Chronic complications and quality of life of patients living with sickle cell disease and receiving care in three hospitals in Cameroon: a cross-sectional study

Abstract

BackgroundSickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. Although sub-Saharan Africa bears 75% of the global burden of SCD, there is a paucity of data on these complications and their effects on the QOL. We aimed to record these chronic complications, to estimate the QOL, and to identify the corresponding risk factors in patients with SCD receiving care in three hospitals in Cameroon.MethodsIn this cross-sectional study, a questionnaire was used to collect data from consecutive consenting patients. Information recorded included data on the yearly frequency of painful crisis, the types of SCD, and the occurrence of chronic complications. A 36-Item Short Form (SF-36) standard questionnaire that examines the level of physical and mental well-being, was administered to all eligible participants. Data were analyzed with STATA® software.ResultsOf 175 participants included, 93 (53.1%) were female and 111 (aged ≥14 years) were eligible for QOL assessment. The median (interquartile range, IQR) age at diagnosis was 4.0 (2.0-8.0) years and the median (IQR) number of yearly painful crisis was 3.0 (1.0–7.0). The most frequent chronic complications reported were: nocturnal enuresis, chronic leg ulcers, osteomyelitis and priapism (30.9%, 24.6%, 19.4%, and 18.3% respectively). The prevalence of stroke and avascular necrosis of the hip were 8.0% and 13.1% respectively. The median (IQR) physical and mental scores were 47.3 (43.9–58.5) and 41.0 (38.8–44.6) respectively. Age and chronic complications such as stroke and avascular necrosis were independently associated with poor QOL.ConclusionsIn this population of patients living with SCD, chronic complications are frequent and their QOL is consequently poor. Our results highlight the need for national guidelines for SCD control, which should include new-born screening programs and strategies to prevent chronic complications.Electronic supplementary materialThe online version of this article (doi:10.1186/s12878-017-0079-7) contains supplementary material, which is available to authorized users.

Estimated Life Expectancy and Income of Patients With Sickle Cell Disease Compared With Those Without Sickle Cell Disease

“…It is well established that health-related quality of life is diminished in individuals with SCD. [41][42][43] Affected individuals experience a variety of SCD symptoms and long-term chronic morbidities, including pain, 25,44 vaso-occlusive crises, 45,46 fatigue, 47,48 depression, 41,49 and reduced social and school functioning, 47,50 that diminish patient well-being. Individuals with SCD report health-related quality of life similar to that of individuals undergoing hemodialysis.…”

Physical and Mental Aspects of Patients With Sickle Cell Disease: a Portrait of Quality of Life in a Developing Country

“…A similar study carried out in Cameroon in 2017 showed means of 47.3 and 41.0 for the physical and mental components, respectively, suggesting worse values in the latter, a finding different from that of the present research study; perhaps this could be explained by the unstable political and social situation in that country. 16 Another study, in Saudi Arabia, revealed even lower values than the Cameroonian study, probably because the SF-36 was even applied with patients during painful episodes. 23 The domain of physical aspects, one of the items of the physical component of the SF-36, is composed of four questions (related to problems with work or some daily activity due to physical health).…”

“…A study conducted in Cameroon, a country with worse socioeconomic conditions than Brazil, showed a median age at diagnosis of 4 years of life, also below that recommended by the CDC. 16 Belgium implemented the screening test for hemoglobinopathies in 1994, as well as one more database of national case registers in 2008, and already reaps fruits, with a mean age at diagnosis of 0.7 years, with an impact on the morbimortality of the disease. 17 It has been demonstrated that the early diagnosis of SCD, together with the capacity of a multiprofessional team and the participation of the family and the Considering that painful seizures begin as early as childhood and, as found in this study, are the most prevalent complications in this population, these young people learn to deal early with the disease and the limitations imposed by it.…”

Health-Related Quality of Life of Children and Adolescents with Sickle Cell Disease in the Middle East and North Africa Region

“…found that pain rates were higher in female children with SCD compared to their male counterparts. 43 , 60 Therefore, further studies are needed to better understand gender differences in pain perception among children and adolescents with SCD so as to tailor pain management strategies accordingly.…”