Pain Rate and Social Circumstances Rather Than Cumulative Organ Damage Determine the Quality of Life in Adults with Sickle Cell Disease.

Previous studies have demonstrated a significantly reduced quality of life (QoL) in sickle cell patients which appeared to be related to the significant morbidity associated with sickle cell disease such as frequent painful crises. With the longevity of sickle cell patients in the Western world chronic organ damage may also be an important factor to the QoL of sickle cell patients. Furthermore, not directly disease related factors such as socioeconomic circumstances may also be important in the general well being of these patients.

First, to determine the contribution of chronic organ damage and cumulative sickle cell related complications to QoL of sickle cell patients in the Netherlands. Second, we analysed the effect of socio-economic circumstances on QoL in our patients.

Consecutive adult sickle cell patients cared for in a teaching hospital were included. QoL was assessed with SF-36 forms and was represented in a physical component scale (PCS) and a mental component scale (MCS). In all patients a systematic evaluation of chronic organ damage was performed including: pulmonary hypertension, renal failure, microalbuminuria, retinopathy and iron overload. In addition, sickle cell related complications including: the frequency of painful crises, chronic leg ulcers, avasculair osteonecrosis, stroke, acute chest syndrome and priapism were assessed in the previous five years by chart review. We studied the QoL scores in relation to presence of chronic organ damage, sickle cell related complications and socioeconomic circumstances (patients occupation and educational level).

A total of 96 patients were included in our study. The presence of chronic organ damage was not related to the QoL of our patients despite the incidence of pulmonary hypertension in 24% of our patients. Of the sickle cell related complications in the previous five years, pain rate was significantly associated with mental component score (MCS) but not with the physical component score (PCS). With respect to the socioeconomic circumstances, 35% of the sickle cell patients were unemployed as compared to 6% of the general population and 16% of immigrants without SCD. Both occupation and the level of education were significantly related to PCS while no relation with MCS or pain rate was found.

In sickle cell patients in Amsterdam, the Netherlands, QoL is mainly determined by pain rate and socioeconomic circumstances such as occupation and educational level. Chronic organ damage, although a major factor determining life expectancy in SCD, was not a determinant of QoL.

No relevant conflicts of interest to declare.