Cystic fibrosis
Overview
Cystic fibrosis is a rare inherited genetic condition that causes breathing and digestive problems. There is currently no cure, but there are medicines and supportive treatments that can help.
Symptoms
If you have cystic fibrosis, your body makes a thick sticky mucus that can affect your lungs and digestive system. This can cause lots of symptoms and make you more likely to get infections. Symptoms affecting your lungs include: a cough that does not go away and brings up mucuswheezing and shortness of breathfrequent sinus infections (sinusitis) and chest infections Symptoms affecting your digestive system include: constipationstomach pain, bloating and swellinglarge, greasy and sticky poos that are hard to flush away Cystic fibrosis can affect other parts of your body and cause other symptoms, such as: in children, not growing as quickly as expectedin adults, losing weight without trying toswollen fingertips and rounder nailsvery salty sweat, which can leave small crystals on your skin Cystic fibrosis is usually found shortly after birth as part of the NHS newborn screening programme. But if the condition goes undiagnosed, symptoms may only become noticeable when a child is a teenager. You may also need other treatments to help relieve symptoms and reduce the risk of complications. These include: physiotherapy – a physiotherapist can teach you ways you can improve your breathing and posture to help clear mucus from your lungsmedical devices – devices such as inhalers and specially designed masks can help with breathing and mucus clearancedietary and nutritional support – a dietitian can provide advice on a high-energy diet that is easy to digest and you may be given supplements to help your digestion Some people may eventually need a lung transplant if other treatments are not controlling their symptoms, although this is not suitable for everyone.
Causes
Cystic fibrosis is an inherited genetic condition. It's caused by an altered gene being passed on to a child by both of their parents. The altered gene causes mucus in the body to become thick and sticky. If the child only gets an altered gene from 1 parent, they will not have symptoms but they could pass the condition on to any children they have, if their partner also has the altered gene. This is called being a carrier. If 2 carriers have a baby, then there is a: 1 in 4 (25%) chance their child will have cystic fibrosis2 in 4 (50%) chance their child will not get cystic fibrosis but will be a carrier1 in 4 (25%) chance their child will not get cystic fibrosis or be a carrier
Treatment
There is currently no cure for cystic fibrosis. But there are treatments that can help ease symptoms. You'll be supported by different healthcare professionals. This may include specialist nurses and doctors, a physiotherapist and a dietitian. Medicines that may be used to treat cystic fibrosis include: medicines called CFTR modulators that help the lungs work better by reducing the effect the altered cystic fibrosis gene has on cells in the lungsmedicines to widen your airways and make breathing easier, or make it easier to cough up mucusantibiotics for infectionssteroid medicine to reduce inflammation in the airways You may also need other treatments to help relieve symptoms and reduce the risk of complications. These include: physiotherapy – a physiotherapist can teach you ways you can improve your breathing and posture to help clear mucus from your lungsmedical devices – devices such as inhalers and specially designed masks can help with breathing and mucus clearancedietary and nutritional support – a dietitian can provide advice on a high-energy diet that is easy to digest and you may be given supplements to help your digestion Some people may eventually need a lung transplant if other treatments are not controlling their symptoms, although this is not suitable for everyone.