Joint Health

FAQs - Joint Health

Having haemophilia means that your blood doesn’t clot as well as it should. This means you are at risk from bleeding inside your body, which might be caused by injury.

There is a risk that you may have a bleed into a joint. Joints are one of the most common sites to bleed – overall, between 7 and 8 out of 10 bleeds in haemophilia are inside a joint.1 Bleeds are most common in the knees, elbows and ankles. But they can also happen in the shoulders, wrists and hips.1

Blood irritates body tissues. Normally, this isn’t a problem because it’s inside the blood vessels. But when it leaks out, it causes inflammation. A bleed inside a joint causes the joint lining to become inflamed, which is painful and damages the joint.2

If it isn’t treated, bleeding into a joint can cause permanent damage.3 Repeated bleeds will wear away the smooth surfaces inside the joint (the cartilage) and cause stiffening of the joint tissues (fibrosis).2 Over time, the joint becomes permanently swollen and deformed.2 This means the joint won’t work as well as it should, and you may become less mobile.2

With better control of bleeding, particularly preventative treatment with factor replacement (prophylaxis), joint deformity is now much less likely to develop.4

A target joint is one that has a tendency to bleed more than others. There are various definitions, but they’re all fairly similar - having at least three or four bleeds into the same joint within six months.2,5 If you have two or fewer bleeds into a joint over 12 months, it is no longer considered a target joint.5

Target joints are more likely to bleed. This is because a type of vicious cycle is set up. When there is a bleed into a joint, the iron in the leaked blood causes inflammation.4 The iron also encourages new blood vessels to grow in the lining of the joint.4 These new blood vessels are very delicate, so they are more likely to be damaged or spontaneously rupture.4 Then more blood leaks into the joint, and so on. Treating with replacement factor can break this cycle and allow a target joint to recover.4,6 Joint bleeds are important because each bleed you have can damage that joint a little more.7  Increasing damage means the joint will take longer to heal and longer for you to get moving again.8 The longer you are off your feet (or not using an arm), the weaker your muscles in that limb will become.8

If you keep bleeding into a target joint, it is likely to become so damaged that the joint becomes permanently swollen and deformed.9 It will become stiff and increasingly difficult to move.9 In turn, the muscles that would normally move the joint weaken.8 A seriously damaged target joint can affect your movement so much that you are permanently disabled.9

Fortunately these days, this degree of disability is less common.10 Improvements in treatments to prevent bleeds (prophylaxis), mean that children with haemophilia can grow up with very little joint damage.4 This is important for adult joint health too.

As well as sticking to your treatment schedule, there are other things you can do to keep your joints healthy. Being active will strengthen the muscles that support your joints and so help to protect them from injury.11-13

If you think you’re developing a target joint, you’ll need to take extra care. Talk to your haemophilia doctor or nurse. They can advise you about what to do – and not do.

There are two main strands to keeping your joints healthy – being diligent with your treatment and being active enough to stay healthy.13,14

Preventing joint damage

If you are on ‘prophylaxis’, you will be having factor replacement regularly to reduce bleeds. This can reduce your risk of joint damage by more than 80%.7  So for people on prophylaxis, an accident or injury is most often the cause of a joint bleed.14 But you will be more likely to have a bleed if you skip treatments.14 You also need to work  with your health team in making sure your factor levels aren’t dipping too low between treatments.14

If you are on prophylaxis, your treatment plan can be personalised to you in order to prevent bleeds as far as possible.6 Your haemophilia team will help you to adapt it to suit, depending on how active you usually are, the sports you take part in, your history of bleeds and your joint health.6

If you have on-demand treatment (factor replacement only if you have a bleed), you are more likely to have spontaneous bleeds - that is bleeds for no apparent reason.14 Whether you have prophylaxis or on-demand treatment, you can help to minimise any damage by treating as soon as you can.14 The quicker you have your treatment, the sooner you will stop the bleeding and minimise any damage to your joints.14

You can also help by:

  • Stick to your treatment plan15
  • Learning to spot the signs of a bleed as early as possible14
  • Communicating with your doctor, nurse and physiotherapist so you work together to keep you healthy14,16 
  • Following their advice if you do have a bleed, in order to reduce your risk of damage14
  • Having factor with you if you’re away from home – ideally a bleed should be treated within two hours15
  • Keeping your Haemtrack record up to date so your specialist has all the information he or she needs in planning and monitoring your treatment15
Keeping your joints healthy

Being active and getting enough exercise is another important part of staying healthy and well.11,13 Keeping yourself fit really does reduce your risk of injury. It builds and strengthens your muscles, which in turn supports your joints.13 Strong muscles reduce the impact on your joints when you are walking or running, for instance.13 Exercise also helps to keep your bones strong – people with haemophilia can be at increased risk of bone thinning (osteoporosis).11 We have more information about staying active when you have haemophilia including a free Active Life fitness support guide that you can download here.

Apart from helping prevent complications of haemophilia, exercise has the same benefits for you as it has for everyone – keeping your weight down and reducing the risk of many chronic illnesses.13

As well as being good for your physical health, we know that exercise is good for your mental health, not least because it’s fun!13 It’s proven to reduce risk of depression.17 Taking part in sports is also a social activity, so encourages friendships too.

Before starting any new activity or sport, it’s best to talk to your health team.11 Depending on what you’re doing, they may recommend that you have an extra dose of factor before exercising.11 This is really important if you have any joints that are prone to bleeds (target joints).11

If you have any concerns that your prophylaxis treatment is not suitably personalised to suit the sports and activities you take part in, contact your haemophilia team.18 They will be pleased that you’re keen to keep yourself well and be just as eager as you to put any problems right.

Between 7 and 8 out of 10 bleeds in haemophilia are inside a joint.1 Over time, joint bleeds can cause serious damage,2 so it’s very important to have the health of your joints assessed regularly.

In order to monitor joint health consistently, physiotherapists use the Haemophilia Joint Health Score (HJHS).19 Your physiotherapist will work through this scoring system with you at least once a year (every 6 months for children).16,20 It will help them assess whether your joint health is stable or getting worse over time.20,21  They will also assess you every time you have a bleed.20 This will help them to pick up any problems early on.20

How the HJHS works

The HJHS looks at the health of the joints most often affected by bleeds: the ankles, elbows and knees.22 Each joint is assessed in eight different ways:

  • Whether there is any swelling21
  • How long there has been swelling21
  • Whether there is any shrinkage (wasting) of surrounding muscles21
  • Whether there is any grating or crackling (either heard or felt) when you move the joint21
  • How well the joint bends21
  • Whether you can fully straighten the joint21
  • Any pain you have in that joint21
  • The strength of the limb21

Your physiotherapist gives you a score for each of these categories for each joint.21 They then score how you walk, run, climb stairs and hop.21

All the scores are added together to give you a total joint health score. Your total for all your joints will be between 0 and 124.21 The lower your score, the healthier your joints are.21

No scoring system will give a complete picture of your health. Your health team will also take into account any bleeds you’ve had and how you feel about your joint health.20 At times, they may also use X-rays, MRI scans or ultrasound to look inside the joint.20,23

Your input is very important - only you know how your joints feel. If you are a parent of a child with haemophilia, pain may be the first sign that there is bleeding into a joint.24 A young child may also limp or favour the other hand, refuse to walk or run, or show more limited movement in that limb.25

If you are at all concerned about your joint health, contact your haemophilia centre, sooner rather than later. The earlier any issues are picked up, the better.

Joints can be affected by different types of bleeds. Traumatic bleeds are caused by an injury or periods of intense activity, such as when playing sports, running or cycling.26,27 Even with mild haemophilia, knocking or falling on a joint might cause bleeding, as blood vessels are damaged.28

Accidents can’t always be prevented and that’s just life. But these days, people with haemophilia can be more active and take part in sports thanks to developments in treatment.11 Your specialist may recommend an extra dose of factor before exercising to reduce the risk of any bleeds.11 Keeping yourself fit can also help to prevent bleeds.13 Being active will strengthen your muscles, which provides support for your joints.13

Spontaneous bleeds happen for no apparent reason.29 These are less likely if you have regular preventative treatment (prophylaxis).14 People with severe haemophilia who have on-demand treatment are much more likely to have spontaneous bleeds.14

Microbleeds are tiny bleeds that are so small that they don’t have any clinical symptoms.4 Even so, over time they can cause damage inside joints.4,30 Again, this is less likely to happen in people with haemophilia who are on prophylaxis.30

Unfortunately, you may still have a bleed even if you’re doing all the right things – keeping yourself fit and making sure you have your prophylaxis if you need it. It’s important not to blame yourself. You’re not doing anything wrong. But it might be a good idea to have a chat with your haemophilia team, just to review your treatment and make sure everything is being done to manage your condition as well as possible.20

The most important thing to do if you think you’ve had a bleed is treat with factor replacement as soon as possible.15 Where at all possible, this should be within two hours of noticing symptoms.15 Even if you think you’re having a joint bleed but are not absolutely sure, it’s best to have a dose of factor.15 You may need to have further doses of factor 12 to 24 hours after a joint bleed.31,32 Contact your haemophilia centre for advice.

Many people with severe haemophilia often have a particular feeling very early on in a bleed, even before there are any clinical signs.15 Doctors call this an ‘aura’.15 Haemophilia specialists advise treating joint bleeds at that point if you can.32 The earlier you treat and stop the bleeding, the less damage will be caused inside the joint.14 That’s why it is so important to always have a factor replacement kit with you.

Apart from having factor, there are other ways you should look after a joint with a bleed. There’s a handy way to remember what to do to minimise stress and help reduce blood flow to the joint. ‘PRICE’ stands for:33

  • Protection – using a splint, for example to prevent movement causing further damage32,33
  • Rest the limb in the position where it is least painful - any movement will encourage blood flow to the joint32
  • Ice – an ice pack or packet of frozen peas wrapped in a towel will cool down the joint and help to numb pain.34 It may also help to reduce swelling.34 NEVER apply ice directly to bare skin.32
  • Compression – applying pressure (such as Tubigrip) helps to keep swelling down by pressing on the blood vessels bringing blood to the joint32
  • Elevation - keeping your leg up or raising an arm on pillows helps to stop bleeding by reducing blood flow to the whole limb32

Do take your usual painkillers.34 Don’t try to be brave. Controlling your pain will help you recover.

While it is important to keep the joint still at first, you need to get it moving as soon as the pain and swelling start to improve.32 Keeping the limb still for too long will mean you lose muscle strength and flexibility in the joint.32 Start by gradually straightening the limb.32 Then move on to exercising the muscles.32 You may need help moving the limb at first.32 You should continue to exercise the limb until you can move and use it normally.32 Don’t go too fast with your exercise regime and do get advice from your physiotherapist.32

Finally, don’t forget to record your bleed and the factor replacement you’ve had on your Haemtrack record.35 Having full details will help your haemophilia specialist to monitor your prophylaxis treatment regime and modify it as necessary, to reduce the risk of future bleeds.36

At any point, if you’re not sure what to do, contact your treatment centre for advice. You should have a 24-hour number to call.

You may notice a number of signs and symptoms if you have a joint bleed. The first may be something health care professionals call an ‘aura’.15 Many people with severe haemophilia have this. It’s a particular feeling very early on in a bleed, even before there are any clinical signs.15 It’s sometimes described as a ‘funny feeling’, tingling or tightness in the joint.32,37

If you are one of those people who has auras, then it’s important to listen to them. Haemophilia specialists advise starting treatment with factor as soon as you notice the aura, rather than waiting for other symptoms.32 Treating early will help to stop bleeding getting worse and minimise damage caused to the inside of the joint.14

Symptoms will vary, depending on how bad the bleed is and how early you are able to treat and control it.

  • The joint may feel warm or very hot38
  • It may be uncomfortable or cause mild, moderate or severe pain38,39
  • There may be slight to severe swelling38,39
  • The area may be red and you may develop some bruising40
  • You may have difficulty moving the joint or be completely unable to move it38,39
  • You may have a feeling of fullness in the joint or it may become very stiff38,39

Joint pain doesn’t always mean that you’ve had a bleed. If you have previous joint damage, you may have arthritic pain in that joint and it’s not always easy to tell the difference.14 Speak to your haemophilia centre for advice – but remember, if in doubt, treat.15

Small children may not be able to explain how their joint is feeling. As a parent, you may need to watch out for other signs, such as:

  • Irritability38
  • Crying38
  • Using the other hand to normal, for example when holding a bottle or toys38
  • Refusing to walk or trying not to bear weight on one leg38
  • Not being able to move the limb as far as normal in any direction25

There are several reasons why it’s important to try and prevent joint bleeds, and to try and minimise the effects of any bleeds you do have.

The main reason is that bleeding causes damage inside your joints.  Any bleeding into a joint will cause inflammation of the joint lining.41 But in research, scientists have demonstrated that permanent joint damage can occur after only a single bleed into the joint.41,42

The inflamed joint lining releases proteins (enzymes) which wear away the smooth cartilage that covers the ends of the bones inside the joint.43 Normally, this cartilage allows the joint to flex and straighten smoothly.43 But as it becomes worn away, there is friction as the two bone ends rub against each other and this is can be very painful.43 These joint changes are similar to the damage caused by severe arthritis and the pain can be similar.39,43

If you have repeated bleeds into the same joint, it will eventually become so damaged that it becomes permanently deformed.2,9 The joint can become misaligned and won’t work properly.2 Understandably, this affects your ability to move freely and you could become permanently disabled.2 Being unable to move a joint is also likely to lead to wasting of the muscles that normally work with that joint.44

Repeated bleeds can also lead to chronic pain in an affected joint.44 Unlike the acute pain you have when you have a bleed, this is permanent so it doesn’t go away when you treat with factor replacement.44

Fortunately, these days this degree of disability is less common.10 Improvements in treatment that prevents bleeds (prophylaxis) means that children with haemophilia usually grow up with little (if any) joint damage.4

Another good reason for preventing joint bleeds is that the more you have, the more there are likely to be in that same joint. When there is a bleed into a joint, new blood vessels grow in the lining of the joint.4 These new blood vessels are very delicate, so they are more likely to be damaged or spontaneously rupture.4 Then more blood leaks into the joint, and so on. Treating with replacement factor can break this cycle and allow a target joint to recover.4,6

Other reasons for preventing joint bleeds may be more immediately obvious to you. They are very painful and seriously affect your day to day life.14,39 When you have a joint bleed, you have to rest until that joint recovers. If you do too much too soon, there’s a risk that it will bleed again.11 Managing your haemophilia well, particularly with preventative factor replacement (prophylaxis) that you can give yourself at home, will mean as little disruption as possible to your school, college, work and social life.14

Joint pain can be put into two categories – acute and chronic. Acute generally means short term and chronic long term. Unfortunately, having pain (whether acute or chronic) is a daily reality for as many as two out of three people with haemophilia.45

Acute joint pain in haemophilia is most often caused by a bleed into a joint. You can usually get it under control quite quickly with factor replacement to control the bleeding.25 The pain inside the joint is caused by swelling and pressure from the bleeding, and by the blood irritating and inflaming the joint tissues.2

It’s important to remember that acute pain doesn’t always mean a bleed.46 People with haemophilia can injure themselves just like anyone else. So sudden pain could be from a fracture or other joint injury.46

Over time, having bleeds can cause permanent damage inside a joint (arthropathy). This results in chronic arthritic pain.47 It can be difficult to tell the difference between chronic arthritic pain and pain caused by a bleed.46 Usually, arthritic pain starts when you are active and goes away after you’ve rested.46

How we feel pain varies a lot between people. What we sometimes call our ‘pain threshold’ can be affected by stress, anxiety, how tired we are and even how much we focus on the pain or manage to distract ourselves from it.48 Older people with haemophilia – who are more likely to have chronic joint damage – often show a remarkable ability to overcome their pain and still have a good quality of life.49 This is probably due to them developing successful ways of coping with it over the years.49 There are successful ways of managing chronic pain, apart from painkillers, although of course, they have a role.50 Exercise, physiotherapy, massage and other complementary therapies can all help.50

Developing chronic pain is no longer inevitable though. With the help of your haemophilia team, it’s now possible to manage and most importantly prevent joint bleeds with good treatment regimes.7,14 If you are able to prevent and minimise damage from joint bleeds, it will prevent chronic joint pain in the future.4

How you manage joint pain depends to some extent on what type of pain it is and what’s causing it. Acute joint pain is usually caused by bleeding into the joint. In this case, the best thing you can do is treat with replacement clotting factor as soon as possible.14 This will stop the bleeding and usually reduces pain quite rapidly.25

Some people sense a bleed is starting very early on. They have an ‘aura’ which is a feeling of fullness or tingling in the joint.32 Haemophilia specialists advise treating with factor at this point if you can.32

You can also help to reduce pain with ‘PRICE’ – Protection, Rest, Ice, Compression and Elevation.33 This means resting the limb, applying an ice pack, applying pressure (with Tubigrip) and raising the limb to reduce blood flow. Be careful when applying ice. You should never put it directly onto bare skin.32 Wrapping a pack of frozen peas in a towel works well. Place over the joint for about 15 minutes at a time and repeat every four to six hours.32

You may need to take painkillers for acute or chronic pain.51 You have to be careful which medicines you take if you have haemophilia.15 Take advice from your haemophilia centre. Never take aspirin because it can make bleeding worse.15 Paracetamol is the painkiller used most often.51 Check with your specialist before taking any over-the-counter anti-inflammatory painkillers such as ibuprofen.15 Many of these can also cause bleeding.15

If you have chronic pain, there are many things that can help. Do talk to your haemophilia team if you have pain, particularly if you feel it is preventing you from living life to the full. Physiotherapy may be beneficial and your physiotherapist will be able to advise on an exercise programme. Movement can help to strengthen muscles and joints and stop them becoming stiffer and less mobile.52 Stronger muscles help to support your joints, reducing the likelihood of injury and bleeds.13,52

Complementary therapies such as massage or hypnosis may help too.50 Your pain is definitely real, but your attitude towards it can make a difference to how it affects your quality of life.49 You can learn to help manage it with techniques including relaxation and distraction.49 Ask for a referral to a pain clinic if you would like further help.

Using a pain rating scale may help you to describe it to health professionals.53 Using one at different times and after different activities can also help you to work out what makes it worse or better. The simplest of these is a scale from one to ten, where one means no pain and ten is the worst pain you can imagine.53,54 There are also versions that children can use, with pictures of happy and unhappy faces that are easier for them to relate to.55 Scales may also ask you to rate how distressing you find your pain and how much it interferes with your sleep, work and everyday activity.53,54 Ask your haemophilia nurse or physiotherapist if you are interested in using a pain rating scale. You can download a free pain rating scale here.

In some cases, if pain is proving very difficult and is seriously interfering with your life, your specialist may suggest other treatments including steroid injections or surgery. Steroid injections are not used often, but they can help to relieve pain in the short term.56 Your specialist may suggest one if you are going to have surgery.56

Your doctor will never suggest surgery lightly. It will usually only be tried if nothing else has helped.23 There are several options, including

  • Aspiration (arthrocentesis) - putting a needle into the joint to draw out the blood.32
  • Arthroscopy - keyhole surgery to release tissues inside the joint that have become stuck together23
  • Synovectomy - removal of the inflamed joint lining or injection to shrink the joint lining23
  • As a last resort, if a severely deformed joint is causing long term severe pain or disability, your specialist may suggest a joint replacement.23 Although this is a big decision to take, it is usually very successful. The operation itself is much safer with modern day factor replacement and brings huge benefits in pain relief and improved mobility.57,58

Please note that the information provided in the above FAQs is accurate as of December 2019.

1. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: Table 1-3: Approximate frequency of bleeding at different sites) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

2. Hemophilia A [Pathophysiology: Hemophilia A] Medscape https://emedicine.medscape.com/article/779322-overview#a3 Last updated January 2019. In.

3. Oxford Handbook of General Practice (4 ed.) Haemophilia and immunology: Haemophilia features] Oxford Medicine Online https://oxfordmedicine.com/view/10.1093/med/9780199671038.001.0001/med-9780199671038-chapter-19#med-9780199671038-chapter-19-div1-6 Published April 2014. In.

4. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Introduction] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

5. Blanchette VS, Key NS, Ljung LR et al Subcommittee on Factor VIII, Factor IX and Rare Coagulation Disorders Definitions in hemophilia: communication from the SSC of the ISTH [Target joint] Journal of Thrombosis and Haemostasis, Nov 2014, vol 12, issue 11, pp1935-9. https://onlinelibrary.wiley.com/doi/full/10.1111/jth.12672. In.

6. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: 1.6 Prophylactic factor replacement therapy) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

7. Haemophilia (Management: Approach) BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/management-approach Last updated June 2019. In.

8. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Management of acute haemarthrosis: Sub-acute phase and rehabilitation.] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

9. Orthopedic Complications and Treatment Related to Chronic Hemarthrosis [Chronic joint arthropathy] Nursing Working Group – Nurses’ Guide to Bleeding Disorders Chapter 9 National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-9-Orthopedic-Complications-Treatment.pdf Published October 2012. In.

10. Haemophilia [Prognosis] BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/prognosis Last updated June 2019. In.

11. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: 1.4 Fitness and physical activity) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

12. Strike K, Mulder K, Michael R. Exercise for haemophilia. [Key results] Cochrane Database of Systematic Reviews 2016, Issue 12. Art. No.: CD011180. DOI: 10.1002/14651858.CD011180.pub2 https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD011180.pub2/epdf/full. In.

13. Wang M, Alvarez-Roman MT, Chowdary P et al Physical activity in individuals with haemophilia and experience with recombinant factor VIII Fc fusion protein and recombinant factor IX Fc fusion protein for the treatment of active patients: a literature review and case reports [Introduction] Blood Coagulation and Fibrinolysis, 2016, vol 27, issue 7, pp737-44. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5054951/#R4. In.

14. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Management of acute haemarthrosis] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

15. Guidelines for the Management of Hemophilia (1.2 Principles of care) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

16. Guidelines for the Management of Hemophilia [1.3 Comprehensive Care] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

17.  Health matters: getting every adult active every day [Benefits of physical activity] Public Health England https://www.gov.uk/government/publications/health-matters-getting-every-adult-active-every-day/health-matters-getting-every-adult-active-every-day Published July 2016. In.

18.  2013/14 NHS Standard contract for haemophilia (all ages) Section B Part 1 - Service Specifications [2.2 Service description/care pathway] NHS England http://haemophilia.org.uk/wp-content/uploads/2017/02/haemophilia_service_specification_pdf.pdf Published October 2013. In.

19.  Richards M, Williams M, Chalmers E et al A United Kingdom Haemophilia Centre Doctors’ Organization guideline approved by the British Committee for Standards in Haematology: guideline on the use of prophylactic factor VIII concentrate in children and adults with severe haemophilia A [Monitoring of prophylaxis: Clinical monitoring] British Journal of Haematology, April 2010, vol 149, issue 4, pp498-507. https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2141.2010.08139.x. In.

20.  Guidelines for the Management of Hemophilia [1.8 Monitoring health status and outcome] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

21.  Hemophilia Joint Health Score Assessment Tool World Federation of Hemophilia http://www1.wfh.org/docs/en/Publications/Assessment_Tools/HJHS_Summary_Score.pdf Last updated February 2011. In.

22. Hemophilia Joint Health Score (HJHS) World Federation of Hemophilia http://elearning.wfh.org/resource/hemophilia-joint-health-score-hjhs/ Last updated February 2011. In.

23.  Guidelines for the Management of Hemophilia [6.1 Musculoskeletal complications] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

24. Caring for your child with hemophilia [Do all children with hemophilia bleed the same?] National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Caring%20for%20Your%20Child.pdf Last updated May 2016. In.

25. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Table 1] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

26.  Playing it Safe: Bleeding disorder, sports and exercise [Before You Start] National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Playing-It-Safe_0.pdf Published April 2017. In.

27. Wang M, Alvarez-Roman MT, Chowdary P et al Physical activity in individuals with haemophilia and experience with recombinant factor VIII Fc fusion protein and recombinant factor IX Fc fusion protein for the treatment of active patients: a literature review and case reports [Physical fitness and activity guidelines for people with haemophilia] Blood Coagulation and Fibrinolysis, 2016, vol 27, issue 7, pp737-44. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5054951/#R4. In.

28. Haemophilia [Classification: Severity of haemophilia A and B based upon plasma levels of factor VIII or IX activity] BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/aetiology Last updated June 2019. In.

29. Guidelines for the Management of Hemophilia [1.1 What is hemophilia] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

30. Manco-Johnson M, Abshire TC, Shapiro AD et al Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe Hemophilia [Discussion] NEJM, August 2007, vol 357, pp545-44. https://www.nejm.org/doi/10.1056/NEJMoa067659?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dwww.ncbi.nlm.nih.gov. In.

31. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Haemostatic management of patients with Haemophilia A and B] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

32. Guidelines for the Management of Hemophilia [5.1 Joint hemorrhage (hemarthrosis)] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1498.pdf Last updated April 2013. In.

33. Guidelines for the Management of Hemophilia (1.5 Adjunctive management) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

34. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Non-haemostatic management] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

35. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: 1.7 Home therapy) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

36. General Information Haemtrack https://apps.mdsas.nhs.uk/Haemtrack/Home/Information Accessed July 2019. In.

37. About Bleeding Disorders: Symptoms and diagnosis World Federation of Hemophilia https://www.wfh.org/en/page.aspx?pid=640 Last updated May 2012. In.

38. Joint Damage [What are the symptoms of a joint bleed?] Hemophilia Federation of America https://www.hemophiliafed.org/understanding-bleeding-disorders/complications/joint-damage/ Accessed July 2019. In.

39. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Table 1. Symptoms and signs of joint bleeds.] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

40. Haemophilia [Diagnosis: Approach: Physical Examination] BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/diagnosis-approach Last updated June 2019. In.

41. Meegeren MER, Roosendaal G, Jansen NWD et al. Blood-Induced Joint Damage: The Devastating Effects of Acute Joint Bleeds versus Micro-Bleeds [Discussion] Cartilage, October 2013, Volume 4, Issue 4, pp313-20. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4297157/. In.

42. Gringeri A, Ewenstein B, Reininger A.  The burden of bleeding in haemophilia: is one bleed too many? [The impact of joint bleeding: Pathophysiology] Haemophilia. Volume 20, pp459–463 Published 2014. https://onlinelibrary.wiley.com/doi/abs/10.1111/hae.12375. In.

43. Joint Damage [How does joint damage occur?] Hemophilia Federation of America https://www.hemophiliafed.org/understanding-bleeding-disorders/complications/joint-damage/ Accessed July 2019. In.

44. Orthopedic Complications and Treatment Related to Chronic Hemarthrosis [Assessment: History and Physical] Nursing Working Group – Nurses’ Guide to Bleeding Disorders Chapter 9 National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-9-Orthopedic-Complications-Treatment.pdf Published October 2012. In.

45. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Introduction] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

46. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Pain is not always a reliable indicator of acute bleeding] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

47. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Chronic pain in haemophilia] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

48. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [What is pain?] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

49. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [How does chronic pain affect quality of life?] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

50. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Beyond medication] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

51. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Analgesic use for acute and chronic pain] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

52. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Physiotherapy and exercise in chronic pain] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

53. Breivik H, Borchgrevink PC, Allen SM et al Assessment of pain [Chronic pain assessment tools] British Journal of Anaesthesia, May 2008, Volume 101, Issue 1, pp17-24. https://academic.oup.com/bja/article/101/1/17/357820#5143824. In.

54. Pain Rating Scale British Pain Society https://www.britishpainsociety.org/static/uploads/resources/files/pain_scales_eng.pdf Published 2006. In.

55. Breivik H, Borchgrevink PC, Allen SM et al Assessment of pain [Assessment of intensity of acute pain] British Journal of Anaesthesia, May 2008, Volume 101, Issue 1, pp17-24. https://academic.oup.com/bja/article/101/1/17/357820#5143824. In.

56. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Management of chronic synovitis and target joints: Intra-articular steroid injection] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

57. Joint replacement surgery in hemophiia [Introduction] World Federation of Hemophilia http://www1.wfh.org/publication/files/pdf-1210.pdf Published June 2010. In.

58. Joint replacement surgery in hemophiia [Conclusions] World Federation of Hemophilia http://www1.wfh.org/publication/files/pdf-1210.pdf Published June 2010. In.

Click here to read more about life beyond haemophilia

Click here to download a joint health discussion guide
 

NP-9305
January 2020

FAQs - Joint Health

Having haemophilia means that your blood doesn’t clot as well as it should. This means you are at risk from bleeding inside your body, which might be caused by injury.

There is a risk that you may have a bleed into a joint. Joints are one of the most common sites to bleed – overall, between 7 and 8 out of 10 bleeds in haemophilia are inside a joint.1 Bleeds are most common in the knees, elbows and ankles. But they can also happen in the shoulders, wrists and hips.1

Blood irritates body tissues. Normally, this isn’t a problem because it’s inside the blood vessels. But when it leaks out, it causes inflammation. A bleed inside a joint causes the joint lining to become inflamed, which is painful and damages the joint.2

If it isn’t treated, bleeding into a joint can cause permanent damage.3 Repeated bleeds will wear away the smooth surfaces inside the joint (the cartilage) and cause stiffening of the joint tissues (fibrosis).2 Over time, the joint becomes permanently swollen and deformed.2 This means the joint won’t work as well as it should, and you may become less mobile.2

With better control of bleeding, particularly preventative treatment with factor replacement (prophylaxis), joint deformity is now much less likely to develop.4

A target joint is one that has a tendency to bleed more than others. There are various definitions, but they’re all fairly similar - having at least three or four bleeds into the same joint within six months.2,5 If you have two or fewer bleeds into a joint over 12 months, it is no longer considered a target joint.5

Target joints are more likely to bleed. This is because a type of vicious cycle is set up. When there is a bleed into a joint, the iron in the leaked blood causes inflammation.4 The iron also encourages new blood vessels to grow in the lining of the joint.4 These new blood vessels are very delicate, so they are more likely to be damaged or spontaneously rupture.4 Then more blood leaks into the joint, and so on. Treating with replacement factor can break this cycle and allow a target joint to recover.4,6 Joint bleeds are important because each bleed you have can damage that joint a little more.7  Increasing damage means the joint will take longer to heal and longer for you to get moving again.8 The longer you are off your feet (or not using an arm), the weaker your muscles in that limb will become.8

If you keep bleeding into a target joint, it is likely to become so damaged that the joint becomes permanently swollen and deformed.9 It will become stiff and increasingly difficult to move.9 In turn, the muscles that would normally move the joint weaken.8 A seriously damaged target joint can affect your movement so much that you are permanently disabled.9

Fortunately these days, this degree of disability is less common.10 Improvements in treatments to prevent bleeds (prophylaxis), mean that children with haemophilia can grow up with very little joint damage.4 This is important for adult joint health too.

As well as sticking to your treatment schedule, there are other things you can do to keep your joints healthy. Being active will strengthen the muscles that support your joints and so help to protect them from injury.11-13

If you think you’re developing a target joint, you’ll need to take extra care. Talk to your haemophilia doctor or nurse. They can advise you about what to do – and not do.

There are two main strands to keeping your joints healthy – being diligent with your treatment and being active enough to stay healthy.13,14

Preventing joint damage

If you are on ‘prophylaxis’, you will be having factor replacement regularly to reduce bleeds. This can reduce your risk of joint damage by more than 80%.7  So for people on prophylaxis, an accident or injury is most often the cause of a joint bleed.14 But you will be more likely to have a bleed if you skip treatments.14 You also need to work  with your health team in making sure your factor levels aren’t dipping too low between treatments.14

If you are on prophylaxis, your treatment plan can be personalised to you in order to prevent bleeds as far as possible.6 Your haemophilia team will help you to adapt it to suit, depending on how active you usually are, the sports you take part in, your history of bleeds and your joint health.6

If you have on-demand treatment (factor replacement only if you have a bleed), you are more likely to have spontaneous bleeds - that is bleeds for no apparent reason.14 Whether you have prophylaxis or on-demand treatment, you can help to minimise any damage by treating as soon as you can.14 The quicker you have your treatment, the sooner you will stop the bleeding and minimise any damage to your joints.14

You can also help by:

  • Stick to your treatment plan15
  • Learning to spot the signs of a bleed as early as possible14
  • Communicating with your doctor, nurse and physiotherapist so you work together to keep you healthy14,16 
  • Following their advice if you do have a bleed, in order to reduce your risk of damage14
  • Having factor with you if you’re away from home – ideally a bleed should be treated within two hours15
  • Keeping your Haemtrack record up to date so your specialist has all the information he or she needs in planning and monitoring your treatment15
Keeping your joints healthy

Being active and getting enough exercise is another important part of staying healthy and well.11,13 Keeping yourself fit really does reduce your risk of injury. It builds and strengthens your muscles, which in turn supports your joints.13 Strong muscles reduce the impact on your joints when you are walking or running, for instance.13 Exercise also helps to keep your bones strong – people with haemophilia can be at increased risk of bone thinning (osteoporosis).11 We have more information about staying active when you have haemophilia including a free Active Life fitness support guide that you can download here.

Apart from helping prevent complications of haemophilia, exercise has the same benefits for you as it has for everyone – keeping your weight down and reducing the risk of many chronic illnesses.13

As well as being good for your physical health, we know that exercise is good for your mental health, not least because it’s fun!13 It’s proven to reduce risk of depression.17 Taking part in sports is also a social activity, so encourages friendships too.

Before starting any new activity or sport, it’s best to talk to your health team.11 Depending on what you’re doing, they may recommend that you have an extra dose of factor before exercising.11 This is really important if you have any joints that are prone to bleeds (target joints).11

If you have any concerns that your prophylaxis treatment is not suitably personalised to suit the sports and activities you take part in, contact your haemophilia team.18 They will be pleased that you’re keen to keep yourself well and be just as eager as you to put any problems right.

Between 7 and 8 out of 10 bleeds in haemophilia are inside a joint.1 Over time, joint bleeds can cause serious damage,2 so it’s very important to have the health of your joints assessed regularly.

In order to monitor joint health consistently, physiotherapists use the Haemophilia Joint Health Score (HJHS).19 Your physiotherapist will work through this scoring system with you at least once a year (every 6 months for children).16,20 It will help them assess whether your joint health is stable or getting worse over time.20,21  They will also assess you every time you have a bleed.20 This will help them to pick up any problems early on.20

How the HJHS works

The HJHS looks at the health of the joints most often affected by bleeds: the ankles, elbows and knees.22 Each joint is assessed in eight different ways:

  • Whether there is any swelling21
  • How long there has been swelling21
  • Whether there is any shrinkage (wasting) of surrounding muscles21
  • Whether there is any grating or crackling (either heard or felt) when you move the joint21
  • How well the joint bends21
  • Whether you can fully straighten the joint21
  • Any pain you have in that joint21
  • The strength of the limb21

Your physiotherapist gives you a score for each of these categories for each joint.21 They then score how you walk, run, climb stairs and hop.21

All the scores are added together to give you a total joint health score. Your total for all your joints will be between 0 and 124.21 The lower your score, the healthier your joints are.21

No scoring system will give a complete picture of your health. Your health team will also take into account any bleeds you’ve had and how you feel about your joint health.20 At times, they may also use X-rays, MRI scans or ultrasound to look inside the joint.20,23

Your input is very important - only you know how your joints feel. If you are a parent of a child with haemophilia, pain may be the first sign that there is bleeding into a joint.24 A young child may also limp or favour the other hand, refuse to walk or run, or show more limited movement in that limb.25

If you are at all concerned about your joint health, contact your haemophilia centre, sooner rather than later. The earlier any issues are picked up, the better.

Joints can be affected by different types of bleeds. Traumatic bleeds are caused by an injury or periods of intense activity, such as when playing sports, running or cycling.26,27 Even with mild haemophilia, knocking or falling on a joint might cause bleeding, as blood vessels are damaged.28

Accidents can’t always be prevented and that’s just life. But these days, people with haemophilia can be more active and take part in sports thanks to developments in treatment.11 Your specialist may recommend an extra dose of factor before exercising to reduce the risk of any bleeds.11 Keeping yourself fit can also help to prevent bleeds.13 Being active will strengthen your muscles, which provides support for your joints.13

Spontaneous bleeds happen for no apparent reason.29 These are less likely if you have regular preventative treatment (prophylaxis).14 People with severe haemophilia who have on-demand treatment are much more likely to have spontaneous bleeds.14

Microbleeds are tiny bleeds that are so small that they don’t have any clinical symptoms.4 Even so, over time they can cause damage inside joints.4,30 Again, this is less likely to happen in people with haemophilia who are on prophylaxis.30

Unfortunately, you may still have a bleed even if you’re doing all the right things – keeping yourself fit and making sure you have your prophylaxis if you need it. It’s important not to blame yourself. You’re not doing anything wrong. But it might be a good idea to have a chat with your haemophilia team, just to review your treatment and make sure everything is being done to manage your condition as well as possible.20

The most important thing to do if you think you’ve had a bleed is treat with factor replacement as soon as possible.15 Where at all possible, this should be within two hours of noticing symptoms.15 Even if you think you’re having a joint bleed but are not absolutely sure, it’s best to have a dose of factor.15 You may need to have further doses of factor 12 to 24 hours after a joint bleed.31,32 Contact your haemophilia centre for advice.

Many people with severe haemophilia often have a particular feeling very early on in a bleed, even before there are any clinical signs.15 Doctors call this an ‘aura’.15 Haemophilia specialists advise treating joint bleeds at that point if you can.32 The earlier you treat and stop the bleeding, the less damage will be caused inside the joint.14 That’s why it is so important to always have a factor replacement kit with you.

Apart from having factor, there are other ways you should look after a joint with a bleed. There’s a handy way to remember what to do to minimise stress and help reduce blood flow to the joint. ‘PRICE’ stands for:33

  • Protection – using a splint, for example to prevent movement causing further damage32,33
  • Rest the limb in the position where it is least painful - any movement will encourage blood flow to the joint32
  • Ice – an ice pack or packet of frozen peas wrapped in a towel will cool down the joint and help to numb pain.34 It may also help to reduce swelling.34 NEVER apply ice directly to bare skin.32
  • Compression – applying pressure (such as Tubigrip) helps to keep swelling down by pressing on the blood vessels bringing blood to the joint32
  • Elevation - keeping your leg up or raising an arm on pillows helps to stop bleeding by reducing blood flow to the whole limb32

Do take your usual painkillers.34 Don’t try to be brave. Controlling your pain will help you recover.

While it is important to keep the joint still at first, you need to get it moving as soon as the pain and swelling start to improve.32 Keeping the limb still for too long will mean you lose muscle strength and flexibility in the joint.32 Start by gradually straightening the limb.32 Then move on to exercising the muscles.32 You may need help moving the limb at first.32 You should continue to exercise the limb until you can move and use it normally.32 Don’t go too fast with your exercise regime and do get advice from your physiotherapist.32

Finally, don’t forget to record your bleed and the factor replacement you’ve had on your Haemtrack record.35 Having full details will help your haemophilia specialist to monitor your prophylaxis treatment regime and modify it as necessary, to reduce the risk of future bleeds.36

At any point, if you’re not sure what to do, contact your treatment centre for advice. You should have a 24-hour number to call.

You may notice a number of signs and symptoms if you have a joint bleed. The first may be something health care professionals call an ‘aura’.15 Many people with severe haemophilia have this. It’s a particular feeling very early on in a bleed, even before there are any clinical signs.15 It’s sometimes described as a ‘funny feeling’, tingling or tightness in the joint.32,37

If you are one of those people who has auras, then it’s important to listen to them. Haemophilia specialists advise starting treatment with factor as soon as you notice the aura, rather than waiting for other symptoms.32 Treating early will help to stop bleeding getting worse and minimise damage caused to the inside of the joint.14

Symptoms will vary, depending on how bad the bleed is and how early you are able to treat and control it.

  • The joint may feel warm or very hot38
  • It may be uncomfortable or cause mild, moderate or severe pain38,39
  • There may be slight to severe swelling38,39
  • The area may be red and you may develop some bruising40
  • You may have difficulty moving the joint or be completely unable to move it38,39
  • You may have a feeling of fullness in the joint or it may become very stiff38,39

Joint pain doesn’t always mean that you’ve had a bleed. If you have previous joint damage, you may have arthritic pain in that joint and it’s not always easy to tell the difference.14 Speak to your haemophilia centre for advice – but remember, if in doubt, treat.15

Small children may not be able to explain how their joint is feeling. As a parent, you may need to watch out for other signs, such as:

  • Irritability38
  • Crying38
  • Using the other hand to normal, for example when holding a bottle or toys38
  • Refusing to walk or trying not to bear weight on one leg38
  • Not being able to move the limb as far as normal in any direction25

There are several reasons why it’s important to try and prevent joint bleeds, and to try and minimise the effects of any bleeds you do have.

The main reason is that bleeding causes damage inside your joints.  Any bleeding into a joint will cause inflammation of the joint lining.41 But in research, scientists have demonstrated that permanent joint damage can occur after only a single bleed into the joint.41,42

The inflamed joint lining releases proteins (enzymes) which wear away the smooth cartilage that covers the ends of the bones inside the joint.43 Normally, this cartilage allows the joint to flex and straighten smoothly.43 But as it becomes worn away, there is friction as the two bone ends rub against each other and this is can be very painful.43 These joint changes are similar to the damage caused by severe arthritis and the pain can be similar.39,43

If you have repeated bleeds into the same joint, it will eventually become so damaged that it becomes permanently deformed.2,9 The joint can become misaligned and won’t work properly.2 Understandably, this affects your ability to move freely and you could become permanently disabled.2 Being unable to move a joint is also likely to lead to wasting of the muscles that normally work with that joint.44

Repeated bleeds can also lead to chronic pain in an affected joint.44 Unlike the acute pain you have when you have a bleed, this is permanent so it doesn’t go away when you treat with factor replacement.44

Fortunately, these days this degree of disability is less common.10 Improvements in treatment that prevents bleeds (prophylaxis) means that children with haemophilia usually grow up with little (if any) joint damage.4

Another good reason for preventing joint bleeds is that the more you have, the more there are likely to be in that same joint. When there is a bleed into a joint, new blood vessels grow in the lining of the joint.4 These new blood vessels are very delicate, so they are more likely to be damaged or spontaneously rupture.4 Then more blood leaks into the joint, and so on. Treating with replacement factor can break this cycle and allow a target joint to recover.4,6

Other reasons for preventing joint bleeds may be more immediately obvious to you. They are very painful and seriously affect your day to day life.14,39 When you have a joint bleed, you have to rest until that joint recovers. If you do too much too soon, there’s a risk that it will bleed again.11 Managing your haemophilia well, particularly with preventative factor replacement (prophylaxis) that you can give yourself at home, will mean as little disruption as possible to your school, college, work and social life.14

Joint pain can be put into two categories – acute and chronic. Acute generally means short term and chronic long term. Unfortunately, having pain (whether acute or chronic) is a daily reality for as many as two out of three people with haemophilia.45

Acute joint pain in haemophilia is most often caused by a bleed into a joint. You can usually get it under control quite quickly with factor replacement to control the bleeding.25 The pain inside the joint is caused by swelling and pressure from the bleeding, and by the blood irritating and inflaming the joint tissues.2

It’s important to remember that acute pain doesn’t always mean a bleed.46 People with haemophilia can injure themselves just like anyone else. So sudden pain could be from a fracture or other joint injury.46

Over time, having bleeds can cause permanent damage inside a joint (arthropathy). This results in chronic arthritic pain.47 It can be difficult to tell the difference between chronic arthritic pain and pain caused by a bleed.46 Usually, arthritic pain starts when you are active and goes away after you’ve rested.46

How we feel pain varies a lot between people. What we sometimes call our ‘pain threshold’ can be affected by stress, anxiety, how tired we are and even how much we focus on the pain or manage to distract ourselves from it.48 Older people with haemophilia – who are more likely to have chronic joint damage – often show a remarkable ability to overcome their pain and still have a good quality of life.49 This is probably due to them developing successful ways of coping with it over the years.49 There are successful ways of managing chronic pain, apart from painkillers, although of course, they have a role.50 Exercise, physiotherapy, massage and other complementary therapies can all help.50

Developing chronic pain is no longer inevitable though. With the help of your haemophilia team, it’s now possible to manage and most importantly prevent joint bleeds with good treatment regimes.7,14 If you are able to prevent and minimise damage from joint bleeds, it will prevent chronic joint pain in the future.4

How you manage joint pain depends to some extent on what type of pain it is and what’s causing it. Acute joint pain is usually caused by bleeding into the joint. In this case, the best thing you can do is treat with replacement clotting factor as soon as possible.14 This will stop the bleeding and usually reduces pain quite rapidly.25

Some people sense a bleed is starting very early on. They have an ‘aura’ which is a feeling of fullness or tingling in the joint.32 Haemophilia specialists advise treating with factor at this point if you can.32

You can also help to reduce pain with ‘PRICE’ – Protection, Rest, Ice, Compression and Elevation.33 This means resting the limb, applying an ice pack, applying pressure (with Tubigrip) and raising the limb to reduce blood flow. Be careful when applying ice. You should never put it directly onto bare skin.32 Wrapping a pack of frozen peas in a towel works well. Place over the joint for about 15 minutes at a time and repeat every four to six hours.32

You may need to take painkillers for acute or chronic pain.51 You have to be careful which medicines you take if you have haemophilia.15 Take advice from your haemophilia centre. Never take aspirin because it can make bleeding worse.15 Paracetamol is the painkiller used most often.51 Check with your specialist before taking any over-the-counter anti-inflammatory painkillers such as ibuprofen.15 Many of these can also cause bleeding.15

If you have chronic pain, there are many things that can help. Do talk to your haemophilia team if you have pain, particularly if you feel it is preventing you from living life to the full. Physiotherapy may be beneficial and your physiotherapist will be able to advise on an exercise programme. Movement can help to strengthen muscles and joints and stop them becoming stiffer and less mobile.52 Stronger muscles help to support your joints, reducing the likelihood of injury and bleeds.13,52

Complementary therapies such as massage or hypnosis may help too.50 Your pain is definitely real, but your attitude towards it can make a difference to how it affects your quality of life.49 You can learn to help manage it with techniques including relaxation and distraction.49 Ask for a referral to a pain clinic if you would like further help.

Using a pain rating scale may help you to describe it to health professionals.53 Using one at different times and after different activities can also help you to work out what makes it worse or better. The simplest of these is a scale from one to ten, where one means no pain and ten is the worst pain you can imagine.53,54 There are also versions that children can use, with pictures of happy and unhappy faces that are easier for them to relate to.55 Scales may also ask you to rate how distressing you find your pain and how much it interferes with your sleep, work and everyday activity.53,54 Ask your haemophilia nurse or physiotherapist if you are interested in using a pain rating scale. You can download a free pain rating scale here.

In some cases, if pain is proving very difficult and is seriously interfering with your life, your specialist may suggest other treatments including steroid injections or surgery. Steroid injections are not used often, but they can help to relieve pain in the short term.56 Your specialist may suggest one if you are going to have surgery.56

Your doctor will never suggest surgery lightly. It will usually only be tried if nothing else has helped.23 There are several options, including

  • Aspiration (arthrocentesis) - putting a needle into the joint to draw out the blood.32
  • Arthroscopy - keyhole surgery to release tissues inside the joint that have become stuck together23
  • Synovectomy - removal of the inflamed joint lining or injection to shrink the joint lining23
  • As a last resort, if a severely deformed joint is causing long term severe pain or disability, your specialist may suggest a joint replacement.23 Although this is a big decision to take, it is usually very successful. The operation itself is much safer with modern day factor replacement and brings huge benefits in pain relief and improved mobility.57,58

Please note that the information provided in the above FAQs is accurate as of December 2019.

1. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: Table 1-3: Approximate frequency of bleeding at different sites) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

2. Hemophilia A [Pathophysiology: Hemophilia A] Medscape https://emedicine.medscape.com/article/779322-overview#a3 Last updated January 2019. In.

3. Oxford Handbook of General Practice (4 ed.) Haemophilia and immunology: Haemophilia features] Oxford Medicine Online https://oxfordmedicine.com/view/10.1093/med/9780199671038.001.0001/med-9780199671038-chapter-19#med-9780199671038-chapter-19-div1-6 Published April 2014. In.

4. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Introduction] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

5. Blanchette VS, Key NS, Ljung LR et al Subcommittee on Factor VIII, Factor IX and Rare Coagulation Disorders Definitions in hemophilia: communication from the SSC of the ISTH [Target joint] Journal of Thrombosis and Haemostasis, Nov 2014, vol 12, issue 11, pp1935-9. https://onlinelibrary.wiley.com/doi/full/10.1111/jth.12672. In.

6. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: 1.6 Prophylactic factor replacement therapy) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

7. Haemophilia (Management: Approach) BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/management-approach Last updated June 2019. In.

8. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Management of acute haemarthrosis: Sub-acute phase and rehabilitation.] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

9. Orthopedic Complications and Treatment Related to Chronic Hemarthrosis [Chronic joint arthropathy] Nursing Working Group – Nurses’ Guide to Bleeding Disorders Chapter 9 National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-9-Orthopedic-Complications-Treatment.pdf Published October 2012. In.

10. Haemophilia [Prognosis] BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/prognosis Last updated June 2019. In.

11. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: 1.4 Fitness and physical activity) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

12. Strike K, Mulder K, Michael R. Exercise for haemophilia. [Key results] Cochrane Database of Systematic Reviews 2016, Issue 12. Art. No.: CD011180. DOI: 10.1002/14651858.CD011180.pub2 https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD011180.pub2/epdf/full. In.

13. Wang M, Alvarez-Roman MT, Chowdary P et al Physical activity in individuals with haemophilia and experience with recombinant factor VIII Fc fusion protein and recombinant factor IX Fc fusion protein for the treatment of active patients: a literature review and case reports [Introduction] Blood Coagulation and Fibrinolysis, 2016, vol 27, issue 7, pp737-44. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5054951/#R4. In.

14. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Management of acute haemarthrosis] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

15. Guidelines for the Management of Hemophilia (1.2 Principles of care) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

16. Guidelines for the Management of Hemophilia [1.3 Comprehensive Care] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

17.  Health matters: getting every adult active every day [Benefits of physical activity] Public Health England https://www.gov.uk/government/publications/health-matters-getting-every-adult-active-every-day/health-matters-getting-every-adult-active-every-day Published July 2016. In.

18.  2013/14 NHS Standard contract for haemophilia (all ages) Section B Part 1 - Service Specifications [2.2 Service description/care pathway] NHS England http://haemophilia.org.uk/wp-content/uploads/2017/02/haemophilia_service_specification_pdf.pdf Published October 2013. In.

19.  Richards M, Williams M, Chalmers E et al A United Kingdom Haemophilia Centre Doctors’ Organization guideline approved by the British Committee for Standards in Haematology: guideline on the use of prophylactic factor VIII concentrate in children and adults with severe haemophilia A [Monitoring of prophylaxis: Clinical monitoring] British Journal of Haematology, April 2010, vol 149, issue 4, pp498-507. https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2141.2010.08139.x. In.

20.  Guidelines for the Management of Hemophilia [1.8 Monitoring health status and outcome] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

21.  Hemophilia Joint Health Score Assessment Tool World Federation of Hemophilia http://www1.wfh.org/docs/en/Publications/Assessment_Tools/HJHS_Summary_Score.pdf Last updated February 2011. In.

22. Hemophilia Joint Health Score (HJHS) World Federation of Hemophilia http://elearning.wfh.org/resource/hemophilia-joint-health-score-hjhs/ Last updated February 2011. In.

23.  Guidelines for the Management of Hemophilia [6.1 Musculoskeletal complications] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

24. Caring for your child with hemophilia [Do all children with hemophilia bleed the same?] National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Caring%20for%20Your%20Child.pdf Last updated May 2016. In.

25. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Table 1] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

26.  Playing it Safe: Bleeding disorder, sports and exercise [Before You Start] National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Playing-It-Safe_0.pdf Published April 2017. In.

27. Wang M, Alvarez-Roman MT, Chowdary P et al Physical activity in individuals with haemophilia and experience with recombinant factor VIII Fc fusion protein and recombinant factor IX Fc fusion protein for the treatment of active patients: a literature review and case reports [Physical fitness and activity guidelines for people with haemophilia] Blood Coagulation and Fibrinolysis, 2016, vol 27, issue 7, pp737-44. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5054951/#R4. In.

28. Haemophilia [Classification: Severity of haemophilia A and B based upon plasma levels of factor VIII or IX activity] BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/aetiology Last updated June 2019. In.

29. Guidelines for the Management of Hemophilia [1.1 What is hemophilia] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

30. Manco-Johnson M, Abshire TC, Shapiro AD et al Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe Hemophilia [Discussion] NEJM, August 2007, vol 357, pp545-44. https://www.nejm.org/doi/10.1056/NEJMoa067659?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dwww.ncbi.nlm.nih.gov. In.

31. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Haemostatic management of patients with Haemophilia A and B] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

32. Guidelines for the Management of Hemophilia [5.1 Joint hemorrhage (hemarthrosis)] World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1498.pdf Last updated April 2013. In.

33. Guidelines for the Management of Hemophilia (1.5 Adjunctive management) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1472.pdf Last updated April 2013. In.

34. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Non-haemostatic management] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

35. Guidelines for the Management of Hemophilia (General Care and Management of Hemophilia: 1.7 Home therapy) World Federation of Hemophilia http://www1.wfh.org/publications/files/pdf-1494.pdf Last updated April 2013. In.

36. General Information Haemtrack https://apps.mdsas.nhs.uk/Haemtrack/Home/Information Accessed July 2019. In.

37. About Bleeding Disorders: Symptoms and diagnosis World Federation of Hemophilia https://www.wfh.org/en/page.aspx?pid=640 Last updated May 2012. In.

38. Joint Damage [What are the symptoms of a joint bleed?] Hemophilia Federation of America https://www.hemophiliafed.org/understanding-bleeding-disorders/complications/joint-damage/ Accessed July 2019. In.

39. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Table 1. Symptoms and signs of joint bleeds.] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

40. Haemophilia [Diagnosis: Approach: Physical Examination] BMJ Best Practice https://bestpractice.bmj.com/topics/en-gb/468/diagnosis-approach Last updated June 2019. In.

41. Meegeren MER, Roosendaal G, Jansen NWD et al. Blood-Induced Joint Damage: The Devastating Effects of Acute Joint Bleeds versus Micro-Bleeds [Discussion] Cartilage, October 2013, Volume 4, Issue 4, pp313-20. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4297157/. In.

42. Gringeri A, Ewenstein B, Reininger A.  The burden of bleeding in haemophilia: is one bleed too many? [The impact of joint bleeding: Pathophysiology] Haemophilia. Volume 20, pp459–463 Published 2014. https://onlinelibrary.wiley.com/doi/abs/10.1111/hae.12375. In.

43. Joint Damage [How does joint damage occur?] Hemophilia Federation of America https://www.hemophiliafed.org/understanding-bleeding-disorders/complications/joint-damage/ Accessed July 2019. In.

44. Orthopedic Complications and Treatment Related to Chronic Hemarthrosis [Assessment: History and Physical] Nursing Working Group – Nurses’ Guide to Bleeding Disorders Chapter 9 National Hemophilia Foundation https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-9-Orthopedic-Complications-Treatment.pdf Published October 2012. In.

45. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Introduction] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

46. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Pain is not always a reliable indicator of acute bleeding] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

47. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Chronic pain in haemophilia] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

48. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [What is pain?] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

49. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [How does chronic pain affect quality of life?] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

50. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Beyond medication] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

51. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Analgesic use for acute and chronic pain] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

52. Auerswald G, Dolan G, Duffy A et al Pain and pain management in haemophilia. [Physiotherapy and exercise in chronic pain] Blood Coagulation and Fibrinolysis, Dec 2016, Volume 27, Issue 8, pp845-54. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087566/. In.

53. Breivik H, Borchgrevink PC, Allen SM et al Assessment of pain [Chronic pain assessment tools] British Journal of Anaesthesia, May 2008, Volume 101, Issue 1, pp17-24. https://academic.oup.com/bja/article/101/1/17/357820#5143824. In.

54. Pain Rating Scale British Pain Society https://www.britishpainsociety.org/static/uploads/resources/files/pain_scales_eng.pdf Published 2006. In.

55. Breivik H, Borchgrevink PC, Allen SM et al Assessment of pain [Assessment of intensity of acute pain] British Journal of Anaesthesia, May 2008, Volume 101, Issue 1, pp17-24. https://academic.oup.com/bja/article/101/1/17/357820#5143824. In.

56. Hanley J, McKernan A, Creagh MD et al Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia [Management of chronic synovitis and target joints: Intra-articular steroid injection] Haemophilia, 2017, 1-10. http://www.ukhcdo.org/wp-content/uploads/2017/03/Guidelines-for-the-management-of-acute-joint-bleeds-and-chronic-synovitis-in-haemophilia.pdf. In.

57. Joint replacement surgery in hemophiia [Introduction] World Federation of Hemophilia http://www1.wfh.org/publication/files/pdf-1210.pdf Published June 2010. In.

58. Joint replacement surgery in hemophiia [Conclusions] World Federation of Hemophilia http://www1.wfh.org/publication/files/pdf-1210.pdf Published June 2010. In.

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NP-9305
January 2020