Transfusion and Iron Overload in Thalassemia and Sickle Cell Disease: Impact of Infusion Chelation Therapy on Quality of Life.

Introduction: Blood transfusions are required as supportive care in thalassaemia and sickle cell patients. Regular blood transfusions may result in iron overload (IO) and, left untreated, in morbidity and earlier morality. Current infusion chelation therapy (ICT) requires 8–12 hour infusions, 5–7 days per week, potentially limiting quality of life (QoL) and inhibiting adherence in patients already limited by thalassaemia and sickle cell disease.

Methods: To assess the impact of ICT on the QoL of patients with thalassemia and sickle cell disease and adherence, a literature review in Medline (539 abstracts; 130 articles), analysis of IO patient transcripts (4 thalassaemia, 1 sickle cell disease) and five IO clinical expert interviews were conducted.

Results: Few published studies (11) used validated QoL instruments and even fewer assessed IO in thalassaemia (4) and sickle cell patients (7). No IO-specific QoL instruments were found. ICT in sickle cell patients may cause additional needle-site pain/discomfort, and may impact on evening social life and sleep, recreational activities, as well as emotional and physical well-being, occupational capacity and feelings of independence. ICT in thalassaemia patients may impact on emotional wellbeing such as self-esteem and worry about the future, family relationships and development of intimate relationships. Published articles and patient and clinician input suggested that QoL impact inhibited prescription of and adherence to ICT and therefore there is a need for easier, efficacious and safe oral chelation therapy (OCT).

Discussion: Limited empirical studies assessed ICT’s impact on QoL in patients with thalassaemia or sickle cell disease, though results indicated that ICT’s QoL impact is significant and prescription and adherence rates would likely improve with OCT. We recommend further qualitative and empirical studies to assess the impact of ICT and OCT in IO patients with thalassaemia and sickle cell patients, using validated instruments.