1 - Serviço de Medicina Física e de Reabilitação, Área de Pediatria, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- 2023 Congress Mediterranean Forum of Physical and Rehabilitation Medicine, Itália (Internacional)
Objectives: Cystic Fibrosis (CF) is a hereditary disease of autosomal recessive transmission caused by a mutation in the CFTR gene. It carries significant implications to various organ systems and quality of life (QoL), particularly chronic lung disease (the leading cause of morbidity & mortality), pancreatic insufficiency and male infertility. The average life expectancy is about 44 years. Early diagnosis and treatment are essential. The CF Reference Center of the University Hospitals of Central Lisbon monitors patients from diagnosis to adulthood in a multi/interdisciplinary way with rehabilitation intervention beginning soon after diagnosis. The present work aims to identify the main points on which a rehabilitation program (RP) should focus to improve the health and QoL in the pediatric age. We also evaluated how the pandemic affected this population.
Materials & methods: Immediately after diagnosis, children are sent to the rehabilitation clinic where a complete history and examination are obtained, focusing on the evolution of respiratory status, posture, muscle strength, functionality and physical activity. The patient is reviewed and receives physiotherapy treatment at least once every 6 months and regular outpatient treatment. A RP is proposed that includes exercises of respiratory and postural corrective kinesitherapy, effort reeducation, regular adapted physical activity and health education with teaching techniques to the patient/caregivers.
Results: Patients with CF who are part of a RP show improvements in respiratory and other organ systems, posture, stress tolerance, muscle strength, endurance, flexibility, functionality and QoL.
Conclusions: The regular follow-up of patients by Rehabilitation improves symptomatology, QoL and functionality while reducing health costs.