Improvements in care make it possible for people with cystic fibrosis to live longer and fuller lives. If you or your child has CF, it might help to know what daily life looks like when you’re living with cystic fibrosis. Please help raise awareness about Cystic Fibrosis by sharing this blog.
CF changes the way the body produces mucus. This thicker mucus causes problems with breathing and digestion, such as lung infections and malnutrition.
There is not yet a cure for cystic fibrosis, but there is reason for hope. We are now able to diagnose CF in infancy, and improved treatments have changed the way people manage their symptoms and live with CF.
What causes Cystic Fibrosis?
Cystic fibrosis is a genetic disease, meaning it is something some people are born with and it isn’t contagious. An abnormal CF gene is carried by 1 out of 20 people in the United States. If two people with an abnormal CF gene have a child together, there is a 1 in 4 chance of that child being born with a cystic fibrosis transmembrane conductance regulator (CFTR) protein. The CFTR protein changes the way the way water and salt move through the body. This causes thicker mucus to be produced, which causes problems by blocking pathways in the body’s organs.
There was a time when someone born with CF was unlikely to live beyond infancy. But advances in early diagnosis and treatment have made cystic fibrosis life expectancy increase drastically. The average life expectancy is now about 37 years, and it continues to rise. Speaking with your doctor about your health and your specific CF mutation can give you an idea of what to expect.
People with CF have more opportunities today than ever before. They go to school, have jobs, and raise families. Everyone’s life is different, but here is a look at what an adult with CF might expect in their daily life.
Cystic Fibrosis & Eating Right
CF damages the digestive system over time, making it harder for the body to absorb nutrients from food. Enzyme capsules taken with every meal and snack make a huge difference, but you still have to be deliberate about how you eat.
An average person with CF needs twice the calories and protein as other people to maintain a healthy weight. By keeping snacks on hand and eating healthy, calorie-rich foods, you can give your body the energy it needs to keep you healthy. Your doctor or nutritionist will help you develop a diet plan that works for you, and they will help you know which vitamins or supplements you need.
Getting enough calories can be tough. We have some tips on having a healthy diet with CF, Cystic Fibrosis Diet.
Cystic Fibrosis & Staying Hydrated
Increasing levels of salt in the body makes a person without CF feel thirsty when they get dehydrated. But CF causes extra salt to escape the body through sweat, so you can get dehydrated without realizing it.
You should always have liquid on hand. Water is always a healthy option, but energy drinks are a great way to rehydrate. There are even energy drink made specifically for people with CF. These drinks are a quick way to stay hydrated while getting some of the nutrients and calories you need.
A big part of living with CF is keeping your airway clear of mucus that makes it difficult to breathe. Getting mucus out of the body helps keep the lungs working and is part of preventing dangerous infections. Medications help to open up the airway and thin out mucus to make it easier to deal with.
Along with medication, there are many treatment options for clearing the lungs. These treatments take time but are worth it for how much they improve your quality of life.
- Huffing, a gentler alternative to coughing. It’s a similar to action to fogging up a window with your breath.
- High-frequency Chest Wall Oscillation, or the Vest. An inflatable vest is vibrated to shake loose mucus from the lungs.
- Postural drainage and percussion (PD&P), also called Chest Physical Therapy (CPT or Chest PT). Clapping on the back, chest, and sides of a person with CF while they are in specific positions helps gravity get mucus out of the body.
Some people are surprised to find out how important working out is for a person with CF. Exercise is a great way to keep the body healthy, especially the lungs, and to clear out the airway. Running and bicycling are two activities that can keep you in shape and be enjoyed throughout life. Speak with your doctor about finding an exercise routine that works for you.
There are many medicines that help manage the symptoms of cystic fibrosis. Being consistent about taking your medication is essential for daily living with CF. Keeping track of every pill and breathing treatment can be challenging, but it will make an enormous difference in how you feel.
Here is a list of some medications a person with CF can expect to need:
- Enzyme capsules to help with digestion
- Vitamins to get enough nutrients
- Bronchodilators to help airflow into the lungs
- Mucolytics or deoxyribonuclease (DNase) to break down mucus
- Antibiotics to combat infection
Many of the medications used for CF are inhaled with a nebulizer during a breathing treatments. This takes time each day, but improvements are continuing to be made to make the treatments shorter. For instance, Cayston is an antibiotic taken in three treatments throughout the day, with each treatment lasting five minutes or less.
Most people take precautions to avoid getting sick, but people with CF have to be even more deliberate. Viruses and bacteria that wouldn’t hurt the majority of people can lead to a serious illness for a person with CF. You work have to work hard to stay healthy.
People with CF need to wash their hands regularly and have easy access to antibacterial hand cleaners. It’s important to stay at least 6 feet away from anyone who is sick. Doing these simple things every day can help you avoid getting sick.
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