What is Cystic Fibrosis

There are over 30,000 individuals in the U.S and 70,000 worldwide that suffer from cystic fibrosis (CF), Physicians diagnose about 1,000 new cases each year.

CF impacts the cells in the body that make sweat, digestive fluids and mucus. Normally, these are very slippery and then systems that keep the body working smoothly. However, if you have CF, they become thick and glue. Causing these funds to black tubes and ducts within the body.
People with CF have chronic and persistent lung infections that make it difficult to breathe over time.

Over time, mucus accumulates inside your airways; This makes breathing a struggle. The mucous traps germs and leads to infection. IT can also cause severe lung damage like a cyst (fluid-filled sacs) and fibrosis (scar tissue). That is how CF got its name.

Symptoms of CF

People with CF have several symptoms, Including;

  • Very Salty -tasting skin
  • Non-stop coughing, at times generating phlegm
  • Frequent lung infections including bronchitis and pneumonia
  • Poor growth or weight gain in spite of a healthy appetite
  • Wheezing or shortness of breath
  • Frequent greasy, or bulky stools, difficulty with bowel movement
  • Male infertility

What Causes Cystic Fibrosis?
It is not contagious; It is produced by a deviation (change) within a single gene described as the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR,) This controls the flow of salt and fluids in and out of your cells. If the CFTR gene does not work as it should, glutinous mucus mounts up throughout your body.
To get CF, you have to receive a mutated copy of the gene from both your parents.
If you only inherit one copy, you will not exhibit any symptoms, But you will be a carrier for the disease. That indicates there’s a possibility you could pass it on to your child one day.
About 10 million Americans are CF carriers. Every time two CF carriers have a baby, there is a 25% (1 and 4) chance that their baby will be born with Cystic Fibrosis. There is a 50% chance the child will be a carrier but not have CF. There is a 25% chance that the child will not carrier CF gene or have CF.

Which Portions of the body does CF affect?
The lung is not the only part of the body that is affected by CF. CF also impacts the Liver, Pancreas, small intestines, large intestine,
and kidneys, Bladder and Reproductive organs.
People with Cystic Fibrosis have the greater risk of developing lung infection because of sticky mucus build up, creates an environment in which germs can thrive and grow. Lung infections are caused mostly by bacteria and tend to be a very serious problem.

  • Liver: If the tubes that transport bile become plugged, your liver get inflamed, and severe scarring or cirrhosis occurs.
  • Pancreas: The thick mucus caused by CF block ducts in your pancreas. This stops digestive enzymes (proteins that break down your food) from reaching your intestine; as a result, your body has a difficult time absorbing the nutrients it needs. Over time this can also lead to diabetes.
    Small intestine- Since breaking down high-acid foods that come from the stomach is difficult, the lining of the small intestine can start to erode.
  • Large intestine: Thincscreations (liquids) in the stomach can make feces very thick. This can trigger blockages, IN some cases the intestine may also start to fold in on itself like an accordion in disorder called intussusception.
  • Bladder: Chronic coughing weakens the bladder muscles, Almost 65 of omen with CF suffer from stress incontinence” This means that you leak urine when you sneeze, cough, laugh or try to lift something, Although this is more common in women, it can occur in men.
  • Kidneys: Some people with CF get kidneys stones. These small, hard mineral deposits can generate nausea, vomiting, and pain. If ignored, they can lead to a kidney infection.
  • Reproductive organs: surplus mucus affects fertility in both men and women. Most men with CF have problems with the tubes that transport their sperm, or what;s called the vasa differentia. Women with CF have very thick cervical mucus, which can make it harder for sperm to fertilize an egg.
  • Other parts of the body: Cystic Fibrosis can also lead to thinning of the bones or osteoporosis ) and muscle weakness. Since CF upsets the balance of minerals in the blood, it can also produce lower blood pressure, fatigue, a fast heart rate and overall feeling of weakness.

Treatments for Cystic Fibrosis:
The type of severity of CF symptoms can differ based on the person. So it’s important to have a treatment plan that contains elements that are unique to each’s situation.
People with CG should work closely with their medical providers and families to create personalized treatment plans.
Look to the CF Foundation, which accredits more than 120 care centers that are staffed by dedicated experts in CF to help care for patients through specialized disease management.

Each day, people with CF typically have a combination of the following therapies:

  • Airway clearance to help loosen and clear away the thick mucus that can build up in the lungs, Some airway clearance techniques may require some help from family, friends or respiratory therapist. Many people with CF utilize an inflatable vest that is worn on the chest and vibrates at a high pulse to help loosen and thin mucus.
  • Inhaled medicines to open the airway or thin the mucus. These are liquid drugs that are made into a mist or aerosol and the inhaled through a nebulizing. Tese medicines include antibiotics to fight lung infection and therapies to help the airways clear.
  • Pancreatic enzyme supplement capsule to help improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF will usually take multivitamins.

Although CF is a serious condition that requires daily care, many treatments for it have advanced. People who have CF live much longer than they use to and the quality of life have improved too.