About Cystic Fibrosis
Learn About The Cystic Fibrosis Symptoms and Treatments
Cystic fibrosis (CF) is a progressive, genetic disease in which the body makes mucus that is thicker and stickier than normal. This mucus clogs the tubes that carry air in and out of your lungs, as well as those that carry digestive enzymes from your pancreas to your small intestine. Because of this, CF can cause problems with both the lungs and the digestive system including:
- Frequent lung infections
- Difficulty breathing
- Problems digesting fat and proteins
- Poor nutrition and growth
More than 30,000 people in the US are living with cystic fibrosis, with an estimated 1,000 new cases diagnosed each year. The vast majority – more than 75 percent – are diagnosed by age 2 and over half of those living with CF today are 18 years of age or older.
The type and severity of cystic fibrosis symptoms can vary greatly from person to person. Because CF affects both the lungs and digestive system, a person may exhibit both respiratory and gastrointestinal symptoms such as:
- Persistent coughing accompanied by a thick mucus
- Frequent lung infections such as pneumonia or bronchitis
- Difficulty breathing, including wheezing or shortness of breath
- Exercise intolerance
- Poor growth or weight gain despite having a good appetite
- Greasy, bulky stools
- Chronic constipation
- Intestinal blockage, especially in newborns
- Male infertility
Because there is no cure for cystic fibrosis, the goal of treatment is to manage the disease, minimize symptoms, and prevent complications. Managing CF involves loosening mucus and ridding it from the lungs; preventing lung infections and intestinal blockages and treating them if they do occur; and ensuring proper nutrition.
Treatment plans for CF are based on the type and severity of a person’s symptoms. No two individuals’ symptoms are exactly alike, which is why treatments are customized to accommodate each person’s unique circumstances.
Most treatment plans, however, include some combination of the following therapies:
- Airway clearance help loosen and get rid of mucus in the lungs. This may include medication, chest physical therapy, vest therapy, and/or pulmonary rehab.
- Inhaled medicines called bronchodilators, help keep the airways open. They are inhaled through a nebulizer and may include antibiotics to treat lung infections and anti-inflammatories to decrease swelling in the airways.
- Oral pancreatic enzyme supplements are usually taken with every meal and snack to help the digestive system absorb vital nutrients. People with CF also usually take multivitamins.
- Getting regular exercise is important to help improve energy, lung function, and overall health.
- Medications are also available to target specific gene mutations. Monitoring CFTR therapy is also a critical part of the treatment plan.
Educating Providers about CF Care
The Cystic Fibrosis Institute is committed to the teaching of cystic fibrosis care to general pediatricians, family medicine physicians, medical students, nurses, respiratory therapists, and other professionals in the healthcare industry.
As faculty at the Northwestern University Feinberg School of Medicine, Dr. Steven Boas is actively training our future CF physician care providers. Formal conferences and lectures on CF are regularly delivered to local medical staffs in Chicago as well as to national audiences.
CFI sponsors a summer internship program for medical students who desire training and experience in CF care. We have assembled and created abundant educational material and resources about CF that can be found in our offices.
- Advocate Condell Medical Center
- Advocate Sherman Hospital
- Alexian Brothers Medical Center
- Ann & Robert H. Lurie Children’s Hospital of Chicago
- Edward Hospital
- NorthShore University HealthSystem
- NorthShore University HealthSystem – Evanston Hospital
- NorthShore University HealthSystem – Glenbrook Hospital
- NorthShore University HealthSystem – Highland Park Hospital
- NorthShore University HealthSystem – Skokie Hospital
- Northwest Community Hospital
- Northwestern Medicine – Lake Forest Hospital
- Northwestern Memorial Hospital
- St. Alexius Medical Center