Youth Issues — Preparing for Life as an Adult with Hemophilia

As young boys with hemophilia grow up, not only do they experience the usual emotional and physical challenges of adolescence and early adulthood, but their relationships with family and caregivers also change when taking on more direct responsibility for their bleeding disorder. Hematologists who look after children will heavily involve parents as much as the young child in decision making. This is very different from the care given to adults, in which the person with hemophilia is encouraged to become independent and accept personal responsibility and ‘ownership’ of the disorder.

Transition between these two models is progressive over many years, emphasized particularly at the time of transfer from pediatric to adult care centres, where separate facilities exist. There may be a structured transition program to introduce you and your family to the new service.

Let us assume that you are a young man who is now cared for by an adult hemophilia centre, and that you are attending for the first time without your family. You are ready to move forward with the excitement of emerging adulthood. What are your expectations and what are those of your family, friends, and the new clinicians who are ready to assume responsibility for your care? It is very natural to be apprehensive. How do you best prepare for the challenges of life as an adult with hemophilia?

The level of care to which you have access will critically determine your opportunities. Being involved in your local hemophilia lobby group or regional chapter is a great support to the improvement and maintenance of your physical and psychological well-being.

Where possible, try to set your life and career goals to maximize your potential: you don’t have to know exactly what you will be doing in ten years or even three, but embarking on a course of study or acquisition of life skills that gives as many opportunities as possible is important. Remember that many physically demanding occupations are difficult and time-limited for men without musculoskeletal disabilities, much more so for men with hemophilia. That’s not to advise a totally sedentary job or lifestyle, far from it; both physical and emotional well-being is best preserved through regular exercise.

Read and learn about your disease to help you make informed discussions with clinicians and family. Although the management of your hemophilia may have seemed to revolve around avoiding bleeds and using factor replacement during your childhood, it is more complex than that. As an adult you will now be responsible for reporting your joint and muscle bleeds and need to know about their common presentations, including some which you may not have experienced yet, for example, back, loin, or hip pain caused by bleeding into the large ileopsoas muscles, which may be associated with the onset of sexual activity.

It is very important that you and your healthcare team develop treatment plans for your bleeds; you may be away from home and have to attend an emergency department where you know more about your condition than any of the clinicians. It’s not as scary if you recognize that you may have a bleed and to request doctors to immediately contact staff at your ‘home’ centre for advice.

Your life beyond the constraints of childhood, with increasing periods of time away from home, can be more chaotic and your bleeding experiences altered. If you are fortunate and receive prophylaxis, your product replacement routines may no longer be appropriate. Discuss your activity timetable openly with hemophilia centre staff—you may need to modify your treatment schedule or your plans to permit safe participation in these activities.

You will also need to think about disclosing your condition to friends and colleagues. These can be complicated discussions and it is best to involve your family and clinicians in making decisions about whom, what, and when you will tell. Find out about the genetic inheritance of your disorder so that you have accurate knowledge to share with your family members and potential life partners.
Transition can be a very exciting time for the young man who is well prepared for the increased responsibility and independence of adulthood with hemophilia. Talk with your family and clinicians, ask them to share your goals, and get out and enjoy!

Alison Street
Hemophilia World, December 2010