Von Hippel-Lindau (VHL) disease has been with me since I was 5 years old, when a benign tumor was found on the optic nerve of my left eye, leaving me blind in that eye. But I didn’t get an official diagnosis of the disease until 2011, when I was 20. By then, it was like a light switch had turned on, and I began developing tumors in my central nervous and endocrine systems, kidneys, and brain. The pace of the growth of new tumors was relentless. Each time I went in for an imaging scan every 3 to 6 months, another new tumor was detected.
By 2011, I was having surgeries in quick succession. The most intense by then was an adrenalectomy to remove benign growths on my adrenal glands. Then, in 2015, I was diagnosed with renal cell carcinoma and in 2016, with pancreatic neuroendocrine carcinoma.
“For my entire life, my mantra has been to stay positive and continue to move forward. Honestly, it’s a message everyone can benefit from, whether you have cancer or not.”— Alexandra Vitale
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These years were probably the hardest for me emotionally because it was the first time the word “cancer” was used to describe the tumors. The surgeries to extract the malignancies, a pancreaticoduodenectomy (more commonly known as a Whipple procedure) to remove the neuroendocrine carcinomas and a partial nephrectomy to remove the renal cell carcinomas in my kidney, were also extremely difficult to recover from physically.
Rolling With the Punches
Prior to undergoing these surgeries, an imaging scan detected yet another tumor, this time on my cerebellum. “I wouldn’t be surprised if we have to do something about that tumor in the coming year,” said my surgeon. To avoid becoming overwhelmed by the seemingly endless development of tumors throughout my body, I decided to focus on the surgeries in front of me and deal with the others later. In December 2016, I had the Whipple and nephrectomy procedures, and 8 months later, I had brain surgery to remove the tumor in my cerebellum, which had begun pushing against my spinal cord, affecting my coordination. Fortunately, that tumor was benign.
Although these tumors were found and removed before they could become life-threatening, the toll they were taking on my mental health was crushing. Every time I had to have a new magnetic resonance imaging scan or a meeting with my physician, I was filled with fear. But I decided to just roll with the punches. What else could I do?
Staying Free of New Tumors
In 2018, another tumor (3 cm) cropped up on one of my kidneys, which made me eligible for a phase II study investigating PT2977, MK-6482, now known as belzutifan, a small-molecule inhibitor of hypoxia-inducible factor 2α, in patients with von Hippel-Lindau disease–associated renal cell carcinoma (ClinicalTrials.gov identifier NCT03401788). The drug has been successful in shrinking the tumor to just 1 cm and stabilizing it, so I most likely will not have to have additional surgery to remove it.
Remarkably, I haven’t had any new tumors develop since I’ve been on belzutifan. I’m still enrolled in the study and most likely will have to remain on this therapy indefinitely.
(Editor’s Note: On August 31, 2021, the U.S. Food and Drug Administration approved belzutifan for adults with von Hippel-Lindau disease who require therapy for associated renal cell carcinoma, central nervous system hemangioblastomas, or pancreatic neuroendocrine tumors not requiring immediate surgery.1)
Remaining Hopeful About the Future
I haven’t known a life without von Hippel-Lindau disease, and I’ve learned to live with that fact, but it has definitely colored how I approach each day. Cancer has a way of prioritizing your goals in life and helping you figure out what is most important to you. I stave off depression by telling myself that tomorrow is not a promised day, and I don’t want to waste time ruminating over circumstances I can’t change. I have to fight every day for my life and keep moving forward. There is no other way to survive this disease. Pain and stress are just reminders to me that I’m still alive.
Since the development of belzutifan, I have never had so much hope for myself and for other survivors of von Hippel-Lindau disease. I’m only 31, and although I know I will never be rid of the effects of the disease, I’m optimistic that research advances will bring us closer to more effective therapies—and maybe even a cure—by the time I’m in my 40s or 50s.
And I’m willing to do my part. Being enrolled in the belzutifan clinical trial has given me a strong appreciation for research, and I am willing to dedicate my body to medical exploration if it will help others with this disease or help to find cures for other cancers. I want oncologists and researchers to know that what they do every day to help patients like me is making a difference in our lives.
For my entire life, my mantra has been to stay positive and continue to move forward. Honestly, it’s a message everyone can benefit from, whether you have cancer or not.
1. U.S. Food and Drug Administration: FDA approves belzutifan for cancers associated with von Hippel-Lindau disease. Available at www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-belzutifan-cancers-associated-von-hippel-lindau-disease. Accessed February 27, 2023.
Ms. Vitale lives in Townsend, Delaware.
Editor’s Note: Columns in the Patient’s Corner are based solely on information The ASCO Post received from patients and should be considered anecdotal.