Facts about cystic fibrosis
On this page, we have collected key information about cystic fibrosis in Norway. For medical and healthcare questions beyond what is written on this page, we recommend contacting the Norwegian Center for Cystic Fibrosis.
Causes, Diagnosis, Inheritance, and Prevalence of Cystic Fibrosis
Cause
Cystic fibrosis is caused by a defect in a protein called the CFTR protein, resulting from a genetic mutation. There are over 2,000 known variants of this mutation. The defect in the CFTR protein leads to the production of excessively thick mucus. Since March 2012, all newborns in Norway have been screened for cystic fibrosis through the newborn screening program.
Diagnosis
In Norway, all newborns are screened for cystic fibrosis via a program for newborn screening that started in March 2012. This makes it possible to diagnose the disease early and start treatment before the symptoms become severe. The diagnosis is usually confirmed by a sweat test that measures the amount of chloride in the sweat, as well as genetic tests to identify CFTR mutations.
Arv
Cystisk fibrose arves autosomalt recessivt. Dette betyr at begge foreldrene må være bærere av en mutasjon i CFTR-genet for at barnet skal kunne få sykdommen. Hvert barn har da 25% sjanse for å arve to muterte gener (ett fra hver forelder) og utvikle cystisk fibrose, 50% sjanse for å bli en frisk bærer (arve ett mutert gen), og 25% sjanse for å ikke arve noen muterte gener.
About 150,000 Norwegians are carriers of the gene responsible for cystic fibrosis, although most are unaware until they have a child with the condition.
Forekomst
On average, 8-10 children are born with cystic fibrosis each year in Norway.
Treatment
Treatment for cystic fibrosis focuses on relieving symptoms and reducing complications. This includes physical therapy to loosen and remove tough mucus from the lungs, medications to reduce mucus production and treat infections, and nutritional therapy to ensure adequate nutrient absorption.
In June 2022, the causative medication Kaftrio was approved for public funding. This applies to those with at least one copy of the most common CFTR mutation (F508del), which accounts for 85% of Norwegians with CF.
For more information about diagnosis, symptoms, treatment, and newborn screening, visit the Norwegian Center for Cystic Fibrosis.
Transplantation as a life-saving treatment in cystic fibrosis
In some, the damage to the lungs will be so great that the disease becomes life-threatening. A lung transplant is then necessary.
- People with cystic fibrosis is represent by the youngest patients (down to the age of 15) who have had a lung transplant in Norway.
- Since 1990, more than 50 people with cystic fibrosis have undergone lung transplantation. A few also need to have other organs transplanted.
- As one of the patient organizations behind the Organ Donation Foundation, we in the Norwegian Cystic Fibrosis Association actively support the work for more organ donors and more transplants.
Competence environments
The Norwegian Center for Cystic Fibrosis in Oslo is the national competence center for cystic fibrosis in Norway. Both patients, relatives, health professionals and other bodies can contact us here.
- Transplantations take place at Rikshospitalet in Oslo.
Contact
If you have any questions, please contact us at redaksjonen@nfcf.no or send us a message via our Facebook page CFNorge. We appreciate being sent online articles and articles where cystic fibrosis is discussed.
The factual framework can be credited/sourced as follows: Norwegian Association for Cystic Fibrosis, www.cfnorge.no.
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