Challenges in care of adult CF patients – the specialist cystic fibrosis team

Goździk J, Majka-Sumner L, Cofta S, Nowicka A, Piorunek T, Batura-Gabryel H

Cystic fibrosis (CF) is the most common life-limiting, autosomal, recessive genetic disorder. The gene which is responsible for the symptoms of this disease is located on the long arm of chromosome 7 and encodes the protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), an apical chloride channel in epithelial cells. CF is a “multi-system” disease. It affects many parts of the body and it has a varied clinical expression. All patients with CF should have access to specialist services and the treatment must be comprehensive and multidisciplinary. The multidisciplinary team approach is important when trying to optimize care given to the patient and their family. The cystic fibrosis team may include personnel from the following specialist areas: medical, nursing, physiotherapy, dietetics, psychological, social / supportive. Close coordination is vital. Ideally, ‘all members of the team’ should have had CF care-related training. The specialist team approach ensures that such specialized multidisciplinary expertise is applied in all aspects of care, better knowledge of individual families, continuity, knowledge of treatment advances and the ability to apply these in daily management. Doubtlessly CF team ensures families a specific point of contact and they know who to talk to.
In 1997 The Department of Pulmonology Diseases in Pozna started running a programme for CF adults at our University – the second CF adult centre in Poland. Members of our CF team have experience in the management of adults who have cystic fibrosis. Its members include physicians, nurses, a physiotherpist, a dietitian, a social worker and a psychologist. We must to build the team approach in CF care and use effectively talents of multiprofessional team members as fully as possible to deliver better services to patients.