High mortality among children with sickle cell anemia and overt stroke who discontinue blood transfusion after transition to an adult program

Abstract

BACKGROUND Chronic blood transfusion is the standard of care in the management of overt stroke due to sickle cell anemia (SS) to prevent recurrence of stroke. The problem arises when children are transitioned to adult care where blood transfusion may be discontinued. The purpose of this study was to report the outcome of 22 patients with SS and overt stroke who were transitioned to our adult program between 1993 and 2009. STUDY DESIGN AND METHODS Transitioned patients were kept on chronic blood transfusion they had as children. Blood bank data were performed and computerized according to FDA and AABB regulations. Records were kept prospectively. Blood counts and percent hemoglobin (Hb)S were obtained before and after transfusion. HbS was kept below 30% after transfusion. Metabolic profiles were obtained every 6 months or more often if needed. Statistical analysis was by the two‐tailed t‐test. RESULTS Patients who were compliant with blood transfusion were rarely hospitalized for painful crises. Alloimmunization and iron overload were the major complications of blood transfusion. Eight patients who refused to be maintained on chronic blood transfusion or who were noncompliant died within 1 to 5 years after transition. Causes of death included stroke in two, sudden in three, and multiorgan failure in three. The overall rate of death after transition was 36% and the major cause was discontinuation of blood transfusion. CONCLUSION Efforts must be made to maintain adequate chronic simple or exchange blood transfusion for children with SS and stroke after transition to adult care.

Supplemental findings of the 2017 National Blood Collection and Utilization Survey

“…Persons with the same race and ethnicity are more likely to have the same major and minor blood antigens and transfusing blood to recipients from donors with the same race and ethnicity can reduce the risk of complications related to mismatched antigens . For example, persons with sickle cell disease can require frequent transfusions and are at a high risk of developing alloantibodies; recruiting donors from racially diverse populations for blood antigen matching can reduce the risk of alloimmunization . New thresholds which have increased the minimum Hgb for male donors may have resulted in more deferrals of black males compared to other races, likely because healthy black males have been reported to have lower Hgb level than white males .…”

Health literacy and knowledge of chronic transfusion therapy in adolescents with sickle cell disease and caregivers

“…These factors create unique educational considerations for caregivers and adolescents preparing for transition to adult care. Inadequate knowledge about SCD and transfusion therapy may be an impediment to adherence and successful adult transition . Additionally, when medical care is transferred or fragmented among multiple institutions, lack of knowledge of past transfusion complications (particularly alloimmunization) may lead to serious and potentially fatal outcomes .…”

Physicians’ Opinion on the Practice of Exchange Blood Transfusion for Treatment of Stroke in Sickle Cell Anaemia

“…Table 2 shows the category of the health institutions investigated as well as the number of registered patients and the frequency distribution of the stroke patients observed in the past one year. However [10] 19.2 % of the respondents had access to all 3 investigative modalities while [8] 15.4% had none. Manual exchange blood transfusion was the commonest modality of managing stroke in sickle cell as reported by 35 (66%) respondents, 9 (16.9%) respondents had access to automated red cell exchange facilities, while simple top up was used additionally by 13 (24.5%) respondents.…”

“…Exchange blood transfusion on the other hand is less associated with iron overload but since it requires more units of blood, may lead to alloimmunization faster. The aim of transfusion therapy is to reduce the percentage of HbS to less than 30% [10].…”