Cystic Fibrosis Research Paper

Cystic Fibrosis

About 1,000 new cases of cystic fibrosis are diagnosed each year and over 70,000 people are already diagnosed worldwide. Cystic Fibrosis is a genetic disorder that affects the lungs’ ability to secrete mucus properly. It’s gene and protein product cause the body to produce unusually thick and sticky mucus (CFF). Mucus is designed to lubricate the lungs, but the lungs of a patient with cystic fibrosis are prone to infection because of the thick mucus that traps bacteria and foreign materials. Instead of being expelled, the materials stay in the lungs and cause infections. The white blood cells die and create an even thicker substance. The mucus in the sinus cavities can also create upper respiratory infections and a nasally

voice (WebMD).
The common symptoms of a subject with cystic fibrosis include: a persistent cough that produces thick spit (sputum) and mucus, wheezing, breathlessness, a decreased ability to exercise, repeated lung infections, and inflamed nasal passages or a stuffy nose (Mayo Clinic).

This disease is usually detected early in infants before symptoms occur, but children grow up not being able to breathe properly or exercise to their full potential because of how this disease restricts their airways. The thick mucus blocks and clogs the lungs, not allowing for all of the surface area to be used for the oxygen and carbon dioxide exchange. This disease can cause other life threatening side effects like pneumothorax, enlargement of the right side of the heart which can lead to heart failure, increased pressure of the lungs, and damaged airways (WebMD). This significantly decreases the ability to breathe; both the dynamic and tidal volumes are smaller than that of a normal

person.
Diagnosis of this disease is usually done when the subject is an infant. The “sweat test” can determine whether a person has cystic fibrosis because their sodium levels are high. Most of the time, if the parent kisses their child, they can taste salt. Parents are tested before the birth of the child to see if they carry the cystic fibrosis gene. This requires only a simple blood test. Both of the parents have to have the recessive gene in order for their child to have cystic fibrosis (Mayo Clinic). If the disease is detected early, treatment can begin immediately.
Cystic fibrosis left untreated is expected to be fatal three to five years after diagnosis.

However, the FDA has approved drugs and there are other treatments available to lengthen the lifespan of the patient. There is chest physical therapy which is a form of shaking up the mucus in the lungs so it can be coughed up. Also there is a nebulized (inhaled) medication that degrades the DNA’s ability to make the sticky mucus. Other antibiotics like tobramycin, colistin, gentamicin, and ceftazidime prevent infection within the lungs and reduce inflammation. Due to the subject’s decreased ability to supply the body with oxygen, supplemental oxygen can be given. Not only is the respiratory system affected, but also the digestive system. Nutrition also needs to be monitored; it is imperative that the patient drinks enough water to promote the removal of the mucus. “Approximately 90% of patients with CF have pancreatic exocrine insufficiency and require oral pancreatic enzyme supplementation with meals”. They are given fat malabsorption in CF, so they are given an enzyme to help with digestion and secretion of the mucus. Other systems affected include the liver, intestines, reproductive tract, and the endocrine system. Cystic fibrosis was considered a fatal childhood disease, but with new medicine and technology, the expected survival age has risen to 37 years. Patients’ quality of life has also improved

(Brunner).