Living with Cystic Fibrosis
Jasmine Melville
Simpson University
Living with Cystic Fibrosis
Imagine knowingly living everyday with something that will most likely kill you before you reach the age of forty. The feeling of needing to carefully make the most of everyday, and to meticulously manage a medication regimen at the same time, are two feelings very familiar to someone who suffers from Cystic Fibrosis (CF). This inherited disorder is defined by the Mayo Clinic as causing, “severe damage to the lungs, digestive system and other organs in the body” (Staff, 2016). Although broad and effective this definition does not encompass all that it means to be diagnosed with, live with, and manage Cystic Fibrosis.
Cystic Fibrosis is most widely known for the devastating effects it has on one’s lungs and respiratory system. The effects are found in the respiratory system because CF causes thickening and hardening of mucus that is normally thin and slippery. However, mucus that is normally thin is also found in other areas of the body such as, the pancreas, gastrointestinal tract, and reproductive organs, all of which can be negatively affected by CF. This hardening and thickening of mucus is due to an inherited mutation found on the transmembrane conductance regulator gene (CFTR), this causes an abnormality in cyclic adenosine monophosphate (cAMP) (Sharma, 2016). Together these irregularities physically translate into an increased amount of sodium and water reabsorption across epithelial cells which results in thin mucus to become hard and sticky (Sharma, 2016).
This destructive and chronic disease is routinely screened for in the United States, and all newborns are subjected to an Immunoreactive Trypsinogen (IRT) blood test to provide early diagnosis and subsequent treatment if needed. Another common test for CF is the chloride sweat test which aims to see if the patient’s sweat is saltier than normal, which can indicate a positive CF test.
After being diagnosed, the patient is quickly immerged into a world of medication regimens, and since CF has no cure, these medications are lifelong. The diversity of medications treat multiple aspects of CF, they include, antibiotics to treat inevitable lung infections, anti-inflammatory medications, mucolytics to promote secretion clearance, inhaled bronchodilators to improve airway diameter, and pancreatic enzymes to support absorption of nutrients in the digestive tract (Staff, 2016). Although these medications help curb the symptoms experienced by CF patients, the medications themselves have side effects and negative aspects to them that can create other health issues.
These patients receive pancreatic enzymes to promote the digestion of dietary fats, carbohydrates, and proteins in an otherwise compromised gastrointestinal tract (Pancrelipase, 2017). Even this simple medication has multiple side effects, one of which is fibrosing colonopathy. This is side effect is a disorder that causes severe abdominal pain, constipation, and nausea and vomiting. These side effects of course have medications that can be taken to treat them, some of which have more side effects. This specific medication does not have many contraindications other than hypersensitivity to hog proteins since these enzymes are procured in swine. Another medication that is important in improving the lives of those who suffer with CF is a mucolytic such as Acetylcysteine. Mucolytics that are inhaled lower the viscosity of mucus in the airways by, degrading mucus and allowing for easier expectoration.
The most promising area of treatment in CF patients is a double lung transplant and it also holds the most potential for complications. Those with CF seeking a lung transplant must meet criteria in three areas, medical, psychological, and financial (Going Through a Evaluation, 2017). During the medical examination, the patient’s organ systems are evaluated for proper function. The psychological criteria evaluate the candidates coping mechanisms, ability to abstain from ingesting intoxicating substances, ability to maintain a medication schedule, and support system (Going Through an Evaluation, 2017). The financial evaluation helps the candidate evaluate what insurance will cover and how to afford things that insurance will not cover (Going Through an Evaluation, 2017). During this arduous process, the applicant can be deemed unqualified for a lung transplant at any time. If found to be qualified for a lung transplant, the candidate is added to a registry list to wait for an unpredictable amount of time.
Emerging studies are beginning to show the benefits of practicing yoga for those who suffer from CF. Although yoga does not specifically help thin mucus or make mucus mobilization easier, it does treat something that most sufferers from CF experience, anxiety. In a study published in the Evidence-Based Complementary and Alternative Medicine journal, patients diagnosed and being treated for CF, experienced, “a notable decrease in immediate anxiety from prior to and just after yoga therapy sessions” (McNamara et al., 2016). This study also showed that joint paint was improved. From this, researches deemed that yoga should be considered a possible alternative therapy to work in conjunction with contemporary medical management.
Frequent hospitalization can be a familiar aspect of life to patients who suffer from CF. Hospitalization is often due to exacerbated respiratory symptoms, lung infections, or other complications caused by to CF. During hospitalization, the CF patient may be able to receive the same nurse if they are familiar with how that nurse treats them, and their family. These patients are often better versed in their chronic diagnosis than the nurse and it is important to employ a nursing theory such as Jean Watson’s Carative Factors to every aspect of care. Special attention should be given to the patients psychosocial, and spiritual well-being. To be attentive to the psychosocial well-being of a patient with CF, Watson’s Carative factor stating, “Promotion and acceptance of the expression of positive and negative feelings” can be used (Wagner, 2010). Patients suffering from CF will have days where they experience acceptance of their disease and other days of anger toward their chronic illness that causes them an immeasurable amount of pain and mental struggle. Therapeutic communication and quality time spent with the patient will help them process through these feelings and will help them feel validated in their happiness and in their fears. This will also help the family support the patient in their journey and the nurse can provide support and validation for the family as well. A carative factor that addresses the spiritual well-being of a patient with CF is, “Allowance for existential- phenomenological forces” (Wagner, 2010). This factor allows space for the unknown to be discussed in an atmosphere of hope and acceptance of miracles. In allowing patients and families to discuss the scary parts of the unknown, provides perspective for all involved to appreciate their life and the lives of the people that surround them.
Cystic Fibrosis is a chronic disease that affects all parts of the patient’s life. Each day they are awakened to take medications that have multiple side effects, and hope to get through the day without needing to be admitted to the hospital. Hope revolves around advancing treatment therapies including lung transplants, which add years to an unfairly shortened life. But this treatment comes with high risks and the possibilities of life threatening complications. Alternative therapies show a way to mitigate anxiety and depression that often riddle the life of someone living with a debilitating chronic illness. Nurses provide a place where these aspects of care meet. Technological advances in treatment must be given a human aspect to have a positive outcome. It is the role of the nurse to provide care that is holistic as well as evidenced based to provide the best care possible to these patients.
Concept Map: Cystic Fibrosis
References
Sharma, G. (2017, August 24). Cystic Fibrosis. Retrieved September 25, 2017, from http://emedicine.medscape.com/article/1001602-overview
Staff, M. (2016, October 13). Cystic fibrosis. Retrieved September 25, 2017, from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/home/ovc-20211890
McNamara, C., Johnson, M., Read, L., Velden, H., Thygeson, M., Liu, M., . . . McNamara, J. (2016, December 19). Yoga Therapy in Children with Cystic Fibrosis Decreases Immediate Anxiety and Joint Pain. Retrieved September 25, 2017, from https://www.hindawi.com/journals/ecam/2016/9429504/
Pancrelipase (Oral Route) Precautions. (2017, March 01). Retrieved September 25, 2017, from http://www.mayoclinic.org/drugs-supplements/pancrelipase-oral-route/precautions/drg- 20065293
Wagner, A. (2010). © 2010 Watson Caring Science Institute Page 7 Assembled by A.L. Wagner. Retrieved September 25, 2017, from https://www.watsoncaringscience.org/files/Cohort%206/watsons-theory-of-human- caring-core-concepts-and-evolution-to-caritas-processes-handout.pdf