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Updated: Dec 10, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Long-term oxygen therapy in children with sickle cell disease and hypoxaemia
Ilaria Liguoro1,2, Michele Arigliani3,4, Bethany Singh2,4
1Department of Medicine, DAME-Division of Paediatrics, Universita degli Studi di Udine, Udine, Italy.
Insights
Nocturnal long-term oxygen therapy (LTOT) is safe and well-tolerated in children with sickle cell disease (SCD) and chronic hypoxemia. This treatment improved oxygen saturation without significant adverse events or worsening of pain crises.
Area of Science:
- Pediatric Hematology
- Pulmonary Medicine
- Critical Care
Background:
- Children with sickle cell disease (SCD) often experience chronic hypoxemia.
- Hypoxemia in SCD can exacerbate disease complications and affect quality of life.
Purpose of the Study:
- To assess the safety and acceptability of nocturnal long-term oxygen therapy (LTOT) for pediatric SCD patients with chronic hypoxemia.
- To evaluate the impact of LTOT on oxygenation parameters and clinical outcomes.
Main Methods:
- Retrospective cohort study of 19 children with SCD receiving LTOT.
- Compared clinical and laboratory data, TCD scans, and overnight oximetry before and after LTOT initiation.
- Adverse events and adherence were monitored.
Main Results:
- No patients discontinued LTOT due to intolerance or poor adherence.
- No major adverse events were reported.
- Significant improvements in overnight oxygen saturation parameters were observed, with no significant changes in hemoglobin, reticulocyte counts, or vaso-occlusive pain events.
Conclusions:
- Nocturnal LTOT is a safe and feasible treatment for children with SCD and chronic hypoxemia.
- LTOT effectively improves oxygenation in this population.
- Further research may explore long-term benefits and optimal management strategies.
Objective:
To evaluate the acceptability and safety profile of nocturnal long-term oxygen therapy (LTOT) in children with sickle cell disease (SCD) and chronic hypoxaemia.
Design:
Retrospective cohort study.
Patients, Setting And Intervention:
Children with SCD who started LTOT from 2014 to early 2019 in two tertiary hospitals in London, UK were retrospectively enrolled. Patients who started disease-modifying therapies <12 months before LTOT or while on LTOT were excluded.
Main Outcome Measures:
Minor and major adverse events during LTOT were reported. Laboratory and clinical data, transcranial Doppler (TCD) scans and overnight oximetry studies performed at steady state within 12 months before and after starting LTOT were compared.
Results:
Nineteen children (10 males; median age 12 years, range 6-15) were included. Nearly half of them (9/19; 47%) were on hydroxyurea at baseline. No child discontinued LTOT because of intolerance or poor adherence. No major adverse events were reported. Laboratory data did not show significant changes in haemoglobin and reticulocyte count after 1 year of follow-up. No statistically significant change in the incidence of vaso-occlusive pain events was noted (median annual rate from 0.5 to 0 episode per patient/year; p=0.062). Overnight oximetry tests performed while on LTOT showed improvements in all oxygen saturation parameters (mean overnight and nadir SpO2, % of time spent with SpO2 <90%) compared with the baseline.
Conclusion:
LTOT is a safe and feasible treatment option for children with SCD and chronic hypoxaemia.
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