Severe haemophilia involves spontaneous bleeding within the musculoskeletal system and mucosal or cerebral hemorrhages at an early age. Hemophilic arthropathy is a long-term, debilitating consequence of repeated haemarthrosis in patients suffering from haemophilia.
The first haemarthrosis, or bleeding into the joint space, can develop between the ages of 1 and 5 years; this typically occurs in the absence of primary prophylaxis which is used to prevent either joint destruction or the progression of joint destruction.
As stated by Lobet et al., patients with severe haemophilia develop deep hemorrhages that mainly affect muscles and large synovial joints. While the knees and elbows are most commonly affected in patients that are over the age of 30 years, the ankles are frequently affected in adolescents and young adults suffering from haemophilia.
The authors classify the stages of haemarthrosis in severe haemophiliacs as – acute haemarthrosis that can be resolved with adequate replacement therapy and rehabilitation, subacute haemarthrosis that occurs after repeated haemarthrosis episodes in the same joint (with clinical signs of joint damage) and lastly, chronic arthropathy which results in a significant loss of muscle function and muscle-tendon contractures.
According to Rodriguez-Merchan, from the Department of Orthopaedic Surgery at La Paz University Hospital, pain around the hip joint, femoral neuropathy and hip flexion contracture could indicate the possibility of an iliopsoas haematoma in a patient with haemophilia.
It is necessary to implement early and effective factor replacement therapy to prevent haemophilia-related complications of the lower limbs. If adequate haemostasis is not achieved, the patient can become susceptible to abnormal bleeding of the muscles which can further raise the risk of nerve injury in patients with inhibitors.
Management strategies for nerve injuries in haemophilia should include prevention of muscle haematomas and haemophilic arthropathy. Initial treatment protocols for compressive neuropathies involve the use of assistive devices such as splints or orthotics for providing support to in the affected muscles.
Susan Geraghty, from the National Haemophilia Foundation, recommends an early orthotic intervention as part of comprehensive rehabilitative programmes for proper positioning of the foot in order to avoid undue stress the lower extremity. It has been reported that this can also help in decreasing pain associated with ankle problems in patients with haemophilia.
By providing limb stability and maintaining balance, MASS4D® foot orthotics can be used as a measure to support foot and ankle movement in patients with haemophilia; these orthotics are designed to reduce the load on the soft tissue structures supporting the joints.
This helps in providing a stable base of support for resistance of body sway while reducing any gait abnormalities that result in an uneven distribution of pressure across the plantar surface of the foot.
MASS4D® also helps in promoting joint stabilisation to improve muscular function and to address postural disparities that could later induce limitations in mobility and as a result, significantly affect an individual’s quality of life.
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