MINI MED SCHOOL: Cystic Fibrosis

Objectives:

At the end of this class, you will be able to:

1. describe cystic fibrosis,

2. discuss the signs of cystic fibrosis, and

3. relate the treatment of this disorder.

Introduction

Cystic fibrosis is a very serious disease that is genetic and it runs in families. This disease causes mucus and secretions to build up and then clog up the lungs and other organs of the body, like the pancreas. The lungs are necessary for sending oxygen to all parts of the body and the body’s cells. The pancreas is one of the organs of the body that is necessary to digest the food we eat.

It is a chronic disease. There is no cure. Even a lung transplant will not help because the defective gene is always in the body.

About 30,000 children and adults in the United States have it and about 70,000 people throughout the world have it. Today, people with this illness can live into their 40s and beyond. In the past, many children with it died while they were still in grade school.

About 1,000 new cases are diagnosed every year
Most (70%) of children with CF are diagnosed with it by the time they reach the age of 2

The Signs of Cystic Fibrosis (CF)

When mucus clogs the lungs, these things happen:

· the person has trouble breathing

· germs enter the lungs and they multiple and build up to the point that an infection can occur

· a low amount on oxygen gets to the body and its cells

· permanent lung damage

These things can happen when this thick mucus clogs the pancreas.

· food is not broken down and digested in the right way because the needed enzymes are not working

Some of the signs of CF are:

· a lot of coughing and coughing spells

· lung infections that occur often

· shortness of breath

· wheezing

· slow or poor growth even though the child has a good appetite

· salty tasting skin

· trouble moving the bowels

· bulky, frequent stools

What Causes Cystic Fibrosis?

Cystic fibrosis is a disease that people get from both of their parents because the genes and DNA from the parents are passed on to the baby. Both parents must pass this gene to the child. The child will not get CF if only one parent has the gene.

About 1 in every 31 people in the United States has the CF gene. It is most common among the white, Caucasian race but it can be found in all other races as well.

How Is the Child Diagnosed?

Newborns and babies before the age of 3 are given a screening test to find out if they have CF. The test that is used is called the “sweat test”. A high level of chloride in the sweat tells the doctor that the baby has CF. A genetic test can also be done by looking for the gene in cells that are taken from the inside of the cheek.

When the baby is diagnosed with CF, treatment begins. Early treatment helps the child to improve their lung function, improve their growth and to avoid infections and having to be in the hospital.

What Can Be Done to Prevent it?

More than 10 million Americans carry the defective gene that can be passed down to any children that they have. Many people get genetic testing before they have any children so they can find out if they can pass it on to their children.

Every time there are two carriers of the CF gene, the chances are:

25% that the child will have cystic fibrosis
50% that the child will carry the CF gene but not have cystic fibrosis
25% that the child will not carry the gene and not have cystic fibrosis

Some parents may decide to adopt a child, rather than take a chance when they both carry the gene. Others decide to go ahead with the pregnancy.

How is CF Treated ?

There is no cure for cystic fibrosis at this time but there is hope that there will be a cure in the future. Now, we use special care, medicine, and good nutrition to treat CF.

People with CF take a pancreatic digestive pill with every meal and snack so that the food is broken down and digested in the correct manner. They also take a multiple vitamin to make sure that their nutrition state is the best that it can be. The person will also do some airway clearing exercise at least one time a day. These exercises loosen the lung mucus so it can be coughed up. Vibrations, or tapping, on the back of the person may be done. They may also use a respiratory drug to keep the airway open and clear.

There are several drugs that are used to help people with CF. They are:

· Bronchodilators. These drugs open up the person’s airway so that they are able to breathe better and cough up mucus.

· Antibiotics. These drugs stop the growth of germs that can lead to serious lung infections like pneumonia.

· Mucus thinners. These drugs make the mucus thinner so that the person is better able to cough it up.

· Anti-inflammatories. These drugs lower swelling of the body’s tissues, including the lungs, when they are affected.

Airway Clearance Techniques (ACTs)

There are several kinds of treatments that are used to clear the airways of people who have CF. They are:

· Coughing

· Huffing.

· Chest PT or postural drainage and percussion or tapping

· Oscillating PEP

· High frequency chest wall oscillation

· Positive expiratory pressure (PEP)

· Active cycle of breathing exercises

· Autogenic drainage

Coughing is the most basic of all these techniques. It clears the mucus up with a person’s own high-speed airflow. Huffing is a kind of cough. The person takes a deep breath in and then exhales in an active way. A mirror will steam up and fog if a person does huffing with a mirror in front of them. It does not involve as much force as a cough and it is less tiring to the person.

For chest physical therapy and postural drainage and percussion, the person lies in different positions while the body is tapped in order to allow mucus to loosen and drain from the different parts of the lungs. This method uses gravity to drain the mucus.

Oscillating positive expiratory pressure (PEP) is also used to clear the airway of the person with CF. The person blows through a tube all the way out several times. This action vibrates the person’s airway so that the mucus can be moved around and coughed up.

High-frequency chest wall oscillation is also called the “vest”. For this method, the person puts on a vest that inflates and is hooked up to a machine that vibrates the chest. Again, the person coughs or huffs during the treatment to get the mucus up and out of the lungs.

Positive expiratory pressure (PEP) pushes air into the person’s lungs so that the airway is kept open. The person wears a mask or a mouthpiece and breathes normally as the air is pushed into the lungs with the machine.

Active cycle of breathing exercises vary according to the needs of the person. Some of these techniques are:

Breathing control. The person relaxes their upper chest and does normal, gentle breathing with the lower part of the chest.
Thoracic expansion exercises. The person takes in deep breaths and hold it in for a couple of seconds. This can be done with chest clapping or vibrating. At the end of this exercise, the person does breathing control.
Forced expiration technique. The person huffs with different lengths of time and then they do breathing control.

Autogenic drainage is “self-drainage.” The mucus moves from smaller to the larger airways. It has three parts:

Dislodging mucus
Collecting mucus
Clearing mucus

Summary

Cystic fibrosis is a chronic disorder that is inherited and without a cure. Good care and treatment is necessary to help the person with cystic fibrosis to have a good quality of life.

References

Cystic Fibrosis Foundation. (2009). “What is Cystic Fibrosis?” [online}. http://www.cff.org/AboutCF/Testing/

Hockenberry, Marilyn J. and David Wilson. (2010).Wong's Essentials of Pediatric Nursing. 8th Edition. Elsevier Mosby.

Nettina, Sandra M. (2009). The Lippincott Manual of Nursing Practice. 7th Ed. Lippincott, Williams and Wilkins.

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