The Diagnosis: Incontinentia Pigmenti 

The patient was diagnosed clinically with the vesicular stage of incontinentia pigmenti (IP), a rare, X-linked dominant neuroectodermal dysplasia that usually is lethal in males. The genetic mutation has been identified in the IKBKG gene (inhibitor of nuclear factor κB; formally NEMO), which leads to a truncated and defective nuclear factor κB. Female infants survive and display characteristic findings on examination due to X-inactivation leading to mosaicism.¹ Worldwide, there are approximately 27.6 new cases of IP per year. Although it is heritable, the majority (65%-75%) of cases are due to sporadic mutations, with the remaining minority (25%-35%) representing familial disease.¹ 

Cutaneous findings of IP classically progress through 4 stages, though individual patients often do not develop the characteristic lesions of each of the 4 stages. The vesicular stage (stage 1) presented in our patient (quiz image). This stage presents within 2 weeks of birth in 90% of patients and typically disappears when the patient is approximately 4 months of age.^1-3 Although the clinical presentation is striking, it is essential to rule out herpes simplex virus infection, which can mimic vesicular IP. Localized herpes simplex virus is most commonly seen in clusters on the scalp and often is not present at birth. Alternatively, IP is most often seen on the extremities in bands or whorls of distribution along Blaschko lines,⁴ as in this patient. 

Stage 2 (the verrucous stage) presents with verrucous papules or pustules in a similar blaschkoid distribution. Areas previously involved in stage 1 are not always the same areas affected in stage 2. Approximately 70% of patients develop stage 2 lesions, usually at 2 to 6 weeks of age.^1-3 Erythema toxicum neonatorum presents in the first week of life with pustules often on the trunk or extremities, but these lesions are not confined to Blaschko lines, differentiating it from IP.⁴ 

The third stage (hyperpigmented stage) lends the disease its name and occurs in 90% to 95% of patients with IP. Linear and whorled hyperpigmentation develops in early infancy and can either persist or fade by adolescence.¹ Pustules and hyperpigmentation in transient neonatal pustular melanosis may be similar to this stage of IP, but the distribution is more variable and progression to other lesions is not seen.⁵ 

The fourth and final stage is the hypopigmented stage, whereby blaschkoid linear and whorled lines of hypopigmentation with or without both atrophy and alopecia develop in 75% of patients. This is the last finding, beginning in adolescence and often persisting into adulthood.¹ Goltz syndrome is another X-linked dominant disorder with features similar to IP. Verrucous and atrophic lesions along Blaschko lines are reminiscent of the second and fourth stages of IP but are differentiated in Goltz syndrome because they present concurrently rather than in sequential stages such as IP. Similar extracutaneous organs are affected such as the eyes, teeth, and nails; however, Goltz syndrome may be associated with more distinguishing systemic signs such as sweating and skeletal abnormalities.⁶ 

Given its unique appearance, physicians usually diagnose IP clinically after identification of characteristic linear lesions along the lines of Blaschko in an infant or neonate. Skin biopsy is confirmatory, which would differ depending on the stage of disease biopsied. The vesicular stage is characterized by eosinophilic spongiosis and is differentiated from other items on the histologic differential diagnosis by the presence of dyskeratosis.⁷ Genetic testing is available and should be performed along with a physical examination of the mother for counseling  purposes.¹ 

Proper diagnosis is critical because of the potential multisystem nature of the disease with implications for longitudinal care and prognosis in patients. As in other neurocutaneous disease, IP can affect the hair, nails, teeth, central nervous system, and eyes. All IP patients receive a referral to ophthalmology at the time of diagnosis for a dilated fundus examination, with repeat examinations every several months initially--every 3 months for a year, every 6 months from 1 to 3 years of age--and annually thereafter. Dental evaluation should occur at 6 months of age or whenever the first tooth erupts.¹ Mental retardation, seizures, and developmental delay can occur and usually are evident in the first year of life. Patients should have developmental milestones closely  monitored and be referred to appropriate specialists if signs or symptoms develop consistent with neurologic involvement.¹ 

The Diagnosis: Incontinentia Pigmenti 

The patient was diagnosed clinically with the vesicular stage of incontinentia pigmenti (IP), a rare, X-linked dominant neuroectodermal dysplasia that usually is lethal in males. The genetic mutation has been identified in the IKBKG gene (inhibitor of nuclear factor κB; formally NEMO), which leads to a truncated and defective nuclear factor κB. Female infants survive and display characteristic findings on examination due to X-inactivation leading to mosaicism.¹ Worldwide, there are approximately 27.6 new cases of IP per year. Although it is heritable, the majority (65%-75%) of cases are due to sporadic mutations, with the remaining minority (25%-35%) representing familial disease.¹ 

Cutaneous findings of IP classically progress through 4 stages, though individual patients often do not develop the characteristic lesions of each of the 4 stages. The vesicular stage (stage 1) presented in our patient (quiz image). This stage presents within 2 weeks of birth in 90% of patients and typically disappears when the patient is approximately 4 months of age.^1-3 Although the clinical presentation is striking, it is essential to rule out herpes simplex virus infection, which can mimic vesicular IP. Localized herpes simplex virus is most commonly seen in clusters on the scalp and often is not present at birth. Alternatively, IP is most often seen on the extremities in bands or whorls of distribution along Blaschko lines,⁴ as in this patient. 

Stage 2 (the verrucous stage) presents with verrucous papules or pustules in a similar blaschkoid distribution. Areas previously involved in stage 1 are not always the same areas affected in stage 2. Approximately 70% of patients develop stage 2 lesions, usually at 2 to 6 weeks of age.^1-3 Erythema toxicum neonatorum presents in the first week of life with pustules often on the trunk or extremities, but these lesions are not confined to Blaschko lines, differentiating it from IP.⁴ 

The third stage (hyperpigmented stage) lends the disease its name and occurs in 90% to 95% of patients with IP. Linear and whorled hyperpigmentation develops in early infancy and can either persist or fade by adolescence.¹ Pustules and hyperpigmentation in transient neonatal pustular melanosis may be similar to this stage of IP, but the distribution is more variable and progression to other lesions is not seen.⁵ 

The fourth and final stage is the hypopigmented stage, whereby blaschkoid linear and whorled lines of hypopigmentation with or without both atrophy and alopecia develop in 75% of patients. This is the last finding, beginning in adolescence and often persisting into adulthood.¹ Goltz syndrome is another X-linked dominant disorder with features similar to IP. Verrucous and atrophic lesions along Blaschko lines are reminiscent of the second and fourth stages of IP but are differentiated in Goltz syndrome because they present concurrently rather than in sequential stages such as IP. Similar extracutaneous organs are affected such as the eyes, teeth, and nails; however, Goltz syndrome may be associated with more distinguishing systemic signs such as sweating and skeletal abnormalities.⁶ 

Given its unique appearance, physicians usually diagnose IP clinically after identification of characteristic linear lesions along the lines of Blaschko in an infant or neonate. Skin biopsy is confirmatory, which would differ depending on the stage of disease biopsied. The vesicular stage is characterized by eosinophilic spongiosis and is differentiated from other items on the histologic differential diagnosis by the presence of dyskeratosis.⁷ Genetic testing is available and should be performed along with a physical examination of the mother for counseling  purposes.¹ 

Proper diagnosis is critical because of the potential multisystem nature of the disease with implications for longitudinal care and prognosis in patients. As in other neurocutaneous disease, IP can affect the hair, nails, teeth, central nervous system, and eyes. All IP patients receive a referral to ophthalmology at the time of diagnosis for a dilated fundus examination, with repeat examinations every several months initially--every 3 months for a year, every 6 months from 1 to 3 years of age--and annually thereafter. Dental evaluation should occur at 6 months of age or whenever the first tooth erupts.¹ Mental retardation, seizures, and developmental delay can occur and usually are evident in the first year of life. Patients should have developmental milestones closely  monitored and be referred to appropriate specialists if signs or symptoms develop consistent with neurologic involvement.¹ 

The Diagnosis: Incontinentia Pigmenti 

The patient was diagnosed clinically with the vesicular stage of incontinentia pigmenti (IP), a rare, X-linked dominant neuroectodermal dysplasia that usually is lethal in males. The genetic mutation has been identified in the IKBKG gene (inhibitor of nuclear factor κB; formally NEMO), which leads to a truncated and defective nuclear factor κB. Female infants survive and display characteristic findings on examination due to X-inactivation leading to mosaicism.¹ Worldwide, there are approximately 27.6 new cases of IP per year. Although it is heritable, the majority (65%-75%) of cases are due to sporadic mutations, with the remaining minority (25%-35%) representing familial disease.¹ 

Cutaneous findings of IP classically progress through 4 stages, though individual patients often do not develop the characteristic lesions of each of the 4 stages. The vesicular stage (stage 1) presented in our patient (quiz image). This stage presents within 2 weeks of birth in 90% of patients and typically disappears when the patient is approximately 4 months of age.^1-3 Although the clinical presentation is striking, it is essential to rule out herpes simplex virus infection, which can mimic vesicular IP. Localized herpes simplex virus is most commonly seen in clusters on the scalp and often is not present at birth. Alternatively, IP is most often seen on the extremities in bands or whorls of distribution along Blaschko lines,⁴ as in this patient. 

Stage 2 (the verrucous stage) presents with verrucous papules or pustules in a similar blaschkoid distribution. Areas previously involved in stage 1 are not always the same areas affected in stage 2. Approximately 70% of patients develop stage 2 lesions, usually at 2 to 6 weeks of age.^1-3 Erythema toxicum neonatorum presents in the first week of life with pustules often on the trunk or extremities, but these lesions are not confined to Blaschko lines, differentiating it from IP.⁴ 

The third stage (hyperpigmented stage) lends the disease its name and occurs in 90% to 95% of patients with IP. Linear and whorled hyperpigmentation develops in early infancy and can either persist or fade by adolescence.¹ Pustules and hyperpigmentation in transient neonatal pustular melanosis may be similar to this stage of IP, but the distribution is more variable and progression to other lesions is not seen.⁵ 

The fourth and final stage is the hypopigmented stage, whereby blaschkoid linear and whorled lines of hypopigmentation with or without both atrophy and alopecia develop in 75% of patients. This is the last finding, beginning in adolescence and often persisting into adulthood.¹ Goltz syndrome is another X-linked dominant disorder with features similar to IP. Verrucous and atrophic lesions along Blaschko lines are reminiscent of the second and fourth stages of IP but are differentiated in Goltz syndrome because they present concurrently rather than in sequential stages such as IP. Similar extracutaneous organs are affected such as the eyes, teeth, and nails; however, Goltz syndrome may be associated with more distinguishing systemic signs such as sweating and skeletal abnormalities.⁶ 

Given its unique appearance, physicians usually diagnose IP clinically after identification of characteristic linear lesions along the lines of Blaschko in an infant or neonate. Skin biopsy is confirmatory, which would differ depending on the stage of disease biopsied. The vesicular stage is characterized by eosinophilic spongiosis and is differentiated from other items on the histologic differential diagnosis by the presence of dyskeratosis.⁷ Genetic testing is available and should be performed along with a physical examination of the mother for counseling  purposes.¹ 

Proper diagnosis is critical because of the potential multisystem nature of the disease with implications for longitudinal care and prognosis in patients. As in other neurocutaneous disease, IP can affect the hair, nails, teeth, central nervous system, and eyes. All IP patients receive a referral to ophthalmology at the time of diagnosis for a dilated fundus examination, with repeat examinations every several months initially--every 3 months for a year, every 6 months from 1 to 3 years of age--and annually thereafter. Dental evaluation should occur at 6 months of age or whenever the first tooth erupts.¹ Mental retardation, seizures, and developmental delay can occur and usually are evident in the first year of life. Patients should have developmental milestones closely  monitored and be referred to appropriate specialists if signs or symptoms develop consistent with neurologic involvement.¹ 

On March 4, 2020, the Supreme Court of the United States (SCOTUS) will hear opening arguments for June Medical Services v. Russo. (Please note that this case was originally referred to as June Medical Services v. Gee. However, Secretary Rebekah Gee resigned from her position on January 31, 2020, and was replaced by Interim Secretary Stephen Russo.) The case will examine a Louisiana law (Louisiana Act 620, or LA 620), originally passed in 2014, that requires physicians to have hospital admitting privileges within 30 miles of where they provide abortion services.¹ When LA 620 was signed into law in 2014, 5 of Louisiana’s 6 abortion clinics would not have met the standards created by this legislation and would have been forced to close, potentially leaving the vast majority of women in Louisiana without access to an abortion provider, and disproportionately impacting poor and rural women. Prior to enactment of this law, physicians at these 5 clinics attempted to obtain admitting privileges, and all were denied. The denials occurred due to two main reasons—because the providers admitted too few patients each year to qualify for hospital privileges or simply because they provided abortion care.² Shortly after this legislation was signed into law, the Center for Reproductive Rights (CRR) challenged the law, citing the undue burden it created for patients attempting to access abortion care.
 

Prior case also considered question of hospital privileges for abortion providers

Interestingly, SCOTUS already has ruled on this very question. In 1992, the Court ruled in Planned Parenthood of Southeastern Pennsylvania v. Casey that it is unconstitutional for a state to create an “undue burden” on a woman’s right to abortion prior to fetal viability.³ And in 2016, when considering whether or not requiring abortion providers to obtain hospital privileges creates an undue burden in Whole Women’s Health (WWH) v. Hellerstedt, the Supreme Court’s answer was yes, it does. WWH, with legal aid from CRR, challenged Texas House Bill 2 (H.B. 2), which similar to LA 620, required abortion providers to have local admitting privileges. Based largely on the precedent set in Casey, SCOTUS ruled 5-3 in favor of WWH.

The Louisiana law currently in question was written and challenged in district court simultaneous to the Supreme Court’s review of WWH. The district court declared LA 620 invalid and permanently enjoined its enforcement, finding the law would “drastically burden women’s right to choose abortions.”⁴ However, the US Court of Appeals for the Fifth Circuit reviewed the case and overturned the district court decision, finding the lower court’s analysis erroneous and stating, “no clinics will likely be forced to close on account of [LA 620].” The Fifth Circuit panel ruled that, despite the precedent of WWH, LA 620 did not create an undue burden because of state-level differences in admitting privileges, demographics, and geography. They also found that only 30% of the 2 million women living in Louisiana would be impacted by the law, predominantly via longer wait times, and argued that this does not represent significant burden. The plaintiffs filed for an emergency stay with SCOTUS, who granted the stay pending a full hearing. On March 4, the Supreme Court will hear arguments to determine if the Fifth Circuit was correct in drawing a distinction between LA 620 and the SCOTUS verdict in WWH.

Targeted restrictions on abortion providers

LA 620 joins a long series of laws meant to enact targeted restrictions on abortion providers, or “TRAP” laws. TRAP laws are written to limit access to abortion under the guise of improving patient safety, despite ample evidence to the contrary, and include such various regulations as admitting privileges, facilities requirements, waiting periods, and parental or partner notification. Many such laws have been enacted in the last decade, and many struck down based on judicial precedent.

How the Supreme Court has ruled in the past

When a case is appealed to the Supreme Court, the court can either decline to hear the case, thereby leaving the lower courts’ ruling in place, or choose to hear the case in full and either affirm or overturn the lower court’s decision. After issuing a ruling in WWH, the 2016-2017 Roberts Court declined to hear challenges from other states with similarly overturned laws, leaving the laws struck down. In electing to hear June Medical Services v. Russo, the court has the opportunity to uphold or overturn the Fifth Circuit Court’s decision. However, today’s Supreme Court differs greatly from the Supreme Court in 2016.

In 2016, the court ruled 5-3 to overturn H.B. 2 in WWH shortly after the death of Justice Antonin Scalia. Scalia was replaced by Justice Neil Gorsuch, a Constitutional originalist who has never directly ruled on an abortion case.⁵ In 2018, Justice Anthony Kennedy, who authored the court’s majority opinion on Casey and was among the majority on WWH, retired, and was replaced by Justice Brett Kavanaugh. Kavanaugh has ruled once on the right to abortion in Garza v. Hargan in 2017, where he argued that precedent states that the government has “permissible interests in favoring fetal life…and refraining from facilitating abortion,” and that significant delay in care did not constitute undue burden.⁶ In regard to the 5-4 stay issued by the court in June Medical Services, Kavanaugh joined Gorsuch in voting to deny the application for stay, and was the only justice to issue an opinion alongside the ruling, arguing that because the doctors in question had not applied for and been denied admitting privileges since the WWH ruling, the case hinges on theoretical rather than demonstrable undue burden.⁷ Appointed by President Donald Trump, both Gorsuch and Kavanaugh are widely considered to be conservative judges, and while neither has a strong judicial record on abortion rights, both are anticipated to side with the conservative majority on the court.

The Supreme Court rarely overturns its own precedent, but concerns are high

The question of precedent will be central in SCOTUS hearing June Medical Services v. Russo so quickly after the WWH decision. Additionally, in hearing this case, the court will have the opportunity to reexamine all relevant precedent, including the Planned Parenthood of Southeastern Pennsylvania v. Casey decision and even Roe v. Wade. With a conservative court and an increasingly charged political environment, reproductive rights advocates fear that the June Medical Services v. Russo ruling may be the first step toward dismantling judicial protection of abortion rights in the United States.

If SCOTUS rules against June Medical Services, stating that admitting privileges do not cause an undue burden for women seeking to access abortion care, other states likely will introduce and enact similar legislation. These TRAP laws have the potential to limit or eliminate access to abortion for 25 million people of reproductive age. Numerous studies have demonstrated that limiting access to abortion care does not decrease the number of abortions but can result in patients using unsafe means to obtain an abortion.⁸

The medical community recognizes the danger of enacting restrictive legislation. The American College of Obstetricians and Gynecologists (ACOG), along with the American Medical Association, the Society of Maternal-Fetal Medicine, the Association for Sexual and Reproductive Medicine, the American Association of Family Practitioners, and many others, filed an amicus curiae in support of the June Medical Services plaintiffs.⁹ These brief filings are critical to ensuring the courts hear physician voices in this important legal decision, and ACOG’s briefs have been quoted in several previous Supreme Court opinions, concurrences, and dissents.
 

Action items

• Although June Medical Services v. Russo’s decision will not be made until early summer 2020, we can continue to use our voices and expertise to speak out against laws designed to limit access to abortion—at the state and federal levels. As women’s health clinicians, we see the impact abortion restrictions have on our patients, especially our low income and rural patients. Sharing these stories with our legislators, testifying for or against legislation, and speaking out in our communities can have a powerful impact. Check with your local ACOG chapter or with ACOG’s state and government affairs office for more information.
• Follow along with this case at SCOTUS Blog.
• Lastly, make sure you are registered to vote. We are in an election year, and using our voices in and out of the ballot box is critical. You can register here.

On March 4, 2020, the Supreme Court of the United States (SCOTUS) will hear opening arguments for June Medical Services v. Russo. (Please note that this case was originally referred to as June Medical Services v. Gee. However, Secretary Rebekah Gee resigned from her position on January 31, 2020, and was replaced by Interim Secretary Stephen Russo.) The case will examine a Louisiana law (Louisiana Act 620, or LA 620), originally passed in 2014, that requires physicians to have hospital admitting privileges within 30 miles of where they provide abortion services.¹ When LA 620 was signed into law in 2014, 5 of Louisiana’s 6 abortion clinics would not have met the standards created by this legislation and would have been forced to close, potentially leaving the vast majority of women in Louisiana without access to an abortion provider, and disproportionately impacting poor and rural women. Prior to enactment of this law, physicians at these 5 clinics attempted to obtain admitting privileges, and all were denied. The denials occurred due to two main reasons—because the providers admitted too few patients each year to qualify for hospital privileges or simply because they provided abortion care.² Shortly after this legislation was signed into law, the Center for Reproductive Rights (CRR) challenged the law, citing the undue burden it created for patients attempting to access abortion care.
 

Prior case also considered question of hospital privileges for abortion providers

Interestingly, SCOTUS already has ruled on this very question. In 1992, the Court ruled in Planned Parenthood of Southeastern Pennsylvania v. Casey that it is unconstitutional for a state to create an “undue burden” on a woman’s right to abortion prior to fetal viability.³ And in 2016, when considering whether or not requiring abortion providers to obtain hospital privileges creates an undue burden in Whole Women’s Health (WWH) v. Hellerstedt, the Supreme Court’s answer was yes, it does. WWH, with legal aid from CRR, challenged Texas House Bill 2 (H.B. 2), which similar to LA 620, required abortion providers to have local admitting privileges. Based largely on the precedent set in Casey, SCOTUS ruled 5-3 in favor of WWH.

The Louisiana law currently in question was written and challenged in district court simultaneous to the Supreme Court’s review of WWH. The district court declared LA 620 invalid and permanently enjoined its enforcement, finding the law would “drastically burden women’s right to choose abortions.”⁴ However, the US Court of Appeals for the Fifth Circuit reviewed the case and overturned the district court decision, finding the lower court’s analysis erroneous and stating, “no clinics will likely be forced to close on account of [LA 620].” The Fifth Circuit panel ruled that, despite the precedent of WWH, LA 620 did not create an undue burden because of state-level differences in admitting privileges, demographics, and geography. They also found that only 30% of the 2 million women living in Louisiana would be impacted by the law, predominantly via longer wait times, and argued that this does not represent significant burden. The plaintiffs filed for an emergency stay with SCOTUS, who granted the stay pending a full hearing. On March 4, the Supreme Court will hear arguments to determine if the Fifth Circuit was correct in drawing a distinction between LA 620 and the SCOTUS verdict in WWH.

Targeted restrictions on abortion providers

LA 620 joins a long series of laws meant to enact targeted restrictions on abortion providers, or “TRAP” laws. TRAP laws are written to limit access to abortion under the guise of improving patient safety, despite ample evidence to the contrary, and include such various regulations as admitting privileges, facilities requirements, waiting periods, and parental or partner notification. Many such laws have been enacted in the last decade, and many struck down based on judicial precedent.

How the Supreme Court has ruled in the past

When a case is appealed to the Supreme Court, the court can either decline to hear the case, thereby leaving the lower courts’ ruling in place, or choose to hear the case in full and either affirm or overturn the lower court’s decision. After issuing a ruling in WWH, the 2016-2017 Roberts Court declined to hear challenges from other states with similarly overturned laws, leaving the laws struck down. In electing to hear June Medical Services v. Russo, the court has the opportunity to uphold or overturn the Fifth Circuit Court’s decision. However, today’s Supreme Court differs greatly from the Supreme Court in 2016.

In 2016, the court ruled 5-3 to overturn H.B. 2 in WWH shortly after the death of Justice Antonin Scalia. Scalia was replaced by Justice Neil Gorsuch, a Constitutional originalist who has never directly ruled on an abortion case.⁵ In 2018, Justice Anthony Kennedy, who authored the court’s majority opinion on Casey and was among the majority on WWH, retired, and was replaced by Justice Brett Kavanaugh. Kavanaugh has ruled once on the right to abortion in Garza v. Hargan in 2017, where he argued that precedent states that the government has “permissible interests in favoring fetal life…and refraining from facilitating abortion,” and that significant delay in care did not constitute undue burden.⁶ In regard to the 5-4 stay issued by the court in June Medical Services, Kavanaugh joined Gorsuch in voting to deny the application for stay, and was the only justice to issue an opinion alongside the ruling, arguing that because the doctors in question had not applied for and been denied admitting privileges since the WWH ruling, the case hinges on theoretical rather than demonstrable undue burden.⁷ Appointed by President Donald Trump, both Gorsuch and Kavanaugh are widely considered to be conservative judges, and while neither has a strong judicial record on abortion rights, both are anticipated to side with the conservative majority on the court.

The Supreme Court rarely overturns its own precedent, but concerns are high

The question of precedent will be central in SCOTUS hearing June Medical Services v. Russo so quickly after the WWH decision. Additionally, in hearing this case, the court will have the opportunity to reexamine all relevant precedent, including the Planned Parenthood of Southeastern Pennsylvania v. Casey decision and even Roe v. Wade. With a conservative court and an increasingly charged political environment, reproductive rights advocates fear that the June Medical Services v. Russo ruling may be the first step toward dismantling judicial protection of abortion rights in the United States.

If SCOTUS rules against June Medical Services, stating that admitting privileges do not cause an undue burden for women seeking to access abortion care, other states likely will introduce and enact similar legislation. These TRAP laws have the potential to limit or eliminate access to abortion for 25 million people of reproductive age. Numerous studies have demonstrated that limiting access to abortion care does not decrease the number of abortions but can result in patients using unsafe means to obtain an abortion.⁸

The medical community recognizes the danger of enacting restrictive legislation. The American College of Obstetricians and Gynecologists (ACOG), along with the American Medical Association, the Society of Maternal-Fetal Medicine, the Association for Sexual and Reproductive Medicine, the American Association of Family Practitioners, and many others, filed an amicus curiae in support of the June Medical Services plaintiffs.⁹ These brief filings are critical to ensuring the courts hear physician voices in this important legal decision, and ACOG’s briefs have been quoted in several previous Supreme Court opinions, concurrences, and dissents.
 

Action items

• Although June Medical Services v. Russo’s decision will not be made until early summer 2020, we can continue to use our voices and expertise to speak out against laws designed to limit access to abortion—at the state and federal levels. As women’s health clinicians, we see the impact abortion restrictions have on our patients, especially our low income and rural patients. Sharing these stories with our legislators, testifying for or against legislation, and speaking out in our communities can have a powerful impact. Check with your local ACOG chapter or with ACOG’s state and government affairs office for more information.
• Follow along with this case at SCOTUS Blog.
• Lastly, make sure you are registered to vote. We are in an election year, and using our voices in and out of the ballot box is critical. You can register here.

On March 4, 2020, the Supreme Court of the United States (SCOTUS) will hear opening arguments for June Medical Services v. Russo. (Please note that this case was originally referred to as June Medical Services v. Gee. However, Secretary Rebekah Gee resigned from her position on January 31, 2020, and was replaced by Interim Secretary Stephen Russo.) The case will examine a Louisiana law (Louisiana Act 620, or LA 620), originally passed in 2014, that requires physicians to have hospital admitting privileges within 30 miles of where they provide abortion services.¹ When LA 620 was signed into law in 2014, 5 of Louisiana’s 6 abortion clinics would not have met the standards created by this legislation and would have been forced to close, potentially leaving the vast majority of women in Louisiana without access to an abortion provider, and disproportionately impacting poor and rural women. Prior to enactment of this law, physicians at these 5 clinics attempted to obtain admitting privileges, and all were denied. The denials occurred due to two main reasons—because the providers admitted too few patients each year to qualify for hospital privileges or simply because they provided abortion care.² Shortly after this legislation was signed into law, the Center for Reproductive Rights (CRR) challenged the law, citing the undue burden it created for patients attempting to access abortion care.
 

Prior case also considered question of hospital privileges for abortion providers

Interestingly, SCOTUS already has ruled on this very question. In 1992, the Court ruled in Planned Parenthood of Southeastern Pennsylvania v. Casey that it is unconstitutional for a state to create an “undue burden” on a woman’s right to abortion prior to fetal viability.³ And in 2016, when considering whether or not requiring abortion providers to obtain hospital privileges creates an undue burden in Whole Women’s Health (WWH) v. Hellerstedt, the Supreme Court’s answer was yes, it does. WWH, with legal aid from CRR, challenged Texas House Bill 2 (H.B. 2), which similar to LA 620, required abortion providers to have local admitting privileges. Based largely on the precedent set in Casey, SCOTUS ruled 5-3 in favor of WWH.

The Louisiana law currently in question was written and challenged in district court simultaneous to the Supreme Court’s review of WWH. The district court declared LA 620 invalid and permanently enjoined its enforcement, finding the law would “drastically burden women’s right to choose abortions.”⁴ However, the US Court of Appeals for the Fifth Circuit reviewed the case and overturned the district court decision, finding the lower court’s analysis erroneous and stating, “no clinics will likely be forced to close on account of [LA 620].” The Fifth Circuit panel ruled that, despite the precedent of WWH, LA 620 did not create an undue burden because of state-level differences in admitting privileges, demographics, and geography. They also found that only 30% of the 2 million women living in Louisiana would be impacted by the law, predominantly via longer wait times, and argued that this does not represent significant burden. The plaintiffs filed for an emergency stay with SCOTUS, who granted the stay pending a full hearing. On March 4, the Supreme Court will hear arguments to determine if the Fifth Circuit was correct in drawing a distinction between LA 620 and the SCOTUS verdict in WWH.

Targeted restrictions on abortion providers

LA 620 joins a long series of laws meant to enact targeted restrictions on abortion providers, or “TRAP” laws. TRAP laws are written to limit access to abortion under the guise of improving patient safety, despite ample evidence to the contrary, and include such various regulations as admitting privileges, facilities requirements, waiting periods, and parental or partner notification. Many such laws have been enacted in the last decade, and many struck down based on judicial precedent.

How the Supreme Court has ruled in the past

When a case is appealed to the Supreme Court, the court can either decline to hear the case, thereby leaving the lower courts’ ruling in place, or choose to hear the case in full and either affirm or overturn the lower court’s decision. After issuing a ruling in WWH, the 2016-2017 Roberts Court declined to hear challenges from other states with similarly overturned laws, leaving the laws struck down. In electing to hear June Medical Services v. Russo, the court has the opportunity to uphold or overturn the Fifth Circuit Court’s decision. However, today’s Supreme Court differs greatly from the Supreme Court in 2016.

In 2016, the court ruled 5-3 to overturn H.B. 2 in WWH shortly after the death of Justice Antonin Scalia. Scalia was replaced by Justice Neil Gorsuch, a Constitutional originalist who has never directly ruled on an abortion case.⁵ In 2018, Justice Anthony Kennedy, who authored the court’s majority opinion on Casey and was among the majority on WWH, retired, and was replaced by Justice Brett Kavanaugh. Kavanaugh has ruled once on the right to abortion in Garza v. Hargan in 2017, where he argued that precedent states that the government has “permissible interests in favoring fetal life…and refraining from facilitating abortion,” and that significant delay in care did not constitute undue burden.⁶ In regard to the 5-4 stay issued by the court in June Medical Services, Kavanaugh joined Gorsuch in voting to deny the application for stay, and was the only justice to issue an opinion alongside the ruling, arguing that because the doctors in question had not applied for and been denied admitting privileges since the WWH ruling, the case hinges on theoretical rather than demonstrable undue burden.⁷ Appointed by President Donald Trump, both Gorsuch and Kavanaugh are widely considered to be conservative judges, and while neither has a strong judicial record on abortion rights, both are anticipated to side with the conservative majority on the court.

The Supreme Court rarely overturns its own precedent, but concerns are high

The question of precedent will be central in SCOTUS hearing June Medical Services v. Russo so quickly after the WWH decision. Additionally, in hearing this case, the court will have the opportunity to reexamine all relevant precedent, including the Planned Parenthood of Southeastern Pennsylvania v. Casey decision and even Roe v. Wade. With a conservative court and an increasingly charged political environment, reproductive rights advocates fear that the June Medical Services v. Russo ruling may be the first step toward dismantling judicial protection of abortion rights in the United States.

If SCOTUS rules against June Medical Services, stating that admitting privileges do not cause an undue burden for women seeking to access abortion care, other states likely will introduce and enact similar legislation. These TRAP laws have the potential to limit or eliminate access to abortion for 25 million people of reproductive age. Numerous studies have demonstrated that limiting access to abortion care does not decrease the number of abortions but can result in patients using unsafe means to obtain an abortion.⁸

The medical community recognizes the danger of enacting restrictive legislation. The American College of Obstetricians and Gynecologists (ACOG), along with the American Medical Association, the Society of Maternal-Fetal Medicine, the Association for Sexual and Reproductive Medicine, the American Association of Family Practitioners, and many others, filed an amicus curiae in support of the June Medical Services plaintiffs.⁹ These brief filings are critical to ensuring the courts hear physician voices in this important legal decision, and ACOG’s briefs have been quoted in several previous Supreme Court opinions, concurrences, and dissents.
 

Action items

• Although June Medical Services v. Russo’s decision will not be made until early summer 2020, we can continue to use our voices and expertise to speak out against laws designed to limit access to abortion—at the state and federal levels. As women’s health clinicians, we see the impact abortion restrictions have on our patients, especially our low income and rural patients. Sharing these stories with our legislators, testifying for or against legislation, and speaking out in our communities can have a powerful impact. Check with your local ACOG chapter or with ACOG’s state and government affairs office for more information.
• Follow along with this case at SCOTUS Blog.
• Lastly, make sure you are registered to vote. We are in an election year, and using our voices in and out of the ballot box is critical. You can register here.

A unique collection of bacteria in the gut may have a strong association with pulmonary arterial hypertension and could be highly predictive of the disease in undiagnosed patients, according to a study published in the journal Hypertension.

This is the first study to show that people with PAH have a common specific gut microbiota profile, wrote lead study author Mohan Raizada, PhD, distinguished professor in the department of physiology and functional genomics at the University of Florida, Gainesville.

The findings have the potential to change how cardiologists diagnose and treat PAH, he added. “While current PAH treatments focus on the lungs, looking at the lung/gut axis could open the door to new therapies centered in the digestive system,” Dr. Raizada said.

The researchers developed a model that found the specific microbiota profile was 83% accurate in predicting the presence or absence of PAH. If a larger study can validate the findings, the researchers wrote, this could lead to a new test for diagnosing PAH that’s less invasive than cardiac catheterization. It could also lead to new treatments that target the gut microbiome.

Study investigators collected stool samples from 18 PAH patients and 12 people without a history of cardiopulmonary disease. The microbiota DNA from the stool samples were isolated and sequenced. The analysis revealed that PAH patients had reduced richness and evenness of the gut bacteria, known as alpha diversity. They had increased levels of bacteria associated with atherosclerosis, and healthy patients had increased levels of bacteria that produced short-chain fatty acids.

Although recent studies have begun to show potential associations between the gut microbiome and cardiovascular diseases, this research is in its infancy, Mariell Jessup, MD, commented. “Even though the study by Dr. Raizada and colleagues predicted pulmonary arterial hypertension based on an individual’s microbiome with some accuracy, it is an observational study, so it does not prove cause and effect. Many other factors, especially diet, affect the gut microbiome,” added Dr. Jessup, Chief Science and Medical Officer for the American Heart Association.

SOURCE: Raizada MK et al. Hypertension. 2020. doi: 10.1161/HYPERTENSIONAHA.119.14294.

A unique collection of bacteria in the gut may have a strong association with pulmonary arterial hypertension and could be highly predictive of the disease in undiagnosed patients, according to a study published in the journal Hypertension.

This is the first study to show that people with PAH have a common specific gut microbiota profile, wrote lead study author Mohan Raizada, PhD, distinguished professor in the department of physiology and functional genomics at the University of Florida, Gainesville.

The findings have the potential to change how cardiologists diagnose and treat PAH, he added. “While current PAH treatments focus on the lungs, looking at the lung/gut axis could open the door to new therapies centered in the digestive system,” Dr. Raizada said.

The researchers developed a model that found the specific microbiota profile was 83% accurate in predicting the presence or absence of PAH. If a larger study can validate the findings, the researchers wrote, this could lead to a new test for diagnosing PAH that’s less invasive than cardiac catheterization. It could also lead to new treatments that target the gut microbiome.

Study investigators collected stool samples from 18 PAH patients and 12 people without a history of cardiopulmonary disease. The microbiota DNA from the stool samples were isolated and sequenced. The analysis revealed that PAH patients had reduced richness and evenness of the gut bacteria, known as alpha diversity. They had increased levels of bacteria associated with atherosclerosis, and healthy patients had increased levels of bacteria that produced short-chain fatty acids.

Although recent studies have begun to show potential associations between the gut microbiome and cardiovascular diseases, this research is in its infancy, Mariell Jessup, MD, commented. “Even though the study by Dr. Raizada and colleagues predicted pulmonary arterial hypertension based on an individual’s microbiome with some accuracy, it is an observational study, so it does not prove cause and effect. Many other factors, especially diet, affect the gut microbiome,” added Dr. Jessup, Chief Science and Medical Officer for the American Heart Association.

SOURCE: Raizada MK et al. Hypertension. 2020. doi: 10.1161/HYPERTENSIONAHA.119.14294.

A unique collection of bacteria in the gut may have a strong association with pulmonary arterial hypertension and could be highly predictive of the disease in undiagnosed patients, according to a study published in the journal Hypertension.

This is the first study to show that people with PAH have a common specific gut microbiota profile, wrote lead study author Mohan Raizada, PhD, distinguished professor in the department of physiology and functional genomics at the University of Florida, Gainesville.

The findings have the potential to change how cardiologists diagnose and treat PAH, he added. “While current PAH treatments focus on the lungs, looking at the lung/gut axis could open the door to new therapies centered in the digestive system,” Dr. Raizada said.

The researchers developed a model that found the specific microbiota profile was 83% accurate in predicting the presence or absence of PAH. If a larger study can validate the findings, the researchers wrote, this could lead to a new test for diagnosing PAH that’s less invasive than cardiac catheterization. It could also lead to new treatments that target the gut microbiome.

Study investigators collected stool samples from 18 PAH patients and 12 people without a history of cardiopulmonary disease. The microbiota DNA from the stool samples were isolated and sequenced. The analysis revealed that PAH patients had reduced richness and evenness of the gut bacteria, known as alpha diversity. They had increased levels of bacteria associated with atherosclerosis, and healthy patients had increased levels of bacteria that produced short-chain fatty acids.

Although recent studies have begun to show potential associations between the gut microbiome and cardiovascular diseases, this research is in its infancy, Mariell Jessup, MD, commented. “Even though the study by Dr. Raizada and colleagues predicted pulmonary arterial hypertension based on an individual’s microbiome with some accuracy, it is an observational study, so it does not prove cause and effect. Many other factors, especially diet, affect the gut microbiome,” added Dr. Jessup, Chief Science and Medical Officer for the American Heart Association.

SOURCE: Raizada MK et al. Hypertension. 2020. doi: 10.1161/HYPERTENSIONAHA.119.14294.

ORLANDO – Knowledge of vaping devices, familiarity with terminology, and the ability to quickly pinpoint individuals at risk of lung injury are just a few skills that can help critical care professionals confronted with patients who may have vaping-associated lung disease, according to a new guidance document.

Andrew D. Bowser/MDedge News

The guidance offers a risk-stratification system that classifies patients into groups based on exposure, symptoms, and imaging results, and provides specific evaluation needs and management strategies for each. The guidance is designed to help critical care professionals efficiently identify those at high risk of respiratory failure.

Physicians also need to communicate with patients to identify what substances are being vaped and develop effective methods to encourage abstinence, according to the authors, led by Craig M. Lilly, MD, FCCP, professor of medicine, anesthesiology, and surgery at the University of Massachusetts, Worcester.

“I would encourage every intensivist, when they leave their intensive care unit at night, [to ask], ‘have I advised against vaping today?’ ” Dr. Lilly said at the Critical Care Congress sponsored by the Society of Critical Care Medicine.

The guidelines, concurrently published as a review article in Critical Care Explorations, propose the term vaping-associated respiratory distress syndrome (VARDS), which the authors say constitutes an acute and progressive respiratory syndrome marked by pathologic changes of lung injury and potentially life-threatening hypoxemic respiratory failure.

They also introduce the three-group Worcester classification system, which is intended to triage vaping-exposed individuals for risk of VARDS based on the presence or absence of vaping-related symptoms and infiltrates, and normal or abnormal oxygen saturation.

“It’s very simple,” said Dr. Lilly, who added that the risk stratification model was developed at the request of Massachusetts public health officials.

Patients with vaping exposure but no symptoms attributable to vaping, such as cough, chest pain, or weight loss, are classified as Worcester Low Risk and testing is not recommended, he said.

By contrast, individuals are considered Worcester Medium Risk if they have vaping exposure, symptoms, and a vaping-associated abnormal pattern on imaging, but no hypoxemia; the presence of hypoxemia would tip the scale toward Worcester High Risk.

“Most patients that have died from vaping have been sent out of emergency rooms when they were noted to be hypoxic,” Dr. Lilly told meeting attendees.

ORLANDO – Knowledge of vaping devices, familiarity with terminology, and the ability to quickly pinpoint individuals at risk of lung injury are just a few skills that can help critical care professionals confronted with patients who may have vaping-associated lung disease, according to a new guidance document.

Andrew D. Bowser/MDedge News

The guidance offers a risk-stratification system that classifies patients into groups based on exposure, symptoms, and imaging results, and provides specific evaluation needs and management strategies for each. The guidance is designed to help critical care professionals efficiently identify those at high risk of respiratory failure.

Physicians also need to communicate with patients to identify what substances are being vaped and develop effective methods to encourage abstinence, according to the authors, led by Craig M. Lilly, MD, FCCP, professor of medicine, anesthesiology, and surgery at the University of Massachusetts, Worcester.

“I would encourage every intensivist, when they leave their intensive care unit at night, [to ask], ‘have I advised against vaping today?’ ” Dr. Lilly said at the Critical Care Congress sponsored by the Society of Critical Care Medicine.

The guidelines, concurrently published as a review article in Critical Care Explorations, propose the term vaping-associated respiratory distress syndrome (VARDS), which the authors say constitutes an acute and progressive respiratory syndrome marked by pathologic changes of lung injury and potentially life-threatening hypoxemic respiratory failure.

They also introduce the three-group Worcester classification system, which is intended to triage vaping-exposed individuals for risk of VARDS based on the presence or absence of vaping-related symptoms and infiltrates, and normal or abnormal oxygen saturation.

“It’s very simple,” said Dr. Lilly, who added that the risk stratification model was developed at the request of Massachusetts public health officials.

Patients with vaping exposure but no symptoms attributable to vaping, such as cough, chest pain, or weight loss, are classified as Worcester Low Risk and testing is not recommended, he said.

By contrast, individuals are considered Worcester Medium Risk if they have vaping exposure, symptoms, and a vaping-associated abnormal pattern on imaging, but no hypoxemia; the presence of hypoxemia would tip the scale toward Worcester High Risk.

“Most patients that have died from vaping have been sent out of emergency rooms when they were noted to be hypoxic,” Dr. Lilly told meeting attendees.

ORLANDO – Knowledge of vaping devices, familiarity with terminology, and the ability to quickly pinpoint individuals at risk of lung injury are just a few skills that can help critical care professionals confronted with patients who may have vaping-associated lung disease, according to a new guidance document.

Andrew D. Bowser/MDedge News

The guidance offers a risk-stratification system that classifies patients into groups based on exposure, symptoms, and imaging results, and provides specific evaluation needs and management strategies for each. The guidance is designed to help critical care professionals efficiently identify those at high risk of respiratory failure.

Physicians also need to communicate with patients to identify what substances are being vaped and develop effective methods to encourage abstinence, according to the authors, led by Craig M. Lilly, MD, FCCP, professor of medicine, anesthesiology, and surgery at the University of Massachusetts, Worcester.

“I would encourage every intensivist, when they leave their intensive care unit at night, [to ask], ‘have I advised against vaping today?’ ” Dr. Lilly said at the Critical Care Congress sponsored by the Society of Critical Care Medicine.

The guidelines, concurrently published as a review article in Critical Care Explorations, propose the term vaping-associated respiratory distress syndrome (VARDS), which the authors say constitutes an acute and progressive respiratory syndrome marked by pathologic changes of lung injury and potentially life-threatening hypoxemic respiratory failure.

They also introduce the three-group Worcester classification system, which is intended to triage vaping-exposed individuals for risk of VARDS based on the presence or absence of vaping-related symptoms and infiltrates, and normal or abnormal oxygen saturation.

“It’s very simple,” said Dr. Lilly, who added that the risk stratification model was developed at the request of Massachusetts public health officials.

Patients with vaping exposure but no symptoms attributable to vaping, such as cough, chest pain, or weight loss, are classified as Worcester Low Risk and testing is not recommended, he said.

By contrast, individuals are considered Worcester Medium Risk if they have vaping exposure, symptoms, and a vaping-associated abnormal pattern on imaging, but no hypoxemia; the presence of hypoxemia would tip the scale toward Worcester High Risk.

“Most patients that have died from vaping have been sent out of emergency rooms when they were noted to be hypoxic,” Dr. Lilly told meeting attendees.

LONDON – Preventive oral health should be high on the agenda when managing babies and children with dystrophic epidermolysis bullosa (DEB), pediatric dentist Susanne Krämer told attendees at the first EB World Congress.

Sara Freeman/MDedge News

While it may not be the first thing on the minds of families coming to terms with their children having a chronic and potentially debilitating skin disease, it is important to consider oral health early to ensure healthy dentition and mouth function, both of which will affect the ability to eat and thus nutrition.

When there are a lot of other health issues, “dentistry is not a priority,” Dr. Krämer acknowledged in an interview at the meeting, organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA). 

Something as simple as brushing teeth can be very distressing for parents of a child with EB, she observed, especially if there is dysphagia and toothpaste may be getting into the airways accidentally.

Oral health was one of the topics that patients with EB and their families said would be good to have some guidance on when they were surveyed by DEBRA International. This led the charity to develop its first clinical practice guideline in 2012. Dr. Krämer was the lead author of the guidelines, which are about to be updated and republished.

The “Oral Health for Patients with Epidermolysis Bullosa – Best Clinical Practice Guidelines” (Int J Paediatr Dent. 2012;22 Suppl 1:1-35) are in the final stages of being revised, said Dr. Krämer, who is head of the department of pediatric dentistry at the University of Chile in Santiago. Although there is not much new evidence since the guidelines were first published, “we do have a lot of new technologies within dentistry that can aid the care of EB,” she said.

LONDON – Preventive oral health should be high on the agenda when managing babies and children with dystrophic epidermolysis bullosa (DEB), pediatric dentist Susanne Krämer told attendees at the first EB World Congress.

Sara Freeman/MDedge News

While it may not be the first thing on the minds of families coming to terms with their children having a chronic and potentially debilitating skin disease, it is important to consider oral health early to ensure healthy dentition and mouth function, both of which will affect the ability to eat and thus nutrition.

When there are a lot of other health issues, “dentistry is not a priority,” Dr. Krämer acknowledged in an interview at the meeting, organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA). 

Something as simple as brushing teeth can be very distressing for parents of a child with EB, she observed, especially if there is dysphagia and toothpaste may be getting into the airways accidentally.

Oral health was one of the topics that patients with EB and their families said would be good to have some guidance on when they were surveyed by DEBRA International. This led the charity to develop its first clinical practice guideline in 2012. Dr. Krämer was the lead author of the guidelines, which are about to be updated and republished.

The “Oral Health for Patients with Epidermolysis Bullosa – Best Clinical Practice Guidelines” (Int J Paediatr Dent. 2012;22 Suppl 1:1-35) are in the final stages of being revised, said Dr. Krämer, who is head of the department of pediatric dentistry at the University of Chile in Santiago. Although there is not much new evidence since the guidelines were first published, “we do have a lot of new technologies within dentistry that can aid the care of EB,” she said.

LONDON – Preventive oral health should be high on the agenda when managing babies and children with dystrophic epidermolysis bullosa (DEB), pediatric dentist Susanne Krämer told attendees at the first EB World Congress.

Sara Freeman/MDedge News

While it may not be the first thing on the minds of families coming to terms with their children having a chronic and potentially debilitating skin disease, it is important to consider oral health early to ensure healthy dentition and mouth function, both of which will affect the ability to eat and thus nutrition.

When there are a lot of other health issues, “dentistry is not a priority,” Dr. Krämer acknowledged in an interview at the meeting, organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA). 

Something as simple as brushing teeth can be very distressing for parents of a child with EB, she observed, especially if there is dysphagia and toothpaste may be getting into the airways accidentally.

Oral health was one of the topics that patients with EB and their families said would be good to have some guidance on when they were surveyed by DEBRA International. This led the charity to develop its first clinical practice guideline in 2012. Dr. Krämer was the lead author of the guidelines, which are about to be updated and republished.

The “Oral Health for Patients with Epidermolysis Bullosa – Best Clinical Practice Guidelines” (Int J Paediatr Dent. 2012;22 Suppl 1:1-35) are in the final stages of being revised, said Dr. Krämer, who is head of the department of pediatric dentistry at the University of Chile in Santiago. Although there is not much new evidence since the guidelines were first published, “we do have a lot of new technologies within dentistry that can aid the care of EB,” she said.

ORLANDO – Medicare beneficiaries are receiving a low number of rehabilitation visits in the early posthospitalization period after critical illness, an analysis of hospital and home health claims data suggests.

The beneficiaries, all discharged directly to home health after an intensive care unit stay, received an average of less than one visit per week in the ensuing month, while a full third received no visits at all, according to authors of the analysis, presented at the Critical Care Congress sponsored by the Society of Critical Care Medicine.

Living alone and living in a rural area were associated with significantly fewer home health rehabilitation visits, according to investigator Jason Raymond Falvey, PT, DPT, PhD, of Yale University, New Haven, Conn.

“We identified that these patients are receiving low doses of rehabilitation in home health care settings, and perhaps doses low enough to not be physiologically adequate to overcome the deconditioning and aerobic capacity concerns that these patients have,” Dr. Falvey said.

These findings reflect an “underrecognition” of the importance of rehabilitation both outside and inside the hospital setting, according to Patricia J. Posa, RN, of Saint Joseph Mercy Hospital, Northville, Mich.

“We even struggle to provide sufficient rehabilitation while they’re in the hospital,” Ms. Posa said in an interview. “So I think that we still have a major gap in providing rehab services across the continuum, and part of that is recognizing the deficits that patients, especially our elderly patients, might be leaving the hospital with.”

Medicare beneficiaries who survive a critical illness are often discharged with referrals for physical, occupational, or speech therapy, yet there are not much data on the delivery of that care or how many visits actually take place, according to Dr. Falvey.

SOURCE: Falvey J et al. Crit Care Med. 2020 Jan;48(1):28.

ORLANDO – Medicare beneficiaries are receiving a low number of rehabilitation visits in the early posthospitalization period after critical illness, an analysis of hospital and home health claims data suggests.

The beneficiaries, all discharged directly to home health after an intensive care unit stay, received an average of less than one visit per week in the ensuing month, while a full third received no visits at all, according to authors of the analysis, presented at the Critical Care Congress sponsored by the Society of Critical Care Medicine.

Living alone and living in a rural area were associated with significantly fewer home health rehabilitation visits, according to investigator Jason Raymond Falvey, PT, DPT, PhD, of Yale University, New Haven, Conn.

“We identified that these patients are receiving low doses of rehabilitation in home health care settings, and perhaps doses low enough to not be physiologically adequate to overcome the deconditioning and aerobic capacity concerns that these patients have,” Dr. Falvey said.

These findings reflect an “underrecognition” of the importance of rehabilitation both outside and inside the hospital setting, according to Patricia J. Posa, RN, of Saint Joseph Mercy Hospital, Northville, Mich.

“We even struggle to provide sufficient rehabilitation while they’re in the hospital,” Ms. Posa said in an interview. “So I think that we still have a major gap in providing rehab services across the continuum, and part of that is recognizing the deficits that patients, especially our elderly patients, might be leaving the hospital with.”

Medicare beneficiaries who survive a critical illness are often discharged with referrals for physical, occupational, or speech therapy, yet there are not much data on the delivery of that care or how many visits actually take place, according to Dr. Falvey.

SOURCE: Falvey J et al. Crit Care Med. 2020 Jan;48(1):28.

ORLANDO – Medicare beneficiaries are receiving a low number of rehabilitation visits in the early posthospitalization period after critical illness, an analysis of hospital and home health claims data suggests.

The beneficiaries, all discharged directly to home health after an intensive care unit stay, received an average of less than one visit per week in the ensuing month, while a full third received no visits at all, according to authors of the analysis, presented at the Critical Care Congress sponsored by the Society of Critical Care Medicine.

Living alone and living in a rural area were associated with significantly fewer home health rehabilitation visits, according to investigator Jason Raymond Falvey, PT, DPT, PhD, of Yale University, New Haven, Conn.

“We identified that these patients are receiving low doses of rehabilitation in home health care settings, and perhaps doses low enough to not be physiologically adequate to overcome the deconditioning and aerobic capacity concerns that these patients have,” Dr. Falvey said.

These findings reflect an “underrecognition” of the importance of rehabilitation both outside and inside the hospital setting, according to Patricia J. Posa, RN, of Saint Joseph Mercy Hospital, Northville, Mich.

“We even struggle to provide sufficient rehabilitation while they’re in the hospital,” Ms. Posa said in an interview. “So I think that we still have a major gap in providing rehab services across the continuum, and part of that is recognizing the deficits that patients, especially our elderly patients, might be leaving the hospital with.”

Medicare beneficiaries who survive a critical illness are often discharged with referrals for physical, occupational, or speech therapy, yet there are not much data on the delivery of that care or how many visits actually take place, according to Dr. Falvey.

SOURCE: Falvey J et al. Crit Care Med. 2020 Jan;48(1):28.

LONDON – Hardened feet are a major determinant of the clinical course of epidermolysis bullosa simplex (EBS), according to research presented by a German team of investigators at the EB World Congress.

In a study of 157 individuals with EBS, 75.8% had plantar keratoderma, a condition associated with a vicious circle of pain, reduced mobility, subsequent weight gain, and further foot problems.

“EBS has severe impacts on various aspects of everyday life,” Antonia Reimer, MD, and associates at the University of Freiburg, Germany, reported in a poster presentation. “Plantar involvement and [plantar keratoderma] are serious complications of all EBS subtypes, correlating with excessive weight gain, pain, local infections, and limited mobility.”

The researchers suggested that “targeting [plantar keratoderma] should be a priority in EBS therapy and research.”

In their retrospective cohort study, clinical and molecular data were retrieved from patient records, and major determinants of the clinical course of EBS investigated. As such, the researchers looked at how weight changes affected EBS, the effect of hardening skin on the feet, pain, mobility, and working life.

“EB simplex is generally regarded as the ‘mildest’ EB type,” Dr. Reimer and colleagues wrote, “however, individuals with EBS report a high disease burden and frequent pain.” The team found that just under 30% of patients (n = 46) experienced frequent pain, particularly those with localized and severe EBS. Of the patients experiencing pain, the majority (75.2%) had plantar keratoderma. Furthermore, those with blisters underneath the hardened skin reported having the most painful lesions.

Palmoplantar hyperhidrosis was present in slightly more than 40% of cases, and was especially common in individuals with localized EBS, Dr. Reimer and colleagues found. They also found that bacterial and fungal infections occurred in 14% and 7% of patients, respectively, and this correlated significantly with diffuse plantar keratoderma.

A third of patients experience mobility problems, and 8.2% required a wheelchair; 16.4% “were in occupational disability,” the team reported.

“Hyperkeratosis is important because it isn’t just about treating the hyperkeratosis, it’s also looking at the mechanical balance of the foot,” Tariq Khan, PhD, said during an unrelated oral presentation. Dr. Khan, a consultant podiatrist specializing in EB at Great Ormond Street Hospital NHS Foundation Trust in London, discussed how to best manage the feet of people with EB.

“Podiatry technology and how we treat can often be detrimental to an EB patient,” Dr. Khan cautioned at the meeting, which was organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA).

For example, “certain devices, certain types of material, will add more friction and pressure and cause more blistering,” he added, making treatment challenging.

SOURCES: EB 2020. Reimer A et al. Poster 26; Khan T. oral presentation; O’Sullivan M et al. Poster 93; Hubbard L. Poster 19.

LONDON – Hardened feet are a major determinant of the clinical course of epidermolysis bullosa simplex (EBS), according to research presented by a German team of investigators at the EB World Congress.

In a study of 157 individuals with EBS, 75.8% had plantar keratoderma, a condition associated with a vicious circle of pain, reduced mobility, subsequent weight gain, and further foot problems.

“EBS has severe impacts on various aspects of everyday life,” Antonia Reimer, MD, and associates at the University of Freiburg, Germany, reported in a poster presentation. “Plantar involvement and [plantar keratoderma] are serious complications of all EBS subtypes, correlating with excessive weight gain, pain, local infections, and limited mobility.”

The researchers suggested that “targeting [plantar keratoderma] should be a priority in EBS therapy and research.”

In their retrospective cohort study, clinical and molecular data were retrieved from patient records, and major determinants of the clinical course of EBS investigated. As such, the researchers looked at how weight changes affected EBS, the effect of hardening skin on the feet, pain, mobility, and working life.

“EB simplex is generally regarded as the ‘mildest’ EB type,” Dr. Reimer and colleagues wrote, “however, individuals with EBS report a high disease burden and frequent pain.” The team found that just under 30% of patients (n = 46) experienced frequent pain, particularly those with localized and severe EBS. Of the patients experiencing pain, the majority (75.2%) had plantar keratoderma. Furthermore, those with blisters underneath the hardened skin reported having the most painful lesions.

Palmoplantar hyperhidrosis was present in slightly more than 40% of cases, and was especially common in individuals with localized EBS, Dr. Reimer and colleagues found. They also found that bacterial and fungal infections occurred in 14% and 7% of patients, respectively, and this correlated significantly with diffuse plantar keratoderma.

A third of patients experience mobility problems, and 8.2% required a wheelchair; 16.4% “were in occupational disability,” the team reported.

“Hyperkeratosis is important because it isn’t just about treating the hyperkeratosis, it’s also looking at the mechanical balance of the foot,” Tariq Khan, PhD, said during an unrelated oral presentation. Dr. Khan, a consultant podiatrist specializing in EB at Great Ormond Street Hospital NHS Foundation Trust in London, discussed how to best manage the feet of people with EB.

“Podiatry technology and how we treat can often be detrimental to an EB patient,” Dr. Khan cautioned at the meeting, which was organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA).

For example, “certain devices, certain types of material, will add more friction and pressure and cause more blistering,” he added, making treatment challenging.

SOURCES: EB 2020. Reimer A et al. Poster 26; Khan T. oral presentation; O’Sullivan M et al. Poster 93; Hubbard L. Poster 19.

LONDON – Hardened feet are a major determinant of the clinical course of epidermolysis bullosa simplex (EBS), according to research presented by a German team of investigators at the EB World Congress.

In a study of 157 individuals with EBS, 75.8% had plantar keratoderma, a condition associated with a vicious circle of pain, reduced mobility, subsequent weight gain, and further foot problems.

“EBS has severe impacts on various aspects of everyday life,” Antonia Reimer, MD, and associates at the University of Freiburg, Germany, reported in a poster presentation. “Plantar involvement and [plantar keratoderma] are serious complications of all EBS subtypes, correlating with excessive weight gain, pain, local infections, and limited mobility.”

The researchers suggested that “targeting [plantar keratoderma] should be a priority in EBS therapy and research.”

In their retrospective cohort study, clinical and molecular data were retrieved from patient records, and major determinants of the clinical course of EBS investigated. As such, the researchers looked at how weight changes affected EBS, the effect of hardening skin on the feet, pain, mobility, and working life.

“EB simplex is generally regarded as the ‘mildest’ EB type,” Dr. Reimer and colleagues wrote, “however, individuals with EBS report a high disease burden and frequent pain.” The team found that just under 30% of patients (n = 46) experienced frequent pain, particularly those with localized and severe EBS. Of the patients experiencing pain, the majority (75.2%) had plantar keratoderma. Furthermore, those with blisters underneath the hardened skin reported having the most painful lesions.

Palmoplantar hyperhidrosis was present in slightly more than 40% of cases, and was especially common in individuals with localized EBS, Dr. Reimer and colleagues found. They also found that bacterial and fungal infections occurred in 14% and 7% of patients, respectively, and this correlated significantly with diffuse plantar keratoderma.

A third of patients experience mobility problems, and 8.2% required a wheelchair; 16.4% “were in occupational disability,” the team reported.

“Hyperkeratosis is important because it isn’t just about treating the hyperkeratosis, it’s also looking at the mechanical balance of the foot,” Tariq Khan, PhD, said during an unrelated oral presentation. Dr. Khan, a consultant podiatrist specializing in EB at Great Ormond Street Hospital NHS Foundation Trust in London, discussed how to best manage the feet of people with EB.

“Podiatry technology and how we treat can often be detrimental to an EB patient,” Dr. Khan cautioned at the meeting, which was organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA).

For example, “certain devices, certain types of material, will add more friction and pressure and cause more blistering,” he added, making treatment challenging.

SOURCES: EB 2020. Reimer A et al. Poster 26; Khan T. oral presentation; O’Sullivan M et al. Poster 93; Hubbard L. Poster 19.

London – A predictable course of hand contracture was seen in a U.K. study of children with recessive dystrophic epidermolysis bullosa (RDEB), with all children experiencing moderate or severe hand deformity by the age of 12 years.

Sara Freeman/MDedge News

This stark finding, reported at the EB World Congress, organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA), highlighted the importance of intervening early with surgical methods that aim to prevent the pseudosyndactyly, or “mitten” hand deformity, which is an unfortunate characteristic of the genetic skin condition.

The investigative team, from the plastic and reconstructive surgery department at Great Ormond Street Hospital for Children NHS Trust, London, presented data from a retrospective case review of 24 children who attended their specialist pediatric EB center between 2010 and 2019. Of these, seven children had surgery to release hand contractures.

A total of 250 hand assessments were made via the novel Assessment of the Component Hand Contractures in Epidermolysis Bullosa (ACE). The assessment provides a hand deformity grade (HDG) – none, mild, moderate, and severe –based on the typical contractures that are seen in RDEB, such as between the fingers (web space contractures), finger flexion contractures, and thumb adduction contractures.

Using the ACE tool, “we found four significant time points regarding hand contracture development,” Catherine Miller, one of the team’s occupational therapists, said during a poster presentation. At birth, none of the children had any signs of hand deformity, but by 2 years of age half had mild hand contracture. By age 6, all children had some form of hand deformity, Ms. Miller said, and by age 12 all had moderate to severe hand deformity, “so adding to the data that hand deformities really are inevitable.”

Other findings were that the thumb and finger web spaces were the first to contract, Ms. Miller said. “So they tend to develop earlier and progress relatively slowly.” By contrast the finger flexion contractures occurred later on, “but progress more relatively rapidly,” she observed.

“Our data are limited as not every child is included at every age, and out tool has not yet been validated,” Ms. Miller and team acknowledged in the poster. “We assume that hand contractures do not improve, and therefore have included operated hands (mean age 6 years) at their last preoperative HDG in order to represent older children and more advanced hand deformities.”

In an interview, Ms. Miller noted that families have a lot going on when their newborn is diagnosed with RDEB, so introducing the idea that there will be substantial hand deformities in the future “is a difficult conversation. We have to take that gently.”

There are nonsurgical approaches to keeping the hands open, such as “encouraging them to open their hands in play, daily stretches; we can make splints with a silicon substance and other thermoplastic materials,” Ms. Miller said.

Hand surgery is a ‘blunt tool’

“The primary problem, of course, is the dermal fibrosis that we see that creates scarring and secondary problems,” said Gill Smith, a plastic surgery consultant who works with Ms. Miller at the hospital.

“In an ideal world, you would bandage up [the children] so that they could never injure their hands, but then they couldn’t use them, they couldn’t grow properly, and they could not develop,” Ms. Smith said in an oral presentation about hand surgery in children with RDEB. “You do not want them to get to the secondary stage, because the secondary stage is a real problem – you get all these impairments of hand function – pseudosyndactyly, finger contraction, and first web contracture, and ending up in a ‘mitten’ hand.”

Surgery is a very “crude” and “blunt tool,” Ms. Smith emphasized. Prevention is key, and perhaps in the future gene therapy, mesenchymal stem cells, and the like will mean that there is less need for hand surgery, she intimated. Until then, there are some things that can be done surgically – such as wrapping the hands, using gloves to protect the skin, stretching out the web spaces of the palm, and using splints. “All of these things we are trying to improve all the time, and come up with new ideas.”

The question is when to intervene? Ms. Smith said that in any other type of hand surgery, particularly in children where growth and function might be affected, the aim would be to “go in early.” In children with RDEB, however, the timing is not so clear: “Should we be going in early, before secondary joint changes, before we get secondary tendon shortening?” Perhaps this would result in less complex surgery, she suggested, but “it is a really huge deal for families and for children. For the moment we are still only really doing it when there [are] quite significant functional difficulties.”

When it comes to the type of surgery done to release the hands, “everyone has variants on the release technique,” but none are known to be better than any other, Ms. Smith said. Surgical release deals with consequences of dermal fibrosis but also creates more fibrosis, she cautioned.

Effects of hand surgery do not last long

How long the surgery’s effect will last is “what everyone wants to know, and I don’t think anyone has found a really good answer. It is variable, but unfortunately it’s a lot shorter than we’d like,” said Ms. Smith.

Indeed, data in another poster presentation by Ms. Smith and colleagues showed that the situation can be ‘back to square one’ within just a couple of years. Of the seven patients who had surgery at a mean 7 years of age (range 6-10 years), “most had returned to their original total score by 2 years post surgery,” the team wrote. All children “were initially happy with both appearance and function after surgery” they added; however, “happiness gradually decreased with time as they lost function and their scores increased with recurrence of contracture.”

The team noted that “sometimes after surgery a different component of the hand contracture worsened but function was preserved.”

While the ACE tool used by the team has not yet been validated, they believe it to be “a systematic tool with a structured method of administration.” As such it can help with informed decision making, they believe, and it could be used with functional measures to see how hand contractures might be impacting hand function and quality of life.

The ACE tool can be downloaded for free from the GOSH website.

SOURCE: Jessop N et al. EB 2020. Posters 42 and 43; Smith G et al. Poster 63.

London – A predictable course of hand contracture was seen in a U.K. study of children with recessive dystrophic epidermolysis bullosa (RDEB), with all children experiencing moderate or severe hand deformity by the age of 12 years.

Sara Freeman/MDedge News

This stark finding, reported at the EB World Congress, organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA), highlighted the importance of intervening early with surgical methods that aim to prevent the pseudosyndactyly, or “mitten” hand deformity, which is an unfortunate characteristic of the genetic skin condition.

The investigative team, from the plastic and reconstructive surgery department at Great Ormond Street Hospital for Children NHS Trust, London, presented data from a retrospective case review of 24 children who attended their specialist pediatric EB center between 2010 and 2019. Of these, seven children had surgery to release hand contractures.

A total of 250 hand assessments were made via the novel Assessment of the Component Hand Contractures in Epidermolysis Bullosa (ACE). The assessment provides a hand deformity grade (HDG) – none, mild, moderate, and severe –based on the typical contractures that are seen in RDEB, such as between the fingers (web space contractures), finger flexion contractures, and thumb adduction contractures.

Using the ACE tool, “we found four significant time points regarding hand contracture development,” Catherine Miller, one of the team’s occupational therapists, said during a poster presentation. At birth, none of the children had any signs of hand deformity, but by 2 years of age half had mild hand contracture. By age 6, all children had some form of hand deformity, Ms. Miller said, and by age 12 all had moderate to severe hand deformity, “so adding to the data that hand deformities really are inevitable.”

Other findings were that the thumb and finger web spaces were the first to contract, Ms. Miller said. “So they tend to develop earlier and progress relatively slowly.” By contrast the finger flexion contractures occurred later on, “but progress more relatively rapidly,” she observed.

“Our data are limited as not every child is included at every age, and out tool has not yet been validated,” Ms. Miller and team acknowledged in the poster. “We assume that hand contractures do not improve, and therefore have included operated hands (mean age 6 years) at their last preoperative HDG in order to represent older children and more advanced hand deformities.”

In an interview, Ms. Miller noted that families have a lot going on when their newborn is diagnosed with RDEB, so introducing the idea that there will be substantial hand deformities in the future “is a difficult conversation. We have to take that gently.”

There are nonsurgical approaches to keeping the hands open, such as “encouraging them to open their hands in play, daily stretches; we can make splints with a silicon substance and other thermoplastic materials,” Ms. Miller said.

Hand surgery is a ‘blunt tool’

“The primary problem, of course, is the dermal fibrosis that we see that creates scarring and secondary problems,” said Gill Smith, a plastic surgery consultant who works with Ms. Miller at the hospital.

“In an ideal world, you would bandage up [the children] so that they could never injure their hands, but then they couldn’t use them, they couldn’t grow properly, and they could not develop,” Ms. Smith said in an oral presentation about hand surgery in children with RDEB. “You do not want them to get to the secondary stage, because the secondary stage is a real problem – you get all these impairments of hand function – pseudosyndactyly, finger contraction, and first web contracture, and ending up in a ‘mitten’ hand.”

Surgery is a very “crude” and “blunt tool,” Ms. Smith emphasized. Prevention is key, and perhaps in the future gene therapy, mesenchymal stem cells, and the like will mean that there is less need for hand surgery, she intimated. Until then, there are some things that can be done surgically – such as wrapping the hands, using gloves to protect the skin, stretching out the web spaces of the palm, and using splints. “All of these things we are trying to improve all the time, and come up with new ideas.”

The question is when to intervene? Ms. Smith said that in any other type of hand surgery, particularly in children where growth and function might be affected, the aim would be to “go in early.” In children with RDEB, however, the timing is not so clear: “Should we be going in early, before secondary joint changes, before we get secondary tendon shortening?” Perhaps this would result in less complex surgery, she suggested, but “it is a really huge deal for families and for children. For the moment we are still only really doing it when there [are] quite significant functional difficulties.”

When it comes to the type of surgery done to release the hands, “everyone has variants on the release technique,” but none are known to be better than any other, Ms. Smith said. Surgical release deals with consequences of dermal fibrosis but also creates more fibrosis, she cautioned.

Effects of hand surgery do not last long

How long the surgery’s effect will last is “what everyone wants to know, and I don’t think anyone has found a really good answer. It is variable, but unfortunately it’s a lot shorter than we’d like,” said Ms. Smith.

Indeed, data in another poster presentation by Ms. Smith and colleagues showed that the situation can be ‘back to square one’ within just a couple of years. Of the seven patients who had surgery at a mean 7 years of age (range 6-10 years), “most had returned to their original total score by 2 years post surgery,” the team wrote. All children “were initially happy with both appearance and function after surgery” they added; however, “happiness gradually decreased with time as they lost function and their scores increased with recurrence of contracture.”

The team noted that “sometimes after surgery a different component of the hand contracture worsened but function was preserved.”

While the ACE tool used by the team has not yet been validated, they believe it to be “a systematic tool with a structured method of administration.” As such it can help with informed decision making, they believe, and it could be used with functional measures to see how hand contractures might be impacting hand function and quality of life.

The ACE tool can be downloaded for free from the GOSH website.

SOURCE: Jessop N et al. EB 2020. Posters 42 and 43; Smith G et al. Poster 63.

London – A predictable course of hand contracture was seen in a U.K. study of children with recessive dystrophic epidermolysis bullosa (RDEB), with all children experiencing moderate or severe hand deformity by the age of 12 years.

Sara Freeman/MDedge News

This stark finding, reported at the EB World Congress, organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA), highlighted the importance of intervening early with surgical methods that aim to prevent the pseudosyndactyly, or “mitten” hand deformity, which is an unfortunate characteristic of the genetic skin condition.

The investigative team, from the plastic and reconstructive surgery department at Great Ormond Street Hospital for Children NHS Trust, London, presented data from a retrospective case review of 24 children who attended their specialist pediatric EB center between 2010 and 2019. Of these, seven children had surgery to release hand contractures.

A total of 250 hand assessments were made via the novel Assessment of the Component Hand Contractures in Epidermolysis Bullosa (ACE). The assessment provides a hand deformity grade (HDG) – none, mild, moderate, and severe –based on the typical contractures that are seen in RDEB, such as between the fingers (web space contractures), finger flexion contractures, and thumb adduction contractures.

Using the ACE tool, “we found four significant time points regarding hand contracture development,” Catherine Miller, one of the team’s occupational therapists, said during a poster presentation. At birth, none of the children had any signs of hand deformity, but by 2 years of age half had mild hand contracture. By age 6, all children had some form of hand deformity, Ms. Miller said, and by age 12 all had moderate to severe hand deformity, “so adding to the data that hand deformities really are inevitable.”

Other findings were that the thumb and finger web spaces were the first to contract, Ms. Miller said. “So they tend to develop earlier and progress relatively slowly.” By contrast the finger flexion contractures occurred later on, “but progress more relatively rapidly,” she observed.

“Our data are limited as not every child is included at every age, and out tool has not yet been validated,” Ms. Miller and team acknowledged in the poster. “We assume that hand contractures do not improve, and therefore have included operated hands (mean age 6 years) at their last preoperative HDG in order to represent older children and more advanced hand deformities.”

In an interview, Ms. Miller noted that families have a lot going on when their newborn is diagnosed with RDEB, so introducing the idea that there will be substantial hand deformities in the future “is a difficult conversation. We have to take that gently.”

There are nonsurgical approaches to keeping the hands open, such as “encouraging them to open their hands in play, daily stretches; we can make splints with a silicon substance and other thermoplastic materials,” Ms. Miller said.

Hand surgery is a ‘blunt tool’

“The primary problem, of course, is the dermal fibrosis that we see that creates scarring and secondary problems,” said Gill Smith, a plastic surgery consultant who works with Ms. Miller at the hospital.

“In an ideal world, you would bandage up [the children] so that they could never injure their hands, but then they couldn’t use them, they couldn’t grow properly, and they could not develop,” Ms. Smith said in an oral presentation about hand surgery in children with RDEB. “You do not want them to get to the secondary stage, because the secondary stage is a real problem – you get all these impairments of hand function – pseudosyndactyly, finger contraction, and first web contracture, and ending up in a ‘mitten’ hand.”

Surgery is a very “crude” and “blunt tool,” Ms. Smith emphasized. Prevention is key, and perhaps in the future gene therapy, mesenchymal stem cells, and the like will mean that there is less need for hand surgery, she intimated. Until then, there are some things that can be done surgically – such as wrapping the hands, using gloves to protect the skin, stretching out the web spaces of the palm, and using splints. “All of these things we are trying to improve all the time, and come up with new ideas.”

The question is when to intervene? Ms. Smith said that in any other type of hand surgery, particularly in children where growth and function might be affected, the aim would be to “go in early.” In children with RDEB, however, the timing is not so clear: “Should we be going in early, before secondary joint changes, before we get secondary tendon shortening?” Perhaps this would result in less complex surgery, she suggested, but “it is a really huge deal for families and for children. For the moment we are still only really doing it when there [are] quite significant functional difficulties.”

When it comes to the type of surgery done to release the hands, “everyone has variants on the release technique,” but none are known to be better than any other, Ms. Smith said. Surgical release deals with consequences of dermal fibrosis but also creates more fibrosis, she cautioned.

Effects of hand surgery do not last long

How long the surgery’s effect will last is “what everyone wants to know, and I don’t think anyone has found a really good answer. It is variable, but unfortunately it’s a lot shorter than we’d like,” said Ms. Smith.

Indeed, data in another poster presentation by Ms. Smith and colleagues showed that the situation can be ‘back to square one’ within just a couple of years. Of the seven patients who had surgery at a mean 7 years of age (range 6-10 years), “most had returned to their original total score by 2 years post surgery,” the team wrote. All children “were initially happy with both appearance and function after surgery” they added; however, “happiness gradually decreased with time as they lost function and their scores increased with recurrence of contracture.”

The team noted that “sometimes after surgery a different component of the hand contracture worsened but function was preserved.”

While the ACE tool used by the team has not yet been validated, they believe it to be “a systematic tool with a structured method of administration.” As such it can help with informed decision making, they believe, and it could be used with functional measures to see how hand contractures might be impacting hand function and quality of life.

The ACE tool can be downloaded for free from the GOSH website.

SOURCE: Jessop N et al. EB 2020. Posters 42 and 43; Smith G et al. Poster 63.

ORLANDO – Individuals with opioid use disorder are more likely to be hospitalized for sepsis and die of sepsis, results of a recent retrospective analysis suggest.

Andrew D. Bowser/MDedge News

The prevalence of opioid use disorder (OUD) has significantly increased over the past 15 years, the analysis further shows.

Results of the study, presented at the Critical Care Congress sponsored by the Society of Critical Care Medicine, further suggested that OUD disproportionately contributes to sepsis deaths in younger, healthier patients.

Together, these findings underscore the importance of ongoing efforts to address the opioid epidemic in the United States, according to researcher Mohammad Alrawashdeh, PhD, MSN, a postdoctoral research fellow with Harvard Medical School and Harvard Pilgrim Health Care Institute, Boston.

“In addition to ongoing efforts to combat the opioid crisis, future public health interventions should focus on increasing awareness, recognition, and aggressive treatment of sepsis in this population,” Dr. Alrawashdeh said in an oral presentation of the study.

This study fills an important knowledge gap regarding the connection between OUD and sepsis, according to Greg S. Martin, MD, MS, FCCM, professor of medicine in pulmonary critical care at Emory University, Atlanta, and secretary for the Society of Critical Care Medicine.

“We’ve not really ever been able to piece together the relationship between opioid use disorders and sepsis,” Dr. Martin said in an interview. “It’s not that people wouldn’t suspect that there’s a connection – it’s more that we have simply not been able to get the kind of data that you can use, like they’ve done here, that really helps you to answer that question.”

The study suggests not only that OUD and sepsis are linked, Dr. Martin added, but that health care providers need to be prepared to potentially see further increases in the number of patients with OUD seen in the intensive care unit.

“Both of those are things that we certainly need to be aware of, both from the individual practitioner perspective and also the public health planning perspective,” he said.

SOURCE: Alrawashdeh M et al. Crit Care Med. 2020 Jan;48(1):28. Abstract 56.

ORLANDO – Individuals with opioid use disorder are more likely to be hospitalized for sepsis and die of sepsis, results of a recent retrospective analysis suggest.

Andrew D. Bowser/MDedge News

The prevalence of opioid use disorder (OUD) has significantly increased over the past 15 years, the analysis further shows.

Results of the study, presented at the Critical Care Congress sponsored by the Society of Critical Care Medicine, further suggested that OUD disproportionately contributes to sepsis deaths in younger, healthier patients.

Together, these findings underscore the importance of ongoing efforts to address the opioid epidemic in the United States, according to researcher Mohammad Alrawashdeh, PhD, MSN, a postdoctoral research fellow with Harvard Medical School and Harvard Pilgrim Health Care Institute, Boston.

“In addition to ongoing efforts to combat the opioid crisis, future public health interventions should focus on increasing awareness, recognition, and aggressive treatment of sepsis in this population,” Dr. Alrawashdeh said in an oral presentation of the study.

This study fills an important knowledge gap regarding the connection between OUD and sepsis, according to Greg S. Martin, MD, MS, FCCM, professor of medicine in pulmonary critical care at Emory University, Atlanta, and secretary for the Society of Critical Care Medicine.

“We’ve not really ever been able to piece together the relationship between opioid use disorders and sepsis,” Dr. Martin said in an interview. “It’s not that people wouldn’t suspect that there’s a connection – it’s more that we have simply not been able to get the kind of data that you can use, like they’ve done here, that really helps you to answer that question.”

The study suggests not only that OUD and sepsis are linked, Dr. Martin added, but that health care providers need to be prepared to potentially see further increases in the number of patients with OUD seen in the intensive care unit.

“Both of those are things that we certainly need to be aware of, both from the individual practitioner perspective and also the public health planning perspective,” he said.

SOURCE: Alrawashdeh M et al. Crit Care Med. 2020 Jan;48(1):28. Abstract 56.

ORLANDO – Individuals with opioid use disorder are more likely to be hospitalized for sepsis and die of sepsis, results of a recent retrospective analysis suggest.

Andrew D. Bowser/MDedge News

The prevalence of opioid use disorder (OUD) has significantly increased over the past 15 years, the analysis further shows.

Results of the study, presented at the Critical Care Congress sponsored by the Society of Critical Care Medicine, further suggested that OUD disproportionately contributes to sepsis deaths in younger, healthier patients.

Together, these findings underscore the importance of ongoing efforts to address the opioid epidemic in the United States, according to researcher Mohammad Alrawashdeh, PhD, MSN, a postdoctoral research fellow with Harvard Medical School and Harvard Pilgrim Health Care Institute, Boston.

“In addition to ongoing efforts to combat the opioid crisis, future public health interventions should focus on increasing awareness, recognition, and aggressive treatment of sepsis in this population,” Dr. Alrawashdeh said in an oral presentation of the study.

This study fills an important knowledge gap regarding the connection between OUD and sepsis, according to Greg S. Martin, MD, MS, FCCM, professor of medicine in pulmonary critical care at Emory University, Atlanta, and secretary for the Society of Critical Care Medicine.

“We’ve not really ever been able to piece together the relationship between opioid use disorders and sepsis,” Dr. Martin said in an interview. “It’s not that people wouldn’t suspect that there’s a connection – it’s more that we have simply not been able to get the kind of data that you can use, like they’ve done here, that really helps you to answer that question.”

The study suggests not only that OUD and sepsis are linked, Dr. Martin added, but that health care providers need to be prepared to potentially see further increases in the number of patients with OUD seen in the intensive care unit.

“Both of those are things that we certainly need to be aware of, both from the individual practitioner perspective and also the public health planning perspective,” he said.

SOURCE: Alrawashdeh M et al. Crit Care Med. 2020 Jan;48(1):28. Abstract 56.

SAN FRANCISCO – Early use of the androgen receptor antagonist apalutamide for metastatic castration-sensitive prostate cancer has a sustained carry-over benefit regardless of subsequent therapy, an exploratory analysis of the TITAN trial suggests.

Susan London/MDedge News

Previous trial results for all 1,052 men randomized showed significant improvements in radiographic progression-free survival and overall survival from adding apalutamide versus placebo to androgen deprivation therapy (N Engl J Med. 2019;381:13-24), leading to recent Food and Drug Administration approval of the drug for metastatic castration-sensitive prostate cancer.

In the new analysis, investigators assessed progression-free survival 2 (PFS2), measured from time of randomization to investigator-determined disease progression or death, among the 277 men who went on to receive a subsequent life-prolonging therapy after progression on their trial therapy.

Results reported at the 2020 Genitourinary Cancers Symposium showed that the risk of PFS2 events was similarly reduced for patients who had initially received apalutamide vs. placebo regardless of whether their next therapy was a new hormonal therapy (32% reduction in risk) or a taxane (37% reduction in risk).

“The PFS2 benefit is an indicator of effective early intensification of treatment, is consistent with the overall survival benefit we have seen with this agent, and together shows totality of the treatment trajectory,” reported Neeraj Agarwal, MD, the study’s lead investigator. “These results may assist with counseling of patients with metastatic castration-sensitive prostate cancer who are contemplating various treatment options.”

The analysis was performed post hoc, and the subsequent therapy was left up to the treating clinicians, acknowledged Dr. Agarwal, professor of medicine and director of the genitourinary oncology program at the Huntsman Cancer Institute, University of Utah, Salt Lake City.

“A small number of events on subsequent therapy and the nonrandomized treatment decision preclude determination of best subsequent therapy based on these data,” he said. “We need further events and for data to mature more in order to pursue additional in-depth analysis.”

Some caveats

As only about a fifth of patients experienced PFS2 events, “these findings need to be interpreted with some caution,” said invited discussant Dana E. Rathkopf, MD, a genitourinary medical oncologist at Memorial Sloan Kettering Cancer Center in New York.

Another caveat is that the cohort analyzed comprised poor responders, who had experienced progression at a median of roughly 12 months whether on apalutamide or placebo, she noted. “If the patients were all on treatment for about 12 months, it does make me wonder why the apalutamide patients responded better than placebo patients to a second hormonal therapy, because you might think that, in the setting of poor response to upfront apalutamide, these patients may develop some type of intrinsic resistance that would suggest they would not respond to a second-line androgen receptor inhibitor relative to a taxane.”

Patients in TITAN were stratified on prior receipt of docetaxel before undergoing randomization. But simply by chance, among those receiving subsequent hormonal therapy, a larger share of those initially treated with apalutamide than of those initially treated with placebo had received the taxane (33% vs. 16%), which may have influenced outcomes, Dr. Rathkopf added.

“Further maturation of the data will tell us more,” she said. “But clearly, apalutamide in both the SPARTAN and TITAN trials improved PFS2 relative to placebo, and this begs the question of how we can better select treatment using predictive markers.”
 

Study details

Among the 277 men experiencing progression on their trial therapy and going on to receive a life-prolonging subsequent therapy, about 30% later received a new hormonal therapy – abiraterone or enzalutamide – and 35% subsequently received a taxane – docetaxel or cabazitaxel.

Overall, PFS2 was significantly better for men initially randomized to apalutamide, compared with counterparts initially randomized to placebo (hazard ratio for events, 0.66; P = .0026), Dr. Agarwal reported at the symposium, which was sponsored by the American Society of Clinical Oncology, ASTRO, and the Society of Urologic Oncology.

In stratified analyses, PFS2 was also significantly better with initial apalutamide vs. placebo whether patients went on to receive a new hormonal therapy (hazard ratio for events, 0.684; P = .0326) or taxane chemotherapy (hazard ratio for events, 0.634; P = .0062). Median values were not reached.

The trial was funded by Janssen Research & Development. Dr. Agarwal and Dr. Rathkopf each disclosed relationships with numerous pharmaceutical companies, including Janssen.

SOURCE: Agarwal N et al. GUCS 2020. Abstract 82.

SAN FRANCISCO – Early use of the androgen receptor antagonist apalutamide for metastatic castration-sensitive prostate cancer has a sustained carry-over benefit regardless of subsequent therapy, an exploratory analysis of the TITAN trial suggests.

Susan London/MDedge News

Previous trial results for all 1,052 men randomized showed significant improvements in radiographic progression-free survival and overall survival from adding apalutamide versus placebo to androgen deprivation therapy (N Engl J Med. 2019;381:13-24), leading to recent Food and Drug Administration approval of the drug for metastatic castration-sensitive prostate cancer.

In the new analysis, investigators assessed progression-free survival 2 (PFS2), measured from time of randomization to investigator-determined disease progression or death, among the 277 men who went on to receive a subsequent life-prolonging therapy after progression on their trial therapy.

Results reported at the 2020 Genitourinary Cancers Symposium showed that the risk of PFS2 events was similarly reduced for patients who had initially received apalutamide vs. placebo regardless of whether their next therapy was a new hormonal therapy (32% reduction in risk) or a taxane (37% reduction in risk).

“The PFS2 benefit is an indicator of effective early intensification of treatment, is consistent with the overall survival benefit we have seen with this agent, and together shows totality of the treatment trajectory,” reported Neeraj Agarwal, MD, the study’s lead investigator. “These results may assist with counseling of patients with metastatic castration-sensitive prostate cancer who are contemplating various treatment options.”

The analysis was performed post hoc, and the subsequent therapy was left up to the treating clinicians, acknowledged Dr. Agarwal, professor of medicine and director of the genitourinary oncology program at the Huntsman Cancer Institute, University of Utah, Salt Lake City.

“A small number of events on subsequent therapy and the nonrandomized treatment decision preclude determination of best subsequent therapy based on these data,” he said. “We need further events and for data to mature more in order to pursue additional in-depth analysis.”

Some caveats

As only about a fifth of patients experienced PFS2 events, “these findings need to be interpreted with some caution,” said invited discussant Dana E. Rathkopf, MD, a genitourinary medical oncologist at Memorial Sloan Kettering Cancer Center in New York.

Another caveat is that the cohort analyzed comprised poor responders, who had experienced progression at a median of roughly 12 months whether on apalutamide or placebo, she noted. “If the patients were all on treatment for about 12 months, it does make me wonder why the apalutamide patients responded better than placebo patients to a second hormonal therapy, because you might think that, in the setting of poor response to upfront apalutamide, these patients may develop some type of intrinsic resistance that would suggest they would not respond to a second-line androgen receptor inhibitor relative to a taxane.”

Patients in TITAN were stratified on prior receipt of docetaxel before undergoing randomization. But simply by chance, among those receiving subsequent hormonal therapy, a larger share of those initially treated with apalutamide than of those initially treated with placebo had received the taxane (33% vs. 16%), which may have influenced outcomes, Dr. Rathkopf added.

“Further maturation of the data will tell us more,” she said. “But clearly, apalutamide in both the SPARTAN and TITAN trials improved PFS2 relative to placebo, and this begs the question of how we can better select treatment using predictive markers.”
 

Study details

Among the 277 men experiencing progression on their trial therapy and going on to receive a life-prolonging subsequent therapy, about 30% later received a new hormonal therapy – abiraterone or enzalutamide – and 35% subsequently received a taxane – docetaxel or cabazitaxel.

Overall, PFS2 was significantly better for men initially randomized to apalutamide, compared with counterparts initially randomized to placebo (hazard ratio for events, 0.66; P = .0026), Dr. Agarwal reported at the symposium, which was sponsored by the American Society of Clinical Oncology, ASTRO, and the Society of Urologic Oncology.

In stratified analyses, PFS2 was also significantly better with initial apalutamide vs. placebo whether patients went on to receive a new hormonal therapy (hazard ratio for events, 0.684; P = .0326) or taxane chemotherapy (hazard ratio for events, 0.634; P = .0062). Median values were not reached.

The trial was funded by Janssen Research & Development. Dr. Agarwal and Dr. Rathkopf each disclosed relationships with numerous pharmaceutical companies, including Janssen.

SOURCE: Agarwal N et al. GUCS 2020. Abstract 82.

SAN FRANCISCO – Early use of the androgen receptor antagonist apalutamide for metastatic castration-sensitive prostate cancer has a sustained carry-over benefit regardless of subsequent therapy, an exploratory analysis of the TITAN trial suggests.

Susan London/MDedge News

Previous trial results for all 1,052 men randomized showed significant improvements in radiographic progression-free survival and overall survival from adding apalutamide versus placebo to androgen deprivation therapy (N Engl J Med. 2019;381:13-24), leading to recent Food and Drug Administration approval of the drug for metastatic castration-sensitive prostate cancer.

In the new analysis, investigators assessed progression-free survival 2 (PFS2), measured from time of randomization to investigator-determined disease progression or death, among the 277 men who went on to receive a subsequent life-prolonging therapy after progression on their trial therapy.

Results reported at the 2020 Genitourinary Cancers Symposium showed that the risk of PFS2 events was similarly reduced for patients who had initially received apalutamide vs. placebo regardless of whether their next therapy was a new hormonal therapy (32% reduction in risk) or a taxane (37% reduction in risk).

“The PFS2 benefit is an indicator of effective early intensification of treatment, is consistent with the overall survival benefit we have seen with this agent, and together shows totality of the treatment trajectory,” reported Neeraj Agarwal, MD, the study’s lead investigator. “These results may assist with counseling of patients with metastatic castration-sensitive prostate cancer who are contemplating various treatment options.”

The analysis was performed post hoc, and the subsequent therapy was left up to the treating clinicians, acknowledged Dr. Agarwal, professor of medicine and director of the genitourinary oncology program at the Huntsman Cancer Institute, University of Utah, Salt Lake City.

“A small number of events on subsequent therapy and the nonrandomized treatment decision preclude determination of best subsequent therapy based on these data,” he said. “We need further events and for data to mature more in order to pursue additional in-depth analysis.”

Some caveats

As only about a fifth of patients experienced PFS2 events, “these findings need to be interpreted with some caution,” said invited discussant Dana E. Rathkopf, MD, a genitourinary medical oncologist at Memorial Sloan Kettering Cancer Center in New York.

Another caveat is that the cohort analyzed comprised poor responders, who had experienced progression at a median of roughly 12 months whether on apalutamide or placebo, she noted. “If the patients were all on treatment for about 12 months, it does make me wonder why the apalutamide patients responded better than placebo patients to a second hormonal therapy, because you might think that, in the setting of poor response to upfront apalutamide, these patients may develop some type of intrinsic resistance that would suggest they would not respond to a second-line androgen receptor inhibitor relative to a taxane.”

Patients in TITAN were stratified on prior receipt of docetaxel before undergoing randomization. But simply by chance, among those receiving subsequent hormonal therapy, a larger share of those initially treated with apalutamide than of those initially treated with placebo had received the taxane (33% vs. 16%), which may have influenced outcomes, Dr. Rathkopf added.

“Further maturation of the data will tell us more,” she said. “But clearly, apalutamide in both the SPARTAN and TITAN trials improved PFS2 relative to placebo, and this begs the question of how we can better select treatment using predictive markers.”
 

Study details

Among the 277 men experiencing progression on their trial therapy and going on to receive a life-prolonging subsequent therapy, about 30% later received a new hormonal therapy – abiraterone or enzalutamide – and 35% subsequently received a taxane – docetaxel or cabazitaxel.

Overall, PFS2 was significantly better for men initially randomized to apalutamide, compared with counterparts initially randomized to placebo (hazard ratio for events, 0.66; P = .0026), Dr. Agarwal reported at the symposium, which was sponsored by the American Society of Clinical Oncology, ASTRO, and the Society of Urologic Oncology.

In stratified analyses, PFS2 was also significantly better with initial apalutamide vs. placebo whether patients went on to receive a new hormonal therapy (hazard ratio for events, 0.684; P = .0326) or taxane chemotherapy (hazard ratio for events, 0.634; P = .0062). Median values were not reached.

The trial was funded by Janssen Research & Development. Dr. Agarwal and Dr. Rathkopf each disclosed relationships with numerous pharmaceutical companies, including Janssen.

SOURCE: Agarwal N et al. GUCS 2020. Abstract 82.