Cystic Fibrosis

By Nese, Emma, & Jordan

What is Cystic Fibrosis you ask?

A condition that affects breathing and digestion.

It’s caused by very thick mucus that builds up in the body. CF is inherited. You have to inherit a gene change for CF from both parents to have CF. If you inherit the gene change from just one parent, you have the gene change for CF, but you don’t have the condition.

NOW I KNOW WHAT YOU ARE THINKING...

How do you prevent getting cystic fibrosis?

Sadly, preventing CF is not possible. In babies with two abnormal CF genes, the disease is already present at birth in some organs, such as the pancreas and liver, but develops only after birth in the lungs. Someday, gene therapy may be used to prevent the lung disease from developing.

How & when is is diagnosed?

The most common test for CF is called the sweat test. It measures the amount of salt (sodium chloride) in the sweat. In this test, an area of the skin (usually the forearm) is made to sweat by using a chemical called pilocarpine and applying a mild electric current. To collect the sweat, the area is covered with a gauze pad or filter paper and wrapped in plastic. After 30 to 40 minutes, the plastic is removed, and the sweat collected in the pad or paper is analyzed. Higher than normal amounts of sodium and chloride suggest that the person has cystic fibrosis.

What types of symptoms or complications will the child have?

they will lose excessive amounts of salt when they sweat. This can upset the balance of minerals in the blood, which may cause abnormal heart rhythms.

Going into shock is also a risk.

Mucus in CF patients is very thick and accumulates in the intestines and lungs.

The result:

~malnutrition

~poor growth

~frequent respiratory infections

~breathing difficulties

~eventually permanent lung damage.

Lung disease is the usual cause of death in most patients. CF can cause various other medical problems.

These include:

~sinusitis

~nasal polyps

~clubbing

~pneumothorax

~hemoptysis (coughing of blood)

~cor pulmonale

~abdominal pain

~discomfort

~gassiness

~rectal prolapse.

Liver disease, diabetes, inflammation of the pancreas, and gallstones also occur in some people with CF.

Is this a curable or treatable birth defect?

Since CF is a genetic disease, the only way to prevent or cure it would be with gene therapy at an early age. Ideally, gene therapy could repair or replace the defective gene. Another option for treatment would be to give a person with CF the active form of the protein product that is scarce or missing. The best that doctors can do is to ease the symptoms of CF or slow the progress of the disease so the patient’s quality of life is improved. This is achieved by antibiotic therapy combined with treatments to clear the thick mucus from the lungs.


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