Surgical Interventions for Patients with Hemophilia A

In some extreme cases, the presence of the hemophilia A in the body might warrant surgical intervention. In such situations, the case might have reached an extreme scenario where the medical practitioners will not have any option but resort to surgery as the only option. Hemophilia A often results in bleeding in the various joints in the body. More specifically, the knees elbows, as well as ankles, are the ones that are mostly affected with the bleeding. In some cases, the severe bleeding might even result in the damage of the subchondral bone and the articular cartilage while chronic synovitis might also arise from the bleeding[1]. Such complications might go ahead to cause far more impairments in the way that the joints in the body work. The patient that has experienced severe bleeding in the joints because of the Hemophilia A might undergo the pain and stiffness that will result from the hemophilic arthropathy or even deformity in some cases[2]. Based on the severity of the complication, the medical practitioners will suggest a surgical procedure to help in the total joint replacements to aid in correcting the damaged joints.

This paper will clarify the appropriate steps taken in the process of undertaking knee replacement because of the severe bleeding in the knee joints. More specifically, the process will focus on the total knee replacement (TKR) that is more appropriate during such times instead. The presence of the clotting factor concentrates often makes the process a bit easier. Before the surgery, the surgical team needs to undertake physical examination to determine the extent of the injury by observing the patient’s gait or even as he or she climb the stairs so as to evaluate how

to approach the procedure[3]. In some cases, the patient might even need more than one operation so that the patient might achieve full recovery. The scenario will arise because of the multiple joint complications that the patient might have. The Radiographic evaluation will analyze the affected part to determine any deformities, strength of muscle as well as the stability of the joint when in active and passive motion[4]. All in all, the peri-operative treatment has various medical considerations such as the factor level of about 60 IU/kg and 20 IU/kg later when it is about four hours after the first dose[5]. The approach helps in raising the factor VIII activity level to about 60-80% after the duration of 72 hours when the surgery has ended[6]. The continuous infusion of the factor also helps in reaching that target. The medical consideration might differ depending on the patient, but the sole purpose is to achieve the required level of 60-80%[7].

Enough information from the pre-operative evaluation makes the surgical procedure a bit easier and successful since the parties would have covered all the issues that might cause any challenges. More importantly, it is a risky and unique procedure that needs the surgical intervention by the surgeons qualified to undertake such a process. Extreme level of arthropathy often results in complex deformities that might challenge the surgeon[8]. The same deformities might also include the angular deformity, the Patellofemoral abnormalities, as well as the lateral subluxation and the femur groove deepening[9]. The presence of such aspects even makes the procedure much more difficult to the surgeons that are expected to undertake the surgical intervention. In the process, they end up identifying any oversized dimensions that might need correction as well as the defects and bone loss that will need prosthetic augments same as the bone grafts that also help the recovery process. In some cases, the cement fixation might be needed that will also raise the need for the proper wound closure that will be tight and has the drains too. After the entire procedure has been undertaken the surgeons can then acknowledge the range of motion that the patient can move and any post-treatment activities.

The factor replacement will still continue even after the surgery, most probably about two weeks after the surgery. The factor will help in the process of maintaining the required level that will enhance the chances of recovery as well. At the same time, any swelling in the place of injury will be treated to reduce chances of complications that might arise after the surgery has taken place. The post-operative period tends to be tedious given that one will have to experience painful episodes arising from the swelling and the recovering wounds as well[10]. The patient will manage the pain through physical therapy, regional anesthesia, and oral narcotics also help in reducing the pain that the patient might be undergoing[11]. Lastly, even the physiotherapy aids the patient in reducing the complications and helping the patient in the recovery program. Bleeding tends to be the most complication that might arise after the operation has ended. Swellings and the excessive wound hematoma are the other complications that might derail the recovery progress[12]. At times, might fail to regain the ability to move after the surgery but the close monitoring by the medical practitioners will help in detecting any other complications and the access to antibiotics might relieve.

In conclusion, the surgical intervention will help to a large extent in terms of reducing the severity that might arise because of the over bleeding in the knee or any other joints in the body. In the event of detecting the complications, various factors are considered by the surgical team in order to determine the extent of the deformities that the patient might be undergoing. In fact, the decision to undertake the surgical approach tends to be the last option since the medicines would have failed at that specific stage instead. Arthropathy often leads to complex deformities that will warrant the surgical intervention. The femur groove deepening, lateral subluxation and the oversized internal knee dimensions are the other deformities that the victims of Hemophilia A undergo. The surgery will entail the replacement of the factor VIII concentrates and the fusion of bone grafts and prosthetic augments depending on the particular deformity that the patient might be experiencing. Even if the surgical procedure is risky, the patient will undergo and the effort that the surgeons will place. In the end, they would have saved the patient from any severe complications.

HRF, INC. is dedicated to provided the best specialty plasma products and meeting or exceeding the best standards in the collection, storage and distribution of said products.  Factor VIII plasma is among the many specialty plasmas products sold by HRF, INC.  Our clients come from all over the world, including the United States, Spain, England and Asian countries.

 

 

Bibliography

Ballal, Rahul D., Marc F. Botteman, Isaac Foley, Jennifer M. Stephens, Caitlyn T. Wilke, and Ashish V. Joshi. “Economic evaluation of major knee surgery with recombinant activated factor VII in hemophilia patients with high titer inhibitors and advanced knee arthropathy: exploratory results via literature-based modeling.” Current medical research and opinion 24, no. 3 (2008): 753-768.

Kasper, Carol K., A. Lois Boylen, Nadia P. Ewing, James V. Luck, and Shelby L. Dietrich. “Hematologic management of hemophilia A for surgery.” Jama 253, no. 9 (1985): 1279-1283.

Lachiewicz, PAUL F., A. E. Inglis, J. N. Insall, T. P. Sculco, M. W. Hilgartner, and J. B. Bussel. “Total knee arthroplasty in hemophilia.” J Bone Joint Surg Am 67, no. 9 (1985): 1361-1366.

Shapiro, Amy D., Gerald S. Gilchrist, W. Keith Hoots, Herbert A. Cooper, and Dennis A. Gastineau. “Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery.” THROMBOSIS AND HAEMOSTASIS-STUTTGART- 80 (1998): 773-778.

Silva, Mauricio, and James V. Luck. “Long-term results of primary total knee replacement in patients with hemophilia.” J Bone Joint Surg Am 87, no. 1 (2005): 85-91.

Teigland, Jens C., Geir E. Tjønnfjord, Stein A. Evensen, and B. Charania. “Knee arthroplasty in hemophilia: 5–12 year follow-up of 15 patients.” Acta Orthopaedica Scandinavica 64, no. 2 (1993): 153-156.

Unger, Anthony S., Craig M. Kessler, and Randall J. Lewis. “Total knee arthroplasty in human immunodeficiency virus-infected hemophiliacs.” The Journal of arthroplasty 10, no. 4 (1995): 448-452.

[1] Shapiro, Amy D., Gerald S. Gilchrist, W. Keith Hoots, Herbert A. Cooper, and Dennis A. Gastineau. “Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery.” THROMBOSIS AND HAEMOSTASIS-STUTTGART- 80 (1998): 773-778.

[2] Lachiewicz, PAUL F., A. E. Inglis, J. N. Insall, T. P. Sculco, M. W. Hilgartner, and J. B. Bussel. “Total knee arthroplasty in hemophilia.” J Bone Joint Surg Am 67, no. 9 (1985): 1361-1366.

[3] Ibid, 1.

[4] Kasper, Carol K., A. Lois Boylen, Nadia P. Ewing, James V. Luck, and Shelby L. Dietrich. “Hematologic management of hemophilia A for surgery.” Jama 253, no. 9 (1985): 1279-1283.

[5] Silva, Mauricio, and James V. Luck. “Long-term results of primary total knee replacement in patients with hemophilia.” J Bone Joint Surg Am 87, no. 1 (2005): 85-91.

[6] Ibid, 5.

[7] Ibid, 4.

[8] Unger, Anthony S., Craig M. Kessler, and Randall J. Lewis. “Total knee arthroplasty in human immunodeficiency virus-infected hemophiliacs.” The Journal of arthroplasty 10, no. 4 (1995): 448-452.

[9] Ibid, 8.

[10] Ibid, 8.

[11] Teigland, Jens C., Geir E. Tjønnfjord, Stein A. Evensen, and B. Charania. “Knee arthroplasty in hemophilia: 5–12 year follow-up of 15 patients.” Acta Orthopaedica Scandinavica 64, no. 2 (1993): 153-156.

[12] Ballal, Rahul D., Marc F. Botteman, Isaac Foley, Jennifer M. Stephens, Caitlyn T. Wilke, and Ashish V. Joshi. “Economic evaluation of major knee surgery with recombinant activated factor VII in hemophilia patients with high titer inhibitors and advanced knee arthropathy: exploratory results via literature-based modeling.” Current medical research and opinion 24, no. 3 (2008): 753-768.