Cystic Fibrosis

Is Cystic Fibrosis curable?

While there is no cure for CF, there has been significant progress in its treatment. Over time, CF causes frequent lung infections due to increased presence of mucous in the airways and, ultimately, respiratory failure. In the pancreas, mucous blocks the release of digestive enzymes, which help the body digest food and absorb its nutrients. In the liver, mucous buildup in create a blockage in the bile duct, causing liver disease. The severity of symptoms differ from patient to patient, so it is important to create an individualized treatment plan with your healthcare providers.

Treatments for CF have traditionally been inhaled through a nebulizer. Newer oral medications are available, as well. Once a therapy is started, it is best to continue it unless you are told otherwise by your healthcare providers. It is essential to stay consistent and continue taking your medications even if you being feeling better. These medications may help prevent future exacerbations or complications.

Beyond medications, there are methods that patients use to assist with loosening the mucous in the chest. Chest percussion is a manual way of doing this, and there are mechanical vests which can also produce vibrations. There are also breathing techniques that can be used.

Does diet affect Cystic Fibrosis?

Eating well every day may help combat growth deficiencies caused by CF. Foods that are high in fat and protein (nuts, cheese, meat with fats) are recommended. Also, when needed, calorie boosters like butter can help. Fat soluble vitamins A, D, E, and K are important because their absorption can be affected by poor nutrition, so taking a multivitamin may be necessary. Patients often will take pancreatic enzymes in the form of a capsule with meals and snacks in order to help absorb the nutrients in their food. If patients are struggling to get their nutrition from conventional means, feeding tubes can be used to supplement.

Cystic Fibrosis-Related Diabetes

In some patients, it is possible to developed Cystic Fibrosis-Related Diabetes (CFRD). CFRD has similar characteristics to both Type 1 and Type 2 diabetes. Patients with CFRD may not be able to make enough insulin in the body, and may not have a typical response to insulin when sick or taking steroids. While CFRD symptoms may not be noticeable prior to diagnosis, symptoms of elevated blood sugar include increased thirst and urination. Symptoms may also include excessive tiredness and unexplained loss of weight or lung function. CFRD treatment is centered on normalizing blood sugar levels, which can be achieved through diet and exercise and insulin injections, if needed.


Key Patient Information

  • CF primarily causes coughing with phlegm, frequent pulmonary infections, and wheezing/shortness of breath.
  • Keep away from germs! Try to stay at least 6 feet from others with CF and anyone with a cold flu or other infection. Wash your hands/ use alcohol-based hand gel, cover your cough, clean and disinfect your nebulizer, avoid frequent contact with dust and dirt, don’t share personal items with others, and get vaccinated.
  • Patients with CF need roughly 1 ½ to 2 times as many calories as non-CF patients. Diet is very important to CF health, and high-fat, high-protein foods are the foundation. These foods include nuts, cheese, and meats with fat.
  • Aerobic exercise and physical activity, in addition to improving sleep and energy levels, can also help to mobilize secretions in the chest. Talk to your doctor about creating an exercise plan that is safe for you.
  • When using a nebulizer, use only approved nebulizers when administering medication. Be sure to clean the nebulizer after use.
  • Most inhaled medications for CF require refrigeration. Medication can be stored at room temperature for different lengths of time based on the drug and recommendations from the manufacturers. Do not use if medicine is cloudy or if there are particles in it.

Living with Cystic Fibrosis

Patients with CF can lead healthy, vibrant lives. Because of this, it is important that patients have plans in place to maintain their physical and mental health. Exercise can help clear mucus from the lungs, improve breathing ability, your mood, and help protect you from heart disease. In addition, exercise provides an opportunity for social interaction. Children are encouraged to get 60 minutes of exercise daily, and the recommended amount for adults is 30 minutes 5 times weekly. Your care team may include a social worker, therapist, or support system to help deal with the emotional challenges that come with such a diagnosis. Taking a multi-centered approach to dealing with CF can help to maximize your quality of life.

Depression

Depression is common in patients with chronic illness. Both adults and children may get depressed.

Symptoms may include:

  • loss of interest in daily activities and/or feeling great sadness
  • Loss of appetite or increased appetite
  • Inability to experience pleasure
  • Sleeping too much or having difficulty sleeping
  • Fatigue
  • Headaches or general aches and pains
  • Feeling guilty or worthless
  • Difficulty completing tasks or concentrating
  • Thoughts of death or suicide
  • Anxiety attacks
  • Tearfulness or being unable to cry
  • Isolation
  • Soft, slow speech

Depression can be successfully managed via medications and counseling. Patients who experience any of the symptoms above should seek medical help.


Overcoming Medication Challenges

It is important for you to know the benefits of your medications. Here at Magellan Rx, we understand your concerns and want to help you with any difficulties you may encounter. For additional information on how to overcome medication challenges, please click here: Overcoming Medication Challenges