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Summer Camps for Children with Sickle Cell Disease.

ABSTRACT: Background:Children with sickle cell disease (SCD) face unique problems that attendance at a camp with their peers is well suited to address. However, because the staff members at ordinary summer camps are not accustomed to accommodating children with chronic diseases, the potential for significant health consequences exists.Methods:We searched the literature in PubMed and CINAHL using the keywords summer camp, camp, sickle, and anemia to identify the unique characteristics of camps for children with SCD and the recommendations for care and/or lessons learned.Results:Published data are limited. Accommodations to avoid triggering sickle vaso-occlusive pain have been developed empirically and extrapolated from other settings. Camp experiences provide peer learning opportunities, positive role models, and a safe area to explore personal growth. The supportive atmosphere of the camp and escape from social stigma are welcome changes from the daily routine. Camp experiences can help with education on self-care and the transition from adolescent to adult healthcare.Conclusion:Camp offers unique opportunities for care coordination. Camps for children with SCD provide a setting for dissemination of best practices for the disease. Community-based organizations should partner in recruiting young adults with SCD as counselors. Educational material now available could be modified for use in the camp setting. All stakeholders in the care of children with SCD should work in unison to ensure these children enjoy the benefits of summer camp.

SUBMITTER: Narcisse L

PROVIDER: S-EPMC6292478 | biostudies-other | 2018

REPOSITORIES: biostudies-other

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Sickle cell disease in children.

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2014-07-01 | E-GEOD-35007 | biostudies-arrayexpress

The genomic architecture of sickle cell disease in children from West Africa

2014-07-01 | GSE35007 | GEO

Neurodevelopmental Outcomes in Preterm Children with Sickle Cell Disease.

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Sensorineural hearing loss in children with sickle cell disease.

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Effects of nutritional intake on disease severity in children with sickle cell disease.

Project description:Children with Sickle Cell Disease (SCD) may show growth failure in comparison to healthy peers. Many factors as hematological status, endocrine and/or metabolic dysfunction, and nutritional status, may play an important role in growth failure. The aim of this study was to assess whether impaired growth and nutritional intake can affect SCD severity during childhood.We conducted an observational study on children with SCD referring to our clinic for routine follow-up visits in a 6-month period. We collected information on weight, height and body mass index (BMI) and calculated their respective standardized scores (z). The nutritional intake was assessed through the last 24-h recall intake of total calories, macro- (proteins, lipids, carbohydrates) and micronutrients (calcium, iron, phosphorus, vitamins B1, PP, A, C, B2). Disease severity was assessed through total hemoglobin (Hb) and fetal hemoglobin (HbF), and lactic dehydrogenase (LDH) levels, and through the total number and days of hospitalizations, as well as the lifetime episodes of acute chest syndrome (ACS).Twenty nine children (14 males, 15 females) with SCD were enrolled; their mean age was 9.95 years (SD 3.50, min 3.72, max 17.18). Z-weight and z-BMI were significantly directly related to total Hb. Food intake resulted significantly unbalanced in terms of total calorie intake, macro- and micronutrients, especially calcium, iron, vitamin B1 and C. Low intake of calcium and vitamin B1 were significantly inversely correlated with number and days of hospitalizations per year. Protein, lipid, phosphorus, and vitamin PP intakes resulted adequate but were inversely correlated with number and days of hospitalizations. Carbohydrate, lipid, iron, phosphorus, vitamins B1 and B2 intakes were significantly inversely correlated to HbF levels.This study showed that, in our population, inadequate nutritional intake, weight and BMI have a significant impact on SCD severity indices.

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Project description:The prevalence of obstructive sleep apnea syndrome (OSAS) is higher in children with sickle cell disease (SCD) as compared with the general pediatric population. It has been speculated that overgrowth of the adenoid and tonsils is an important contributor.The current study used MRI to evaluate such an association. We studied 36 subjects with SCD (aged 6.9 ± 4.3 years) and 36 control subjects (aged 6.6 ± 3.4 years).Compared with control subjects, children with SCD had a significantly smaller upper airway (2.8 ± 1.2 cm(3) vs 3.7 ± 1.6 cm(3), P < .01), and significantly larger adenoid (8.4 ± 4.1 cm(3) vs 6.0 ± 2.2 cm(3), P < .01), tonsils (7.0 ± 4.3 cm(3) vs 5.1 ± 1.9 cm(3), P < .01), retropharyngeal nodes (3.0 ± 1.9 cm(3) vs 2.2 ± 0.9 cm(3), P < .05), and deep cervical nodes (15.7 ± 5.7 cm(3) vs 12.7 ± 4.0 cm(3), P < .05). Polysomnography showed that 19.4% (seven of 36) of children with SCD had OSAS compared with 0% (zero of 20) of control subjects (P < .05) and that in children with SCD the apnea-hypopnea index correlated positively with upper airway lymphoid tissues size (r = 0.57, P < 001). In addition, children with SCD had lower arterial oxygen saturation nadir (84.3% ± 12.3% vs 91.2% ± 4.2%, P < .05), increased peak end-tidal CO(2) (53.4 ± 8.5 mm Hg vs 42.3 ± 5.3 mm Hg, P < .001), and increased arousals (13.7 ± 4.7 events/h vs 10.8 ± 3.8 events/h, P < .05).Children with SCD have reduced upper airway size due to overgrowth of the surrounding lymphoid tissues, which may explain their predisposition to OSAS.

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TRANSCRIPTIONAL RESPONSES IN CHILDREN WITH SICKLE CELL

Project description:To date, little is known about lung disease pathogenesis in patients with sickle cell disease (SCD). Acute chest syndrome (ACS) is a significant cause of morbidity and premature.mortality in SCD, but preventative, diagnostic, and therapeutic options are limited. Further, ACS and acute vasoccclusive pain crises (VOC) can have overlapping features such as chest pain, which causes a diagnostic dilemma for clinicians. We aimed to explore changes in gene expression profiles in patients with SCD at baseline and during hospitalization for VOC or ACS in order to better understand ACS disease pathogenesis.

2019-11-05 | GSE139912 | GEO

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