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Tuesday, August 30, 2016
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.
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.
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.
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.
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.
This article is a NetWellness exclusive.
Last Reviewed: Jun 24, 2010
John S Heintz, MD
Clinical Assistant Professor of Pediatrics
College of Medicine
The Ohio State University