Assessing the quality of life of adolescents with sickle cell disease and their transition to adult medicine: experience in a remote area of the Democratic Republic of Congo

Sickle cell disease is the most common genetic disease in the world, affecting mainly populations in sub-Saharan Africa. It causes high morbidity and mortality rates and is a real public health problem in the Democratic Republic of the Congo. Regular medical follow-up improves the quality of life of patients, especially adolescents, and raises the issue of their transition to adult medicine. This study aims to assess the quality of life and transition of sickle-cell adolescents, identifying key factors influencing their well-being. This was a cross-sectional descriptive and observational study conducted in the form of interviews with 106 sickle cell adolescents in Mbujimayi. The SF-36 short questionnaire was used to assess quality of life. A closed questionnaire (dichotomous or nominal) was used to assess the transition period. The results revealed an overall optimal quality of life, with an average SF-36 score of 60.6 ± 8. Improvement in pain management (75.21 ± 23.49) and misperception of role limitations related to emotional issues (47.79 ± 27.53). Pain is the main factor affecting quality of life. It is significantly associated with physical functioning (p-value 0.014), energy/fatigue p-value 0.014), functional/physical role (p 0.001), emotional well-being (p-value 0.000), social functioning (p-value 0.000) and pain itself (p-value 0.000). The majority, 92% of the adolescents, were unaware of the transition, and 68% did not feel ready to move from pediatric care to adult care. These findings highlight the importance of considering the quality of life of sickle cell adolescents and their transition to adult medicine in Mbujimayi, highlighting the challenges they face and the factors that affect their well-being in general. The most significant factors impacting quality of life were pain and fatigue. While many interviewees recognized the transition to adult care, they felt unprepared and lacked sufficient financial resources.