Health-Related Quality of Life of Adolescents with Sickle Cell Disease on Hydroxyurea

Hassan, Abdul Kareem F.; Mones, HayfaaMohammed; Ahmed, BahaaAbd Al Hussein

doi:10.4103/joah.joah_7_21

Cited by 3 publications

(1 citation statement)

References 38 publications

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“…Handheld dynamometry is considered a valid, reliable, simple tool for the objective measurement of HGS [9,10]. HRQL is a crucial outcome measure that provides insight into the well-being of children with SCD [11]. The Childhood Health Assessment Questionnaire (CHAQ) is commonly used to assess health status in children, and the updated versions showed improved validity in a variety of musculoskeletal problems [12,13].…”

Section: Introductionmentioning

confidence: 99%

Safety and efficacy of monthly high-dose vitamin D3 supplementation in children and adolescents with sickle cell disease

Hanna,

Kamal,

Fawzy

et al. 2024

Eur J Pediatr

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Little is known about the impact of vitamin D supplementation on hand grip strength (HGS) and health-related quality of life (HRQoL) in children and adolescents with sickle cell disease (SCD). We aimed to evaluate the safety and efficacy of monthly high-dose vitamin D3 supplementation and its implications on bone mineral density (BMD), HGS, and HRQoL in patients with SCD and healthy controls. The study included 42 children with SCD and 42 healthy matched controls. The study participants were supplemented with high-dose monthly oral vitamin D3. Changes in the serum level of 25(OH) vitamin D3, maximum HGS, and BMD from baseline to 6 months were assessed, and the HRQoL questionnaire and Childhood Health Assessment Questionnaire (CHAQ) were used to evaluate the functional capacity. At baseline, SCD subjects had poorer growth status indicated by negative Z scores. Suboptimal BMD was detected by significantly lower Z score, and lower HGS and worse HRQL parameters were found compared to the controls (P < 0.001). Median 25(OH) vitamin D3 was significantly lower in SCD patients compared to controls (16.5 vs. 28 ng/mL, respectively (P < 0.001)). After 6 months of vitamin D supplementation, there was significant improvement in the DEXA Z-score (P < 0.001), limitation of physical health (P = 0.02), pain scores (P < 0.001), and CHAQ grades (P = 0.01) in SCD patients. A significant improvement in HGS (P < 0.001 and P = 0.005) as well as the CHAQ score (P < 0.001 and P = 0.003) was detected in the SCD group and controls, respectively. There were no reported clinical adverse events (AEs) or new concomitant medications (CMs) during the study duration, and safe levels of Ca and 25 (OH) D3 were observed at 3 and 6 months for both groups. There was a significant positive correlation between HGS and total physical score (r = 0.831, P < 0.001) and a negative correlation with CHAQ score (r = − 0.685, P < 0.001). We also detected a significant positive correlation between vitamin D levels at 6 months and HGS (r = 0.584, P < 0.001), pain score (r = 0.446, P < 0.001), and a negative correlation with CHAQ score (r = − 0.399, P < 0.001). Conclusion: Monthly oral high-dose vitamin D supplementation was safe and effective in improving vitamin D levels, HGS, and HRQoL in SCD children and healthy subjects, and BMD scores in SCD patients. Further randomized controlled trials are warranted to assess an optimal dosing strategy and to investigate the impact on clinically significant outcomes in children and adolescents with SCD and their healthy counterparts. Trial registration: ClinicalTrials.gov, identifier NCT06274203, date of registration: 23/02/2024, retrospectively registered. What is known:• Several studies have reported a high prevalence of vitamin D deficiency and suboptimal bone mineral density (BMD) in sickle cell disease (SCD) patients.• Musculoskeletal dysfunction is reported in SCD patients with a negative impact on physical activity and health-related quality of life (HRQL).• Little is known regarding the impact of vitamin D3 supplementation in children and adolescents with SCD. What is new:• We found that monthly oral high-dose vitamin D3 supplementation was safe, tolerated, and effective in improving serum vitamin D levels, HGS, BMD scores, and HRQL in SCD patients.

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Section: Introductionmentioning

confidence: 99%

Safety and efficacy of monthly high-dose vitamin D3 supplementation in children and adolescents with sickle cell disease

Hanna,

Kamal,

Fawzy

et al. 2024

Eur J Pediatr

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Quantifying muscle strength, size, and neuromuscular activation in adolescent and young adult survivors of musculoskeletal sarcoma: Identifying correlates and responses to functional strengthening

Rock

Addison²,

Gray³

et al. 2023

The Knee

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Low Uptake of Hydroxyurea among a Cohort of Sickle Cell Disease Patients in Nigeria

Okoye,

Efobi,

Ommunakwe

et al. 2023

Nigerian Journal of Medicine

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Background: Hydroxyurea (HU) is the first disease-modifying drug used in the treatment of sickle cell disease (SCD). Despite its well-documented benefits, adoption and drug adherence have remained difficult. Aim: This study focused on determining the uptake rate of HU among SCD patients and the effects of HU on the disease outcome. Methodology: This was a retrospective hospital-based study conducted in four Nigerian tertiary institutions from October 2016 to September 2021. Data on their basic demographics, steady-state hematologic parameters, acute and chronic SCD-related events, and the use of HU were retrieved and analysed using the Statistical Package for the Social Sciences (SPSS) version 21. Statistics were both descriptive and inferential. Results: Of 509 SCD patients’ data analysed, 251 were male (49.3%), while 258 were female (50.7%). Their mean age was 26.9 (standard deviation ± 8.11) years; range: 18–63 years. Only 28 (5.5%) of the 509 patients were receiving or had ever received HU therapy. The steady-state hematological variables between individuals who are on hydroxyurea and those who are not hydroxyurea showed a statistically significant difference. Individuals who experience priapism (21.4%) and stroke (10.7%) were considerably more likely to be on HU (P = 0.001, 0.043), whereas patients not on HU were more likely to experience pulmonary hypertension (2.5%) (P = 0.001). Conclusion: The uptake of HU among SCD patients was low. Further prospective studies are encouraged to ascertain the factors responsible for this. There is a need for improved patients’ education and counselling to increase the level of awareness of HU and its benefits among patients.

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Health-Related Quality of Life of Adolescents with Sickle Cell Disease on Hydroxyurea

Cited by 3 publications

References 38 publications

Safety and efficacy of monthly high-dose vitamin D3 supplementation in children and adolescents with sickle cell disease

Safety and efficacy of monthly high-dose vitamin D3 supplementation in children and adolescents with sickle cell disease

Quantifying muscle strength, size, and neuromuscular activation in adolescent and young adult survivors of musculoskeletal sarcoma: Identifying correlates and responses to functional strengthening

Low Uptake of Hydroxyurea among a Cohort of Sickle Cell Disease Patients in Nigeria

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