116). This point is also made by Goozner (2004) who suggests, "Even when a genetic flaw causes disease, it doesn't automatically mean that it can be treated by replacing the defective or missing protein with its biotechnologically created equivalent. Cystic fibrosis is the classic example" (p. 30).
The treatment protocols that are currently in use are therefore designed to address the immediate symptoms of the condition, which in many cases also require some truly aggressive therapy. For example, according to Schubert and Murphy, "Those who are pancreatic insufficient rely on the frequent ingestion of enzyme supplements (often as many as 40 pills per day) to aid digestion and reduce stomach cramping, the use of dietary supplements to aid in weight gain, and, for some, the increased use of supplemental feeding tubes" (Schubert & Murphy, p. 36). The Mayo Clinic reports that still other current treatments for cystic fibrosis include the use of antibiotics to control infections, mucus-thinning drugs and bronchodilators to make clearing of the lungs easier, bronchial airway drainage, and analgesics (Treatments and drugs, 2008).
According to the clinicians at the Mayo Clinic, since the genetic mutation that causes the disease was identified in 1989, researchers have been seeking to find ways to insert copies of the normal gene into cells of the respiratory tract but there have been some profound constraints involved. As these researchers emphasize, "The challenge has been to find a reliable way to deliver the normal genetic material to affected cells that line the airways. Several methods have been developed as delivery systems, including using modified viruses, fat capsules (liposomes) and synthetic vectors. Clinical trials are under way to test the effectiveness of these delivery systems" (Treatments and drugs, p. 3). Likewise, Reid points out that, "Although scientists have made considerable progress, early detection and treatments for cystic fibrosis does not always guarantee the patient will live a long normal life. However, researchers are constantly searching for new and improved methods to complement or replace current therapies in an attempt to at least improve the patient's quality of life" (p. 28). According to Drotar, "Major advances in the diagnosis and treatment of the disease have been made over the past two decades, significantly increasing life expectancy to a median age of 32. However, reduction in the mortality of CF patients has been based on arduous, time-consuming treatment regimens prescribed two to four times a day depending on illness severity" (p. 383). Not surprisingly, a number of researchers cite a low adherence rate for compliance...
Because cystic fibrosis is a genetic disease, researchers have increasingly sought gene-replacement therapies that can cure rather than treat the disease. Unfortunately, the research was also consistent in emphasizing that such a cure remains a distant goal that is perhaps decades away. In the meantime, clinicians recommend a multidisciplinary approach that embraces a multifaceted treatment protocol for the wide range of symptoms that are typically associated with the disease, and these techniques have provided many CF sufferers with a longer and better life. The cumulative effects of cystic fibrosis, though, tend to make such symptomatic treatments less effective over time, and opportunistic infections remain the primary cause of premature death among this population. While the race for a cure continues, though, the enormously debilitating effects of cystic fibrosis on the individual as well as the other family members involved means that the incidence of CF is not restricted to CF patients alone, but rather extends to those around them as well in ways that can adversely affect the quality of life for all concerned. Therefore, it just makes good sense to extend future research directions into how family members can provide the levels of support needed for CF sufferers while protecting themselves from the burnout that is typically associated with the provision of such demanding day-to-day healthcare needs for others.
References
Drotar, D. (2000). Promoting adherence to medical treatment in chronic childhood illness: Concepts, methods, and interventions. Mahwah, NJ: Lawrence Erlbaum Associates.
Goozner, M. (2004). The $800 million pill: The truth behind the cost of new drugs. Berkeley, CA: University of California Press.
Levinson, R. & Reiss, M.J. (2003). Key issues in bioethics: A guide for teachers. New York: RoutledgeFalmer.
Reid, C.D. (1996, October). Probing the pancreas. FDA Consumer, 30(8), 27-28.
Schubert, J.D. & Murphy, M. (2005). The struggle to breathe: Living at life expectancy with cystic fibrosis. The Oral History Review, 32(1), 35-36.
Treatment and drugs. (2008). The Mayo Clinic. [Online]. Available: http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=treatments-and-drugs.
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