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2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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IMPLEMENTATION OF A JOINT DISEASE SURVEILLANCE PROGRAM IN HAEMOPHILIA PATIENTS – EXPERIENCE OF A PORTUGUESE PEDIATRIC TERTIARY CENTRE

Rosário Perry da Câmara1, Sara Batalha1, Paula Kjöllerström1, Isabel Seixo2, Elsa Soeiro3, Raquel Maia1

1 - Paediatric Haematology Unit;
2 - Rehabilitation and Physical Medicine Unit;
3 - Ambulatory Care Unit - Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central

Team Haemophilia Education Meeting. Frankfurt, maio 2016 (comunicação)
VII Simpósio de Coagulopatias Novo Nordisk. Lisboa, novembro 2016 (comunicação)

Introduction: Although primary prophylaxis drastically reduced the number of joint bleeds in children with severe haemophilia, a few still occur, as well as subclinical events that may result in articular damage. Early detection using validated tools is key to detect slight joint disease and standardize evaluations. In September 2014, a Multidisciplinary Joint Disease Surveillance Program, involving a Paediatric Haematology specialist, a Rehabilitation and Physical Medicine specialist and a dedicated nurse, was implemented to systematize evaluations and optimize follow-up.
Methods: Descriptive study involving severe haemophilia patients followed-up in a Portuguese paediatric tertiary centre. Demographic data, phenotype, therapeutic regimen and HJHS were collected.
Results: 22 severe haemophilia patients were included; median age 12 (1-19 y.o.); median age of diagnosis and beginning of prophylaxis were 9 and 21 months, respectively; 19 patients (86% - all over 5 years old) attended the Multidisciplinary consult; 37%have a HJHS of 0; average score was 3; 53% have joint impairment (medium joint score 2) and 37% have gait changes (medium gait score 1).
Discussion: Although the global score was low, similarly to recent studies, most children revealed some degree of joint impairment. The Multidisciplinary consult decreased hospital visits and promoted inter-specialty communication and better management (prophylaxis; physiotherapy). 

Palavras-chave: Hemophilia, Joint Disease Surveillance Program, Pediatrics