Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease that develops when a gene that controls the movement of salt in and out of cells doesn’t work properly, resulting in a build-up of mucus and digestive secretions in the lungs, making it difficult for your child to breathe. His digestion is also affected because digestive fluids aren’t able to reach his intestines.

One of the main signs of CF in babies can be a failure to pass meconium, the sticky black first bowel movement they normally have within their first 24 hours. Other signs include problems breathing, coughing and wheezing, and failure to thrive. In older children you may notice recurrent bouts of wheezing and coughing similar too the common cold, but if this is paired with poor growth your child may be suffering from CF. Other symptoms in children are a big appetite but no weight gain, salty tasting skin and large, greasy stools. If you notice any of these symptoms in your child consult your pediatrician, who can arrange diagnostic tests.

Treatment

If you have a family history of CF you can have genetic testing in pregnancy to determine whether you’re likely to pass it onto your baby. Newborn screening for CF can be carried out via a heel prick test but not all states check for it, so if you have a family history of the condition request that your baby’s blood sample be analyzed for CF. Your pediatrician may also recommend what’s called a sweat test, where your baby’s sweat is tested for abnormally high levels of salt. It’s important to identify whether your baby has CF early so that treatment can begin as soon as possible.

A child with CF will need an ongoing program of care and treatment. Babies may need an urgent operation to unblock their bowels and it’s vital you also get your baby immunised against common childhood diseases. Other treatment includes medication and physiotherapy to help to clear the lungs of mucus, enzyme tablets to aid digestion, and exercise to boost general health.

There’s no cure for CF, but life expectancy for children with the condition has increased hugely in the last 20 years, and a baby born with it can expect to live beyond his mid-thirties.

CF and fertility

The fertility of both men and women can be affected if they have CF. In men, the sticky mucus can block the tubes along which sperm travels; in women it can cause low weight, which may delay puberty and make menstrual cycles irregular therefore playing havoc with ovulation. Make an appointment with your physician or a genetic counselor if you have CF and are finding it hard to become pregnant, or are worried about passing it on to your baby.

The information in this feature is intended for educational purposes only. If you have any concerns about your health, the health of your child or the health of someone you know, please consult with a doctor or other healthcare professional.