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Cystic Fibrosis

Adults with Cystic Fibrosis

How does cystic fibrosis affect adults?

Only over the past several decades has this question become relevant. Twenty or thirty years ago, most individuals with CF did not survive into adulthood. Fortunately, improved medication, care, and understanding of the disease now assure that almost all people with CF will live to become adults. Most will even be healthy, well, and able to pursue the same goals that those without CF dream of.

It remains true that those with CF can expect a shortened life, but those that are currently children may expect to live until middle age, or even a normal lifespan. Those who are now adults and healthy can count on the opportunity to pursue careers, raise families, and enjoy most leisure activities. Those who are currently adults with more advanced illness will have fewer opportunities, a shorter expected lifespan, and a more restricted lifestyle. However, these adults can still benefit from improved therapies, less frequent severe health problems, and can expect further advances to better their lives over the next several years.

Nonetheless, it is important to be realistic; progressive, severe lung disease, difficulty breathing, and relatively early death are still the eventual outcome for those that either do not choose to, or cannot undergo lung transplantation.

Is CF ever diagnosed in adults?

While CF is usually diagnosed when individuals are children or teens, occasionally a person with what has been thought to be:

will come to the attention of a health professional who suspects that the problem might not be that simple. A sweat test or genetic testing will confirm the diagnosis of CF.

Confronting the prospect of a new, life-shortening disease at that point can be very difficult. It can be psychologically difficult and involves incorporating a complex and time consuming new treatment regimen into an already busy life. Often, the person will need an initial hospitalization to receive IV antibiotics and training.

The benefit of diagnosis is often relief, as the individual is finally able to identify why they have been more ill than expected. Usually, the improved level of health and energy offsets the new difficulties in the long run.

How is cystic fibrosis treated in adults?

The treatment of CF in adults is very similar to that of children and teens with CF. 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:

in order to continue the best possible health. These requirements are difficult to fit into a busy schedule, especially involving post-secondary education, parenting, and work. Thus, it is important to clarify with the care team which activities are crucial, and which can slide a little. Those that are determined to be very important really must be fit in, or the person's health and ability to enjoy other life pursuits will suffer.

Treatments may consist of very few medications and therapies in a lucky few. More typically though, they involve a half-hour of inhaled medication and lung clearance activities once or twice per day, as well as oral medication to improve absorption of food before meals and snacks. Other medications and treatments depend on the level of care needed.

It is critical to monitor for worsening of problems early on, before they become severe enough to cause more permanent damage. Individuals can also expect the occasional need for oral or intravenous antibiotic medication 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% to 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. A certain number of 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 almost certainly due to a number of factors. It is important for adults with CF to 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: Approximately 25% of children and adults with CF have some degree of liver abnormality, although it is mild in the majority of that 25%. Only a small number 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. However, the possibility of liver problems makes the need to keep alcohol intake at a very 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. It is best not to be treated with any habit-forming or narcotic medications.

Are people with CF able to have families?

Yes. There are, of course, some factors that affect this. Women with CF may experience slightly greater than usual difficulties conceiving, but usually can do so. Women need to consider their overall health before conceiving, with attention to both their ability to stay healthy during pregnancy and their ability to stay healthy 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, until tested for fertility, men should NOT assume infertility, to avoid surprises. Men can have biological children through the use of alternative methods involving sperm harvesting and artificial insemination. Both men and women need to consider their health and life expectancy in their decisions about caring and providing for a family.

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. There are some career paths that require careful consideration, and may be difficult to follow, such as health care fields involving contact with ill individuals. However, the CF team can help sort out these issues.

What are the problems facing adults with advanced illness?

As mentioned above, virtually all adults with CF will face increasing lung disease and eventual death at some point. Fortunately, the option of lung transplantation is available to many. This can tremendously improve life expectancy and the quality of life. For those who are either not able to undergo transplantation, or choose not to do so, it is important to resolve legal, family, and personal issues. It is important for the individual to experience the end-of-life period according to his or her wishes.

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Last Reviewed: Dec 15, 2006

The Ohio State University John S. Heintz, MD
Clinical Assistant Professor
Director, Adult Cystic Fibrosis Center
Division of Pediatric Pulmonology
Department of Pediatrics (Nationwide Children's Hospital)
College of Medicine
The Ohio State University
John S. Heintz, MD