Researching Cystic Fibrosis Disease

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Table of Contents

Summary
Causes
Treatment
Medical side effects
Cures
Gender and age range affected
Kvp and Mas for an x-ray
Position and projections used from the x-ray tube
References

Summary

Cystic fibrosis is a progressive, chronic, and fatal genetic disorder that affects the body’s mucous glands. The disease causes thick and sticky mucus to form in different body organs including the lungs, pancreas, and reproductive system. Individuals with the disease get to live for an average of 37 years. The primary body systems that cystic fibrosis effects are the digestive system and reproductive system. Statistics show that about 30,000 Americans have the disorder and that there are approximately 1000 new cases every year (Hodson, Geddes, & Bush, 2012).

Causes

Cystic fibrosis is a genetic disorder that occurs when a mutation in a gene results in changes to the protein responsible for the regulation of salt levels in the body cells. For cystic fibrosis to develop, a child must inherit the defective gene from their parent. Children born of two carriers have a 25 percent chance of having cystic fibrosis. They also have a 25 percent chance of not having the disorder and a 50 percent chance of being carriers (Hodson et al., 2012). The symptoms and severity of the disease vary from one person to another. This variation is due to the different mutations of the cystic fibrosis gene in every individual.

Research shows that cystic fibrosis is a biochemical abnormality caused by the mutation of the gene responsible for the production of a protein called cystic fibrosis transmembrane regulator (Hodson et al., 2012). This protein handles the movement of chloride ions in and out of the body cells. Abnormality of the CFTR protein results in the blockage of the circulation of water and chloride ions into the cells as well as the production of abnormal mucous (Giddings, 2009). However, CFRT abnormality is only responsible for about seventy percent to eighty percent of cystic fibrosis cases. There are more than 400 other mutations that might result in cystic fibrosis.

Treatment

There is no known cure for cystic fibrosis. However, various treatment therapies are available. These therapies aim at controlling lung infection and inflammation. They also aim at reducing the amount of mucus in the lungs and preventing intestinal blockage. Additionally, the treatments are used to improve the flow of air in and out of the lungs and to maintain high nutritional status among the patients. The treatment therapies include the use of drugs, physical therapy, surgery and nutritional therapy. Studies show that patients have over the past years benefited from treatment regimes that utilize a blend of different treatments (Giddings, 2009).

Drug therapy involves administering different classes of medicine including antibiotics, oral pancreatic enzymes, and Bronchodilators. These drugs help loosen mucus from the lungs and relax the bronchial tube muscles. The most effective drugs to treat the lung infections and inflammation associated with the disorder are antibiotics. Other drugs used include steroids, hypertonic saline, and mucolytic drugs. Physical therapy is used to dislodge a large amount of mucous in the chest and help the patient breathe with ease (Giddings, 2009). The physical therapies include the forced expiration technique and the active cycle method. The use of mechanical instruments such as tubes, masks, and vibrating vests has also become popular. Procedures used in surgical therapy include lung transplant, nasal polyp removal, and bowel surgery.

Medical side effects

The drugs used to treat cystic fibrosis include bronchodilators, oral corticosteroids, inhaled corticosteroids, antibiotics and mucolytic agents. Bronchodilators help relax the muscles around the airways. The common side effects associated with this class of drugs include a fast heartbeat, shaky hands, and headaches. Oral corticosteroids and inhaled corticosteroids act by preventing inflammation, swelling and mucus accumulation in the airways. Their side effects include fluid retention, increased appetite, hoarseness, and stomachaches. The side effects of using antibiotics are yeast infections, stomach upsets, voice changes and diarrhea (Giddings, 2009).

Cures

At the moment, there is no known cure for the disease. However, new drugs and therapies for the treatment of the illness are at different stages of development. Areas that offer hope for a cure include pharmacogenomics, nanotechnology and utilization of rDNA (Hodson et al., 2012).

Gender and age range affected

As mentioned earlier, cystic fibrosis occurs equally among females and males. However, diagnosis of the disease occurs earlier in males compared to females. One possible explanation for the late diagnosis among females is the gender-based bias among physicians and parents. Diagnosis of most cases takes place before the age of ten years. Additionally, the predicted median survival age for patients with the disorder is 37.4 years (Hodson et al., 2012). Therefore, the age range with the highest number of cystic fibrosis patients is 0 to 37 years.

Kvp and Mas for an x-ray

Bruno and Abujudeh (2012) point out that chest x-rays can be used to monitor the onset and progression of lung infections in cystic fibrosis patients. They suggest 50 mAs and 100 kVp. They state that it is important to consider lower settings for children.

Position and projections used from the x-ray tube

The position used in chest x-rays for cystic fibrosis is the conventional PA (frontal, post-anterior). The patient sits or stands in an erect position and full inspiration. The distance from the x-ray machine should be six feet (Bruno & Abujudeh, 2012).

References

Bruno, M., & Abujudeh, H. (2012). Quality and safety in radiology. New York: Oxford University Press.

Giddings, S. (2009). Cystic fibrosis. New York: Chelsea House.

Hodson, M., Geddes, D., & Bush, A. (2012). Cystic fibrosis (3rd ed.). New York: CRC Press.

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