Introduction Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and reduced life expectancy. Hydroxyurea (HU) has been shown to reduce the frequency and severity of vaso-occlusive episodes in SCD. Hypoxaemia and intermittent nocturnal oxygen desaturations occur frequently in children with SCD and contribute to the associated morbidity, including risk of cerebrovascular disease.
Objective To evaluate the effect of HU on oxygen saturation (SpO2) overnight and on daytime SpO2 spot checks in children with SCD.
Methods A retrospective review of children with SCD and respiratory problems who attended two UK tertiary sickle respiratory clinics and were treated with HU. Longitudinal data were collected from 2 years prior and up to 3 years after the commencement of HU.
Results Forty-three children, 23 males (53%) with a median age of 9 (range 1.8–18) years were included. In the 21 children who had comparable sleep studies before and after starting HU, mean SpO2 was higher (95.2% from 93.5%, p=0.01) and nadir SpO2 was higher (87.2% from 84.3%, p=0.009) when taking HU. In 32 of the children, spot daytime oxygen saturations were also higher (96.3% from 93.5%, p=0.001).
Conclusion Children with SCD had higher oxygen saturation overnight and on daytime spot checks after starting HU. These data suggest HU may be helpful for treating persistent hypoxaemia in children with SCD pending more evidence from a randomised clinical trial.
- sickle cell disease
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LvG and MA contributed equally.
Contributors AG conceptualised and designed the study, led on the analysis and interpretation of the data and contributed to the manuscript. LvG contributed to the design of the study, carried out the data collection and drafted the initial manuscript. MA contributed to the analysis, contributed to the manuscript and drafted the subsequent revisions. BS carried out the data collection, analysis and reviewed the initial manuscript. CJB, SC, BI, WK, GR and DR contributed to the study design and critical revision of the initial manuscript as well as subsequent revisions. All authors approved the final manuscript as submitted.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement Data are available on reasonable request. Deidentified participant data are available on reasonable request, contacting Dr Atul Gupta, King’s College Hospital, Denmark Hill, London, UK, SE5 9RS (firstname.lastname@example.org), 020 3299 4574.