Prevalence of Musculoskeletal Complications among Hemophilia Patients as Seen at Kenyatta National Hospital
Maina, Edward, N
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Hemophilia is an X-linked heritable coagulopathy that is characterized by quantitative or qualitative deficiency of protein factors VIII and IX. Musculoskeletal manifestations are common in hemophilia patients, accounting for 70-80% of hemorrhages in severe hemophilia. These bleeds are usually of childhood onset, either as spontaneous or trauma induced hemorrhagic episodes, and may progressively lead to musculoskeletal complications with significant chronic disability, morbidity and reduced quality of life. This necessitates frequent need for restorative physical therapy and orthopedic surgical procedures. In Kenya 654 people are registered as living with Hemophilia with 81.8% having Hemophilia A and 18.2% Hemophilia B. However there is paucity of information in regard to the prevalence and pattern of musculoskeletal manifestations and complications in this population. Study objective: To determine the prevalence and pattern of musculoskeletal complications in hemophilia patients visiting Kenyatta National Hospital. Study Design: Descriptive Cross-sectional study. Study Setting: Kenyatta National Hospital hematology outpatient clinics and Wards. Materials and method: A sample of 37 hemophilic patients visiting KNH were recruited into the study. For each patient clinical history and examination was undertaken to establish presence or history of musculoskeletal complications i.e. Hemarthrosis, Arthropathy, Muscle hematoma, Pathological fracture, Pseudotumor, and Nerve palsy. Evaluation of affected joints was carried out based on the Gilbert Joint Scoring System. All the data was entered in the data collection sheet and later transferred to a secure database. Statistical analysis: Data was analysed using Statistical Package for Social Sciences version 24. Descriptive statistics methods were utilized to analyze quantitative variables. Pearson’s r correlation technique was used for empirical relationship between variables. Statistical significant p-value of 0.05 and 95% confidence level was used. Data was presented in tables and graphs. Results: All 37 patients were males. 91.9% had Hemophilia A and 8.1% had Hemophilia B. The average age was 21.5 (SD=13.9) years with the youngest being 5 years old and the oldest 61 years old. The average age at the time of diagnosis was 2.7 (SD=2.8) years with 56.7% diagnosed within the first year of life. 70.3% had severe hemophilia while 29.7% had moderate hemophilia. 97% of the patients presented with various orthopedic manifestations. 86.5% of the patients reported recurrent hemarthrosis, 75.7% had loss of Range of Motion in various joints, and 70.3% had fixed flexion joint contractures occurring in single or multiple joints. 67.3% reported history of muscle hematoma. One patient had a fracture and none were found to have peripheral nerve palsy or pseudotumor. There was a significant association between the reported hemophilia severity levels and the Global Joint Score (p value = 0.001) and between the frequency and severity of joint bleeds and Global Joint score (p value = 0.001). There was no significant relationship between the age at diagnosis of the patient and the Global Joint Score (p value = 0.575), and the household income level and the Global Joint Score (p value = 0.603). Conclusion: The study demonstrated a wide prevalence of various orthopaedic complications in hemophilia patients visiting KNH. There was a significant association of severity of orthopaedic complications and the prevailing severity of disease. Therefore there is need for prophylactic factor replacement therapy and early involvement of orthopaedic surgeons in management and follow up of hemophilia patients.
University of Nairobi
SubjectPrevalence of Musculoskeletal Complications among Hemophilia Patients as Seen at Kenyatta National Hospital
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