Patient portal
A multidisciplinary team for the diagnosis, treatment and training of our patients and their families in the management of cystic fibrosis.
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.
Accreditations
Reference unit for neonatal screening programme in Catalonia, designated by the Public Health Agency of the Department of Health of the Generalitat de Catalunya, since the programme began in September 1999.
We are a Pediatric Cystic Fibrosis Unit of Reference in Spain, with the distinction of Excellence, by the Sociedad Española de Fibrosis Quística (SEFQ - Spanish society of cystic fibrosis) and the Federación Española de Fibrosis Quística (FEFQ - Spanish federation of cystic fibrosis).
Reference unit within the framework of the Network of Expert Clinical Units (XUEC) in paediatric respiratory minority diseases of the Department of Health of the Generalitat de Catalunya.
Why SJD Barcelona Children's Hospital?
We work in coordination with multiple Hospital services under the coordination of the reference pediatric pulmonologist
Multidisciplinar team
We work in coordination with multiple Hospital services under the coordination of the reference pediatric pulmonologist
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.
We have tools that favor direct contact between healthcare teams and patients and their families
Close and effective monitoring
We have tools that favor direct contact between healthcare teams and patients and their families
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.
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 experience
Attended patients in the Cystic Fibrosis Unit
Outpatient medical appointments
Outpatient nurse appointments
The rehabilitation physiotherapy sessions
Year 2022
Our professionals
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. The Cystic Fibrosis Unit participates with the Clinical Research Unit of the SJD Barcelona Children's Hospital in pharmacological and non-pharmacological clinical trials focused on this disease. These are some of the clinical trials that have been active over the last five years:
- GS-US-205-1850: Randomized, double-blind, Phase 3 Trial to evaluate the safety and efficacy of treatment regiments of aztreonam 75mg powder and solvent for nabulizer solution/aztreonam for inhalation solution (AZLI) in pediàtric subjects with cystic fibrosis (CF) and new onset respiratory tract Pseudomonas aeruginosa (PA) infection/colonization (Alpine2). Gilead, 2017-2021.
- POWERPATCH Parche cutáneo autónomo e inteligente para el diagnóstico de la fibrosis quística. Versión 1.0 de 13 de diciembre de 2019. Horizon 2020 ERC-2019-PoC: POWER-PATCH_Self-powered skin patch for cystic fibrosis diagnosis, 2019-2021.
- VX19-445-117: A phase 3b open-label study to assess the effect of elexacaftor/tezacaftor/ivacaftor on glucose tolerance in cystic fibrosis subjects with abnormal glucose metabolism. Vertex, 2021- 2022.
- VX20-445-121: A Phase 3b Open-label Study Evaluating the Safety of Elexacaftor/Tezacaftor /Ivacaftor Combination Therapy in Cystic Fibrosis Subjects. Vertex, 2022-2023.
- VX20-121-102: A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-121Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for F508del and a Minimal Function Mutation (F/MF) Vertex, 2022-2023.
- VX21-445-124: A Phase 3 Double-blind, Randomized, Placebo-controlled Study Evaluating the Efficacy and Safety of ELX/TEZ/IVA in Cystic Fibrosis Subjects 6 Years of Age and Older With a Non-F508del ELX/TEZ/IVA-responsive CFTR Mutation. Vertex, 2022-2023.
- VX21-445-125: A Phase 3 Open-label Study Evaluating the Long-term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects With Non-F508del CFTR Genotypes, 2023.
- VX20-121-104: A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-121 Combination Therapy in Subjects With Cystic Fibrosis, 2023.
In addition, the unit's team actively participates as speakers at specific congresses on the disease, in scientific publications, as well as in different national and international working groups. We highlight:
- European Cystic Fibrosis Society (ECFS): in the European patient registry and in nursing working group.
- Sociedad Española de Neumología Pediátrica (the Spanish society for pediatric respiratory medicine): with the cystic fibrosis working group.
- Sociedad Española de Gastroenterología, Nutrición y Hepatología Pediátrica (Spanish society for paediatric gastroenterology, nutrition and hepatology): with the cystic fibrosis working group.
- Sociedad Española de Fibrosis Quística (Spanish cystic fibrosis society).
Resources for families
The Catalan Cystic Fibrosis Association was formed in 1988 by a group of relatives of cystic fibrosis patients who saw the advantages of working together for the benefit of patients and their families.
The spanish cystic fibrosis federation brings together and represents 15 cystic fibrosis associations from the different autonomous communities of Spain, with more than 4,000 members.
Since its founding in 1955, the organisation has worked alongside the cystic fibrosis community to transform this genetic disease.