Cystic Fibrosis: Increased Life Expectancy And New Hope

For most people, breathing is not something they think about. But imagine feeling like you cannot get enough air into your lungs, and you are suffocating. That is what life can sometimes be like for people with cystic fibrosis. Cystic fibrosis can make breathing difficult and also lead to additional health problems. On the positive side, the life expectancy for the condition has doubled in the last 50 years. 

Gene Mutation and Cystic Fibrosis

Cystic fibrosis is a genetic condition, which means it is something you are born with. 

Excess thick mucus is produced. When you think of mucus, you may think it only affects the nose and the lungs. But mucus can also build up in the pancreas and other organs.

The cause of the condition is a mutation in a gene, which generates CFTR protein. CFTR regulates water and salt levels in and out of the body. A mutation in the gene damages the normal production of CFTR protein. In order to develop cystic fibrosis, a child must have inherited the defective gene from both parents. Although the gene, which causes cystic fibrosis, has been identified, the reason it mutates in some people is still being researched.

Not every person who is a carrier of the disease will produce symptoms. 

In some people, symptoms start early in childhood. When symptoms start early in life, their lung function often starts to decrease by middle childhood.

Symptoms and Prognosis

In people who have cystic fibrosis, their mucus becomes thick and often sticky and is produced in excess. With thick mucus sticking to the lungs, breathing becomes difficult. The increase in mucus also creates an environment in the lungs where bacteria tend to grow easily. People with cystic fibrosis, often develop frequent respiratory infections, such as pneumonia. 

Mucus is not only present in the lungs. It can also build up in the ducts of the pancreas. Digestive enzymes produced in the pancreas are not able to research the small intestines, due to the excess mucus clogging the ducts. The enzymes breaks food down so nutrients can be absorbed by the body. Because the enzymes don’t reach the small intestines, proteins and fats cannot be completely absorbed.

The condition also increases the amount of salt in the sweat. When people with cystic fibrosis sweat, they may lose excess amounts of salt, which can lead to additional health problems. Salt is one of the minerals needed in the body and plays a role in proper fluid balance. When fluid balance in the body is disrupted, problems, such as dehydration and low blood pressure can develop.  

See Also: Living with Cystic Fibrosis

In addition to the health problems mentioned above, cystic fibrosis usually causes infertility in men and difficulty becoming pregnant in women. Both men and women with cystic fibrosis are also at an increased risk of bone loss as they age.

Living With Cystic Fibrosis

Although there is not currently a cure for cystic fibrosis, there is treatment. Patients can also take an active role in their health. There are several things a person with cystic fibrosis can do to control symptoms and increase their quality of life.

Increasing quality of life with Cystic Fibrosis

Quality of life may mean different things to different people. But for everyone a decrease in symptoms and the ability to function as well as possible is the goal.

Although a pediatrician or internal medicine specialist may have some knowledge about cystic fibrosis, a pulmonologist is a lung specialist who should be aware of the latest treatments, research and clinical trials for the condition. You may also want to receive care at one of the 110 Cystic Fibrosis Foundation accredited care centers located throughout the United States.

In addition to seeking the right care team, one way to improve your wellbeing is by paying attention to what you eat. Many people with cystic fibrosis are encouraged to eat a high fat, high-calorie diet. Since people with cystic fibrosis often do not absorb adequate level of nutrients, they need to take in extra calories. In addition, doctors often recommend taking vitamins and pancreatic enzyme supplements after eating to increase the absorption of nutrients.

Exercise can improve lung health in anyone, and people with cystic fibrosis are no different. Speak with your doctor about the type of exercise you should participate in and how to get started. Lastly, if you have cystic fibrosis, avoid smoking and being around secondhand smoke, which can damage your lungs even further.

Treatments Increases Life expectancy 

According to the National Heart, Lung and Blood Institute, there are many people with cystic fibrosis living into their 40s and 50s. Fifty years ago, children with the condition usually did not make it to their teens.There has been an increase in life expectancy due to improved understanding of the condition and more effective treatments.

Treatment for cystic fibrosis includes airway clearance techniques. This type of treatment involves the use of varied equipment to help loosen mucus from the lungs. Patients and their families are taught how to perform the various techniques at home to increase adherence to the regimen.

Inhaled medication is also often prescribed to people with cystic fibrosis. Various types of inhaled medications are used, which ease symptoms. For example, medication is available, which helps patients cough up mucus. Other inhaled medications contain antibiotics, which helps treat lung infections. In addition to inhaled medication, oral antibiotics are also often needed to treat respiratory infections.

See Also: Cystic Fibrosis: Causes & Risk factors

Recent advances in the treatment of cystic fibrosis include a medication, which treats the underlying cause of the condition. The drug is being tried on a small number of patients, and additional research is needed to determine its effectiveness. Although more work needs to be done to identify a definitive cure for cystic fibrosis, progress is being made.