Quality of Life (QOL) in Sickle Cell Disease (SCD).

Introduction Assessment of QOL is important for clinicians, as it will allow them to provide the patients with better care. The objective of this study is to assess the QOL in SCD patients. Methods A convenience sample of 34 patients with SCD (50% female) with a mean age of 21 years (SD= 9.4 y), completed a Dartmouth Care Cooperative Chart System (COOP) questionnaire (Table 1) during a comprehensive clinic visit at Children’s Hospital & Research Center in Oakland. High scores (patient ratings of 4 or 5) represent unfavorable levels of health or major limitations. A score of 3 represents moderate impairment while scores of 1 or 2 indicate no impairment. A brief chart review was completed to obtain medical history. Results 60% of the transfused patients (Tx) had severe impairment in at least one domain and this was the case in 71% of non-transfused patients (Non-Tx). As shown in table 2, the most affected domains were different between the two groups of Tx and non-Tx. In non-Tx patients the most affected domains were those of pain, overall health, and social support. In Tx patients, the most affected domains were pain, daily activities, and change in health. Although both groups had a similar amount of pain (non-Tx=42% and Tx=50%), Tx patients reported a better overall QOL (question 9). The most common complications that non-Tx patients suffered from were AVN (71%), vaso-occlusive episodes (46%), acute chest syndrome (46%), pneumonia (17%), reactive airways disease (17%), chest pain (13%), headaches (13%), and sepsis episodes (13%). Data showed that 30% of the Tx patients suffered from strokes previous to start of transfusion. The most common complications found in these patients were acute chest syndrome (30%), reactive airways disease (50%), urinary tract infections (20%), and depression (20%). Conclusions Both transfused and non-transfused SCD patients suffer from serious impairments in QOL. We recommend all SCD patients undergo QOL assessments and intervention which focuses on affected domains. Studies are needed to determine the impact of complications on QOL.