Understanding the Prognosis for People with Cystic Fibrosis

Cystic fibrosis (CF) is a disease that people can be born with. While there is no cure, people with CF may live well into adulthood if they receive the appropriate treatment and medical monitoring.

Cystic fibrosis (CF) is a rare genetic disorder that causes thick, sticky mucus to build up in the lungs and digestive system. Around 30,000 people in the United States currently have CF, and around 1,000 new cases are diagnosed every year.

Diagnosis and treatment have improved greatly since CF was first discovered. As a result, people with the disease are living longer.

Here’s more about living with CF, the average life expectancy with the condition, and strategies that may help people avoid life threatening complications.

Learn more about cystic fibrosis.

What is the prognosis or outlook for people with cystic fibrosis?

Respiratory failure is the most common cause of death for people with CF. This condition typically develops after a long history of inflammation from CF that has damaged the lung tissue. People with CF are also more prone to developing certain complications that may shorten their lifespan, such as diabetes and cancer.

While there is no cure for CF, regular medical care may help slow the progression of the disease. Treatment aims to address the symptoms, prevent complications, and improve quality of life.

What is the general life expectancy for people with cystic fibrosis?

According to the Cystic Fibrosis Foundation’s 2019 Annual Report, the mean life expectancy for people with CF is 48.4 years .

Life expectancy has increased greatly over the years thanks to earlier treatment and improved therapies.

When the disease was first discovered in 1938, it was typically diagnosed after death in children less than 18 months old. In 1995, the mean life expectancy was just 28.9 years . Today, more than 50% of people living with CF are adults, and more than half of them may live beyond 40 years old .

CF patient registry data suggests that people born with CF between 2018 and 2022 may live to age 56 years or older. And half of those born between 2021 and today may have a life expectancy of up to 65 years.

What quality of life can people who have cystic fibrosis expect?

People living with CF experience a buildup of mucus in their lungs. The mucus clogs the lungs and may cause wheezing , coughing, and difficulty breathing . People with CF may also experience chronic lung infections.

When the mucus clogs someone’s digestive tract , they may experience issues such as malnutrition , slowed growth, jaundice (yellowing of the skin), and changes in their bowel movements ( diarrhea , constipation , or a foul smell ).

Treatment with medication and physical therapy may help manage these issues. But with longer life expectancy comes an increased risk for additional complications that may become serious or even life threatening.

Possible complications include :

• osteoporosis
• diabetes
• nasal polyps
• sinus infections
• liver or kidney issues
• fertility issues
• bladder control issues
• heart failure
• chronic lung infections
• salt loss syndrome
• cancer in the digestive tract

Overall, people who address their CF symptoms, maintain a healthy lifestyle, avoid infections and complications, and take care of their mental health may have a good quality of life.

What factors may determine quality of life for people with cystic fibrosis?

According to the National Heart, Lung, and Blood Institute (NHLBI) , living with CF involves attending regular medical appointments, closely following a prescribed treatment plan, and understanding how CF affects overall health. Vaccines for the flu , COVID-19 , pertussis , and pneumonia may also help people avoid serious infections.

Lifestyle is another major factor and can help with both managing symptoms and preventing complications. The NHLBI recommends following a healthy diet and exercise plan, managing stress levels, and practicing good sleep hygiene habits.

To prevent pancreatitis and ensure that CFTR modulator drugs continue working, people should also avoid smoking and secondhand smoke exposure and limit alcohol intake.

Along with physical health, mental health is an important piece of the puzzle. People with CF may have a greater risk of developing depression , anxiety , and other mental health conditions.

Advanced cystic lung disease

The chronic inflammation from CF may eventually lead to more advanced cystic lung disease. This disease and the resulting respiratory failure is the most common cause of death for people with CF.

Improving quality of life at this stage involves working closely with doctors to address symptoms, looking into lung transplantation , or seeking palliative or hospice care when necessary.

Frequently asked questions

How long will you live with cystic fibrosis?

Half of people with CF may live past 40 years old . How long you may live depends on factors such as what stage of CF you are in and any other health complications you may have.

Can someone with CF live a full and happy life?

Yes. While avoiding infections and treating symptoms is an everyday reality for people with CF, many people who live with the disease can hold jobs, go to college, and raise families.

What’s does “safe distance” mean for individuals with cystic fibrosis?

According to the Cystic Fibrosis Foundation , people with CF should stay 6 feet away from people who have a cold or the flu. This rule also applies to any contact with others who have CF to avoid transmitting or contracting germs that may cause serious infection.

Takeaway

There is no cure for CF, but advances in diagnosis and treatment may help people live longer than they would have in previous generations. These days, people with CF may go on to earn college degrees, start careers, and live full, happy lives — and the potential for longer life expectancy continues to extend each year.

If you have CF, you can work with your doctor to manage your symptoms and create a plan to avoid complications that may lead to more serious health issues. Finding a support network may also help you maintain your mental health and quality of life. If you cannot find a local support group , there are many opportunities to connect online .