Just 30 years ago individuals with cystic fibrosis didn’t live past the age of seven. Today, due to the improved diagnosis and treatment protocols, people are living into their 30’s and 40’s. Most are healthy enough to pursue the same goals and dreams that those without cystic fibrosis do.
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 are necessary to stop the stem of infection and mucus build up that are at the root of sinusitis, allergies, asthma and digestive problems.
But the diagnosis is also a 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 the best possible health. Treatment protocols will include good 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 toilet 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 toilet will consist of postural percussion and drainage as well as specific medications that are designed to break up the mucous and treat the infections. These treatments will sometimes require an admission to the hospital in order to gain full benefit from the protocol.
People who suffer from cystic fibrosis can also develop other conditions that are more common in those who experience the increased mucous and digestive issues. 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. In the beginning most people are able to be treated with oral medications for their diabetes but eventually they will require insulin.
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 but, at this time, researchers and doctors aren’t clear as to why it happens. About 25% also have trouble with their liver as well as a significant number who 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 Chest in April 2001 researchers found that as a group adults who suffered from cystic fibrosis didn’t demonstrate any significant levels of depression, anxiety or other psychopathology. Overall adults who reported healthy psychological functioning also had better lung function and stronger social support which has implications for clinical treatment protocols. (1)
University of Michigan: Cystic Fibrosis
Canadian Medical Association: Cystic Fibrosis in Adults
Current Opinion in Pulmonary Medicine: Manifestations of Cystic Fibrosis Diagnosed in Adulthood