Cognitive & Behavioral Outcomes

Cognitive and Behavioral Outcomes in Children with Hemophilia

By Dr. David Clark

5/8/24 

The eTHINK study looked at cognitive, behavioral and adaptive functions in children and young adults with hemophilia A or B in the US.  The study included 551 male children (433 As and 101 Bs) plus 17 subjects whose disorder was not specified) from 21 US HTCs.  The subjects hadhemophilia of any severity, with or without inhibitors, and their ages ranged from 1 – 21 years. 

They found that performance on cognitive tests was generally comparable to or better than age-matched children in the general population, with a few exceptions.  Two age groups, 4 – 5 and 10 – 21 years, performed worse in attention measures and processing speed.  The authors also found no effect of hemophilia severity. 

They did find a higher prevalence of attention-deficit/hyperactivity disorder (ADHD) and learning disorders than in the general population, especially in boys aged 13 – 17 years. This shows the importance of screening for ADHD and similar conditions.  Interestingly, children on prophylactic treatment showed fewer ADHD symptoms and emotional problems than those receiving on-demand treatment. 

Two other interesting findings are that children whose mothers had higher education levels tended to have higher intellectual function and better attention.  Similarly, lower family income was associated with lower intellectual, attention, executive, behavioral and emotional function and adaptive skills.  Executive function is the ability to set and carry out goals.  These findings suggest the importance of the child’s environment and point to the need to consider that in treating patients. 

These results were significantly better than those found in a similar study performed over 30 years ago and probably reflect improvements in hemophilia treatment.  The authors point out that the study did not include females because there was insufficient data available and that further research is needed to determine whether there is a difference in the cognitive and behavioral effects between male and female children.  [Mrakotsky C et al., J. Pediatr, online ahead of print 5/28/24]