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Cystic Fibrosis: Defining the Illness and Its Treatment

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This paper discusses cystic fibrosis. It considers whether the use of pharmacological method is a promising approach for curing cystic fibrosis by responding to the following questions: What causes cystic fibrosis? What’s the pathophysiology of this chronic disorder? What’s the prognosis of this disease?

Definition, Incidence, and Prevalence

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Cystic fibrosis is a hereditary disorder that affects the respiratory system and the digestive system. It involves production of abnormal thick mucus that leads to blockage of both the lungs and the pancreas. According to Sanders and Fink (2016), prevalence of cystic fibrosis is at least 30,000 people in the United States and approximately 1,000 incidence cases are reported each year.

Etiology

Cystic fibrosis is a genetic hereditary disease. It is passed from parents to offspring as either dominant or recessive genes. Offspring that receive the dominant genes, phenotypically express the disease while those that have the recessive genes are carriers of the disease. According to a study by Morteza, Mandana, Amir and Leila (2017), cystic fibrosis is caused by chromosomal mutations on the cystic fibrosis transmembrane conductance regulator (CTTR) gene. These mutations occur during the prophase stage of the mitosis cell division. During the mutation, 3bp chromosome is deleted in the 10th exon resulting in loss of phenylalanine amino acid. Absence of this amino acid affects the type of mucus produced by the body. The body produces mucus that is extremely thick and sticky which can result in chronic disorder (Morteza et al., 2017).

Normal Anatomy and Physiology

In a normal healthy person, both the lungs and the pancreas lack the excessively thick mucus. In the lungs, the respiratory tract remains clear with no obstruction. This increases the amount of air inhaled and exhaled from the lungs. Therefore, gaseous exchange in the lungs is optimized. Also, respiratory cilia work best in absence of thick mucus. These cilia sweep pathogens and dust particles out of the lungs ensuring the lungs are pathogen free. In the pancreas, the pancreatic duct delivers pancreatic enzyme into the duodenum in absence of the thick mucus. This enzyme is then used to digest carbohydrates, fats and proteins. Digestion of these compounds ensures the body maximumly absorbs and benefits from the food ingested.

Pathophysiology

Cystic fibrosis causes functional changes in both the lungs and the pancreas. Presence of extremely thick and sticky mucus results in both blockage of the pancreatic duct and obstruction of the lungs’ airway. According to a study by Kulkarni, Kansra and Karande (2019), the cystic fibrosis transmembrane conductance regulator (CTTR) helps in transportation of ions such as chloride and bicarbonate across the cell membrane. When these CTTR proteins are denatured due to genetic mutation, ionic balance of salts inside and outside a cell is affected. This imbalance leads to production of the extremely thick and sticky mucus in the lungs and pancreas (Kulkarni, Kansra and Karande, 2019). Sticky mucus in the lungs can impair gaseous exchange while in the pancreas, the amount of pancreatic enzyme transported to the duodenum is reduced.

Diagnostics

There are many tests which can be used to diagnose cystic fibrosis disorder. Some of these tests include nanoduct sweat testing and carrier testing. According to Desax, Ammann, Schoeni and Barben (2008), nanoduct sweat testing measures elevated sweat chloride levels in body sweat. Higher levels of chloride above normal range usually confirm cystic fibrosis. However, this method is ineffective when used in young babies because they may not sweat enough. Thus, babies usually need more than one test to confirm cystic fibrosis (Desax et al., 2008). On the other hand, carrier testing checks genetic composition being passed down from parents to offspring. These genes can either be homozygous or heterozygous depending on the parents’ genetic composition.

Manifestations

Signs and symptoms of cystic fibrosis are manifested differently in the early, middle and late stages of the disease process. According to Alma (2019), the early stages of cystic fibrosis are manifested by: salty sweat, constant coughing, weight gain, thick phlegm, and smelly stools that are greasy and pale colored. With time, cystic fibrosis progresses into the middle stage. This stage is manifested by lung infections, weight loss, poor growth and respiratory problems. During these two stages, the body deteriorates due to breathing difficulties and digestion problems (Alma, 2019). This leads to progression of the disease into the late stage. According to a study by Veras and Groninger (2013), the late stages of cystic fibrosis are more associated with the damage inflicted on the body organs. This damage includes cysts, abscesses, fibrosis of lungs and airway, obstruction of pancreatic duct and dyspnea (Veras and Groninger, 2013).

Disease Management

Cystic fibrosis can be managed by use of pharmacological and nonpharmacological methods. Pharmacological methods use medications to manage this disease. Examples of medications used include antibiotics, anti-inflammatory, bronchodilators, pancreatic enzymes, mucolytics and CTTR modulators (Sharma, 2019). Nonpharmacological methods manage cystic fibrosis without any use of medications. Examples of nonpharmacological methods used include regular exercising to improve lung functioning, high calories and protein intake to provide body with enough nutrients and massage to ease breathing difficulties. Also, anti-inflammatory oils can be used in aromatherapy to manage this disease.

Complications of Disease and Treatment

Complications can arise either due to disease process or treatment used in managing cystic fibrosis. During the disease process, complications arise because of severe damages inflicted on the body organs by the disease. According to Sharma (2019), cystic fibrosis causes complications such as bronchiectasis, atelectasis, pneumothorax, hemoptysis, pancreatitis and end-stage lung disease. These complications vary in different individuals because of how CFTR genes mutate (Sharma, 2019). Moreover, both pharmacological and nonpharmacological treatments can cause complications. Pharmacological use of medications such as antibiotics can result in mutation of the bacteria in the lungs. This can cause the bacteria to be drug resistant. Nonpharmacological method of exercising can cause fatigue when overdone. Fatigue affects breathing patterns thus interfering with the gaseous exchange in the lungs.

Treatment Outcome Desired

The overall goal of treating cystic fibrosis is to ensure maintenance of good health in patients living with the disease. This is achieved by slowing down the rate at which the disease deteriorates. Using antibiotics and mucus-thinning medications help in achieving this desired outcome. Antibiotics reduce infections in the lungs while mucus-thinning medications keep the airway free of obstruction. Thus, normal gaseous exchange in the lungs is maintained. Besides, using pancreatic enzyme therapy increases the quantity of digestive enzymes available to digest carbohydrates, fats and proteins. Consequently, the body maximumly digests and absorbs the required nutrients hence promoting good health and wellness in patients living with the disease.

Prognosis

The disease process of cystic fibrosis is different when it’s treated from when it’s untreated. When treated, the rate at which cystic fibrosis worsens from one stage to the next stage is decreased. Also, normal body physiological processes such as gaseous exchange and digestion are maintained. Thus, treated patients live longer and healthier than untreated patients. Untreated patients have a shorter lifespan because of decreased body immunity, increased lung infections, and increased pancreas complications. According to Henderson (2017), cystic fibrosis patients undergoing treatment live approximately 37.5 years while those without treatment live approximately 10 years.

Professional Resources

To gain deeper knowledge about cystic fibrosis, it is advisable to go to web-based resources. These resources include American Lung Association and National Heart, Lung, and Blood Institute (NHLBI). American Lung Association can be accessed by going to: https://www.lung.org/lung-health-and-diseases/lung-disease-lookup/cystic-fibrosis/. Likewise, NHLBI can be accessed by going to https://www.nhlbi.nih.gov/health-topics/cystic-fibrosis. These two resources provide primary care providers with comprehensive information about cystic fibrosis. This information comprises of causes, treatment, risk factors, diagnosis, signs and symptoms of the disease. By use of this extensive information, primary care providers improve on the quality of care offered to patients.

Client Education

Educating clients about the disease enables them to make informed decisions. When patients are educated about their care and potential treatment options, they are empowered to improve their health status. Some of the resources which can be used include Cystic Fibrosis Source (CFS) and Cystic Fibrosis Foundation (CFF). Both sources provide clients with information on how to maintain good health when down with the disease. This can be achieved by exercising regularly, eating nutritious diet and drinking a lot of fluids.

Concepts

Cystic fibrosis affects oxygenation, digestion and reproduction processes in the body. Oxygenation is affected when thick mucus obstructs the airway passage in both the bronchioles and lungs. Hence, the body’s oxygenation level decreases because of reduced gaseous exchange in the lungs. Also, digestion is affected because of reduced amount of pancreatic enzymes produced due to clogging of the pancreatic duct. Consequently, nutrients aren’t completely digested. Lastly, cystic fibrosis affects reproduction. Women with cystic fibrosis have thicker vaginal mucus that hinder sperms movement.

Conclusion

Cystic fibrosis cannot be cured by used of pharmacological methods. However, it can be easily managed if detected in early stages of life. It is highly recommended newborn babies to be screened for the disease.

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