Choroba policystyczna nerek
Charakterystyka, pielęgnacja i opieka
Choroba policystyczna nerek (PKD) to genetyczne schorzenie charakteryzujące się tworzeniem licznych torbieli w nerkach, prowadzących do ich powiększenia i postępującej niewydolności. Dotyka około 12,5 miliona osób na świecie, z 50% ryzykiem rozwoju schyłkowej niewydolności nerek (ESRD) oraz wysokim odsetkiem powikłań, takich jak nadciśnienie tętnicze (80%), bolesne powikłania nerkowe (60%) i pęknięcie tętniaka śródczaszkowego (3%). Opieka pielęgniarska koncentruje się na monitorowaniu funkcji nerek, ciśnienia tętniczego, bilansu płynów i elektrolitów, a także na kontroli masy ciała i ocenie miejsc dostępu do dializy. Leczenie jest głównie wspomagające, obejmujące kontrolę bólu, leczenie nadciśnienia (preferowane inhibitory ACE, ARB, beta-blokery, blokery kanału wapniowego) oraz szybkie zwalczanie zakażeń układu moczowego. W 2018 r. FDA zatwierdziła tolvaptan, antagonista receptora wazopresyny, który spowalnia progresję ADPKD u wybranych pacjentów.
- Wprowadzenie do choroby policystycznej nerek
- Opieka pielęgniarska w chorobie policystycznej nerek
- Ocena i monitorowanie stanu pacjenta
- Zarządzanie objawami i powikłaniami
- Edukacja pacjenta i jego rodziny
- Leczenie farmakologiczne
- Modyfikacje stylu życia i dieta
- Kompleksowa opieka multidyscyplinarna
- Badania przesiewowe i poradnictwo genetyczne
- Monitorowanie długoterminowe
- Badania kliniczne i nowe kierunki leczenia
- Podsumowanie zasad opieki pielęgniarskiej
Wprowadzenie do choroby policystycznej nerek
Choroba policystyczna nerek (PKD – Polycystic Kidney Disease) to genetyczne schorzenie charakteryzujące się tworzeniem licznych torbieli wypełnionych płynem w nerkach. Torbiele te stopniowo rosną, prowadząc do powiększenia nerek, upośledzenia ich funkcji i ostatecznie do niewydolności nerek. Jest to najczęstsza zagrażająca życiu choroba jednogenowa, dotykająca około 12,5 miliona osób na całym świecie, z 50% ryzykiem rozwoju schyłkowej niewydolności nerek, 80% ryzykiem nadciśnienia tętniczego, 60% ryzykiem bolesnych powikłań nerkowych i 3% ryzykiem pęknięcia tętniaka śródczaszkowego.1
Jest to najczęstsza dziedziczna choroba nerek i częsta przyczyna przewlekłej choroby nerek. Niektóre osoby rozwijają nadciśnienie tętnicze i niewydolność nerek w wyniku PKD.2 Obecnie nie ma leku na PKD, ale wczesne wykrycie i leczenie może zmniejszyć lub zapobiec niektórym powikłaniom.
Opieka pielęgniarska w chorobie policystycznej nerek
Opieka pielęgniarska w przypadku pacjentów z chorobą policystyczną nerek skupia się na monitorowaniu progresji choroby i wdrażaniu interwencji, gdy objawy lub oznaki wskazują na potrzebę interwencji.3 Plany opieki pielęgniarskiej pomagają monitorować i zarządzać progresją choroby, identyfikując odpowiednie pomiary funkcji związanych z nerkami, specyficzne dla każdego stadium choroby.
Ocena i monitorowanie stanu pacjenta
Kluczowym elementem opieki pielęgniarskiej jest dokładna ocena stanu pacjenta i regularne monitorowanie:4
- Zbieranie wywiadu i przeprowadzanie oceny stanu pacjenta
- Monitorowanie parametrów życiowych, szczególnie ciśnienia tętniczego
- Monitorowanie funkcji nerek i wydalania moczu, nawodnienia, równowagi płynów i elektrolitów
- Kontrola dziennej masy ciała
- Ocena obrzęków i dbanie o integralność skóry
- Monitorowanie miejsca dostępu do dializy (jeśli dotyczy)
Pielęgniarki śledzą ilość wydalanego moczu w stosunku do przyjmowanych płynów. Jeśli pacjent nie oddaje wystarczającej ilości moczu, personel medyczny może zastosować leki moczopędne lub wprowadzić cewnik.5
Zarządzanie objawami i powikłaniami
W chorobie policystycznej nerek ważne jest wczesne rozpoznanie i leczenie objawów oraz powikłań:6
- Kontrola bólu za pomocą leków przeciwbólowych, w tym opioidów w przypadku przewlekłego bólu
- Leczenie nadciśnienia tętniczego, które jest najczęstszym objawem PKD
- Szybkie leczenie zakażeń układu moczowego antybiotykami
- Monitorowanie pod kątem krwiomoczu i zwiększenie podaży płynów w przypadku jego wystąpienia
- Zarządzanie powikłaniami takimi jak kamienie nerkowe
W przypadku PKD leczenie jest głównie wspomagające, ponieważ nie ma lekarstwa na tę chorobę. Stosowane leki obejmują leki przeciwbólowe, antybiotyki na infekcje oraz inhibitory ACE lub ARB, aby pomóc w kontroli nadciśnienia.6
Edukacja pacjenta i jego rodziny
Edukacja pacjenta i jego rodziny jest kluczowym elementem opieki pielęgniarskiej w PKD:8
- Informowanie o naturze choroby, możliwych powikłaniach i prawdopodobnym rokowaniu
- Edukacja na temat znaczenia przestrzegania zaleceń dotyczących leków i diety
- Wyjaśnienie genetycznych implikacji dla członków rodziny
- Zachęcanie do regularnych badań kontrolnych
- Wskazówki dotyczące zdrowego stylu życia, w tym aktywności fizycznej, kontroli masy ciała i ograniczenia soli
Edukacja powinna obejmować również informacje na temat konieczności regularnych badań kontrolnych, nawet przy braku objawów klinicznych, z badaniami ultrasonograficznymi co 6-12 miesięcy w zależności od indywidualnego przypadku, aby ocenić progresję torbieli i wykryć przewlekłą chorobę nerek we wczesnym stadium.10
Leczenie farmakologiczne
Kontrola nadciśnienia tętniczego jest kluczowym elementem leczenia PKD. Wczesna i skuteczna kontrola nadciśnienia jest bardzo ważna, aby zmniejszyć zachorowalność i śmiertelność pacjentów z PKD.11 Najlepszymi lekami w leczeniu nadciśnienia u pacjentów z PKD są inhibitory układu renina-angiotensyna-aldosteron (RAAS):
- Inhibitory konwertazy angiotensyny (ACE)
- Blokery receptora angiotensyny II (ARB)
- Blokery kanału wapniowego
- Beta-blokery
- Rozważne stosowanie leków moczopędnych
W kwietniu 2018 r. FDA zatwierdziła nowy lek o nazwie tolvaptan do leczenia autosomalnej dominującej postaci choroby policystycznej nerek (ADPKD). Lek ten może być stosowany w celu spowolnienia spadku funkcji nerek u dorosłych zagrożonych tym typem PKD.13 Tolvaptan to antagonista receptora wazopresyny, który wykazał zdolność do spowolnienia spadku funkcji nerek i zmniejszenia objawów bólu u osób z ADPKD.14
Zastosowanie tolvaptanu wymaga starannego wyboru pacjentów kwalifikujących się do leczenia, w oparciu o ryzyko progresji. Pacjenci będą potrzebować wsparcia podczas rozpoczęcia leczenia i długoterminowego zarządzania.11
Leczenie innych objawów i powikłań
Oprócz kontroli nadciśnienia, leczenie PKD obejmuje także:
- Leczenie bólu – w przypadku długotrwałego bólu lekarz może zalecić silniejsze leki, takie jak opioidy lub gabapentynę, które mogą pomóc w bólu neuropatycznym15
- Leczenie zakażeń układu moczowego – szybkie leczenie antybiotykami, aby zapobiec dalszym powikłaniom16
- Leczenie kamicy nerkowej – odpowiednie nawodnienie i leki przeciwbólowe17
- Leczenie anemii – żelazo i erytropoetyna12
- Zapobieganie chorobie metabolicznej kości – suplementy wapnia, środki wiążące fosforany i leki hamujące przytarczyce12
- Hormon wzrostu – aby przeciwdziałać ograniczającemu wzrost wpływowi mocznicy12
Modyfikacje stylu życia i dieta
Zdrowy styl życia może znacznie pomóc w zarządzaniu PKD. Zalecenia dla pacjentów obejmują:18
- Dieta przyjazna nerkom – zwykle zaleca się dietę niskosodową, niskobiałkową (choć nie zawsze) i ograniczającą cholesterol1914
- Regularna aktywność fizyczna – zaleca się umiarkowaną aktywność fizyczną, jednak należy unikać sportów kontaktowych, które mogą być potencjalnie szkodliwe dla nerek1320
- Kontrola masy ciała – pacjenci z nadwagą lub otyłością mają szybszą progresję choroby nerek w ADPKD14
- Odpowiednie nawodnienie – picie dużej ilości wody przez cały dzień jest zalecane, aby pomóc nerkom wypłukiwać toksyny7
- Unikanie kofeiny we wszystkich napojach7
- Zaprzestanie palenia – palenie może przyspieszyć progresję PKD21
Zmiana diety może pomóc w zarządzaniu niektórymi objawami. Lekarz może zalecić picie dodatkowych płynów, aby pomóc nerkom wypłukiwać odpady.22 Zmniejszenie spożycia soli pomaga kontrolować ciśnienie krwi u pacjentów z PKD, którzy mają nadciśnienie.13
Dializoterapia i przeszczep nerki
W przypadku postępu choroby do schyłkowej niewydolności nerek (ESRD), pacjenci wymagają dializy lub przeszczepu nerki.14
- Dializa – procedura wykonywana rutynowo u osób cierpiących na niewydolność nerek. Proces polega na usuwaniu z krwi substancji odpadowych i płynów, które normalnie są eliminowane przez nerki23
- Przeszczep nerki – procedura chirurgiczna polegająca na zastąpieniu chorej nerki zdrową nerką od innej osoby. Nerka może pochodzić od zmarłego dawcy lub od żywego dawcy23
Osoby z PKD, które mają przeszczep nerki, zwykle dobrze sobie radzą. Jednak nadal potrzebują dożywotniej opieki, aby monitorować nową nerkę i ogólny stan zdrowia.24
Kompleksowa opieka multidyscyplinarna
Opieka nad pacjentem z PKD wymaga podejścia zespołowego, w którym umiejętności nefrologa są wykorzystywane wraz z umiejętnościami innych specjalistów medycznych:25
- Nefrolodzy (specjaliści od chorób nerek)
- Gastroenterolodzy
- Urolodzy
- Chirurdzy transplantolodzy
- Radiolodzy
- Doradcy genetyczni
- Specjaliści od żywienia
- Pracownicy socjalni
- Psychiatrzy
- Wyspecjalizowane pielęgniarki
Zespołowe podejście, w którym wykorzystywane są umiejętności różnych specjalistów, zapewnia optymalną kompleksową opiekę.25 Pacjenci z PKD powinni również znaleźć nefrologa, który ma doświadczenie w leczeniu pacjentów z PKD.13
Wsparcie psychologiczne i grupy wsparcia
Osoby z PKD mogą potrzebować wsparcia psychologicznego w radzeniu sobie z chorobą przewlekłą:27
- Pozwolenie pacjentowi na wyrażenie swoich uczuć i obaw, szczególnie związanych z możliwą progresją choroby i niewydolnością nerek
- Zapewnienie wsparcia i poradnictwa
- Skierowanie pacjenta i jego rodziny do służb społecznych i socjalnych w celu uzyskania wsparcia
Warto rozważyć dołączenie do grupy wsparcia dla osób z chorobą policystyczną nerek. Może być bardzo pomocne usłyszeć, jak inni radzili sobie z tymi samymi problemami.28 Zapytaj zespół medyczny o grupy wsparcia w twojej okolicy.29
Badania przesiewowe i poradnictwo genetyczne
Badania przesiewowe są dostępne dla krewnych osoby z autosomalną dominującą postacią choroby policystycznej nerek (ADPKD). Decyzja o poddaniu się badaniu przesiewowemu w kierunku ADPKD powinna być omówiona z doświadczonym pracownikiem służby zdrowia i powinna obejmować pełną dyskusję na temat potencjalnych zagrożeń i korzyści.14
Zaleca się badanie członków rodziny pod kątem choroby policystycznej nerek. Ta choroba występuje rodzinnie. Chorobą można lepiej zarządzać, jeśli zostanie wykryta wcześnie.28
Jeśli masz PKD i zastanawiasz się nad posiadaniem dzieci, doradca genetyczny może pomóc ci poznać ryzyko przekazania choroby swoim dzieciom.30
Monitorowanie długoterminowe
Po rozpoznaniu PKD konieczne jest regularne monitorowanie w celu oceny funkcji nerek i progresji choroby:9
- Regularne wizyty kontrolne u nefrologa31
- Badania krwi w różnych odstępach czasu w celu monitorowania funkcji nerek16
- Badania obrazowe (USG, MRI, CT) w celu monitorowania wzrostu torbieli i funkcji nerek32
- Monitorowanie ciśnienia krwi – pielęgniarki monitorujące PKD będą regularnie mierzyć ciśnienie krwi33
- Badanie moczu w celu oceny krwiomoczu, białkomoczu i infekcji27
Częstotliwość wizyt ambulatoryjnych u nefrologa zależy od stopnia dysfunkcji nerek i od powikłań, takich jak brak przyrostu masy ciała, trudności żywieniowe i z karmieniem, nadciśnienie, zaburzenia elektrolitowe, infekcje dróg moczowych i zwłóknienie wątroby (tj. nadciśnienie wrotne).25
Badania kliniczne i nowe kierunki leczenia
Badania nad PKD koncentrują się na znalezieniu skutecznych metod leczenia, które mogą spowolnić postęp choroby.34 Trwające badania próbują odkryć nowe mutacje genetyczne związane z torbielami nerek, odkryć nowe biomarkery przebiegu choroby lub przeprowadzić kliniczne pomiary wyników poprzez dostęp do danych klinicznych i laboratoryjnych pacjentów, którzy wyrazili zgodę, w sposób pozbawiony identyfikacji.
Po omówieniu z lekarzem, pacjenci mogą rozważyć udział w badaniach klinicznych dotyczących nowych terapii PKD. Klinika PKD oferuje pacjentom możliwość uczestniczenia w badaniach medycznych mających na celu zbadanie nowych terapii mających na celu spowolnienie lub zatrzymanie wzrostu torbieli i dysfunkcji nerek.35
Zrozumienie czynników predykcyjnych szybkiej progresji tej choroby stało się coraz ważniejsze wraz z pojawieniem się potencjalnych nowych metod leczenia.20
Podsumowanie zasad opieki pielęgniarskiej
Opieka pielęgniarska w chorobie policystycznej nerek powinna obejmować:36
- Zapobieganie i/lub spowolnienie progresji choroby – identyfikowanie i zarządzanie czynnikami ryzyka jest najlepszym sposobem na zapobieganie lub spowolnienie progresji PKD
- Promocja dobrostanu fizycznego i psychospołecznego – zapewnienie edukacji dostosowanej do pacjenta, aby pomóc mu przejąć kontrolę nad swoim stanem i promować dobre samopoczucie
- Monitorowanie choroby i powiązanych procesów – badanie zdolności pacjenta do samodzielnego zarządzania przed i po sesjach edukacyjnych
Plany opieki opracowane wspólnie przez zaangażowanych pacjentów i zespół opieki mogą poprawić wyniki.37 Ważne jest również, aby pamiętać, że pacjenci z PKD są narażeni na wiele problemów psychospołecznych, takich jak lęk, depresja i stres.
W opiece pielęgniarskiej nad pacjentem z PKD należy monitorować potencjalne powikłania (takie jak przewodnienie, zaburzenia elektrolitowe i anemia), które będą wymagały leczenia.37 W przypadku pacjentów z zaawansowanym stadium PKD, którzy otrzymują hemodializę lub dializę otrzewnową, należy ocenić miejsca dostępu pod kątem wszelkich problemów.
Plany opieki pielęgniarskiej i interwencje mogą pomóc spowolnić postęp choroby nerek i wydłużyć funkcjonowanie nerek. Choć nie można odwrócić PKD, interwencje takie jak plan opieki pielęgniarskiej mogą pomóc spowolnić postęp i zapobiec wtórnym stanom, takim jak choroba serca.33
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Materiały źródłowe
- #1 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK373382/
Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threatening single-gene disease. It affects up to 15 million people worldwide with 50% risk for end-stage kidney disease, 80% risk for hypertension, 60% risk for painful kidney complications, 20% risk for symptomatic polycystic liver disease and 3% risk for intra-cerebral aneurysm rupture. […] For a long time, the treatment and management strategies of this disease have not progressed in comparison with the treatment of other kidney diseases. Recently, there have been new therapeutic hopes with identification of specific drugs based on the mechanisms of kidney progression. This chapter reviews the treatment and management of ADPKD progression, and the identification of ADPKD patients with rapidly progressing disease, hypertension, and extrarenal complications.
- #2 Kidneys – polycystic kidney disease (PKD) | Better Health Channelhttps://www.betterhealth.vic.gov.au/health/conditionsandtreatments/kidneys-polycystic-kidney-disease-pkd
Polycystic kidney disease (PKD) is an inherited condition characterised by the growth of cysts on the kidneys. […] There is currently no cure for PKD, but early detection and treatment can reduce or prevent some complications. […] PKD is the most common inherited kidney disease and is a common cause of Chronic Kidney Disease. […] Some people develop high blood pressure and kidney failure as a result of PKD. […] Currently there is no cure for PKD. However, early detection and treatment can reduce or prevent some of the complications of PKD. […] Self-care suggestions for PKD generally include: changing your diet this may help to manage some symptoms. […] Your doctor or healthcare professional will discuss best healthcare choices in managing this condition. Be guided by your doctor.
- #3 Chronic Kidney Disease Nursing Care Planshttps://www.healthline.com/health/kidney-disease/kidney-failure-care-plan
Nursing care plans for chronic kidney disease help nurses monitor the progression of the disease and provide treatment when signs or symptoms indicate that intervention is needed. […] Nursing care plans help healthcare professionals treat people with CKD. These plans provide guidelines for monitoring and maintaining specific levels of markers such as blood pressure, blood glucose, and fluid balance. […] Following CKD care plans can help relieve symptoms, prevent complications (such as secondary heart conditions), and slow disease progression. […] Nursing care plans help healthcare professionals monitor and manage CKD progression by identifying appropriate measurements of kidney-related functions specific to each stage of the disease. […] If your GFR does not fall within the identified range for your stage of CKD, healthcare professionals can begin interventions to help address the issue.
- #4 Nursing Interventions for Polycystic Kidney DiseaseNursing File | Nursing Filehttps://nursingfile.com/nursing-care-plan/nursing-interventions/nursing-interventions-for-polycystic-kidney-disease.html
Take patient history and perform assessment. […] Monitor vital signs especially blood pressure. […] Monitor renal function and urine elimination, hydration, fluid and electrolyte balance. […] Monitor daily weights. […] Assess edema and promote skin integrity. […] Access site for dialysis (if appropriate). […] Give prescribed drugs, including ACE inhibitors to control hypertension (if giving diuretics, obtain specimens for serum electrolyte levels, especially potassium, which may be decreased). […] Provide comfort measures, including opioid analgesics; assist the patient with relaxation techniques and the use of TENS. […] Provide fluids and foods based on the patients condition, encourage increased fluids if the patient has a urinary tract infection, and restrict fluids if the patient has renal failure.
- #5 Chronic Kidney Disease Nursing Care Planshttps://www.healthline.com/health/kidney-disease/kidney-failure-care-plan
Nurses keep track of output, which is how much and how often you urinate in relation to the amount of liquid you consume throughout the day. […] If you are not urinating enough, healthcare professionals may use interventions such as diuretic medication or insertion of a catheter. […] A nurse might also take steps to prevent infection, such as cleaning your pelvic area, and might provide education to you and your loved ones about urinary elimination in CKD. […] If you have CKD, nurses will continuously monitor you for this issue by weighing you, measuring your urine output, assessing any swelling, and listening to your breath sounds. […] If they find symptoms of excess fluid retention, they will begin interventions such as: restricting fluids, giving you diuretic medications, treating any areas of swelling to prevent skin breakdown, preparing you for dialysis treatments if you have stage 5 CKD.
- #6 Polycystic Kidney Disease, Glomerulonephritis – LevelUpRNhttps://leveluprn.com/blogs/medical-surgical-nursing/renal-4-polycystic-kidney-disease-glomerulonephritis?srsltid=AfmBOoqNpiALsL843WmRCk91SyV_o6BoAM-odj7NxcEgAUEkrDgSaJRB
Polycystic kidney disease is an autosomal dominant genetic disorder that causes the formation of fluid-filled cysts in the kidneys. And these cysts will compress and replace the healthy kidney tissue, and that results in decreased kidney function and eventually causes kidney failure. So signs and symptoms of this disorder include abdominal distension, flank or back pain, hypertension, and hematuria, so blood in the urine due to rupturing of the cyst. […] In terms of treatment, treatment is largely supportive because there is no cure for this condition. Medications that can be used include analgesics, antibiotics for infection, as well as ACE inhibitors or ARBs to help manage hypertension. […] In terms of nursing care, we’re going to want to closely monitor our patients’ eyes and nose as well as their daily weight, and then we may need to decrease their fluid, sodium, and protein intake until their kidneys heal.
- #7 Polycystic kidney disease (PKD) – Symptoms, causes, treatment | National Kidney Foundationhttps://www.kidney.org/kidney-topics/polycystic-kidney-disease
Polycystic kidney disease (PKD) causes fluid-filled cysts in the kidneys, leading to kidney damage and failure. […] PKD cysts can slowly replace much of the kidneys, reducing kidney function and leading to kidney failure. […] High blood pressure is the most common sign of PKD. […] Because high blood pressure can cause kidney damage, it is very important to treat it. In fact, treatment of high blood pressure can help slow or even prevent kidney failure. […] At present, there is no cure for PKD. However, a lot of research is being done. […] Studies also suggest that some treatments may slow the rate of kidney disease in PKD, but further research is needed before these treatments can be used in patients. […] In the meantime, many supportive treatments can be done to control symptoms, help slow the growth of cysts, and prevent or slow down the loss of kidney function in people with PKD. These include: Careful control of blood pressure, Prompt treatment with antibiotics for bladder or kidney infection, Drinking lots of fluid when blood in the urine is first noted, Medication to control pain, Healthy lifestyle with regard to quitting smoking, exercise, weight control and reduced salt intake, Drinking lots of plain water throughout the day, Avoiding caffeine in all beverages.
- #8 14 | The VN’s guide to caring for patients with polycystic kidney disease — Veterinary Internal Medicine Nursinghttps://www.veterinaryinternalmedicinenursing.com/blog/episode-14
We cant do anything to treat the patients polycystic kidney disease – draining the cysts is not practical, since there are so many, and they will continue to refill. Instead, we manage the chronic kidney disease our patient has as a result – our focus is going to be on correcting dehydration, acidosis and hypokalaemia, providing nutritional support, administering antiemetics and other supportive medications as needed, and managing any complications associated with the patients renal disease – such as anaemia, hypertension, and proteinuria. […] In terms of nursing care specific to PKD, the biggest area where we can support our patient and their families is in education. […] This starts at a young age – for example, if youre seeing an affected breed in for a puppy or kitten check, discussing potential screening.
- #9 Polycystic kidney disease (Causes, Symptoms and Treatment)https://patient.info/doctor/autosomal-dominant-polycystic-kidney-disease
Diagnosis of ADPKD has implications both for the patient and for their family. Consider the information needs of the patient and their family members. Provide written information and sources of support relating to the disease and be prepared to discuss the nature of the disease, possible complications and likely prognosis as well as the genetic implications for other family members. […] Care and monitoring would usually be undertaken jointly by primary care and renal teams. […] Current advice is that blood pressure should be controlled to a target of 130/80 mm Hg. Ongoing research is investigating whether a lower target (110/75 mm Hg) is effective at slowing disease progression in those with preserved function. […] Treat UTIs as they occur, as for the general population. […] Patients should become familiar with self-management for straightforward repeated episodes.
- #10 14 | The VN’s guide to caring for patients with polycystic kidney disease — Veterinary Internal Medicine Nursinghttps://www.veterinaryinternalmedicinenursing.com/blog/episode-14
And if their pet already has evidence of PKD, theyll need regular follow up (even in the absence of clinical signs) – with ultrasounds every 6-12 months depending on the individual, to evaluate progression of those cysts, and detect CKD at an early stage – so we can slow the progression of it as much as possible. […] Though theres nothing we can do to treat PKD itself, careful client education and communication, and regular reassessments to detect CKD at an early stage are vital. […] By performing genetic testing, we can not only detect patients with PKD and allow for prompt recognition of renal dysfunction, but we can also eliminate them from breeding programs, reducing the number of PKD patients in the future. […] Once a patient shows clinical signs for their PKD, theyre managed as we would any other CKD patient – and as we know, there are lots of opportunities to use our nursing skills with them.
- #11 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK373382/
Ensuring that Tolvaptan is used in a safe and effective manner requires multiple considerations, including the careful selection of patients eligible for treatment, based on risk of progression. Patient will need to be supported throughout treatment initiation and long-term management. […] Early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients. […] The best treatment of this disease is RAAS inhibitors with angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs). These agents remain the most recommended drugs to treat hypertension in patients with ADPKD. […] Therefore, early control of hypertension is very important in patients with ADPKD to slow down kidney disease progression and prevent occurrence of cardiovascular events.
- #12 Pediatric Polycystic Kidney Disease Treatment & Management: Approach Considerations, Long-Term Monitoringhttps://emedicine.medscape.com/article/983281-treatment
Survival of neonates depends on neonatal artificial ventilation and intensive care, as well as the degree of pulmonary hypoplasia. In order to optimize ventilation, fluid overload can be managed with diuretics, continuous renal replacement therapy, and nephrectomy. […] Systemic hypertension should be aggressively treated with antihypertensive medication. Angiotensin-converting enzyme (ACE) inhibitors are the drugs of choice. Calcium channel blockers, beta blockers, and the judicious use of diuretics are also potential options. Antibiotics are used to treat urinary tract infections. […] Once children with ARPKD develop chronic kidney disease, they require management of anemia with iron and erythropoietin; prevention of metabolic bone disease with calcium supplements, phosphate binders, and parathyroid-suppressing medication; and growth hormone to counter the growth-limiting effects of uremia.
- #13 Polycystic kidney disease (PKD) – Symptoms, causes, treatment | National Kidney Foundationhttps://www.kidney.org/kidney-topics/polycystic-kidney-disease
In April 2018, the FDA approved a new drug called tolvaptan for the treatment of autosomal dominant polycystic kidney disease (ADPKD). The drug can be used to help slow kidney function decline in adults at risk for this type of PKD. […] Reducing salt intake helps control blood pressure in patients with PKD who have high blood pressure. […] Physical exercise is recommended for people with PKD, however exercises that are potentially harmful to the kidney, such as contact sports, should be avoided. […] In addition to your primary care doctor, you should also find a nephrologist who has experience treating patients with PKD.
- #14 Patient education: Polycystic kidney disease (Beyond the Basics) – UpToDatehttps://www.uptodate.com/contents/polycystic-kidney-disease-beyond-the-basics
Low-salt diet â The amount of salt (sodium) ingested on a daily basis has been linked to more rapid progression of kidney disease in patients with ADPKD. […] Weight control â Patients who are overweight (a body mass index [BMI] of 25 to 30) and patients with obesity (BMI greater than 30) have faster progression of kidney disease in ADPKD. […] Tolvaptan â Tolvaptan is a type of drug called a vasopressin receptor antagonist. It has been shown to slow the decline in kidney function and reduce pain symptoms in people with ADPKD. […] End-stage kidney disease â Patients with ADPKD who progress to end-stage kidney disease require either dialysis or kidney transplantation. […] POLYCYSTIC KIDNEY DISEASE SCREENING â Screening tests are available to relatives of a person with autosomal dominant polycystic kidney disease (ADPKD). The decision to have a screening test for ADPKD should be discussed with an experienced health care provider and should include a full discussion of the potential risks and benefits.
- #15 Polycystic Kidney Disease (PKD) Symptoms, Treatments & Causes â American Kidney Fund (AKF)https://www.kidneyfund.org/all-about-kidneys/types-kidney-diseases/polycystic-kidney-disease
Polycystic kidney disease (PKD) is a genetic disorder that causes many fluid-filled cysts to grow in your kidneys, leading to kidney damage. […] There is no cure for ADPKD. Your doctor can recommend strategies to manage some of the symptoms and health problems caused by ADPKD. […] If you have ADPKD and are in pain, there are many ways to help you feel better. […] For long-lasting pain, your doctor might recommend stronger medicines like opioids or gabapentin, which can help with nerve pain. […] It’s important to treat hypertension because controlling high blood pressure has shown not slow the growth of kidney cysts in ADPKD. […] If you have hypertension and PKD, you can take steps to control your high blood pressure, which includes quitting smoking or using tobacco products and taking prescribed medicines for hypertension.
- #16https://www.nhs.uk/conditions/autosomal-dominant-polycystic-kidney-disease-adpkd/treatment/
There’s currently no cure for autosomal dominant polycystic kidney disease (ADPKD), and it’s not possible to stop cysts forming in the kidneys. […] If you’re diagnosed with ADPKD, you’ll usually see a kidney specialist, who can help draw up a suitable treatment plan. […] Medication is usually used to treat high blood pressure in people with ADPKD. […] In many cases of ADPKD, any pain you experience can be relieved by treating the underlying cause, such as kidney stones or a urinary tract infection (UTI). […] If you have severe, persistent or frequently recurring UTIs, you may need surgery to remove 1 or both of your kidneys, followed by dialysis or a kidney transplant. […] You’ll have blood tests at varying intervals to monitor your kidney function. […] Tolvaptan is a medication that’s recommended by the National Institute for Health and Care Excellence (NICE) to treat ADPKD in adults. […] If you have ADPKD, your kidneys will be more vulnerable to injury.
- #17 Polycystic Kidney Disease (PKD) | Tampa General Hospitalhttps://www.tgh.org/institutes-and-services/conditions/polycystic-kidney-disease-pkd
PKD treatment can vary based on the severity of the condition and the resulting symptoms. […] Possible options include: […] Healthy lifestyle practices, such as consuming a low-salt diet, exercising regularly, losing excess body weight and maintaining proper hydration levels to improve kidney health […] Medication to control high blood pressure […] Antibiotics to resolve urinary tract infections […] Surgery to remove painful kidney stones.
- #18 Polycystic Kidney Disease (PKD): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/5791-polycystic-kidney-disease
Polycystic kidney disease (PKD) is a genetic disorder that causes cysts to grow in the kidneys. Most people with PKD will eventually need dialysis or a kidney transplant. If you receive a PKD diagnosis, it’s important to work with your healthcare provider on a treatment plan to manage complications of the disease. […] There’s no cure for PKD. The goal of treatment is to slow the progression of the disease and control the symptoms it causes. The most common treatments for PKD include blood pressure management, dialysis, and kidney transplant. […] Living a healthy lifestyle can go a long way in managing PKD. Some tips for healthy living include eating a kidney-friendly diet, exercising, managing blood pressure, and taking all of your medication exactly as your provider prescribes. […] It’s important to work with healthcare providers who have experience treating polycystic kidney disease.
- #19https://myhealth.alberta.ca/Health/aftercareinformation/pages/conditions.aspx?hwid=uh4640
Finding out that you have kidney disease can be very upsetting. […] You have a type that causes fluid-filled bubbles (or cysts) to grow inside your kidneys. […] Your doctor can help you set up a treatment plan that can ease pain and help you stay active. Changes in your diet along with a good exercise program should help you stay healthy. […] Follow-up care is a key part of your treatment and safety. […] Take your medicines exactly as prescribed. […] Work with your doctor and dietitian to set up a diet that will be healthy for you. […] Your doctor may advise you to eat a low-protein diet, although this is not always recommended. […] Your doctor may recommend that you drink extra fluids to help your kidneys flush out the wastes. […] Talk to your doctor about joining a support group for people with polycystic kidney disease. […] Have your family members tested for polycystic kidney disease. This condition runs in families. The disease can be managed better if it is found early. […] Watch closely for changes in your health, and be sure to contact your doctor or nurse advice line if: You do not get better as expected.
- #20 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK373382/
Understanding predictors for rapid progression of this disease has become increasingly important with the emergence of potential new treatments. […] Patients with ADPKD should avoid violent sports. […] Strategies of treatment and management should be individualized for each ADPKD patient. […] Improvements in screening and diagnosis of ADPKD have allowed earlier diagnosis of disease, later onset of ESKD and better survival. However, the main and most effective therapy remains control of hypertension. Therefore, early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients.
- #21 Polycystic Kidney Disease – Melbourne Kidney Carehttps://www.melbournekidneycare.com.au/conditions/polycystic-kidney-disease/
Dietary changes. These may include reducing salt, protein, cholesterol, alcohol and caffeine. […] Smoking cessation. Smoking can speed the progression of PKD. […] Exercise. Movement assists with weight control and reduces the risk of complications. […] Antibiotic treatment for infection or UTIs and pain medications for pain that may accompany severe back pain, bleeding, stones or infection. […] Transplant or dialysis is used where End Stage Kidney Disease develops, which usually occurs over many years. […] A new medication has recently been approved and PBS listed with early stage CKD (2 to 3) and rapidly progressing ADPKD.
- #22 Polycystic Kidney Disease: Care Instructions | Kaiser Permanentehttps://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.polycystic-kidney-disease-care-instructions.uh4640
Finding out that you have kidney disease can be very upsetting. […] Your doctor can help you set up a treatment plan that can ease pain and help you stay active. Changes in your diet along with a good exercise program should help you stay healthy. […] Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments, and call your doctor if you are having problems. It’s also a good idea to know your test results and keep a list of the medicines you take. […] Work with your doctor and dietitian to set up a diet that will be healthy for you. Your doctor may advise you to eat a low-protein diet, although this is not always recommended. […] Your doctor may recommend that you drink extra fluids to help your kidneys flush out the wastes. Be sure to drink the amount of fluid your doctor advises.
- #23 Polycystic Kidney Disease Treatment | Baptist Health | Arkansashttps://www.baptist-health.com/services/kidney-care/polycystic-kidney-disease
Polycystic kidney disease (PKD) is a genetic disorder characterized by the growth of numerous cysts filled with fluid in the kidneys. PKD cysts can reduce kidney function, leading to kidney failure. People with PKD can also have cysts in the liver and problems in other organs such as the heart and blood vessels in the brain. Treatment for polycystic kidney disease may include pain medication, surgery, treatment for high blood pressure and others. […] Treatment for polycystic kidney disease may include pain medication, surgery to shrink cysts and relieve pain, treatment for high blood pressure, treatment for urinary tract infections and in some cases, dialysis and kidney transplantation. […] Dialysis is a procedure that is performed routinely on people who suffer from kidney (renal) failure. The process involves removing waste substances and fluid from the blood that are normally eliminated by the kidneys. […] A kidney transplant is a surgical procedure performed to replace a diseased kidney with a healthy kidney from another person. The kidney may come from a deceased donor or from a living donor. Individuals who donate a kidney can live healthy lives with the kidney that remains.
- #24 Polycystic Kidney Disease (PKD) | University of Utah Healthhttps://healthcare.utah.edu/kidney-nephrology/polycystic-kidney-disease
Medication can help people with PKD manage their symptoms: […] People with PKD should maintain a healthy weight and follow a balanced, low-sodium diet: […] If you develop kidney failure, you will need dialysis to remove waste and extra fluid from your blood. You will also need a kidney transplant to prolong your life. […] People with PKD who have a kidney transplant tend to do well. However, they still need lifelong care to monitor their new kidney and overall health. […] PKD prevents your kidneys from removing waste from your body. This waste can build to toxic levels and cause a condition called uremia. You may develop kidney failure and need a transplant if uremia progresses. Our nephrologists will work closely with our kidney transplant surgeons to give you high-quality, seamless care.
- #25 Pediatric Polycystic Kidney Disease Treatment & Management: Approach Considerations, Long-Term Monitoringhttps://emedicine.medscape.com/article/983281-treatment
Once children are in end-stage renal disease, dialysis or transplantation is the only option. Renal transplantation may be necessary in a large number of patients with ARPKD. […] The primary care physician and consulting nephrologist should participate in the care of children and adults with polycystic kidney disease. Once polycystic kidney disease is diagnosed, the frequency of outpatient follow-up with the nephrologist depends on the degree of renal dysfunction and on complicating features, such as a failure to thrive, nutritional and feeding difficulties, hypertension, electrolyte disturbances, urinary infections, and hepatic fibrosis (ie, portal hypertension). […] A team approach in which the skills of the nephrologist are used together with those of other medical specialists (eg, gastroenterologist), specialized nurses, nutritionists, social workers, psychiatrists, and other support staff provides optimal comprehensive care.
- #26https://umiamihealth.org/en/treatments-and-services/nephrology/polycystic-kidney-disease
Polycystic kidney disease is an inherited condition in which clusters of cysts, or fluid-filled sacs, develop in the kidneys. As cysts continue to multiply over time, they can cause the kidneys to enlarge and lose their function. These cysts can become infected or they can bleed and rupture. When the kidneys are massively enlarged, patients can develop flank pain or discomfort. Complications from this disease can lead to cardiovascular disease and kidney failure. […] University of Miami Health System nephrologists (kidney specialists) provide advanced treatments for polycystic kidney disease, including treatments to help slow the rate of kidney cyst growth, prevent declining kidney function, and manage the signs, symptoms, and complications of polycystic kidney disease. […] The ADPKD Center of the University of Miami provides comprehensive, multidisciplinary and individualized care for patients and families affected by ADPKD. Our integrated care team includes key specialists with expertise in ADPKD, including nephrologists, hepatologists, cardiologists, geneticists and genetic counselors, interventional radiologists, pain management specialists, obstetricians (high-risk pregnancy), neurologists and neurosurgeons.
- #27 Nursing Interventions for Polycystic Kidney DiseaseNursing File | Nursing Filehttps://nursingfile.com/nursing-care-plan/nursing-interventions/nursing-interventions-for-polycystic-kidney-disease.html
Provide supportive care to minimize symptoms. […] Obtain specimens for urinalysis and culture and sensitivity as ordered to evaluate for hematuria, proteinuria, and infection; obtain specimens for laboratory tests, such as electrolyte levels, as ordered. […] Individualize patient care, as appropriate. […] Allow the patient to verbalize his feelings and concerns, especially related to possible progression of the disease and renal failure; provide support and guidance. […] Prepare the patient for dialysis or renal replacement therapy as indicated. […] Encourage the parents of a child with the infantile form to obtain genetic counseling. […] Prepare the patient and his family for possible renal transplant or surgery. […] Refer the patient and his family to community and social services for support.
- #28 Polycystic Kidney Disease: Care Instructions | Kaiser Permanentehttps://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.polycystic-kidney-disease-care-instructions.uh4640
Talk to your doctor about joining a support group for people with polycystic kidney disease. It can be very helpful to hear how others have dealt with the same problems. […] Have your family members tested for polycystic kidney disease. This condition runs in families. The disease can be managed better if it is found early. […] Watch closely for changes in your health, and be sure to contact your doctor if you do not get better as expected.
- #29 Polycystic kidney disease – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/diagnosis-treatment/drc-20352825
Our caring team of Mayo Clinic experts can help you with your polycystic kidney disease-related health concerns […] Treating polycystic kidney disease involves dealing with the following symptoms and complications in their early stages: […] Early treatment offers the best chance of slowing the progress of polycystic kidney disease. […] Ask your healthcare team about support groups in your area. […] Your care team is likely to ask you questions, such as: […] For polycystic kidney disease, questions to ask include:
- #30 Polycystic kidney disease – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/symptoms-causes/syc-20352820
Polycystic kidney disease (PKD) is a condition in which clusters of cysts grow in the body, mainly in the kidneys. Over time, the cysts may cause the kidneys to get bigger and stop working. PKD is most often passed through families. This is called an inherited condition. […] Lifestyle changes and treatments might help reduce damage to the kidneys. […] If you have polycystic kidney disease and you’re thinking about having children, a genetic counselor can help you know your risk of passing the disease to your children. […] Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. It’s most important to manage your blood pressure. […] Here are some tips for keeping your blood pressure in check: Take the blood pressure medicines your healthcare professional prescribes as directed. Eat a low-salt diet that has plenty of fruits, vegetables and whole grains. Get to and stay at a healthy weight. Exercise regularly. Aim for at least 30 minutes of moderate physical activity most days of the week. Limit alcohol use. Don’t smoke. […] Polycystic kidney disease care at Mayo Clinic.
- #31 Polycystic Kidney Disease (PKD) Symptoms, Treatments & Causes â American Kidney Fund (AKF)https://www.kidneyfund.org/all-about-kidneys/types-kidney-diseases/polycystic-kidney-disease
After you find out that you have ADPKD, it is important to work with your doctor to understand your risk for progressing to kidney failure and learn about what you can do to manage it. […] Work with your doctor to prevent or manage diabetes and high blood pressure. […] Talk to your doctor about all the options to manage your ADPKD. You can also ask your doctor if there are any clinical trials studying treatments for ADPKD that you could take part in. […] Caregivers can become an essential part of life for people with ADPKD who have kidney failure. […] Taking care of yourself is just as important as taking care of your loved one.
- #32 Polycystic Kidney Disease Clinic – Nephrology | Loma Linda University Healthhttps://lluh.org/services/nephrology/polycystic-kidney-disease-clinic
Our clinic primarily focuses on diagnosing, managing, and treating PKD and related conditions, including: Autosomal dominant polycystic kidney disease, Chronic kidney disease (CKD), High blood pressure (hypertension), Urinary tract infections (UTIs), Kidney stones, Hematuria (blood in urine), Liver cysts, Pain management, End-stage renal disease. […] Comprehensive evaluation, including imaging studies such as ultrasounds, MRIs, and CT scans to monitor kidney cyst growth and function. […] Assistance in understanding the genetic aspects of PKD, predicting risks for family members, and making informed decisions about family planning. […] Monitoring and treatment plans for controlling hypertension, a common complication in PKD patients. […] Addressing and alleviating pain caused by growing cysts or related complications through various therapeutic methods.
- #33 Chronic Kidney Disease Nursing Care Planshttps://www.healthline.com/health/kidney-disease/kidney-failure-care-plan
Nurses will monitor your lab results and other vital signs daily to maintain this balance. […] If they suspect an imbalance, they may begin interventions such as providing lactose solution, giving diuretic medications, and suggesting dietary changes. […] Nurses monitoring your CKD will take your blood pressure regularly. […] Interventions might include diuretic medications and education on dietary and lifestyle changes. […] Its important to work closely with your medical team to manage symptoms of CKD. […] If your healthcare team has advised you to take these steps, its important to track your results and report any changes to your care team. […] Nursing care plans and interventions can help slow the progression of kidney disease and prolong kidney function. […] You cant reverse CKD, but interventions such as a nursing care plan can help slow the progression and prevent secondary conditions such as heart disease.
- #34 Polycystic Kidney Disease Clinic – Division of Nephrologyhttps://nephrology.wustl.edu/patient-care/clinics/polycystic-kidney-disease-clinic/
Ongoing research attempts to uncover novel genetic mutations associated with kidney cysts, discover new biomarkers of disease course, or perform clinical outcomes measures by accessing clinical and laboratory data of consented patients in a de-identified manner. […] Collaboration with the clinical services allows for the recruitment of patients interested in helping researchers investigate the cause and mechanisms of cystic kidney diseases (currently more than 200 patients).
- #35 Polycystic Kidney Disease Clinic – Division of Nephrologyhttps://nephrology.wustl.edu/patient-care/clinics/polycystic-kidney-disease-clinic/
The Washington University Polycystic Kidney Disease (PKD) Clinic specializes in the care of patients with PKD, a common genetic disease affecting more than 12 million people worldwide. […] The focus of this clinic encompasses not only the kidney manifestations, but also extends to include the management of hypertensive, liver and vascular complications of the disease. […] Our close interaction with the transplant team also allows patients with progressive disease to minimize or even eliminate the need for dialysis as a bridge to transplantation. […] The clinic also provides patients with the opportunity to participate in medical trials designed to investigate novel therapies to slow or halt cyst growth and renal dysfunction. […] The PKD clinic is headed by Dr. Seth Goldberg. […] The Division of Nephrology at Washington University School of Medicine is at the cutting edge of research on cystic diseases affecting the kidney at both the basic science level and in clinical applications by translating these discoveries to patient care.
- #36 Adults with chronic kidney disease: Overview and nursing care goalshttps://www.myamericannurse.com/adults-with-chronic-kidney-disease-overview-and-nursing-care-goals/
Nurses care for adults with various stages of CKD in a variety of inpatient and outpatient settings. Regardless of CKD stage, the three main nursing care goals are: […] The three main nursing care goals are preventing and/or slowing disease progression, promoting physical and psychosocial well-being, and monitoring disease and related processes. […] Identifying and managing risk factors is the best way to prevent or slow CKD progression. […] Provide patient-specific education to help patients take charge of their condition and promote well-being. […] Throughout disease progression, ask patients about their future treatment plans. […] Maintaining a healthy weight is key to CKD self-management. […] For patients receiving dialysis, provide education about access site protection. […] Focus education for patients receiving a kidney transplant on preventing organ rejection.
- #37 Adults with chronic kidney disease: Overview and nursing care goalshttps://www.myamericannurse.com/adults-with-chronic-kidney-disease-overview-and-nursing-care-goals/
Care plans developed collaboratively between engaged patients and the care team can improve outcomes. […] Assess patients self-management abilities before and after educational sessions. […] Keep in mind that patients with CKD are at risk for many psychosocial issues, such as anxiety, depression, and stress. […] CKD can result in several complications (such as fluid overload, electrolyte imbalances, and anemia) that will require treatment. […] For patients with late-stage CKD who are receiving hemodialysis or peritoneal dialysis, assess access sites for any issues. […] Patients always have the right to refuse treatment or choose palliative care.