Adult Cystic Fibrosis

Just 30 years ago individuals with cystic fibrosis (CF) often did not live past the age of seven. Today, due to the improved diagnosis and treatment protocols, including the use of airway clearance devices, many cystic fibrosis patients are living into their 30’s and 40’s. And according to a recent article, in some countries, the life expectancy is expected to increase to age 75 in the next 10 years due to advances in medication and treatment. As the life expectancy of CF patients has risen, new issues of care management have arisen. Now it is not unusual for people with CF to live as independent adults.

Previously, CF patients were mostly cared for by their parents. Now as they are starting to live much longer, they may no longer be able to rely on their parents for care and help with daily chores such as airway clearance. A major advancement in the treatment of CF has been the introduction of airway clearance devices such as the Hill-Rom The Vest® Airway Clearance System. The key to surviving appears to be a consistent, daily regiment that involves exercise, a healthy diet, good hygiene and airway clearance therapy.

Because cystic fibrosis can present in a variety of ways, and individuals can have a variety of severity of those symptoms, there will be those people who will have very small number of side effects from the disease while others will suffer from progressive lung disease and a relatively early death.

Cystic fibrosis is usually diagnosed in infancy but occasionally there are those individuals who slip past and are found in early adulthood. Other diagnoses can include chronic asthma, sinusitis, allergies, diarrhea, and low weight. These diagnoses are co-morbid with the diagnosis of cystic fibrosis because of the nature of the disease.

A sweat test or genetic testing will confirm the diagnosis of cystic fibrosis but a diagnosis at this late stage in life will also present a challenging psychological experience. And this new diagnosis will also add more complex and time consuming treatment regimens into a busy life. IV antibiotics and training will are necessary to stop the stem of infection and mucus build up that are at the root of sinusitis, allergies, asthma and digestive problems.

Even though the diagnosis can be devastating to parents and patients alike, it can also come as relief because it gives an answer to why individuals were ill for so long and gives them a path to improved health and energy.

Treatment for adults with cystic fibrosis, whether the individual is diagnosed at infancy or at a later age, is much the same as for children or teens. The difference lies in the compliance rate of adults, who must be more compliant than teens so they can maintain good health. Treatment protocols will include excellent nutrition that is low in fat and high in protein to make the most of the digestive issues. This improved nutrition should help the individual to maintain a normal weight that will help the body to have the reserve to fight infections.

Adults should also add pulmonary therapy into their daily schedules. This will help the lungs to stay clear of mucous and secretions which will also reduce the risk of infection and lung damage. Pulmonary therapy consists of postural percussion and drainage as well as specific medications that are designed to break up the mucous and treat any infections. In addition, or in lieu of percussion therapy, airway clearance devices such as the Hill-Rom Vest can be an essential part of a daily regiment for CF patients. Regardless of which therapy is used, it must be maintained regardless of how well the CF patient is feeling on a particular day. Regularity and treatment compliance appear to be key factors in maintaining and improving lung function.

People who suffer from cystic fibrosis can also develop other medical conditions the first, diabetes, is most common. It is estimated that between 15 and 45% of people older than 30 who have CF also have diabetes.

Osteoporosis is almost universally common in adults who have cystic fibrosis and can even be found in teens. Thin bones that are more at risk for fracture can be caused from a number of factors. About 25% of CF patients also have trouble with their liver and often suffer from joint pain and arthritis.

Adults who suffer from CF and want a family should consider whether or not they’ll be available to help care for their family as they grow. While most men are infertile because of blocked tubes from the testicles men should NOT assume infertility until tested. Women may have a greater problem getting pregnant but are usually able to successfully achieve pregnancy.

In a research study published in the April 2001 Magazine ‘Chest’, researchers found that adults CF patients didn’t demonstrate any significantly higher levels of depression, anxiety or other psychopathology. Overall adults with CF that reported healthy psychological functioning also had better lung function and stronger social support.