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Patient education: Cystic fibrosis (The Basics)

Patient education: Cystic fibrosis (The Basics)

What is cystic fibrosis? — Cystic fibrosis ("CF") is a disease that some children are born with. It causes thick mucus and other fluids to build up and clog different parts of the body, including the lungs, pancreas, liver, and intestine (figure 1):

The thick mucus in the lungs causes people with CF to get frequent lung infections. Over time, these infections damage the lungs.

The thick fluids in the pancreas and liver keep the intestine from absorbing certain nutrients from food. This affects a child's growth and causes problems with bowel movements.

CF is a lifelong condition. Doctors can't cure the disease, but they can use different treatments to help with symptoms.

People with CF often have health problems and don't live as long as people without the disease. But better treatments are helping people with CF be much healthier and live longer. To help manage CF, work closely with your (or your child's) doctors or nurses.

What causes CF? — CF is caused by an abnormal gene. To get the disease, a person needs to get the gene from both of their parents.

If a person gets the abnormal gene from only 1 parent, they will not have CF. But they will have a chance of passing on the abnormal gene to their children. Having 1 copy of the gene is called being a "carrier."

What are the symptoms of CF? — People can have different symptoms at different times. Most people start having symptoms as a baby or young child. A few people start having symptoms as teens or adults. The symptoms usually get worse over time.

Common symptoms of CF include:

Not growing or gaining weight normally

Long-lasting cough – The cough usually brings up mucus and sounds "wet." Some people cough up blood.

Trouble breathing or breathing that sounds like whistling (wheezing)

Frequent infections of the lungs or sinuses – The sinuses are hollow areas in the bones of the face.

Skin that tastes salty – For example, if your child has CF, you might taste salt when you kiss them.

Belly pain, diarrhea, or constipation (trouble having bowel movements)

Bowel movements that are oily, bad-smelling, and float in the toilet bowl

Infertility – Infertility is when a person has trouble getting pregnant or getting a partner pregnant.

Is there a test for CF? — Yes. In the US and many other countries, every baby is tested, or "screened," for CF at birth. Because of this, most people with CF are diagnosed when they are babies and do not yet have symptoms.

Other times, a child or adult might be tested if they start having symptoms of CF. A pregnant person might also choose to test their unborn baby for CF.

The different tests for CF include:

Newborn screening – This test is done soon after birth. It involves taking a few drops of blood to check for signs of CF and several other health problems. If the result is abnormal, the baby might need other tests to find out for sure if they have CF.

"Sweat test" – During this test, the doctor makes a person sweat and then measures the amount of salt in the sweat.

Blood tests to check for the abnormal gene

How is CF treated? — People with CF can have different treatments to help with symptoms and prevent further problems.

Treatments to help the lungs can include:

Medicines called "CTFR modulators" – These medicines (brand names: Trikafta, Symdeko, Kalydeco, Orkambi) can help with the symptoms caused by the abnormal CF gene. A doctor can do a gene test to see if 1 of these medicines might work for you or your child.

Antibiotic medicines to help prevent or treat infections

Medicines to help open up the airways in the lungs

Medicines to help thin out the mucus in the lungs – A person breathes these medicines in from a machine called a "nebulizer."

Chest physiotherapy – This involves breaking up and loosening the mucus in the lungs. There is more than 1 way to do this, and your child's doctor or nurse can show you the different ways. One way involves thumping your child's chest and back to loosen the mucus. Other ways involve using special devices. These include a vibrating vest or devices children breathe into.

Lung transplant – This is surgery in which a doctor replaces a person's diseased lungs with healthy lungs. A lung transplant is done only if people have severe disease and meet certain conditions. But the doctor or nurse might start discussing this with a child and their family well before it is time to consider a transplant. That way, you will know what to expect and how to prepare for it if the time comes.

Treatments to help the digestive system can include:

Taking medicines (called "enzymes") to help break down food in the intestines – For most people, this involves taking enzymes at every meal and snack.

Following a special diet or taking a supplement that has extra calories

Taking vitamins

How can I help my child stay as healthy as possible? — There are things that can help a person with CF stay as healthy as possible. They include:

Working to gain enough weight. Ask the doctor or nurse which foods are best.

Not letting anyone smoke in the home

Getting regular exercise

Following all of the doctor's instructions about treatments and visits

These things can help lower the risk of infection:

Making sure the person gets all of the vaccines that their doctor recommends. Vaccines can prevent certain serious or deadly infections.

Washing hands often

Following instructions about protection in the hospital or doctor's office. For example, the doctor or nurse might have you wear a face mask while at the hospital to help keep germs out. Doctors and nurses might also wear special gowns or gloves when treating people with CF.

Trying not to touch or get too close to other people with CF. People with CF can have infections that are especially dangerous to other people with CF. They can give these infections to each other by touching or sitting close together. They, their family members, and friends should wash their hands often.

How can I learn more about CF? — To learn more about CF, talk with your child's doctor or nurse. The Cystic Fibrosis Foundation also has a lot of information. Their website is www.cff.org. Their toll-free phone number in the US is 1-800-344-4823.

More on this topic

Patient education: Coughing up blood (The Basics)
Patient education: Pneumonia in adults (The Basics)
Patient education: Poor weight gain in babies and children (The Basics)
Patient education: Vaccines for babies and children age 0 to 6 years (The Basics)
Patient education: Vaccines for children age 7 to 18 years (The Basics)
Patient education: Coping with high drug prices (The Basics)
Patient education: Brand versus generic medicines (The Basics)
Patient education: Bronchiectasis in children (The Basics)
Patient education: Lung transplant (The Basics)

Patient education: Poor weight gain in infants and children (Beyond the Basics)
Patient education: Asthma inhaler techniques in children (Beyond the Basics)

This topic retrieved from UpToDate on: Oct 01, 2023.
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