Niccum Haag Highlights Importance of Patient Education For Patients With VHL-Associated Tumors


Heather Niccum Haag, BSN, RN, CCRN, shares how her institution improved patient education with the treatment belzutifan.

Heather Niccum Haag, BSN, RN, CCRN

Heather Niccum Haag, BSN, RN, CCRN

Clear and concise patient education is important for all patients with cancer, but especially for those with rare tumors, according to Heather Niccum Haag, BSN, RN, CCRN.

During the 48th Annual Oncology Nursing Society Congress, Niccum Haag shared her experience collaborating with a neuro-oncology pharmacist, neuro-oncologist, and patient education department at her institution to create the patient handout based on the FDA drug label for belzutifan (Welireg) for the treatment of von Hippel-Lindau (VHL) disease. The handout includes key information, such as how medicine doses are decided, how treatment will be given, how this medication should be stored in a patient’s home, the possible adverse events (AEs), and when the patient should call their doctor.

Prior to belzutifan’s approval in 2021, VHL was treated with either radiation or surgery, Niccum Haag explained. Since belzutifan is the first systemic treatment that these patients will have receive for their cancer, readily available education is of paramount importance.

“What I want others to take away from this is that even when something is new and there’s limited information available, you need to take what you have available so that your patients can understand the risks and benefits of the treatment options your team is providing them,” Haag said.

In an interview with Oncology Nursing News®, Niccum Haag, a clinic nurse at The Ohio State University Comprehensive Cancer Center Wexner Medical Center — James Cancer Hospital and Richard J. Solove Research Institute, discussed challenges with treating VHL and ways to better educate patients on the disease as well as treatments with belzutifan.

Oncology Nursing News: What is belzutifan’s mechanism of action?

Niccum Haag: We all have 2 copies of the VHL gene. VHL disease occurs when someone inherits 1 pathogenic variant of that disease and then throughout their lifetime the normal gene develops the variant. When 2 copies are present, you start to see the tumorigenesis in multiple organ systems within the body.

When that gene has a pathogenic variant it develops a couple of proteins, 2 of which are the hypoxia inducible factor 1-alpha and the hypoxia inducible factor-2 alpha [HIF2α]. Belzutifan binds to that receptor on the HIF2α—blocking the transcription between those 2 proteins to inhibit tumorigenesis.

What are some of the biggest questions that patients have that you wanted to educate them about in this format?

The biggest questions are how do they take the medications and what AEs [should they] expect? What’s unique about belzutifan is that it is a first-in-class drug and this patient population has never had any medical management before—aside from surgical interventions and, possibly, radiation along the way; they have never had a medication that they can take at home by mouth [or] an IV infusion in an infusion clinic—this is a first for them.

A lot of the questions generated are: “What are your other patients like?” and “What are they experiencing on this drug?” Other questions that occur are: “What happens if I have an AE and I have to stop [treatment], is the tumor going to grow out of control and have that rebound effect?”

How did the different reviewers come together for this project?

I led this project as the clinic nurse managing the day-to-day care of these patients, but we had our education department [and] clinical nurse specialist who helped us connect all the parts to get the patient facing education document together. In a larger academic institution, there are more avenues and pathways you have to take [compared with what] a smaller rural hospital would use.

Our clinic pharmacists helped us make sure that we were using the FDA drug label with prior research that’s been done for this population to highlight the AEs and major interactions that are known. Our neuro-oncologist oversees the medical management of these patients, so having all of those vantage points provides a comprehensive picture for the patient.

What are some of the greatest challenges in treating patients with VHL-associated tumors?

Because this is a rare disease, there are not as many centers that are equipped to care for these patients. A lot of patients travel far to come to our clinic. These patients also have seen neurosurgery in the past. [Additionally, because of] the renal cell cancer that presents in this disease process too, they see urology, endocrinology, and ophthalmology.

When patients are coming from a long distance, coordinating all those appointments between different providers is challenging and because this is an autosomal dominant inheritance pattern, coordinating care among families has helped facilitate some of that adherence. Any education that we are providing to patients helps facilitate adherence, and when patients are adherent to their regimens as intended that helps improve their outcomes.

What would you like colleagues to take away from this work?

The work that I put together for this project with my colleagues is very specialized to our patient population, but there are other rare diseases out there. There are always going to be new drugs coming to the market and new medical devices. Answering patients’ questions so that they can appropriately call you and notify you if they’re having any AEs [is important] so you can intervene as appropriate and facilitate their adherence.

One thing I would like to highlight is [that] even though this drug is only indicated for the VHL population right now for their central nervous system hemangioblastomas, there are many other studies going on right now to see if there is any benefit for other cancer populations. So we may see this, in the future, applied to different patient populations.


Niccum Haag H. New drug, old problems: development of an education plan for patients and nurses for new to market drug, belzutifan, for management of von-Hippel Lindau tumors. Poster presented at: 48th Annual Oncology Nursing Society Congress; April 26-30, 2023; San Antonio, TX. Accessed May 30, 2023.

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