SuperTerry destroys the evil monster Huntington’s disease

Fifteen-year-old San Diegan Terry Leach wants to destroy Huntington’s disease, the condition that has devastated him since his toddler years and threatens to take his life very soon.

Terry’s story deeply moved San Diego artist Lee Ellingson to imagine a different outcome. In Lee’s Superman-like comic-book-style rendition (below), Terry has overcome HD by becoming “SuperTerry.” SuperTerry knocks out Huntington’s and saves the world from the ravages of the deadly disease that afflicts an estimated 30,000 Americans and could devastate as many as 250,000 more Americans who live at risk.

SuperTerry is vigorous, powerful, and triumphant. He beams with the joy of restored health and newfound happiness that the real-life Terry – along with every other victim of HD and juvenile HD – hopes for as scientists seek effective treatments and a cure.

“Wouldn’t it be great if this Huntington’s disease was like some kind of monster and Terry had super powers and could defeat the monster?” Lee told me in an interview. “That was my idea. It just kills me that a kid like Terry can have a disease like this at such a young age.”

Lee recently learned of Terry because of his own son Arnold’s struggle to live. Terry and Arnold attend the same after-school care program for disabled children and teens.

Arnold, who turns 13 later this month, was born hydrocephalic, a condition once known as “water on the brain.” He had an emergency operation immediately after birth to insert a shunt that drains fluid from the brain. He has had 23 more operations to adjust the shunt. Arnold also has autism and cerebral palsy.

Although Lee has made drawings for Arnold, SuperTerry is his very first piece illustrating a disease. He was shocked to learn that HD can affect children.

“He’s aware of what’s going on, but his body doesn’t do what it should be doing,” Lee, whose work includes background layout for the 1990s TV series Attack of the Killer Tomatoes and pieces for the San Diego Museum of Natural History, said of Terry. “That’s what’s especially heartbreaking for me. He’s a normal 15-year-old kid inside.”

Lee imagined the monster by thinking of the horrors of HD.

“He’s kind of like a blob,” Lee said. “He’s transparent and green. Slimy! I just wanted him to look real mean.

“I’ve always been pretty religious, but seeing all these kids really tests your faith,” Lee continued. “Kids like Terry and Arnold are the closest things on earth to an angel. Terry will never steal or cheat or rob or hurt anyone.”

Lee made the illustration based on photographs. On August 2 the two met in person for the first time at a fundraising event for the San Diego Chapter of the Huntington’s Disease Society of America (HDSA-San Diego). Lee presented Terry with the illustration.

“Terry was very appreciative of the picture,” his mother Angela told me. “He liked it a lot.”

Lee Ellingson (left) and Terry Leach

A feeding tube, operations, and looming death

SuperTerry HD treatments are needed now.

HD patients constantly struggle to maintain weight because they burn large amounts of calories and suffer from severely hampered swallowing. In 2010, in an end-of-life measure, the real Terry started taking meals and water through a feeding tube connected several times each day to a surgically produced hole in his abdomen. Before the operation, he weighed only 67 pounds. He now weighs 100, enough to help extend his life but still way below the average of 126 pounds for a 15-year-old male.

Terry’s body reacts to the hole as if it were an ulcer, causing him to produce large amounts of saliva that he wipes away with a towel constantly at hand. Doctors will inject botox into his salivary glands to diminish their output. He’s also gotten botox in his arms and legs to relieve pain.

Terry has undergone leg and foot operations to further relieve pain and tightness, correct deformities, and allow him, with assistance, to occasionally leave his wheelchair and walk. (For further background on Terry, please click here and here.)

Terry 

Because he can’t talk, Terry partially communicates through a language program on his iPad, which allows him to interact with people as the device’s speaker pronounces words and phrases that he selects. He takes regular classes at Madison High School, where he is starting tenth grade. Last fall he made the honor roll by carrying a grade-point average of at least 3.5. In middle school he received a number of other awards.

Terry loves computer games. During my visit to the Leach household, he played Club Penguin while I spoke to his mother for 90 minutes.

 “He’s always happy,” said Angela, a single parent who depends on Medi-Cal and other programs for financial assistance and, when she misses work because of Terry’s medical appointments and crises, the generosity of her employer, the San Diego Convention and Visitors Bureau. “He’s always wanting to give me a hug. And he’s so strong. He deals with everything and never quits fighting. He never complains, either. He’s always wanting to help. Everybody that knows Terry sees the light within him.”

However, Angela knows that, unless treatments become available very soon, Terry will die of HD. Recently a cousin of Terry’s with juvenile HD died at the age of 23. Other juvenile HD patients die in their teens or even childhood.

Angela Leach with the original drawing of SuperTerry (photo by Gene Veritas)

Raising awareness, saving the children

Knowing how little time remains for Terry, both Angela and Lee want to use SuperTerry to raise funds for HD research and increase awareness about HD and the difficult issues surrounding it.

Lee plans to expand SuperTerry into a comic strip and perhaps even a graphic novel about HD. Angela hopes to sell SuperTerry t-shirts in collaboration with HDSA.

“I don’t want any fame or glory,” Lee said. “I just want to help raise money. I want Terry to be the star.” As research progresses, science will also find ways to cure other diseases, too, he added. Such research could also benefit Arnold.

Above, San Diego Chargers football stars Philip Rivers (left rear) and Antonio Gates with Arnold Ellingson (left foreground) and Terry at HDSA-San Diego fundraiser in spring 2012. Below, friends Arnold and Terry enjoy Disneyland together.

Angela hopes that her and Lee’s efforts will help inform HD families about the option of genetic testing so that couples can avoid passing the disease onto their children through the use of preimplantation genetic diagnosis (PGD) or in the event of pregnancy – and depending on the couple’s personal and religious beliefs – early termination of the pregnancy. She wants SuperTerry to “save the children” from HD.

“I would never want another mother to go through what I did,” explained Angela, whose husband at the time (now an HD patient living in a nursing home in Indiana) did not tell her about the disease in his family until after Terry was born and the husband himself developed symptoms. HD families need to “get more proactive” about testing and family planning, she added.

“Terry’s life shows you what you face if you take a chance” by conceiving without all of the information, genetic counseling, and other resources available to HD families, she said.

“It would be best to be proactive to minimize that situation,” Angela continued. “It’s a lifelong situation. I’ve watched Terry grow up and deteriorate. There are so many challenges. We’re forever fighting.

“I don’t want his life to be in vain,” she concluded, recalling how an old friend’s son who had been in baby photos with Terry was now an imposing teenage football player. “I was happy for her, but it broke my heart. That could have been Terry. He could have been a football player. Or at least talk.”

An angel fighting for the cure (Huntington’s disease and abortion – Part I)

Lying in a hospital birthing room, Christina Wright held her premature infant son Timothy John awhile in her arms and, after he died, handed his tiny body over for research that might help save her husband and thousands of others from Huntington’s disease.

On August 28, 2011, Timothy became an “HD angel,” yet another mourned but deeply loved victim of this killer brain disease. His brain was donated to a tissue bank at the University of British Columbia’s renowned research program on Huntington’s.

After the fetus tested positive for the abnormal gene that causes Huntington’s, Christina and her husband Michael prayed at their local church in Coral Springs, FL, and then decided to abort their child in the 22nd week of the pregnancy. Hoping all along that they might carry the baby to term, they had already given him a name.

For more than ten years, Michael has helped care for his 54-year-old mother Gail Suvino, now residing in a nursing home with late-stage HD. In October 2010 Michael himself tested positive for the disease. He is 34, and he worries constantly about when his own symptoms will start.

Christina and Michael Wright (family photo)

The ‘toughest decision’

Christina and Michael could not bear the thought of their son facing the threat of a life burdened with severe disabilities and ultimately cut short by years, if not decades.

“It was very hard for me,” Christina said with great emotion in a phone interview on December 8. “I didn’t know that he was going to be born alive. He was strong and a fighter and wanted to live, and that breaks our hearts.”

“He looked just like me when he was born,” said Michael, who had stood by Christina during the birth. “He had my big feet and chicken legs.”

“This was the toughest decision we ever had to make,” he continued. “I bring it up every day. A lot of people don’t know what this disease does to a family. It does massive damage to a family.”

A birth with a research goal

With medical assistance, a baby delivered at 22 weeks would have a 10 percent chance of survival, explained Christina, a pediatric nurse. Because this was an abortion, the medical team did not intervene to save Timothy. He died of heart and lung failure.

“I did not have a clinical abortion,” Christina said, noting that in a standard abortion the mother is put under anesthesia and the fetus terminated and removed in pieces with the help of forceps. Under those circumstances, the brain could not have been used for research.

“I went through the birth to be able to donate the brain,” Christina continued. “The doctors did insert pills into my cervix to induce my labor. I pushed him out. I delivered my son with the purpose of fighting this disease.

With great pain in her voice, Christina spoke of how she will miss her son.

“No one wants to hurt their child,” she said. “We loved him so much. We always will. Even if we have ten children, we’re always going to be missing one. He was our son and always will be. We have pictures of him. We have his ashes in an urn. He will live in our hearts forever.

“I feel like he’s fighting in a different place,” she added. “He’s my husband’s angel. My son can help save my husband. He’s a warrior. I couldn’t be more proud as a mom, to know that my son helped find a cure to beat this. Some moms want their children to be a doctor or a lawyer. Mine might help find the cure for a disease that destroys families.”

Painful memories of another test

When I read Christina’s Facebook posting about Timothy on August 29, I felt terrible. I decided immediately that I would soon write an article for this blog about their plight. This is the first of two articles about HD and abortion.

Never an easy decision, abortion is even tougher for people of faith like the Wrights and a pediatric nurse like Christina. The disabling, ultimately deadly nature of HD further complicates that decision.

Interviewing the Wrights was one of the most difficult moments in my 13-plus years as an HD activist.

Listening to them sent my mind reeling back to the winter of 1999-2000, when my wife and I tested our own baby for HD in the womb six months after I had tested positive for HD. As I told the Wrights, their experience closely resembled ours – except for the all-important fact that our daughter tested negative.

As I pictured Christina holding Timothy, I imagined what it would have been like if our daughter had died in her mother’s arms immediately after birth.

I can’t find the words to describe the pain I felt. I shared our family’s story with Christina and Michael. I told them that I supported their decision and thanked them for donating Timothy’s brain for such a worthy cause. “I appreciate that,” Christina said.

In recounting the interview to my wife, I explained how the Wrights had oscillated between continuing and terminating the pregnancy. I then recalled our own situation. “Nobody knows for sure until they’re actually faced with the decision,” my wife said. I agreed.

An HD-free child

I am immensely relieved that our “miracle baby” is HD-free.

On several evenings this past week she and I happened to watch a recording of The Heart of Christmas, a new feature film based on the story of Dax Locke, a toddler whose battle against a rare form of leukemia mobilized the community to support his family and celebrate Christmas early so that he could enjoy it one more time before he died in 2009. Thinking of the Wrights, I felt so privileged to share these moments with her.

Last Saturday morning, after we accompanied her to a testing center for a private school entrance exam, I reflected on her progress in life and her immense potential.

The most powerful drive in life – more than the sex drive or anything else – is the need to nurture and protect a child. No calamity wounds a family more than the death of a child.

An imperfect situation

The Wrights’ story reveals the tragedy of HD, the wrenching decisions it forces upon families, and the imperfect conditions under which they must make and carry out those decisions.

After meeting through Match.com and dating awhile, Christina and Michael married on St. Patrick’s Day of this year. Michael, a beverage merchandiser who had to quit college in part to help care for his mother, had revealed to Christina that he had tested positive for HD but so far had not symptoms. Christina witnessed the tough reality of HD by meeting Gail.

“A lot of people would run the other way,” Michael said. “She really loves me.”

Christina, now 38, had been told by doctors that she couldn’t get pregnant because of an irregular menstrual cycle since the age of 14 and, later, ten years of failed attempts to conceive in a prior relationship. She and Michael didn’t use contraceptives.

The pregnancy caught the Wrights completely by surprise. Thus they hadn’t had the opportunity to seek genetic counseling, nor could they resort to PGD (preimplantation genetic diagnosis), which would have allowed them to choose embryos without HD for implantation into her uterus.

Pondering the results

An amniocentesis was done on the baby at sixteen and a half weeks, the earliest possible moment. But the results of the HD test took three more weeks to arrive.

Michael’s abnormal gene has 42 CAG repeats, whereas a normal huntingtin gene has only ten to 29. Those extra repeats cause the disease. Timothy had 40 repeats. Statistically speaking, Michael, who is 34, will probably develop symptoms sometime in his forties or fifties. Someone with 40 repeats might develop them a bit later, although, according to Michael, Gail’s symptoms started around the age of 36 or 37, and she has only 36 repeats.

Although they are finding more clues, scientists still don’t know exactly how and when the disease starts. Anybody with 40 repeats or more will definitely develop symptoms at some point.

The Wrights had learned that the child of a gene-positive man has a much greater chance of developing juvenile Huntington’s disease, which can start as early as the toddler years. But that’s because the father can often pass on more repeats, whereas a mother usually passes on her number. In this case, Timothy actually had fewer repeats.

“I almost don’t want to know that, if that’s the case,” Christina told me after I explained my understanding of that connection between repeats and juvenile HD. “We very much wanted our baby. We feel that we gave him back to God, especially because we’re Catholic.”

‘We were bonding’

The process of learning about their child’s fate was awkward. The Wrights received the results of Timothy’s HD test from an on-call obstetrician, rather than from their regular doctor, who was out of town. No social workers or geneticists were on hand to help clarify the information, and it was the first time the obstetrician had ever advised a patient on an amniocentesis involving HD.

The Wrights faced additional time pressure because in Florida a fetus at 24 weeks is considered viable, Christina explained. Had Timothy reached that age, the couple would have had to travel to another state for an abortion.

“We were both crying and both devastated,” she said of the impact the news of Timothy’s gene-positive result. “It was very difficult for us. I didn’t think God would give us more than we could handle, and so I wanted to have our son.

“It wasn’t just a pregnancy at that point. It was our son. We saw his picture on the ultrasound. I was gaining weight. I could feel him move. We were bonding with him. We started picking names. It was just very hard.”

Medical advice

But the obstetrician told her that she “was being selfish and not considering the life of the child” if she went through with the pregnancy, she said.

Other doctors that the Wrights knew at the University of Florida in Gainesville – where Michael travels regularly to participate in an HD research study – couldn’t tell the couple when the symptoms would occur and how bad they might be.

“We wanted to consult with them,” Christina said, explaining that they spoke with these physicians via cell phone. “Their opinion meant more than an obstetrician’s. They agreed that it would be better not to have the child.”

She added that these doctors did not mention the relationship between the CAG repeats and the age of onset.

All of the doctors led the Wrights to conclude that they would have a “sick baby,” Christina said.

Timothy’s purpose

Sitting in a pew at their church, the Wrights prayed and cried for more than an hour. The parish priest appeared and asked what was wrong, and, without mentioning HD or a genetic test, the couple explained that they had gotten “bad news” at the doctor’s office and would have to deliver their child prematurely.

“He came into our room (at the hospital) and said a prayer and held our son and put some holy water on him and christened (baptized) him,” Christina said, adding that Timothy was probably dead at that point. “It was very touching for us.”

Timothy lived for about 90 minutes. The medical personnel needed to get his brain on dry ice in 30 or so minutes, and they shipped it to the University of British Columbia’s Huntington Disease BioBank. There scientists study tissue samples to understand “the way DNA changes associated with Huntington’s disease affect the actual physical characteristics and proteins of the brain and tissues.”

“That was one thing we were adamant about,” Christina said. “If we were going to lose our son, we wanted something to come from this.

“We’re not ashamed of what we did. We just want to help find a cure.”

The couple wants to share their story to raise awareness about Huntington’s and support the quest for a cure not just of HD, but other devastating neurological conditions such as Alzheimer’s and Parkinson’s, Michael added.

Timothy is now part of that quest.

“We felt that was his purpose,” Christina said.

Opinions on testing

The Wrights still want a child and are now saving to afford PGD, which can cost upwards of $10,000 and even twice that amount, depending on the circumstances. Their insurance doesn’t cover it.

They believe that PGD is “the responsible thing to do,” Christina said. She strongly disagrees with families that, when pregnant, choose not to test for HD via amniocentesis.

“With all that science has to offer, it’s (also) the responsible thing to do,” she said.

“Just think about the long-term effects if they don’t do the right thing,” Michael said. In using the latest scientific techniques, he and Christina aren’t seeking the “perfect baby, but a healthy baby,” he added. “You owe it to your family” to assure healthy genes, he said.

“God gives us these vessels, these instruments, this technology to guide us and help us along, especially in the medical field,” Michael said. “Don’t go through it blind-folded.”

More HD families need to “come out of the closet,” Michael continued. HD is “not something to be ashamed of. People look at Mom and think it’s a stroke. It hurts."

Learning from the Wrights’ example

In concluding the interview, I told Michael that he and I were “brothers” in the fight to stay healthy and support the cause for the cure. I told Christina that she was my “sister” in the cause.

I’ve been meditating on Timothy and his parents. I pray that others don’t have to face their terrible predicament, although many surely will until the medical community achieves greater awareness of the genetics of HD.

Ultimately, effective treatments or a cure would liberate people from this predicament.

We can learn much from Timothy John Wright. His parents gave us his brain for research – to serve the greater, common good. Understanding the bigger picture, Christina and Michael committed the ultimate act of human solidarity.

We in the HD community also need to see the greater good. Whenever possible, we need to participate in research. To do so, we must exit the terrible “HD closet.” I myself remained pseudonymous, in the closet until 2010, and in February of this year I came out by delivering the keynote speech to HD specialists from around the world gathered at a key conference in Palm Springs.

As Michael pointed out, we need to rise above the difficult feelings surrounding HD and gain heart from the fact that our participation in the quest for treatments and a cure is helping to create the dawn of a new era. Along with the scientists, we are taking a quantum leap in the study of the brain. We are the pioneers pointing the way to better brain health. We are doing something profoundly good and important.

From suffering to progress for all

In the past, many people – including my own family – have viewed HD as a matter of suffering. But today, with scientific progress, HD is about the human quest for a longer and more fulfilling life for all.

In the Bible, Abraham was willing to sacrifice his own son in a sign of complete commitment and love. Our collective human sorrow is captured In Michelangelo’s Pietà, with Mary holding the body of her son Jesus after his crucifixion.

Michelangelo's Pietà, in St. Peter's Basilica in Rome


The mini-urn containing Timothy's ashes (family photo)

We in the HD community are all Timothy John Wrights in our mothers’ arms.

Indeed, we in the HD community endure great suffering. “You think, ‘God can’t be that cruel,’” Michael said.

But, like the Wrights, none of us should feel ashamed. This community has lots of love – and it shows it just as the Wrights have done.

(The second part of this series will tell the story of Katelyn Sandbulte, a 20-year-old juvenile Huntington’s disease patient in the first trimester of pregnancy.... At this special time of year, please remember to donate to the Huntington’s Disease Society of America.)