Largest gathering of people with Acromegaly meets in Milpitas

Last Saturday, The Milpitas Beat attended the world’s largest gathering of people living with Acromegaly (also called “Gigantism” in cases where the disease begins before patients have reached puberty) at Embassy Suites in Milpitas. Acromegaly is a rare endocrine disease made famous by people including pro wrestler Andre the Giant, actor Fred Gwynne (Herman Munster), and actor Ted Cassidy (Lurch from “The Addams Family”).

The event is held every two years. This most recent one was the first since the start of the pandemic, and was co-hosted by Acromegaly Community and Stanford Medicine. One hundred and fifty Acromegaly patients from around the world attended, along with top physicians specializing in Acromegaly/endocrine disorders and biotech/pharma companies developing new Acromegaly treatments.

The Beat spoke with Jill Sisco from Oklahoma, Acromegaly Community’s president, who explained how the organization attained nonprofit status in 2010, but had started out via Facebook groups around 2008. The first thing Jill (who herself was diagnosed in 2005) explained was that most people with Acromegaly get the disease post-puberty, which means they don’t shoot up in height like in the more famous cases cited above. This makes the disease less impactful in terms of physical appearance but much harder to diagnose. 

Ninety-seven percent of the time, Acromegaly is caused by pituitary tumors. Surgery is the first line of treatment. But said Jill, “It gets missed all the time.” This means that by the time the tumors are operated on, they often have grown large.

“Everything on us continues to grow,” Jill explained. “Our bones, instead of growing in length, are growing in girth. We are the only true big-boned humans.” 

People with Acromegaly experience their shoes and rings growing tighter, along with joint pain as a result of their bone growth.  

One attendee, Monica, has had two surgeries (a not uncommon experience) since her 2018 diagnosis, along with gamma knife radiation. “The first surgery,” she shared, “they fully removed the tumor, and it grew back within 9 months…” 

She’s now managing the disease with medication, but explained how it can create a state where “Every joint hurts.”

“It also affects all our organs,” Jill added. For example, the heart can outgrow its valves, a major cause of death in Acromegaly patients. 

But both women said their meds are mostly effective.

“There’s over 7,000 rare conditions out there,” Jill said, adding that only 5% of them have treatments. “We’re lucky to have the treatments that we have.”

They also commented on Acromegaly’s mental health impacts, which are hormonal as well as psychological: “When you look into a mirror and don’t recognize what’s looking back at you,” Jill said, “you don’t want to go into public, you don’t want people to see you. It’s a very isolating disease.”

With normal hormone levels, life expectancy among Acromegaly patients is the same as that of average people, but the disease brings with it many comorbidities, as growth hormones speed up every process in the body. So even a slow-growing cancer like colon cancer can advance quickly in someone with excess growth hormones, meaning people with Acromegaly are advised to get more frequent colonoscopies.

Another attendee, Burt from Nebraska, explained how he’d been diagnosed with obstructive sleep apnea (leading to soft palate and uvula surgery), diabetes, and congestive heart failure before being diagnosed with Acromegaly. 

He had his pituitary tumor surgery in 2010: “It was within two months of my diagnosis.” 

“Two years later,” he said, “I had a residual tumor return. Ever since then I’ve been on medication…Now I’m doing good. It’s controlled.” 

But: “The pain’s not controlled.” Reason being, the meds can control growth hormone, but not the damage already done to joints and organs. As a result, Burt is constantly in a lot of joint pain. “It’s always there,” he said. “This morning I’d give it a scale of 10; right now I’d give it a scale of 2.”

Bonnie, a hair stylist, had chronic headaches for over 20 years before her Acromegaly diagnosis: “I had headaches since I was in my 20s. And it was always kind of brushed under the rug. When a doctor dismisses that, you think, OK, maybe it is stress. Eventually, I stopped telling anyone I had headaches…” 

Later, after other health issues came up, she got diagnosed and headed into tumor surgery. Surrounding her surgery, her doctor noted the chronic headaches cited years before in her record. He wondered why they hadn’t been taken care of. Bonnie had tried to, but “Everyone dismissed that.” 

Meanwhile, her physical changes were so gradual that “Even my husband, he didn’t really pick up on it.” But eventually, her endocrinologist asked to look at her driver’s license, so as to compare it to her present appearance. Noting a difference, he began to suspect Acromegaly. “He really insulted me,” Bonnie recalled, “because the first thing he said was, ‘You know Andre the Giant? This disease is what he had.’” 

As a woman, she felt affronted. “You’re making me feel really good!” she said, sarcastically.

Dr. Larry Katznelson, M.D., of Stanford was on hand, too, having come to give a lecture on the various dimensions of Acromegaly. A professor of medicine and neurosurgery, Katznelson specializes in neuroendocrinology, particularly as it pertains to pituitary disorders.

He explained some of the physical changes that can take place: “The jaw can grow outwards. The tongue can get huge.” Moreover, “Some of these tumors can wrap around major arteries or begin to grow into the brain.” In the meantime, said Katznelson, “The international success rate of surgery is about 50%.” The smaller the tumor, the easier the surgery. He also added, “Medical therapies take care of most but not all patients.”

Katznelson broke down Acromegaly as follows: “We have two problems here: one is the tumor itself, which can be pushing on structures that are important. The other part is that the tumor’s making this growth hormone. So you either want to lower that level or block the level.

“The problem is untreated Acromegaly. It’s critical that we get that growth hormone managed.” 

Optimistically, Katznelson said, based on existing and upcoming treatments (including a recently FDA-approved oral medication), “We really should be able to control the majority [of patients].”

**

Jill Sisco looked out at her guests with pride. She recalled the moment when it first crossed her mind to start the Acromegaly Community. After her own diagnosis, she sought support on an online message board. A random commenter wrote, “Big hands. Big feet. Sounds like a bunch of big fuglies to me.”

It cut her deeply. But no one removed the comment. For two whole days, it just sat there. So she messaged the admin. When the admin replied, they said, “Sorry, sometimes the crazies get by me.”

Jill was firm in her resolve. She thought there and then, If I run a support group, the crazies won’t get by me.

Monica sounded a note of support for the Acromegaly Community and all Jill has achieved with it:

“I don’t know what I would have done,” she said, tearing up, “without Jill.”

Learn more about the Acromegaly Community here.