Anxiety and apprehension within a family that has experienced an intracranial bleed in their child is understandable. Cendie Wood’s guest blog on the Hemophilia Society of Colorado blog (http://cohemo.blogspot.com/2011/02/story-of-survival-cendie-woods-family.html) documents the dilemma facing parents and grandparents of a child with severe hemophilia. Head bleeds in hemophilia may occur outside the skull such as a scalp hematoma, or within the skull, an intracranial hemorrhage. Bleeding into the brain is one of the leading causes of death in hemophilia. The lifetime risk of intracranial hemorrhage in severe hemophilia before AHF replacement therapy became available, has been estimated at 2-10% with a mortality rate of 30%. One of the earliest reports of a head bleed was in Queen Victoria’s eighth child, Leopold. He died at age 31 in 1884 after he struck his head in a fall in Cannes, France.
Prophylactic infusions of AHF are intended to minimize the frequency of spontaneous intracranial hemorrhages in small children, such as Cendie Wood’s grandson, Brett, who have hemophilia. However, the instance will not be totally eliminated for intracranial hemorrhages are known to occur in persons who do not have a deficiency of clotting factors. Hopefully her grandson, who will soon be three years of age, will be free of intracranial bleeds with regular AHF prophylactic replacement.
Recurrences of intracranial do occur in severe hemophilia. They may be located subdurally or intracerebrally. In my book, Doctor Guilt?, Roger a five-year-old boy who had severe hemophilia with an intracerebral hemorrhage is described. He was one of the first children to survive after receiving cryoprecipitate in 1965 while undergoing cranial surgery. The introduction of replacement therapy with cryoprecipitate in 1964 by Dr. Judith Pool at Stanford University was a remarkable event allowing survival of head bleeds in hemophilia. Roger’s head bleed recurred and he underwent head surgery a second time. When his intracranial bleed recurred a third and a fourth time he was not returned to surgery but did receive cryoprecipitate AHF replacement therapy that allowed him to survive to adulthood.
With the support of the excellent and experienced hemophilia care in Denver by Doctor Marilyn Manco-Johnson and the Hemophilia Treatment Center staff, you should convert your energy from agony to optimism for a good life for your grandson. Good luck and enjoy your grandson, whom I hope will grow to become a strong, handsome boy to make his grandmother proud of him.