Patient portal
A multidisciplinary team for the diagnosis and treatment of cystic fibrosis and training our patients and their families on its management.
The Cystic Fibrosis Unit at the SJD Barcelona Children's Hospital is a reference unit for the diagnosis and management of pediatric patients with cystic fibrosis from birth until 18 years of age.
As well as performing neonatal screening, we also handle the multidisciplinary management and diagnosis of patients with cystic fibrosis (CF), as well as other variations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein.
Cystic fibrosis, also known as mucoviscidosis, is a severe illness that requires close, multidisciplinary monitoring of the child to improve both their quality of life and their prognosis, as they require multiple kinds of treatments, many of which are lifelong.
Accreditations
Reference unit for neonatal screening for cystic fibrosis in Catalonia, since the programme began in September 1999, awarded by the Public Health Agency of Catalonia (Department of Health, Generalitat de Catalunya).
National reference unit in Pediatric Cystic Fibrosis, with a Distinction of Excellence, awarded by the Spanish Society of Cystic Fibrosis (SEFQ) and the Spanish Federation of Cystic Fibrosis (FEFQ).
Reference unit within the framework of the Network of Units of Clinical Expertise (XUEC) in rare pediatric respiratory diseases, awarded by the Generalitat de Catalunya's Department of Health.
Why the SJD Barcelona Children's Hospital?
We work in close collaboration with several 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 several tools to promote direct contact between healthcare teams, the patient and their family.
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.
We offer comprehensive care to patients and their family, with educational training programmes to get them involved in managing the disease.
Our experience
Attended patients in the Cystic Fibrosis Unit
Outpatient medical appointments
Outpatient nurse appointments
The rehabilitation physiotherapy sessions
Our 2022 Figures
Team
Research and scientific production
There are constantly new therapeutic options to fight cystic fibrosis, thanks to the countless studies and clinical trials being conducted with drugs designed to correct the dysfunctional CFTR protein. The Cystic Fibrosis Unit collaborates with the Clinical Research Unit at the SJD Barcelona Children's Hospital on clinical trials for pharmacological and non-pharmacological treatments for the disease. These are some of the active clinical trials we have been involved in over the last five years:
- GS-US-205-1850: Randomized, Double-Blind, Phase 3B Trial to Evaluate the Safety and Efficacy of 2 Treatment Regimens of Aztreonam 75 mg Powder and Solvent for Nebulizer Solution / Aztreonam for Inhalation Solution (AZLI) in Pediatric 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 [An automated, intelligent skin patch for diagnosing cystic fibrosis]. Version 1.0, 13 December 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 Subjects With Cystic Fibrosis Vertex, 2022-2023.
- VX20-121-102: A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-121 Combination 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 team on the unit are active participants in various disease-specific conferences as speakers, they are involved in scientific publications, as well as being part of various state and international working groups. In particular:
- European Cystic Fibrosis Society (ECFS): in the registration of European patients and in the nursing working group.
- Spanish Society of Pediatric Pulmonology: with the working group in cystic fibrosis.
- Spanish Society of Pediatric Gastroenterology, Nutrition and Hepatology: with the working group in cystic fibrosis.
- Spanish Society of Cystic Fibrosis.
Resources for families
Founded in 1988 by a group of families affected by cystic fibrosis who realised the benefits of working together to better the lives of patients and their families.
A body consisting of and representing 15 different cystic fibrosis associations across the Spanish Autonomous Communities, with more than 4,000 members.
Since it was founded in 1955, this entity has worked hand in hand with the cystic fibrosis community to help transform the genetic disease.