Authors (including presenting author) :
Ng CS(1), Chan YH(1), Yip HT(1), Lam PL(1), Wong SC(2)
Affiliation :
(1) Physiotherapy Department, Queen Mary Hospital
(2) Department of Paediatrics & Adolescent Medicine, Queen Mary Hospital
Introduction :
Cystic fibrosis (CF) is a genetic disorder causing dysregulation in airway mucociliary clearance and is one of the most common genetic diseases among Caucasian, with an incidence of around 1 in 2500–3500 newborns. Contrastingly, it is considerably less prevalent in Hong Kong, with an estimated incidence of approximately 1 in 300,000 live births. Medications and modulator therapy are available for controlling CF, while physiotherapy remains essential in managing complications and maintaining quality of life of the CF population.
Objectives :
Herein, we describe the case of a 1-month-old female neonate diagnosed with CF in Queen Mary Hospital (QMH).
Methodology :
Born to a British non-consanguineous couple, the child was first presented with meconium ileus – an early manifestation of CF occurring in approximately 20% of CF patients, and was admitted to the neonatal intensive care unit (NICU) since birth. Later genetic tests confirmed the diagnosis of CF. Condition was complicated by episodes of chest infections.
To address the complex clinical picture and the patient’s family’s preference for the United Kingdom’s National Health Service standard care for CF, an international multidisciplinary team involving local professionals at QMH and CF specialists at the Addenbrooke's Hospital was formed. Regular online joint consultations were arranged with roles of physiotherapy clearly delineated. Physiotherapy was delivered according to the “Standards for the clinical care of children and adults with cystic fibrosis in the UK” adopted by the Addenbrooke’s team, which included airway clearance techniques, positive expiratory pressure therapy, pulmonary exercise and inhalation therapy. Additionally, carer education was performed to ensure the family’s competence in undertaking long-term caring responsibilities.
Result & Outcome :
The structured physiotherapy program facilitated optimisation of the patient’s lung condition and empowered caregivers to actively participate in physiotherapy management even in the NICU. Such collaborative care model promoted parent-infant interaction and enabled smooth transition from hospital to home care, in turn shortened the NICU length of stay.
The rarity of CF among local population has traditionally posted challenges for physiotherapists in Hong Kong to deliver CF-specific care. The presented case illustrated the importance of physiotherapy in neonatal CF care and demonstrated how cultural and logistical barriers can be overcome by bridging local expertise with established international models. This report aims to serve as a foundation for future development of rigorous physiotherapy guideline in CF care applicable to the context of Hong Kong, with the goal of advancing our quality of CF care to an internationally-recognised standard.
