May is Cystic Fibrosis Awareness Month
WHAT IS CYSTIC FIBROSIS (CF)?
CF is a progressive, life-shortening, genetic disease that affects the lungs, pancreas, and other organs.
There are close to 40,000 children and adults living with CF in the US, an estimated 105,000 people have been diagnosed with CF across 94 countries, and CF can affect people of every racial and ethnic group.
The patient registry in the US shows:
- There are close to 40,000 children and adults living with CF in the US
- Approximately 1,000 new cases of CF are diagnosed each year
- More than 75% of people with CF are diagnosed by age 2
- More than have of the CF population is age 18 or older
There has been significant progress in treating this disease. There still is no cure and too many lives are cut far too short.
WHAT ARE THE SYMPTOMS?
Cystic Fibrosis is a complex disease and the symptoms, and the severity can differ widely from person to person. Many different factors can affect a person’s health and the course the disease runs, including the age when you are diagnosed.
CF is not contagious. You cannot “catch” CF from someone. The defective CTFR gene causes unusually thick, sticky mucus that can clog the lungs, pancreas, and other organs. This build up can cause severe respiratory and digestive problems. It affects everyone differently and the severity varies. Some people with CF are in good health while others are severely limited and chronically ill.
Symptoms can include:
- Very salty tasting skin
- Persistent coughing, sometimes with phlegm
- Frequent lung infections including pneumonia or bronchitis
- Wheezing and shortness of breath
- Frequent coughing and clearing of mucus
- Poor growth or weight gain despite good appetite and intake
- Frequent greasy, bulky stools or difficulty with bowel movements
- Nasal polyps
- Chronic sinus infections
- Male infertility
HOW IS IT DIAGNOSED?
Cystic Fibrosis is diagnosed by the age of 2, however, some are not diagnosed until they are adults. CF diagnosis is a multistep process and should include:
- Newborn Screening
- Sweat Test
- Genetic or carrier testing
- Clinical evaluation at a CF Foundation-accredited care center
HOW IS IT MANAGED?
Because the symptoms can widely differ from person to person, treatment plans can contain many of the same elements and are tailored to each person’s unique situation. People with CF, their families, and their CF care teams work closely together to develop and maintain treatment plans that align with personal life goals and health goals.
Each day, people with CF complete a combination of the following treatments, therapies, and medication regimens:
- Airway Clearance – helps loosen and get rid of the thick, sticky mucus that builds up in the lungs.
- Inhaled Medications – opens airways or thins mucus. These are liquid medications that are made into mists or aerosols and inhaled through nebulizers. They can include antibiotics to fight lung infections as well as medications such as Albuterol, mucolytics, and corticosteroids.
- Pancreatic Enzymes – these capsules improve absorption of vital nutrients. Because CF affects the pancreatic function, absorption is a problem. The enzymes help the body absorb the nutrients from the food consumed. The enzymes are taken with every meal and snack.
- Multivitamins – potent multivitamins are also necessary to replace lost nutrients.
- CFTR Modulators – these medications target the underlying defect in the CFTR protein and work as a “corrector.” Though this is not a cure, it is significantly slowing the progression of the disease process. Because there are so many different mutations of CF, the modulators are only effective for people with specific mutations.
- Nutrition – this is very important for persons with CF. Many require a high-fat, high-calorie diet to maintain good weight gain.
- Exercise - keeping the body, especially the lungs, strong and healthy is vital in maintaining good health for CF’ers.
- Infection prevention – staying away from others (6 feet apart) with colds, flu, or any other infection is necessary to prevent infection. Frequent handwashing, not sharing utensils or drinkware and using alcohol-based hand gels frequently when in public helps to keep other’s germs from transferring.
The Cystic Fibrosis Foundation leads the way in research and development of new treatments and medications and has made great strides over the years to provide life-changing measures for the CF community. To find out more about Cystic Fibrosis, the disease process, treatments, research and how you can help support the CF community, visit www.cff.org.
*Facts and information were found at www.cff.org
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