Impact of Aging on Bones, Joints and Muscles in Hemophilia
Impact of Aging on Bones, Joints and Muscle in Hemophilia
The widespread availability of replacement factor concentrates has significantly contributed to the extended lifespan of persons with hemophilia.1 The availability of factor concentrate has also had an impact on the quality of life, particularly with primary (early) prophylaxis, when a program of regular administration of factor concentrate is established before significant joint bleeding occurs. Primary prophylaxis is now established as the treatment of choice for children with severe hemophilia and usually starts before or shortly after the first joint bleed. However, persons with hemophilia who are now reaching their 40s, 50s and beyond did not have easy access to early prophylaxis during their youth, or prophylaxis was not considered standard of care during that time, and now face joint disease that is the consequence of recurrent hemarthrosis.2
Progression of Joint Disease
Hemarthrosis, or joint bleeding, is the hallmark of hemophilia. Most commonly affecting the knees, elbows and ankles, hemarthrosis can occur in any joint, and recurrent bleeding in the same joint leads to development of a target joint.3 Acute hemarthrosis may be extremely painful due to the increased intra-articular pressure. Many patients will hold the affected joint in a semi-flexed position of comfort in order to minimize pain associated with excess movement.
Left untreated, hemarthrosis can progress into chronic synovitis, characterized by inflammation of the synovial membrane that lines the joints. These inflammatory processes – if left unchecked, result in nonspecific damage to the synovium and cartilage, leading to development of scar tissue, and degradation of the protective cartilage surrounding the bones. The debilitating impact to the affected joints during the chronic synovitis stage may be masked by the milder pain and greater mobility of joints than experienced during the acute hemarthrosis stage. It is therefore critically important to adequately diagnose and address the condition by approaches such as: education; regular musculoskeletal screening during clinic visits; identifying potential causes perpetuating the chronic synovitis; relative rest and activity modification and of course medical management, as underlying inflammatory processes may continue to promote further damage to the joints and bones.
Treatment of chronic synovitis and bone and joint diseases in hemophilia are targeted toward management of pain and prevention of disease progression. It is important to encourage patients to speak with their hemophilia treatment teams to develop personalized therapies for each of these aims. These therapies should be individualized based on a patient’s history of bleeding events and factor concentrate treatment, as well as responsiveness or adverse effects of therapies used to treat prior events.
A number of different treatment options are available for pain management, ranging from pharmacological therapies, such as analgesics, non-steroidal anti-inflammatory drugs (NSAIDS), COX-2 inhibitors, and opioids.4,5,6 A number of non-pharmacologic therapies are also thought to be effective in managing pain, including RICE (Rest, Ice, Compression, Elevation) and physical therapy approaches including fitness or exercise regimens.7,8
Surgical interventions may be considered when other therapies are not effective. However, for patients with bleeding disorders, invasive surgery, in the form of synovectomies or hip or knee replacements, should proceed with careful monitoring and administration of replacement factor concentrate.3
Finally, to limit progression of joint disease, late or secondary, prophylaxis may be recommended. Secondary prophylaxis is usually initiated after a careful review of potential benefits. Some studies have demonstrated a reduction in the number of joint bleeds in adults with hemophilia, but its long-term effect on reversing arthropathy is less clear.9,10
Talking to Your Hemophilia Treatment Team
Persons with hemophilia should be encouraged to discuss joint health and potential therapy with their physician and hemophilia treatment team. The treatment team will have the expertise to individualize personal treatment plans, specific to a patient’s needs.
1. Franchini, M., Tagliaferri, A. & Mannucci, P.M. The management of hemophilia in elderly patients. Clinical interventions in aging 2, 361-8 (2007).
2. Jansen, N.W.D., Roosendaal, G. & Lafeber, F.P.J.G. Understanding haemophilic arthropathy: an exploration of current open issues. British journal of haematology 143, 632-40 (2008).
3. Madhok, R., York, J. & Sturrock, R.D. Haemophilic arthritis. Annals of the Rheumatic Diseases 50, 588-591 (1991).
4. Schilling, A., Corey, R., Leonard, M. & Eghtesad, B. Acetaminophen: old drug, new warnings. Cleveland Clinic Journal of Medicine 77, 19-27 (2010).
5. World Federation of Hemophilia Guidelines for the Management of Hemophilia. World Federation of Hemophilia (2005).
6. National Hemophilia Foundation MASAC Recommendations on Use of Cox-2 Inhibitors in Persons with Bleeding Disorders. MASAC Recommendation #118 (2001). Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=295
7. Jarvinen, T. et al. Muscle injuries: optimising recovery. Best Practices & Research Clinical Rheumatology 21, 317-31 (2007).
8. Gomis, M., Querol, F., Gallach, J., Gonzalez, L. & Aznar, J. Exercise and sport in the treatment of haemophilic patients: a systematic review. 15, 43-54 (2009).
9. Tagliaferri, A., Perna, C.D. & Rivolta, G.F. Secondary prophylaxis in adolescent and adult haemophiliacs. Blood Transfusion 6, 17-20 (2008). 10. Dolan, G. The challenge of an ageing hemophilic population. Haemophilia 16, 11-16 (2010).
LIT CODE: G.PH.SM.Hem.2012-05-30.267 exp 30-May-2014