Breaking the vicious cycle: investing prophylactic factor use in functional body mass
Age: 27 Severe Hemophilia A
Note: I have been fortunate to have had access to prophylaxis since I was 5 and extremely supportive parents. I have no doubt that these are the two largest contributing factors to my healthy joints and ability to participate in triathlons, snowboarding, and other high energy sports. As I have gotten older I recognize that with hemophilia, and many other conditions, everyone is not dealt the same cards. We may be playing a similar game, but we’re on a spectrum of advantage in the game. Circumstances such as insurance, inhibitors, and target joints can have a dramatic effect on the whole family’s experience with hemophilia. I acknowledge these disparities between my experience and others, and hope that some of these general thoughts on “using prophylaxis to its full potential” are relevant and could help (even in some small way) you or a hemophiliac you care about, play the cards you/they have been dealt.
After a large iliopsoas bleed landed me in the hospital for my 5th birthday, we would always do prophylactic infusions on Mondays and Thursdays because they seemed like the best days to keep my factor levels up for while I was at school. Prophylaxis was done because the need for it was there: I was going to play kickball, play on the monkey bars, and practice flying jumps off the swings. Little did I realize at the time, that the preventive treatment coupled with the activity would keep me out of the vicious cycle of atrophy, injury, being sedentary, and more atrophy. That was also long before I realized how valuable each of those $1500 vials were that brought me up from 0% factor 8 to 60%. A “get out of jail free card”, a chance to do things with the assurance of significant protection for the next 24 hours, and dwindling protection after that.
As I got older, my appetite for pushing my limits remained and I found myself following in my dad’s footsteps to start running cross country and track in high school. Throughout all the miles of running my parents and I would often regroup about how I was feeling: distinguishing normal fatigue and soreness from precursors of bleeding episodes from the training.
I ran off and on for fitness during college and started swimming more seriously. At 24 I found myself on one of the best collegiate triathlon teams in the country and I fell in love with the sport. The variety in training for swimming, biking, or running suited my body very well and I found that the two days of rest between training blocks for one sport were great for recovering. Continuing to discuss any aches and pains with my parents, we reached a consensus that it would be in my best interest to run on days that my factor level was pretty high to contain any micro-bleeds, overly strained muscles, or turned ankles quickly. From these discussions came the concept of using prophylaxis to help keep up my training momentum. I believe this has led to more resilient joints that are constantly getting stimulus tostay coordinated and supported. From then on, I promised myself that I would use each prophylactic dose as opportunity. Though the factor would have a fleeting presence in my body, but its benefit would not.
From my peak of fitness in early 2010, and my now current attempts to sustain it as a twenty something in the working world, I am humbled by how efficient the body is at eliminating energetically expensive muscle it does not think is needed. It needs a constant reminder of “hey! This stuff is important!”, and for hemophiliacs doubly so. Without constant reminders that ankle and leg muscles are important muscles will atrophy and leave one exposed to more easily turned ankles and vulnerable knees from lack of joint support during flexion and more impact on the skeleton from running and walking.
It may seem like a lot of work to maintain this, but with the increasing awareness of sport specific and functional training, people are enjoying the benefits of their workouts and find the fitness easier to sustain. The general premise of sport specific or functional training is that strength exercises should be designed around a goal, I want the motions of: running, walking, swimming, bike riding, basketball, skiing, playing soccer to be easier. By strengthening with specific weight lifting, rubber bands, pilates, or stretching motions, the body is reminded that muscles, nerves, ligaments, and tendons required for these motions are important.
So how does prophylaxis fit into this? Well as some us of us (particularly the severe hemophiliacs among us) can attest to, there is annoyingly large variety of mundane motions and activities that cause bleeding episodes. By saving for high factor level days some of these more vigorous “reminders” of exercise stimulus for our bodies to hang on to tissue that is quite functional and helpful for living an active life, the vicious cycle is transformed into the fitness investment cycle: infuse, build and maintaining functional body mass, protection of coordination and strength, live it up, infuse.
By traveling “light” and shaping most of the mass we carry into being functional help us do the activities that we love, hemophiliacs can take a fleeting, yet appreciated protection such as factor and have it pay out more permanently as smarter tissue that helps to protect the body at lower factor levels.
“He who would travel happily must travel light.” — Antoine de St. Exupery