About 18,000 Americans (1 in 7500 males) have hemophilia; over half are young. While advances have reduced the risk for blood-borne infections (hepatitis, HIV) transmission in human blood-derived clotting factor, many youth with hemophilia do not understand or practice preventive care.  Optimal care involves measures to prevent joint disability (e.g., exercise for muscle strength to protect joints, prompt treatment of bleeding episodes) and infections (e.g., vaccination, sexual activity, no illegal drug use). 

A 45-minute telephone survey was developed and conducted to determine preventive care knowledge and self-management practices.  Participants were recruited from 20 of the 134 federally-funded hemophilia treatment centers and eight randomly selected hemophilia foundation chapters.  Among these youth, ages 13 through 21 years, 110 consented to the interview. The average age was 16.7 years; nearly half were non-Hispanic White, and another one-third were Hispanic. 

The two complications of highest concern in this group were joint disease (41%) and bleeding episodes (23%).  One-third did not believe that joint disease was preventable.  Sixty percent said they limited physical activity to control complications, and only 27% used exercise for preventive joint care.  One-fourth (24%) reported using prophylaxis (clotting factor administration) regularly or before sports/exercise. 

Only 31% reported treated a bleeding episode within one hour. About 30% indicated that the main reason for delayed care was not recognizing a bleeding episode, and another 25% did not have clotting factor at hand. 
Infections were not their top concerns; 12% named HIV and 10% named hepatitis.  Among these participants, 70% had a severe form of hemophilia and another 3% had a moderate form which meant that 73% have been at risk for viral infections through blood products.  Patient records indicated that over half (53%) of the participants already had hepatitis C.  However, 78% reported not knowing how hepatitis C is spread, and 67% did not know how hepatitis B is transmitted. 

The findings were used to develop a national education campaign.  Youth focus groups helped create the slogan “Do the 5!” for these key messages: annual comprehensive checkup, vaccinations for hepatitis A and B, early treatment of bleeding episodes, regular exercise to protect joints, and routine monitoring for blood-borne infections.

(Nazzaro AM et al. Am J Public Health 2006; 96(9):1618-1622)

Commment: The National Hemophilia Foundation Nursing Working Group is a resource for nurses who have patients with hemophilia – check http://www.hemophilia.org/resources/nurses/index.htm.     --J.O.