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WHAT IT IS

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system, and other organs.

CAUSES

CFTR cells produce a protein that functions as an ion channel on cell surfaces, primarily allowing chloride ions to pass through. This process helps draw water outside the cell, thinning mucus and making it easier for the body to function by making it slippery. In Cystic Fibrosis, a mutation in CFTR cells prevents this from happening, forcing the mucus to stay sticky and thick.

Although cystic fibrosis is progressive and requires daily care, people with CF are usually able to attend school and work

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There are two types of Cystic Fibrosis:

The one that affects multiple organs, is called Classic Cystic Fibrosis, and it's usually diagnosed in the first years of life.

The other one is called Atypical Cystic Fibrosis, and it could probably only affect one of your organs, the symptoms are not consistent, and it's usually diagnosed later in your life.

The Symptoms of Cystic Fibrosis:

Trouble Breathing.

Slow physical growth.

Nagging coughs.

Sinus infections.

Breathing problems.

Lung Infections, like pneumonia or bronchitis.

The Symptoms of Atypical Cystic Fibrosis:

Dehydration.

Heatstroke due to strange electrolyte.

levels.

Pancreatitis.

Extreme Weight loss.

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How is cystic fibrosis (CF) treated?

You'll probably have a healthcare team that includes a specialist in cystic fibrosis and many other types of caregivers. There is no cure for cystic fibrosis, but your team will help you manage the disease. The major focus of management is keeping your airways clear. Your provider will also prescribe medicine when needed.

Keeping airways clear

You can help to keep your airways clear if you have cystic fibrosis in a number of ways:

You can learn special ways of coughing and breathing.

You can use devices that fit into your mouth or therapy vests that rely on vibrations to loosen mucus.

You can learn something called chest physical therapy, also known as postural drainage and percussion to loosen mucus. With this method, you move into certain positions so that your lungs can drain. Another person claps their hand on your chest and/or your back to help loosen the mucus. You might combine this with coughing.

Medications for cystic fibrosis

Your provider may prescribe these medicines, which won't cure CF, but which will help you in certain situations. They include:

Antibiotics to treat lung infections or prevent them.

Inhaled bronchodilators to make breathing easier by opening and relaxing your airways.

Inhaled medicine to make mucus thinner and easier to get rid of.

Anti-inflammatory drugs, including steroids and non-steroidal anti-inflammatories.

Medications to treat the cause of cystic fibrosis in people with certain gene variants.

Pancreatic enzymes to aid in digestion.

Stool softeners to help with constipation.

Surgeries for cystic fibrosis

You may need surgery for cystic fibrosis or one of its complications. These might include:

Surgery on your nose or sinuses.

Bowel surgery to remove blockages.

Transplant surgery, including a double lung transplant or a liver transplant.

Why is a high-calorie, high-fat diet needed for people with cystic fibrosis (CF)?

People with cystic fibrosis have nutritional needs that aren't the same as the needs of people without CF. People with CF may need 1.5 to 2 times the number of calories as people without CF. You need the extra calories if you have CF because you use more energy than other people to breathe, fight lung infections and maintain your strength.

You also need more calories and fat because cystic fibrosis stops the digestive enzymes made by your pancreas from working completely. This means nutrients and fats from foods aren't fully absorbed by your intestines.

Although the enzyme capsules that are taken before all meals and snacks helps digest fats, proteins and starches, a certain amount of nutrients and fats don't get absorbed. If your body doesn't absorb enough fats, then fat-soluble vitamins aren't being fully absorbed either, and these vitamins are needed to protect the lungs.

It's also important to stress that people with cystic fibrosis should keep a higher than normal weight starting in early childhood. Researchers have shown that young people with CF who maintain a higher weight grow faster and taller up to puberty and again grow taller when they hit their growth spurt at puberty.

Young people with CF who started life at a lower weight did not grow as many inches, started puberty at a later age and never got that same puberty growth spurt. Reaching your full genetic potential - getting as tall as possible with lungs as large as possible - is another reason why higher-than-normal weight in young people with CF is so important.

Another common misbelief is that salt (sodium) is unhealthy for all people. This isn't true for children and adults with CF. People with CF lose a lot of salt in their sweat. Although there's not a set standard, healthcare providers generally tell people with CF to eat salty foods. This is true especially during hot, humid weather and exercise. If you have CF, you can probably add salt to meals and snacks as desired. Ask your provider or a registered dietician about the amount of salt you need each day.

What are the complications of cystic fibrosis (CF)?

The complications of CF include the following:

Adults who have CF can have problems with breathing, digestion and their reproductive organs.

The thick mucus present in people who have CF can hold bacteria, which can lead to more infections.

People who have CF have a higher risk of developing diabetes or the bone-thinning conditions like osteopenia and osteoporosis.

Men who have CF are not able to father children without the aid of alternative reproductive technology. Women who have CF can have a decrease in fertility (the ability to have children) and complications in pregnancy.

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Cystic Fibrosis (CF): Causes, Symptoms, Diagnosis & Treatment

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Cystic fibrosis is an inherited disease that causes sticky mucus to build up in organs like the lungs and the pancreas. People with CF need high-fat, high-calorie foods.

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There’s no cure for cystic fibrosis. But it can be managed, it is a disease and its symptoms can be extreme but a cystic fibrosis specialist and other healthcare team can help keep the patient comfortable.

Management involves:

Keeping airways clear and open with breathing techniques and devices to loosen mucus.

Medicines can also be used to help correct issues with protein imbalance like the regulator (CFTR) protein

Ensuring you get enough of the right kinds of calories from food.

Avoiding smoke, dust, dirt, household chemicals, and mold.

There is no contact with other CF patients who are not part of the family ( this can cause the exchange of infections).

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https://youtu.be/3NNY6Tb3vQ4?si=eIanREU3eLbeJUMh

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cystic fibrosis explanation of cause video - Google Search

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Gene. From each parent to develop cystic fibrosis. If they only inherit one copy from one parent.MoreGene. From each parent to develop cystic fibrosis. If they only inherit one copy from one parent. They won’t develop. It. However they will be a carrier of that mutated gene.

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Chalmers, Sarah. “Cystic Fibrosis .” Mayo Clinic, Mayo Clinic, 23 Nov. 2021, www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700.

“Cystic Fibrosis (CF): Causes, Symptoms, Diagnosis & Treatment.” Cleveland Clinic, my.clevelandclinic.org/health/diseases/9358-cystic-fibrosis#management-and-treatment

.Cystic Fibrosis: Video, Anatomy, Definition & Function | Osmosis. www.osmosis.org/learn/Cystic_fibrosis.

“Everything You Need to Know about Cystic Fibrosis | Everyday Health.” EverydayHealth.com, 2 July 2019, www.everydayhealth.com/cystic-fibrosis/guide/.

“What Is Cystic Fibrosis? | Duke Health.” Www.youtube.com,www.youtube.com/watch?v=3NNY6Tb3vQ4. Accessed 28 Oct. 2023.