Adults with Cystic Fibrosis

How does cystic fibrosis affect adults?

Just 20 to 30 years ago, most individuals with cystic fibrosis (CF) did not survive into adulthood. Fortunately, most children and teens with CF today will live to become adults thanks to improved medication, care, and understanding of the disease. Most will even be healthy enough to pursue the same goals that those without CF dream of.

Today’s children may be able to live until middle age, or even to a normal life expectancy. Currently healthy adults with CF pursue careers, raise families, and enjoy most leisure activities. But adults with more advanced illness do have fewer opportunities, a shorter expected lifespan, and a more restricted lifestyle. However, these adults can still benefit from improved therapies that reduce the frequency of severe health problems. All patients can expect further scientific advances to better their lives over the next several years.

Nonetheless, it is important to be realistic. Progressive lung disease, breathing difficulty, and relatively early death are still the eventual outcomes without a lung transplant.

Is CF ever diagnosed in adults?

While CF is usually diagnosed in children or teens, previously undiagnosed adults will occasionally come to the attention of health professionals. One or more of the following conditions may be seen in these individuals:

A sweat test or genetic testing will confirm the diagnosis of CF. Confronting the prospect of a new, life-shortening disease can be challenging. It can be psychologically difficult and it involves adding a complex and time consuming new treatment regimen into an already busy life. Often, the person will be admitted to the hospital to receive initial IV antibiotics and training.

It can also be a relief to get the diagnosis of CF because individuals are finally able to identify why they have been ill. In the long run, improved health and energy usually offset the new difficulties.

How is cystic fibrosis treated in adults?

Treatment of CF in adults is similar to that of children and teens. In adults, sticking to therapies is at least as important, if not more so, than when younger. Inevitably, lung damage is greater in adulthood than earlier in life. Therefore, it is critically important to maintain the best possible health. Steps you should take include the following:

Maintain excellent nutrition
Be of normal weight
Keep lungs as clear of mucous and secretions as possible
Treat infections promptly
Follow regularly with an accredited CF center

These requirements are difficult to fit into a busy schedule, especially one that involves school, parenting, and/or work. Thus, you should clarify with the care team which activities are crucial, and which can slide a little. Those that are very important must be fit in, or your health and ability to enjoy other life pursuits will suffer.

Treatments may consist of very few medications and therapies in a lucky few. More likely, treatments involve 30 minutes of inhaled medication and lung clearance activities once or twice a day. Oral medication is also needed to improve absorption of food before meals and snacks. Other medications and treatments depend on the level of care required.

It is critical to monitor problems early before they worsen and cause permanent damage. Oral or intravenous antibiotic medication may be needed at times to decrease the level of lung or sinus infection. This will sometimes require admission to the hospital.

What other kinds of problems occur in adults along with CF?

Diabetes: Diabetes, known as CF-Related Diabetes Mellitus (CFRDM) is more common in adults with CF than in those without. It is estimated that 15-45% of individuals older than 30 in the CF population have CFRDM. The primary source of the CFRDM appears to be an inability of the pancreas to produce enough insulin. The usual treatment involves the use of injected insulin. Some people are able to be treated with oral medications, at least early in the process. Often, these people will eventually require the use of insulin.

Osteoporosis: Thin bones are almost universally common in adults and even teens with CF. It is still unclear exactly why this occurs, and is almost certainly caused by a number of factors. Adults with CF should have routine bone density evaluation (DEXA scans). If needed, adults should be treated with weekly or monthly medication for treatment of osteoporosis, in order to prevent broken bones.

Liver Problems: About 25% of children and adults with CF have some degree of liver abnormality, although it is mild for most. Only a small number in this group will have problems that affect their health. Occasionally, these problems will require daily medication. Even less commonly, they will cause cirrhosis and the need for liver transplantation. The possibility of liver problems makes it a necessity to keep alcohol intake at a moderate level, at most.

Joint pain/arthritis: A significant minority of people with CF will have some degree of joint pain, and sometimes actual arthritis. This can range from mild, occasional discomfort to chronic arthritis pain. This is always best treated with the usual arthritis medications, and sometimes with the assistance of an arthritis specialist. Avoid treatment with any habit-forming or narcotic medications.

Are people with CF able to have families?

Yes, but some CF-related factors can have an effect. Both men and women need to consider their health and life expectancy in their decisions about caring and providing for a family.

Women with CF may have greater difficulty conceiving, but usually can do so. Before conceiving, women should consider both their ability to stay healthy during pregnancy and while caring for a demanding infant.

Most men (95%) with CF cannot conceive through usual intercourse due to a blockage or absence of the tubes that carry sperm from the testicles. However, men should NOT assume infertility until tested for fertility to avoid surprises. Men can have biological children through the use of alternative methods involving sperm harvesting and artificial insemination.

Are people with CF able to have careers?

Almost all teens and adults with CF are able to pursue educational and work goals, and should plan to do so. Keep in mind that some career paths require careful consideration and may be difficult to follow. For example, health care fields involve contact with ill individuals, which can be risky for a CF patient. Your CF team can help sort out these issues.

What are the problems facing adults with advanced illness?

Virtually all adults with CF will face increasing lung disease and possibly the need for lung transplantation. This can tremendously improve life expectancy and the quality of life. For those who are either unable or unwilling to get a transplant, legal, family, and personal issues should be resolved in advance. It is important for individuals to experience the end of life period according to their wishes.

For more information:

Go to the Cystic Fibrosis health topic.