Exon Publications, Brisbane, Australia
Polycystic Kidney Disease (PKD) is a genetic disorder that causes the growth of numerous cysts in the kidneys, leading to a decline in kidney function and various complications. This chapter focuses on the medical management of PKD, highlighting the importance of managing kidney function, controlling hypertension, and using appropriate medications. The role of medications like ACE inhibitors and tolvaptan is highlighted. This chapter also explores advanced treatment options for PKD, including dialysis, and kidney transplantation. Dialysis is necessary when kidney function declines significantly, with hemodialysis and peritoneal dialysis being the primary types. Kidney transplantation offers a more permanent solution by replacing a damaged kidney with a healthy one from a donor.
Keywords: ACE inhibitors for PKD; Blood pressure management in PKD patients; Chronic kidney disease progression in PKD; Controlling hypertension in Polycystic Kidney Disease; Dialysis options for Polycystic Kidney Disease; End-stage renal disease management in PKD; Hemodialysis for PKD patients; Kidney function monitoring in PKD; Kidney transplantation benefits for PKD; Living donor kidney transplant for PKD; Managing pain in Polycystic Kidney Disease; Medications for Polycystic Kidney Disease symptoms; Monitoring cyst growth in PKD; Peritoneal dialysis for Polycystic Kidney Disease; Tolvaptan treatment for PKD; Transplant surgery for PKD patients; Urinary tract infection prevention in PKD
Correspondence: Exon Publications, Brisbane, Australia. books@exonpublications.com
Cite as: Treatment and Management of Polycystic Kidney Disease. In: Polycystic Kidney Disease: Public Education. Brisbane (AU): Exon Publications, 2024. Online first 2024 May 28. ISBN: 978-0-6458663-8-4.
Doi: https://doi.org/10.36255/treatment-management-polycystic-kidney-disease
Copyright: Exon Publications.
License: This article is published under Attribution-NonCommercial-NoDerivatives license (BY-NC-ND 4.0) https://creativecommons.org/licenses/by-nc-nd/4.0/
Polycystic Kidney Disease (PKD) significantly affects the kidneys, causing them to develop numerous fluid-filled cysts that impair their function. Managing PKD requires a comprehensive approach that includes regular monitoring of kidney health, controlling high blood pressure, and using medications to address symptoms and complications. This chapter aims to provide a clear understanding of the strategies and treatments available for managing PKD. By focusing on monitoring kidney function, managing hypertension, and utilizing medications, individuals with PKD can take proactive steps to maintain their health and slow the progression of the disease. This chapter focuses on two key advanced treatments: dialysis, and kidney transplantation. Dialysis helps manage kidney failure by removing waste products and excess fluids from the blood. Kidney transplantation provides a more permanent solution by replacing the diseased kidney with a healthy donor kidney (1–9).
PKD often leads to high blood pressure, or hypertension, which is one of the most common and serious symptoms of the disease. Controlling hypertension is crucial because it helps slow down the progression of kidney damage and reduces the risk of heart disease and stroke. The kidneys play a key role in regulating blood pressure by balancing fluid and salt levels in the body. When the kidneys are damaged by cysts, they struggle to perform this function, leading to hypertension. Managing blood pressure helps slow the progression of kidney damage and reduces the risk of other complications, such as heart disease and stroke.
Doctors often prescribe medications to help control blood pressure in people with PKD. These medications may include ACE (Angiotensin converting enzyme) inhibitors, angiotensin II receptor blockers (ARBs), or other antihypertensive drugs. It is important to take these medications as prescribed and to follow up regularly with your healthcare provider to monitor their effectiveness and adjust dosages if needed.
Monitoring blood pressure at home is an effective way to keep track of how well your treatment plan is working. Home blood pressure monitors are widely available and easy to use. Keeping a record of your readings and sharing them with your healthcare provider can help adjust your treatment plan as needed.
Apart from medications for controlling high blood pressure mentioned above, an important medication for PKD is tolvaptan. Tolvaptan is specifically designed to slow the growth of cysts in the kidneys. It works by blocking a hormone called vasopressin, which plays a role in the formation and growth of cysts. Clinical studies have shown that tolvaptan can slow the progression of kidney function decline in individuals with PKD. However, tolvaptan is not suitable for everyone and can have side effects, so it is important to discuss this option with a healthcare provider to determine if it is appropriate.
In addition to hypertension, PKD can cause other symptoms that need to be managed. Pain is a common symptom. As cysts grow and press against surrounding tissues, they can cause significant discomfort. Over-the-counter pain relievers, such as acetaminophen, are often recommended for mild to moderate pain. However, it is important to avoid nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen, as they can further damage the kidneys. For more severe pain, doctors may prescribe stronger pain medications. It is important to work with a healthcare provider to find the most effective and safe pain management strategy.
Blood in the urine, or hematuria, is another symptom of PKD. This can occur when cysts burst and bleed into the urinary tract. Hematuria can be alarming, but it is important to seek medical advice to determine the cause and appropriate treatment. Recurrent urinary tract infections (UTIs) are also common in people with PKD. Drinking plenty of water, practicing good hygiene, and taking prescribed antibiotics can help prevent and treat UTIs.
Kidney stones are more common in people with PKD. These stones form when minerals in the urine crystallize and can cause severe pain, particularly if they obstruct the urinary tract. Staying hydrated and avoiding foods high in oxalates, such as spinach and nuts, can help prevent kidney stones. If kidney stones do occur, medical treatment may be necessary to remove them.
In advanced stages of PKD, when kidney function declines significantly, individuals may develop anemia, a condition where the blood has a lower than normal number of red blood cells. Anemia can cause fatigue, weakness, and other symptoms. To treat anemia, doctors may prescribe medications that stimulate the production of red blood cells or recommend iron supplements to increase iron levels in the blood.
Dialysis is a treatment that takes over the function of the kidneys, removing waste products and excess fluids from the body. Dialysis is typically indicated for individuals with PKD when their kidneys can no longer perform their essential functions, a condition known as end-stage renal disease (ESRD). ESRD occurs when the kidneys are functioning at less than 10-15% of their normal capacity. Symptoms that may indicate the need for dialysis include severe fatigue, persistent nausea, swelling in the legs and feet, shortness of breath, and dangerously high levels of waste products in the blood. Blood tests measuring creatinine and blood urea nitrogen levels help doctors determine when it is time to start dialysis.
There are two main types of dialysis: hemodialysis and peritoneal dialysis. Both methods aim to remove waste products and excess fluids from the blood, but they do so in different ways.
Hemodialysis is the most common form of dialysis. It involves using a machine to filter the blood. During hemodialysis, blood is taken from the body, passed through a filter in the dialysis machine (called a dialyzer), and then returned to the body. The dialyzer acts as an artificial kidney, removing waste products and extra fluids. Hemodialysis is usually performed at a dialysis center, although it can sometimes be done at home with the right training and equipment.
The procedure for hemodialysis requires access to the bloodstream. This is typically achieved through a minor surgical procedure to create a fistula or graft in the arm. A fistula is created by connecting an artery directly to a vein, which allows for a stronger and more durable blood vessel for dialysis. A graft involves inserting a soft plastic tube to join an artery and a vein. In some cases, a catheter may be used for short-term access, but fistulas and grafts are preferred for long-term use because they are less prone to infection and clotting.
Each hemodialysis session typically lasts about four hours and is done three times a week. During the session, patients can read, watch TV, or even take a nap. While hemodialysis can effectively manage kidney failure, it also requires a significant time commitment and lifestyle adjustments.
Peritoneal dialysis is another type of dialysis that uses the lining of the abdomen, called the peritoneum, to filter the blood. During peritoneal dialysis, a sterile solution called dialysate is introduced into the abdominal cavity through a catheter. The dialysate absorbs waste products and excess fluids from the blood vessels in the peritoneum. After a few hours, the used dialysate, now containing waste products, is drained out and replaced with fresh dialysate.
There are two main types of peritoneal dialysis: Continuous Ambulatory Peritoneal Dialysis (CAPD) and Automated Peritoneal Dialysis (APD). CAPD is done manually, usually four to five times a day, and each exchange takes about 30-40 minutes. APD is done using a machine called a cycler, typically overnight while the patient sleeps. The cycler automatically performs multiple exchanges throughout the night.
Peritoneal dialysis offers more flexibility and independence compared to hemodialysis, as it can be done at home, at work, or while traveling. However, it requires the patient to be more actively involved in their treatment and maintain a sterile environment to prevent infections.
Choosing between hemodialysis and peritoneal dialysis depends on various factors, including the patient’s lifestyle, medical condition, and personal preference. Both types of dialysis have their advantages and disadvantages, and the decision should be made in consultation with healthcare providers.
Living with dialysis requires significant adjustments. It is important to follow dietary recommendations, as certain foods can affect the balance of fluids and electrolytes in the body. Regular monitoring of blood pressure, fluid intake, and overall health is crucial. Support from family, friends, and healthcare providers can help manage the emotional and physical challenges of dialysis.
In conclusion, dialysis is a life-saving treatment for individuals with PKD who have reached ESRD. Understanding the indications for dialysis, the different types available, and how the procedures work can help patients make informed decisions about their care. Whether choosing hemodialysis or peritoneal dialysis, working closely with healthcare providers and maintaining a supportive network are essential for managing PKD and improving quality of life.
Kidney transplantation involves replacing a diseased kidney with a healthy one from a donor. A kidney transplant is typically indicated for individuals with PKD who have reached ESRD, meaning their kidneys are functioning at less than 10-15% of their normal capacity. Symptoms that suggest the need for a transplant include severe fatigue, persistent nausea, swelling in the legs and feet, difficulty breathing, and dangerously high levels of waste products in the blood. Blood tests measuring creatinine and blood urea nitrogen levels help doctors determine when a kidney transplant is necessary.
There are two main types of kidney transplants: living donor transplants and deceased donor transplants. Both types have their own benefits and considerations.
A living donor transplant involves receiving a kidney from a living person, usually a family member or close friend. Living donor transplants offer several advantages. The surgery can be scheduled at a convenient time. Living donor kidneys typically last longer than those from deceased donors. However, finding a compatible living donor can be challenging, and the donor must undergo extensive testing to ensure they are healthy enough to donate.
A deceased donor transplant involves receiving a kidney from someone who has recently died and whose family has agreed to organ donation. Deceased donor kidneys are allocated based on a waiting list managed by a transplant organization. The waiting time for a deceased donor kidney can vary, sometimes taking several years. Despite the longer wait, deceased donor transplants are a vital option for many patients with ESRD.
The kidney transplant procedure involves several steps. Before the surgery, the recipient undergoes a thorough evaluation to ensure they are a suitable candidate for transplantation. Once a compatible donor kidney is available, the transplant surgery is scheduled. The procedure usually takes three to four hours and is performed under general anesthesia. In most cases, the recipient's own diseased kidneys are not removed unless they are causing severe problems such as pain or infection.
After the transplant surgery, the patient is closely monitored in the hospital for several days. The new kidney usually starts working immediately, although it may take a few days or weeks for it to function fully.
The patient will need to take immunosuppressive medications for the rest of their life to prevent their body from rejecting the new kidney. These medications help suppress the immune system to stop it from attacking the transplanted kidney as a foreign object.
Living with a transplanted kidney requires ongoing care and monitoring. Regular follow-up appointments are essential to check kidney function, manage immunosuppressive medications, and monitor for any signs of rejection or infection. Blood tests are routinely performed to measure kidney function and ensure that the immunosuppressive medications are at the correct levels.
Managing PKD involves a combination of regular monitoring, lifestyle adjustments, and the use of medications to control symptoms and slow disease progression. Regular check-ups and tests help track kidney function and detect complications early. Controlling high blood pressure with medications and lifestyle changes is essential to prevent further kidney damage. Pain management, infection prevention, and addressing complications like anemia and kidney stones are also critical aspects of care. Dialysis is essential for those with severe kidney function decline, offering life-saving support through hemodialysis and peritoneal dialysis. Kidney transplantation presents a long-term solution, restoring kidney function with a healthy donor kidney.
This article is part of our public education series. It was written by professional medical writers for the public using simple terms based on our medical book Polycystic Kidney Disease and articles indexed in PubMed, and further peer-reviewed for factual accuracy by independent experts. It is intended solely for informational purposes and is not to be considered medical advice. The views and opinions expressed in this article are believed to be accurate at the time of publication, but the publisher, editors, or authors cannot be held responsible or liable for any errors, omissions, or consequences arising from the use of the information contained in this article. The publisher makes no warranties, implicit or explicit, regarding the contents of this article or its use.
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