Family Perceptions of Barriers and Facilitators of Treatment Protocols for Secondary Stroke Prevention in Children with Sickle Cell Disease

Abstract 4805

Children with sickle cell anemia (SCA) and a primary overt stroke are at high risk of recurrent (secondary) stroke. Chronic blood transfusion (CBT) dramatically reduces but does not eliminate this high risk, and results in transfusion-related hemosiderosis. We previously reported the use of hydroxyurea/phlebotomy as an alternative to CBT to reduce the risk of secondary stroke and improve management of iron overload (Ware et al. J Pediatr 2004). This study examines the caregiver and child experience with secondary stroke prevention. Individual semi-structured interviews were conducted with primary caregivers and children/adolescents (age > 5 years) recruited from the Duke Pediatric Sickle Cell Program. The interviewer (E.P.) asked about perceptions of risk of recurrent stroke and iron overload with and without therapy and facilitators and barriers of therapy. Interviews were coded and analyzed independently by two investigators (E.P and C.T.). The sample included 14 youth (10 males) with a median age of 12.5 years (range 3–17). All primary caregivers were female. Twelve children had a history of overt stroke and 2 had a history of silent stroke. All children had experience with CBT and 9 were receiving CBT at the time of the interview. Eleven children had experience taking hydroxyurea and 5 were taking hydroxyurea at the time of the interview. All caregivers agreed that their child was at risk of recurrent stroke, identified benefit of current treatment and reported high motivation to adhere to treatment protocols. They noted significant impact that stroke had on school functioning, attention, personality, participation in sports and overall quality of life. Caregiver-reported barriers to CBT and hydroxyurea fell into three main categories: (1) missed work and school and related consequences; (2) unexpected resource-related challenges; and (3) inconvenience of clinic appointments, all of which contributed to burden on the family and sometimes missed clinic appointments and treatments. There were higher levels of concern expressed by caregivers of children on CBT related to the higher frequency and longer length of medical appointments compared with those taking hydroxyurea. The primary child-reported barrier was dislike of needles or shots (although this decreased with age as expected); those taking hydroxyurea also noted that they sometimes forgot to take the medication if they were busy with other activities or fell asleep. Caregiver-reported facilitators of CBT and hydroxyurea included: (1) understanding importance of stroke prevention and connection to consistent treatment; (2) ancillary benefits of treatments in addition to stroke prevention; (3) link between treatment and long-term benefits. Caregivers were able to overcome treatment barriers via the following: (1) logistical supports including appointment and medication reminders; (2) shared responsibility with other family members including the child; (3) trust in medical staff; and (4) faith. Although children disliked needles and shots, many enjoyed the clinic visits due to fun activities in the clinic setting and rewards. In addition, iron overload was a significant concern for caregivers. For those with children on CBT, knowledge of the risks of iron overload motivated adherence with oral iron chelation. Automatic refills facilitated adherence with chelation therapy, but the taste of the medication was a major barrier to adequate iron chelation. Caregivers of children taking hydroxyurea noted the benefit of avoiding iron overload. Of those who had undergone phlebotomy, in-home phlebotomy was noted as a facilitator, though requirement for IV contributed to negative perception. In summary, as clinicians review options for secondary stroke prevention with families, they should discuss family perceptions and individual barriers and facilitators which may impact adherence with therapy and long-term outcome. Future research should also investigate whether these family perceptions predict actual adherence to protocols and treatment outcomes.