Term Paper: Cystic Fibrosis. There Are Eleven

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[. . .] On the cellular level, cystic fibrosis patients find a "particular cell protein can not do its job and fight off bacterial infection. A genetic error deletes a single amino acid, which is just one of 15-hundred that make up the protein, causing the protein to become misshapen and preventing it from traveling to the cell's surface to begin its infection fighting work (http://www.hopkinsmedicine.org/healthnewsfeed/hnf_1053.htm)." Researchers have discovered a gene which makes the protein that "transports chloride ions, one of salt's two components, in and out of cells. Because the movement of sodium ions, salt's other component, is naturally linked to the transport of chloride, the protein mutated in cystic fibrosis regulates sodium transport indirectly (Damaris)."

Cystic fibrosis affects the "epithelium, the layer of cells that lines the passages in the body's organs. Normally, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues, however in cystic fibrosis suffers, the thick mucus does not move easily, clogging passages in many organs (http://kidshealth.org/teen/diseases_conditions/digestive/cystic_fibrosis.html)." Tissues are damaged when the chemical makeup of the mucus needed to protect them is changed, causing blockage in the airways and ducts (http://www.healingwell.com/library/cysticfibrosis/info1.asp).

Understanding Sodium and Cystic Fibrosis

The abnormal ion transport system of sodium is thought to attribute to the thick mucus seen in cystic fibrosis sufferers, as well as preventing them from properly removing the mucus from their lungs. Researchers suspect that the "normal protein, called cystic fibrosis transmembrane conductance regulator (CFTR), may also directly or indirectly regulate the passage of various other molecules into and out of cells (Damaris)."

The atypical CFTR protein can not "properly regulate sodium transport by lung cells, allowing the cells to keep drawing in sodium - and water, pulled by osmotic pressure, follows. The liquid layer coating the lungs thins as its water is absorbed by cells, preventing cilia from beating effectively, resulting in mucus clogging the lungs (Damaris)." Cystic fibrosis patients are unable to generate productive coughs due to a reduction in this lubrication.

There are different theories concerning sodium and treatment therapies. One theory suggests that "adding salt to airways will pull water out of cells and increase the depth of the liquid layer that underlies mucus, which would boost a patient's rearing of mucus. A conflicting older theory suggests that the abnormal chloride channel in cystic fibrosis results in airway-surface fluid loaded with salt (Damaris)."

One study found that "defensins, some of the compounds the body uses to kill off invading microbes, are less effective in salty solutions, and that adding salt could be harmful, while reducing the concentration of salt in airway fluid would help defensins attack infections (Damaris)."

New Theory

There is currently a new theory concerning the pathology of cystic fibrosis. Researchers at the University of New Mexico in Albuquerque suggest a link between "abnormally acidic conditions inside cystic fibrosis lung cells to the ease which bacteria can grow and live inside the respiratory tract. All cells attach sugars to proteins on their surface, and these sugars serve as molecular flags to identify cells. The cystic fibrosis lung cells attach some atypical sugars, which P. aeruginosa and other bacteria can use as footholds while they attack the lung cells (Damaris)."

Treatments

There is currently no cure for cystic fibrosis, however there are a number of treatments available to patients to help alleviate their suffering and hopefully prolong their lives.

The principal treatments for cystic fibrosis sufferers are "antibiotic therapy for pulmonary exacerbations, and chronic suppressive therapy, airway clearance and exercise, therapy with mucolytic agents, bronchodilators, and anti-inflammatory agents, supplemental oxygen, and nutritional support, which are similar for children and adults. Because of the potential complexity, all aspects of the medical regimen should be reviewed on a regular basis with an assessment of adherence and potential side effects from medications (David)."

While researchers, sufferers and their families all agree that the foremost goal of all research is to find a cure, they stress the importance of providing comforting treatment in the meantime. One new treatment that is currently in a trial phase is the drug - choloroquine. The drug's primary use has been for the treatment of malaria, with few side effects noted. Researchers are hopeful that the drug, "when inhaled, will lower the acidity of the lungs, thus preventing bacteria from gaining a foothold (Damaris)."

Some drugs used to treat cystic fibrosis, such as steroids, can have adverse side effects such as causing diabetes and osteoporosis. In an effort to reduce these side effects by lowering the medication doses, "researchers have devised a way of encapsulating the drugs inside a patient's own red blood cells, and since there is no peak of concentration, the patient always has a low but clinically relevant dose that can be one-tenth to one-twentieth the oral dose (unknown)." In a recent study, ten cystic fibrosis patients had blood removed for a clinical trial. The red blood cells were isolated and placed in a "dilute saline solution that makes the cells porous, permitting a synthetic steroid to diffuse into the cells. The steroid-beating cells were then reinjected into their donors (unknown)." When researchers checked the patient's blood a month later, they found "low but clinically effective concentrations of the drug present (unknown)."

Functioning in the World patient with cystic fibrosis will find it hard to lead a basically normal life. While most people take breathing for granted and never really concentrate on the act, those suffering from cystic fibrosis can find themselves fighting for every breath.

Patients with cystic fibrosis are not able to reproduce like the general population. In 98-99% of men with cystic fibrosis diagnosed prior to adulthood, the abnormalities of the vas deferens render them sterile. While women suffering from the condition have a "normal reproductive anatomy, they are less fertile than women without the disease (David)."

While adults with cystic fibrosis will find there are "few absolute restrictions on choice of employment, present and future physical limitations should be considered (David)."

Prognosis

Cystic fibrosis is a fatal disease with no known cure currently available. The prognosis of the length of survival depends on the "severity of cystic fibrosis, which is determined largely by how much the lungs are affected (http://www.cysticfibrosis.com/info/index.html)."

While "deterioration is inevitable, leading to debility and eventually death, the outlook has improved steadily over the past 25 years, mainly because treatments can now postpone some of the changes that occur in the lungs (http://www.cysticfibrosis.com/info/index.html)." Currently, there is a life expectancy of greater than 28 years in half of all cystic fibrosis sufferers. The "long-term survival rate is somewhat better in males, people who don't have pancreatic problems, and people whose initial symptoms are restricted to the digestive system. Despite their many problems, people with cystic fibrosis usually attend school or work until shortly before death (http://www.cysticfibrosis.com/info/index.html)."

Conclusion

Cystic fibrosis is a fatal disease which prevents its sufferers from leading completely normal lives due to conditions such as difficulty breathing and failure to adsorb nutrients properly. Researchers are constantly looking for new therapies which can ease symptoms, prolong life, and have hopes of eventually curing this debilitating disease.

Works Cited

Family Guide to Cystic Fibrosis. (accessed 14 July 2004). http://www.phd.msu.edu/DNA/cf_fam.pdf).

Andolsek, Kathryn M. A review of approaches to diagnosis of cystic fibrosis. American Family Physician. (1997): 01 June.

Chromosomal Patterns of Inheritance. (accessed 14 July 2004). http://med.usd.edu/som/genetics/curriculum/1DCHROM2.htm).

Cystic Fibrosis. (accessed 14 July 2004). http://kidshealth.org/teen/diseases_conditions/digestive/cystic_fibrosis.html).

A cystic fibrosis.com. (accessed 13 July 2004). http://www.cysticfibrosis.com/info/index.html).

Damaris, Christensen. The persistent problem of cystic fibrosis: why are people with this disease plagued by lung infections. Science News. (2002): 26 January.

David, Rodman. Cystic fibrosis adult care*: consensus conference report. Chest.

2004): 01 January.

The Cellular Problem Behind Cystic Fibrosis. (accessed 14 July 2004). http://www.hopkinsmedicine.org/healthnewsfeed/hnf_1053.htm).

Tsui, Lap-Chee. Cystic fibrosis: genotypic and phenotypic variations. Annual Review of Genetics. (1995): 01 January.

Unknown. Shuttling medicines via blood cells. Science News. (2002): 15 June.

What is Cystic Fibrosis. (accessed 14 July 2004). http://www.healingwell.com/library/cysticfibrosis/info1.asp). [END OF PREVIEW]

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