Exercise guidelines do not exist for individuals with sickle cell anemia (SCA) despite the impact of disease-related complications on physical functioning. Thirteen subjects (mean 15.1 ± 2.8 years old) with SCA were prescribed three exercise sessions/week for 12 weeks on a stationary bicycle placed at home. In total, 77% of subjects completed 89% of prescribed sessions without exercise-related adverse events, thus meeting feasibility and safety criteria. Adherence to prescribed duration and target heart rate during training decreased during the second half of the study. Future trials are warranted to further evaluate training benefits associated with regular exercise in children with SCA.

Children with SCD are active at moderate to vigorous intensity for shorter durations. Negative personal beliefs about disease impact and poor physical functioning represent barriers to PA in SCD.

Objective To examine heart rate recovery (HRR) as an indicator of autonomic nervous system (ANS) dysfunction following maximal exercise testing in children and young adults with sickle cell anemia (SCA). Study design Recovery phase heart rate (HR) in the first 5 minutes following maximal exercise testing in 60 subjects with SCA and 30 matched controls without SCA was assessed. The difference between maximal HR and HR at both 1-minute (ΔHR1min) and 2-minute (ΔHR2min) recovery was our primary outcome. Results Compared with controls, subjects with SCA demonstrated significantly smaller mean ΔHR1min (23 bpm, 95% CI [20, 26] vs. 32 bpm, 95% CI [26, 37], p = 0.006) and ΔHR2min (39 bpm, 95% CI [36, 43] vs. 48 bpm, 95% CI [42, 53], p = 0.011). Subjects with SCA also showed smaller mean changes in HR from peak HR to 1 minute, from 1 minute to 2 minutes and from 2 through 5 minutes of recovery by repeated measures testing. In a multivariable regression model, older age was independently associated with smaller ΔHR1min in subjects with SCA. Cardiopulmonary fitness and hydroxyurea use, however, were not independent predictors of ΔHR1min. Conclusions Children with SCA demonstrate impaired HRR following maximal exercise. Reduced post-exercise HRR in SCA suggests impaired parasympathetic function, which may become progressively worse with age, in this population.

We demonstrated the feasibility and effectiveness of a low-cost QI intervention to increase TCD screening. This approach was more successful than other related intervention models and is easily implemented by smaller sickle cell programs without full-time personnel.