Study of the degree of disability in children suffering from Hemophilia- A using PedHAL score in south Gujarat area

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Original Article

Author Details : Vijay B Shah, Kirti Mehta*, Silky Agrawal

Volume : 5, Issue : 4, Year : 2019

Article Page : 138-145

https://doi.org/10.18231/j.ijmpo.2019.031

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Abstract

Introduction: The severe forms of haemophilia are characterized mainly by frequent haemarthroses leading to chronic crippling haemarthropathy when not treated very early or prophylactically. The occurrence of disability in developing and resource limited country like India is more common and severe as compared to those countries where prophylactic treatment home based factor therapy is available. PedHAL Score helps in assessment of disability in paediatric population based on activities of daily living.
Objectives: the objective of the study was to find out the degree of disability in children (4-14 years) suffering from Haemophilia in South Gujarat region using PedHAL score. The other objectives were to assess the effect of severity of disease on degree of disability in children suffering from hemophilia and to assess the effect of different factors (therapeutic and demographic) on degree of disability.
Materials and Methods: The study was conducted among the pediatric hemophilia patients between 4 to 14 years of age group. A predesigned questionnaire Pediatric hemophilia activity list (PedHAL) parent version 0.11 was selected to measure the impact of hemophilia on daily life activities as perceived by child’s parent. The PedHAL is pediatric version, to assess functional abilities in children, to measure the impact of hemophilia on self-perceived functional abilities in children. The median PedHAL total score was 100 with Inter Quartile Range of 85 to 100.
Results: Based on the median value the worst affected function are sitting, kneeling and standing (median 94) followed by other functioning of leg. (median 94.55). Mean PedHAL Score was slightly lower in illiterate cases (83.1) compare to cases educated up to primary (86.55). Mean PedHAL Score was 86.44 in severe cases and it was 74.17 in moderate cases. Mean PedHAL Score was slightly higher in cases having more than 20 bleed till date (88.87) compare to those having less bleeds (87.876). (p<0>
Conclusion: The mean PedHAL score is not associated with age of the patient; nutritional status of patient; education of patient; severity of disease; number of bleeds till date; distance from home to hospital; habit of exercise; regularity of exercise; type of family of patient; family history of haemophilia; education of patients’ mother; and family income of the patient. Early onset of symptoms is associated with lower PedHAL score and thus higher disability. Cases having 6 or more bleed per year had lower PedHAL score and thus higher disability. Higher PedHAL score and thus lower disability is associated with cases taking only factor 8 compared to cases taking other medication along with factor VIII.

Keywords: Hemophilia A, Degree of disability, Pediatric hemophilia activity list (PedHAL).

How to cite : Shah V B, Mehta K, Agrawal S , Study of the degree of disability in children suffering from Hemophilia- A using PedHAL score in south Gujarat area. IP Int J Med Paediatr Oncol 2019;5(4):138-145

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