A Global Perspective on Milestones of Care for Children with Sickle Cell Disease

Sainati, Laura; Montanaro, Maria; Colombatti, Raffaella

doi:10.5772/64656

Sickle Cell Disease - Pain and Common Chronic Complications 2016

DOI: 10.5772/64656

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A Global Perspective on Milestones of Care for Children with Sickle Cell Disease

Laura Sainati¹,

Maria Montanaro²,

Raffaella Colombatti³

Abstract: Sickle cell disease (SCD) is one of the most common severe and monogenic disorders worldwide. Acute and chronic complications deeply impact the health of children with SCD. Milestones of treatment include newborn screening, comprehensive care and prevention of cerebrovascular complications.

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2022

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

Hassan

Mones

Ahmed

2022

Journal of Applied Hematology

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BACKGROUND: Sickle cell disease (SCD) is a chronic multisystem disorder associated with acute and chronic complications that may negatively impact the quality of life (QoL). The study aimed to assess the health-related QoL (HRQoL) of patients with SCD on hydroxyurea (HU) and the factors affecting HRQoL domains. MATERIALS AND METHODS: This case-control study included 174 patients with SCD (12–18-year-old); 87 were on HU for at least 1 year and 87 were not receiving HU. It also included 174 healthy adolescents of the same age group. The HRQoL was assessed using the Short Form 36 Health Survey version 2 (SF-36v2). A multivariate linear regression analysis was performed to assess the independent effect of studied variables on HRQoL dimensions. RESULTS: Patients with SCD who were not on HU were found to have significantly lower SF-36 v2 scores (all domains) compared to those on HU and control group, P < 0.001. While patients on HU had significantly lower SF-36 v2 scores in physical functioning, role physical (RP), general health, and physical health component score only compared to the control group, P < 0.001. Multivariate linear regression analysis revealed significant associations between duration of HU therapy and RP (R2 = 0.208, P = 0.021) and mental health component score (R2 = 0.389, P = 0.047) and between hemoglobin levels with social functioning (R2 = 0.370, P = 0.023). CONCLUSIONS: HU has improved the HRQoL of SCD patients, in almost all domains, in addition to the improvement in many disease-related complications, mainly painful episodes. The positive impact of HU on HRQoL was significantly associated with the duration of therapy.

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

Hassan

Mones

Ahmed

2022

Journal of Applied Hematology

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A Global Perspective on Milestones of Care for Children with Sickle Cell Disease

Cited by 1 publication

References 77 publications

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

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

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