Quality of Life and Clinical Assessment of Joint Health in Children with Hemophilic Arthropathy

Abstract

Abstract Hemophilic arthropathy results in pain, deformity, and disability with severe impairments, activity limitation, and thus productivity loss. Also patterns of treatment interfere with patients' life, so quality of life should be assessed when evaluating treatment. The aim of this work was to assess the quality of life of children with hemophilic arthropathy and its relation to clinical joint health if any. The study was carried out on fifty boys aged 4 to 16 years, with hemophilic arthropathy. All patients were subjected to thorough local clinical assessment of the most affected and/or target joint using Haemophilia Joint Health Score (HJHS 2.1), assessment of quality of life using Haemo-Qol questionnaire kids' and parents' versions for three age groups: Group I: 4-7 years (21 items covering 8 dimensions), Group II: 8-12 years (64 items covering 10 dimensions), Group III: 13-16 years (77 items covering 12 dimensions) and complete blood count measurement. Among the fifty hemophilic patients, 36 (72%) patients were hemophilia A and 14 (28 %) patients were hemophilia B. All patients were receiving on demand replacement therapy using plasma derived Factor concentrate or fresh frozen plasma (FFP) according to availability. The age at first joint bleeding ranged from 1-8 years with a mean of 2.40±1.78. While the number of joints affected in the studied patients ranged from 2-13 joints with a mean of 7±3.25, and the most common target joint being the knee (72%), followed by the ankle (10%) and then the elbow (4%). Twenty three patients (46%) had severe, and 27 (54 %) had moderate hemophilia. The results of the present study showed that there was significant difference between moderate and severe hemophilic patients as regards age of onset of joint bleeding (z = -2.747, p = 0.006) and number of joints affected (t = -3.855, p < 0.001). There was statistical significant difference between the three studied age groups as regards HJHS (F= 9.843, P< 0.001), and Global gait score (F= 8.939, P<0.001). However there was no significant difference between the studied age groups as regards Kid and Parent Haemo-QOL scores (P=0.234 and 0.273 respectively). Forty four (88%) patients had different degrees of anemia. There was significant correlation between kid Haemo-QOL with degree of anemia (r = 0.291, p = 0.040), mainly with view and school dimensions. There were significant correlations between kid and parent Haemo-QOL and HJHS with each of the following: factor activity level, duration of the disease, duration of joint disease, number of bleeding attacks last year and number of joints affected. Five (10%) of the studied patients did synovectomy. The Haemo-QOL score was statistically significantly lower after the intervention (p=0.043) mainly with physical health, feeling, view, family, school and sports, treatment and dealing dimensions. In the current study there were significant positive correlations between kid and parent Haemo-QOL with global gait (r = 0.671, p <0.001 for kid and r = 0.656 p <0.001 for parents) and HJHS scores (r = 0.620, p <0.001 for kid and r = 0.630, p <0.001 for parents). Several factors affect the quality of life in patients with hemophilic arthropathy including synovectomy, anemia ,mode of treatment and joint health. Table Table. Figure Figure. Disclosures Hassab: Eli Lilly and Company: Research Funding.