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Cystic fibrosis (CF) is a common genetic disease that causes mucus in the body to become thick and sticky. The mucus builds up and causes problems in many of the body's organs, especially the lungs and the pancreas. People who have CF can have serious breathing problems and lung disease.
CF is caused by a change, or mutation, in a gene. The changed gene is passed down in families. To pass on this disease, both parents must be carriers of the changed gene.
Some common symptoms in a baby who has CF include a blocked small intestine, breathing problems, not growing or gaining weight even with a good appetite, salty skin, and diarrhea. Other symptoms in children may include clubbing (rounding and flattening) of the fingers and growths in the nose or sinuses.
Babies are tested for CF right after birth. The doctor may also notice the signs of the disease during a routine exam. If your child has a positive screening test or symptoms of CF, the doctor will order a sweat test and a genetic test.
After a child is diagnosed with CF, a team of health professionals will build a treatment plan. The plan is based on the child's specific health problems. Following a treatment plan will help your child live a longer, healthier life.
A personal treatment plan will quickly become part of your child's daily routine. It will include airway clearance techniques (ACT), medicines, a fitness plan, and nutritional therapies.
Regular visits with the team involved in your child's care are important. Lab tests can help the doctor know how serious the disease is and how it is affecting your child's body.
Some treatments are still being tested and developed. Talk to your doctor about clinical trials and new treatments.
The best treatment available is often found at cystic fibrosis care centers. These centers address medical, nutritional, and emotional needs. Contact the Cystic Fibrosis Foundation at www.cff.org to find one.
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CF is caused by a changed (mutated) gene that a child inherits from both parents. The changed gene causes problems with the way salt and water move in and out of the cells that make mucus. This causes the mucus to be thick and sticky. This glue-like mucus builds up and causes problems in many of the body's organs, especially the lungs and the pancreas.
Many people don't know they have the changed gene.
CF is a genetic condition. It is an autosomal recessive disease. This means that to get the disease, you must inherit the changed (mutated) gene from both parents. Keep in mind:
Symptoms of CF are usually caused by the production of thick, sticky mucus throughout the body. The symptoms aren't the same for everyone.
Common symptoms in a baby who has CF include:
Other symptoms may also develop in childhood, such as:
CF causes mucus in the body to become thick and sticky. The mucus buildup can cause problems in many of the body's organs and systems. These include:
People with CF have thick and sticky mucus that traps bacteria. This causes lots of lung infections and often causes permanent lung damage. Bronchiectasis, which is caused by long-lasting airway inflammation, is common.
Mucus can interfere with how the pancreas works. This can make it hard for the child to absorb nutrients from food.
You may notice that your child has unusually salty skin. CF can cause a person to become easily dehydrated or to have very low salt levels.
Almost all men who have cystic fibrosis are unable to father a child. Women who have CF may have more difficulty getting pregnant than other women.
People who have CF may have weaker bones than other people. This is because their bones contain lower levels of minerals. Weakened bones can lead to bone fractures or osteoporosis. CF can also cause swollen or painful joints (arthropathy or arthritis). These problems are more common in adults than in children.
The life expectancy for people with CF has been steadily increasing. Because of newborn screening, many babies with CF are diagnosed before they develop symptoms. Early diagnosis and personalized treatment can help them feel better and live longer.
It's important to diagnose and treat CF early. Call your doctor if your child:
Call your doctor if your child who has been diagnosed with CF gets worse in any way. Typically, this is when your child:
A medical history and a physical exam are often the first steps in diagnosing CF. This is followed by screening or lab tests.
The diagnosis of CF requires one of the following:
Also, there must be at least one of the following:
Both newborns and adults can be tested for the changed (mutated) gene that causes CF. These tests include:
After your child is diagnosed, a team of health professionals will work with you to build a treatment plan. The plan is based on your child's specific health problems. It may include respiratory therapy, medicines, a fitness plan, and nutritional therapies. Following this plan, along with getting regular medical care and taking steps to reduce infection, can help your child lead a longer, healthier life.
The best treatment available is often found at CF care centers. These centers address medical, nutritional, and emotional needs. Contact the Cystic Fibrosis Foundation at www.cff.org to find one.
Regular visits with the team involved in your child's care are important. Your doctor will want to make sure that your child eats well and gains weight and grows at a normal rate. The doctor will record your child's weight, height, and head size. This lets the doctor keep track of how your child develops over time.
Lab tests can help your doctor know how serious the disease is and how it is affecting your child's body.
Your doctor will ask you about your child's immunizations. The doctor will schedule any shots that are needed. Children with CF should have all the recommended shots, plus pneumococcal shots.
Respiratory therapy is any treatment that slows down lung damage and improves breathing.
People with CF have thick and sticky mucus that traps bacteria. This causes lots of lung infections and often causes permanent lung damage. The focus is to reduce infection and get rid of mucus to keep the lungs healthy. Medicines may be used to control the amount and thickness of mucus.
Other ways to help remove mucus from the lungs involve airway clearance techniques (ACT). These include coughing and breathing exercises, wearing a percussive vest, and using positive expiratory pressure (PEP) devices.
This treatment works to replace certain digestive enzymes. It helps to make sure the body absorbs all the vitamins and minerals it needs. This treatment also can prevent or treat intestinal blockages. It involves:
You and your child will struggle at times with this illness. But having a good attitude and lots of support will help you and your child cope.
Being the daily caregiver of a family member with special healthcare needs isn't easy. It's important to find support so that you can give your child the best care possible.
Consider joining a support group in your area, or even online. These groups can help you connect with other parents who have a child with the same condition. They can also help you learn what resources are available in your area.
Support groups can be a source of emotional support, but you may also find counseling useful. It can help you understand and deal with the wide range of emotions you may feel.
Your child will need help too. Providing emotional support for your child can help them cope with the difficulties they may face. And meeting other kids with the same condition can help your child feel like they are part of a community.
Medicines for CF help keep the lungs as healthy as possible, reduce and control mucus in the lungs, and replace digestive enzymes. There are also medicines that can improve lung function for those with certain gene changes.
Some medicines cost a lot, but there may be programs to help pay for them.
Surgery may be needed to treat problems from CF. These procedures may include:
If a child has CF and isn't able to get all the nutrients they need from food, a feeding tube may be placed in the child's stomach.
CF causes mucus to become thick and sticky, which can clog the lungs and cause serious problems. You can help your child maintain lung function and avoid problems from mucus buildup and blockage by doing airway clearance techniques. As your child gets older, they'll be able to do this on their own.
Airway clearance techniques (ACT) include special breathing and coughing exercises. It may involve using postural drainage and percussion. An older child or adult may use a percussive vest or use a positive expiratory pressure (PEP) device. These all help with clearing mucus from the lungs.
Your child's care team will make a personal treatment plan that includes ACT. These therapies are one part of a larger treatment plan to help keep your child's lungs clear.
It is important for your child to visit their doctor regularly and make any needed changes in treatment.
Current as of:
March 3, 2021
Author: Healthwise StaffMedical Review: John Pope MD - PediatricsKathleen Romito MD - Family MedicineR. Steven Tharratt MD, MPVM, FACP, FCCP - Pulmonology, Critical Care Medicine, Medical Toxicology
Current as of: March 3, 2021
Author: Healthwise Staff
Medical Review:John Pope MD - Pediatrics & Kathleen Romito MD - Family Medicine & R. Steven Tharratt MD, MPVM, FACP, FCCP - Pulmonology, Critical Care Medicine, Medical Toxicology
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