The Cystic Fibrosis Unit at SJD Barcelona Children’s Hospital is a reference unit for the diagnosis and management of cystic fibrosis in children and young people aged 0 to 18. It has been recognised by the Department of Health of the Catalan Government since the neonatal screening programme in Catalonia began in September 1999.
Therefore, in addition to screening, we carry out the diagnosis and multidisciplinary management of patients with cystic fibrosis (CF), as well as other forms of dysfunction of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein .
Cystic fibrosis, or mucoviscidosis, is a serious disease that requires close multidisciplinary monitoring of the children to improve their quality of life and prognosis, since it requires multiple treatments, most of them for life.
Cystic fibrosis requires great teamwork, with the involvement of several of the Hospital’s departments, including Pneumology, Gastroenterology, Hepatology and Nutrition, Rehabilitation and Physical Medicine, Otorhinolaryngology, Endocrinology, Nephrology, Pharmacy, Psychiatry and Psychology, Social Work, Laboratory and Diagnostic Imaging. The coordination of teams and follow-up is always carried out by a pediatric respiratory medicine specialist with the support of the specialist nurse in the unit.
Close and effective monitoring
The management of the disease is complex, so very close and centralised monitoring is carried out at the hospital Cystic Fibrosis Unit, with effective communication with the primary care centre where the patient will need to go for more ordinary procedures. Patients are also allocated a nurse as a contact person to answer any questions by phone or email and access to the Hospital’s Patient Portal.
Patient and family training
The Unit runs a health education programme to teach the patient and their family about the disease and its optimal management at home. When the patient reaches adolescence, special emphasis is placed on self-care so that they can gradually become responsible for their own treatment and speak for themselves. This process culminates in transfer to the Adult Unit from the age of 18.
Our outcomes of 2018
Research and scientific production
New therapeutic options for dealing with cystic fibrosis are constantly opening up thanks to clinical studies and trials with drugs aimed at correcting CFTR function. Since 2013 the SJD Barcelona Children’s Hospital Cystic Fibrosis Unit:
- Has participated in 4 clinical trials.
- Has published 13 articles in scientific journals and book chapters on CF.
- Has presented 27 papers at national and international courses, seminars and conferences.
In addition, the Unit actively participates in a number of national and international working groups. These include:
- European Cystic Fibrosis Society (ECFS): in the European patient registry and in nurse groups.
- Sociedad Española de Neumología Pediátrica (the Spanish society for pediatric respiratory medicine): we have collaborated with the cystic fibrosis working group in drawing up the Spanish guidelines for management of patients with cystic fibrosis diagnosed by neonatal screening.
- Sociedad Española de Gastroenterología, Nutrición y Hepatología Pediátrica (Spanish society for pediatric gastroenterology, nutrition and hepatology): with the cystic fibrosis working group.