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Treating Other Symptoms of Wilson Disease

Sheila is 51 and is a headmistress in a local primary academy as well as being a regional officer the Primary Heads’ Association. Sheila enjoys her job but in recent years has found it increasingly stressful. Sheila lives with her husband John and they have been married for 24 years, he is often absent on business as he is an engineer involved in many overseas projects. They have two children, Daisy is twenty and currently studying law at university in Swansea. Philip is nineteen and is studying geography in Dundee. Apart from visits to her GP for symptoms of anxiety, for which she has refused medication, Sheila has no medical history of note.

Sheila has not returned to work following the autumn half-term break. The school secretary phones her home and gets no answer, she tries to contact John but he is overseas and his phone goes straight to voicemail. Concerned she drives the short distance to Sheila’s home and sees the car in the drive, looking through the lounge window she sees Sheila sprawled on the sofa with an empty vodka bottle at her side. She dials 999. Sheila is unconscious with a GCS of 9 following an overdose of alcohol, she is taken to hospital and admitted to ICU where she is stabilised overnight

Observations on Admission to A&E

Airway: Unable to maintain airway, airway adjunct inserted by paramedics.

Breathing: Irregular breaths. RR: 7bpm. Oxygen Saturation: 98% on high flow oxygen attached to airway.

Circulation: HR: 28bpm, shallow and irregular pulse, BP: 96/42mmHg

Disability: AVPU: Unconscious but with some response to pain, GCS assessed at 9.

Exposure: Temperature 35.3C, BM 2.8mmol/L, she has pale skin with a slight blue tinge apparent. There are traces of vomit around her mouth and on her clothes. Serum bilirubin 25µmol/l, ALT 45iu/l, Prothrombin Time 21seconds.

Problem 1:

Using the A-E approach identify the nursing priorities for Sheila’s care.

  • Consider both her current presentation and the potential for any further deterioration and how this could be
  • Consider Sheila as a whole person, considering everything we know about her and not just her presenting

The A-E is a very systematic approach which helps in recognizing the life threatening conditions early. As mentioned by (), this approach helps in implementing critical interventions fast. The elements of A-E approach are Airway with cervical spine immobilization.

A. Airway: The patient is required to be kept in recovery position, laid on side and 30 o tilted. There is an increased risk of obstruction as Sheila is not enough conscious to sit. It is required considering the security of airway while minimizing the force to the cervical spine. In doing so, the nurse will require to place Sheila in a proper position so that the volume of air pressure can be achieved which is responsible for effective ventilation. The mandible and submandibular structures will be required to be lifted.

B. Breathing: Additionally, an irregular breathing has been observed in the patient where oxygen saturation is 98% which is attached to the airway. The respiratory rate is required to be monitored. Can be provided with face mask while vomiting, 3Liters of oxygen will be sufficient for the patient. In order to stabilize the patient from this condition, the nurse will be required to ensure that there is enough supply of oxygen to the lungs of Sheila. The patient can be given oxygen at 24% via a Venturi mask through maintaining a limit of nasal cannula at 1-2 L/minute.

C. Circulation: In case circulation with breathing control and IV fluids, it has been observed that Pulse rate is 28 beats per minute where the normal rate of heart beat is 60 bpm. Pulse is referred to the number of heart beats within 1 min. The patient will be provided with pharmacotherapy for restoring the pulse condition. The patient will be observed for 20 minutes for checking the blood pressure. The patient is also required to be encouraged to follow a healthy life style with quitting tobacco consumption and other substances. The pharmacological intervention might also be required to be implemented where oxygen therapy and inhaled bronchodilators might stabilize the shallowness and irregularity of pulse rate. The patient’s blood pressure is also low which is required to be restored by reducing the trigger situation to anxiety. The environment is required to be created less threatening and more comfortable for the client.

D. Disability: The brain and spinal functions are also needed to be assessed and protected. The AVPU (Alert, Voice, Pain, Unresponsive) assessment has shown that the patient is unconscious though response to pain is present. The Glasgow Coma Scale (GCS) has also been assessed at 9. As mentioned by (), a patient can score from 3 to 15 in a Glasgow Coma Scale (GCS) where scoring 3-8 indicates to be in a coma which is not the current case. Therefore, it can be stated that Sheila has had a moderate head injury. The nurse involved in the care intervention process of Sheila is required to make sure that there is always adequate airway. The nurse will be involved constantly in clearing mouth of the patient, insert oral airway, intubation assistance and continual therapy to restore the respiratory system of the patient.

E. Exposure: Exposure is another component of A-E approach applied in nursing. Considering this element it can be suggested to check the low level of vitamin K. On the other hand, no surgery can be possible during the recovery process of head injury as the clotting of blood might become evident in the case of Sheila. The patient is likely to have a liver failure which will be prioritized in the treatment plan of Sheila. As there is a trace of vomit around the mouth of Sheila, the level of bilirubin is required to be maintained.

Problem 2: 1000

Provide a rationale for the care you have suggested in Problem 1

  • Use the pathophysiology of liver failure and understanding of Sheila’s overall

Pathophysiology

It can be stated that in the case of liver failure, malfunction of the multiple organisms might take place. Triantafyllou et al. (2018) mentioned that Hyperbilirubinemia might become present which is an indicator of severe failure of the liver. The hepatic synthesis of the coagulation factors is impaired due to which Coagulopathy may occur.

The airway positioning is required to be optimized for reducing the risk of aspiration. As aspiration increases the risk of aspiration pneumonia and excessive liquid in the lungs can result in pulmonary edema that might create harm upon lungs. The increased level of aminotransferase can indicate towards Hepatocellular necrosis. Another functional issue that may take place includes peripheral vascular resistance and decrease in blood pressure. This induces hyperdynamic circulation along the increase of the heart rate and cardiac output. The impact upon cerebral locations also takes place where ammonia is produced increasingly by nitrogen substances is in the spine. This condition is considered as Portosystemic encephalopathy. Patients that have the health condition of severe encephalopathy may be commonly affected by cerebral edema. This might coincide with the health condition of Sheila as there are indications that the current patient may be suffering from liver failure. Uncal herniation can occur which can be fatal.

The oxygen saturation level is assessed to detect hypoxemia which leads to oxygenation deficiency in the blood. Additionally, this helps assessing the percentage of hemoglobin bound to oxygen in the blood processing through artery that can be denoted as SpO2. If kidneys are unable to receive oxygen in enough amounts those can spread signal in erythropoietin form. Apart from that lower oxygen saturation may lead to cell death and organ failure ultimately leading to cell injury. It is also required to test if the client is suffering from hepatic encephalopathy due to which they can develop marked amnesia, confusion or disorientation.

Patients like Sheila that are suffering from liver failure may also have acute injury to the kidney, though the reason behind it is unclear (Olson & Karvellas, 2017). The hepatic synthetic function is responsible for successfully allow the blood urea nitrogen (BUN) level become optimized which can be low in a misleading manner. Depending on this, the level of creatinine indicates towards injury to the kidney. The patient presenting the problems might have the hepatorenal syndrome, where decrease in the level of excretion of urine sodium and fractional sodium may occur. This can happen when the diuretics are not used and there is no evidence of tubular injury.

Apart from the above stated pathologies immunologic pathology may also occur. Acute liver failure may lead to immune system defects. The opsonization may get defected, immune deficiencies like Complement deficiency and others along with dysfunctional white cells and killer cells as well. As mentioned by Villanueva‐Millán et al. (2017), an increased level of bacterial translocation from gastrointestinal tract can also be observed in the patients with liver failure. Infections might also occur in the respiratory structures and urinary tract along with which, sepsis is common. Here, different pathogens may facilitate these infections which include fungus, virus or bacteria.

Additionally, the metabolic system may also be considered to be dysfunctional as a result of liver failure. Different metabolic conditions that occur to the patients having liver failure may include deficiency of Alpha-I antitrypsin and others. Apart from that, as per the views of Li et al. (2020), Hypokalemia is common in some parts of the liver among the patients with liver failure. It may take place due to decreased sympathetic tone and increased use of diuretics. As a reason of hepatic glycogen, Hypoglycemia may also take place. Additionally, it has also been observed that impairement of gluconeogenesis and insulin degradation may also take place. Moreover, due to the liver failure, Noncardiogenic pulmonary edema may also take place.

Pharmacology

Considering pharmacology in the case of liver failure, it is required to be assured that the patient is able to absorb the medicines prescribed to her. It is due to the fact that, ineffective and unnecessary medication may lead to reduction of concentration of plasma along with a reduction in efficacy. As mentioned by Camarata & Schilsky (2018), drugs like furosemide may become difficult to be absorbed among the patients with acute liver failure cases. This is very crucial as the ability of absorption of these kinds of drugs cannot be detected through pharmacokinetic data of the healthy individuals. The water solubility of the drugs is also required to be considered important in the cases like Sheila. It is due to the fact that as the liver condition of Sheila has been depicted in the case study, there are traces of vomit around her mouth and on her clothes and the level of Serum bilirubin is 25µmol/l.

  • Remember to refer to relevant pharmacology

This may be caused by the distribution of the drug into the ascitic fluid. It might result in the decrease of drug concentration in different parts of the body which may include its site of action. In order to stabilize the symptoms of Sheila load of doses may be required to be provided. As a result of chornic liver disease, the albumin levels may be lowered. Therefore, hypoalbuminaemia may affect highly protein bound drugs. This leads to increase in the proportion of unbound drugs which will be responsible for increased adverse effects. The nurses and associated healthcare providers are also required to be aware, if the drugs provided to Sheila can be metabolized by her liver. As mentioned by Gluchowski et al. (2019), the hepatic cell mass can reduce due to liver failure which will be responsible for subsequent reduction in the enzymes that metabolizes drugs. These are responsible for increased adverse drug effects. The extraction ration of the drug will also be required to be considered by the nurses while providing medication. The liver architecture of the patients with liver failure may lead to portal hypertension which is responsible for developing collateral circulation. This might lead the patient to face adverse therapeutic effects. The international normalised ratio is also required to be assessed for Sheila to identify if the metabolic capacity of Shelia is impaired.

Apart from that, the metabolic impairment may also be observable with reduced albumin and encephalopathy. Therefore, Sheila might require reduction in the maintenance doses of the drugs that are hepatically metabolized (Dinis-Oliveira, 2016).

It is very important in the context of liver failure to assess if the excretion of the drugs taking place as recommended. Drugs that undergo biliary excretion may be abandoned as in patients like Sheila with liver failure or disease elimination through the biliary route may get reduced which leads to accumulation resulting in negative medication outcome and increased risk of damage to the kidney.

Developing Scenario

Despite being stabilised initially Shelia’s liver function continues to decline causing an ongoing deterioration in her condition that progresses to end-stage and six weeks after her admission Sheila dies because of liver failure.

Problem 3: Discuss the nursing management of Sheila during this time

  • Consider the likely symptoms of end-stage liver disease and principles relating to end-of-life care
  • What referral options are available for managing Sheila in her final days?
  • What support might be available for Sheila’s family after her death?

The liver disease end stage symptoms might include the following:

  • Easy bleeding and bruising. The patients with liver disease may experience vomiting blood. Apart from that Lloyd, (2017) mentioned patient’s skin and eyes can also become yellow persistently or in recurrent manner.
  • Abdominal pain is the common among the patients with liver disease along with which, loss of appetite may also take place. The initial symptoms that patients with liver disease experience include nausea swelling due to the building up of fluids in the legs or abdomen.
  • There may also some psychological symptoms presents including cognitive issues such as problems with memorizing and concentrating (Lyu et al., 2018).

The underlying causes behind the above stated symptom may be respectively enlargement of the veins in the connecting tube to stomach and throat. The psychological symptoms may take place due to the damage of brain and nervous system.

 It can be stated that the end-of-life care is referred to the nursing care that is focused upon the highest possible comfort to the patient so that their course of living until can be made meaningful. The core principles of end-of-life care include showing respect to the patient by the healthcare providers and others. The nurses will also require being sensitive to the wishes of the patient and their family members and the choice of the patient needs to be respected always. The pain symptoms may become common in the patients with liver disease which are also required to be comforted and required to be given priority. The patients might go through inability of managing psychological, social and spiritual issues. Therefore, they are required to be assessed and therapeutic approaches can be applied likewise (de Castro & Calder, 2018).

The choice and desire of the patient is required to be provided with high importance where patients that desire continuity of care and a thorough assessment of the needs and requirements are also required to be considered in the end of life care.

The current condition of the patient where there are traces of vomit around Sheila’s mouth and on her clothes has been observed along with Serum bilirubin 25µmol/l, ALT 45iu/l, Prothrombin Time 21seconds, it is required to refer Sheila to a hepatologist. This is due to the fact that the patient is demonstrating jaundice and severe fatigue. Sheila can also be referred to the neurologist to assess if the neural damages are severe enough to conduct an operation. Additionally, Sheila can be referred to dietician where she can be guided about the daily meals without harming the liver further.

The responsibility of the nurses includes helping the patient’s family to cope with the grief after death of the patient. Therefore, nurses are required to focus that the family members are required to be returned in the normal life. In doing so, family members can be encouraged to take care of their health, taking meal in timely manner, be hydrated as much as possible. The family members are likely to demonstrate irritability or intolerance to the environment to which nurses are required to respond sensitively (Booth, Macdonald & Youssef, 2018).

Reference List for Sheila Campbell Case Study

Booth, A. T., Macdonald, J. A., & Youssef, G. J. (2018). Contextual stress and maternal sensitivity: A meta-analytic review of stress associations with the Maternal Behavior Q-Sort in observational studies. Developmental Review48, 145-177.

Camarata, M., & Schilsky, M. L. (2018). Treating Other Symptoms of Wilson Disease: The Liver. In Management of Wilson Disease (pp. 87-106). Humana Press, Cham.

de Castro, G. S., & Calder, P. C. (2018). Non-alcoholic fatty liver disease and its treatment with n-3 polyunsaturated fatty acids. Clinical nutrition37(1), 37-55.

Dinis-Oliveira, R. J. (2016). Metabolomics of methadone: clinical and forensic toxicological implications and variability of dose response. Drug metabolism reviews48(4), 568-576.

Gluchowski, N. L., Gabriel, K. R., Chitraju, C., Bronson, R. T., Mejhert, N., Boland, S., ... & Walther, T. C. (2019). Hepatocyte Deletion of Triglyceride‐Synthesis Enzyme Acyl CoA: Diacylglycerol Acyltransferase 2 Reduces Steatosis Without Increasing Inflammation or Fibrosis in Mice. Hepatology70(6), 1972-1985.

Ivanova, D., Zhelev, Z., Getsov, P., Nikolova, B., Aoki, I., Higashi, T., & Bakalova, R. (2018). Vitamin K: redox-modulation, prevention of mitochondrial dysfunction and anticancer effect. Redox biology16, 352-358.

Jain, S., & Iverson, L. M. (2020). Glasgow Coma Scale. In StatPearls [Internet]. StatPearls Publishing.

Li, X., Hu, C., Su, F., & Dai, J. (2020). Hypokalemia and clinical implications in patients with coronavirus disease 2019 (COVID-19). MedRxiv.

Lloyd, J. K. (2017). Minimising stress for patients in the veterinary hospital: Why it is important and what can be done about it. Veterinary sciences4(2), 22.

Lyu, J., Zhang, J., Mu, H., Li, W., Champ, M., Xiong, Q., ... & Cui, M. (2018). The effects of music therapy on cognition, psychiatric symptoms, and activities of daily living in patients with Alzheimer’s disease. Journal of Alzheimer's Disease64(4), 1347-1358.

Olson, J. C., & Karvellas, C. J. (2017). Critical care management of the patient with cirrhosis awaiting liver transplant in the intensive care unit. Liver Transplantation23(11), 1465-1476.

Smith, D., & Bowden, T. (2017). Using the ABCDE approach to assess the deteriorating patient. Nursing Standard (2014+)32(14), 51.

Stonerock, G. L., & Blumenthal, J. A. (2017). Role of counseling to promote adherence in healthy lifestyle medicine: strategies to improve exercise adherence and enhance physical activity. Progress in cardiovascular diseases59(5), 455-462.

Triantafyllou, E., Woollard, K. J., McPhail, M. J., Antoniades, C. G., & Possamai, L. A. (2018). The role of monocytes and macrophages in acute and acute-on-chronic liver failure. Frontiers in Immunology9, 2948.

Villanueva‐Millán, M. J., Pérez‐Matute, P., Recio‐Fernández, E., Lezana Rosales, J. M., & Oteo, J. A. (2017). Differential effects of antiretrovirals on microbial translocation and gut microbiota composition of HIV‐infected patients. Journal of the International AIDS Society20(1), 21526.

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