Cystic Fibrosis

Cystic Fibrosis is one of the most common life-

threatening autosomal-recessive disorders plaguing the

world today. This paper discusses many aspects of cystic

fibrosis, from the clinical manifestations and specific

complications of the disease to the daily lifestyles of CF

patients. This paper will also cover the demographics of

patients suffering from CF; including age, race, and

gender. Information regarding treatment options and

preventative screening available for the disease will

follow.

Cystic Fibrosis is a tragic disease that affects the

respiratory, digestive, and reproductive systems. Thick,

sticky mucus is formed that inhabits the lungs,

intestines, and reproductive linings and allows an ideal

breading ground for bacteria. This over-production of mucus

causes frequent infections, breathing difficulties,

malnutrition, poor growth, and lung disease. Many CF

patients are characterized as "talking through their nose"

or having a persistent cough with no results. The

accumulated mucus is so thick that simple coughing does not

dislodge it. Daily respiratory treatments, exercise, chest

physical therapy, and vigorous percussion are some of the

therapies that help to decrease and shift mucus secretions.

My aunt, Linda Hegberg, suffered from cystic fibrosis and

my mother used to jump and pound on her back to help

dislodge the mucus. This form of vigorous percussion helped

maintain what health Linda had left in her lungs. (K.

Workman, personal interview, June 9, 2005).

CF also causes respiratory problems such as atelactesis

(collapsed lung), pneumothorax, hemoptysis (coughing up blood),

sinusitis, and nasal polyps.

In the intestinal tract, mucus can also build up and

prevent proper digestion of foodstuffs. With inadequate

digestion and limited pancreatic enzymes, the patient

suffers from malnutrition and frequent obstructions. The

pancreatic channels that normally carry important

enzymes to digest food are blocked, resulting in a limited

ability to absorb necessary nutrients and fats. The

patient cannot gain weight regardless of a hearty appetite.

When they are able to form and pass stool, it is pale to

clay-colored with an extremely foul smell. (Cystic Fibrosis,

n.d.)

A patient suffering from cystic fibrosis may also

experience abdominal pain and discomfort, gassiness, cor

pulmonale (enlargement of the right side of the heart),

rectal prolapse, gallstones, liver disease, and clubbing of

the fingers and toes. Depending on the severity of each

patient's disease, they may suffer from one or all of these

symptoms. (Cystic Fibrosis, n.d.).

A CF patient leads a life much unlike that of a normal,

healthy person. The disease carries not only physical changes,

but social and psychological. Each day brings fear, worry,

resentment, and depression; all of which adversely affect the

patient. Each day is a challenge, but the hardest to overcome

is finding the good through all the bad. For example, my aunt

Linda was diagnosed with cystic fibrosis when she was five years

old. She had to sleep in an "oxygen tent" at night, took

numerous medications daily, was sick all the time, and had to be

beaten on the back to loosen up the mucus in her lungs. She led

a confined life; she had a hard time partaking in sports and

activities because she couldn't breathe well. With every

negative aspect staring her in the face, she still remained a

happy little girl. She had a smile that could melt your heart,

a personality that intrigued, and an outlook that beat all the

odds. It was difficult, but she realized that she should spend

the time she had being happy and enjoying life. She got married

and had a beautiful son, Gregory. She passed away in March 1983

at the age of 28.

Cystic Fibrosis