What is Cystic Fibrosis (CF)?

What is Cystic Fibrosis (CF)?

Cystic Fibrosis is an inherited chronic disease that causes the lungs and digestive system to become clogged with thick, sticky mucus. The mucus makes it difficult to breathe, eat, or fight certain infections because the body is unable to cough up or swallow this excess mucus. Cystic Fibrosis can result in serious health problems including lung damage and infections.

Symptoms and Treatment Options for Cystic Fibrosis

The primary symptom of Cystic Fibrosis is malabsorption of nutrients and chronic diarrhea from pancreatic insufficiency.

The severity of symptoms can be determined by the number of mutations in the CFTR gene.

Treatment options are dependent on the individual’s age or how serious their symptoms are.

In children who have cystic fibrosis, treatment includes antibiotics and chest physical therapy to maintain lung function as long as possible before a lung transplant. In adults who have cystic fibrosis, treatment includes intravenous antibiotics for lung infections and a high-calorie diet for weight gain.

Respiratory signs and symptoms

Cystic Fibrosis is a rare genetic disorder that can affect many organs, including the lungs, liver and pancreas. The symptoms of Cystic Fibrosis typically worsen over time.

Symptoms may include:

  • frequent lung infections due to mucus buildup in the lungs
  • wheezing, coughing or shortness of breath
  • excess phlegm production (spit), which is sometimes green or yellow

“There are also three respiratory signs associated with CF: a prolonged cough with phlegm or mucus; trouble breathing; and persistent bronchitis.”

Digestive signs and symptoms

Since cystic fibrosis causes mucous to build up and clog the lungs and digestive tract. This results in digestive complications as a result of the buildup of mucous in the digestive tract.

Symptoms:

  • Excessive gas

  • Bloating

  • Diarrhea

  • Abdominal pain,

  • Nausea

  • Enlarged liver or spleen due to cirrhosis of the liver

 

Different Types of Cystic Fibrosis

Cystic Fibrosis is a rare, recessive genetic disease that causes problems with the lungs, digestive track, and sweat glands. There are more than 1,500 different types of cystic fibrosis. The most common types are Cystic Fibrosis Northern European (CFE), and Cystic Fibrosis Southern European (CFS). These two account for 50% of all cases worldwide.

The difference between Cystic Fibrosis Northern European vs. Southern European

Northern European is a genetic mutation. This mutation causes the CFTR protein to not form correctly, which affects the body’s ability to regulate fluids and salt.

Southern European is a rare gene mutation that is most prominent in people of southern European ancestry. This mutation causes an individual to have less than half the normal amount of the protein cystic fibrosis transmembrane conductance regulator (CFTR) in their body. Cystic fibrosis Southern European can lead to other symptoms, such as infant breathing problems, digestive complications, and lung infections.

What causes CF?

Cystic fibrosis is a genetic disease that affects the lungs, pancreas and digestive system of someone with the condition. Cystic fibrosis is caused by a mutation in a gene called CFTR, which encodes for a protein that regulates salt and water balance in cells.

Children need to inherit one copy of the gene from each parent in order to have the disease. If they inherit two copies, they will develop cystic fibrosis and not be able to pass it on. But if they only inherit one copy they can become carriers which means that their descendants may also develop it.

How to diagnose CF?

There are many ways to diagnose Cystic Fibrosis with a genetic test. However, this process can be expensive and invasive. The most common way to diagnose Cystic Fibrosis is a sweat test that detects the abnormal sodium level in the sweat of people with the disease.

The best way to prevent Cystic Fibrosis is by having children tested for it before they are born.

Diet for CF

Adults with cystic fibrosis often have trouble absorbing fats, getting the right nutrients, or maintaining self-sustaining appetites. If you are afflicted with this condition, there are some important steps you can take to ensure your body stays nourished.”

We are all aware that there is no cure for Cystic Fibrosis yet. According to the Cystic Fibrosis Foundation, Cystic Fibrosis patients have an increased risk of developing lung disease, pancreatitis, and heart disease.

Cystic Fibrosis

 

A good diet can help keep various CF symptoms in check:

  • Protein: In order to prevent muscle loss, it is important to make sure that you are getting protein from beef, chicken, fish, eggs, or soy regularly.

  • Zinc: Foods high in zinc include kidney beans, beef, spinach, liver, eggs and seafood. These are all great for preventing infection.

  • Salt: People with cystic fibrosis have salty sweaters, which leads to an electrolyte imbalance and dehydration. Drinking salty snacks, pickles, and sports drinks can help replace the salt in your body. You can also cook vegetables in chicken broth instead of water.

  • Calcium: Foods that are rich in calcium can be beneficial for people with CF. Dairy products, such as high-fat yogurt, milk, and higher fat cheese, have all been shown to have many health benefits including reduced risk of osteoporosis.

  • Iron: Iron aids in fighting off infection-causing bacteria and provides us with red blood cells that transport oxygen around our bodies. Giving your body iron can be achieved by eating foods such as liver, tuna, turkey, seafood, eggs and beef.

  • Antioxidants: Fruits and vegetables contain antioxidants that may help fight off inflammation in your lungs. This could lead to bacterial infection, which is a serious problem for those with cystic fibrosis.

  • Fiber: Fiber helps with digestion by absorbing water and producing short-chain fatty acids that help nourish the gut lining and reduce inflammation in the body.

Please call Superior Compounding Pharmacy in Plymouth Michigan to speak with one of our licensed pharmacists today at 734-404-6065. We can help answer any medication questions that you may have.