Occupational History for Forty Adults with Sickle Cell Disease Compared with Healthy Siblings

Introduction: Despite high rates of unemployment and disability in adults with sickle cell disease (SCD), these issues remain understudied. Since SCD mostly affect people of African descent, the potential risk factors for poor job performance and unemployment in adults with SCD are a complex combination of disease, demographic, and socio-economic variables. Recurrent acute vaso-occlusive crises and organ dysfunction related to SCD can limit employment options and interfere with job retention. Potential associates of unemployment and poor job performance in adults with SCD include health-related (pain frequency and intensity, health-care utilization) and psychosocial (support system, coping mechanism). The goal of this study is to compare patients’ rates of unemployment and disability with those of their unaffected siblings. We compare patients with siblings so as to adjust for some socioeconomic variables.

Patients and Methods: Patients on chronic transfusion therapy and those with additional disabling conditions were excluded. Surveys were administered during routine clinic visits. Along with demographic data, the survey also assessed use of hydroxyurea, employment status, length of employment, annual income, and job satisfaction. Moreover, patients were asked to provide the same occupational data for a sibling without SCD and whose age was within five years of the patient’s age. Clinical data including number of hospitalizations in the recent year were also gathered from the patient’s medical record.

Results: A cohort of 40 adult SCD patients (28 hemoglobin SS, 8 hemoglobin SC, 1 Sβ⁰ and 3 Sβ⁺; age: median = 29, range 19-56 years; sex: 20 males, 20 females) completed surveys concerning occupational history for themselves and a sibling. Of the patients, twenty-three out of forty (57.5%) were on hydroxyurea therapy and 24/40 (60%) had 3 or more hospitalizations in the previous one year. Ten out of forty SCD patients (25%) are currently employed, compared to 26/40 (65%) of their siblings (p < .001; all tests reported are McNemar’s Exact (binomial) test). Five out of forty SCD patients (12.5%) have been employed for more than 2 years as compared to 20/40 (50%) of their siblings (p <.001). Twenty-six (65%) of SCD patients are on disability, while this is true for only four (10%) of the siblings (p< .001). It is important to note that 20 out of 26 (77%) SCD patients who are currently on disability have previously had a job. Fifteen of the twenty-six (58%) SCD patients on disability reported that they missed more than 1 day per week of work due to their SCD. Of the unemployed patients, twenty-three out of 30 (77%) wished they had a job.

Conclusions: In this sample, patients with SCD have notably lower rates of current employment and sustained employment when compared with similar-age siblings without SCD.Despite the fact that 32/40 (80%) of the SCD patients have previously had a job, only 25% are currently employed. Our future research studies will focus on development of specific interventions to improve job accommodation of SCD patients.