Description of a Colocated Comprehensive Care Model for People With Sickle Cell and Comorbid Pulmonary Disease.

Comorbid pulmonary complications in people with sickle cell disease (pwSCD) are associated with high rates of morbidity and mortality, and poor access to care contributes to poor outcomes among this particularly high-risk pwSCD. Our purpose was to describe the population served and the resources required for hematology, pulmonary, nursing, respiratory therapy, social work, genetics, psychology, and school liaison providers to see these patients in an integrated clinic. We abstracted demographic, medication, clinical, and diagnostics data of the pwSCD seen at least once in this clinic from February 1, 2014 to December 10, 2020 from the electronic medical record and identified 145 unique pwSCD. Abnormal lung function and bronchodilator responsiveness were detected in 31% and 42% of participants respectively. Sleep abnormalities were found in over two-thirds of those screened and 65% had ≥1 previous acute chest syndrome episode. This clinic also allowed for direct provider communication and required relatively limited resources to serve a large number of severely affected pwSCD. Given the degree of abnormal respiratory variables detected and the limited resources required to implement this model, studies are warranted to evaluate whether it has the potential to improve outcomes in high-risk populations.

Outcomes before and after providing interdisciplinary hematology and pulmonary care for children with sickle cell disease.

People with sickle cell disease (pwSCD) are at risk of developing lung conditions that complicate their SCD but often face health care access barriers. An interdisciplinary clinic providing pulmonary care for pwSCD was created in 2014 at the Nationwide Children's Hospital (NCH) to address access barriers that may prevent optimized treatment. We hypothesize that pwSCD and pulmonary disease would have fewer hospitalizations for acute chest syndrome (ACS), asthma, and vaso-occlusive episodes in the 2 years after their initial SCD-pulmonary clinic visit compared with the 2 years before. From 2014 to 2020, 119 pwSCD were evaluated in the SCD-pulmonary clinic and followed up at the NCH for at least 2 years before and after this initial visit. Acute care outcomes, pulmonary function, polysomnography, echocardiogram, laboratory, and medication prescribing data were collected and analyzed using the Wilcoxon signed ranked and McNemar tests. The median number of acute care visits for ACS (P < .001) and asthma (P = .006) were significantly lower during the 2 years after pwSCD's initial SCD-pulmonary clinic evaluation compared with the 2 years before. Asthma and allergic rhinitis were more frequently diagnosed and prescriptions for hydroxyurea (P = .005) and inhaled corticosteroids (P = .005) were more common in the post-SCD-pulmonary clinic period. The median number of prescribed systemic corticosteroids was lower in the 2 years after SCD-pulmonary clinic evaluation (P < .0001). Lactate dehydrogenase and white blood cell counts also significantly decreased. Implementing a multidisciplinary SCD-pulmonary clinic is feasible and may allow improved management of pulmonary problems and lead to improvements in the usage of health and acute care.

Tumor lysis Syndrome in a Patient with Metastatic Breast Cancer Treated with Alpelisib.

PURPOSE: Tumor lysis syndrome (TLS) is a rare but life-threatening phenomenon that occurs mainly in patients with aggressive hematologic or highly chemotherapy sensitive solid tumors such as high-grade neuroendocrine carcinoma or testicular cancer. Tumor lysis syndrome is exceedingly rare in hormone receptor-positive, HER2-negative breast cancer. Furthermore, TLS following treatment with alpelisib, a novel phosphatidylinositol 3-kinase (PI3K) inhibitor used to treat PIK3CA-mutated (gene encoding p110α subunit of PI3K), hormone receptor positive advanced breast cancer, has never been described in patients with nonhematologic malignancies. METHODS: In the following case, we present a patient with hormone receptor-positive, HER2-negative, PIK3CA-mutated metastatic breast cancer who developed TLS 12 days after starting fulvestrant and alpelisib. RESULTS: Patient was promptly treated with improvement in her renal function to baseline without requiring renal replacement therapy. Alpelisib was resumed at a reduced dose with no further complications. CONCLUSION: Through this case, we discuss the potential complications of TLS and the importance of prompt recognition and treatment.

Mental health history and social barriers impacting caregivers of infants with cystic fibrosis.

OBJECTIVES: Caregivers of infants with cystic fibrosis (CF) carry a heavy treatment burden for their child along with the inherent difficulties of raising an infant. This study investigated the impact of self-reported caregiver mental health diagnoses and social barriers during the 1st year of life on clinical outcomes. METHODS: A retrospective chart review was conducted for infants seen in a large tertiary hospital CF clinic over a 5-year period. Baseline characteristics were collected, and documentation from physician and social work notes were reviewed. Demographics and clinical characteristics were compared by the presence or absence of self-reported mental health diagnoses, social barriers, and "emotional concern." RESULTS: Analyses were conducted on 71 patients. Thirty-five percent of caregivers disclosed mental health diagnoses, 52% identified social barriers to care, and 55% reported feeling upset or fatigued. Having a caregiver with a self-reported mental health diagnosis was associated with tobacco smoke exposure (p < .001) and increased odds of hospitalizations (odds ratio [OR], 3.01; 95% confidence interval [CI], 1.49-6.06), emergency department/urgent care visits (OR, 3.17; 95% CI, 1.32-7.64), and longer lengths of stay (OR, 1.93; 95% CI, 1.69-2.20). Caregivers who expressed emotional concern had infants with significantly lower weight-for-length percentiles (p = .012). DISCUSSION: Caregiver mental health and social barriers to care are important determinants to address as they may impact clinical outcomes in infants with CF. Identifying barriers and struggles early increases the likelihood that clinical teams can intervene and provide support. Further research into mental health and socioeconomic barriers faced by caregivers of infants with CF is crucial.

Guillain-Barré syndrome hyponatremia: is it SIADH or pseudohyponatremia?

Approximately 5% of hospitalized patients with Guillain-Barré syndrome (GBS) experience SIADH; but pseudohyponatremia has also been reported in patients treated with IVIG. We present a case of a 51-year-old male with GBS who developed acute hyponatremia the day after initiation of IVIG; his sodium levels began to improve within 24 h of completion of IVIG. Differentiating between pseudohyponatremia caused by the IVIG treatment and SIADH caused by GBS was the key to successfully treating this patient. This case exemplifies the importance of pursuing further studies to determine the exact cause of hyponatremia in GBS in order to prevent further neurologic damage to the patient.

Cutaneous amyloidosis.

Amyloidosis is generally classified as either systemic or cutaneous, with both primary and secondary forms. There are also heredofamilial and hemodialysis-associated varieties of amyloidosis, all with specific amyloid fibril derivatives. Nodular cutaneous amyloidosis is the most rare form of primary cutaneous amyloidosis. Lesions typically present as a crusted nodule on the face, extremities, or acral sites. The amyloid fibrils are immunoglobulin-derived and either kappa or lambda light chains. Systemic involvement is dependent on plasma cell amyloid protein deposition. Lesions may otherwise be classified as a local plasma cell clone or cutaneous plasmacytoma. Recent reports state that there is <10% risk of systemic progression. Workup should include at least a full history and physical examination; serum protein electrophoresis and urine protein electrophoresis; and gingival, rectal, or abdominal fat pad biopsies to rule out the presence of extracutaneous amyloid deposition. Management of nodular cutaneous amyloidosis is challenging, as there is no consistently effective treatment and local recurrence is common.