Cystic Fibrosis Chest Physiotherapy

Most cystic fibrosis patients suffer from excessive mucus build up in the lungs and respiratory organs. This mucus is a normal substance found in every person but in patients with cystic fibrosis it becomes abnormally thick and sticky. It sticks to the lining of the passageways that go to the lungs and digestive tracts and disrupts the work of those organs.

What Is It and How Does It Work?

The thick mucus that is clinging to the linings of the air passages and lungs must be dislodged and removed. There are several ways removing the mucus. Many have been used since the disease was first diagnosed and named in the 1940’s. New treatments are being discovered every year and the average life span of a CF patient has risen from 16 to the late 30’s.

Tests for Diagnosing Cystic Fibrosis

The standard and most common test for cystic fibrosis is the sweat test. Mothers often are performing this test in a maternal way when they kiss their infant and realize they have a salty taste. The sweat test measures the chloride or sodium (salt) is in the child’s sweat.

The test is normally painless and is conducted by placing a chemical that induces sweat to a portion of the body, usually an arm or leg. Then an electrode is attached to that spot and a mild electric current are passed through the area causing that spot to sweat. The small amount of current may feel tingly or cause that area to feel warm but is harmless and weak. Sweat is collected and sent to a laboratory for analysis for the mutant gene that carries cystic fibrosis.

Sampling

Preventing Infections in Cystic Fibrosis Patients

There is no known cure for cystic fibrosis so the main purpose of treatment is to reduce the severity of the symptoms and give the patient the best possible quality of life. New antibiotics developed recently are more effective in fighting infections and killing the bacteria that causes them. Faster acting inhalers deliver the medications straight to the airways. Long-term users of antibiotics have the potential of developing a resistance to drug therapy.

The role of white blood cells in the body is to attack bacteria. When the DNA in the cells is released it makes the mucus thicker. There is a new aerosol drug that can be prescribed to breaks up the DNA into pieces and makes the mucus thinner. The new drug is called dornase alfa and it does have side effects. The drug can cause sore throat and increased irritation in the airways.

Medication

Cystic Fibrosis and Pregnancy

Scientists and researchers recently released a study showing a decline in birthrates of babies with cystic fibrosis since genetic testing became available. The general public has more knowledge and understanding of the disease that was a mystery for so long. In medieval times the people believed a baby was “bewitched” when they displayed symptoms of what we now know is cystic fibrosis.

With new research and new methods of genetic testing, many hope there will be an another reduction in the birthrate for CF babies. Cystic fibrosis in men will make them sterile, but a woman may still conceive and carry a child to full-term or near full-term.

Genetic Factors

A child cannot be born with cystic fibrosis unless both of his or her parents are carriers of the mutant gene. That gene was identified in 1988 and is called the cystic fibrosis transmembrane conductance regulator gene. It is associated with the number seven chromosomes. If only one parent is a carrier of the gene, there is a 25 percent chance their child will be a carrier for the disease.

Who Discovered Cystic Fibrosis?

Cystic fibrosis was a disease that has been around since the Middle Ages. Many infants with “salty skin” often died in infancy or young. These babies and children were considered “bewitched” because they died at such an early age.

The Swiss pediatrician Guido Fanconi first named the disease in 1936, when he refers to it as cystic fibrosis with Bronchiectasis. A scientist from Columbia University first developed a complete profile and description of cystic fibrosis symptoms.

Dr. Dorothy Hansine Anderson

Genetics and Cystic Fibrosis

Do you know if you are a carrier for the cystic fibrosis gene? If you are parents and want to start a family or are planning on having another child and are concerned because there is cystic fibrosis in your family history, consider genetic testing. Cystic fibrosis is an inherited disease a child could get if both parents carry the recessive gene that contains cystic fibrosis.

You could be a carrier without showing signs of the disease and pass it on to your child. If your spouse is not a carrier, there is still a 25 percent chance your child will also be a carrier. You have a higher risk of being a carrier of cystic fibrosis if you are a Caucasian and descendent from a family in Northern Europe. Although other ethnic groups can be affected by cystic fibrosis, they have much less risk than a Caucasian.

Risk Profile

The difference in ethnic groups who are carriers of this mutant gene are shown by these statistics:

    • Caucasians have a 1 in 29 chance of carrying the cystic fibrosis gene
    • People of Latino background have a 1 in 46 chance of carrying the gene

What Causes Cystic Fibrosis?

The Cystic Fibrosis Foundation estimates there are over 30,000 Americans with cystic fibrosis. This disease affects mostly Caucasians whose ancestors came from northern Europe. It affects all racial and ethnic groups but white Caucasians are more at risk for developing or being a carrier of the defective gene that carries cystic fibrosis.

Average lifespan of a patient diagnosed with cystic fibrosis is 30 years. New technology and scientific advances are making life better and improving predictions of life spans for cystic fibrosis patients.

Cystic fibrosis has the following signs and symptoms but will be different for each individual patient. Patients with cystic fibrosis are often diagnosed before the age of three but diagnoses have been given to teens and adults also. One of the symptoms of cystic fibrosis is a delay in the onset of puberty.