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Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder, and is the fourth leading cause of end-stage renal disease in Canada.


Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder and is the fourth leading cause of end-stage renal disease in Canada.

A lifelong disease, patients develop clusters of cysts -- noncancerous round sacs containing fluid. The disease is quite variable, from minimal impact on kidney function to rapidly progressive disease that results in kidney failure at a young age.

There is no cure for ADPKD at this time, but research has led to improved tools for diagnosing and predicting the renal outcome of the disease, as well as treatments that may slow the progression of ADPKD in some people.  

As part of BC Renal's overarching priorities of optimizing patient experience and outcomes as well as innovation and research in renal care, we have developed and implemented a provincial ADPKD strategy in BC's renal programs that supports equitable and sustainable care for patients and families living with ADPKD.

This includes:

  • An ADPKD advisory group of interdisciplinary renal health professionals from across the province to support program development and implementation
  • A framework for delivery of best practices in ADPKD care within BC Kidney Clinics, which complements and enhances existing Kidney Care Clinic best practices
  • Educational tools and resources that support delivery of interdisciplinary ADPKD care in collaboration with other healthcare disciplines.
  • A comprehensive provincial registry of ADPKD patients that informs ongoing quality improvement and research efforts.
  • A dissemination strategy that supports BC Renal knowledge sharing and research efforts.

For more information and education on management of PKD view the ADPKD presentations at https://ukidney.com/nephrology-resources/adpkd-channel 

Guidelines & Tools

The ADPKD best practice guideline outlines how the existing experience and strong network of Kidney Care Clinics across the province can be utilized as the framework for the care of patients with ADPKD. These best practices complement the existing Kidney Care Clinic best practices by providing tools and strategies tailored to the needs of patients and families living with ADPKD.  This framework will enable consistent and sustainable delivery of best-practices in ADPKD care within the scope of existing Kidney Care Clinic services. 

Multiple ADPKD-specific clinic tools and staff resources as well establishment of the ADPKD clinic network have been created to support this standardized delivery of interdisciplinary ADPKD care.  These guidelines and clinical tools are made for use in every Kidney Clinic in BC, allowing patients and families to access best practices in ADPKD care wherever they live and receive their usual kidney care.  

  • Relevant Publications
    • Bevilacqua, M. U., Hague, C. J., Romann, A., et al. CT of Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: Accuracy, Reproducibility, and Radiation Dose. Radiology, 2019; 291(3), 660–667. doi: 10.1148/radiol.2019181830.  https://pubs.rsna.org/doi/abs/10.1148/radiol.2019181830 
    • Soroka S, Alam A, Bevilacqua M, et al. Updated canadian expert consensus on assessing risk of disease progression and pharmacological management of autosomal dominant polycystic kidney disease. Can J Kidney Health Dis. 2018;5:2054358118801589. http://journals.sagepub.com/doi/10.1177/2054358118801589 
    • Chebib, F. T., Perrone, R. D., Chapman, A. B., et al. A Practical Guide for Treatment of Rapidly Progressive ADPKD with Tolvaptan. Journal of the American Society of Nephrology (JASN), 2018; 29(10), 2458–2470. doi: 10.1681/asn.2018060590. http://www.jasn.org/lookup/doi/10.1681/ASN.2018060590
    • Chebib, F. T., & Torres, V. E. Recent Advances in the Management of Autosomal Dominant Polycystic Kidney Disease. Clinical Journal of the American Society of Nephrology, 2018; 13(11), 1765–1776. doi: 10.2215/cjn.03960318. http://cjasn.asnjournals.org/lookup/doi/10.2215/CJN.03960318


ADPKD Registry

BC has developed a first of its kind registry that aims to include all British Columbians with polycystic disease, regardless of disease or treatment status. The comprehensive clinical data made available through the registry will support improved individual patient care and improve our understanding of the disease, which will benefit all people living with ADPKD.

The registry is housed within PROMIS, a powerful information system managed by BC Renal that serves as a database for British Columbians with kidney disease. In the past, people living with ADPKD were enrolled in PROMIS at later stages of the disease when they required more specialized nephrology care. While this model works well for other forms of kidney disease, in ADPKD, new treatments and changes in care are likely to be of most benefit when initiated early in the disease course. Enhancing data capture at this stage of the disease will enable better patient care.  

Through the ADPKD registry, aggregate and anonymized data on patient demographics, clinical characteristics, treatments and outcomes will be available to all nephrologists in the province to help assess patient care and enable quality improvement. As the data steward, BC Renal will ensure best practices and patient privacy.

For patients or family members living with ADPKD who have questions about this registry, please see the frequently asked questions document below. 

Information on Enrollment in the ADPKD Registry
At present, registration in the database is not automatic; this must be completed at the point of care.  For clinical staff involved in this process, the following link contains instructions for enrolment of your patients in the ADPKD registry. To enhance the data collected for the registry, we ask programs to complete and information form when patients are seen in the office, and fax it or the current clinic dictation in for upload into the registry.  This information sheet is also available below.

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Tolvaptan for ADPKD

The vasopressin receptor antagonist tolvaptan has been approved for use in Canada for the treatment of ADPKD. Given limited data, the medication is not indicated for all patients with ADPKD as only a subset of ADPKD patients may benefit. Based on its mechanism of action and the current studies available, patients most likely to benefit are those who are younger, with evidence of enlarged kidneys but preserved kidney function. Patients with slowly progressive disease who are not likely to reach ESRD are not likely to benefit from tolvaptan and the drug has not yet been studied in those with advanced renal dysfunction.

For information and suggestions regarding the use of tolvaptan in ADPKD, please refer to the patient and prescriber FAQ documents linked below.  For more detailed information on assessing renal prognosis in ADPKD and identifying candidates for treatment, refer to the Canadian consensus recommendations on ADPKD management found here.

Mandatory monitoring must be completed when using tolvaptan.  A consent to drug use and hepatic monitoring are required, and bloodwork must be performed prior to each refill. Details of this process are outlined in the documents linked below, including the consent form, a request for bloodwork monitoring and a pre-printed prescription form.

To further enhance safety and outcome monitoring, we ask that all patients on tolvaptan be registered in PROMIS

At this time, tolvaptan is not funded by either BC PharmaCare or the BC Renal Network. Some private insurers have listed tolvaptan; we suggest that potential patients discuss this with their drug insurance provider prior to initiating treatment with tolvaptan.  They should inquire about coverage as well as any lifetime maximums that may apply.  Once a prescription is completed, the distributor will also ensure that funding is in place before dispensing tolvaptan.  This may result in a delay of up to several weeks between prescribing and the first delivery of tolvaptan. 

 

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