Patients Dissatisfied With Acute Sickle Cell Disease Care

Julie Kanter, MD

Based on the findings of a new survey, a negative perception of care may be a barrier for patients living with sickle cell disease to address the condition.

Julie Kanter, MD, and colleagues from the Sickle Cell Disease Implementation Consortium assessed the sickle cell disease-related medical care experience of adolescents and adults with the disease. Through a survey evaluating pain interference, quality of healthcare, and self-efficacy of 440 adults and adolescents with sickle cell disease, the team found most participants were pleased with their usual care physicians but had negative experiences in acute care settings.

The Sickle Cell Disease Implementation Consortium comprises 8 academic or clinical sites and 1 data coordinating center distributed in locations across the US with high populations of those with sickle cell disease. Activities are divided into 2 phases: a needs assessment and implementation studies. The assessment included surveys, focus groups, and interviews with individuals with sickle cell disease and with clinicians.

The survey captured individual-level data using previously validated surveys collected using a standard approach. It used patient-reported measures in 4 major domains: pain interference and experience, quality of healthcare, social determinants of health, and self-efficacy regarding sickle cell disease. The domains were chosen to evaluate specific areas of care, experiences, and coping that could be targeted by interventions across different geographic locations and for different age groups.

Patients with sickle cell disease were eligible to participate in the survey if they had a confirmed diagnosis, lived in the geographic region of 1 of the 8 sites, were between 15 and 50 years old, and were not experiencing acute symptoms of sickle cell disease. Participants were recruited by clinicians and through websites, flyers, recruitment letters, health fairs, and clinical programs.

Each patient reported their age, gender, race, ethnicity, and sickle cell disease phenotype. Participants indicated the frequency of their pain episodes experienced over the previous year, including the extent of any interference with usual daily activities.

To measure pain, each participant completed the Patient Reported Outcomes Measurement Information System four-item Pain Interference Short Form. On the form, patients indicated how much pain interfered with daily activities in the previous 7 days, including work around the house, social activities, and household chores. Items were scored on a five-point Likert scale—1 indicated “not at all” and 5 indicated “very much.”

The Adult Sickle Cell Quality of Life Measurement Information System quality of care measure was used to assess the healthcare experience of the participants. The form had 27 questions but allowed respondents to complete the survey in as few as 5 questions if they did not have sickle cell disease-related pain in the previous 12 months and never sought emergency of ambulatory care.

In an effort to assess self-efficacy to understand the perceived ability to deal with daily aspects of sickle cell disease such as pain and fatigue, the investigators used the nine-item Sickle Cell Self-Efficacy Scale. Responses ranged from 1 (not sure at all) to 5 (very sure).

Among 440 patients, a majority (55.7%) were female and African American (97.3%) with a mean age of 27.8 years old. Most patients (70.3%) had hemoglobin SS or hemoglobin S β⁰-thalassemia.

A majority of respondents (82.6%) had access to non-acute (usual) sickle cell disease care, and 92.1% noted satisfaction with their usual care physician. More than half (66%) reported needing an emergency department visit for acute pain in the previous year.

The respondents were not as pleased with their emergency department care. Just half of the participants reported being satisfied with or perceiving having adequate quality care in the emergency department. Participants mentioned when they experienced severe pain or a clinician lack of empathy, it was associated with negative quality of care. Younger patients (<19 years old vs 19-30 and 31-50 years old) reported better emergency department experiences.

The results suggested the need to improve communication and co-management to prevent the perceived negative experiences of care in the acute setting. Further, the Sickle Cell Disease Implementation Consortium should use its projects to identify and help individuals engage with a knowledgeable clinician.

The study, “Perception of US Adolescents and Adults With Sickle Cell Disease on Their Quality of Care,” was published online in JAMA Network Open.