Prioritizing Musculoskeletal Care for the Patients with Hemophilia A

The care and the management of the complication arising from the hemophilia A have been effective in the current years. However, the bleeding in the joints have continued to be a concern for the patients since it might be occurring while the patient is still unaware of the bleeding. In fact, medical insights show that the bleeding is common in the musculoskeletal system that is an essential part of the human body. More likely, the occasional bleeding might even lead to the joint damage or even the haemophilic arthropathy that have adverse to the body of the patient[1]. As a result of the challenges that might stem from the excess bleeding in the joints, it is advisable that the medical practitioners engage in early identification and management of the bleeding in the musculoskeletal system. In extreme cases, the bleeding in the joints will even lead to dysfunction and deformities that have adverse complications to the patient. Hence, there is the need to adopt the management strategies that will reduce the bleeding in the joints and help the patients with or without inhibitors to enhance their musculoskeletal systems.

The bleeding might occur only once into a specific part that is also known as a ‘target joint’ or it might occur frequently resulting in some other orthopaedic complications as well. The bleeding might occur because of trauma or it might occur randomly on its own. The severe bleeding in the joints is dangerous since it also results in an increase in the clotting factor level that puts the body functioning at risk. More specifically, the bleeding takes place in the joints especially the knees, elbows and the ankles while the muscles affected include the calf, forearm muscles and even the iliopsoas[2]. Evidently, such areas are critical for the body functioning of a person. For instance, when the knees, elbows, and ankles are affected, motion and any other kind of movement will be a problem. The manifestation of the joint bleeding is evident when one experiences tenderness, swelling, severe pain or even the reduced mobility[3]. Besides that, the complications arise because the synovial tissue retains the blood released into the joints. In this case, there is the need to control the bleeding so that the patient will retain the ability to move and have the muscle strength too.

When the management of the musculoskeletal bleeding is initiated at an early stage it ensures that the patient will not reach any severe bleeding scenarios. In fact, medical records prove that prophylaxis that is introduced at the early periods of the bleeding episodes results in the patients retaining the joint function that would otherwise be undermined by the presence of severe bleeding episodes[4]. In fact, the same early prophylaxis is far more effective than the on-demand therapy in terms of reducing the bleeding in the joints of the patients. In the process, they help in conserving the musculoskeletal and the joint functions that might be experiencing severe haemophilia. Apart from the prophylaxis, the factor replacement therapy is also effective in managing the bleeding that is harmful to the joint parts of the patients. Apart from the early prophylaxis that starts mostly in children that might have haemophilia, the secondary prophylaxis is effective in later stages of life[5]. Often, it does not prevent the bleeding but tries to reduce the extent of the joint damage that is present in the joints. In the process of delaying the damage, secondary prophylaxis also helps in reducing the bleeding also not only the existing joint damage. It can be effective in managing the complications especially for the patients that do not have the inhibitors that might reduce the likelihood of progress in terms of the recovery and the management of the complications. It might also help in reducing the joint inflammation that might arise because of the lack of proper medication and therapy that might reduce the severity of the bleeding[6].

The on-demand therapy is another option often picked when the scenario has reached acute bleeding instead. The therapy is administered only if the clinical observation identifies any extreme extent of the internal joint bleeding in questions. More importantly, it involves the use of the factor VIII concentrates in sequential doses in order to raise the factor to reach the required levels[7]. The required level, on the other hand, depends on that particular case that will involve the severity of the part affected and the severity as well. The patients that have inhibitors require some extra medication that entails the use of prophylaxis that also needs the use of bypassing agents[8]. The combination of the two therapies is essential to help in reducing the severity that might also result in the arthropathy. However, research on the patients without inhibitors has enough information that has clarified any of the issues that are considered.

In conclusion, the adoption of management therapies is essential in reducing any musculoskeletal complications and enhancing the function of the joints, movement as well as the strength of the muscles in the body. The issue of joint disease that might result from the joint bleeding in patients with hemophilia A continues to be a persistent issue that is even a challenge to the medical doctors. To make it worse, even the patients with inhibitors and those without inhibitors all are exposed to the dangers of the joint diseases. With the risks of crippling deformities arising from the joint bleeding, there is the need to undertake early detection and management of the strategies as well[9]. The management of the bleeding all depends on the severity and stage of the joint bleeding. In the early stages, it is advisable if the patient adopts the early prophylaxis since it prevents the joint complications from spreading. Later, the secondary prophylaxis helps in managing the complications arising from the advanced stages of chronic joint disease. In such, scenarios it will not prevent but rather reduce the severity of the complications. In other cases, the same secondary prophylaxis will fail to apply as well based on the acute bleeding and the prompt measures needed. In such scenarios, the on-demand therapy is the one that plays an important role instead.

 

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Bibliography

Carcao, Manuel, Pamela Hilliard, Miguel A. Escobar, Luigi Solimeno, Johnny Mahlangu, and Elena Santagostino. “Optimising musculoskeletal care for patients with haemophilia.” European journal of haematology 95, no. S81 (2015): 11-21.

Carulli, C., R. Civinini, C. Martini, S. Linari, M. Morfini, M. Tani, and M. Innocenti. “Viscosupplementation in haemophilic arthropathy: a long‐term follow‐up study.” Haemophilia 18, no. 3 (2012): e210-e214.

Fernandez-Palazzi, Federico, Salvador Rivas Hernandez, Norma B. De Bosch, and Arlette R. De Saez. “Hematomas within the iliopsoas muscles in hemophilic patients: the Latin American experience.” Clinical orthopaedics and related research 328 (1996): 19-24.

Hermann, George, Marvin S. Gilbert, and I. Fikry Abdelwahab. “Hemophilia: evaluation of musculoskeletal involvement with CT, sonography, and MR imaging.” AJR. American journal of roentgenology 158, no. 1 (1992): 119-123.

Hoyer, Leon W. “Hemophilia A.” New England Journal of Medicine 330, no. 1 (1994): 38-47.

Rodriguez-Merchan, E. Carlos. “Musculoskeletal complications of hemophilia.” HSS journal 6, no. 1 (2010): 37-42.

 

[1] Carcao, Manuel, Pamela Hilliard, Miguel A. Escobar, Luigi Solimeno, Johnny Mahlangu, and Elena Santagostino. “Optimising musculoskeletal care for patients with haemophilia.” European journal of haematology 95, no. S81 (2015): 11-21.

[2] Carulli, C., R. Civinini, C. Martini, S. Linari, M. Morfini, M. Tani, and M. Innocenti. “Viscosupplementation in haemophilic arthropathy: a long‐term follow‐up study.” Haemophilia 18, no. 3 (2012): e210-e214.

[3] Ibid, 1.

[4] Fernandez-Palazzi, Federico, Salvador Rivas Hernandez, Norma B. De Bosch, and Arlette R. De Saez. “Hematomas within the iliopsoas muscles in hemophilic patients: the Latin American experience.” Clinical orthopaedics and related research 328 (1996): 19-24.

[5] Ibid, 2.

[6] Hermann, George, Marvin S. Gilbert, and I. Fikry Abdelwahab. “Hemophilia: evaluation of musculoskeletal involvement with CT, sonography, and MR imaging.” AJR. American journal of roentgenology 158, no. 1 (1992): 119-123.

[7] Rodriguez-Merchan, E. Carlos. “Musculoskeletal complications of hemophilia.” HSS journal 6, no. 1 (2010): 37-42.

[8] Hoyer, Leon W. “Hemophilia A.” New England Journal of Medicine 330, no. 1 (1994): 38-47.

[9] Ibid, 6.