The day after Christmas in 2016 brought unwelcome news for Wasilla resident Josh McCool.

McCool, who was just 26 years old at the time, was diagnosed with a rare neuroendocrine tumor – called pheochromocytoma – that had spread from his adrenal glands, which produce hormones that the body needs to function properly.

His journey

More inside

McCool was originally diagnosed with a tumor on one of his adrenal glands, which was removed in 2017 due to complications during surgery. Despite not expecting it to return, he developed persistent symptoms, which worsened and took a toll on the former journeyman electrician and father of two young sons.

“My resting heart rate was very elevated. I was super weak, unable to play with my kids, and spending 16 to 18 hours a day in bed. On top of that, I lost 100 to 110 pounds over six months,” said McCool. “I would be out of breath just walking from our couch to the kitchen.”

Thankfully, McCool’s mother, Wendy, who is an Anchorage resident – along with his oncology nurse at Alaska Native Medical Center, Rowen Mulvey, RN – became relentless advocates and eventually helped him get Alaska Medicaid to approve his treatment and travel to the Seattle Cancer Care Alliance (SCCA) Endocrine Neoplasia Clinic. After six months of evaluation, McCool’s specialist at SCCA knew cancer therapy was needed, so he was referred to the Neuroendocrine Tumor program at Virginia Mason Medical Center.

When I first met McCool, he was very weak and, at times, even needed a wheelchair. He was in significant pain and his cancer was secreting adrenaline, which was causing even more symptoms. I knew he urgently needed treatment, so we considered the options – including chemotherapy, radiation and Peptide Receptor Radionuclide Therapy (PRRT) with lutetium Lu-177 dotatate (Lutathera®).

To find out which treatment would be best for him, we did a special type of PET-CT scan, called Netspot®, which measures the amount of somatostatin receptors (a hormone) on the cancer cells. When the tumor cells lit up on the PET-CT, we knew PRRT with lutetium Lu-177 dotatate would be the ideal treatment for McCool.

So, we quickly worked with Mulvey in Anchorage and were able to start treating him within a month.

The treatment

Amazingly, just one week after his first treatment in early February 2019, McCool noticed that his symptoms started to dissipate. Improved appetite was followed by markedly more energy, increased activity and – within two or three weeks – a significant decrease of pain in his back.

Side effects were minimal since the lutetium Lu-177 dotatate therapy directly targets cancer cells by binding to the somatostatin protein on the cancer cells, wherever they may be in the body.

A total of four treatments are needed, once every two months. Since PRRT is not available in Alaska, McCool flies to Virginia Mason in Seattle. After each treatment, he has to take precautions for two weeks to ensure his family is not exposed to radiation.

When I spoke with McCool after his second infusion in early April, he said he had completely tapered off pain medicine and was now able to play with his 4- and 8-year-old sons. He has also put 40 pounds back on and his life is returning to normal.

Pheochromocytomas and paragangliomas

According to the National Cancer Institute, pheochromocytomas and paragangliomas arise at an average age of 24 in hereditary cases and 43 in sporadic cases. The incidence is equal between males and females.

When they develop in the adrenal glands, these glands – which are located above the kidneys in the back of the upper abdomen – produce high amounts of hormones causing high blood pressure, headaches, excessive sweating and heart palpitations.

About 10 to 15 percent of pheochromocytomas may be malignant. Unfortunately, McCool’s pheochromocytomas turned out to be cancerous.

The Virginia Mason Neuroendocrine Tumor program specializes in treating patients with neuroendocrine malignancies by providing surgery, radiation, systemic therapy, genetic counseling and clinical research trials. It also includes the largest PRRT treatment program in the Pacific Northwest.

Perspectives and a goal

Despite the challenging therapy, McCool feels very lucky. “Getting the green light to move ahead with the treatment was like winning the lottery,” said McCool. “It has definitely been a character-building experience and one I couldn’t have gotten through without the amazing support system of family and friends that I’ve been blessed with all along the way.”

McCool is especially grateful for the unending love and support from his fiancé, Shannon, who has been working three jobs to help their family get through this very challenging experience.

As the oncologist who oversees a team of amazing clinicians who have been helping care for McCool, the dramatic improvements in his health are remarkable and the benefits he has received from PRRT are impressive, even for a treatment that has been shown to reduce cancer progression by 80 percent.

Although the treatment is not a cure, we expect that McCool will be able to survive years longer with his cancer.

“I will be done with my treatments in August and my goal is to take my family to Disneyland in 2020,” said a grateful and optimistic McCool.

Hagen F. Kennecke, MD, MHA, FRCPC, is a medical oncologist and medical director of the Virginia Mason Cancer Institute. His areas of clinical expertise include neuroendocrine tumors and gastrointestinal oncology. Dr. Kennecke practices at Virginia Mason Hospital and Seattle Medical Center. Twitter: @HKennecke

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