Prophylaxis with factor concentrates has been shown, if started early enough, to reduce the effects of haemophilic arthropathy. However, many adults with severe haemophilia did not have the benefits of prophylaxis as children and therefore have significant joint disease and other factors associated with repeated bleeding joints such as deformity, muscle weakness, and impaired balance. These can cause difficulty with mobility and result in pain on a regular basis. Also, the combination of joint disease and advancing age can leave people with haemophilia at an increased risk of falls and social isolation due to reduced mobility. To keep joints in the best possible shape as we get older requires a coordinated approach.
Firstly, it may be necessary to use appropriate pain relief for arthropathy. Options that are common in people with haemophilia are:
- Paracetamol (used cautiously in patients with concomitant liver disease).
- COX-2 inhibitors (these are an anti-inflammatory drug that has minimal effect on platelet function).
Note: Paracetamol and COX-2 inhibitors can cause bleeding in the stomach and it is important that their use is monitored carefully by your treatment centre.
Other methods of pain relief, such as rest-ice-compression-elevation (RICE), splinting and walking aids (e.g. crutches, canes, orthotics, etc) are reliable and can provide some help when carrying out daily activities. Also, light daily exercise and physiotherapy are important with joints that have severe or repeated bleeds.
Joint bleeding accounts for more than 90% of all serious bleeding events in persons with severe haemophilia. This risk of bleeding persists throughout life, and the potential benefits of prophylaxis in some form may be experienced by all age groups. The use of prophylaxis in adulthood has increased in recent years. It may be recommended to continue, restart, or even start prophylaxis on an ongoing basis or a short-term basis for a number of reasons. This will help to:
- Prevent bleeds.
- Preserve joint function or slow down the progression of joint damage.
- Relieve pain associated with bleeding and/or synovitis.
- Allows for gentle exercise and reconditioning.
There is some evidence that bleeds can increase after the age of 50 and prophylaxis will reduce the likelihood of this happening.
A synovectomy is a surgical procedure that can also be used to maintain joint function and/or reduce pain in target joints. Steroid injections and total joint replacement are other interventions for relieving severe pain and disability. The results of hip and knee joint replacement surgery in people with haemophilia, under 55 years old, are similar to the general population. It is important to note that people with haemophilia, particularly those with HIV, may be at increased risk for infectious and non-infectious complications after surgery.
Finally, being overweight can put extra strain on your joints, which can lead to further problems with arthritis, so trying to maintain an ideal body weight is important for preserving joint health.
There may be an increased risk of osteoporosis in individuals with haemophilia. As a result, this may make the replacement of joints more problematic. Another aspect of ageing is that although prophylaxis may prevent haemophilic arthropathy, it is unlikely to have an impact on the most common type of arthropathy in older individuals, i.e. degenerative or osteoarthritis.
The best prevention is to build up peak bone density by 35 years of age. However, if osteoporosis has already developed it is possible to prevent further deterioration and reduce the risk of fractures by:
- Exercise: weight-bearing exercise may help to retain minerals in the bone. Activities such as walking, hiking, swimming, and pilates are low impact and may improve bone density and lower the risk for developing bone disease.
- Lifestyle changes: remove risk factors such as smoking and ensure a moderate alcohol and caffeine intake. A diet containing calcium, phosphate and vitamin D will help to improve bone strength.
- Medication: a specialist doctor may advise firstly taking calcium and vitamin D supplements, but if the fracture risk is significantly increased, other drugs are available.
- Steroids: should be avoided if possible.
- Surgery: may be required, especially if a joint is damaged.
Joint Replacement
Joint replacement is more common in men with haemophilia. These are usually knee and hip replacements. Due to the complexity of the ankle joint, the bones in the joint are usually fused together using pins. However, in recent years there have been some success with ankle replacements.
On this page, two men with haemophilia share their stories of life before and after joint replacement.
Story 1:
Having haemophilia attack my knee joint relentlessly for over forty-six years and despite availing of every option offered to keep my target knee functioning within a manageable pain threshold, the day finally came when I had no choice but to go for a total knee replacement. I was very apprehensive about the prospects of surgery, considering my medical complications and the generally poor condition of my knee joint, but having built up over the years a strong working relationship with the team in St. James’s Hospital, I felt confident about their reassurances that they had the expertise and experience to deal with any complications that may arise.
Total knee replacement is major surgery even if you do not have haemophilia. Preparations included intensive physiotherapy and regular visits to the orthopaedic surgeon in an attempt to build up my knee as much as possible for the operation. This involved a great deal of commitment on my part, but in the attempt of ensuring that there was little difference for my surgery and recovery as you would expect for an average non-haemophiliac, it was certainly worth it. I spent approximately five weeks in hospital and a week of intensive physiotherapy in an orthopaedic hospital. Yes, I have to state that it was a difficult time for me and involved some pain and discomfort, but nothing like the constant pain I had become used to living with my crippled joint. I have nothing but praise for the attention, care and advice given to me at this time by the full team that enabled me to make a steady but slow progress towards recovery.
I expected to be up and running within weeks, but I soon discovered that it is much more realistic to start slowly and build up an exercise programme that fits in with your physical state and lifestyle over a longer period of time to ensure that you can sustain your programme into your new and more active future. Remember, if you receive a new knee, it will take time to grow strong and stable. It will also have limitations that you will have to learn to cope with over time.
In total, I was out of work for four months and my G.P had to be very forceful in making me see the reality that rushing back to work should not be my priority, but instead I should concentrate on working hard to make a full recovery, including making simple lifestyle changes that not alone would greatly improve the stability of my new joint, but would also give me the added bonus of improving my general health now and into the future.
From this process, I have learned the lesson that you must listen to your body if you are to make steady and sustainable progress and try to avoid attempting to constantly push your body to the limit. Sixteen months later, I am enjoying my new joint as I enter a new painless phase of my life.
Story 2:
It’s three months today since the surgery and it’s only now I can finally write something about it. This is partly due to the brain haze created by my painkillers but mostly due to the fact that it’s only now I’m starting to fully realise what it actually means.
Before I explain the difference, I’ll skip back a bit to give a quick synopsis of the background as to why a hip replacement was needed. I had always been active and reasonably fit. I had numerous bleeds in my ankles and the occasional knee or back bleed over the years. Then in 2003, I started to feel an ache in my right hip. However, compared to the other joints, it didn’t really get rated as an issue. I only felt any ache after a lot of exercise, such as a day walking or a few hours in the gym and after a short rest it was fine and I was good to go. It never prevented me from doing any daily activities or going anywhere.
Gradually, it started to become more prominent and by 2006, it was starting to be a bit uncomfortable. I was starting to arrange days in such a way that I was getting everything done a bit more efficiently. Instead of randomly meeting up with friends during the week, I would meet two or three of them at the weekend. I was still able to keep reasonably fit but I switched from cycling to swimming. They were minor adjustments but it was the start of a slippery slope. In 2007, it continued to deteriorate and by the end of the year, especially going into the winter, it was really starting to cause problems on a day to day basis. It had gotten a lot sorer and even walking to the shops was difficult. On the up side, when I wanted some chocolate, the effort to get to and back from the shop wasn’t worth it so I ate better and less which has to be good for me. By January 2008, I had to get a car to get round as getting to the bus stop or train station was too much of a struggle. After a fairly intense few weeks of physiotherapy, I gained back some movement in the joint and I was walking with some soreness but I was walking again. On a side note, if you have to go to a new physiotherapist, get an idea of what you’re in for by asking who they treat. If county footballers and provincial rugby players are on the list, prepare yourself properly as it will most likely feel great afterwards, but will hurt during. Although I was walking, it was still restricted. Anything over 40 minutes left my back and left leg sore from compensating for my right hip and the right hip would be sore and weak and become extremely stiff afterwards. At the start of March, it had reached the point where I needed it checked out properly and I went to see the orthopaedic surgeon. I was informed it was gone and it needed to be replaced.
By July 2009, I had to change my car to something higher just to be able to get in and out and drive safely. I wasn’t sleeping through the night and walking was getting more difficult. Shots of pain would shoot from my hip and I’d drop to the floor faster than Irish bank shares. I was prescribed strong painkillers and anti-inflammatories for when I needed them. They worked at the start but at the start of 2010, whenever I needed them became every day and the small amount quadrupled (not because I was fast becoming a drug addict).
In April 2010, I got my new hip and the morning after surgery, the physiotherapist arrived to get me out of bed and on my feet for the first time. Top tip for after surgery: if you have a morphine pump as I did, press it as many times as you can when you see the physiotherapist coming. You will be a lot more mobile and it won’t hurt as much afterwards. That is a joke, but there is some truth in it, as the more you move, the quicker things get better. After a week, I was let home. The adjustments had been made to the flat and there were no steps which made it easier. Another great tip: try not to drop the pickup stick on the floor. It is pretty frustrating. After two weeks, I could already do stuff I had not been able to do in a year such as stretching to the side without pain and my personal favourite – sleeping.
After two months, I could walk further than I had in a year and every day, it got stronger.
Looking back now, I have no idea why I was bargaining to try and keep the affected hip any longer. It was like banging your head against a brick wall and wondering why you had a headache. The improvement thankfully up until now is unbelievable. I can walk without pain (some muscle soreness, but that’s nothing). I can drive, I can stand long enough to iron a load of washing (so it’s not all good news) and most importantly, I can plan. I can plan to meet friends and not have to cancel and I can plan holidays. One of the other things I now realise is how much energy goes into your day when trying to just get through and maintain what you have. Fighting to stand still is so much more difficult than fighting to move forward. I feel full of energy that I haven’t felt in so long.
When it comes down to it, I wasn’t left with any real choice to get it done. The quality of my life is so much more important and there wasn’t any quality before the replacement. If you need a replacement of any type and you’re unsure about it, sit down and list the top 5 or 10 things that are most important to you whether it is going to the cinema or walks with the family etc. Think about what you could do before any pain and now, and the answer will come to you pretty quickly.
Slips, Trips & Falls
Slips, trips and falls can happen to anyone, but they are more common and more significant as we get older. This is even more important in people with haemophilia due to repeated bleeding, joint deformities, muscle weakness and impaired balance. About 10% of falls in people with haemophilia result in serious injury. Many falls are preventable and injuries from falls can be minimised.
Physiotherapy and an active lifestyle are important for the preservation of joint function and improving your quality of life. They also play an important role in reducing the risk of falls. Inactive or unfit people tend to have poorer balance and weaker muscles and can be unsteady when walking. Over time, this actually makes you more likely to fall because your muscles get weaker, your joints stiffen, and your balance gets worse.
Physical activity can:
- Improve balance
- Improve muscle strength and flexibility
- Keep bones strong
- Increase energy levels
- Help with sleeping problems
- Help to control blood pressure, blood sugar levels and weight
- Help you to feel good about life
What you can do:
- Be physically active every day. Whatever your age, aim to do at least 30 minutes of activity, at least 5 times a week.
- The activity should make you breathe a bit faster and your heart pump a bit harder, but you should still be able to talk while doing the activity.
- Exercises and activities that make you both stronger and improve your balance lower the risk of having a fall.
- Some examples are Tai Chi, dancing, or group exercise programmes that include balance and muscle strengthening.
- Talk to a physiotherapist about what balance and strengthening exercises or activities will best suit you, and how to manage any pain you might have.
- A physiotherapist can also advise you about whether a walking aid, such as a stick or frame, would help you. Using a walking aid can increase your steadiness and confidence to walk more. It is important that walking aids are properly adjusted for you, and that they are properly maintained (e.g. replace worn stoppers).
- If you have arthritis, being active helps to control pain, weakness and stiffness.
- If you are unable walk, it is still important to get outside and socialise. Aids such as mobility scooters should be considered