVID-19 vaccines are now included in the emergency use authorizations for children who have completed primary or booster vaccination. The Moderna vaccine is authorized as a single-dose booster for children as young as 6 years and the Pfizer-BioNTech vaccine can be given as a single booster dose in children as young as 5 years, the FDA said.
“These bivalent COVID-19 vaccines include an mRNA component of the original strain to provide an immune response that is broadly protective against COVID-19 and an mRNA component in common between the omicron variant BA.4 and BA.5 lineages,” the FDA said.
With the third autumn of the COVID era now upon us, the discussion has turned again to a possible influenza/COVID twindemic, as well as the new-for-2022 influenza/COVID/respiratory syncytial virus tripledemic. It appears, however, that COVID may have missed the memo.
For the sixth time in the last 7 weeks, the number of new COVID cases in children fell, with just under 23,000 reported during the week of Oct. 14-20, according to the American Academy of Pediatrics and the Children’s Hospital Association. That is the lowest weekly count so far this year, and the lowest since early July of 2021, just as the Delta surge was starting. New pediatric cases had dipped to 8,500, the lowest for any week during the pandemic, a couple of weeks before that, the AAP/CHA data show.
Weekly cases have fallen by almost 75% since over 90,000 were reported for the week of Aug. 26 to Sept. 1, even as children have returned to school and vaccine uptake remains slow in the youngest age groups. Rates of emergency department visits with diagnosed COVID also have continued to drop, as have new admissions, and both are nearing their 2021 lows, according to the Centers for Disease Control and Prevention.
New vaccinations in children under age 5 years were up slightly for the most recent week (Oct. 13-19), but total uptake for that age group is only 7.1% for an initial dose and 2.9% for full vaccination. Among children aged 5-11 years, 38.7% have received at least one dose and 31.6% have completed the primary series, with corresponding figures of 71.2% and 60.9% for those aged 12-17, the CDC said on its COVID Data Tracker.
Despite the low overall numbers, though, the youngest children are, in one respect, punching above their weight when it comes to vaccinations. In the 2 weeks from Oct. 6 to Oct. 19, children under 5 years of age, who represent 5.9% of the U.S. population, received 9.2% of the initial vaccine doses administered. Children aged 5-11 years, who represent 8.7% of the total population, got just 4.2% of all first doses over those same 2 weeks, while 12- to 17-year-olds, who make up 7.6% of the population, got 3.4% of the vaccine doses, the CDC reported.
On the vaccine-approval front, the Food and Drug Administration recently announced that the new bivalent COVID-19 vaccines are now included in the emergency use authorizations for children who have completed primary or booster vaccination. The Moderna vaccine is authorized as a single-dose booster for children as young as 6 years and the Pfizer-BioNTech vaccine can be given as a single booster dose in children as young as 5 years, the FDA said.
“These bivalent COVID-19 vaccines include an mRNA component of the original strain to provide an immune response that is broadly protective against COVID-19 and an mRNA component in common between the omicron variant BA.4 and BA.5 lineages,” the FDA said.
Sexual health care for disabled youth: Tough and getting tougher
The developmentally disabled girl was just 10 years old when Margaret Thew, DNP, medical director of adolescent medicine at Children’s Wisconsin, Milwaukee, helped care for her. Providing that care was not emotionally easy. “Her brother’s friend sexually assaulted her and impregnated her,” Dr. Thew said.
The girl was able to obtain an abortion, a decision her parents supported. The alternative could have been deadly. “She was a tiny little person and would not have been able to carry a fetus,” Dr. Thew, a nurse practitioner, said.
Dr. Thew said she’s thankful that tragic case occurred before 2022. After the United States Supreme Court overturned Roe v. Wade in June, Wisconsin reverted to an 1849 law banning abortion. Although the law is currently being challenged, Dr. Thew wonders how the situation would have played out now. (Weeks after the Supreme Court’s decision, a similar case occurred in Ohio. In that case, a 10-year-old girl had to travel out of the state to obtain an abortion after having been raped.)
Talking to adolescents and young adults about reproductive health, whether regarding an unexpected pregnancy, the need for contraception, or to provide information about sexual activity, can be a challenge even for experienced health care providers.
The talks, decisions, and care are particularly complex when patients have developmental and intellectual disabilities. Among the many factors, Dr. Thew said, are dealing with menstruation, finding the right contraceptives, and counseling parents who might not want to acknowledge their children’s emerging sexuality.
Statistics: How many?
Because the definitions of disabilities vary and they represent a spectrum, estimates for how many youth have intellectual or developmental disabilities range widely.
In 2019, the National Survey of Children’s Health found that 1 in 4 children and adolescents aged 12-17 years have special health care needs because of disability. The American Community Survey estimates more than 1.3 million people aged 16-20 have a disability.
Intellectual disabilities can occur when a person’s IQ is below 70, significantly impeding the ability to perform activities of daily living, such as eating, dressing, and communicating. Developmental disabilities are impairments in physical, learning, language, and behavior, according to the United States Centers for Disease Control and Prevention. Among the conditions are attention-deficit/hyperactivity disorder, autism spectrum disorders, fragile X syndrome, learning and language problems, spina bifida, and other conditions.
Addressing common issues, concerns
April Kayser is a health educator for the Multnomah County Health Department, Portland, Ore. In 2016, Ms. Kayser and other experts conducted interviews with 11 youth with developmental and intellectual disabilities and 34 support people, either parents or professionals who provide services. The survey was part of the SHEIDD Project – short for Sexual Health Equity for Individuals with Intellectual/Developmental Disabilities – at Oregon Health and Science University (OHSU).
From their findings, the researchers compiled guidelines. They provided scenarios that health care providers need to be aware of and that they need to be ready to address:
- A boy, 14, who is unclear about what to do when he feels sexually excited and wants to masturbate but isn’t at home. He has been told that masturbation is appropriate in private.
- A 20-year-old woman who lives in a group home is pregnant. She confesses to her parents during a visit that another resident is her boyfriend and that he is the father of the child she is expecting.
- A 17-year-old boy wants to ask out another student, who is 15.
Some developmentally and intellectually disabled youth can’t turn to their parents for help. One person in the survey said his father told him, “You don’t need to worry about any of that stuff. You’re too young.” Another said the job of a health care provider was to offer reproductive and sex education “to make sure you don’t screw up in some bad way.”
One finding stood out: Health care providers were at the top of the list of those whom young people trusted for information about reproductive and sexual health, Ms. Kayser said. Yet in her experience, she said, health care professionals are hesitant to bring up the issues with all youth, “especially those with intellectual and developmental disabilities.”
Health care providers often talk both to the patient and to the parents. Those conversations can be critical when a child is developmentally or intellectually disabled.
Women with disabilities have been shown to have a higher risk for adverse outcomes of pregnancy, said Willi Horner-Johnson, PhD, associate professor at OHSU–Portland State University School of Public Health.
In a recent study, she and her colleagues analyzed data from the CDC’s National Survey of Family Growth that included self-reported disability status. They found that the number of women with disabilities who give birth is far higher than was previously thought.
The researchers found that 19.5% of respondents who gave birth reported at least one sensory, cognitive, or mobility-related disability, a rate that is much greater than the less than 1%-6.6% estimates that are based on hospital discharge data.
Her group reported other troubling findings: Women with disabilities are twice as likely to have smoked during their pregnancy (19% vs. 8.9%) and are more likely to have preterm and low-birthweight babies.
Clinicians play an important role
Dr. Horner-Johnson agreed with the finding from the Multnomah County survey that health care providers play an important role in providing those with intellectual and developmental disabilities reproductive health care that meets their needs. “Clinicians need to be asking people with disabilities about their reproductive plans,” she said.
In the Multnomah County report, the researchers advised health care providers to recognize that people with disabilities are social and sexual beings; to learn about their goals, including those regarding sex and reproductive health; and to help youth build skills for healthy relationships and sexual activity.
Dr. Horner-Johnson pointed out that the American College of Obstetricians and Gynecologists “recommends that clinicians discuss reproductive plans at every visit, for example, by asking one key question – ‘Would you like to become pregnant in the next year?’ – of every woman of reproductive age.”
Some women will not be able to answer that question, and health care providers at times must rely on a caregiver for input. But many women, even those with disabilities, could answer if given a chance. She estimated that only about 5% of disabled people are unable to communicate. “Clinicians defer to the caregiver more than they need to,” she said.
Clinicians are becoming better at providing care to those with disabilities, Dr. Horner-Johnson said, yet they have a way to go. Clinician biases may prevent some from asking all women, including those with disabilities, about their reproductive plans. “Women with disabilities have described clinicians treating them as nonsexual, assuming or implying that they would not or should not get pregnant,” she writes in her report.
Such biases, she said, could be reduced by increased education of providers. A 2018 study in Health Equity found that only 19.3% of ob.gyns. said they felt equipped to manage the pregnancy of a woman with disabilities.
Managing sexuality and sexual health for youth with disabilities can be highly complex, according to Margaret Thew, DNP, medical director of adolescent medicine at Children’s Wisconsin, Milwaukee. Challenges include the following:
- Parents often can’t deal with the reality that their teen or young adult is sexually active or may become so. Parents she helps often prefer to use the term “hormones,” not contraceptives, when talking about pregnancy prevention.
- Menstruation is a frequent concern, especially for youth with severe disabilities. Some react strongly to seeing a sanitary pad with blood, for example, by throwing it. Parents worry that caregivers will balk at changing pads regularly. As a result, some parents want complete menstrual suppression, Dr. Thew said. The American Academy of Pediatrics outlines how to approach menstrual suppression through methods such as the use of estrogen-progestin, progesterone, a ring, or a patch. In late August, the American College of Obstetricians and Gynecologists released its clinical consensus on medical management of menstrual suppression.
- Some parents want to know how to obtain a complete hysterectomy for the patient – an option Dr. Thew and the AAP discourage. “We will tell them that’s not the best and safest approach, as you want to have the estrogen for bone health,” she said.
- After a discussion of all the options, an intrauterine device proves best for many. “That gives 7-8 years of protection,” she said, which is the approved effective duration for such devices. “They are less apt to have heavy monthly menstrual bleeding.”
- Parents of boys with disabilities, especially those with Down syndrome, often ask for sex education and guidance when sexual desires develop.
- Many parents want effective birth control for their children because of fear that their teen or young adult will be assaulted, a fear that isn’t groundless. Such cases are common, and caregivers frequently are the perpetrators.
Ms. Kayser, Dr. Horner-Johnson, and Dr. Thew have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.
The developmentally disabled girl was just 10 years old when Margaret Thew, DNP, medical director of adolescent medicine at Children’s Wisconsin, Milwaukee, helped care for her. Providing that care was not emotionally easy. “Her brother’s friend sexually assaulted her and impregnated her,” Dr. Thew said.
The girl was able to obtain an abortion, a decision her parents supported. The alternative could have been deadly. “She was a tiny little person and would not have been able to carry a fetus,” Dr. Thew, a nurse practitioner, said.
Dr. Thew said she’s thankful that tragic case occurred before 2022. After the United States Supreme Court overturned Roe v. Wade in June, Wisconsin reverted to an 1849 law banning abortion. Although the law is currently being challenged, Dr. Thew wonders how the situation would have played out now. (Weeks after the Supreme Court’s decision, a similar case occurred in Ohio. In that case, a 10-year-old girl had to travel out of the state to obtain an abortion after having been raped.)
Talking to adolescents and young adults about reproductive health, whether regarding an unexpected pregnancy, the need for contraception, or to provide information about sexual activity, can be a challenge even for experienced health care providers.
The talks, decisions, and care are particularly complex when patients have developmental and intellectual disabilities. Among the many factors, Dr. Thew said, are dealing with menstruation, finding the right contraceptives, and counseling parents who might not want to acknowledge their children’s emerging sexuality.
Statistics: How many?
Because the definitions of disabilities vary and they represent a spectrum, estimates for how many youth have intellectual or developmental disabilities range widely.
In 2019, the National Survey of Children’s Health found that 1 in 4 children and adolescents aged 12-17 years have special health care needs because of disability. The American Community Survey estimates more than 1.3 million people aged 16-20 have a disability.
Intellectual disabilities can occur when a person’s IQ is below 70, significantly impeding the ability to perform activities of daily living, such as eating, dressing, and communicating. Developmental disabilities are impairments in physical, learning, language, and behavior, according to the United States Centers for Disease Control and Prevention. Among the conditions are attention-deficit/hyperactivity disorder, autism spectrum disorders, fragile X syndrome, learning and language problems, spina bifida, and other conditions.
Addressing common issues, concerns
April Kayser is a health educator for the Multnomah County Health Department, Portland, Ore. In 2016, Ms. Kayser and other experts conducted interviews with 11 youth with developmental and intellectual disabilities and 34 support people, either parents or professionals who provide services. The survey was part of the SHEIDD Project – short for Sexual Health Equity for Individuals with Intellectual/Developmental Disabilities – at Oregon Health and Science University (OHSU).
From their findings, the researchers compiled guidelines. They provided scenarios that health care providers need to be aware of and that they need to be ready to address:
- A boy, 14, who is unclear about what to do when he feels sexually excited and wants to masturbate but isn’t at home. He has been told that masturbation is appropriate in private.
- A 20-year-old woman who lives in a group home is pregnant. She confesses to her parents during a visit that another resident is her boyfriend and that he is the father of the child she is expecting.
- A 17-year-old boy wants to ask out another student, who is 15.
Some developmentally and intellectually disabled youth can’t turn to their parents for help. One person in the survey said his father told him, “You don’t need to worry about any of that stuff. You’re too young.” Another said the job of a health care provider was to offer reproductive and sex education “to make sure you don’t screw up in some bad way.”
One finding stood out: Health care providers were at the top of the list of those whom young people trusted for information about reproductive and sexual health, Ms. Kayser said. Yet in her experience, she said, health care professionals are hesitant to bring up the issues with all youth, “especially those with intellectual and developmental disabilities.”
Health care providers often talk both to the patient and to the parents. Those conversations can be critical when a child is developmentally or intellectually disabled.
Women with disabilities have been shown to have a higher risk for adverse outcomes of pregnancy, said Willi Horner-Johnson, PhD, associate professor at OHSU–Portland State University School of Public Health.
In a recent study, she and her colleagues analyzed data from the CDC’s National Survey of Family Growth that included self-reported disability status. They found that the number of women with disabilities who give birth is far higher than was previously thought.
The researchers found that 19.5% of respondents who gave birth reported at least one sensory, cognitive, or mobility-related disability, a rate that is much greater than the less than 1%-6.6% estimates that are based on hospital discharge data.
Her group reported other troubling findings: Women with disabilities are twice as likely to have smoked during their pregnancy (19% vs. 8.9%) and are more likely to have preterm and low-birthweight babies.
Clinicians play an important role
Dr. Horner-Johnson agreed with the finding from the Multnomah County survey that health care providers play an important role in providing those with intellectual and developmental disabilities reproductive health care that meets their needs. “Clinicians need to be asking people with disabilities about their reproductive plans,” she said.
In the Multnomah County report, the researchers advised health care providers to recognize that people with disabilities are social and sexual beings; to learn about their goals, including those regarding sex and reproductive health; and to help youth build skills for healthy relationships and sexual activity.
Dr. Horner-Johnson pointed out that the American College of Obstetricians and Gynecologists “recommends that clinicians discuss reproductive plans at every visit, for example, by asking one key question – ‘Would you like to become pregnant in the next year?’ – of every woman of reproductive age.”
Some women will not be able to answer that question, and health care providers at times must rely on a caregiver for input. But many women, even those with disabilities, could answer if given a chance. She estimated that only about 5% of disabled people are unable to communicate. “Clinicians defer to the caregiver more than they need to,” she said.
Clinicians are becoming better at providing care to those with disabilities, Dr. Horner-Johnson said, yet they have a way to go. Clinician biases may prevent some from asking all women, including those with disabilities, about their reproductive plans. “Women with disabilities have described clinicians treating them as nonsexual, assuming or implying that they would not or should not get pregnant,” she writes in her report.
Such biases, she said, could be reduced by increased education of providers. A 2018 study in Health Equity found that only 19.3% of ob.gyns. said they felt equipped to manage the pregnancy of a woman with disabilities.
Managing sexuality and sexual health for youth with disabilities can be highly complex, according to Margaret Thew, DNP, medical director of adolescent medicine at Children’s Wisconsin, Milwaukee. Challenges include the following:
- Parents often can’t deal with the reality that their teen or young adult is sexually active or may become so. Parents she helps often prefer to use the term “hormones,” not contraceptives, when talking about pregnancy prevention.
- Menstruation is a frequent concern, especially for youth with severe disabilities. Some react strongly to seeing a sanitary pad with blood, for example, by throwing it. Parents worry that caregivers will balk at changing pads regularly. As a result, some parents want complete menstrual suppression, Dr. Thew said. The American Academy of Pediatrics outlines how to approach menstrual suppression through methods such as the use of estrogen-progestin, progesterone, a ring, or a patch. In late August, the American College of Obstetricians and Gynecologists released its clinical consensus on medical management of menstrual suppression.
- Some parents want to know how to obtain a complete hysterectomy for the patient – an option Dr. Thew and the AAP discourage. “We will tell them that’s not the best and safest approach, as you want to have the estrogen for bone health,” she said.
- After a discussion of all the options, an intrauterine device proves best for many. “That gives 7-8 years of protection,” she said, which is the approved effective duration for such devices. “They are less apt to have heavy monthly menstrual bleeding.”
- Parents of boys with disabilities, especially those with Down syndrome, often ask for sex education and guidance when sexual desires develop.
- Many parents want effective birth control for their children because of fear that their teen or young adult will be assaulted, a fear that isn’t groundless. Such cases are common, and caregivers frequently are the perpetrators.
Ms. Kayser, Dr. Horner-Johnson, and Dr. Thew have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.
The developmentally disabled girl was just 10 years old when Margaret Thew, DNP, medical director of adolescent medicine at Children’s Wisconsin, Milwaukee, helped care for her. Providing that care was not emotionally easy. “Her brother’s friend sexually assaulted her and impregnated her,” Dr. Thew said.
The girl was able to obtain an abortion, a decision her parents supported. The alternative could have been deadly. “She was a tiny little person and would not have been able to carry a fetus,” Dr. Thew, a nurse practitioner, said.
Dr. Thew said she’s thankful that tragic case occurred before 2022. After the United States Supreme Court overturned Roe v. Wade in June, Wisconsin reverted to an 1849 law banning abortion. Although the law is currently being challenged, Dr. Thew wonders how the situation would have played out now. (Weeks after the Supreme Court’s decision, a similar case occurred in Ohio. In that case, a 10-year-old girl had to travel out of the state to obtain an abortion after having been raped.)
Talking to adolescents and young adults about reproductive health, whether regarding an unexpected pregnancy, the need for contraception, or to provide information about sexual activity, can be a challenge even for experienced health care providers.
The talks, decisions, and care are particularly complex when patients have developmental and intellectual disabilities. Among the many factors, Dr. Thew said, are dealing with menstruation, finding the right contraceptives, and counseling parents who might not want to acknowledge their children’s emerging sexuality.
Statistics: How many?
Because the definitions of disabilities vary and they represent a spectrum, estimates for how many youth have intellectual or developmental disabilities range widely.
In 2019, the National Survey of Children’s Health found that 1 in 4 children and adolescents aged 12-17 years have special health care needs because of disability. The American Community Survey estimates more than 1.3 million people aged 16-20 have a disability.
Intellectual disabilities can occur when a person’s IQ is below 70, significantly impeding the ability to perform activities of daily living, such as eating, dressing, and communicating. Developmental disabilities are impairments in physical, learning, language, and behavior, according to the United States Centers for Disease Control and Prevention. Among the conditions are attention-deficit/hyperactivity disorder, autism spectrum disorders, fragile X syndrome, learning and language problems, spina bifida, and other conditions.
Addressing common issues, concerns
April Kayser is a health educator for the Multnomah County Health Department, Portland, Ore. In 2016, Ms. Kayser and other experts conducted interviews with 11 youth with developmental and intellectual disabilities and 34 support people, either parents or professionals who provide services. The survey was part of the SHEIDD Project – short for Sexual Health Equity for Individuals with Intellectual/Developmental Disabilities – at Oregon Health and Science University (OHSU).
From their findings, the researchers compiled guidelines. They provided scenarios that health care providers need to be aware of and that they need to be ready to address:
- A boy, 14, who is unclear about what to do when he feels sexually excited and wants to masturbate but isn’t at home. He has been told that masturbation is appropriate in private.
- A 20-year-old woman who lives in a group home is pregnant. She confesses to her parents during a visit that another resident is her boyfriend and that he is the father of the child she is expecting.
- A 17-year-old boy wants to ask out another student, who is 15.
Some developmentally and intellectually disabled youth can’t turn to their parents for help. One person in the survey said his father told him, “You don’t need to worry about any of that stuff. You’re too young.” Another said the job of a health care provider was to offer reproductive and sex education “to make sure you don’t screw up in some bad way.”
One finding stood out: Health care providers were at the top of the list of those whom young people trusted for information about reproductive and sexual health, Ms. Kayser said. Yet in her experience, she said, health care professionals are hesitant to bring up the issues with all youth, “especially those with intellectual and developmental disabilities.”
Health care providers often talk both to the patient and to the parents. Those conversations can be critical when a child is developmentally or intellectually disabled.
Women with disabilities have been shown to have a higher risk for adverse outcomes of pregnancy, said Willi Horner-Johnson, PhD, associate professor at OHSU–Portland State University School of Public Health.
In a recent study, she and her colleagues analyzed data from the CDC’s National Survey of Family Growth that included self-reported disability status. They found that the number of women with disabilities who give birth is far higher than was previously thought.
The researchers found that 19.5% of respondents who gave birth reported at least one sensory, cognitive, or mobility-related disability, a rate that is much greater than the less than 1%-6.6% estimates that are based on hospital discharge data.
Her group reported other troubling findings: Women with disabilities are twice as likely to have smoked during their pregnancy (19% vs. 8.9%) and are more likely to have preterm and low-birthweight babies.
Clinicians play an important role
Dr. Horner-Johnson agreed with the finding from the Multnomah County survey that health care providers play an important role in providing those with intellectual and developmental disabilities reproductive health care that meets their needs. “Clinicians need to be asking people with disabilities about their reproductive plans,” she said.
In the Multnomah County report, the researchers advised health care providers to recognize that people with disabilities are social and sexual beings; to learn about their goals, including those regarding sex and reproductive health; and to help youth build skills for healthy relationships and sexual activity.
Dr. Horner-Johnson pointed out that the American College of Obstetricians and Gynecologists “recommends that clinicians discuss reproductive plans at every visit, for example, by asking one key question – ‘Would you like to become pregnant in the next year?’ – of every woman of reproductive age.”
Some women will not be able to answer that question, and health care providers at times must rely on a caregiver for input. But many women, even those with disabilities, could answer if given a chance. She estimated that only about 5% of disabled people are unable to communicate. “Clinicians defer to the caregiver more than they need to,” she said.
Clinicians are becoming better at providing care to those with disabilities, Dr. Horner-Johnson said, yet they have a way to go. Clinician biases may prevent some from asking all women, including those with disabilities, about their reproductive plans. “Women with disabilities have described clinicians treating them as nonsexual, assuming or implying that they would not or should not get pregnant,” she writes in her report.
Such biases, she said, could be reduced by increased education of providers. A 2018 study in Health Equity found that only 19.3% of ob.gyns. said they felt equipped to manage the pregnancy of a woman with disabilities.
Managing sexuality and sexual health for youth with disabilities can be highly complex, according to Margaret Thew, DNP, medical director of adolescent medicine at Children’s Wisconsin, Milwaukee. Challenges include the following:
- Parents often can’t deal with the reality that their teen or young adult is sexually active or may become so. Parents she helps often prefer to use the term “hormones,” not contraceptives, when talking about pregnancy prevention.
- Menstruation is a frequent concern, especially for youth with severe disabilities. Some react strongly to seeing a sanitary pad with blood, for example, by throwing it. Parents worry that caregivers will balk at changing pads regularly. As a result, some parents want complete menstrual suppression, Dr. Thew said. The American Academy of Pediatrics outlines how to approach menstrual suppression through methods such as the use of estrogen-progestin, progesterone, a ring, or a patch. In late August, the American College of Obstetricians and Gynecologists released its clinical consensus on medical management of menstrual suppression.
- Some parents want to know how to obtain a complete hysterectomy for the patient – an option Dr. Thew and the AAP discourage. “We will tell them that’s not the best and safest approach, as you want to have the estrogen for bone health,” she said.
- After a discussion of all the options, an intrauterine device proves best for many. “That gives 7-8 years of protection,” she said, which is the approved effective duration for such devices. “They are less apt to have heavy monthly menstrual bleeding.”
- Parents of boys with disabilities, especially those with Down syndrome, often ask for sex education and guidance when sexual desires develop.
- Many parents want effective birth control for their children because of fear that their teen or young adult will be assaulted, a fear that isn’t groundless. Such cases are common, and caregivers frequently are the perpetrators.
Ms. Kayser, Dr. Horner-Johnson, and Dr. Thew have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.