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Gardner, J. (2007). What you need to know about cystic fibrosis. Nursing 2007, p. 52-55.

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1. The nurse performs a sweat chloride test on a patient and the result was 72 mEq/L. This is an indicator that the patient has:
a. Diaphoresis
b. COPD
c. Cystic Fibrosis
d. GERD

2. When doing chest physiotherapy including postural drainage and percussion, how long should each section of the lungs be percussed?
a. 1-3 minutes
b. 3-5 minutes
c. 8-10 minutes
d. 10-15 minutes

3. Can Cystic Fibrosis be cured?
a. Yes
b. No

4. Patients with Cystic Fibrosis should increase what in their diet?
a. Sugar
b. Protein
c. Fat
d. Salt

5. Cystic Fibrosis affects the following systems: (select all that apply)
a. Respiratory
b. Cardiac
c. Reproductive
e. Gastrointestinal
f. Endocrine

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Encourage your female patients that are considering pregnancy to get tested to see if they are a carrier of the mutated gene.

Let parents know that children tend to bruise easily due to their inability to absorb fat-soluble vitamins.

Encourage Cystic Fibrosis patients to include daily exercise in their routine to stimulate coughing.

Teach your patients and their parents about support groups

Watch for signs and symptoms of the other complications that can arise with Cystic Fibrosis.

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Respiratory problems

Hemothorax, pneumothorax or even atelectasis can occur.

Gastroesophageal reflux disease
Pancreatic problems

Blocked pancreatic ducts can lead to pancreatitis.

Intestinal obstruction
Diabetes
Reproductive problems

Most men are sterile due to the mucus blocking sperm in the vas deferens.

Women may experience difficulty getting pregnant the traditional way. Optional ways are artificial insemination and in vitro fertilization.

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Acapella airway clearance device.  How to use by Aryia

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Aryia is 10 years old and has Cystic Fibrosis. She is demonstrating how she blows into her Acapella device to move mucus up and out of her lungs.

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The goal of treatments are to help patients with Cystic Fibrosis cough up all the mucus. There are four ways to to this.
1. Chest Physiotherapy - done twice a day, morning and evening.
2. Postural Drainage and Percussion - each lobe of the lungs needs to be percussed for 3-5 minutes. This can be done by a parent or a nurse if they are hospitalized.
3. Positive Expiratory Pressure - uses a breathing devise with a special expiratory valve that creates a positive pressure in the airways while exhaling.
4. Flutter Mucus Clearance Device - a small handheld plastic pipe with a mouthpiece and a stainless-steel ball inside.
5. Surgery may be necessary to remove a bowel obstruction.
A lung transplant could be an option but it is not very successful. 90% survive one year, and 50% survive at least five years.
For the GI system:

Lactulose (prevents constipation)

Ursodeoxycholic Acid - used to treat and possibly prevent progressive liver disease.

For the Respiratory System:

Bronchodilators used before chest physiotherapy.

Dornase Alfa (Pulmozyme) - improve pulmonary function and decrease risk of infection.

Aerosol antibiotics (gentamicin, ticarcillin, tobramycin) - given via nebulizer after chest physiotherapy

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This video describes the required materials, setup, and technique to do bubble positive expiratory pressure (Bubble PEP.)

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Men and women are equally affected however, more white people get Cystic Fibrosis then other races. 40% of these patients in the United States are 18 or older. 80% of Cystic Fibrosis patients are diagnosed under age 3, as early as 2 days old.

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Blood testing that looks for an elevated serum level of the pancreatic enzyme trypsinogen, especially in newborns.

Sweat chloride test which simulates the production of sweat, then measures the amount of sweat electrolytes. Normal is less than 40 mEq/L and greater than 60 mEq/L would indicate Cystic Fibrosis.

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Cystic Fibrosis is a genetic disease. This leads to the body producing to much mucus that is really thick and unwavering. It not only effects the respiratory system but also the GI and reproductive as well.

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In younger people you can expect to see:

clubbing of the fingers and toes

cyanosis

chronic purulent discharge from the nose

recurrent episodes of bronchitis and bronchopneumonia

chronic weight and nutritional problems