Impact of Severe Hemophilia A in causing Joint Pains

The joint disease has been instrumental in the complications that arise in the joints and even lead to the deformities in some extreme cases. The extent of the joint disease is often severe and causes the deviation of the joint range of motion of the different joints affected by the bleeding from the haemophilia A. The joint diseases even results in some painful episodes where they can no longer function effectively or even move easily. More specifically, the joints affected tend to be the ankles, knees and elbows as well. A closer analysis of the patient affected with the bleeding into the joints reveals how they have to undergo the swellings, painful episodes and the inflammation that they encounter when the bleeding starts. In extreme cases, the bleeding might even limit the range of motion that might even lead to the loss of mobility of the patient.

The bleeding in the joints is often significant and without the proper management of the complications, the severity is more likely to increase as time goes by. Often, the bleedings occur randomly or they might even be affected by trauma that will cause the same bleeding still. Besides that, the same bleeding often affects only part at a time and move to another body part as well. The initial bleeding might even start when the child begins walking instead. However, when they fail to initiate any management, it will advance to the severe extents. At the early age, the hemarthrosis might be rarely occurring but if left unattended to they might even result to around thirty hemarthroses in a single year[1]. The location of the bleeding and the frequency as well tends to differ based on the patient’s age at the time of diagnosis. For instance, most of the young adults will have bleeding around the ankles often. The older adults will have bleedings at multiple areas that include knees and elbows often.

At times, acute hemarthrosis might cause the patient to undergo acute pains that will result from the occasional bleeding in the body. In fact, the acute hemarthrosis will be characterized by painful episodes and the local discomfort around the target joints. In such cases, the replacement therapy is the most likely intervention that will help end the joint bleeding right from the beginning[2]. In fact, the acute begins with the bleeding and within a few hours it would have resulted into a hot and swollen joint. The joint undergoes various challenges at that point where it becomes hard to bear the weight of the whole body especially if the affected joint is the knee. In the process, even the patient loses the ability to move since the joint movement is what results into mobility. The pain is often severe in that the patient might use various coagulation factors but the pain will subside at a very low rate[3]. In fact, the use of the coagulation factors will fail to reduce the inflammation as well as the loss of mobility. The blood in the joint might also decide the extent of the pain and the severity of the pain that one will experience. In some cases, the bleeding might result in Subacute hemarthroses that occurs because of the frequent episodes of hemarthrosis that will keep occurring at the same joint repeatedly[4]. The target joint will make the patient undergo extreme pain since reaching complete recovery is often a problem. The joint damage will be evident in the joint swelling and decreased mobility since the bleeding will not end easily as one expects. In the process, the complications extend in the capsular contractures, palpation and the ligament as well.

The hemophilic arthropathy arises when the hemarthrosis episodes increases in number and goes ahead to harm the joint. The destructive chronic synovitis is one of the complications that even result in the hemophilic arthropathy[5]. The occurrence of these complications is often characterized by acute pain that controlling and managing might seem a bit challenging as well. Besides that, the hemophilic arthropathy tends to vary based on that specific joint and the knee has the joint hypertrophy. The knee hypertrophy often destroys the joint and even messes with the mobility because of the way it reduces t range of motion meaning that movement will be a challenge as well. The complications keep extending to severe forms where the genu valgum, subluxation as well as the deformation limits the ability of the joint to move thereby leading to disability in the way that one cannot move easily as expected[6]. The ankle, on the other hand, results in complications in the tibio-talar while is some cases it might affect the subtalar joint or even both[7]. Such complications often weaken the joints and thereby leading to the process of weight bearing a bit challenging for the joints that are expected to carry all the weight of the body. At that point, the pain and joint stiffness makes the patient find it challenging to undertake various important activities. The elbow also experiences the chronic arthropathy that lead to joint instability and the degradation of the inner surfaces increasing the pain and inflammation that are typical in such cases as well[8]. The stiffness might be present right when the patient is waking up since resting makes it a bit painful.

In conclusion, haemophilia A tends to have bleeding in the joints that will result in joint diseases that will cause swollen body parts and tenderness that are painful. In the process, it becomes hard for the patient to move easily because the joint disease messes with the range of motion of the joints that then leads to the loss of mobility in the long-run. With such complications, the movement of the elbows, knees, and ankles becomes hard and the mobility will not be a guarantee. Later, the stiffness in the joints and the pain is often immense in the way that the patients could find it hard to move easily. Apart from the movement, the inflammation also is a challenge and makes the patient to have a hard time coordinating multiple activities instead.

 

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Bibliography

Acharya, Suchitra S. “Exploration of the pathogenesis of haemophilic joint arthropathy: understanding implications for optimal clinical management.” British journal of haematology 156, no. 1 (2012): 13-23.

Dunn, A. L. “Pathophysiology, diagnosis and prevention of arthropathy in patients with haemophilia.” Haemophilia 17, no. 4 (2011): 571-578.

Hermans, C., P. De Moerloose, K. Fischer, K. Holstein, R. Klamroth, T. Lambert, G. LAVIGNE‐LISSALDE, R. Perez, M. Richards, and G. Dolan. “Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations.” Haemophilia 17, no. 3 (2011): 383-392.

Knobe, Karin, and Erik Berntorp. “Haemophilia and joint disease: pathophysiology, evaluation and management.” Journal of Comorbidity 1, no. 1 (2011): 51-59.

Lundin, Björn, M. L. Manco‐Johnson, D. M. Ignas, R. Moineddin, V. S. Blanchette, A. L. Dunn, S. V. Gibikote et al. “An MRI scale for assessment of haemophilic arthropathy from the International Prophylaxis Study Group.” Haemophilia 18, no. 6 (2012): 962-970.

Rodriguez-Merchan, E. Carlos. “Cartilage damage in the haemophilic joints: pathophysiology, diagnosis and management.” Blood Coagulation & Fibrinolysis 23, no. 3 (2012): 179-183.

[1] Knobe, Karin, and Erik Berntorp. “Haemophilia and joint disease: pathophysiology, evaluation and management.” Journal of Comorbidity 1, no. 1 (2011): 51-59.

[2] Acharya, Suchitra S. “Exploration of the pathogenesis of haemophilic joint arthropathy: understanding implications for optimal clinical management.” British journal of haematology 156, no. 1 (2012): 13-23.

[3] Ibid, 2.

[4] Hermans, C., P. De Moerloose, K. Fischer, K. Holstein, R. Klamroth, T. Lambert, G. LAVIGNE‐LISSALDE, R. Perez, M. Richards, and G. Dolan. “Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations.” Haemophilia 17, no. 3 (2011): 383-392.

[5] Dunn, A. L. “Pathophysiology, diagnosis and prevention of arthropathy in patients with haemophilia.” Haemophilia 17, no. 4 (2011): 571-578.

[6] Lundin, Björn, M. L. Manco‐Johnson, D. M. Ignas, R. Moineddin, V. S. Blanchette, A. L. Dunn, S. V. Gibikote et al. “An MRI scale for assessment of haemophilic arthropathy from the International Prophylaxis Study Group.” Haemophilia 18, no. 6 (2012): 962-970.

[7] Rodriguez-Merchan, E. Carlos. “Cartilage damage in the haemophilic joints: pathophysiology, diagnosis and management.” Blood Coagulation & Fibrinolysis 23, no. 3 (2012): 179-183.

[8] Ibid, 7.