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Family of terminally-ill child finds hope in the face of rare genetic condition

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Updated Jan 19, 2019; By Mark Tower | mtower@mlive.com

HUDSONVILLE, MI –Gabriel Dehen gazes lovingly up at his mother. She speaks to him in a soft, tranquil voice.

A gentle coo escapes the handsome child’s half-pursed lips. His mother’s hands shake as she tries to capture a video of the moment on her smartphone.

It would be a treasured moment for any parent. But, for David and Christina Dehen, the value of that “coo” is beyond measure.

“That’s rare,” Christina says. “We don’t get that often.”

In fact, Christina said, occasions where they get to experience their beloved Gabe “talking” with them is increasingly uncommon, despite the fact he is now almost 3 years old — a time when most toddlers have a rapidly expanding vocabulary and are speaking in short sentences.

Their son is hooked up to medical equipment that helps him breathe, eat, digest his food and monitor his vitals. He cannot lift his head, walk, talk or sit up. Gabe has already spent months of his life in the hospital and is always one bad day away from being readmitted.

In his short life, he has already had 19 hospital stays and survived nine bouts with pneumonia.

Gabe suffers from a rare and fatal genetic mutation that causes his brain and brain stem to degenerate.

Dr. Seth DeVries, the pediatric neurologist who diagnosed Gabe with the condition, explains that genetic testing only recently became sophisticated enough to find the PIGA mutation on Gabe’s X chromosome, meaning it’s difficult to determine exactly how rare his condition is. In his research, DeVries said, he only found mention of about a dozen such cases and only full reports on three or four.

“Too few to count,” he said.

That means predicting how the mutation will impact Gabe’s development is also very difficult.

A ‘world of uncertainty’

“It’s incredibly rare,” said Dr. Bradd Hemker, palliative care director at Spectrum Health’s Helen DeVos Children’s Hospital. “There is no one else in West Michigan and probably very few in the country that have exactly what he has.”

Hemker works with the Dehens as they are forced to make tough choices about complex medical treatments. But, unlike in the case of more well-documented medical conditions, not enough is known about Gabe’s position to predict how long of a life he should expect or to be certain which treatments will work best.

“We simply can’t do that for Gabe and his parents,” Hemker said. “They live sort of in a world of uncertainty.”

Since his condition is a neurodegenerative disorder, the doctors can make no promises to the family about Gabe’s future.

“That means it gets worse,” Hemker said.

Gabe has good days and bad, though the medical team has made great strides in reducing the seizures that once very frequently overtook his tiny body.

Now on three seizure medications, his parents say Gabe has gone almost two years without one.

Other problems remain, like the difficulty Gabe has digesting his food and his need for frequent breathing treatments.

His father describes how difficult it was to watch the pain and misery expressed in his son’s face when medical teams struggled to find a vein to insert an IV.

“I felt like I was looking at heaven going through hell,” David said. “To see such a little guy going through so much was agonizing.”

Despite all they’ve been through, David and Christina still vividly recall the joy they felt when they learned Christina was pregnant with Gabe.

Because of a kidney transplant, David believed he was sterile.

“We knew we were going to have issues from the beginning,” Christina said.

They later learned David’s sperm was viable, and explored various options for fertility treatments. Ultimately, they pursued in vitro fertilization.

Christina was in a work meeting when the clinic called her with the good news.

“I walked out of the manager’s meeting,” she said. “Of course, I was crying. I was so excited.”

Her pregnancy was largely uneventful.

“I wasn’t sick,” Christina said. “I think I had a couple days that I got real nauseous and I figured out that I couldn’t have spicy foods, which is rough for me because I love spicy food.”

‘He wasn’t breathing’

Gabe arrived on Oct. 16, 2014. It was only after his birth the new parents had the first hint something might be wrong.

“He wasn’t breathing,” Christina said. “They said he was having seizure activity and they were going to take him away. Of course, I didn’t see him either. I didn’t get to see him. They just whisked him out pretty quickly.”

David described the shock that hit them in that moment.

“You go from a moment of indescribable joy to unimaginable fear in a split second,” he said.

Gabe spent his first weeks in the neonatal intensive care unit at Helen DeVos Children’s Hospital, where he was taken shortly after his birth at Metro Health Hospital. Christina saw her son briefly before he was taken from one hospital to the next.

Six weeks later, on the day before Thanksgiving, Christina and David were able to take Gabe home. Soon after, it was obvious to them something was still wrong with their son.

“All he did is cry,” Christina said. “He cried all the time.”

She brought him to her pediatrician and eventually Gabe was readmitted to the hospital.

“There was a lot of optimism that he would snap out of all this,” David said.

Doctors started looking for the seizures Gabe was experiencing. Once they pinpointed them, further tests suggested something else might be at the root of the problem.

Christina had shared that her extended family has a history of male children who died at a young age.

“We decided right away we needed to get genetic testing for this,” Dr. DeVries said.

Naming ‘the beast’

That is how doctors identified the genetic mutation.

David said he will never forget how DeVries phrased it, saying they needed to “name the beast.”

“Something was trying to devour my son,” he said.

David expressed just how difficult it is to find himself powerless to solve the problem.

“As a husband, I want to be able to fix things,” he said. “I can’t fix this.”

There’s no question Gabe’s illness has taken its toll on the family. Though many of their medical expenses have been covered, there are still plenty of others to worry about, like the cost of a van to ferry Gabe and his equipment to medical appointments.

His condition is a constant demand on their time and attention, but David and Christina still treasure every moment they get to spend with their son.

But they sometimes find it difficult to do even the simplest of things, from mopping the kitchen floor to mowing the lawn.

‘Strength to fight on’

Friends, family and strangers have all stepped up to help, something for which they express deep gratitude and hope will serve as an example for others. When they came home from the hospital to find a kind neighbor had cut their grass, they said, it filled them with warmth.

“It gives you strength to fight on,” David said. “It helps to know somebody cares about this little guy and cares about this family.”

The Dehens are grateful for the help they have received from countless healthcare professionals in making Gabe’s life as happy and comfortable as possible. Recently, the Dehens were approved for in-home nursing, which has given them some much-needed breathing room.

Dr. DeVries said most people have no idea the burden placed on parents who have children with special needs. Though it can be unimaginable difficult, he said, providing the around-the-clock care their child requires is not an option for families like the Dehens.

“These children deserve that care, deserve that love,” DeVries said. “They deserve every opportunity that they can get.”

Gabe is popular with most everyone he meets. The doctors and nurses at the hospital. David and Christina’s friends and family.

There are moments when Gabe’s personality unquestionably shines through. The rare “coo,” an ear-to-ear smile or a reaction to an illustration in one of his favorite books all bring the Dehens the joy any parent knows.

David said he sees Gabe’s personality most clearly when his medications start wearing off.

“If this disease wasn’t there, he’d probably be a cut-up,” he said.

Sharing their story

The couple said part of the reason they feel compelled to tell their story is because they hope others can learn from what they’ve been through. They hope it inspires others to see the needs in the lives of those around them and to do something to help.

“The easy things are right in front of you,” Christina said.

It’s the same reason David and Christina gave approval for Dr. DeVries to publish details about their son’s case. Those data points, they hope, could help the next family who learns their child suffers from Gabe’s condition.

They also hope telling their story will benefit others in less tangible ways. The couple finds comfort in reading stories about families in similar situations, and Christina has found relief in talking with other mothers she’s connected with through the hospital’s neonatal intensive care unit.

“If it weren’t for other people telling their story, we wouldn’t be able to get that comfort and support,” Christina said.

The best advice

The best advice he can give parents in their position, DeVries said, is the same advice he would give any parent.

“Love your children unconditionally and fiercely,” he said.

That perfectly describes the love David and Christina have for Gabe, DeVries said.

“This was not something that they signed up for,” he said. “But they’re following through with it. They’re caring for him and they’re loving him.”

At the heart of that unshakable love and devotion in such a difficult situation, they say, is their faith in God. Without it, they don’t know where they’d be.

One Bible verse in particular gives David hope in dark moments — Psalm 3:3:

But You, O Lord, are a shield for me,
My glory and the One who lifts up my head.

Gabe cannot lift his head on his own, meaning the verse echoes the Dehen family’s own prayers.

“It’s very specific of the miracle I need for my son,” David said.

It is prayer his parents credit with where they are today — with Gabe relatively happy and comfortable, and currently on one of his longest streaks out of the hospital since he was born.

His last hospital stay ended on April 10. His third birthday is less than three months away and they are already making plans for how to celebrate the milestone as a family.

The Dehens never know how long they’ll have with their son. That fact inspires them to treasure every Mother’s Day, every birthday, every smile and every coo.

“Until he breathes his last,” David said. “I’m never going to give up hope.”

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