Prophylactic treatment in severe hemophilia A

The prophylaxis intervention plays an important role in the process of reducing the frequency of the bleeding and also the severity of the arthropathy that leads to the patient seeing an improvement in the life. At times, the medical practitioners might even decide to use secondary prophylaxis that helps adults in the process of reducing the complications associated with the arthropathy in the body[1]. The application of the prophylaxis as treatment of the haemophilia A has been successful so far. In fact, its use has been incorporated at the early ages of children as primary prophylaxis while the adult patients will benefit from the secondary prophylaxis that differs based on the differences in ages between the two groups in question. Its main purpose is also to enhance the joint function since the healthy movement of the joints helps in the mobility of the patient. In some extreme cases, the application of the tertiary prophylaxis might be needed since it also helps in reducing the number of target joints and severe bleeding that they might be experiencing.

The adoption of the prophylaxis has proved to be an essential aspect in the process of reducing the bleeding evident in the joints of the children and adults that have severe haemophilia A. More importantly, the efficient prophylaxis needs the consideration of various factors that are of importance in the process. First, the medical practitioners will need to acknowledge the presence of the trough levels and the factor concentrate available that will help in serving the patient[2]. Another case is looking at the cause of the bleeding such as the chronic synovitis or even the severe arthropathy. The approach to consider these factors will be essential in the process of how to handle the bleeding and the appropriate steps to take in correcting the complications. In the process, it is advisable if the medical practitioners are focused on minimizing the joint arthropathy. Another aspect about the prophylaxis is that the treatment is supposed to progress even if the target joints had healed and the bleeding had ceased[3]. Such a scenario is what leads to the improved quality of the prophylaxis treatment adopted. Prophylaxis was introduced right when the children are still young and the complications arising from the haemophilia A are not that severe. In fact, research suggests that Prophylaxis that is introduced before the children reach 2 years by the time the child reaches 6 or even years, the outcome will be significant based on the reduction of the severity in the complications that the patient will be experiencing at that particular time[4].

The joint arthropathy is influenced by the frequency of the bleeds that the patient will experience in a year. The patients that experience more joint bleeds in a year are more likely to suffer from severe joint arthropathy instead. The patients that might be having about twenty to fifty bleeds in a single year and depend on the on-demand therapy are more likely to develop the joint arthropathy in the early stages of life. Shockingly, the same patients experiencing the joint bleeds but, they use the prophylaxis as a treatment approach might end up developing a joint disease like joint arthropathy even after twenty years or so[5]. Such a scenario proves that prophylaxis is an effective treatment approach that has far more effective benefits that can be used in the process of helping the patient to be immune to other complications that might end up being harmful to him or her. In fact, a research that was comparing the on-demand therapy and prophylaxis on young children affected by the haemophilia A and experiencing the joint bleeds suggested the same. More importantly, magnetic resonance imaging proved that nearly 45% of the children that were using the on-demand therapy showed the signs of the joint arthropathy while just 7% of those that were being treated by prophylaxis had any signs of the joint arthropathy[6]. The research proves that the use of prophylaxis is effective to the patients since it has endless benefits that are also more likely to benefit most of the patients affected by haemophilia A and especially the joint bleeds that have been more common and challenged the patients even the younger children.

The use of intensive prophylaxis approaches have proved to be effective in helping the patients with joint diseases to handle the complications they are experiencing. In fact, the prophylaxis programs shows that a patient they are treating will even experience only one joint bleed in a period of two years. Such a low frequency of the joint bleeds proves how effective it can be in controlling the many complications associated with the joint diseases[7]. The only challenge is the slow research in identifying the clinical manifestations of the various joint diseases that would have led to an opportunity to identify the complications early enough before things got out of hand. However, the use of lifelong prophylaxis is tends to be far more effective if it is used right when the patient is a young child as opposed to being used when people are all grown up[8]. Such scenarios often mean that the patient has reached serious extents where reducing the joint arthropathy will be much difficult. In fact, such cases might reveal the prophylaxis to be ineffective yet it is not. He only issue is using it starting from an early age before continuing with it to adulthood in a bid to use a lifelong prophylaxis that will prove to be effective in the end[9]. The use of the regimens seems a bit complicated in adulthood and the researchers have failed to identify the pattern and how it works as well.

In conclusion, the use of prophylaxis as an approach to manage the complications has proved to be effective and its use in treating the joint bleeds has shown long-term positive changes. In fact, it has shown that it reduces the chances of joint arthropathy when the younger children are introduced to the prophylaxis treatment approach. Instead, the children that will be treated by on-demand therapy are more likely to experience the joint arthropathy very early in the life stages. The scenario shows that the prophylaxis as a treatment approach is far more effective instead. The decline of the joint bleeds because of the prophylaxis treatment has proved to be far more effective as well.

 

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Bibliography

Altisent, C., M. Martorell, A. Crespo, L. Casas, C. Torrents, and R. Parra. “Early prophylaxis in children with severe haemophilia A: clinical and ultrasound imaging outcomes.” Haemophilia (2015).

Blobel, Carl P., Coline Haxaire, George D. Kalliolias, Edward DiCarlo, Jane Salmon, and Alok Srivastava. “Blood-Induced Arthropathy in Hemophilia: Mechanisms and Heterogeneity.” In Seminars in thrombosis and hemostasis, vol. 41, no. 8, pp. 832-837. 2015.

Federici, A. B. “Prophylaxis in patients with von Willebrand disease: who, when, how?.” Journal of Thrombosis and Haemostasis 13, no. 9 (2015): 1581-1584.

Ljung, Rolf, and Nadine Gretenkort Andersson. “The current status of prophylactic replacement therapy in children and adults with haemophilia.” British journal of haematology 169, no. 6 (2015): 777-786.

Oldenburg, Johannes. “Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens.” Blood 125, no. 13 (2015): 2038-2044.

Tagliaferri, Annarita, Giulio Feola, Angelo Claudio Molinari, Cristina Santoro, Gianna Franca Rivolta, Dorina Bianca Cultrera, Fabio Gagliano et al. “Benefits of prophylaxis versus on-demand treatment in adolescents and adults with severe haemophilia A: the POTTER study.” Thrombosis and haemostasis 114, no. 1 (2015): 35-45.

 

[1] Oldenburg, Johannes. “Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens.” Blood 125, no. 13 (2015): 2038-2044.

[2] Ljung, Rolf, and Nadine Gretenkort Andersson. “The current status of prophylactic replacement therapy in children and adults with haemophilia.” British journal of haematology 169, no. 6 (2015): 777-786.

[3] Altisent, C., M. Martorell, A. Crespo, L. Casas, C. Torrents, and R. Parra. “Early prophylaxis in children with severe haemophilia A: clinical and ultrasound imaging outcomes.” Haemophilia (2015).

[4] Ibid, 2.

[5] Federici, A. B. “Prophylaxis in patients with von Willebrand disease: who, when, how?.” Journal of Thrombosis and Haemostasis 13, no. 9 (2015): 1581-1584.

[6] Ibid, 1.

[7] Tagliaferri, Annarita, Giulio Feola, Angelo Claudio Molinari, Cristina Santoro, Gianna Franca Rivolta, Dorina Bianca Cultrera, Fabio Gagliano et al. “Benefits of prophylaxis versus on-demand treatment in adolescents and adults with severe haemophilia A: the POTTER study.” Thrombosis and haemostasis 114, no. 1 (2015): 35-45.

[8] Blobel, Carl P., Coline Haxaire, George D. Kalliolias, Edward DiCarlo, Jane Salmon, and Alok Srivastava. “Blood-Induced Arthropathy in Hemophilia: Mechanisms and Heterogeneity.” In Seminars in thrombosis and hemostasis, vol. 41, no. 8, pp. 832-837. 2015.

[9] Ibid, 1.