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A care pathway that patients can understand and use
The day began with a presentation on the development of an ADPKD care pathway based on the KDIGO clinical practice guideline and developed through collaboration involving KDIGO and ERKNet.
The central idea is deliberately simple: a care pathway should provide an accessible map of what should happen at each stage of the disease. It should show where care can appropriately be delivered, when specialist expertise is needed, what information should be provided, and which discussions should take place with patients and families. The ambition is to make this available in a clear A4 format that can be used by patients, clinicians and healthcare authorities.
The pathway is organised around two axes. The first follows disease progression—from presentation and diagnosis through to advanced kidney disease, dialysis and transplantation.
The second describes the appropriate level of care, from primary care and general nephrology to specialist and expert-centre involvement.
Participants strongly supported the principle that expert centres are essential, but should not become the only place in which routine care can be delivered. The number of specialists is limited and many academic centres are already under significant pressure. Good care therefore depends on effective cooperation between primary care, general nephrologists and expert teams.
Primary-care physicians and general nephrologists can undertake many important elements of care, including taking a family history, arranging initial imaging, monitoring blood pressure, performing urine tests and providing routine chronic kidney disease management. However, the pathway also needs to make clear when referral to an expert centre should be accelerated.
The “red flags” discussed included:
- very early or unusually severe disease;
- a rapid decline in kidney function;
- syndromic or atypical features;
- severe extra-renal manifestations, including significant polycystic liver disease;
- complex questions about genetics or diagnosis;
- family planning, reproductive options and pregnancy;
- assessment of an at-risk child or other family member.
There was broad agreement that every person with ADPKD should ideally have access to an expert consultation at the beginning of follow-up or, at minimum, at least once during their lifetime. Such a consultation should not focus only on kidney measurements. It should provide comprehensive information, consider the wider family, identify individual risks and open the door to clinical trials, registries and emerging treatment options.
The pathway also underlines the importance of multidisciplinary care. Pain, cyst infection, liver involvement, kidney stones, intracranial aneurysms, genetics, fertility, pregnancy, psychological support, dialysis and transplantation may each require different expertise.
Transition from paediatric to adult care was highlighted as an especially vulnerable period.
Between approximately 15 and 25 years of age, young people are making major educational, professional, social and personal decisions. Losing them to follow-up during this period can have lifelong consequences.
Participants welcomed the plan for a simplified patient version of the pathway, written in clear language and suitable for translation. A pathway is not only a clinical tool: it can help patients advocate for themselves, ask informed questions and show healthcare authorities where services or coordination are missing.
At the same time, the discussion identified an important omission. Psychosocial support may be described as essential, but it is still rarely embedded in routine care or adequately reimbursed. Participants argued that the pathway should make this need more visible and support advocacy for appropriately resourced psychological and social care.