To the Editor:

A 38-year-old woman was diagnosed with common variable immune deficiency (CVID) by an immunologist at an outside institution 1 year prior to the current presentation. The diagnosis was based on history of severe recurrent sinopulmonary tract, inner ear, Clostridium difficile, urinary tract, and herpes zoster infections of approximately 6 years’ duration, as well as persistently low IgG, IgA, and IgM levels of 530 mg/dL (reference range, 690–1400 mg/dL), 29 mg/dL (reference range, 88–410 mg/dL), and 30 mg/dL (reference range, 34–210 mg/dL), respectively, with failure to respond to vaccinations (ie, Haemophilus influenzae type B, Streptococcus pneumoniae, diphtheria IgG antibody, tetanus antibody). She was started on replacement intravenous immunoglobulin (IVIG) 40 g monthly (400 mg/kg) for CVID. She had a family history of CVID diagnosed in her son and sister.

One year after the CVID diagnosis, she was diagnosed with Sweet syndrome (SS) by a physician at our institution via biopsy of a lesion on the left arm (Figure 1) that showed dense dermal infiltrate of neutrophils with scattered background apoptotic nuclear debris without evidence of vasculitis (Figure 2). Gram stain and microbial biopsy cultures were negative for mycobacterial, fungal, and bacterial organisms. Cutaneous lesions failed to respond to courses of intravenous antibiotics. Sarcoidosis workup was unremarkable and was pursued to exclude the association with SS. Other negative testing included antinuclear antibody, human immunodeficiency virus, rheumatoid factor, thyroid-stimulating hormone, Ro and La autoantibodies, cytoplasmic antineutrophil cytoplasmic antibody, perinuclear antineutrophil cytoplasmic antibody, antimitochondrial antibody, and urinalysis. Occult malignancy was excluded with negative bone marrow biopsy; cerebrospinal fluid analysis; esophagogastroduodenoscopy; colonoscopy; and computed tomography of the chest, abdomen, and pelvis.

Sweet syndrome flares in this patient began with a prodromal syndrome of fever, chills, fatigue, diarrhea, and severe local neuropathic pain. Cutaneous lesions erupted 2 days later, most frequently on the arms and fingers. Preemptive treatment with prednisone 30 to 40 mg when the prodrome was present did not arrest cutaneous lesion development. Flares initially occurred every 3 to 5 weeks.

She initially was successfully treated with high-dose prednisone 100 mg daily during SS flares. Prolonged low-dose prednisone maintenance (10–20 mg) and hydroxychloroquine failed to control her frequent exacerbations. Dapsone was intolerable secondary to an adverse reaction. She continued to have frequent exacerbations of the SS requiring hospitalizations.

During SS flares, CVID was stable with infrequent systemic infections. Although a causal relationship between CVID and SS was unclear, an empiric increase in IVIG dose was made by her immunologist to test if it would decrease the frequency of the cutaneous flares. Subsequently, the IVIG dose was increased to 60 g monthly followed by 200 g monthly after approximately 4 months with a partial initial response in the beginning of therapy for the first 6 months. However, episodes resumed with increasing frequency with cutaneous lesion flares every 2 to 3 weeks. In a 3-month period, the patient had at least 4 hospitalizations for SS flares. Finally, 18 months after the diagnosis of SS was made, she was started on metronomic cyclophosphamide at a daily oral dose of 100 mg, later reduced to 50 mg daily after she developed mild neutropenia. She was continued on monthly IVIG replacement at a higher dose of 200 g divided over 2 days for CVID throughout the course of the disease and to the present time. Since then, the frequency of SS flares has notably reduced. She required 1 hospitalization after cyclophosphamide was initiated. She uses short-pulse prednisone (1 mg/kg) for 3 to 5 days when new skin lesions appear in addition to cyclophosphamide.

Common variable immune deficiency, the most common primary immunodeficiency, initially can present in adulthood.^1,2 Its hallmarks include low levels of serum immunoglobulin, most notably IgG with most patients having concurrent deficiencies of IgA and IgM, and impaired antibody responses with recurrent or atypical infections. It has been associated with autoimmune diseases, granulomatous disease, and inflammatory disorders.² Failure to mount protective levels of antibody titer after vaccination demonstrates the deficiency of antibody production.¹ Lack of recognition of this clinical spectrum may lead to delayed diagnosis and more importantly stalls the initiation of immunoglobulin replacement therapy.¹ The customary dose of immunoglobulin replacement is 400 mg/kg given in a single monthly infusion²; however, doses should be individualized and based on clinical response.¹

Sweet syndrome is characterized by the constellation of pyrexia; leukocytosis; and eruption of painful, edematous, dermal, and neutrophil-dense plaques that occur in the setting of infection or malignancy or are drug induced.^3,4 Although not fully elucidated, the pathogenesis is thought to involve the effects of cytokines that precipitate neutrophil activation and infiltration inducing a hypersensitivity reaction and escalation of the immunologic cascade.³ Because SS can represent a paraneoplastic phenomenon or a dermal manifestation of a solid neoplasm or hematologic dyscrasia, it is important to rule out occult malignancy.³ The mainstay of treatment is systemic corticosteroids to which classical SS lesions readily respond. Alternatively, topical or intralesional corticosteroids may be used as adjuvant therapy. Alternate first-line treatments include potassium iodide and colchicine. Second-line therapies include indomethacin, cyclosporine, dapsone, and other immunosuppressive agents.⁵ The lesions may become superinfected with bacterial pathogens requiring antimicrobials.³ Spontaneous resolution seldom occurs. The risk for relapse is lifelong following spontaneous or therapy-induced clinical remission.³ There is a growing body of literature of SS-associated conditions.

Common variable immune deficiency is a collection of disorders resulting in antibody deficiency and recurrent infections.⁶ Despite the humeral defects in CVID, patients paradoxically may develop various autoimmune, hematologic, and inflammatory disorders.⁷ Sweet syndrome, first described in 1964, is a constellation of fever, neutrophilia, and neutrophilic dermatosis of unknown pathogenesis.⁸ Copresentation of CVID and SS has not been commonly reported. O’Regan et al⁸ described a 17-year-old adolescent boy with both SS and CVID but SS preceded the diagnosis of CVID. In our case, the patient presented with CVID first and then manifested SS 1 year later.

Common variable immune deficiency is the most frequent symptomatic primary immunodeficiency in adults. Because adults with CVID have varied manifestations, CVID is thought to be late-onset antibody failure. The genetic basis of these disorders has not been identified in the majority of individuals. More than 100 genetic defects have been ascribed to primary immunodeficiencies,⁹ though none are consistently found to be associated with CVID. The majority of CVID cases are sporadic, but the positive family history in our patient suggests a familial form. Approximately 10% to 20% of patients have an identified heritable cause of CVID.¹⁰ Our patient’s diagnosis of CVID was confirmed by meeting the diagnostic triad set by the European Society for Immunodeficiencies¹¹ of marked reduction of IgG and IgA or IgM plus onset after 2 years of age, recurrent infections, and defective vaccination response. Additional complications including autoimmunity, malignancy, and granulomatous inflammation were extensively ruled out.

The etiology of SS is unknown and its pathogenesis not fully elucidated, though it is presumed to be a hypersensitivity reaction.¹² Sweet syndrome can be classified into 3 major subtypes: classical or idiopathic, malignancy associated, or drug induced.³ Our patient’s presentation is consistent with the classical variant, as malignancy was ruled out and the patient was not on any medication other than IVIG at the time of diagnosis. The treatment of SS consists of systemic steroids, initially high dose followed by a prolonged taper over 4 to 6 weeks.³ This treatment causes a pronounced and sustained decrease in serum IgG due to increased catabolism during drug administration and decreased synthesis during and for a variable time after drug administration.¹³ In refractory cases, intravenous pulse administration of methylprednisolone sodium succinate for 3 to 5 days may enhance the response to standard therapies.⁵

The concurrent development of neutrophilic dermatoses/SS in an individual with CVID has not been fully described. There is a credible association of SS with infections, inflammatory bowel disease, pregnancy, malignancy, and medications, as well as a possible association with Behçet disease, erythema nodosum, relapsing polychondritis, rheumatoid arthritis, sarcoidosis, and thyroid disease.⁵ The association between immunoglobulin deficiencies and SS is markedly unusual. Despite regular IVIG replacement, adequate treatment of CVID did not seem to modulate SS flares in our patient. A case report in a pediatric patient does not provide specific guidance regarding treatment options.⁸

A particularly challenging aspect of our case was tailoring a treatment regimen to suppress SS flares. We have attained partial response to the refractory cutaneous lesions (decreased frequency and amplitude of outbreaks) with IVIG replacement 200 g every 4 weeks in combination with metronomic cyclophosphamide 50 mg daily (use of a repetitive, low-dose daily chemotherapy regimen to minimize side effects). Intermittent SS flares were managed acutely with pulse high-dose steroids. We report a case of SS with CVID, raising the plausibility of correlated pathogenesis. However, the exact mechanisms remain undefined.

To the Editor:

A 38-year-old woman was diagnosed with common variable immune deficiency (CVID) by an immunologist at an outside institution 1 year prior to the current presentation. The diagnosis was based on history of severe recurrent sinopulmonary tract, inner ear, Clostridium difficile, urinary tract, and herpes zoster infections of approximately 6 years’ duration, as well as persistently low IgG, IgA, and IgM levels of 530 mg/dL (reference range, 690–1400 mg/dL), 29 mg/dL (reference range, 88–410 mg/dL), and 30 mg/dL (reference range, 34–210 mg/dL), respectively, with failure to respond to vaccinations (ie, Haemophilus influenzae type B, Streptococcus pneumoniae, diphtheria IgG antibody, tetanus antibody). She was started on replacement intravenous immunoglobulin (IVIG) 40 g monthly (400 mg/kg) for CVID. She had a family history of CVID diagnosed in her son and sister.

One year after the CVID diagnosis, she was diagnosed with Sweet syndrome (SS) by a physician at our institution via biopsy of a lesion on the left arm (Figure 1) that showed dense dermal infiltrate of neutrophils with scattered background apoptotic nuclear debris without evidence of vasculitis (Figure 2). Gram stain and microbial biopsy cultures were negative for mycobacterial, fungal, and bacterial organisms. Cutaneous lesions failed to respond to courses of intravenous antibiotics. Sarcoidosis workup was unremarkable and was pursued to exclude the association with SS. Other negative testing included antinuclear antibody, human immunodeficiency virus, rheumatoid factor, thyroid-stimulating hormone, Ro and La autoantibodies, cytoplasmic antineutrophil cytoplasmic antibody, perinuclear antineutrophil cytoplasmic antibody, antimitochondrial antibody, and urinalysis. Occult malignancy was excluded with negative bone marrow biopsy; cerebrospinal fluid analysis; esophagogastroduodenoscopy; colonoscopy; and computed tomography of the chest, abdomen, and pelvis.

Sweet syndrome flares in this patient began with a prodromal syndrome of fever, chills, fatigue, diarrhea, and severe local neuropathic pain. Cutaneous lesions erupted 2 days later, most frequently on the arms and fingers. Preemptive treatment with prednisone 30 to 40 mg when the prodrome was present did not arrest cutaneous lesion development. Flares initially occurred every 3 to 5 weeks.

She initially was successfully treated with high-dose prednisone 100 mg daily during SS flares. Prolonged low-dose prednisone maintenance (10–20 mg) and hydroxychloroquine failed to control her frequent exacerbations. Dapsone was intolerable secondary to an adverse reaction. She continued to have frequent exacerbations of the SS requiring hospitalizations.

During SS flares, CVID was stable with infrequent systemic infections. Although a causal relationship between CVID and SS was unclear, an empiric increase in IVIG dose was made by her immunologist to test if it would decrease the frequency of the cutaneous flares. Subsequently, the IVIG dose was increased to 60 g monthly followed by 200 g monthly after approximately 4 months with a partial initial response in the beginning of therapy for the first 6 months. However, episodes resumed with increasing frequency with cutaneous lesion flares every 2 to 3 weeks. In a 3-month period, the patient had at least 4 hospitalizations for SS flares. Finally, 18 months after the diagnosis of SS was made, she was started on metronomic cyclophosphamide at a daily oral dose of 100 mg, later reduced to 50 mg daily after she developed mild neutropenia. She was continued on monthly IVIG replacement at a higher dose of 200 g divided over 2 days for CVID throughout the course of the disease and to the present time. Since then, the frequency of SS flares has notably reduced. She required 1 hospitalization after cyclophosphamide was initiated. She uses short-pulse prednisone (1 mg/kg) for 3 to 5 days when new skin lesions appear in addition to cyclophosphamide.

Common variable immune deficiency, the most common primary immunodeficiency, initially can present in adulthood.^1,2 Its hallmarks include low levels of serum immunoglobulin, most notably IgG with most patients having concurrent deficiencies of IgA and IgM, and impaired antibody responses with recurrent or atypical infections. It has been associated with autoimmune diseases, granulomatous disease, and inflammatory disorders.² Failure to mount protective levels of antibody titer after vaccination demonstrates the deficiency of antibody production.¹ Lack of recognition of this clinical spectrum may lead to delayed diagnosis and more importantly stalls the initiation of immunoglobulin replacement therapy.¹ The customary dose of immunoglobulin replacement is 400 mg/kg given in a single monthly infusion²; however, doses should be individualized and based on clinical response.¹

Sweet syndrome is characterized by the constellation of pyrexia; leukocytosis; and eruption of painful, edematous, dermal, and neutrophil-dense plaques that occur in the setting of infection or malignancy or are drug induced.^3,4 Although not fully elucidated, the pathogenesis is thought to involve the effects of cytokines that precipitate neutrophil activation and infiltration inducing a hypersensitivity reaction and escalation of the immunologic cascade.³ Because SS can represent a paraneoplastic phenomenon or a dermal manifestation of a solid neoplasm or hematologic dyscrasia, it is important to rule out occult malignancy.³ The mainstay of treatment is systemic corticosteroids to which classical SS lesions readily respond. Alternatively, topical or intralesional corticosteroids may be used as adjuvant therapy. Alternate first-line treatments include potassium iodide and colchicine. Second-line therapies include indomethacin, cyclosporine, dapsone, and other immunosuppressive agents.⁵ The lesions may become superinfected with bacterial pathogens requiring antimicrobials.³ Spontaneous resolution seldom occurs. The risk for relapse is lifelong following spontaneous or therapy-induced clinical remission.³ There is a growing body of literature of SS-associated conditions.

Common variable immune deficiency is a collection of disorders resulting in antibody deficiency and recurrent infections.⁶ Despite the humeral defects in CVID, patients paradoxically may develop various autoimmune, hematologic, and inflammatory disorders.⁷ Sweet syndrome, first described in 1964, is a constellation of fever, neutrophilia, and neutrophilic dermatosis of unknown pathogenesis.⁸ Copresentation of CVID and SS has not been commonly reported. O’Regan et al⁸ described a 17-year-old adolescent boy with both SS and CVID but SS preceded the diagnosis of CVID. In our case, the patient presented with CVID first and then manifested SS 1 year later.

Common variable immune deficiency is the most frequent symptomatic primary immunodeficiency in adults. Because adults with CVID have varied manifestations, CVID is thought to be late-onset antibody failure. The genetic basis of these disorders has not been identified in the majority of individuals. More than 100 genetic defects have been ascribed to primary immunodeficiencies,⁹ though none are consistently found to be associated with CVID. The majority of CVID cases are sporadic, but the positive family history in our patient suggests a familial form. Approximately 10% to 20% of patients have an identified heritable cause of CVID.¹⁰ Our patient’s diagnosis of CVID was confirmed by meeting the diagnostic triad set by the European Society for Immunodeficiencies¹¹ of marked reduction of IgG and IgA or IgM plus onset after 2 years of age, recurrent infections, and defective vaccination response. Additional complications including autoimmunity, malignancy, and granulomatous inflammation were extensively ruled out.

The etiology of SS is unknown and its pathogenesis not fully elucidated, though it is presumed to be a hypersensitivity reaction.¹² Sweet syndrome can be classified into 3 major subtypes: classical or idiopathic, malignancy associated, or drug induced.³ Our patient’s presentation is consistent with the classical variant, as malignancy was ruled out and the patient was not on any medication other than IVIG at the time of diagnosis. The treatment of SS consists of systemic steroids, initially high dose followed by a prolonged taper over 4 to 6 weeks.³ This treatment causes a pronounced and sustained decrease in serum IgG due to increased catabolism during drug administration and decreased synthesis during and for a variable time after drug administration.¹³ In refractory cases, intravenous pulse administration of methylprednisolone sodium succinate for 3 to 5 days may enhance the response to standard therapies.⁵

The concurrent development of neutrophilic dermatoses/SS in an individual with CVID has not been fully described. There is a credible association of SS with infections, inflammatory bowel disease, pregnancy, malignancy, and medications, as well as a possible association with Behçet disease, erythema nodosum, relapsing polychondritis, rheumatoid arthritis, sarcoidosis, and thyroid disease.⁵ The association between immunoglobulin deficiencies and SS is markedly unusual. Despite regular IVIG replacement, adequate treatment of CVID did not seem to modulate SS flares in our patient. A case report in a pediatric patient does not provide specific guidance regarding treatment options.⁸

A particularly challenging aspect of our case was tailoring a treatment regimen to suppress SS flares. We have attained partial response to the refractory cutaneous lesions (decreased frequency and amplitude of outbreaks) with IVIG replacement 200 g every 4 weeks in combination with metronomic cyclophosphamide 50 mg daily (use of a repetitive, low-dose daily chemotherapy regimen to minimize side effects). Intermittent SS flares were managed acutely with pulse high-dose steroids. We report a case of SS with CVID, raising the plausibility of correlated pathogenesis. However, the exact mechanisms remain undefined.

To the Editor:

A 38-year-old woman was diagnosed with common variable immune deficiency (CVID) by an immunologist at an outside institution 1 year prior to the current presentation. The diagnosis was based on history of severe recurrent sinopulmonary tract, inner ear, Clostridium difficile, urinary tract, and herpes zoster infections of approximately 6 years’ duration, as well as persistently low IgG, IgA, and IgM levels of 530 mg/dL (reference range, 690–1400 mg/dL), 29 mg/dL (reference range, 88–410 mg/dL), and 30 mg/dL (reference range, 34–210 mg/dL), respectively, with failure to respond to vaccinations (ie, Haemophilus influenzae type B, Streptococcus pneumoniae, diphtheria IgG antibody, tetanus antibody). She was started on replacement intravenous immunoglobulin (IVIG) 40 g monthly (400 mg/kg) for CVID. She had a family history of CVID diagnosed in her son and sister.

One year after the CVID diagnosis, she was diagnosed with Sweet syndrome (SS) by a physician at our institution via biopsy of a lesion on the left arm (Figure 1) that showed dense dermal infiltrate of neutrophils with scattered background apoptotic nuclear debris without evidence of vasculitis (Figure 2). Gram stain and microbial biopsy cultures were negative for mycobacterial, fungal, and bacterial organisms. Cutaneous lesions failed to respond to courses of intravenous antibiotics. Sarcoidosis workup was unremarkable and was pursued to exclude the association with SS. Other negative testing included antinuclear antibody, human immunodeficiency virus, rheumatoid factor, thyroid-stimulating hormone, Ro and La autoantibodies, cytoplasmic antineutrophil cytoplasmic antibody, perinuclear antineutrophil cytoplasmic antibody, antimitochondrial antibody, and urinalysis. Occult malignancy was excluded with negative bone marrow biopsy; cerebrospinal fluid analysis; esophagogastroduodenoscopy; colonoscopy; and computed tomography of the chest, abdomen, and pelvis.

Sweet syndrome flares in this patient began with a prodromal syndrome of fever, chills, fatigue, diarrhea, and severe local neuropathic pain. Cutaneous lesions erupted 2 days later, most frequently on the arms and fingers. Preemptive treatment with prednisone 30 to 40 mg when the prodrome was present did not arrest cutaneous lesion development. Flares initially occurred every 3 to 5 weeks.

She initially was successfully treated with high-dose prednisone 100 mg daily during SS flares. Prolonged low-dose prednisone maintenance (10–20 mg) and hydroxychloroquine failed to control her frequent exacerbations. Dapsone was intolerable secondary to an adverse reaction. She continued to have frequent exacerbations of the SS requiring hospitalizations.

During SS flares, CVID was stable with infrequent systemic infections. Although a causal relationship between CVID and SS was unclear, an empiric increase in IVIG dose was made by her immunologist to test if it would decrease the frequency of the cutaneous flares. Subsequently, the IVIG dose was increased to 60 g monthly followed by 200 g monthly after approximately 4 months with a partial initial response in the beginning of therapy for the first 6 months. However, episodes resumed with increasing frequency with cutaneous lesion flares every 2 to 3 weeks. In a 3-month period, the patient had at least 4 hospitalizations for SS flares. Finally, 18 months after the diagnosis of SS was made, she was started on metronomic cyclophosphamide at a daily oral dose of 100 mg, later reduced to 50 mg daily after she developed mild neutropenia. She was continued on monthly IVIG replacement at a higher dose of 200 g divided over 2 days for CVID throughout the course of the disease and to the present time. Since then, the frequency of SS flares has notably reduced. She required 1 hospitalization after cyclophosphamide was initiated. She uses short-pulse prednisone (1 mg/kg) for 3 to 5 days when new skin lesions appear in addition to cyclophosphamide.

Common variable immune deficiency, the most common primary immunodeficiency, initially can present in adulthood.^1,2 Its hallmarks include low levels of serum immunoglobulin, most notably IgG with most patients having concurrent deficiencies of IgA and IgM, and impaired antibody responses with recurrent or atypical infections. It has been associated with autoimmune diseases, granulomatous disease, and inflammatory disorders.² Failure to mount protective levels of antibody titer after vaccination demonstrates the deficiency of antibody production.¹ Lack of recognition of this clinical spectrum may lead to delayed diagnosis and more importantly stalls the initiation of immunoglobulin replacement therapy.¹ The customary dose of immunoglobulin replacement is 400 mg/kg given in a single monthly infusion²; however, doses should be individualized and based on clinical response.¹

Sweet syndrome is characterized by the constellation of pyrexia; leukocytosis; and eruption of painful, edematous, dermal, and neutrophil-dense plaques that occur in the setting of infection or malignancy or are drug induced.^3,4 Although not fully elucidated, the pathogenesis is thought to involve the effects of cytokines that precipitate neutrophil activation and infiltration inducing a hypersensitivity reaction and escalation of the immunologic cascade.³ Because SS can represent a paraneoplastic phenomenon or a dermal manifestation of a solid neoplasm or hematologic dyscrasia, it is important to rule out occult malignancy.³ The mainstay of treatment is systemic corticosteroids to which classical SS lesions readily respond. Alternatively, topical or intralesional corticosteroids may be used as adjuvant therapy. Alternate first-line treatments include potassium iodide and colchicine. Second-line therapies include indomethacin, cyclosporine, dapsone, and other immunosuppressive agents.⁵ The lesions may become superinfected with bacterial pathogens requiring antimicrobials.³ Spontaneous resolution seldom occurs. The risk for relapse is lifelong following spontaneous or therapy-induced clinical remission.³ There is a growing body of literature of SS-associated conditions.

Common variable immune deficiency is a collection of disorders resulting in antibody deficiency and recurrent infections.⁶ Despite the humeral defects in CVID, patients paradoxically may develop various autoimmune, hematologic, and inflammatory disorders.⁷ Sweet syndrome, first described in 1964, is a constellation of fever, neutrophilia, and neutrophilic dermatosis of unknown pathogenesis.⁸ Copresentation of CVID and SS has not been commonly reported. O’Regan et al⁸ described a 17-year-old adolescent boy with both SS and CVID but SS preceded the diagnosis of CVID. In our case, the patient presented with CVID first and then manifested SS 1 year later.

Common variable immune deficiency is the most frequent symptomatic primary immunodeficiency in adults. Because adults with CVID have varied manifestations, CVID is thought to be late-onset antibody failure. The genetic basis of these disorders has not been identified in the majority of individuals. More than 100 genetic defects have been ascribed to primary immunodeficiencies,⁹ though none are consistently found to be associated with CVID. The majority of CVID cases are sporadic, but the positive family history in our patient suggests a familial form. Approximately 10% to 20% of patients have an identified heritable cause of CVID.¹⁰ Our patient’s diagnosis of CVID was confirmed by meeting the diagnostic triad set by the European Society for Immunodeficiencies¹¹ of marked reduction of IgG and IgA or IgM plus onset after 2 years of age, recurrent infections, and defective vaccination response. Additional complications including autoimmunity, malignancy, and granulomatous inflammation were extensively ruled out.

The etiology of SS is unknown and its pathogenesis not fully elucidated, though it is presumed to be a hypersensitivity reaction.¹² Sweet syndrome can be classified into 3 major subtypes: classical or idiopathic, malignancy associated, or drug induced.³ Our patient’s presentation is consistent with the classical variant, as malignancy was ruled out and the patient was not on any medication other than IVIG at the time of diagnosis. The treatment of SS consists of systemic steroids, initially high dose followed by a prolonged taper over 4 to 6 weeks.³ This treatment causes a pronounced and sustained decrease in serum IgG due to increased catabolism during drug administration and decreased synthesis during and for a variable time after drug administration.¹³ In refractory cases, intravenous pulse administration of methylprednisolone sodium succinate for 3 to 5 days may enhance the response to standard therapies.⁵

The concurrent development of neutrophilic dermatoses/SS in an individual with CVID has not been fully described. There is a credible association of SS with infections, inflammatory bowel disease, pregnancy, malignancy, and medications, as well as a possible association with Behçet disease, erythema nodosum, relapsing polychondritis, rheumatoid arthritis, sarcoidosis, and thyroid disease.⁵ The association between immunoglobulin deficiencies and SS is markedly unusual. Despite regular IVIG replacement, adequate treatment of CVID did not seem to modulate SS flares in our patient. A case report in a pediatric patient does not provide specific guidance regarding treatment options.⁸

A particularly challenging aspect of our case was tailoring a treatment regimen to suppress SS flares. We have attained partial response to the refractory cutaneous lesions (decreased frequency and amplitude of outbreaks) with IVIG replacement 200 g every 4 weeks in combination with metronomic cyclophosphamide 50 mg daily (use of a repetitive, low-dose daily chemotherapy regimen to minimize side effects). Intermittent SS flares were managed acutely with pulse high-dose steroids. We report a case of SS with CVID, raising the plausibility of correlated pathogenesis. However, the exact mechanisms remain undefined.

Greed kills babies. Children’s lives matter. Children over profit.

These were the slogans proclaimed by signs carried by protesters outside of MedStar Franklin Square Medical Center in Baltimore in early May of 2018 to protest the closure of the dedicated pediatric emergency department and inpatient pediatric unit.

But administrators at Franklin Square Medical Center had made their decision long before the glue had dried on the signs, and the protests of patients and community officials fell on deaf ears. Eight doctors and 30 other staff had already lost their jobs, including the chair of pediatrics, Scott Krugman, MD.¹

And this was just another drop in a slow ooze of pediatric inpatient units based in community hospitals that have seen the ax fall on what was thought to be a vital medical resource for their communities – yet not vital enough to survive its lack of profitability. From Taunton, Mass., to Chicago, Ill., to rural Tennessee, pediatric inpatient units in community hospitals have failed to even flirt with breaking even, let alone show profitability. Many community pediatric inpatient units are saddled with rock-bottom reimbursements offered by state Medicaid programs, the overwhelmingly prevalent payer for pediatric hospitalizations, which is compounded by the seasonality and unpredictability of pediatric inpatient volumes, so many have seen a glowing red bottom line lead to their demise.

What does this mean for pediatric health in underserved and rural communities? The closure of the pediatric inpatient unit at MedStar Franklin Square Medical Center meant the loss of physicians and nurses staffing the child protection team helping to assist the local district attorney in child abuse cases. Sometimes described as “secondary care,” community pediatric hospitalists also serve as a link between primary care providers and tertiary care subspecialists; they can serve as pediatric generalists throughout a hospital and provide newborn nursery care, delivery room resuscitations, ED consultations, procedural sedations, psychiatric unit support, surgical comanagement, and informal or formal outpatient consultations.² Losing even a small inpatient pediatric unit can have a ripple effect on inpatient and outpatient pediatric services in a health system and community.

For patients and their caregivers, the loss of pediatric inpatient services in their community hospital can erect additional hurdles to appropriate health care. The need to travel longer distances to urban centers or even the other side of town can be challenging given the difficulties posed by long distances, traffic congestion, public transportation, or just parking.³ For patients suffering from longer hospitalizations caused by medical complexity or chronic illnesses, traveling long distances can exacerbate the caregiver stress from attempting to care for a family at home while participating in the care of a hospitalized child. Longer travel times can also worsen family stress by increasing a caregiver’s absence from home and increased nonmedical expenses, not to mention loss of wages.⁴ Comfort levels with inpatient providers can also suffer because most pediatric units in community hospitals are staffed by either community general pediatricians or very small pediatric hospitalist groups, which breeds familiarity with frequently admitted patients and their caregivers. This familiarity can be lacking in large academic centers, with confusing and ever-rotating teams of academic hospitalists, residents, and medical students.⁵

What is driving the slow drumbeat of pediatric inpatient unit closures? On a macroeconomic scale, pediatric hospitalizations have been dropping yearly, driven down by immunizations (despite the best efforts of certain celebrities), antibiotic stewardship, and improved access to outpatient care. In 2006, there were 6.6 million hospitalizations for children aged 17 years and younger,⁶ but by 2012 this had dropped to 5.9 million hospitalizations.⁷ In the same age group, the rate of hospitalization from the ED dropped from 4.4% in 2006 to 3.2% in 2015.⁸

On a hospital level, the presence of multiple small pediatric units in a region may not make sense from a cost standpoint, and a larger, merged unit may provide higher quality because of its higher volumes. On a state and local level, alternative payment models have been implemented with the best of intentions but have led administrators at community general hospitals to look at pediatric units as the lowest hanging money-losing fruit in their efforts to survive a brave new world of hospital payment.

The most extreme (or advanced, depending on your viewpoint) model is in Maryland: Since 2014, acute care hospitals have been only able to receive a fixed amount of revenue from all payers, including Medicare, Medicaid, and commercial insurers.⁹ Known as an all-payer global budget, it incentivizes lowering unnecessary costs of care, such as readmissions, but also encourages cauterization of cost centers hemorrhaging money – such as inpatient pediatrics. Even the venerable Johns Hopkins Children’s Center has seen its profitability pale in comparison to the expansion team Johns Hopkins All Children’s Hospital in St. Petersburg, Fla., which is the second-most profitable hospital in the Hopkins system, only edged out by Sibley Memorial Hospital – which also sports an out-of-state location in the District of Columbia.¹⁰

But all hope is not lost for your comfy local pediatric inpatient unit. In other states and regions where a more favorable (to hospitals) payer mix exists, large pediatric hospitals are still engaged in turf battles with other local competitors to grab market share. In these regions, community pediatric units have survived by partnering with large pediatric institutions, either through affiliations or wholesale transplantation of the larger pediatric institution’s providers, nurses, and EHRs into essentially what is a leased floor. In addition, large pediatric institutions that participate in capitated models such as accountable care organizations have paradoxically found it financially favorable to direct “bread-and-butter” pediatric hospitalizations to community pediatric units, which often provide the same care at a lower cost.

Utilizing community inpatient pediatric units was “initially … a means of expanding their market share and ‘downstream’ revenue from transfers, but more commonly now [is] a way of alleviating the costs associated with admitting low to moderate acuity patients to the main tertiary sites,” said Francisco Alvarez, MD, associate chief of Regional Pediatric Hospital Medicine Programs at Lucile Packard Children’s Hospital in Palo Alto, Calif. “The cost of care provided by pediatric hospitals has always been higher than the average cost for nonpediatric hospitals in regard to caring for pediatric patients due to their highly skilled specialties and services. These have become more scrutinized by private and government insurance plans and, in some cases, have led to lower reimbursements and therefore a lower or deficient net revenue for certain patient populations.”

For community pediatric hospitalists, the shifting sands of reimbursement on which pediatric inpatient care is built can be a motion illness–inducing experience. In addition to concerns over community health care, job security, and population health, care provided in community hospitals can often be subtly undercut by tertiary and quaternary care pediatric hospitals.

“The focus of pediatric residency programs in freestanding children’s hospitals has created a situation where new pediatricians have less opportunity to develop respect for community pediatric hospital medicine,” said Beth Natt, MD, director of pediatric hospital medicine in the Regional Programs at Connecticut Children’s Medical Center, Hartford. “We are the nameless ‘OSH,’ the place that gets ‘Monday-morning quarterbacked’ in resident morning reports without having a voice at the table. Add this to residents learning ‘only’ protocolized care as opposed to a spectrum of appropriate care, and we create a culture of ‘wrong and right’ with the backward nonprotocol driven community docs looking like they are practicing medicine in the Wild West.”

What’s a community pediatric hospitalist to do, faced with an uncertain future and diminishing respect? Continuing to partner with local pediatric providers, community leaders, and local health care advocacy groups will help to enmesh inpatient providers in the fabric of a community’s health care. But making the value case to hospital administrators is critical for community pediatric hospitalists, as adult hospitalists realized soon after the inception of the hospitalist field.

Goals valued by hospital administrators are pursued on a daily basis by community pediatric hospitalists, and these successes need to be brought to light. Achieving value and quality metrics, pursuing high-value care, reducing readmission rates, championing EHRs, and improving documentation are goals that community pediatric hospitalists and hospital administrators can work toward together.¹¹ By pursuing and sharing success in meeting these shared goals, perhaps the local community pediatric inpatient unit can survive – and thrive.

As for Dr. Krugman, he has moved on and is soon to be gainfully employed again. But he continues to be focused, as always, on the health of his patients.

“What are we going to do to take care of kids in their own communities?” Dr. Krugman asked. “It’s going to be an increasing challenge over the next decade due to the consolidation of children’s hospitals and low payments, especially for hospitals that are adult-focused. Unless we find a way to pay for pediatric care as a country.”

Dr. Chang is a pediatric hospitalist at Baystate Children’s Hospital in Springfield, Mass., and is the pediatric editor of The Hospitalist.

References

1. McDaniels A. (2018). Protesters denounce reduction in pediatric services at Baltimore’s MedStar Franklin Square hospital. Baltimore Sun. Available at: http://www.baltimoresun.com/health/health-care/bs-hs-franklin-square-hospital-protest-20180508-story.html.

2. Roberts KB. Pediatric hospitalists in community hospitals: Hospital-based generalists with expanded roles. Hosp Pediatr. 2015 May;5(5):290-2.

3. Georgia Health News. (2018). A hospital crisis is killing rural communities. This state is ‘Ground Zero’. Available at: http://www.georgiahealthnews.com/2017/09/hospital-crisis-killing-rural-communities-state-ground-zero/.

4. DiFazio RL et al. Non-medical out-of-pocket expenses incurred by families during their child’s hospitalization. J Child Health Care. 2013 Sep;17(3):230-41.

5. Gunderman R. Hospitalist and the decline of comprehensive care. N Engl J Med. 2016 Sep 15; 375(11):1011-3.

6. Statistical Brief #56. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb56.jsp.

7. Overview of Hospital Stays for Children in the United States, 2012 #187. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb187-Hospital-Stays-Children-2012.jsp.

8. Trends in Hospital Inpatient Stays by Age and Payer, 2000-2015 #235. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb235-Inpatient-Stays-Age-Payer-Trends.jsp.

9. Maryland All-Payer Model | Center for Medicare & Medicaid Innovation. (2018). Retrieved from https://innovation.cms.gov/initiatives/Maryland-All-Payer-Model/.

10. The effects of Maryland’s unique health care system. (2018). Retrieved from https://www.axios.com/johns-hopkins-finances-maryland-1518553853-722c2195-731e-4e02-ab1e-94e4211ba945.html.

11. The Increasing Need for Hospitalist Programs to Demonstrate Value | SCP. (2018). Retrieved from https://www.schumacherclinical.com/providers/blog/the-increasing-need-for-hospitalist-programs-to-demonstrate-value.
 

Greed kills babies. Children’s lives matter. Children over profit.

These were the slogans proclaimed by signs carried by protesters outside of MedStar Franklin Square Medical Center in Baltimore in early May of 2018 to protest the closure of the dedicated pediatric emergency department and inpatient pediatric unit.

But administrators at Franklin Square Medical Center had made their decision long before the glue had dried on the signs, and the protests of patients and community officials fell on deaf ears. Eight doctors and 30 other staff had already lost their jobs, including the chair of pediatrics, Scott Krugman, MD.¹

And this was just another drop in a slow ooze of pediatric inpatient units based in community hospitals that have seen the ax fall on what was thought to be a vital medical resource for their communities – yet not vital enough to survive its lack of profitability. From Taunton, Mass., to Chicago, Ill., to rural Tennessee, pediatric inpatient units in community hospitals have failed to even flirt with breaking even, let alone show profitability. Many community pediatric inpatient units are saddled with rock-bottom reimbursements offered by state Medicaid programs, the overwhelmingly prevalent payer for pediatric hospitalizations, which is compounded by the seasonality and unpredictability of pediatric inpatient volumes, so many have seen a glowing red bottom line lead to their demise.

What does this mean for pediatric health in underserved and rural communities? The closure of the pediatric inpatient unit at MedStar Franklin Square Medical Center meant the loss of physicians and nurses staffing the child protection team helping to assist the local district attorney in child abuse cases. Sometimes described as “secondary care,” community pediatric hospitalists also serve as a link between primary care providers and tertiary care subspecialists; they can serve as pediatric generalists throughout a hospital and provide newborn nursery care, delivery room resuscitations, ED consultations, procedural sedations, psychiatric unit support, surgical comanagement, and informal or formal outpatient consultations.² Losing even a small inpatient pediatric unit can have a ripple effect on inpatient and outpatient pediatric services in a health system and community.

For patients and their caregivers, the loss of pediatric inpatient services in their community hospital can erect additional hurdles to appropriate health care. The need to travel longer distances to urban centers or even the other side of town can be challenging given the difficulties posed by long distances, traffic congestion, public transportation, or just parking.³ For patients suffering from longer hospitalizations caused by medical complexity or chronic illnesses, traveling long distances can exacerbate the caregiver stress from attempting to care for a family at home while participating in the care of a hospitalized child. Longer travel times can also worsen family stress by increasing a caregiver’s absence from home and increased nonmedical expenses, not to mention loss of wages.⁴ Comfort levels with inpatient providers can also suffer because most pediatric units in community hospitals are staffed by either community general pediatricians or very small pediatric hospitalist groups, which breeds familiarity with frequently admitted patients and their caregivers. This familiarity can be lacking in large academic centers, with confusing and ever-rotating teams of academic hospitalists, residents, and medical students.⁵

What is driving the slow drumbeat of pediatric inpatient unit closures? On a macroeconomic scale, pediatric hospitalizations have been dropping yearly, driven down by immunizations (despite the best efforts of certain celebrities), antibiotic stewardship, and improved access to outpatient care. In 2006, there were 6.6 million hospitalizations for children aged 17 years and younger,⁶ but by 2012 this had dropped to 5.9 million hospitalizations.⁷ In the same age group, the rate of hospitalization from the ED dropped from 4.4% in 2006 to 3.2% in 2015.⁸

On a hospital level, the presence of multiple small pediatric units in a region may not make sense from a cost standpoint, and a larger, merged unit may provide higher quality because of its higher volumes. On a state and local level, alternative payment models have been implemented with the best of intentions but have led administrators at community general hospitals to look at pediatric units as the lowest hanging money-losing fruit in their efforts to survive a brave new world of hospital payment.

The most extreme (or advanced, depending on your viewpoint) model is in Maryland: Since 2014, acute care hospitals have been only able to receive a fixed amount of revenue from all payers, including Medicare, Medicaid, and commercial insurers.⁹ Known as an all-payer global budget, it incentivizes lowering unnecessary costs of care, such as readmissions, but also encourages cauterization of cost centers hemorrhaging money – such as inpatient pediatrics. Even the venerable Johns Hopkins Children’s Center has seen its profitability pale in comparison to the expansion team Johns Hopkins All Children’s Hospital in St. Petersburg, Fla., which is the second-most profitable hospital in the Hopkins system, only edged out by Sibley Memorial Hospital – which also sports an out-of-state location in the District of Columbia.¹⁰

But all hope is not lost for your comfy local pediatric inpatient unit. In other states and regions where a more favorable (to hospitals) payer mix exists, large pediatric hospitals are still engaged in turf battles with other local competitors to grab market share. In these regions, community pediatric units have survived by partnering with large pediatric institutions, either through affiliations or wholesale transplantation of the larger pediatric institution’s providers, nurses, and EHRs into essentially what is a leased floor. In addition, large pediatric institutions that participate in capitated models such as accountable care organizations have paradoxically found it financially favorable to direct “bread-and-butter” pediatric hospitalizations to community pediatric units, which often provide the same care at a lower cost.

Utilizing community inpatient pediatric units was “initially … a means of expanding their market share and ‘downstream’ revenue from transfers, but more commonly now [is] a way of alleviating the costs associated with admitting low to moderate acuity patients to the main tertiary sites,” said Francisco Alvarez, MD, associate chief of Regional Pediatric Hospital Medicine Programs at Lucile Packard Children’s Hospital in Palo Alto, Calif. “The cost of care provided by pediatric hospitals has always been higher than the average cost for nonpediatric hospitals in regard to caring for pediatric patients due to their highly skilled specialties and services. These have become more scrutinized by private and government insurance plans and, in some cases, have led to lower reimbursements and therefore a lower or deficient net revenue for certain patient populations.”

For community pediatric hospitalists, the shifting sands of reimbursement on which pediatric inpatient care is built can be a motion illness–inducing experience. In addition to concerns over community health care, job security, and population health, care provided in community hospitals can often be subtly undercut by tertiary and quaternary care pediatric hospitals.

“The focus of pediatric residency programs in freestanding children’s hospitals has created a situation where new pediatricians have less opportunity to develop respect for community pediatric hospital medicine,” said Beth Natt, MD, director of pediatric hospital medicine in the Regional Programs at Connecticut Children’s Medical Center, Hartford. “We are the nameless ‘OSH,’ the place that gets ‘Monday-morning quarterbacked’ in resident morning reports without having a voice at the table. Add this to residents learning ‘only’ protocolized care as opposed to a spectrum of appropriate care, and we create a culture of ‘wrong and right’ with the backward nonprotocol driven community docs looking like they are practicing medicine in the Wild West.”

What’s a community pediatric hospitalist to do, faced with an uncertain future and diminishing respect? Continuing to partner with local pediatric providers, community leaders, and local health care advocacy groups will help to enmesh inpatient providers in the fabric of a community’s health care. But making the value case to hospital administrators is critical for community pediatric hospitalists, as adult hospitalists realized soon after the inception of the hospitalist field.

Goals valued by hospital administrators are pursued on a daily basis by community pediatric hospitalists, and these successes need to be brought to light. Achieving value and quality metrics, pursuing high-value care, reducing readmission rates, championing EHRs, and improving documentation are goals that community pediatric hospitalists and hospital administrators can work toward together.¹¹ By pursuing and sharing success in meeting these shared goals, perhaps the local community pediatric inpatient unit can survive – and thrive.

As for Dr. Krugman, he has moved on and is soon to be gainfully employed again. But he continues to be focused, as always, on the health of his patients.

“What are we going to do to take care of kids in their own communities?” Dr. Krugman asked. “It’s going to be an increasing challenge over the next decade due to the consolidation of children’s hospitals and low payments, especially for hospitals that are adult-focused. Unless we find a way to pay for pediatric care as a country.”

Dr. Chang is a pediatric hospitalist at Baystate Children’s Hospital in Springfield, Mass., and is the pediatric editor of The Hospitalist.

References

1. McDaniels A. (2018). Protesters denounce reduction in pediatric services at Baltimore’s MedStar Franklin Square hospital. Baltimore Sun. Available at: http://www.baltimoresun.com/health/health-care/bs-hs-franklin-square-hospital-protest-20180508-story.html.

2. Roberts KB. Pediatric hospitalists in community hospitals: Hospital-based generalists with expanded roles. Hosp Pediatr. 2015 May;5(5):290-2.

3. Georgia Health News. (2018). A hospital crisis is killing rural communities. This state is ‘Ground Zero’. Available at: http://www.georgiahealthnews.com/2017/09/hospital-crisis-killing-rural-communities-state-ground-zero/.

4. DiFazio RL et al. Non-medical out-of-pocket expenses incurred by families during their child’s hospitalization. J Child Health Care. 2013 Sep;17(3):230-41.

5. Gunderman R. Hospitalist and the decline of comprehensive care. N Engl J Med. 2016 Sep 15; 375(11):1011-3.

6. Statistical Brief #56. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb56.jsp.

7. Overview of Hospital Stays for Children in the United States, 2012 #187. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb187-Hospital-Stays-Children-2012.jsp.

8. Trends in Hospital Inpatient Stays by Age and Payer, 2000-2015 #235. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb235-Inpatient-Stays-Age-Payer-Trends.jsp.

9. Maryland All-Payer Model | Center for Medicare & Medicaid Innovation. (2018). Retrieved from https://innovation.cms.gov/initiatives/Maryland-All-Payer-Model/.

10. The effects of Maryland’s unique health care system. (2018). Retrieved from https://www.axios.com/johns-hopkins-finances-maryland-1518553853-722c2195-731e-4e02-ab1e-94e4211ba945.html.

11. The Increasing Need for Hospitalist Programs to Demonstrate Value | SCP. (2018). Retrieved from https://www.schumacherclinical.com/providers/blog/the-increasing-need-for-hospitalist-programs-to-demonstrate-value.
 

Greed kills babies. Children’s lives matter. Children over profit.

These were the slogans proclaimed by signs carried by protesters outside of MedStar Franklin Square Medical Center in Baltimore in early May of 2018 to protest the closure of the dedicated pediatric emergency department and inpatient pediatric unit.

But administrators at Franklin Square Medical Center had made their decision long before the glue had dried on the signs, and the protests of patients and community officials fell on deaf ears. Eight doctors and 30 other staff had already lost their jobs, including the chair of pediatrics, Scott Krugman, MD.¹

And this was just another drop in a slow ooze of pediatric inpatient units based in community hospitals that have seen the ax fall on what was thought to be a vital medical resource for their communities – yet not vital enough to survive its lack of profitability. From Taunton, Mass., to Chicago, Ill., to rural Tennessee, pediatric inpatient units in community hospitals have failed to even flirt with breaking even, let alone show profitability. Many community pediatric inpatient units are saddled with rock-bottom reimbursements offered by state Medicaid programs, the overwhelmingly prevalent payer for pediatric hospitalizations, which is compounded by the seasonality and unpredictability of pediatric inpatient volumes, so many have seen a glowing red bottom line lead to their demise.

What does this mean for pediatric health in underserved and rural communities? The closure of the pediatric inpatient unit at MedStar Franklin Square Medical Center meant the loss of physicians and nurses staffing the child protection team helping to assist the local district attorney in child abuse cases. Sometimes described as “secondary care,” community pediatric hospitalists also serve as a link between primary care providers and tertiary care subspecialists; they can serve as pediatric generalists throughout a hospital and provide newborn nursery care, delivery room resuscitations, ED consultations, procedural sedations, psychiatric unit support, surgical comanagement, and informal or formal outpatient consultations.² Losing even a small inpatient pediatric unit can have a ripple effect on inpatient and outpatient pediatric services in a health system and community.

For patients and their caregivers, the loss of pediatric inpatient services in their community hospital can erect additional hurdles to appropriate health care. The need to travel longer distances to urban centers or even the other side of town can be challenging given the difficulties posed by long distances, traffic congestion, public transportation, or just parking.³ For patients suffering from longer hospitalizations caused by medical complexity or chronic illnesses, traveling long distances can exacerbate the caregiver stress from attempting to care for a family at home while participating in the care of a hospitalized child. Longer travel times can also worsen family stress by increasing a caregiver’s absence from home and increased nonmedical expenses, not to mention loss of wages.⁴ Comfort levels with inpatient providers can also suffer because most pediatric units in community hospitals are staffed by either community general pediatricians or very small pediatric hospitalist groups, which breeds familiarity with frequently admitted patients and their caregivers. This familiarity can be lacking in large academic centers, with confusing and ever-rotating teams of academic hospitalists, residents, and medical students.⁵

What is driving the slow drumbeat of pediatric inpatient unit closures? On a macroeconomic scale, pediatric hospitalizations have been dropping yearly, driven down by immunizations (despite the best efforts of certain celebrities), antibiotic stewardship, and improved access to outpatient care. In 2006, there were 6.6 million hospitalizations for children aged 17 years and younger,⁶ but by 2012 this had dropped to 5.9 million hospitalizations.⁷ In the same age group, the rate of hospitalization from the ED dropped from 4.4% in 2006 to 3.2% in 2015.⁸

On a hospital level, the presence of multiple small pediatric units in a region may not make sense from a cost standpoint, and a larger, merged unit may provide higher quality because of its higher volumes. On a state and local level, alternative payment models have been implemented with the best of intentions but have led administrators at community general hospitals to look at pediatric units as the lowest hanging money-losing fruit in their efforts to survive a brave new world of hospital payment.

The most extreme (or advanced, depending on your viewpoint) model is in Maryland: Since 2014, acute care hospitals have been only able to receive a fixed amount of revenue from all payers, including Medicare, Medicaid, and commercial insurers.⁹ Known as an all-payer global budget, it incentivizes lowering unnecessary costs of care, such as readmissions, but also encourages cauterization of cost centers hemorrhaging money – such as inpatient pediatrics. Even the venerable Johns Hopkins Children’s Center has seen its profitability pale in comparison to the expansion team Johns Hopkins All Children’s Hospital in St. Petersburg, Fla., which is the second-most profitable hospital in the Hopkins system, only edged out by Sibley Memorial Hospital – which also sports an out-of-state location in the District of Columbia.¹⁰

But all hope is not lost for your comfy local pediatric inpatient unit. In other states and regions where a more favorable (to hospitals) payer mix exists, large pediatric hospitals are still engaged in turf battles with other local competitors to grab market share. In these regions, community pediatric units have survived by partnering with large pediatric institutions, either through affiliations or wholesale transplantation of the larger pediatric institution’s providers, nurses, and EHRs into essentially what is a leased floor. In addition, large pediatric institutions that participate in capitated models such as accountable care organizations have paradoxically found it financially favorable to direct “bread-and-butter” pediatric hospitalizations to community pediatric units, which often provide the same care at a lower cost.

Utilizing community inpatient pediatric units was “initially … a means of expanding their market share and ‘downstream’ revenue from transfers, but more commonly now [is] a way of alleviating the costs associated with admitting low to moderate acuity patients to the main tertiary sites,” said Francisco Alvarez, MD, associate chief of Regional Pediatric Hospital Medicine Programs at Lucile Packard Children’s Hospital in Palo Alto, Calif. “The cost of care provided by pediatric hospitals has always been higher than the average cost for nonpediatric hospitals in regard to caring for pediatric patients due to their highly skilled specialties and services. These have become more scrutinized by private and government insurance plans and, in some cases, have led to lower reimbursements and therefore a lower or deficient net revenue for certain patient populations.”

For community pediatric hospitalists, the shifting sands of reimbursement on which pediatric inpatient care is built can be a motion illness–inducing experience. In addition to concerns over community health care, job security, and population health, care provided in community hospitals can often be subtly undercut by tertiary and quaternary care pediatric hospitals.

“The focus of pediatric residency programs in freestanding children’s hospitals has created a situation where new pediatricians have less opportunity to develop respect for community pediatric hospital medicine,” said Beth Natt, MD, director of pediatric hospital medicine in the Regional Programs at Connecticut Children’s Medical Center, Hartford. “We are the nameless ‘OSH,’ the place that gets ‘Monday-morning quarterbacked’ in resident morning reports without having a voice at the table. Add this to residents learning ‘only’ protocolized care as opposed to a spectrum of appropriate care, and we create a culture of ‘wrong and right’ with the backward nonprotocol driven community docs looking like they are practicing medicine in the Wild West.”

What’s a community pediatric hospitalist to do, faced with an uncertain future and diminishing respect? Continuing to partner with local pediatric providers, community leaders, and local health care advocacy groups will help to enmesh inpatient providers in the fabric of a community’s health care. But making the value case to hospital administrators is critical for community pediatric hospitalists, as adult hospitalists realized soon after the inception of the hospitalist field.

Goals valued by hospital administrators are pursued on a daily basis by community pediatric hospitalists, and these successes need to be brought to light. Achieving value and quality metrics, pursuing high-value care, reducing readmission rates, championing EHRs, and improving documentation are goals that community pediatric hospitalists and hospital administrators can work toward together.¹¹ By pursuing and sharing success in meeting these shared goals, perhaps the local community pediatric inpatient unit can survive – and thrive.

As for Dr. Krugman, he has moved on and is soon to be gainfully employed again. But he continues to be focused, as always, on the health of his patients.

“What are we going to do to take care of kids in their own communities?” Dr. Krugman asked. “It’s going to be an increasing challenge over the next decade due to the consolidation of children’s hospitals and low payments, especially for hospitals that are adult-focused. Unless we find a way to pay for pediatric care as a country.”

Dr. Chang is a pediatric hospitalist at Baystate Children’s Hospital in Springfield, Mass., and is the pediatric editor of The Hospitalist.

References

1. McDaniels A. (2018). Protesters denounce reduction in pediatric services at Baltimore’s MedStar Franklin Square hospital. Baltimore Sun. Available at: http://www.baltimoresun.com/health/health-care/bs-hs-franklin-square-hospital-protest-20180508-story.html.

2. Roberts KB. Pediatric hospitalists in community hospitals: Hospital-based generalists with expanded roles. Hosp Pediatr. 2015 May;5(5):290-2.

3. Georgia Health News. (2018). A hospital crisis is killing rural communities. This state is ‘Ground Zero’. Available at: http://www.georgiahealthnews.com/2017/09/hospital-crisis-killing-rural-communities-state-ground-zero/.

4. DiFazio RL et al. Non-medical out-of-pocket expenses incurred by families during their child’s hospitalization. J Child Health Care. 2013 Sep;17(3):230-41.

5. Gunderman R. Hospitalist and the decline of comprehensive care. N Engl J Med. 2016 Sep 15; 375(11):1011-3.

6. Statistical Brief #56. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb56.jsp.

7. Overview of Hospital Stays for Children in the United States, 2012 #187. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb187-Hospital-Stays-Children-2012.jsp.

8. Trends in Hospital Inpatient Stays by Age and Payer, 2000-2015 #235. (2018). Retrieved from https://www.hcup-us.ahrq.gov/reports/statbriefs/sb235-Inpatient-Stays-Age-Payer-Trends.jsp.

9. Maryland All-Payer Model | Center for Medicare & Medicaid Innovation. (2018). Retrieved from https://innovation.cms.gov/initiatives/Maryland-All-Payer-Model/.

10. The effects of Maryland’s unique health care system. (2018). Retrieved from https://www.axios.com/johns-hopkins-finances-maryland-1518553853-722c2195-731e-4e02-ab1e-94e4211ba945.html.

11. The Increasing Need for Hospitalist Programs to Demonstrate Value | SCP. (2018). Retrieved from https://www.schumacherclinical.com/providers/blog/the-increasing-need-for-hospitalist-programs-to-demonstrate-value.
 

Digital technology use by parents during family activities may exacerbate internalizing or externalizing behavior in their children, according to results published in Pediatric Research.

In a study of 183 couples with children aged 5 years or younger, mothers perceived an average of 1.65 devices as interfering in their interactions with their child at least once per day, compared with an average of 1.43 devices per day for fathers. In addition, 56% of mothers and 43% of fathers reported that two or more devices interrupted their parent-child activities on a daily basis.

Higher technology interference (“technoference”) was associated with greater externalizing and internalizing behaviors in children and higher parenting stress for both mothers and fathers. Technoference also was associated with lower coparenting quality in fathers only, and greater parent depressive symptoms in mothers only, reported Brandon T. McDaniel, PhD, of Illinois State University, Normal, and Jenny S. Radesky, MD, of the University of Michigan, Ann Arbor.

Zinkevych/iStock/Getty Images

SOURCE: McDaniel B et al. Pediatr Res. 2018. doi: 10.1038/s41390-018-0052-6.

Digital technology use by parents during family activities may exacerbate internalizing or externalizing behavior in their children, according to results published in Pediatric Research.

In a study of 183 couples with children aged 5 years or younger, mothers perceived an average of 1.65 devices as interfering in their interactions with their child at least once per day, compared with an average of 1.43 devices per day for fathers. In addition, 56% of mothers and 43% of fathers reported that two or more devices interrupted their parent-child activities on a daily basis.

Higher technology interference (“technoference”) was associated with greater externalizing and internalizing behaviors in children and higher parenting stress for both mothers and fathers. Technoference also was associated with lower coparenting quality in fathers only, and greater parent depressive symptoms in mothers only, reported Brandon T. McDaniel, PhD, of Illinois State University, Normal, and Jenny S. Radesky, MD, of the University of Michigan, Ann Arbor.

Zinkevych/iStock/Getty Images

SOURCE: McDaniel B et al. Pediatr Res. 2018. doi: 10.1038/s41390-018-0052-6.

Digital technology use by parents during family activities may exacerbate internalizing or externalizing behavior in their children, according to results published in Pediatric Research.

In a study of 183 couples with children aged 5 years or younger, mothers perceived an average of 1.65 devices as interfering in their interactions with their child at least once per day, compared with an average of 1.43 devices per day for fathers. In addition, 56% of mothers and 43% of fathers reported that two or more devices interrupted their parent-child activities on a daily basis.

Higher technology interference (“technoference”) was associated with greater externalizing and internalizing behaviors in children and higher parenting stress for both mothers and fathers. Technoference also was associated with lower coparenting quality in fathers only, and greater parent depressive symptoms in mothers only, reported Brandon T. McDaniel, PhD, of Illinois State University, Normal, and Jenny S. Radesky, MD, of the University of Michigan, Ann Arbor.

Zinkevych/iStock/Getty Images

SOURCE: McDaniel B et al. Pediatr Res. 2018. doi: 10.1038/s41390-018-0052-6.

Over the last decade, three series published in the Lancet on child development have increased global awareness of the importance of early brain development and highlighted the critical role nurturing care plays in the first 3 years of a child’s life. Yet, as practitioners and policy makers in low- and middle-income countries increasingly acknowledge the influence early development has on later developmental, educational, and socioeconomic trajectories, there has been less agreement regarding the most appropriate methods and measures for screening and monitoring child development over time.

Mr. Small is with the Oregon Research Institute, Eugene. Dr. Hix-Small is with Portland (Ore.) State University. The authors have stated that they had no interests that might be perceived as posing a conflict or bias. Dr. Hix-Small has worked as a paid ASQ trainer. Email them at [email protected].

Over the last decade, three series published in the Lancet on child development have increased global awareness of the importance of early brain development and highlighted the critical role nurturing care plays in the first 3 years of a child’s life. Yet, as practitioners and policy makers in low- and middle-income countries increasingly acknowledge the influence early development has on later developmental, educational, and socioeconomic trajectories, there has been less agreement regarding the most appropriate methods and measures for screening and monitoring child development over time.

Mr. Small is with the Oregon Research Institute, Eugene. Dr. Hix-Small is with Portland (Ore.) State University. The authors have stated that they had no interests that might be perceived as posing a conflict or bias. Dr. Hix-Small has worked as a paid ASQ trainer. Email them at [email protected].

Over the last decade, three series published in the Lancet on child development have increased global awareness of the importance of early brain development and highlighted the critical role nurturing care plays in the first 3 years of a child’s life. Yet, as practitioners and policy makers in low- and middle-income countries increasingly acknowledge the influence early development has on later developmental, educational, and socioeconomic trajectories, there has been less agreement regarding the most appropriate methods and measures for screening and monitoring child development over time.

Mr. Small is with the Oregon Research Institute, Eugene. Dr. Hix-Small is with Portland (Ore.) State University. The authors have stated that they had no interests that might be perceived as posing a conflict or bias. Dr. Hix-Small has worked as a paid ASQ trainer. Email them at [email protected].

ORLANDO – New research suggests it’s feasible for hospitals to use an automated closed-loop insulin delivery system – free of prandial boluses and carbohydrate counts – to improve glucose control in noncritical patients with type 2 diabetes mellitus (T2DM).

The findings, released at the annual scientific sessions of the American Diabetes Association and via simultaneous publication in The New England Journal of Medicine, don’t examine cost or clinical outcomes. However, “our results suggest this new technology might be another approach to manage in-patient hypoglycemia in a safe and effective way, lead author Lia Bally, MD, PhD, of the division of endocrinology, diabetes, and clinical nutrition, Bern (Switzerland ) University Hospital, said in an interview.

For the open-label trial, the researchers recruited 136 adults with T2DM under noncritical care at two hospitals (one in the England and the other in Switzerland). Some patients had undergone surgery, Dr. Bally said, and some others were being treated for systemic infections. Comorbidities were significantly more severe in the closed-loop group, and 43% had sepsis.

All of the subjects required subcutaneous insulin therapy.

From 2016 to 2017, patients were randomly assigned to receive normal subcutaneous insulin therapy (n = 70) or closed-loop insulin delivery (n = 66).

It took about 15 minutes to perform the procedure to implement the closed-loop insulin delivery system, Dr. Bally said. It featured a subcutaneous cannula inserted into the abdomen, a continuous glucose monitor (a device also used in the control group), and a trial insulin pump.

This was not a hybrid system, and it did not include prandial insulin boluses or input of the timing and carbohydrate content of meals. One reason behind the choice to adopt a fully automated system was to relieve the burden on both health care professionals and patients, coauthor Hood Thabit, PhD, of Wellcome Trust–MRC Institute of Metabolic Science, the Manchester Academic Health Science Center, and University of Manchester, said in an interview.

For up to 15 days or until discharge, researchers tracked how much of the time sensor glucose measurements were in a target range of 100 mg/dL to 180 mg/dL.

In the closed-loop group, glucose measurements were in the target range 66 mg/dL ± 17% of the time compared to 42 mg/dL ± 17% in the control group, a difference of 24 mg/dL ± 3% (95% confidence interval, 19-30; P less than .001).

For the closed-loop group, the average glucose level was 154 mg/dL, and it was 188 mg/dL in the control group (P less than .001).

The researchers didn’t find a statistically significant difference between the groups in duration of hypoglycemia or amount of insulin delivered.

None of the patients suffered from severe hypoglycemia or clinically significant hyperglycemia with ketonemia.

There were 18 incidents of clinically significant hyperglycemia events (capillary glucose levels of more than 360 mg/dL) in the closed-loop group, compared with 41 such events in the control group. (P = .03)

Three patients in each group had adverse trial-related device effects.

Of 62 patients in the closed-loop group who completed the trial, 87% reported being pleased by their glucose levels, and all but one reported being happy to have their levels monitored automatically. All 62 patients said they’d recommend the system to others.

Going forward, the researchers hope to launch a multicenter trial that will examine clinical outcomes such as postoperative complications, infections, mortality, and glucose control after hospital discharge, according to Dr. Bally.

The study was supported by Diabetes UK, the Swiss National Science Foundation, the European Foundation for the Study of Diabetes, the JDRF, the National Institute for Health Research Cambridge Biomedical Research Center, and a Wellcome Strategic Award. Abbott Diabetes Care supplied equipment and guidance regarding connectivity, and representatives reviewed the manuscript before submission.

Dr. Bally reported funding from the University Hospital Bern, University of Bern and the Swiss Diabetes Foundation. Dr. Thabit reported no disclosures. Other authors report no disclosures or various disclosures.

SOURCE: Bally L et al. ADA 2018 Abstract 350-OR. Published simultaneously in The New England Journal of Medicine. June 25, 2018

ORLANDO – New research suggests it’s feasible for hospitals to use an automated closed-loop insulin delivery system – free of prandial boluses and carbohydrate counts – to improve glucose control in noncritical patients with type 2 diabetes mellitus (T2DM).

The findings, released at the annual scientific sessions of the American Diabetes Association and via simultaneous publication in The New England Journal of Medicine, don’t examine cost or clinical outcomes. However, “our results suggest this new technology might be another approach to manage in-patient hypoglycemia in a safe and effective way, lead author Lia Bally, MD, PhD, of the division of endocrinology, diabetes, and clinical nutrition, Bern (Switzerland ) University Hospital, said in an interview.

For the open-label trial, the researchers recruited 136 adults with T2DM under noncritical care at two hospitals (one in the England and the other in Switzerland). Some patients had undergone surgery, Dr. Bally said, and some others were being treated for systemic infections. Comorbidities were significantly more severe in the closed-loop group, and 43% had sepsis.

All of the subjects required subcutaneous insulin therapy.

From 2016 to 2017, patients were randomly assigned to receive normal subcutaneous insulin therapy (n = 70) or closed-loop insulin delivery (n = 66).

It took about 15 minutes to perform the procedure to implement the closed-loop insulin delivery system, Dr. Bally said. It featured a subcutaneous cannula inserted into the abdomen, a continuous glucose monitor (a device also used in the control group), and a trial insulin pump.

This was not a hybrid system, and it did not include prandial insulin boluses or input of the timing and carbohydrate content of meals. One reason behind the choice to adopt a fully automated system was to relieve the burden on both health care professionals and patients, coauthor Hood Thabit, PhD, of Wellcome Trust–MRC Institute of Metabolic Science, the Manchester Academic Health Science Center, and University of Manchester, said in an interview.

For up to 15 days or until discharge, researchers tracked how much of the time sensor glucose measurements were in a target range of 100 mg/dL to 180 mg/dL.

In the closed-loop group, glucose measurements were in the target range 66 mg/dL ± 17% of the time compared to 42 mg/dL ± 17% in the control group, a difference of 24 mg/dL ± 3% (95% confidence interval, 19-30; P less than .001).

For the closed-loop group, the average glucose level was 154 mg/dL, and it was 188 mg/dL in the control group (P less than .001).

The researchers didn’t find a statistically significant difference between the groups in duration of hypoglycemia or amount of insulin delivered.

None of the patients suffered from severe hypoglycemia or clinically significant hyperglycemia with ketonemia.

There were 18 incidents of clinically significant hyperglycemia events (capillary glucose levels of more than 360 mg/dL) in the closed-loop group, compared with 41 such events in the control group. (P = .03)

Three patients in each group had adverse trial-related device effects.

Of 62 patients in the closed-loop group who completed the trial, 87% reported being pleased by their glucose levels, and all but one reported being happy to have their levels monitored automatically. All 62 patients said they’d recommend the system to others.

Going forward, the researchers hope to launch a multicenter trial that will examine clinical outcomes such as postoperative complications, infections, mortality, and glucose control after hospital discharge, according to Dr. Bally.

The study was supported by Diabetes UK, the Swiss National Science Foundation, the European Foundation for the Study of Diabetes, the JDRF, the National Institute for Health Research Cambridge Biomedical Research Center, and a Wellcome Strategic Award. Abbott Diabetes Care supplied equipment and guidance regarding connectivity, and representatives reviewed the manuscript before submission.

Dr. Bally reported funding from the University Hospital Bern, University of Bern and the Swiss Diabetes Foundation. Dr. Thabit reported no disclosures. Other authors report no disclosures or various disclosures.

SOURCE: Bally L et al. ADA 2018 Abstract 350-OR. Published simultaneously in The New England Journal of Medicine. June 25, 2018

ORLANDO – New research suggests it’s feasible for hospitals to use an automated closed-loop insulin delivery system – free of prandial boluses and carbohydrate counts – to improve glucose control in noncritical patients with type 2 diabetes mellitus (T2DM).

The findings, released at the annual scientific sessions of the American Diabetes Association and via simultaneous publication in The New England Journal of Medicine, don’t examine cost or clinical outcomes. However, “our results suggest this new technology might be another approach to manage in-patient hypoglycemia in a safe and effective way, lead author Lia Bally, MD, PhD, of the division of endocrinology, diabetes, and clinical nutrition, Bern (Switzerland ) University Hospital, said in an interview.

For the open-label trial, the researchers recruited 136 adults with T2DM under noncritical care at two hospitals (one in the England and the other in Switzerland). Some patients had undergone surgery, Dr. Bally said, and some others were being treated for systemic infections. Comorbidities were significantly more severe in the closed-loop group, and 43% had sepsis.

All of the subjects required subcutaneous insulin therapy.

From 2016 to 2017, patients were randomly assigned to receive normal subcutaneous insulin therapy (n = 70) or closed-loop insulin delivery (n = 66).

It took about 15 minutes to perform the procedure to implement the closed-loop insulin delivery system, Dr. Bally said. It featured a subcutaneous cannula inserted into the abdomen, a continuous glucose monitor (a device also used in the control group), and a trial insulin pump.

This was not a hybrid system, and it did not include prandial insulin boluses or input of the timing and carbohydrate content of meals. One reason behind the choice to adopt a fully automated system was to relieve the burden on both health care professionals and patients, coauthor Hood Thabit, PhD, of Wellcome Trust–MRC Institute of Metabolic Science, the Manchester Academic Health Science Center, and University of Manchester, said in an interview.

For up to 15 days or until discharge, researchers tracked how much of the time sensor glucose measurements were in a target range of 100 mg/dL to 180 mg/dL.

In the closed-loop group, glucose measurements were in the target range 66 mg/dL ± 17% of the time compared to 42 mg/dL ± 17% in the control group, a difference of 24 mg/dL ± 3% (95% confidence interval, 19-30; P less than .001).

For the closed-loop group, the average glucose level was 154 mg/dL, and it was 188 mg/dL in the control group (P less than .001).

The researchers didn’t find a statistically significant difference between the groups in duration of hypoglycemia or amount of insulin delivered.

None of the patients suffered from severe hypoglycemia or clinically significant hyperglycemia with ketonemia.

There were 18 incidents of clinically significant hyperglycemia events (capillary glucose levels of more than 360 mg/dL) in the closed-loop group, compared with 41 such events in the control group. (P = .03)

Three patients in each group had adverse trial-related device effects.

Of 62 patients in the closed-loop group who completed the trial, 87% reported being pleased by their glucose levels, and all but one reported being happy to have their levels monitored automatically. All 62 patients said they’d recommend the system to others.

Going forward, the researchers hope to launch a multicenter trial that will examine clinical outcomes such as postoperative complications, infections, mortality, and glucose control after hospital discharge, according to Dr. Bally.

The study was supported by Diabetes UK, the Swiss National Science Foundation, the European Foundation for the Study of Diabetes, the JDRF, the National Institute for Health Research Cambridge Biomedical Research Center, and a Wellcome Strategic Award. Abbott Diabetes Care supplied equipment and guidance regarding connectivity, and representatives reviewed the manuscript before submission.

Dr. Bally reported funding from the University Hospital Bern, University of Bern and the Swiss Diabetes Foundation. Dr. Thabit reported no disclosures. Other authors report no disclosures or various disclosures.

SOURCE: Bally L et al. ADA 2018 Abstract 350-OR. Published simultaneously in The New England Journal of Medicine. June 25, 2018

ORLANDO – Liana K. Billings, MD, an endocrinologist at the University of Chicago and the NorthShore University HealthSystem in Skokie, Ill., loves the thrill of letting patients know they have a rare kind of diabetes. “Once you do this once, you don’t want to stop,” she told colleagues in a presentation at the annual scientific sessions of the American Diabetes Association. “I hope you all have the experience of diagnosing someone with MODY. It’s phenomenal.”

Yes, it’s true: There’s a diabetes diagnosis that spawns good feelings like delight and relief. The cause for celebration is a condition known as monogenetic diabetes, also known as maturity-onset diabetes of the young (MODY) if it develops after the neonatal period.

“The reason that getting a diagnosis of MODY can be a ‘good’ diagnosis is because the three most common forms of MODY have gene-specific treatments that typically improve patients’ glycemic control and are less onerous than the treatments patients were previously receiving when they were thought to have type 1 or type 2 diabetes,” explained Miriam S. Udler, MD, PhD, of Massachusetts General Hospital and Harvard Medical School, Boston, in an interview.

Dr. Udler and Dr. Billings spoke to colleagues about monogenetic diabetes in their presentation at the ADA meeting.

Research has suggested that 1%-4% of people with diabetes have the monogenetic form, in which the condition is caused by changes in a single gene. A 2017 British study of 1,407 patients with diabetes reported that “the minimum prevalence of monogenic diabetes is 3.6% of patients diagnosed at age 30 years or younger.”

The study, which tested a screening regimen, also turned up 17 new diagnoses of monogenetic diabetes among the 1,407 patients, doubling the total. The findings reflect an apparent fact about monogenetic diabetes: Physicians often don’t look for it, even though a diagnosis can be a godsend – especially for those who were previously diagnosed with type 1 or type 2 and placed on treatment regimens that are unnecessary at best and harmful at worst. (Diabetes Care. 2017 Aug;40[8]: 1017-25)

Patients with the MODY variant in the GCK gene, for example, “can generally stop all medications because they are not at risk for clinically significant complications of diabetes,” Dr. Udler said. “Patients with HNF1A and HNF4A variants can often be switched from insulin injections to ... a sulfonylurea, which is easier to take than insulin injections, and patients generally have better glycemic control after switching to pills.”

Unfortunately for doctors and patients, it can be complicated and costly to test for monogenetic diabetes. But screening tools are available to help physicians make choices about whether to launch testing in the first place, according to Dr. Udler and Dr. Billings.

There are two forms of monogenetic diabetes – neonatal diabetes, which is diagnosed by age 6-9 months, and MODY, which is typically diagnosed in those aged between 10 and 25 years, noted Dr. Billings.

Reasons to suspect MODY include early onset of diabetes (under 35 years), a family history of diabetes, a lack of obesity, and negative islet-cell antibodies, she said.

Obese patients may also have the condition: A 2017 American study of 488 overweight and obese children and adolescents diagnosed with type 2 diabetes found that 4.5% actually had monogenetic diabetes. (Genet Med. 2018 Jun;20[6]:583-90).

Once a physician suspects MODY, physicians may consult the University of Exeter’s risk calculator. It provides guidance about whether a test is a good idea. Dr. Billings cautioned, however, that the value of a calculator’s estimate of risk is not all-encompassing. “You should never use the calculator by itself as a reason to not pursue your intuition,” she said.

Dr. Udler noted that the University of Exeter calculator has important limitations, such as its reliance on specific genes, its lack of consideration of family history outside of parents, and its reliance on the experiences of white European patients.

As for tests, the University of Chicago and the University of Exeter both offer free genetic testing for neonatal diabetes, Dr. Billings said in her presentation.

Monogenetic diabetes tests in older children and adults are not free. However, Dr. Udler said the tests are often covered by insurance companies whether done for one or more genes.

At least one company offers a direct-to-consumer monogenetic diabetes test, according to Dr. Udler, but she recommended against it, especially in light of a curious online notice that says the test isn’t intended to be diagnostic. “I’m not sure what this would be useful for then,” she said.

For her part, Dr. Billings cautioned that test results may be inconclusive, and tests may offer different answers. She also recommended referring patients to genetic counseling.

Dr. Udler reported a board member/advisory panel relationship with Encompass Bioscience. Dr. Billings reported relationships with Novo Nordisk, Sanofi, and Dexcom.
 

ORLANDO – Liana K. Billings, MD, an endocrinologist at the University of Chicago and the NorthShore University HealthSystem in Skokie, Ill., loves the thrill of letting patients know they have a rare kind of diabetes. “Once you do this once, you don’t want to stop,” she told colleagues in a presentation at the annual scientific sessions of the American Diabetes Association. “I hope you all have the experience of diagnosing someone with MODY. It’s phenomenal.”

Yes, it’s true: There’s a diabetes diagnosis that spawns good feelings like delight and relief. The cause for celebration is a condition known as monogenetic diabetes, also known as maturity-onset diabetes of the young (MODY) if it develops after the neonatal period.

“The reason that getting a diagnosis of MODY can be a ‘good’ diagnosis is because the three most common forms of MODY have gene-specific treatments that typically improve patients’ glycemic control and are less onerous than the treatments patients were previously receiving when they were thought to have type 1 or type 2 diabetes,” explained Miriam S. Udler, MD, PhD, of Massachusetts General Hospital and Harvard Medical School, Boston, in an interview.

Dr. Udler and Dr. Billings spoke to colleagues about monogenetic diabetes in their presentation at the ADA meeting.

Research has suggested that 1%-4% of people with diabetes have the monogenetic form, in which the condition is caused by changes in a single gene. A 2017 British study of 1,407 patients with diabetes reported that “the minimum prevalence of monogenic diabetes is 3.6% of patients diagnosed at age 30 years or younger.”

The study, which tested a screening regimen, also turned up 17 new diagnoses of monogenetic diabetes among the 1,407 patients, doubling the total. The findings reflect an apparent fact about monogenetic diabetes: Physicians often don’t look for it, even though a diagnosis can be a godsend – especially for those who were previously diagnosed with type 1 or type 2 and placed on treatment regimens that are unnecessary at best and harmful at worst. (Diabetes Care. 2017 Aug;40[8]: 1017-25)

Patients with the MODY variant in the GCK gene, for example, “can generally stop all medications because they are not at risk for clinically significant complications of diabetes,” Dr. Udler said. “Patients with HNF1A and HNF4A variants can often be switched from insulin injections to ... a sulfonylurea, which is easier to take than insulin injections, and patients generally have better glycemic control after switching to pills.”

Unfortunately for doctors and patients, it can be complicated and costly to test for monogenetic diabetes. But screening tools are available to help physicians make choices about whether to launch testing in the first place, according to Dr. Udler and Dr. Billings.

There are two forms of monogenetic diabetes – neonatal diabetes, which is diagnosed by age 6-9 months, and MODY, which is typically diagnosed in those aged between 10 and 25 years, noted Dr. Billings.

Reasons to suspect MODY include early onset of diabetes (under 35 years), a family history of diabetes, a lack of obesity, and negative islet-cell antibodies, she said.

Obese patients may also have the condition: A 2017 American study of 488 overweight and obese children and adolescents diagnosed with type 2 diabetes found that 4.5% actually had monogenetic diabetes. (Genet Med. 2018 Jun;20[6]:583-90).

Once a physician suspects MODY, physicians may consult the University of Exeter’s risk calculator. It provides guidance about whether a test is a good idea. Dr. Billings cautioned, however, that the value of a calculator’s estimate of risk is not all-encompassing. “You should never use the calculator by itself as a reason to not pursue your intuition,” she said.

Dr. Udler noted that the University of Exeter calculator has important limitations, such as its reliance on specific genes, its lack of consideration of family history outside of parents, and its reliance on the experiences of white European patients.

As for tests, the University of Chicago and the University of Exeter both offer free genetic testing for neonatal diabetes, Dr. Billings said in her presentation.

Monogenetic diabetes tests in older children and adults are not free. However, Dr. Udler said the tests are often covered by insurance companies whether done for one or more genes.

At least one company offers a direct-to-consumer monogenetic diabetes test, according to Dr. Udler, but she recommended against it, especially in light of a curious online notice that says the test isn’t intended to be diagnostic. “I’m not sure what this would be useful for then,” she said.

For her part, Dr. Billings cautioned that test results may be inconclusive, and tests may offer different answers. She also recommended referring patients to genetic counseling.

Dr. Udler reported a board member/advisory panel relationship with Encompass Bioscience. Dr. Billings reported relationships with Novo Nordisk, Sanofi, and Dexcom.
 

ORLANDO – Liana K. Billings, MD, an endocrinologist at the University of Chicago and the NorthShore University HealthSystem in Skokie, Ill., loves the thrill of letting patients know they have a rare kind of diabetes. “Once you do this once, you don’t want to stop,” she told colleagues in a presentation at the annual scientific sessions of the American Diabetes Association. “I hope you all have the experience of diagnosing someone with MODY. It’s phenomenal.”

Yes, it’s true: There’s a diabetes diagnosis that spawns good feelings like delight and relief. The cause for celebration is a condition known as monogenetic diabetes, also known as maturity-onset diabetes of the young (MODY) if it develops after the neonatal period.

“The reason that getting a diagnosis of MODY can be a ‘good’ diagnosis is because the three most common forms of MODY have gene-specific treatments that typically improve patients’ glycemic control and are less onerous than the treatments patients were previously receiving when they were thought to have type 1 or type 2 diabetes,” explained Miriam S. Udler, MD, PhD, of Massachusetts General Hospital and Harvard Medical School, Boston, in an interview.

Dr. Udler and Dr. Billings spoke to colleagues about monogenetic diabetes in their presentation at the ADA meeting.

Research has suggested that 1%-4% of people with diabetes have the monogenetic form, in which the condition is caused by changes in a single gene. A 2017 British study of 1,407 patients with diabetes reported that “the minimum prevalence of monogenic diabetes is 3.6% of patients diagnosed at age 30 years or younger.”

The study, which tested a screening regimen, also turned up 17 new diagnoses of monogenetic diabetes among the 1,407 patients, doubling the total. The findings reflect an apparent fact about monogenetic diabetes: Physicians often don’t look for it, even though a diagnosis can be a godsend – especially for those who were previously diagnosed with type 1 or type 2 and placed on treatment regimens that are unnecessary at best and harmful at worst. (Diabetes Care. 2017 Aug;40[8]: 1017-25)

Patients with the MODY variant in the GCK gene, for example, “can generally stop all medications because they are not at risk for clinically significant complications of diabetes,” Dr. Udler said. “Patients with HNF1A and HNF4A variants can often be switched from insulin injections to ... a sulfonylurea, which is easier to take than insulin injections, and patients generally have better glycemic control after switching to pills.”

Unfortunately for doctors and patients, it can be complicated and costly to test for monogenetic diabetes. But screening tools are available to help physicians make choices about whether to launch testing in the first place, according to Dr. Udler and Dr. Billings.

There are two forms of monogenetic diabetes – neonatal diabetes, which is diagnosed by age 6-9 months, and MODY, which is typically diagnosed in those aged between 10 and 25 years, noted Dr. Billings.

Reasons to suspect MODY include early onset of diabetes (under 35 years), a family history of diabetes, a lack of obesity, and negative islet-cell antibodies, she said.

Obese patients may also have the condition: A 2017 American study of 488 overweight and obese children and adolescents diagnosed with type 2 diabetes found that 4.5% actually had monogenetic diabetes. (Genet Med. 2018 Jun;20[6]:583-90).

Once a physician suspects MODY, physicians may consult the University of Exeter’s risk calculator. It provides guidance about whether a test is a good idea. Dr. Billings cautioned, however, that the value of a calculator’s estimate of risk is not all-encompassing. “You should never use the calculator by itself as a reason to not pursue your intuition,” she said.

Dr. Udler noted that the University of Exeter calculator has important limitations, such as its reliance on specific genes, its lack of consideration of family history outside of parents, and its reliance on the experiences of white European patients.

As for tests, the University of Chicago and the University of Exeter both offer free genetic testing for neonatal diabetes, Dr. Billings said in her presentation.

Monogenetic diabetes tests in older children and adults are not free. However, Dr. Udler said the tests are often covered by insurance companies whether done for one or more genes.

At least one company offers a direct-to-consumer monogenetic diabetes test, according to Dr. Udler, but she recommended against it, especially in light of a curious online notice that says the test isn’t intended to be diagnostic. “I’m not sure what this would be useful for then,” she said.

For her part, Dr. Billings cautioned that test results may be inconclusive, and tests may offer different answers. She also recommended referring patients to genetic counseling.

Dr. Udler reported a board member/advisory panel relationship with Encompass Bioscience. Dr. Billings reported relationships with Novo Nordisk, Sanofi, and Dexcom.
 

As a physician you are the embodiment of delayed gratification. You spent more than 20 years in school before you earned a degree that then allowed you spend another 3-plus years in training before anyone would consider you a “real” doctor. Somewhere along that long and shallow trajectory someone may have said, “You must have done really well on the marshmallow test.”

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected].

As a physician you are the embodiment of delayed gratification. You spent more than 20 years in school before you earned a degree that then allowed you spend another 3-plus years in training before anyone would consider you a “real” doctor. Somewhere along that long and shallow trajectory someone may have said, “You must have done really well on the marshmallow test.”

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected].

As a physician you are the embodiment of delayed gratification. You spent more than 20 years in school before you earned a degree that then allowed you spend another 3-plus years in training before anyone would consider you a “real” doctor. Somewhere along that long and shallow trajectory someone may have said, “You must have done really well on the marshmallow test.”

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected].