Part I
Cystic Fibrosis (CF) is a recessive disease that an individual inherits and causes the buildup of mucus that turns destructive to other organs. It cause damage of the body’s respiratory systems and may cause severe problems in the patient’s digestive system. The mucus produced is abnormally thick and its accumulation may cause serious damage to the lung. Every individual has two copies of the CF transmembrane conductance regulator (CFTR). When an individual inherits two other copies of CFTR genes that have mutations, each from the mother and father, them he or she develops CF (Gupta, Hong & Bai, 2019). Both two copies of CF should have mutations; if one does not have mutation, then the individual comes a CF carrier. A CF carriers do not have the disease but can transfer the defective gene to their children. Gene mutations produce CFTE protein that regulates salt and fluids flow in the body cells. In patients of CF, the mutations disrupt the process of CFTR protein production. Malfunctioning of CFTR in body lungs cells and other body parts cause infections. It may as well cause destruction of the pancreas and other organs.
Diagnosis of CF takes several steps and procedures. There are diverse tests including sweat chlorine test, a carrier test, and clinical evaluation. Besides, there are processes of newborn screening of children after their birth at an early stage of two years of age. The sweat test is a proven and reliable test for CF in newborn babies. It involves a test on the salt concentration in an individual’s sweat. A high level of chloride may confirm the presence of CF. If the sweat that has a chloride level of less than or at 2 mmol/L, there is less likelihood that CF. Chloride levels between 30 and 59 mmol/L shows possibilities of CF but further diagnosis is necessary. A chloride level of more hat 60 mmol/L reveals a high likelihood of CF being diagnosed.
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How genetic testing can be used to make predictions
When parents are either CF patients or carriers and a sweat chloride level is either at 60 mmol/L or more, then there is high likelihood of CF in the patient. CF gene mutation testing is important to test whether an individual is a carrier. Individuals who have both positive CF gene mutation test show positive chloride level test results, the results confirm a positive test CF. CF gene mutation test, therefore, is useful to predict if an individual is a carrier.
The pathophysiology of Cystic Fibrosis. How it affects the respirator and GI system
There are different types of CF complications, based on the degree of CFTR mutations . However, i n some CF patients, the do not experience all pathological changes in their systems.
CF patients may experience acute failure in the functioning of their respiratory systems. They experience nasal polyps and, in some instances, sinus infections. The presences of asialoGM1 molecules in CF patients’ PH, which are critical receptors for bacteria in the respiratory system, which promotes the binding of P aeruginosa and S aureus (Grossman & Grossman, 2005). Reduced CFTR amounts cause bacterial presence and colonization in the patient’s airways. Airways obstruction case an expansion of the alveoli and air trapping occurs.
CF mostly affects the pancreases as part of the GI system. The CFTR renders the secretion from the pancreas to be thick and, therefore, cause a blockage of the duct. Abnormally thick secretions from the pancreas cause duct blockage and compromises the release of critical enzymes. CF patients have difficulties in digesting fats, other proteins, and vitamins that are soluble in fats.
Manifestations that Sam is the early stages of ARDS
There are no bowel sounds and the heart tones are normal. These are manifestations that Sam is in the early stages of ARDS. If the condition was at advanced stages, it would be evident from collapsed lungs (pneumothorax). Sam would be experiencing severely short breath and critical difficulties while breathing. He would have extreme tiredness and fatigue.
Pathophysiology of the three stages of ARDS
The three stages of ARDs include exudative, proliferative, and fibrotic (Force, Ranieri, & Rubenfeld, 2012). At the exudation stage, there is leakage of proteinaceous fluids. Neutrophils cells migrate into the endothelial surfaces. At proliferative stage, there is the secretion off extracellular matrix proteins and attachment of interstitium on the surface of membranes that are damaged. Besides, there is intra-alveolar fibrosis occurring at this stage. It is further manifested b type II collagen. At the fibrotic stage, there is type I collagen, which consists of highly thick fibrils.
Top three nursing priorities with rationale at this time
The first nursing priority is to help Sam maintain an adequate gaseous exchange. Second, help Sam regain the lost blood during surgery. Third, help prevent further secretion of mucus from Sam’s lungs.
Lab volumes changes after units of PRBC are given
When packed red blood cells (PRBC) are given to Sam, the blood pressure level may change.
A plan for Sam for the next 12 hours
Assessment
Sam has ground glass appearance in the lower lobes. NA 17 meq/L., K 3.9 me/L, Mg 2 me/L, WBC, 12, 000 mm3, H/H 15 g/dl and 42%, Lactic Acid 20 mg/dl.
Diagnosis
Ineffective breathing pattern. Low red blood cells.
Expected Outcome
As outcome for the plan of care, Sam will achieve effective breathing with mechanical ventilation. He will have adequate cardiac output.
Planning
Allow Sam to have uninterrupted rest
Monitor lung sounds every 2 hours
Implementation
Asses Sam’s level of exhaustion every 2 hours
Monitor the ease of breathing
Evaluation
Sam is breathing using ventilator and PRBC is introduced.
Part II
The information can be used to come to the conclusion that Sam is now in septic shock include excessive inflammatory response to pathogens that are infectious.
The mean arterial pressure (MAP) and ml/hr for the Docutamine drip and Levophed drip will start from 2 to 20 µg/kg/min.
Top three priorities, with rationale, at this time
At this time, the priority is to put Sam under high-flow nasal cannula (HFNC) to enable him have humidified oxygen. HFNC is important because, at this time, Sam has severe sepsis (Kim & Hong, 2016). Second, prone positioning would be effective is a priority because Sam will be in critical demand for oxygenation. Third, extracorporeal life support would be important at this time to help Sam cope with his condition.
Ethical dilemma you identify in relation to Sam’s parents request
In Sam’s case, there is the question whether it is morally acceptable to act on the parent’s will after 24 hours, instead of the 4 days stated in Sam’s living will. First, Sam’s autonomy as stated in the will not to be on the ventilator for more than 4 days without improvement should be respected. The proposal by his parents to have the treatment continued, however, violates Sam’s autonomy expressed in the living will. The principle of beneficence regards the good of the patient as important and critical in situations that raise ethical dilemma ( Shaha et al., 2018) . No harm should be done to the patient and his or her whish should be respected. Based on the principle of beneficence, it is not justified to do as per the request of Sam’s parents. Instead, it is better to wait for the 4 days while monitoring Sam’s condition.
References
Force, A. D. T., Ranieri, V. M., & Rubenfeld, G. D. (2012). Acute respiratory distress syndrome. JAMA, 307 (23), 2526-2533.
Grossman, S., & Grossman, L. C. (2005). Pathophysiology of cystic fibrosis implications for critical care nurses. Critical care nurse, 25 (4), 46-51.
Gupta, A., Hong, Z., & Bai, H. J. (2019). Autosomal Recessive Inhertance Cystic Fibrosis: A Literature Review. Social Science and Humanities Journal, 883-891.
Kim, W. Y., & Hong, S. B. (2016). Sepsis and Acute Respiratory Distress Syndrome: Recent Update. Tuberculosis and respiratory diseases, 79 (2), 53–57. doi:10.4046/trd.2016.79.2.53
Shaha, K. K., Patra, A. P., Chaudhari, V. A., Das, S., & Das, S. K. (2018). A review on Applications of Principles of Medical Ethics in the Practice of Surgery. South-East Asian Journal of Medical Education, 12 (1), 13-18.
