Polycystic Kidney Disease Pathophysiology and Nursing Care
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Introduction
The kidneys are significant physiological organs that filter waste materials and extra fluid from circulation. However, the kidneys can occasionally go wrong, which can cause a number of medical issues. There is a range of kidney-related problems. One of these conditions is polycystic kidney disease. Multiple fluid-filled cysts develop in your kidneys as a result of the hereditary ailment polycystic kidney disease (PKD). PKD cysts can alter your kidneys' structure, potentially making them significantly bigger, unlike the often-benign simple kidney cysts that might develop in the kidneys later in life. A hereditary abnormality of the kidneys is known as polycystic kidney disease (PKD). Multiple cysts growing inside the kidneys, which may ultimately result in renal failure, is how it is described. Because PKD may be inherited in an autosomal dominant manner, a kid who has a parent with the condition has a 50% chance of acquiring it as well. Both autosomal dominant PKD (ADPKD) and autosomal recessive PKD are different kinds of PKD (National Institute of Diabetes and Digestive and Kidney Diseases, 2017). The following paper will offer an analysis of polycystic kidney disease by probing the causes, signs, diagnoses, and prognosis. Additionally, the paper will probe into the nursing care plan and the teaching priorities.
Pathophysiology
Causes, Etiology of Polycystic Kidney Disease
Mutations in certain genes are the root cause of the hereditary condition called polycystic kidney disease (PKD). There are two primary genetically based kinds of PKD, each with a distinct phenotype:
Autosomal Dominant PKD (ADPKD)
The polycystin-1 and polycystin-2 proteins, which are important in controlling cell growth and division, are made according to the instructions provided by these genes. The creation of many cysts in the kidney is caused by mutations in these genes, which cause tubular epithelial cells to proliferate abnormally (The American Kidney Fund, 2022).Being an autosomal dominant condition, ADPKD has a 50% risk of developing in everyone who inherits one copy of the defective gene from an affected parent.
Autosomal Recessive PKD (ARPKD)
ARPKD is an uncommon type of PKD that generally manifests in infancy or early childhood. It is brought on by changes in the PKHD1 gene, which codes for the production of the fibrocystin protein. The development of cilia, which are hair-like structures on the surface of cells and are crucial for cell communication and function, is facilitated by fibrocystin (Chebib & Hogan, 2018). Mutations in the PKHD1 gene lead to improper cilia production, leading in the development of cysts in the kidneys. Because ARPKD is an autosomal recessive condition, a person must inherit two copies of the defective gene—one from each parent—in order to be affected. Both kinds of PKD can cause kidney cysts to develop and reproduce, which can cause kidney enlargement, reduced kidney function, and ultimately end-stage renal disease (Chebib & Hogan, 2018).
Since ARPKD is an autosomal recessive condition, a person must inherit two versions of the defective gene, one from each parent, in order to be affected. Both kinds of PKD can cause kidney cysts to develop and reproduce, which can cause kidney enlargement, reduced kidney function, and ultimately end-stage renal disease.
Signs and Symptoms
The signs and symptoms of polycystic kidney disease (PKD) might vary depending on the kind and complexity of the illness.
Common PKD warning signs and symptoms include (NHS, 2017):
a) Abdominal or flank pain: Pain in the side or back is a typical PKD symptom and may be brought on by cyst-related kidney enlargement.
b) High blood pressure: PKD has been linked to high blood pressure, which can worsen kidney impairment and raise the risk of heart disease.
c) Kidney cysts can result in kidney stones developing, which can be painful and uncomfortable.
d) urinary problems. An increased desire to urinate, especially at night, might result from PKD.
e) The growth of cysts in the urinary system puts people with PKD at an increased risk of getting urinary tract infections.
f) Kidney enlargement owing to cyst formation is a potential side effect of PKD and can be identified via imaging tests like an MRI or ultrasound.
g) Reduced kidney function: PKD has the potential to, over time, produce a reduction in renal function, which may result in symptoms including weakness, weariness, and swelling in the legs and ankles.
Diagnosis
The development of many cysts in the kidneys is a hallmark of polycystic kidney disease (PKD), a hereditary illness that is part of other nephrotic illnesses (NHS, 2017). Genetic tests, imaging scans, physical examinations, and medical histories can all be used to identify it. The symptoms one is experiencing, the family history, and the medical history will all be discussed with the
doctor (NHS, 2017). Since PKD is a hereditary condition, it's critical to find out whether any family members have a history of either renal illness or PKD. Examining a patient's belly, especially when a doctor will examine for any odd tumors or larger kidneys.
Imaging Tests: Imaging tests can aid in the visualization of kidney cysts. The imaging tests that are most often utilized include (NHS, 2017).
a) The most used imaging test for PKD diagnosis is ultrasound. The kidneys are represented as sound waves (Hwang et al., 2018).
b) CT scan: This imaging procedure produces fine-grained pictures of the kidneys using Xrays and computer technologies.
c) MRI: This imaging procedure produces fine-grained pictures of the kidneys using a magnetic field and radio waves.
d) Genetic testing: Because PKD is a hereditary condition, the diagnosis can be confirmed by genetic testing. (Hwang et al., 2018) The PKD-causing genes' mutations can be found via a blood test.
In order to assess the severity of the condition and keep track of kidney function, more tests may be required once PKD has been definitively diagnosed. A blood test to assess kidney function and a urine test to look for protein and blood in the urine are examples of these that may be performed.
Treatment
The goals of treatment are to reduce symptoms and avoid consequences. Urinary tract infections and high blood pressure are both treated with medications. Growth can be enhanced using growth hormones. Once kidney failure sets in, dialysis and transplantation are plausible options
Whilst there is currently no cure for PKD, there are a number of therapeutic options that can help control symptoms and halt the disease's development.
a) Medication: Specific drugs can be used to treat discomfort, high blood pressure, and urinary tract infections that are PKD symptoms. ARBs, painkillers, antibiotics, and ACE inhibitors are a few examples of possible treatments (Hwang et al., 2018).
b) Altering the diet and exercise regimen might also help patients control the symptoms of PKD. Regular exercise and a low-sodium diet can both assist to lower blood pressure and maintaining the function of your kidneys.
c) Dialysis: If your kidneys are no longer working correctly, dialysis can assist clear the blood of waste and extra fluid.
d) Kidney transplant: A kidney transplant may be required in severe PKD sufferers. A healthy kidney from a donor is surgically implanted into the patient's body during a kidney transplant.
It's critical to remember that PKD treatment is extremely customized and is based on the disease's severity and the patient's specific symptoms. Working together with your medical team will help you choose the most effective course of therapy.
Prognosis
The degree and type of polycystic kidney disease (PKD) affect the prognosis of the disease. When compared to autosomal recessive PKD (ARPKD), which can cause symptoms as early as infancy and proceed more swiftly, autosomal dominant PKD (ADPKD) often progresses slowly and may not show symptoms until middle life (Hwang & Pei, 2018). The kidneys may continue to operate properly in the early stages of ADPKD, and people may not exhibit any symptoms. However, if the kidneys expand due to cyst growth over time, elevated blood pressure,
proteinuria, and ultimately renal failure may result. Some ADPKD patients could require dialysis or a kidney transplant. Due to the disease's accelerated progression and potential for serious kidney and liver damage, the prognosis for ARPKD is often poor (Hwang et al., 2018). Infants with ARPKD may need urgent care and are at a higher risk of dying soon after birth. It's crucial to remember that with early discovery and suitable care, the prognosis for PKD can be improved. A nutritious diet, controlling blood pressure, quitting smoking, and limiting alcohol intake are just a few examples of lifestyle modifications that can reduce the disease's course (Hwang & Pei, 2018). For those with PKD, regular monitoring and medical treatment of symptoms can also aid the prognosis.
Nursing Implications
Nursing Care Plan
PKD's first priority nursing diagnosis is the risk of excess fluid volume due to decreased urine output and impaired renal function. This nursing diagnosis is appropriate because the condition leads to decreased urine output, which can cause an imbalance in fluid and electrolyte levels. The fluid accumulation can lead to edema, hypertension, and other complications (Johnson, 2020). The broad goal is to maintain fluid and electrolyte balance and prevent complications associated with fluid volume excess. The SMART patient outcome is that the patient will maintain adequate fluid and electrolyte balance by achieving a urine output of at least 30 mL/hour, maintaining a serum electrolyte level within normal limits, and reporting any signs of fluid overload, such as edema or shortness of breath.
The nursing interventions will include monitoring fluid intake and output. Accurately measuring the patient's fluid intake and output is essential to determine the fluid balance. The nurse should monitor the patient's urine output hourly and document the amount. A urine output
of less than 30 mL/hour requires prompt intervention (National Kidney Foundation, 2022). Additionally, the nurse should monitor the patient's weight daily to detect any changes that may indicate fluid overload. The second intervention is to assess vital signs and electrolyte levels.
Vital signs, including blood pressure, heart rate, and respiratory rate, should be monitored frequently to detect any signs of fluid overload (Johnson, 2020). The nurse should also monitor the patient's serum electrolyte levels, including potassium, sodium, and calcium, and report any imbalances to the physician.
The third intervention is to administer medication as prescribed. The nurse should administer medications as prescribed, including diuretics, antihypertensives, and electrolyte supplements. The nurse should monitor the patient's response to medication, including any side effects, and report any adverse reactions to the physician. Besides, nurses can implement measures to prevent infection. Patients with PKD are at increased risk of developing urinary tract infections. The nurse should implement measures to prevent infection, such as encouraging the patient to maintain good hygiene and drink adequate fluids to promote urine output (Johnson, 2020). Lastly, the nurse should educate the patient on fluid and sodium restriction. The patient should be encouraged to limit their fluid intake and avoid foods high in sodium, such as processed foods, canned soups, and fast food (National Kidney Foundation, 2022). The nurse should also provide information on the importance of adhering to the medication regimen and attending follow-up appointments with the physician.
Another priority nursing diagnosis for a patient with Polycystic Kidney Disease (PKD) is impaired gas exchange related to respiratory muscle weakness, lung involvement, and decreased oxygen-carrying capacity of the blood. This nursing diagnosis is appropriate for a patient with PKD because it can lead to respiratory distress and impaired gas exchange (Johnson, 2020). The
broad goal is to improve gas exchange, maintain oxygen saturation within normal limits, and prevent respiratory complications. The SMART Patient Outcome is that the patient will maintain oxygen saturation above 95%, breathe comfortably, and report any signs of respiratory distress.
Several nursing interventions align with this diagnosis. The first is to assess the respiratory status of the patient. The nurse should assess the patient's respiratory status frequently, including respiratory rate, depth, pattern, and oxygen saturation level. The nurse should also monitor for signs of respiratory distress, such as dyspnea, chest pain, cough, and wheezing (Johnson, 2020). The second intervention is to encourage deep breathing and coughing. The nurse should encourage the patient to perform deep breathing exercises and coughing to promote lung expansion and secretion clearance. To reduce respiratory distress, the nurse should also educate the patient on proper breathing techniques, such as pursed-lip breathing. Thirdly, a nurse should intervene by administering oxygen therapy as prescribed, including a nasal cannula, face mask, or mechanical ventilation, to maintain oxygen saturation within normal limits. The nurse should monitor the patient's response to oxygen therapy and adjust the flow rate or device as needed. Another intervention is to position the patient for optimal breathing. The nurse should position the patient for optimal breathing, such as elevating the head of the bed, to reduce the workload on the respiratory muscles and promote lung expansion (Johnson, 2020). The nurse should also encourage patients to change positions frequently to prevent respiratory complications. Nurses also need to inform patients about selfcare. The nurse should educate the patient on self-care measures to prevent respiratory complications, such as avoiding smoking, getting vaccinated against flu and pneumonia, and practicing good hygiene to prevent infections. Lastly, a nurse should monitor and manage pain. Patients with PKD may experience pain from cysts in the kidneys or surrounding organs
(National Kidney Foundation, 2022). The nurse should monitor the patient's pain level and administer pain medications as prescribed. Pain management can improve respiratory function by reducing anxiety, improving lung expansion, and promoting deep breathing.
Teaching Priorities
Patients with PKD require ongoing education on managing the disease and preventing complications. There are several teaching priorities for patients with PKD. First, a patient needs to understand the disease. Patients with PKD should receive education on the disease process, including its causes, signs and symptoms, and potential complications (Johnson, 2020). They should also be informed about the genetic nature of the disease and its potential impact on family members. Secondly, PKD patients should learn the lifestyle modifications they must make to manage the disease. Patients with PKD should receive education on lifestyle modifications, including a low-sodium diet, maintaining a healthy weight, avoiding smoking and alcohol, and regular exercise (National Kidney Foundation, 2022). These modifications can help slow the progression of the disease and prevent complications such as high blood pressure and cardiovascular disease. Besides, medication management is a teaching priority. Patients with PKD often require medication to manage their symptoms and prevent complications. Patients should receive education on their medication regimen, including proper dosages, administration, and potential side effects. Another teaching priority is monitoring and follow-ups. Patients with PKD should be educated on the importance of regular monitoring and follow-up with their healthcare provider. This includes regular blood and urine tests, imaging studies, and monitoring blood pressure (National Kidney Foundation, 2022). The practice will allow for early detection and management of any potential complications. Additionally, support and coping are vital teaching
priorities. Patients with PKD may experience emotional and psychological distress due to the impact of the disease on their daily life. Patients and their families should receive education on coping strategies and support resources, including counseling, support groups, and community resources.
There are several educational and community resources for patients with PKD. These include the PKD Foundation, National Kidney Foundation, and Genetic Counseling. The PKD Foundation is a nonprofit organization that provides support and education for patients and families affected by PKD (PKD Foundation, 2022). They offer educational materials, support groups, and a patient registry to connect patients with clinical trials. The National Kidney Foundation provides educational resources and support for patients with kidney disease, including PKD. They offer educational materials, a helpline, and support groups. Lastly, patients with PKD and their families may benefit from genetic counseling to better understand the inheritance pattern and implications of the disease for future generations.
Conclusion
Polycystic Kidney Disease (PKD) is an inherited condition that can hugely affect those that get it. The disease can hugely affect patients, and one needs to seek medical attention when the signs and symptoms of PKD surface. Developing a comprehensive nursing care plan that addresses the patient's needs and improves their quality of life is also essential. Besides, patients with PKD require ongoing education on how to manage the disease and prevent complications. Educational and community resources are available to support patients and families affected by PKD.
References
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Hwang, Y., & Pei, Y. (2018). Autosomal dominant polycystic kidney disease management. Oxford Medicine
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https://doi.org/10.1093/med/9780199592548.003.0309_update_001
Hwang, Y., Barua, M., McNaught, A., Khalili, K., & Pei, Y. (2018). Imaging-based diagnosis of autosomal dominant polycystic kidney disease. Polycystic Kidney Disease, 133-142. https://doi.org/10.1007/978-1-4939-7784-0_7
Johnson, A. M. (2020). Polycystic Kidney Disease. StatPearls Publishing.
https://www.ncbi.nlm.nih.gov/books/NBK430836/
National Institute of Diabetes and Digestive and Kidney Diseases. (2017, January 17). Autosomal dominant polycystic kidney disease.
https://www.niddk.nih.gov/health-information/kidney-disease/polycystickidney-disease/autosomal-dominant-pkd
National Kidney Foundation. (2022). Polycystic Kidney Disease.
https://www.kidney.org/atoz/content/polycystic
NHS. (2017, October 20). Autosomal dominant polycystic kidney disease - Treatment. nhs.uk. https://www.nhs.uk/conditions/autosomal-dominant-polycystic-kidneydisease-adpkd/treatment/
NHS. (2017, October 20). Autosomal dominant polycystic kidney disease - Diagnosis.
nhs.uk. https://www.nhs.uk/conditions/autosomal-dominant-polycystic-kidneydisease-adpkd/diagnosis/
PKD Foundation. (2022). About Us. https://pkdcure.org/about-us/
The American Kidney Fund. (2022, December 5). Polycystic kidney disease (PKD) symptoms, treatments & causes. American Kidney Fund.
https://www.kidneyfund.org/all-about-kidneys/types-kidney-diseases/ polycystic-kidney-disease
