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What Every Parent Should Know About Cystic Fibrosis in Children

Adam Secory

mother comforts daughter, who is wearing a breathing mask

You may not even remember it, but in the first few days after your baby was born, they were screened for cystic fibrosis. All 50 states have newborn screening programs that check for the genetic disorder.

Unless your baby had a positive test, you likely just moved on to the other newborn issues you had to worry about. While you may be in the clear with this child, there's a chance your next could get cystic fibrosis.

Cystic fibrosis is a genetic disease inherited from both parents.

Both parents must be a carrier of the gene mutation that causes cystic fibrosis. One in 31 people carries the mutation. If both you and your spouse are carriers, there's a 25 percent chance your child will have CF. And that probability applies to every child you have.

Every person with cystic fibrosis is born with it — it happens at conception. Overall, about one in every 3,500 babies has the genetic disease that causes the production of thick, sticky mucus. The mucus builds up in your child's lungs, digestive tract and other parts of the body. Over time, the build-up can clog their lungs and lead to lung infections and respiratory illnesses. The same thing can happen in the pancreas, creating a blockage that prevents your child from breaking down the food they eat.

While the gene mutation is passed on at conception, only 10 to 15 percent of babies have symptoms at birth. But the most common signs typically show up before the age of two:

  • Wheezing, coughing and increased mucus in the lungs
  • Pneumonia
  • Salty-tasting skin
  • Slow weight gain
  • Enlarged fingertips and toes
  • Polyps inside the nose
  • Abnormal bowel movements and rectal prolapse

These signs are similar to many other health problems, which can make identifying the disease difficult in cases where a diagnosis was missed in newborn screening.

Sweat is the key to diagnosing CF.

Parents are often the first to notice cystic fibrosis symptoms in their baby. When you smother your baby with kisses, the salty taste is a giveaway.

Salty skin is the marker for cystic fibrosis because the disease changes how the exocrine glands handle salt. So there's a high amount of salt in the sweat. A test to measure the salt content of your child's sweat will tell you if they have CF. When there's a positive result on the newborn screening at the hospital or you notice the symptoms yourself, your baby will get a sweat test to diagnose CF.

Because cystic fibrosis is hereditary, it's important to test your other children, even if they show no symptoms. The age when symptoms show up varies, so your child could still have CF even if you haven't seen any signs. Or they might not have the disease but are still a carrier of the mutated gene, meaning their children could get cystic fibrosis.

Any future children could, too. Since CF starts at conception, prenatal genetic screening tests are available to pregnant women to check for the disease before baby is born.

Cystic fibrosis has long-term effects on physical development and health.

Salty sweat and other symptoms lead to a diagnosis but those aren't the only problems that result from CF. While the severity of each case varies, your child will get sick more often and need more medical attention. Cystic fibrosis is a life-long disease, so its effects will present challenges throughout your child's whole life, including:

  • Slow growth and physical development – Thick, sticky mucus mucks up the digestive system, blocking tubes inside the pancreas and making it hard to digest food. Your kid won't be able to absorb the nutrients needed to grow and stay healthy.
  • Breathing problems – The mucus also makes it much harder for your child to breathe and increases their risk of infection. They will cough, wheeze and hack up mucus more often. They will catch infections that don't normally affect healthy babies and children.
  • Lung infections and lung disease – As your kid grows up, respiratory infections can get worse and cause serious damage, resulting in lung disease.
  • Dehydration and heat stroke – With the loss of more salt through sweat, your child is more likely to get dehydrated or succumb to heat stroke. You'll have to take extra measures to keep them hydrated and cool in hot weather.
  • Infertility – Further down the road, cystic fibrosis can lead to fertility issues in adulthood. Men can become sterile and women can produce so much mucus that it's difficult for sperm to reach their eggs.

Early detection and early treatment are key.

There's a reason Iowa and every other state has standardized newborn screening programs to check for cystic fibrosis. When CF is caught early, your baby's healthcare team can develop a treatment plan to manage symptoms, aid growth, prevent complications and slow lung damage.

The most important treatments address the lung and digestive issues that are common with cystic fibrosis. Your kid may need mucus thinners, antibiotics and other medications to breathe better and prevent infections. If they fall behind in their development, medicine that helps their body absorb nutrients and a diet of healthy, high-calorie meals are often necessary.

Your child's treatment plan will depend on their symptoms and the severity of the disease. In addition to managing the treatment plan, there's a lot more to do as a parent, especially in the early years.

  • Infants – Babies and infants need more care and are more susceptible to infection. You'll want to ensure daycare, babysitters and family members are educated about CF and your child's needs. Take extra precautions with cleanliness to help prevent the spread of bacteria and infections and constantly monitor their breathing.
  • Toddlers – Developing good eating habits is important for all children between the ages of 3 and 5, but especially for kids with cystic fibrosis. Consult a dietitian for help with creating a positive mealtime environment, establishing healthy food choices or just getting cranky tots to eat.
  • School-age children – Once your kid is off to kindergarten, they may have to start doing a lot of the care themselves, like taking medicines at mealtime, staying hydrated and getting proper nutrition. Absences from school are common due to frequent infections, or in some cases, hospitalization, making it important to help them keep up with their schoolwork.

Cystic fibrosis is no longer just for kids.

And that's a good thing. Cystic fibrosis was once known as a disease of children. The life expectancy for children born with the disease was so low that they didn't reach adulthood.

Medical advances have changed that. Now, of the 30,000 people living with cystic fibrosis in the United States, half are adults. Cystic fibrosis is being diagnosed and treated sooner, so people are living longer. The life expectancy for people with cystic fibrosis today is about 38 years, with more and more people living even longer.

If you're concerned about cystic fibrosis in your child, talk to your pediatrician about the symptoms you've seen. While newborn genetic screening tests are effective at catching CF early, they don't catch every case. If someone else in your family has CF and you're worried that you or your spouse are carriers, consult your primary care provider for your testing options.

Adam Secory

AdamSecory , DO

Adam Secory, DO has been with The Iowa Clinic since 2013 and practicing as a board-certified pediatrician in Des Moines since 2008. The culture, the ability to grow the Pediatric Department, and working closely with subspecialists is what drew him to The Iowa Clinic. 

Dr. Secory enjoys working with kids and their families from birth to adolescence, making pediatrics the perfect fit. His patients motivate him to be a better doctor with their smiles, hugs, and steadfast honesty. He has been fascinated by pediatric cardiology and its complexity ever since a mission trip to the Yucatan for patients born with congenital heart defects. Other clinical interests include sports medicine and dermatology.

When he is not practicing medicine, Dr. Secory spends time with his wife and two girls. Together they golf, bike, play tennis and participate in any other outdoor activities whenever they get the chance.