Living Longer With Cystic Fibrosis


CLEVELAND, Ohio. (Ivanhoe Newswire) — More than 30,000 Americans are living with cystic fibrosis, or CF, for short. Years ago most kids with this disease didn’t make it into their teens, but now, more and more are living longer.

John O’Neil stays busy with a demanding job, a wife, and three young girls.

“I like to tell people it’s basically like a circus that I live in 24/7,” O’Neil told Ivanhoe.

He also has cystic fibrosis, or CF, a genetic disease that causes mucus to build up in his lungs and other organs. John takes a handful of pills every day and needs breathing treatments to keep his lungs clear. But he doesn’t let it get him down.

“I’m never going to let this disease define who I am as a person,” said O’Neil.

Years ago, the outlook for cystic fibrosis was grim.

“If you look back into the 1950s, a child born with CF, the parents would be told that they’d be lucky if they made it to elementary school,” detailed Elliott Dasenbrook, MD, the Director of Adult Cystic Fibrosis Center at Cleveland Clinic.

Today, the average age of survival is about 47, with many living longer! Doctors credit better care and nutrition. Also, for the first time, newer drugs that target the gene defect have recently become available.

Dr. Dasenbrook said “There are now more adults in the United States with CF than there are children.”

But there are challenges: adults with CF are more likely to develop infections, diabetes, arthritis, fertility problems, and a resistance to antibiotics. That’s why specialty centers that treat adults only can be helpful.

John feels better than ever and has big plans for his future.

“My aspiration is to live to see my daughters grow up and walk them down the wedding aisle one day and live into grandparenthood with my wife,” said O’Neil.

A goal that he has a good shot at making!

People with CF have inherited two copies of a defective gene, one from each parent. Both parents must have at least one copy of the defective gene for a child to have CF.  People with one copy of the defective gene are known as carriers but they don’t have the disease.

Contributors to this news report include: Julie Marks, Producer; Robert Walko, Editor. 

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BACKGROUND: Cystic fibrosis is an uncommon genetic disorder that primarily affects the respiratory and digestive systems. Symptoms often include chronic cough, lung infections, and shortness of breath. Children with cystic fibrosis may also have trouble gaining weight and growing. Roughly 30,000 people in the United States have cystic fibrosis. The disease affects about 1 in 2,500 to 3,500 newborns. About 75 percent of diagnoses happen before age 2. It is now mandatory for all doctors in the United States to screen newborns for cystic fibrosis. The test involves collecting a blood sample from a heel prick. A positive test can be followed up with a “sweat test” to measure the amount of salt in the sweat, which can help secure the diagnosis. Treatment revolves around keeping the airway clear and maintaining adequate nutrition. Health problems can be managed, but there’s no known cure for this progressive illness. Until late in the 20th century, few people with cystic fibrosis lived beyond childhood. Improvements in medical care have increased life expectancy by decades.


SYMPTOMS AND TREATMENT: The symptoms of cystic fibrosis can vary depending on the person and the severity of the condition. Symptoms may appear at infancy, but for other children, symptoms may not begin until after puberty or even later in life. One of the first signs of cystic fibrosis is a strong salty taste to the skin. Other symptoms of cystic fibrosis result from complications that can affect the lungs, pancreas, liver and other glandular organs. Some current treatments being used are medications, surgical procedures and home care. Antibiotics may be prescribed to get rid of a lung infection and to prevent another infection from occurring in the future. They’re usually given as liquids, tablets, or capsules. In more severe cases, injections or infusions of antibiotics can be given intravenously. Chest therapy can help loosen the thick mucus in the lungs. A common technique involves placing the head over the edge of a bed and clapping with cupped hands along the sides of the chest. Cystic fibrosis can prevent the intestines from absorbing necessary nutrients from food. A person might need up to 50 percent more calories per day than people who don’t have the disease. They may also need to take pancreatic enzyme capsules with every meal. Your doctor may also recommend antacids, multivitamins, and a diet high in fiber and salt.


FUTURE FINDINGS: The outlook for people with cystic fibrosis has improved dramatically in recent years, largely due to advances in treatment. Today, many people with the disease live into their 40s and 50s, and even longer in some cases. However, there’s no cure for cystic fibrosis, so lung function will steadily decline over time. Scientists at Boston University’s Center for Regenerative Medicine have made two stem cell discoveries that could help doctors treat cystic fibrosis without having to resort to transplants. They prevented stem cells from creating cells for a lot of organs at once and producing only the lung cells they need for cystic fibrosis work. They also converted immature lung cells into airway cells by turning off a molecular pathway known as the ‘WNT’ signaling pathway. These two discoveries could pave the way for personalized medicine in cystic fibrosis, the team said.

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