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Case Study Cystic Fibrosis

Cystic Fibrosis: Case Study

The patient is a 7-year-old female. Due to the fact that the patient is a minor, her mother walked into the ER with her. The mother explained that her symptoms consist of persistent coughing throughout the day and it becomes worse at night, the coughing often results in spitting out phlegm. Along with wheezing, stuffy nose, loss of appetite, pain in the abdomen, and the taste of salty skin. The patient has been up coughing and complaining about the pain for 5 hours. Since the patient’s mother had thought it was a regular cold, she had been giving her children’s Tylenol for the past 3 days, 5 mL every 4 hours. Once the patient was taken in, a physical assessment was performed. The questions that were asked to the patients mother were about family history of CF, history of bowel obstruction as an infant, stool, and eating habits (nurses labs 2018). Because of the fact that the patient’s mom mentioned her skin having a salty taste, a sweat test has been recommended to check for cystic fibrosis. The sweat test measures the amount of chloride in sweat while the genetic test detects chromosomal mutations (Very Well Health 2019). Patient has now been diagnosed with cystic fibrous and will be admitted to the hospital for further instructions.

Pathophysiology

The pathophysiology of cystic fibrosis is based on the defects in the cystic fibrosis gene, which codes for protein transmembrane conductance regulator (CFTR) that functions as a chloride channel and is regulated by cyclic adenosine monophosphate (cAMP) (Nurselabs 2018). Cystic fibrosis is often caught at birth but there are cases where people begin to develop it later in life. If it is detected as birth, many persons with cystic fibrosis acquire a lung infection which incites an inflammatory response, the infection becomes established with a distinctive bacterial flora (Nurselabs 2018). If there is a case of a person developing cystic fibrosis as they get older, there are many symptoms that come along with it and it does affect many parts of your body. It can affect your GI tract, pancreas and your sweat glands which causes the skin to taste salt like. Symptoms may include pain in the abdomen, having a chronic cough that may include blood or phlegm, any gastrointestinal problems such as diarrhea, fat in the stool, heartburn, severe constipation, or bulky stools. When it affects your respiratory system, it can cause pulmonary hypertension, shortness of breath, sinusitis, wheezing, acute bronchitis, and/or pneumonia. There might also be a delay in puberty, growth, and in development. Other commons side effects include deformity of nails, different infections, male infertility, nasal polyps or weight loss (Mayo Clinic 2020).

History

The symptoms presented by the patient include coughing up phlegm, wheezing, stuffy nose, pain in the abdomen, salty skin and loss of appetite. In the past the patient has had pneumonia and was hospitalized for a couple of days due to this condition. When the doctor said, she might be diagnosed with cystic fibrosis the patients mother was heartbroken but not in complete shock because of all the symptoms that her daughter has been having.

Physical Assessment

The patient has been wheezing and has shortness of breath, is in fact coughing with phlegm, and pain in the abdomen. Vital signs were taken as well as a skin assessment, the skin around the rectum was examined for irritation and breakdown from frequent foul stools (Nurses lab 2018). The sweat chloride test results have come in. For the sweat test the results came back positive and with 60 mmol/L of chlorine detected. The principal diagnostic test to confirm CF is a sweat chloride test using the pilocarpine iontophoresis method to collect sweat and perform a chemical analysis of its chloride content is currently considered to be the only adequately sensitive and specific type of sweat test (Nurseslab 2018).

Related treatments

There are a couple actions and medications that can be taken to help with cystic fibrosis such as diet, exercise, monitoring and vaccines. Medication wise there are pancreatic enzymes, vitamins, bronchodilators, antibiotics, mucolytic, and cystic fibrosis transmembrane conductance regulator (CFTR). Patients are monitored in the CF clinic every 2-3 months to achieve maintenance of growth and development, maintenance of as nearly normal lung function as possible, intervention and retardation of the progression of lung disease, clinical assessment to monitor gastrointestinal tract involvement, and monitoring for complications and their treatment (Nurses lab 2018).

Nursing Diagnosis and Treatment Goal

Based on the assessment the nurse’s diagnosis is ineffective airway clearance, ineffective breathing pattern, risk for infection, imbalanced nutrition: less than body requirements, anxiety, compromised family coping, and deficient knowledge of the caregiver. (Nurses lab 2018). In regards to the airway clearance it pertains to the thick mucus production, which is why the patient coughs up so much phlegm, and the breathing pattern pertains to the tracheobronchial obstruction, and the anxiety is from the stress of being in the hospital. There are five major goals set out for the patient, which are maintaining adequate oxygenation, remaining free from infection, relieving the anxiety, improving nutritional status, and relieving immediate respiratory distress (Nurses lab 2018).

Nursing Interventions

There are many ways to help the patient reach the goal of getting healthier but these are the most important. Preventing infection, providing family support, maintaining adequate nutrition, reducing the child’s anxiety, improving breathing and improving the airway clearance. By preventing infection, if there is anyone who is sick they mustn’t visit the patient to prevent the illness from getting worse. Whoever does come into contact with the patient they must wash hands before engaging and practice good hygiene. Vital signs should be taken every 4 hours as well as taking the antibiotics that have been prescribed. By the patient’s family providing support its gives the family and the child opportunities to voice the fears and anxiety; it’s good for family members to provide emotional support throughout the entire hospital stay (Nurses lab 2018). While maintaining the adequate nutrition for the child it is greatly encouraged to be increasing the caloric intake and to do so you can provide the child with snacks that include peanut butter. It is also good to administer pancreatic enzymes with all the meals given throughout the day. Report and changes in bowel movement and try to weigh the child weekly. When attempts in reducing the child’s anxiety it’s good to provide the child with age related activities that’ll get rid of the stress and boredom. When attempting the improve the child’s breathing Maintain the child in a semi-Fowler’s position; use pulse oximetry; maintain oxygen saturation higher than 90%; administer oxygen as ordered; administer mouth care every 2 to 4 hours; perform chest physiotherapy every 2 to 4 hours as ordered; plan nursing and therapeutic activities and diversional activities; and teach them to exercise to help loosen the thick mucus (nurses lab 2018). And lastly when improving the airway clearance, it is important to monitor the child for any signs of respiratory distress, as well as teaching the child to cough effectively. By doing so, it is easier for more mucus to come out of the child’s system and its important for you to exam the mucus that has been spit out. Encourage the child to increase the fluid intake or drink extra fluids.

Evaluation

Overall the patient seems to be having early stages of cystic fibrosis and doesn’t seem to be too severe. Pertaining to the nursing interventions, if the patients mother does everything that was mentioned, it will help her daughter recover smoothly and efficiently. The goals that must be achieved are relief of immediate respiratory distress, maintenance of adequate oxygenation, freedom from infection, relief of anxiety and improvement of nutritional status (Nurses lab 2018). With all the tips, recommendations and medication provided, the patient should be able to slowly start to recover.

Recommendations

In the future, the nurse should make it mandatory to make calls to check in on the patient and ask for vitals, color and consistency of phlegm and stool, asking if symptoms have progressed or worsened, ask about caloric intake and weight status as well as any other things that may be important. Follow up calls should be a priority to ensure that the patient is doing everything necessary to become healthier.

References
About cystic Fibrosis; what is cystic fibrosis?. Cystic fibrosis foundation (N.d) retrieved on December 31, 2020 from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/

Cystic fibrosis; overview. Mayoclinc (March 14, 2020) retrieved on December 31, 2020 from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700?utm_source=Google&utm_medium=abstract&utm_content=Cystic-fibrosis&utm_campaign=Knowledge-panel

Cystic Fibrosis; pathophysiology. Marianna Belleza, R.N September 3, 2018) Retrieved on December 31, 2020 from https://nurseslabs.com/cystic-fibrosis/#nursing_management

How cystic fibrosis is diagnosed, Lori Alma (August 07, 2019) retrieved on December 31, 2020 from https://www.verywellhealth.com/cystic-fibrosis-diagnosis-998227#:~:text=There%20are%20two%20tests%20commonly,treatment%2C%20newborns%20are%20routinely%20screened

Sweat test; the gold standard. Cystic fibrosis foundation (N.d) retrieved on December 31, 2020 from https://www.cff.org/What-is-CF/Testing/Sweat-Test/#:~:text=People%20with%20CF%20have%20more,by%202%20weeks%20of%20age.

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