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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.

A lifelong disease, patients develop clusters of cysts -- noncancerous round sacs containing water-like 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 in recent years has led to improved tools for diagnosing and predicting the course of the disease, as well as new treatments that may slow the progression of ADPKD in some people.  

With input from nephrologists across the province, the BC Renal Agency is undertaking a number of initiatives to evaluate and improve the care of people living with ADPKD across the province, including:

  • Improving understanding of ADPKD in BC
  • Improving access to accurate and reproducible diagnostics
  • Guiding and evaluating use of new treatments
For more information and education on management of PKD view the ADPKD presentations at ukidney.com 

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 the BC Renal Agency 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, the BC Renal Agency 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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New Imaging Tools

Kidney imaging plays a key role in diagnosing and predicting the course of kidney disease in patients with ADPKD. 

Based on recent medical advances, two initiatives are underway to improve access and optimize the utility of renal imaging of patients with polycystic kidney disease in BC.

  • Standardization of radiographic reporting in ADPKD: Standardized criteria in radiographic diagnosis and reporting of images in ADPKD will support equitable access to assessments across all clinical sites in BC, and will reassure clinicians that the reports are validated, reproducible and can reliably inform decisions.

 

  • Standardized evaluation of Total Kidney Volume (TKV) in patients with ADPKD: TKV has emerged as a strong indicator of disease status, prognosis and progression in ADPKD and accurate TKV reporting will help BC nephrologists provide individualized care to patients with ADPKD. An evaluation of the accuracy and feasibility of different methods of TKV reporting is currently underway with the goal of making standardized and accessible TKV measurement available across the province. 

Below is a link to an online calculator for the Mayo Imaging Classification of ADPKD.  This classification is a validated method to use imaging to classify patients into categories that predict the rate of progression of their kidney disease. 

While standardization of kidney volume reporting in BC is in progress, discussion with your local radiologist when using currently available imaging modalities can yield the information necessary to use imaging based classifications. 

Online tool for TKV calculation and Mayo imaging classification of ADPKD

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 Agency. 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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