Cystic Fibrosis, Research Paper Example

Cystic fibrosis is a disease that affects the exocrine glands, that includes the glands responsible for mucus and sweat formation. Persons who are diagnosed with cystic fibrosis inherit two mutated genes, one from each parent. However, the parents who transmit the disease to the child might not necessarily suffer from the disease itself. From a genetic point of view, there are several potential scenarios, including a diagnosis of cystic fibrosis, serving as a carrier, or no genetic prevalence of the condition at all (American Lung Association, 2011).

This disease primarily affects lungs, intestines, sinuses, sex organs, and the pancreas. The signs and symptoms differ from individual to individual, but two of the early warning signs are that a baby’s skin tastes salty when kissed, and there are difficulties in passing a stool after birth. Other signs and symptoms include diarrhea, liver disease, and severe constipation, amongst others (Government, 2010).

From a statistical perspective, cystic fibrosis is identified in approximately 1,000 new patients on an annual basis, with the large majority of these patients being diagnosed before the age of two (Cystic Fibrosis Foundation, 2011). Although medical research has expanded the life span of many cystic fibrosis patients to their mid 30s, the condition still remains largely difficult to treat, and no cure has been discovered to date (Cystic Fibrosis Foundation, 2011). In addition, “The steady rise of the median predicted age of survival suggests how improvements in treatment and care are advancing the lives of those with CF. In 1955, children with CF were not expected to live long enough to attend grade school. Today, thanks to continued Foundation-supported research and specialized care, an increasing number of people with cystic fibrosis are living into adulthood and leading healthier lives that include careers, marriage and families of their own” (Cystic Fibrosis Foundation, 2011). Therefore, with proper physician oversight and nursing care, it is likely that patients suffering from cystic fibrosis have a much greater chance of improving the quality of their lives for a longer period of time. This requires an understanding of the depth of a patient’s condition and the level of care that is required to ensure that these efforts are maximized to ensure effective treatment outcomes for as long as possible (Cystic Fibrosis Foundation, 2011).

Cystic fibrosis is a highly complex condition that dramatically reduces the quality of life and life span for many patients. Typically, “The thick, sticky mucus produced by a person with CF blocks the airways in the lungs. This makes breathing difficult. Bacteria grows in the mucus, leading to life-threatening lung infections that can damage the lungs. The mucus also clogs the pancreas, which prevents normal digestion and leads to malnutrition” (American Lung Association, 2011). This is a critical factor in promoting an effective clinical understanding of cystic fibrosis, since patients with this condition face significant challenges in their efforts to be successful in managing the condition and its progression (American Lung Association, 2011). For many patients who do not survive cystic fibrosis, the primary cause of death is typically some form of respiratory failure, as breathing becomes impossible in the disease’s late stages (American Lung Association, 2011).

Cystic fibrosis causes nutrition complications because it increases the body’s  requirements for nutrients required in energy formation, development and maintenance. In addition, the disease makes it be difficult for the patient to obtain these nutrients in their body due to its significant impact on the digestive process. Both children and adults suffering from cystic fibrosis must consume a diet rich in a high calorie, high fat content foods along with vitamin supplements and pancreatic enzymes, which are required in the facilitation of digestion (Springhouse, 2007).

To boost the digestive system patients must take pancreatic enzyme supplements. These enzymes will aid in the rapid digestion of foods and the absorption of nutrients. The cystic fibrosis patient requires food rich in high calories for the following reasons: 1) they need additional energy to assist them in breathing; 2) large amounts of energy are wasted in fighting primary symptoms, such as fever; 3) patients often experience digestion problems which lead to a lack of absorption of nutrients into the body. For example, a child suffering from this disease should consume 120 to 150 percent more calories compared to a healthy child without the disease (Government, 2010).

Patients suffering from cystic fibrosis also have tremendously high protein requirements. This is due to the fact that protein is useful in energy production, growth of body tissues and maintenance, and proteins are also responsible in strengthening the body’s immune system. Proteins sources may include milk, beans, eggs, fish, and meat, amongst others. In addition, these patients need to take foods rich in vitamins, or should alternatively take vitamin supplements to increase vitamin absorption in their bodies (Springhouse, 2007).

Another nutrient requirement for cystic fibrosis patients is salt, because large amounts of sodium are lost during sweating. The primary source of this nutrient is obtained from the consumption of sodium chloride in almost every type of food.

Cystic fibrosis patients should avoid foods that require a lengthy absorption period in the digestive tract. This is due to the fact that the disease causes digestion complication in most patients, resulting from the lack of proper functioning of the pancreas, small intestines, and other organs responsible for the digestion process. Cystic fibrosis patients should avoid foods rich in saturated fat because fat digests very slowly. However, with the introduction of enzyme supplements, this is less of a problem because these enzymes speed up the digestion of fats and assist in fast absorption of nutrients (Government, 2010).

From a physiological perspective, it is important to recognize that cystic fibrosis creates mucus buildup in the lungs that essentially suffocates after a period of time and does not allow a patient to breathe effectively (PubMed Health, 2011). In this context, the condition leads to the inevitable problems associated with a severe decline in lung function and digestion on a gradual basis (PubMedHealth, 2011). The lack of lung function and tissue destruction leads is likely to occur because oxygen cannot freely flow to organ cells and tissues, thereby leading to the degradation and destruction over time (PubMed Health, 2011). Therefore, as systems begin to break down, the quality of life of these patients gradually deteriorates, thereby leading to other physical challenges for this patient population (PubMed Health, 2011). Therefore, the primary consideration of nursing care must incorporate a variety of strategies to support improving the quality of life for these patients (PubMed Health, 2011). With cystic fibrosis, the outcomes are generally consistent; however, how the disease presents within different patients is unique in each case (Medline Plus, 2011). Therefore, additional considerations must be made that will be effective in treating these patients according to their specific level of disease progression (Medline Plus, 2011).

The primary goal of nurses working with cystic fibrosis patients is to help these patients manage their health condition with less difficulty. The nurse care plan to the patient includes three components: medication, nutrition, and exercise. The nurse should always ensure that the patient is receiving the required medication to alleviate some of the common symptoms. Secondly, another important consideration is diet, as nurses should ensure that patients consume the most nutrient-rich diet, putting into consideration their age and the stage of disease progression. Finally, a nurse care plan should incorporate exercises into a patient’s recovery program. In conclusion, nurses play a significant role in supporting the treatment of cystic fibrosis, primarily aimed at assisting the patient in management of significant symptoms (Springhouse, 2007).

In a general context, there must be a high level quality of care associated with the treatment of patients with cystic fibrosis. Since their life spans are generally short compared to healthy individuals, it is important for nurses to maximize this level of care and to demonstrate an effective capacity to make adjustments to the care plan as needed. In a study conducted by Kerem, Conway, Elborn, and Heijerman (2005), cystic fibrosis and a subsequent reduced quality of life requires nurses to provide a holistic level of care that is based upon the ability to expand the level of patient care that is provided to this patient population. It is important to provide patients with a comprehensive level of care that is based upon consistent evaluations and symptom monitoring to ensure that patient care is not compromised in any way. It is known that “standards of care define the optimal service provision necessary to deliver the best outcomes possible for patients” (Kerem et.al, 2005); however, many standards of care are not uniform and consistent with the needs of this population group (Kerem et.al, 2005). Therefore, additional measures must be considered to ensure that patients are protected from unnecessary risks during the course of their treatment, which could be compromised when standards are not consistent (Kerem et.al, 2005).

In order for cystic fibrosis treatment to be deemed successful, it is important to note the following: “Cystic fibrosis is a demanding disease to manage for both the patient and the CF Team. Patient and family advocacy is one of the most important roles for the specialist nurse. Patient wellbeing and satisfaction are a particular focus in nursing care and successful advocacy will help achieve this” (Kerem et.al, 2005, p. 9). From this perspective, it is important to determine how to best approach this patient population to ensure that their needs are met, while also considering how nurses might integrate new concepts of care that will improve patient quality of life for this difficult condition (Kerem et.al, 2005). By providing a multifaceted plan of care, patients are likely to experience improved quality of life and the desired level of care for their remaining years (Kerem et.al, 2005).

From an adult perspective, providing a high quality level of care for patients with cystic fibrosis is essential to the provision of improving their lives, given the challenges and limitations that they face. One of the key considerations to make is that cystic fibrosis is a debilitating and terminal disease, yet many adults are living longer and longer as a result of effective treatment and provisions of care (Yankaskas et.al, 2004). Therefore, it is important to recognize these opportunities and the different methods of improving care for the adult patient population to ensure that their care is maximized at all levels (Yankaskas et.al, 2004). It is known that “The health-care needs of adults with CF are considerable.² Each year, they make an average of 4.7 CF clinic visits, experience 1.5 acute exacerbations, and are admitted to the hospital 1.0 times. In response to the increasing numbers of adults with CF, the number of adult CF programs has grown from < 10 in 1992 to > 79 in 2002…People who receive diagnoses of CF as adults may be overwhelmed by the implications of a disease that leads to premature death for many children and young adults. It is important for the CF care team to educate such patients about the disease. In particular, they should be informed that patients who receive diagnoses in adulthood often have much better prognoses than patients who receive diagnoses during early childhood” (Yankaskas et.al, 2004). In this context, it is important to recognize the value of effective nursing care and treatment for adult cystic fibrosis treatments, and to support their course of treatment from the beginning until the end (Yankaskas et.al, 2004).  This is a critical time to consider the different approaches to care and treatment that will be most effective for adult patients, and for nurses to take the steps that are necessary to improve the quality of care that is provided to them (Yankaskas et.al, 2004). These efforts are instrumental in shaping how to best manage the condition and the reality of its symptoms and the prognosis (Yankaskas et.al, 2004).

Nursing care and treatment for cystic fibrosis must emphasize improving the quality of life of this patient population, while also considering other factors which may be effective in treating the condition and in supporting patients in their struggle. It is necessary for nurses to recognize not only the physical burdens of cystic fibrosis, but also the toll that the disease takes on patients with respect to their emotional and psychological wellbeing. Therefore, treatment methods must be consistent with improving these different areas of the chosen plan of care to ensure that patients are treated as best as possible.

References

American Lung Association, Cystic Fibrosis. Retrieved November 4, 2011 from http://www.lungusa.org/lungdisease/cysticfibrosis/?gclid=CNCVjtKutKwCFQIDQAodCi49Hg

Cystic Fibrosis Foundation, What you need to know-What is Cystic Fibrosis? Retrieved November 4, 2011 from http://www.cff.org/AboutCF/?gclid=CLKRiI2vtKwCFYbrKgodinIXIw

Government ( 2010, August 13). Cystic Fibrosis. Retrieved October 14, 2011, from Medecinenet.com: http://www.medicinenet.com/cystic_fibrosis/article.htm

Kerem, E., Conway, S., Elborn, S., and Heijerman, H. (2005). Standards of care for patients with cystic fibrosis: a European consensus. Journal of Cystic Fibrosis, 4: 7-26.

Pub Med Health  (2011).  Cystic fibrosis. Retrieved October 31, 2011 from http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/

Springhouse (2007). Visual nursing: a guide to diseases, skills, and treatments. New York: Lippincott Williams & Wilkins.

U.S. National library of medicine (2011). Cystic Fibrosis. Retrieved November 4, 2011 from, http://vsearch.nlm.nih.gov/vivisimo/cgi-bin/query-meta?query=cystic+fibrosis&v%3Aproject=nlm-main-website

Yankaskas, J.R., Marshall, B.C., Sufian, B., Simon, R.H., and Rodman, D. (2004). Cystic fibrosis adult care: consensus conference report. Chest, 125.1(Suppl1S): 1S-39S.