Haemophilia: Signs, risks and treatment of internal bleeding

People living with haemophilia are prone to slow, steady and continuous bleeding after minor trauma1  – or after no trauma at all, in the case of severe haemophilia2.

The amount of clotting factor in the blood directly determines the seriousness of the condition3. Almost all people with haemophilia are men, and just over one third of them have severe haemophilia. The rest have the mild or moderate type4.

This is general disease awareness and should not be understood as medical advice. If you experience symptoms of COVID-19 or have questions, doubts or concerns, you should contact your doctor. Always follow the advice of local authorities.


Published 04 May 2020 | 3 min read
 


While people with haemophilia can experience bleeding both externally and internally, this blog post focuses on internal bleeds as these are the biggest concern for people with haemophilia5.

The seriousness of bleeds can vary significantly – from superficial bruising, which is treatable at home, to bleeds requiring visits to the hospital or haemophilia treatment centre such as head, throat or stomach bleeds6. In some cases, a bleed may take several weeks to recover7.

The seriousness of bleeds can vary significantly

When it comes to joint bleeds, the elbow and knee joints are particularly susceptible8, but bleeds can occur in multiple parts of the body. Here are a handful of other areas commonly affected:

 

  • the area just under the skin (superficial bruising)
  • the area some distance beneath the skin (deeper bruising, which may result in lumps or haematomas)
  • the area deep in the muscles
  • the nasal mucosa (the inside of the nose)

 

When it comes to frequency, people with severe haemophilia may have 2-3 bleeds per month, while those who are moderately affected may have 5-6 bleeds per year10. However, even in those living with severe haemophilia, the bleed frequency may vary a lot and is likely to depend on such things as activity. People with mild haemophilia experience bleeds even less frequently, with one study citing just one (joint) bleed per year in a patient with mild haemophilia A11.

Because of the potential after-effects of a bleed, people with haemophilia cannot just wait them out12. If joint bleeds are repeatedly left untreated, the long-term effects can be arthritis or destruction of the joint. All bleeds should be given immediate attention and sometimes immediate intervention13.

 

How can a bleed be detected?

Before you can respond to a bleed, you obviously need to know what one looks and feels like. So, what are the early signs of a bleed? When there are no visual symptoms, a warm, tingling or prickly sensation in the affected area can be evidence of bleeding. Other signs are soreness, pain or a feeling of pressure14.

In the specific case of joint bleeds, the experience is a little different. Early signs of such an episode include:

 

● a “funny”, tingling or bubbling sensation in the joint

● pain, most commonly in a leg or arm

● reluctance to use a joint fully

● warmth in the affected area15

 

Later, a joint bleed sometimes results in:

 

● stiffness and/or swelling in the joint

● the joint being warm to the touch16

 

To minimise the restrictions such symptoms may impose, people commonly alter and compensate for the familiar or natural use of their limbs17. In the case of a joint bleed in the right elbow, for example, a right-handed person may choose to use the left arm instead. Becoming aware of such compensatory use, haemophilia patients as well as carers should be alert.

What should be the response to bleeds?

As stated above, some bleeds don’t require any medical attention.

But in case of uncertainty about either 1) what is going on internally and/or 2) the necessity of medical attention, it’s best to check with the haemophilia treatment centre18. A healthcare professional may choose to administer clotting factor concentrate, which is known as factor replacement therapy19. While superficial bruises don’t need any medical treatment, treatment is occasionally needed in the case of deeper bruises or bruises in tight areas (such as the forearm – to avoid nerve compression).

Illustration: Man playing with a ball inside a house. Text: Physical activity has a number of benefits. It can prevent bleeds and joint damage if done properly.

In the event of a slow bleed, the word “PRICE” is a useful summing-up of the kind of First Aid treatment that people with haemophilia should self-apply (or be treated to from carers)20:

 

● P stands for Protection

● R stands for Rest

● I stands for Ice

● C stands for Compression

● E stands for Elevation

 

In other words, to reduce swelling and prevent further bleeding, implement immediate Protection and Rest while applying Ice and a Compressive bandage to the affected and preferably Elevated area. By Protection we mean reducing stress or weight bearing on the affected joint or muscle, for instance by using crutches or other supports21

The PRICE routine should be continued throughout the healing process, which can take several days or even weeks.

In general, clotting factor treatment may be needed for joint and muscle bleeds22.  Again, people living with haemophilia ought to get in touch with their haemophilia treatment centre if they need medical attention or wish to understand their general condition better.

All bleeds should be given immediate attention and sometimes immedite intervention.

Living with haemophilia

Living with haemophilia does not mean having to give up on having a fulfilling and active life23,  but it does mean having an understanding of one’s condition, individual limitations and medical needs. Caring properly for bleeds is one of the essential parts of maintaining a healthy life with haemophilia.

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References

  1. Jarrah Ali Al-Tubaikh, Internal Medicine: An Illustrated Radiological Guide (Springer Science & Business Media, 2010), 303.
  2. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A
  3. https://www.hemophilia.org/sites/default/files/document/files/The-Basics-of-Hemophilia.ppt, 10.
  4. WFH source provided by client states that, out of 1,125,000 male PwH, 418,000 are severely affected. 418/1,125 = appr. 37%. Here’s a link: https://www.ncbi.nlm.nih.gov/pubmed/31499529
  5. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia/Haemophilia-booklet.pdf, 3. This source, p. 3, also establishes that “bleeds” occur internally: “Internal bleeding episodes or ‘bleeds’ are the main problem for most people with haemophilia.” That “internal bleeding is the biggest concern for people with haemophilia” is supported by https://www.haemophilia.org.au/about-bleeding-disorders/faqs/general-questions
  6. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 2 and 15.
  7. https://www.hemophilia.ca/files/Chapter%2004.pdf, 4.
  8. Jarrah Ali Al-Tubaikh, Internal Medicine: An Illustrated Radiological Guide (Springer Science & Business Media, 2010), 303.
  9. These bullet points are indebted to two sources:

    (1) https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf and

    (2) Jarrah Ali Al-Tubaikh, Internal Medicine: An Illustrated Radiological Guide (Springer Science & Business Media, 2010), 303.

  10. Jarrah Ali Al-Tubaikh, Internal Medicine: An Illustrated Radiological Guide (Springer Science & Business Media, 2010), 303.
  11. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6113607/ This source states: “A patient with a FVIII activity of 20% [this is within the “mild” range] is predicted to have 1 joint hemorrhage per year.”
  12. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 17.
  13. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 1 and 3.
  14. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 4.
  15. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 6.
  16. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 6.
  17. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 6.
  18. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diag nosed/Newly-diagnosed-Chapter-3.pdf, 6.
  19. https://www.hemophilia.ca/files/Chapter%2004.pdf, 3.
  20. https://www.hemophilia.ca/files/Chapter%2004.pdf, 4, and https://www.haemophilia.org. au/HFA/media/Documents/Haemophilia%20Folder%20-%20Newly%20Diagnosed/Newly-diagnosed-Cha pter-3.pdf, 5.
  21. http://www.haemophilia.org.nz/bleeding-disorders/haemophilia/treatment/p-r-i-c-e-/
  22. https://www.hemophilia.ca/files/Chapter%2004.pdf, 4. Also, http://www1.wfh.org/publications/files/pdf-1494.pdf, 6.
  23. https://www.haemophilia.org.au/HFA/media/Documents/Haemophilia/Haemophilia-booklet.pdf, 2 and 17.