Before there was Google, there was the Encyclopedia Britannica.
“I just had a patient this morning in clinic who told me that when he was seven or eight he pulled the Encyclopedia Britannica down off the shelf and looked up hemophilia, and it said you would live a very painful life and you would die by the time you were 20,” said Dana Francis, social worker with the adult hemophilia program at the University of California, San Francisco
But today that patient, a doctor with a family, is in his early 60s, Francis said at a recent webinar hosted by the National Hemophilia Foundation (NHF).
Such stories inspired “Navigating Time and Space: Experiences of Aging with Hemophilia,” a study that surveyed and interviewed the first generation of hemophilia patients to reach their golden years. The research, which also included caregivers, captured life stories to better understand patient struggles and how improved treatments impacted their lives.
“If you’re over 50 you were born at a time when there was no (clotting) factor, no treatment for hemophilia except icing the joint, elevating the joint and whole blood transfusions…there really wasn’t much for you.” Francis said. “Your life span back then, if you lived to be 40, that was old.”
Contrast that with today’s emerging treatments, including potential gene therapies.
The future was unknown for those who are senior citizens today. Patients – most of whom are men – would spend their lives questioning the reality of tomorrow, making each life decision based on a mantra of having just two years left, said one male participant in the study, age 67. On top of that, they faced stigma due to physical limitations, cruelty from peers, painful joint bleeds, fear and discrimination, said Tam Perry, faculty member at the Wayne State University School of Social work in Detroit, Michigan. Women with hemophilia were met with silence.
“Very few women get acknowledged, they get ignored,” said a female patient, age 60. Medical professionals would just say women can’t have hemophilia.
In the 1980s and 1990s, early clotting factor treatments brought hope only to be dashed by products tainted with Hepatitis C and human immunodeficiency virus (HIV). The question of life span was fraught with emotional complexity, trauma and uncertainty, said Sara L. Schwartz, Clinical Associate Professor at the University of Southern California School of Social Work.
Since then, improved technology helped ensure product safety and fueled a feeling of cautious optimism.
“This is really the first chance that a lot of hemophiliacs have been able to age…they didn’t expect to have this longevity so there was little preparation,” said an anonymous nurse.
This unique population would benefit from additional support, including mental health services, holistic health care and even retirement planning, the researchers recommended. Separate studies have found that aging patients encounter problems typical of old age compounded by hemophilia, which can cause bleeding in joints among other problems. Obesity is also a concern for people with hemophilia as they age – something that can worsen joint bleeds and cause mobility issues.