Hemophilia is a chronic condition that is identified at birth. It is a health condition where frequently upon injury, sometimes even spontaneously, blood clots slowly. The bleed can be controlled by replacing the protein "Factor" that is missing in hemophilia. An uncontrolled bleed leads to accumulation of blood in large spaces within the body such as the joints- knee, elbow, ankle, etc.
Multiple bleeds, or even a single uncontrolled and prolonged bleed damages joints. Now how and why does this happen? Briefly, the accumulated blood exerts pressure on the joint leading to pain, swelling and deformity of the joint. The synovium, a membrane that lines the joint and provides nourishment, begins to absorb the excess fluid in the joint and thickens and also becomes more vascularized. The cartilage in the end of the bones in the joint gets eroded by the enzymes from the swollen synovium. Eventually, the joint becomes 'bone-on-bone' and joint function is diminished and the range of motion of the joint is affected. This process is decribed in great detail here.
Its imperative for a hemophiliac to start becoming active once a bleed starts to restolve. This activity can range from simply extending or moving the joint through the available range of its motion. This could also involve tightening and relaxing the muscles supporting the joint on either side. For example, for the knee this would be the thigh and the calf muscles. Hence the rehabilitation of the joint takes several forms of physical activity or therapy (PT). A PT must closely supervise the actions of the patient. This becomes particularly important when the patient is sent back with a home program of exercises. The movements have to be gradual upto the point of pain, but not beyond. Any over-exercising of the joint or incorrect positioning or stretching can lead to a joint bleed- even in the presence of circulating Factor ("breakthrough bleeds") which is counter-productive. So here's the classic Catch-22* patients with hemophilia face- too little exercise weakens the joint, too much causes a bleed. There is no testbook definition of what is the 'right amount'. What I have experienced is to go easy, go gradual and use pain and discomfort as an indication to stop. I have found value in frequency- working out 3-4 times a week repeating the same set of exercise at least twice a week.
Due to several years of untreated hemophilia I have severely damaged all my joints. This includes my right knee and right shoulder. These are target joints which have had recurring bleeds. I have neuromuscular weakness on both legs, but predominantly in my right leg. Both right knee and right shoulder have 'bone-on-bone' arthritis. This affects the way I get up from a seated position. I force my right and left hand to get up. The right shoulder, being a Target Joint, often has a bleed. Now if I shift the load on to the legs instead, the right knee bleeds for the same reason. Another example of the dilemma as what is the best way to get up.
Typically when a joint gets to mine in stage there is chronic pain due to the 'bone-on-bone' condition and the doctors recommend a total knee-replacement (TKR) surgery. I had no pain but was considering TKR expecting it benefit from improved gait and mobility. My thinking was if I became more mobile, I could get a lot more strength back in my leg muscles. At the pre-op visit for TKR it was determined that due to my pre-existing 'kidney disease' I was at a significantly higher risk of infection of the knee. I had to effectively cancel the surgery. This was a vicious circle where my mobility could not improve without TKR. Not quite a classic Catch-22 but a clear impact of a comorbidity.
My belief is that as the patient with hemophilia ages, we will be faced with more such situations and we will figure out way to adapt to the new challenges we encounter.
Due to several years of untreated hemophilia I have severely damaged all my joints. This includes my right knee and right shoulder. These are target joints which have had recurring bleeds. I have neuromuscular weakness on both legs, but predominantly in my right leg. Both right knee and right shoulder have 'bone-on-bone' arthritis. This affects the way I get up from a seated position. I force my right and left hand to get up. The right shoulder, being a Target Joint, often has a bleed. Now if I shift the load on to the legs instead, the right knee bleeds for the same reason. Another example of the dilemma as what is the best way to get up.
Typically when a joint gets to mine in stage there is chronic pain due to the 'bone-on-bone' condition and the doctors recommend a total knee-replacement (TKR) surgery. I had no pain but was considering TKR expecting it benefit from improved gait and mobility. My thinking was if I became more mobile, I could get a lot more strength back in my leg muscles. At the pre-op visit for TKR it was determined that due to my pre-existing 'kidney disease' I was at a significantly higher risk of infection of the knee. I had to effectively cancel the surgery. This was a vicious circle where my mobility could not improve without TKR. Not quite a classic Catch-22 but a clear impact of a comorbidity.
My belief is that as the patient with hemophilia ages, we will be faced with more such situations and we will figure out way to adapt to the new challenges we encounter.
*“catch-22
noun
noun: catch-22; plural noun: catch-22s
- a dilemma or difficult circumstance from which there is no escape because of mutually conflicting or dependent conditions.
- "a catch-22 situation"
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chicken-and-egg problem
"he wanted to do the right thing, but either decision would hurt somebody—it was a classic catch-22"
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Origin
1970s: title of a novel by Joseph Heller (1961), in which the main character feigns madness in order to avoid dangerous combat missions, but his desire to avoid them is taken to prove his sanity.
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