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Life with Cystic Fibrosis

Cystic Fibrosis (CF) is an inherited disease that causes thickened mucus to form in the lungs, pancreas and other organs. In the lungs, this mucus blocks the airways, causing lung damage and making it hard to breathe. CF is a life-threatening condition, but thanks to advances in treatment and care, the average life expectancy has been steadily increasing and quality of life with cystic fibrosis has improved.

You or your loved one with CF might experience a wide range in severity of CF symptoms. Even within the same family, siblings can have differing disease severity.

Symptoms of CF can be classified into two main categories: symptoms of respiratory tract disease and symptoms of gastrointestinal disease. [Click here for a list of the most common symptoms of cystic fibrosis.]

What to Expect

You will meet with the care team (physicians, CF care coordinator, nurses, respiratory therapists, nutritionist, and social worker) who will explain the condition, map out a CF care strategy, and discuss resources.

Some children will need baseline lab work or X-rays at the first visit, depending on their age. Throat cultures are obtained four to six times per year. Newborns are seen very frequently until a feeding regimen, enzyme dosage, and weight gain are established.

Hospitalizations may be required for complications, such as intestinal blockage or pulmonary exacerbations. Ongoing medical treatment tries to keep these to a minimum, and address them in the outpatient setting whenever possible.

Managing Cystic Fibrosis

Management of CF centers on nutrition and lung health.

Maintaining normal weight is very important, as it has been shown to be linked to lung health. A person with CF needs to take in adequate calories. This is sometimes hard to achieve without the use of nutritional supplements or other means, such as a feeding tube. You may also need to take in more salt, especially if you are sweating. An appropriate enzyme and vitamin dose is also very important in the management of CF.

People with CF usually need to do daily airway clearance therapy (ACT) and may need to use inhaled medications or even antibiotics when necessary. It is also important to exercise and stay active for your lung health. You should also make sure any respiratory tract infections are treated promptly. If you think you have an infection, call your doctor, so you can receive medication.

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