Shedding Light on a Rare Disease : Woman Hopes O.C. Group Will Increase Awareness of Life-Threatening Effects of Excessive-Growth Illness
Jill Meer doesn’t avoid mirrors, but they are a constant reminder that her appearance has changed. “I gave up worrying about my looks,” she says. “I had to. It was hurting too much.”
In addition to a puffy and “fuller” face, Meer’s ring size has gone from a 7 to a 9 1/2 and her feet from 7 1/2 narrow to 9 1/2 medium. Nearly 20 years after graduating from high school, she has grown 1 1/2 inches.
Meer’s condition is caused by acromegaly, a rare and potentially life-threatening disease brought on when a tumor on the pituitary gland creates an overproduction of growth hormone. Acromegaly is characterized by excessive skeletal growth and soft tissue enlargement, the most obvious symptoms being the growth of hands, feet, nose and jaw.
The biblical giant Goliath probably provided the first description of the disease, which is also known as gigantism when the tumor appears before the end of puberty and dramatic bone growth occurs; Carel Struycken, the 7-foot-tall actor who played Lurch in the recent “Addams Family” movie, is an acromegalic.
The disease strikes three to four people per million each year. So far it has been diagnosed in more than 40,000 Americans, typically not until eight to 10 years after the onset of the tumor. But many cases remain undiagnosed. And if untreated, the disease can cause diabetes, heart disease, hypertension, kidney-related problems, cancer, blindness, arthritis and other serious conditions.
Meer, however, feels fortunate.
Her pituitary tumor was discovered and surgically removed nine years ago. That halted further growth. But as often happens, Meer’s tumor returned last year.
“I was devastated,” says Meer, 37. “It made me very frightened because I didn’t understand how or why it came back, but with the support group now I understand it could always be with me.”
The support group is the Acromegaly Network Assn., the world’s first nonprofit organization dedicated to supporting acromegaly patients and their families, educating both the public and the medical community and funding research for the early diagnosis and treatment of the disease.
The group, which was formed early this year, holds monthly meetings at Cedars-Sinai Medical Center in Los Angeles. The goal of the 100-plus member association is to become a worldwide network: Meer is starting an Orange County chapter, and other chapters are opening in Texas and Canada.
“The importance of this group is that today we have the means of treating them, and they don’t die,” said Dr. Shlomo Melmed, director of endocrinology and metabolism at Cedars-Sinai. “The association provides them with social and psychological support, access to medical options and information about the latest drug trials.”
Says member Struycken, whose acromegaly was diagnosed two decades ago when he was 20: “This group definitely serves a very important function. What I’ve always noticed going through the medical mill is that there are people--even endocrinologists who should know their stuff--who are really very uneducated as far as acromegaly is concerned.”
Melmed, one of the Acromegaly Network Assn.'s founding medical advisers, acknowledges that most people are unfamiliar with the disease.
“People know about cancer, they know about heart disease, lupus and diabetes, but they don’t know about the rare diseases,” he said. “It doesn’t matter how rare that disease is, for that individual patient it is the No. 1 concern. And there are thousands of patients with acromegaly in this country.”
Indeed, for Meer, attending the association’s meetings have provided blessed relief.
“I’ve been carrying this weight alone for nine years,” she says. “Until I found this group, I felt embarrassed to have it. I felt very ugly. I felt very different.”
Meer remembers joking with her mother a decade ago, ‘ “Gosh, Mom, you’re shrinking.’ She measured me on the wall like when you’re a kid, and she said, ‘You’re 5-(foot)-5 and a half.’ I said, ‘Oh, mom, don’t be silly. I’m 5-4.’ ”
Meer discounted the increase in height at the time, figuring “maybe we measured wrong all along.” But looking back, she says, “I see a lot of indicators (of the disease) that I didn’t understand.”
Meer’s acromegaly wasn’t diagnosed until after she went in for her annual checkup with her gynecologist in 1983.
At the time, she was recently divorced and, she recalls, “I jokingly said, ‘I’ve got to tell you this divorce is taking a toll on me. I just feel wiped out all over. Look at my hands, they’re swollen.’ And my feet hurt like I’d walked all day shopping. They were very tender.”
Her head felt equally tender. And she was experiencing migraine headaches, arthritic symptoms in her joints and severe fatigue.
“Just getting through the day was difficult,” says Meer, who recalls her gynecologist telling his nurse, “It sounds like acromegaly or rheumatoid arthritis to me.”
That fact that her gynecologist even recognized the symptoms of acromegaly is unusual.
“Another doctor could have dismissed it,” says Meer, who is on the association’s national board of directors. “That’s one of the key problems. Statistics show that basically people are walking around with this tumor undiagnosed and undetected for eight to 10 years, and until doctors are able to recognize acromegaly by sight they don’t get tested.”
After having a full body X-ray and a CAT scan, Meer was informed by a neurosurgeon that she had a tumor the size of a golf ball on her pituitary.
“As I’m about to jump out the seventh floor with this news,” Meer recalls, “he said, ‘The good news is at least I don’t have to tell you to enjoy the next three months. This is doable. We can cure this.’ ”
The word “cure” is significant, Meer says.
“I was told the tumor more than likely wouldn’t come back (after surgery). I was told more than likely I was cured. In fact, I wasn’t,” she says. “It turns out people have these tumors two and three times and have two and three surgeries.”
Meer’s doctors figure she probably had the tumor for five years, dating back to when she was taking medication for infertility problems. As she later learned, infertility is another symptom of the disease.
Within six months of the surgery, Meer says, her swelling went down, and she began feeling much better.
Every six months for the next five years she had blood tests done to check for an elevation in her growth-hormone level. By the sixth year, she says, “it was starting to elevate, but the doctors were still telling me I’m OK. If it elevated beyond the normal range they knew the tumor had returned.”
Living with the disease hasn’t been easy.
“There are a lot of really scary aspects to this,” Meer says. “One is that there’s still a lot they don’t know about this disease. I feel real grateful because I’m under treatment, so I’m being monitored.”
But, she concedes, “there was a real self-esteem issue: I was growing. I was now a different person. My whole build was different and, as a woman, that was difficult. But the real debilitating thing is when this goes undiagnosed and untreated. Then you get the major cardiovascular problems, you get grotesque-looking facial features: protruding forehead, really bulbous noses. You get a real coarsening in the cheek bones. Your hands get even thicker than mine. You have a terrible weight problem.”
Meer, who remarried 5 1/2 years ago--"when I was in my ‘cure’ stage"--and has a 13-year-old son from her first marriage, says that learning last December that her tumor had returned “was very scary for us as a family.”
“I’m a positive person. I believe you’ve just got to go forward. But there’s an element of regret--and a great element of sadness. I felt like I’d been ravaged. I felt, ‘Boy, God really doesn’t love me to get it a second time.’ And yet, I swear it’s been a real gift, if you can imagine. It’s gotten me to a higher place in a sense.”
One day, she recalls, “I was crying and my husband (Ron) wrote on my vanity mirror in lipstick, ‘DSLS,’ and then left for the day. I had to call him and say, ‘What does that mean?’ It means ‘Don’t Sweat the Little Stuff,’ and that’s the motto in this family: The little stuff doesn’t matter, and that’s what the tumor has given us.”
Although she and her husband would like to have another child, they are now considering adoption. Meer has been told the pituitary doubles in size with a normal pregnancy. And with the tumor she risks bleeding problems.
Meer also has had to modify her lifestyle. She can no longer play a full game of tennis or do aerobics. “I can’t do that bouncing up and down because my joints are sore.”
She and her family have learned to accept that.
“Once we knew I wasn’t going to die from it, we all relaxed a lot,” she says. “We’ve all come to terms with it. I take my medicine. We know I need to get plenty of rest. I’m a real go-getter--I’m involved in a lot of community kinds of things--and they just know when I’m wiped out.”
But that has begun to change now that she’s on new medication.
Wanting to avoid a second surgery, which offers no guarantees, Meer last month began taking a new drug produced in Western Europe. It can shrink the tumor in 35% of the cases, and in 95% of the cases it will at least reduce the growth hormone to a normal level.
“I feel like I haven’t felt in years,” says Meer, “and the swelling is going down.”
Meer, who is a member of the Costa Mesa Child Care Committee and the Orange County Board of Supervisors’ Child Development Council, in addition to being heavily involved in Republican politics, is now putting her energy into forming an Orange County chapter of the Acromegaly Network Assn.
And she’s approaching it with missionary zeal, with plans to provide the medical community with patient-education packets and to conduct educational seminars in community hospitals.
“I’ve got to get the word out,” she says. “I don’t want people to suffer. I’ve suffered for nine years.”
For more information about the Orange County chapter, call Meer at (714) 645-0204.