Adherence to clinical surveillance guidelines in von Hippel-Lindau (vHL) disease: Evaluating the impact of a coordinated care centre implementation in Canada.

Abstract

460 Background: VHL is a rare hereditary cancer syndrome, predisposing to the development of multiorgan tumour growth, including bilateral and recurrent clear cell renal cell carcinoma. While guidelines for the screening and management of these patients exist, adherence to guidelines is variable. The development of coordinated clinical care centres with multidisciplinary expertise in rare diseases has been demonstrated to improve outcomes. Methods: In this study, we reviewed adherence to surveillance guidelines prior to and after development of a Canadian provincial vHL clinic at our institution, British Columbia (BC) Cancer. Care in this clinic is provided both in-person and through telehealth. We sought to evaluate whether patient geographic distance from the clinic impacted time to being seen for consultation, and whether there was a change in management plan after being seen in the clinic. Results: Between June 2022, and April 2024, 57 patients were seen in the vHL clinic (median age 34, 58% female, and 42% male). Of these, 13 lived within 50km from the clinic, 7 within 50-100km, 8 within 100-500km, and 29 lived more than 500km away. We found significant improvement in adherence to nearly all surveillance guidelines after the development of the vHL Clinic (table). 10.5% of patients had never had germline confirmation of vHL and were referred for expedited testing after being seen. We did not find any difference in time from referral to consultation based on patient geographic proximity to the clinic, F(3,53)=1.10, p =0.35 nor the modality of initial consultation (t(55)=1.07, p =0.29; in person vs telehealth). Finally, after initial consultation, 87% of patients had a change in their management plans, most commonly involving referral for further subspecialty evaluation (n=41) or initiation of pharmaceutical treatment (n=24). Conclusions: Our results add to existing evidence that coordinated care centres with multidisciplinary expertise in vHL are feasible in the Canadian public healthcare system and can result in improvements in quality of care. Our clinic model has a robust multidisciplinary care team that utilizes technology to reach patients for equitable access to care. Future work will evaluate potential cost savings to the healthcare system with this model, as well as patient satisfaction with their care pre and post participation in the vHL clinic. Adherence to clinical screening guidelines for patients with vHL before and after consultation with the BC cancer provincial vHL clinic. Screening Guideline Parameter Pre connection with vHL clinic Post connection with vHL clinic Central nervous system imaging 51.8% 100%* Abdomen/Pelvis imaging 44.6% 100%* Metanephrines 8.9% 64.3%* Ophthalmologic 75.0% 85.7% Audiology 21.4% 85.7%* *Statistically significant difference ( p <0.05).