When a child is born with cystic fibrosis, symptoms usually appear in the first year of life, although occasionally they can develop later. The symptoms and related problems of cystic fibrosis may vary from person to person, and may be worse for some than for others.
The main symptoms are detailed below.
Troubling cough and wheeze - this is caused by the thick mucus in the lungs, which your body tries to shift by coughing it up.
Recurring chest and lung infections - these can be quite severe, such as bronchitis or pneumonia. They are caused by the continual build up of mucus in your lungs, which provides an ideal breeding ground for bacteria. To prevent the risk of cross infection, it is recommended that people with cystic fibrosis do not come into close contact with one another.
Malnutrition - this is due to the mucus clogging your digestive system. The mucus blocks the ducts in your pancreas that produce a food-digesting enzyme, so not enough of this enzyme reaches your intestines. As a result, your body cannot digest much of the essential nutrients in your food, and you may struggle to put and keep on weight. In children with cystic fibrosis, this can even result in delayed puberty if they are severely underweight.
Clubbed fingers and toes - 'clubbing' is where the ends of the fingers and toes become swollen and rounded. This deformity is commonly seen in congenital heart disease patients, but it is also sometimes evident in those who have certain lung conditions, such as cystic fibrosis, and appears as the condition progresses. However, the exact reason for it occurring is not fully understood.
Polyps inside the nostrils - these are small, soft growths in your nasal passages. They are caused by inflammation of your nose and sinuses due to a build up of mucus.
Large, odorous stools - this is a result of poor digestion caused by mucus build up in your digestive system. As much of the food you eat cannot be digested, stools tend to be greasy and bulky, and may contain undigested food stuffs.
Salty tasting skin - you may first notice your child's symptoms when you kiss them as their skin can have a salty taste. This is caused by high levels of salt in their sweat.
There are also several other health problems that can occur as a result of cystic fibrosis. These include:
- diabetes,
- liver damage,
- infertility, and
- osteoporosis (weak and brittle bones).
Due to improvements in treatment, as many as 75% of children affected by cystic fibrosis live to young adulthood, and at present, the average life expectancy is between 30 and 40 years. This is expected to keep rising as research into gene therapy and treatment continues.
