Food insecurity, housing instability, and dietary quality among children with sickle cell disease: Assessment from a single urban center.

BACKGROUND: Food insecurity and housing instability, both social determinants of health (SDoH), disproportionately affect economically unstable, under-resourced US communities in which children with sickle cell disease (SCD) live. Association between these SDoH markers and dietary quality among children with SCD is unknown. PROCEDURES: We assessed a cross-sectional sample of dyadic parent-child patients and young adult patients up to age 21 from one pediatric SCD center. Food insecurity, housing instability, and dietary quality were measured using validated US instruments and a food frequency questionnaire. Better dietary quality was defined using US dietary guidelines. Multivariate regression assessed for associations among dietary quality and food insecurity with or without (±) housing instability and housing instability alone. RESULTS: Of 100 enrolled participants, 53% were Black and 43% Hispanic; mean age 10.6 ± 5.6 years. Overall, 70% reported less than or equal to one economic instability: 40% housing instability alone and 30% both food insecurity and housing instability. Eighty percent received more than or equal to one federal food assistance benefit. Compared to no economic instability, food insecurity ± housing instability was significantly associated with higher intake of higher dairy and pizza, while housing instability alone was significantly associated with higher dairy intake. Food insecurity ± housing instability was significantly associated with lower intake of whole grains compared to housing instability alone. CONCLUSIONS: Our sample reported high frequencies of both food insecurity and housing instability; having more than or equal to one SDoH was associated with elements of poorer diet quality. Screening families of children with SCD for food insecurity and housing instability may identify those with potential nutrition-related social needs.

"I've Weathered Really Horrible Storms Long Before This ": The Experiences of Parents Caring for Children with Hematological and Oncological Conditions during the Early Months of the COVID-19 Pandemic in the U.S.

The societal impact of COVID-19 is vast, thus it is imperative to understand how vulnerable groups, such as children with chronic medical conditions are affected. This understanding can prepare psychologists and other healthcare providers to meet their current and future needs. A convenience sample of 11 parents of children with hematological/oncological conditions was recruited. They participated in semi-structured interviews on the effect of the COVID-19 pandemic on their children. Qualitative analysis identified common themes. Parental responses focused on the pandemic's impact on children's general daily life and healthcare. Themes of caution, uncertainty, adaptation, and the role of the healthcare providers and early medical experiences emerged. Concerns about vulnerability, changes in routine, the importance of virtual connections, and the pivotal role of providers have implications for children with and without medical conditions. The adaptation and resilience of the families provide a sense of hope in an uncertain time.

Acupuncture for pediatric sickle cell pain management: A promising non-opioid therapy.

OBJECTIVE: To describe the use of acupuncture for pain management in children with sickle cell disease. DESIGN: A retrospective chart review of a single-institution experience on the use of acupuncture in pediatric patients with sickle cell disease was evaluated between 2012-2019. Demographic characteristics, presenting pain location, pain scores pre- and post-acupuncture, and adverse events were collected. SETTING: Columbia University Medical Center, NewYork-Presbyterian Morgan Stanley Children's Hospital Pediatric Hematology outpatient and inpatient units. INTERVENTIONS: Acupuncture was performed by six licensed acupuncturists. Point prescriptions were based on pain location, philosophies of Traditional Chinese Medicine and Japanese Style of Kiiko Matsumoto acupuncture. MAIN OUTCOME MEASURES: Pain reduction as measured by two Verbal Pain Scales. RESULTS: Ninety acupuncture treatments were administered to twenty-four patients with sickle cell disease: median age 17.5 years, 62 % female, 37.5 % African American, 50 % Hispanic. The mean treatment duration was 18.5 ±â€¯4.8 min. Fifty-five treatments had documented pre/post-acupuncture pain scores. Pain reduction was achieved in 65.5 % of these treatments. A 0-10 pain scale used in 13 treatments reported a mean pre-acupuncture score of 7.31 ±â€¯1.75, post-acupuncture score of 6.08 ±â€¯1.85, and a mean pain score change of 1.23 ±â€¯1.09 (p = 0.11); A 0-4 pain scale used in 42 treatments reported a mean pre-acupuncture pain score of 3.31 ±â€¯0.72, post-acupuncture score of 2.33 ±â€¯0.98, and a mean pain score change of 0.98 ±â€¯0.99 (p < 0.0001). No adverse events were noted. CONCLUSION: Acupuncture therapy decreased pain for our patients with sickle cell disease, providing a safe non-opioid therapeutic option.

A Scoping Review of Validated Tools to Measure Incivility in Healthcare Settings.

OBJECTIVE: To identify and compare validated tools used to assess incivility in healthcare settings. BACKGROUND: Incivility in the workforce is associated with poor quality outcomes, increased employee turnover, and decreased job satisfaction. Validated tools are essential for accurate measurement of incivility. The aim of this study was to compare characteristics of validated tools for use in a busy clinical setting. METHODS: In a scoping review, English language research studies using incivility tools published in PubMed or CINAHL between March 1, 2013, and March 14, 2018, were assessed for sound psychometric properties and feasibility of use (eg, short, easy to administer). RESULTS: After screening 869 articles and full text review of 244, 5 identified tools met the criteria; the Short Negative Acts Questionnaire seemed best suited for use in a busy healthcare setting. CONCLUSION: Adoption of a standardized and validated incivility tool makes it possible to compare across clinical settings and track progress over time.

Response to Long-term Vitamin D Therapy for Bone Disease in Children With Sickle Cell Disease.

Patients with sickle cell disease (SCD) are at risk for bone fragility from multiple factors including vitamin D deficiency. To date, no studies have evaluated the efficacy and safety of long-term vitamin D therapy for bone disease in children with SCD. We report a cohort of 4 children with SCD found to have severe vitamin D deficiency, secondary hyperparathyroidism, and abnormal bone mineral density treated with monthly high-dose oral cholecalciferol over 2 years. All patients exhibited a positive response to therapy without hypervitaminosis D or hypercalcemia. Further studies are needed to standardize guidelines for optimal vitamin D dosing and prevention of toxicity.

Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy.

PURPOSE: Based on standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A) detect more-frequent retinopathy in adolescents with SCD. DESIGN: Cross-sectional study. METHODS: Setting: Institutional. SUBJECTS: Sixteen adolescents with SCD, aged 10-19 years (mean age 14.9 years), and 5 age-equivalent controls (mean age 17.4 years). OBSERVATION PROCEDURES: Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT, and OCT-A were performed. MAIN OUTCOME MEASURES: Sickle retinopathy defined by biomicroscopic changes, Goldberg stages I-V, Penman scale, flow void on OCT-A, or macular thinning on SD-OCT. RESULTS: While 22 of 32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4 of 24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20 of 24 eyes (83.3%) had peripheral arteriovenous anastomoses (Goldberg II) in addition. No patients had Goldberg stages III-V. By SD-OCT and OCT-A, thinning of the macula and flow voids in both the superficial and deep retinal capillary plexus were found in 6 of 30 (20%) eyes. CONCLUSIONS: All 24 eyes with adequate UWFA studies demonstrated sickle retinopathy. SD-OCT and OCT-A, which have not been previously reported in the adolescent population, detected abnormal macular thinning and flow abnormalities undetected by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected. If these findings are confirmed with larger cross-sectional and prospective analyses, these approaches may enhance early screening for sickle retinopathy.

Vitamin D deficiency and acute vaso-occlusive complications in children with sickle cell disease.

BACKGROUND: Vitamin D is increasingly recognized for its roles in non-skeletal disorders. Patients with sickle cell disease (SCD) have a high prevalence of vitamin D deficiency but data are limited with respect to possible associations between low vitamin D and acute vaso-occlusive complications. We examined whether vitamin D deficiency is associated with acute pain and acute chest syndrome (ACS) in children with SCD. PROCEDURE: A cross-sectional study was conducted in 95 children with SCD who had serum 25-hydroxyvitamin D (25-OHD) measured during comprehensive care examinations. History of acute pain and ACS within two years of obtaining 25-OHD was collected. Associations between 25-OHD levels and acute vaso-occlusive events were analyzed by logistic regression. Odds ratios and 95% confidence intervals were calculated for the risk of pain and ACS associated with vitamin D deficiency (25-OHD <20 ng/ml). RESULTS: Subjects were 3-20 years old (median 10.6); 48 males, 47 females; 46 African, 49 Hispanic; 72 SS, 20 SC, 1 S/ß(0) Thalassemia, and 2 S/ß(+) Thalassemia. Median 25-OHD was 16 ng/ml. Fifty-six (59%) were vitamin D-deficient. Thirty-one (33%) and 29 (31%) had at least one episode of pain and ACS, respectively. Serum 25-OHD was significantly associated with pain (P = 0.0121) but not with ACS (P = 0.628). Of those with pain, 73% (23/31) were vitamin D-deficient while 26% (8/31) had 25-OHD ≥20 ng/ml (P = 0.04, OR = 2.7, 95%CI = 1.05-6.94). CONCLUSIONS: Our findings emphasize the high prevalence of vitamin D deficiency and its potential association with acute pain in SCD. Correcting low vitamin D may offer a simple, low-cost intervention to help reduce acute vaso-occlusive complications.

Sickle cell disease: time for a closer look at treatment options?

Tremendous progress has been made in the care of individuals with sickle cell over the past several decades. Major successes have been comprehensive infection prophylaxis, prediction and prevention of stroke, and better transfusion care, the latter including both prevention of alloimmunization and treatment of iron overload. However, definitive therapies remain limited to hydroxycarbamide (hydroxyurea) and stem cell transplantation, both of which have been in use for at least two decades. Despite knowing the progressive natural history of the disease with organ dysfunction, failure, and ultimately death at a young age, definitive therapies are considered for only a small proportion of individuals. Consequently, while life expectancy has improved dramatically from the last century, the ongoing pace of advancement has slowed or stalled. We believe that it is time to broaden the use of definitive therapy for those with asymptomatic disease, being cautiously more aggressive in our approach.

Fetal hemoglobin levels in African American and Hispanic children with sickle cell disease at baseline and in response to hydroxyurea.

The degree of fetal hemoglobin (HbF) expression is a major determinant of phenotypic severity of sickle cell disease (SCD). Genetic regulation of HbF production is complex and can vary among ethnic groups. The pediatric sickle cell population at our institution is approximately half Hispanic, nearly all from the Dominican Republic. Hydroxyurea (HU) is the only Food and Drug Administration (FDA)-approved drug to ameliorate symptoms of SCD. We retrospectively compared baseline and HU-induced percent HbF (%HbF) in African American (AA) and Hispanic (H) patients aged 4 to 21 years with homozygous Sickle hemoglobin or HbSß(0)Thalassemia. No significant differences were detected in average baseline %HbF between AA (N=48) and H (N=58) patients (P=0.63). In the subset of children taking HU who reached maximum tolerated dose (MTD), no differences were found between the ethnic groups in laboratory response to drug, measured by %HbF at MTD (P=0.28), the increase in %HbF (P=0.31) or mean red cell volume (MCV) (P=0.93), or the MTD of HU (P=0.95). Regulation of HbF at baseline and in response to HU are comparable between Hispanics and African Americans at our center. If generalizable, our results support combining these 2 groups in future clinical and translational analyses focused on HbF and response to HU in this ethnically mixed patient population.

Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis.

OBJECTIVES: Children with sickle cell anemia (SCA) and moyamoya syndrome carry a significant risk of ischemic stroke. Given the success of encephaloduroarteriosynangiosis (EDAS) or pial synangiosis in the treatment of moyamoya disease, the purpose of this study was to examine whether it reliably and durably protected children with SCA and moyamoya syndrome against cerebrovascular complications. METHODS: The authors retrospectively reviewed a series of 12 patients with SCA who developed clinical and/or radiological evidence of moyamoya syndrome and underwent EDAS. RESULTS: Eleven patients (92%) presented following a cerebrovascular accident (CVA), transient ischemic attack (TIA), or seizure. Magnetic resonance (MR) imaging or angiography suggested moyamoya vascular changes, and cerebral angiography confirmed the diagnosis in all 12 patients. At the time of surgery, the median age was 12.3 years (range 6.8-19.4 years). Ten (83%) of 12 patients had a history of CVA, and 4 of these patients were compliant with a transfusion protocol at the time of their CVA. Bilateral (7 patients) or unilateral (5 patients) EDAS was performed without complications. The mean follow-up period was 46.8 months (range 8.1-106 months). During the follow-up period, 2 patients (16.7%) suffered cerebrovascular events. One patient, who was stroke-free preoperatively, suffered a CVA 3 weeks after the procedure. The other patient suffered a single left lower-extremity TIA 18 months following right-sided EDAS. She returned to her neurological baseline condition and remains stable 53 months postoperatively. Seven patients underwent follow-up angiography or MR angiography, and evidence of revascularization was noted in all cases. At this time, no patient has developed progressive disease requiring a contralateral procedure after unilateral EDAS. CONCLUSIONS: The EDAS procedure is a safe and effective treatment option in patients with SCA who develop moyamoya syndrome.