A crowning moment for Huntington’s disease

Shelby Lentz is competing for the Miss Michigan title. But since her diagnosis, she’s now competing in a race against time.

Shelby Lentz will be competing for the Miss Michigan title next summer.

But her goal isn’t to be crowned the most beautiful woman in the state. Her goal is to spread awareness of something that robs her and her family of their dignity.

Beauty may be skin deep, but it’s what lies deeper inside her brain that has her concerned. Terrified, really.

The 20-year-old Kalamazoo resident isn’t worried about applying makeup. She’s worried about her genetic makeup.

Lentz’ grandfather and 42-year-old father suffer from Huntington’s disease. Her grandpa’s mom also likely had the disease. Lentz’ 11-year-old sister, Breanna, diagnosed with juvenile Huntington’s disease, started showing symptoms three years ago.

“When you get it at the juvenile level, it’s far more aggressive than the adult version,” Lentz said.

Lentz, who holds the title of Miss Spirit of the State for the Miss Michigan organization, uses her pageant platform to speak about Huntington’s disease, trying to raise awareness of the disease that robbed her loved ones of muscle and brain power.

Learning her probable fate

Since Huntington’s disease is genetic, Lentz felt the need to know her destiny. Would she escape the neurodegenerative disorder that kills brain cells, affects muscle coordination and leads to mental decline and severe behavioral changes? Or, would she stand alone on the runway, wearing an immunity crown.

“I was pretty adamant about getting the testing,” Lentz said. “For me, not knowing is far worse than knowing because I tend to worry and stress. I told myself one of two things would happen when I got the results back—I would find out I was negative and I’d be able to take care of my sister and focus on her, or, I’d be able to start fighting the disease sooner at a younger age.”

Last school season, Lentz attended college out of state. Her mom encouraged her to wait to get the testing until she returned to Michigan.

“I didn’t want to get the test results and go back to school all alone,” Lentz said.

Last summer, after her final semester, she returned home and went to Spectrum Health Medical Genetics for genetic counseling and testing.

Spectrum Health certified genetic counselor Kathleen Delp, MSW, delivered the dreaded news. Lentz tested positive for the Huntington’s disease gene.

She had dreamed of being crowned Miss Michigan and, someday, Miss America. But now she’s crowned with this disease of thorns, which is primed to pierce life as she knows it. It’s not a matter of if. It’s only a matter of when.

“If you have the gene, then you will develop symptoms eventually,” Lentz said. “They just can’t tell you when or how severe.”

A dreaded disease

Delp said Huntington’s disease typically progresses over a 15- to 20-year period, causing involuntary motor movements, mood disorders, cognitive decline and difficulties with swallowing, balance and coordination.

It affects 1 in 10,000 people with an estimated 30,000 people nationwide currently battling the disease. Another 100,000 to 200,000 people are at risk.

I’ve definitely gone through a roller coaster of emotions. … It is scary. I don’t know what my fate is and when this disease will hit me.

Shelby Lentz

“As a result of genetic testing, more individuals are identified with the (Huntington’s disease) gene, even those without a family history whose parents may have died prior to manifesting any symptoms,” Delp said.

There’s a 50 percent risk of transmission from parent to child.

“The gene can expand, contract or remain the same size when transmitted from one generation to another,” Delp said. “If the gene is transmitted from the father, there is a greater likelihood for a larger expansion in the child, which could result in a child showing symptoms in childhood or the teenage years.”

Lentz is not showing symptoms at this time. But she will. It’s a sad, undeniable and inevitable truth she carries with her every day.

Her father started showing symptoms in his 20s.

“I don’t know when I’ll become symptomatic,” she said. “It typically happens between your 30s and 40s. I don’t have any physical symptoms, but sometimes I worry about the cognitive. Anxiety and depression are far worse in people with (this gene). There are a lot of unknowns and that’s the stressful part. They can’t tell you much else other than, ‘You’re sick and you’re going to get worse.’”

Like her father and sister, when Lentz begins showing symptoms, she plans to rely on Spectrum Health for care.

“Unless you’re already showing signs and symptoms, there isn’t much they can do,” Lentz said. “But they’re doing a lot of research into treatments.”

Lentz is well aware how those signs and symptoms manifest. Like monsters. She’s seen them demonize her family members.

Holding onto hope

“I’ve definitely gone through a roller coaster of emotions between a state of shock and a state of positivity,” she said. “I’m still going through that even though a few months have gone by (since her genetic testing results). It is scary. I don’t know what my fate is and when this disease will hit me.”

While she continues to hope and pray for a cure, she’s conscious of the reality of now. A now that has no cure, no answer for the disease that looms in her future like a dark shadow from which she cannot escape.

“I’m constantly in fear,” Lentz said. “I don’t know how many days I have of being healthy. It was taken from my sister so quickly and that scares me. Every time I go to a … conference I hear doctors and researchers say there’s never been a better time to have (the disease) because they’re very close. Hopefully within the next decade or so we’ll have something, whether it’s treatment or cure.”

Until then, Lentz is focused on raising awareness about the disease. First, for her grandpa, dad and sister. More recently, for herself, too.

“I’ve been trying to process the information for the last five or six months,” Lentz said. “I feel fortunate because the (Huntington’s) community is supportive. It’s a genetic disorder, so you’re never alone. The Miss America organization is based on community service and scholarships. Every woman has something that she speaks about during her reign. I chose (Huntington’s disease) because it’s something near and dear to my heart.”

A passionate platform

She’s talked about the disease on television programs and at conferences. But she’s aiming for an even larger audience.

“My goal is to hopefully become Miss Michigan someday and go on to become Miss America,” she said. “I want to talk to leaders in every state. When I found out about this disease, I didn’t even know what it was, and here it was running in my family. It’s interesting how much can change in a short amount of time.”

The one constant for Lentz, though, is competing in pageants.

She’s following in her mom’s high-heeled footsteps.

“My mother competed and volunteered with Miss Michigan,” Lentz said. “I’ve always loved singing. One of the portions of competition is the talent portion. When I got old enough, my mom told me, ‘You can get scholarship money just for singing.’ I started out because of scholarships and being able to perform. Then I started growing fonder and fonder about what that organization stands for and what it’s about.”

Lentz has captured three titles in her short career—Miss Allegan County (where she attended high school), Miss Greater Kalamazoo and currently, Miss Spirit of the State.

She even writes her own songs to perform during pageants.

Her favorite? “Champion.”

“I co-wrote it with a girl in Nashville,” Lentz said. “I wanted a song that was empowering like, ‘We can take on the world.’ A year after I wrote that, my dad and sister were diagnosed. Then, I was diagnosed. The song really hit home for me.”

She introduced the song to the Huntington’s disease community.

“It’s kind of become like the … anthem,” she said. “It now means more to me than I ever thought it would.”

And thanks to her pageant work, she has a prime platform for sharing the song and her soul-felt passion for a disease that has her in its sights.

“Having a crown on your head gives you the opportunity to open more doors you wouldn’t be able to open on your own,” Lentz said.

And for her little sister, who often requires a wheelchair and has difficulty speaking, the crown means even more.

“She thinks I’m an actual princess,” Lentz said. “After I won my current title in July, I let her try on my crown. I told her I talked about her in my interview and she thought that was the greatest thing ever.”

Delp said she appreciates and admires Lentz.

“Shelby has incredible courage and determination to be willing to share her personal story and promote (Huntington’s) awareness in our state,” Delp said. “By making … awareness her platform in pageant events, Shelby is educating others and increasing awareness of (this disease) with the hope to promote funding for effective treatments and a cure.

“Shelby is fearless and intensely motivated with a depth of compassion for those who suffer with this disease.”

A support group, HD Living Positive, meets monthly in the Grand Rapids area. For more information on support groups in Michigan, visit the Huntington’s Disease Society of America site.

To learn more about genetic counseling and testing at Spectrum Health, call Kathleen Delp at 616.391.8664 or visit Spectrum Health Medical Genetics.

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