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Respiratory Complications of Cystic Fibrosis

Respiratory Complications of Cystic Fibrosis

What is cystic fibrosis? — Cystic fibrosis 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 cystic fibrosis 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 abnormal bowel movements.
Cystic fibrosis is caused by an abnormal gene. To get the disease, people need to get the abnormal gene from both their mother and father. If people get the abnormal gene from only 1 parent, they will not have cystic fibrosis. But they will have a chance of passing on the abnormal gene to their children.
Cystic fibrosis is a lifelong condition. As of now, doctors can't cure the disease, but they can use different treatments to help with symptoms.
People with cystic fibrosis don't live as long as people without the disease. But better treatments are helping people with cystic fibrosis live longer. To help manage your child's disease for as long as possible, it's important to work closely with his or her doctor.
What are the symptoms of cystic fibrosis? — 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. A person's symptoms usually get worse over time.
Common symptoms of cystic fibrosis include:
Not growing or gaining weight normally
A long-lasting cough – The cough usually brings up mucus (it 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, you might taste salt when you kiss your child)
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 woman has trouble getting pregnant, and a man has trouble getting a woman pregnant.
Is there a test for cystic fibrosis? — Yes. If a person has symptoms of cystic fibrosis, or if he or she has a family member with the disease, his or her doctor can do a test to check for the disease. The different tests include:
A "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
Cystic fibrosis testing can also happen at other times. Some pregnant women choose to test their unborn baby for the disease. Plus, every baby in the United States is tested for the disease at birth.
How is cystic fibrosis treated? — People with cystic fibrosis can have different treatments to help with symptoms and prevent further problems.
Treatments to help the lungs can include:
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
Medicines called "CTFR modulators" – These medicines (brand names: Trikafta, Symdeko, Kalydeco, Orkambi) can help with the symptoms caused by the abnormal cystic fibrosis gene. A doctor can do a gene test to see if any of these medicines might work for you or your child.
Airway clearance therapy – This involves breaking up and loosening the mucus in the lungs. It is sometimes called "chest physiotherapy." 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.
A 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 your child's doctor might start discussing this with you 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 the food in the intestines – For most children, this involves taking enzymes at every meal and snack.
Following a special diet
Taking vitamins
How can I help my child stay as healthy as possible? — There are things you can do to help your child stay as healthy as possible. You can:
Help your child gain enough weight. Ask the doctor or nurse which foods are best.
Not let anyone smoke in the home.
Make sure your child gets regular exercise.
Follow all your doctor's instructions about treatments and visits.
To help protect your child from getting an infection:
Make sure your child gets all the vaccines his or her doctor recommends. Vaccines can prevent certain serious or deadly infections.
Wash your hands and your child's hands often.
Follow instructions about protecting your child in the hospital or doctor's office. For example, the doctor or nurse might have your child 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 cystic fibrosis.
Teach your child to try not to touch or get too close to other people with cystic fibrosis. Both children and adults with cystic fibrosis can have infections that are especially dangerous to other people with the condition. They can give these infections to each other by touching or sitting close together. Have your child, family members, and friends wash their hands often.
How can I learn more about cystic fibrosis? — To learn more about cystic fibrosis, talk with your child's doctor or nurse. The Cystic Fibrosis Foundation also has a lot of information. Their website is Their toll-free phone number in the US is 1-800-344-4823.
All topics are updated as new evidence becomes available and our peer review process is complete.
This topic retrieved from UpToDate on: Mar 30, 2020.
Topic 16906 Version 16.0
Release: 28.2.2 - C28.105
© 2020 UpToDate, Inc. and/or its affiliates. All rights reserved.

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