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Eniola Adewale

MedicalNigeria.com




Newsletter

Saturday, July 22, 2017

What is Cystic Fibrosis and its significance?

Cystic fibrosis is of great significance in our world today.           

Most cases of cystic fibrosis in the UK are now identified through

laboratory screening test procedures after birthIt’s discovered that 1 in every 2,500 babies born in the UK has cystic fibrosis.


More than 30,000 people in the U.S. live with cystic fibrosis (CF.) Doctors diagnose about 1,000 new cases each year.

CF affects the cells in your body that make mucus, sweat, and digestive fluids. Normally, these are very thin and slippery to keep systems in your body running smoothly. But if you have CF, they become thick and glue-like. This blocks tubes and ducts throughout your body.
Over time, mucus builds up inside your airways. This makes breathing a struggle. The mucus traps germs and leads to infections. It can also cause severe  lung damage like cyst (fluid-filled sacs) and fibrosis (scar tissue). That’s how CF got its name.


  What Causes Cystic Fibrosis?

It’s caused by a mutation (change) in a single gene called 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 doesn’t work like it should, a sticky mucus builds up throughout your body. It’s not contagious.To get CF, you have to inherit a mutated copy of the gene from both your parents.If you only inherit one, you won’t have any symptoms. But you’ll be a “carrier” of the disease. That means there’s a chance you could pass it on to your own child one day.About 10 million Americans are CF carriers. Every time two CF carriers have a baby, there’s a 25% (1 in 4) chance that their baby will be born with CF.


Parts of the Body Does Cystic Fibrosis Affect?The lungs aren’t the only part of the body that’s harmed by CF. The disease also affects the following organs:

Pancrease: The thick mucus caused by CF blocks 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 hard time absorbing the nutrients it needs. Over time, this can also lead to diabetes.

Liver: If the tubes that remove bile become clogged, your liver gets inflamed and severe scarring (cirrhosis) occurs.

Small intestine: Because breaking down high-acid foods that come from the stomach is a challenge, the lining of the small intestine can erode.

Large intestine: Thick secretions (liquids) in your stomach can make feces (poop) very thick. This can cause blockages. In some cases, the intestine may also start to fold in on itself like an accordion (called “intussusception”).

Bladder: Chronic coughing weakens the bladder muscles. Almost 65% of women with CF have what’s called “stress incontinence.” This means that you leak urine when you cough, sneeze, laugh, or lift something. Though more common in women, men can have it, too.

kidneys: Some people with CF get kidney stones. These small, hard mineral deposits can cause nausea, vomiting, and pain. If ignored, they can lead to a kidney infection.

Reproductive organs: Excess 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 deferentia.” Women with CF have very thick cervical mucus, which can make it harder for a sperm to fertilize an egg.


Other parts of the body: CF can also lead to thinning of the bones (osteoporosis) and muscle weakness. Because it upsets the balance of minerals in the blood, it can also bring about low blood pressure, fatigue, a fast heart rate, and a general feeling of weakness.
Although CF is a severe condition that needs daily care, many treatments for it have improved. People who have CF live much longer than they used to and the quality of life has improved as well. 





Treating cystic fibrosis 
     There's currently no cure for cystic fibrosis, but it's possible to help control the symptoms, prevent or reduce complications, and make the condition easier to live with. A person with cystic fibrosis will be supported by a team of healthcare professionals at a specialist cystic fibrosis centre. A care plan will be drawn up that's tailored to their individual needs.A range of treatments may be used and sometimes treatment in hospital will be needed. Regular appointments to monitor the condition will also be recommended.Some of the main treatments for cystic fibrosis include: 

  • ·       Dietary and nutritional advice
  •     Lung transplants
  •      Medications to treat and prevent lung problems
  •      Airway clearance techniques to remove mucus from the lungs.
  •      Treatments for associated problems, such as diabetes 
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