Hearts for Ezra: How this baby boy’s death could change your life

Ezra Bessey was born May 30, 2012, and died a little more than eight months later. In that time, he was loved by his mother, Emily; father, Ethan; and sister, Daisy, now 4, and was largely content with the world. He smiled often and charmed people with his dimples, long eyelashes and big, bright brown eyes.

Now, he is having a positive effect on those who never met him.

Hallowell, Maine -- Oct. 2012 -- Ezra Bessey is pictured shortly after he was diagnosed with spinal muscular atrophy. After he died on Feb. 5, 2013, his parents, Emily and Ethan Bessey, started the nonprofit Hearts for Ezra to raise awareness and fund research for a cure.

Hallowell, Maine — Oct. 2012 — Ezra Bessey is pictured shortly after he was diagnosed with spinal muscular atrophy. He died on Feb. 5, 2013, and his parents, Emily and Ethan Bessey, started the nonprofit Hearts for Ezra to raise awareness and fund research for a cure.

Wednesday, Feb. 5, marked the one-year anniversary of his death from spinal muscular atrophy, a genetic disorder that destroys the nerves controlling muscle movement. As many as one in every 6,000 babies is born with the disease, and one in 40 people is estimated to be a carrier of the gene that causes it.

The passing of a year doesn’t make it easier to reconcile the loss of a baby, said Emily, 38, of Hallowell.

“A part of me doesn’t want it to feel better. My child is a part of me, so the sadness is, I don’t know, not that I welcome it. It’s what I would expect, to lose a part of your family, this little, beautiful, perfect child. It should feel sad,” she said.

For the Bessey family, time often felt like it was running out. That’s because spinal muscular atrophy, also called SMA, has no cure.

Hallowell, Maine -- Oct. 2012 -- Ezra Bessey is pictured with his sister, Daisy, after he was diagnosed with spinal muscular atrophy. After he died on Feb. 5, 2013, his parents, Emily and Ethan Bessey, started the nonprofit Hearts for Ezra to raise awareness and fund research for a cure.

Hallowell, Maine — Oct. 2012 — Ezra Bessey is pictured with his sister, Daisy, after he was diagnosed with spinal muscular atrophy. 

About four months after he was born, Emily noticed Ezra wasn’t applying pressure through his feet or holding up his head. Their pediatrician sent them to a pediatric neurologist, who did the blood work necessary to make a diagnosis. All the while, Emily and Ethan prepared themselves for the fact that their son might have to grow up in a wheelchair or that they might have to retrofit the house. They thought they could figure it out.

They didn’t expect to be told he had Type 1 SMA, the most severe form, and that there was no hope for their son’s life.

“It was just like nothing we had ever expected. The furthest thing from our minds was that our child would have a terminal illness and die four months later,” Emily said.

At the time, she wanted to know many things, including what to expect about his death. How would they know it was approaching? What would it be like for Ezra? She remembers the doctor telling her the disease creeps up: “They’ll be fine, and then they’ll be gone.” So every time she put him down to sleep, she wondered whether he would wake up. When would the disease steal him away?

She began to chronicle the experience, capturing her memories, photos and videos in a blog she named “Sweet Ezra.” In one post she describes why she had to write: “The pressure I already felt at recognizing how fast our kids grow up was compounded by the reality of having such fleeting time with Ezra. Now these photos and videos are what bring us comfort. They help replace the images of Ezra dying in our arms and allow us to remember our boy as he was for the majority of his life — full of vibrant joy.”

Hallowell, Maine -- Jan. 14, 2013 -- Ezra Bessey with his mother, Emily Bessey, about three weeks before he died of spinal muscular atrophy.

Hallowell, Maine — Jan. 14, 2013 — Ezra Bessey with his mother, Emily Bessey, about three weeks before he died of spinal muscular atrophy.

Preserving Ezra’s memory was also one of many reasons Emily and Ethan decided to start a volunteer-run nonprofit called The Hearts for Ezra Foundation, which helps them spread the word about SMA and raise money for research to find a cure. Most of their funds go to Families of SMA, a charity that funds and directs research and provides support for families with loved ones who have the disease.

“People around the world know him and love him. They know his smile and the brightness of his eyes. For an 8-month-old baby to be able to touch people around the world gives me a little bit of peace,” Emily said.

She wants the public to know about SMA, so families can prepare. If both parents have the gene, there’s a one in four chance their baby will have SMA. That means a couple could already have a healthy child and not realize they are carriers. It’s easy to get screened to see if you have the gene. Your doctor can do it, or you can go through a genetic testing company such as Counsyl.

Despite the relative prevalence of the disorder, Emily said she finds few people are aware of SMA. That’s another reason why she sees room for improvement in the way physicians prepare patients trying to have a child. Shouldn’t doctors do more to inform women and men planning to have children about the possibility of SMA and other genetic disorders and how to test for them?

Federal legislation — The Newborn Screening Saves Lives Reauthorization Act of 2013, of which Sen. Susan Collins, R-Maine, is a co-sponsor — would create a timeline for evaluating which disorders will be recommended for infant screenings. While the bill would make it slightly easier for SMA to be added to the screenings done after a baby’s birth — thereby helping detect the disease before symptoms show up — there is debate about whether having every infant screened is worth the cost, especially if there’s no effective treatment.

What is clear is that it’s helpful for parents to know whether they are carriers before they decide to have children, so they can make choices that are right for them.

Emily said that, to her, information is power. Before she had Ezra, she didn’t know she and her husband were both carriers. “I’d rather know what to do, so I can plan for it,” she said. “I’ve seen that everything isn’t always OK.”

Hallowell, Maine -- Nov. 2012 -- Ezra Bessey, 5 months, with his father, Ethan; his mother, Emily; and his sister, Daisy.  Photo by Jeff Dachowski

Hallowell, Maine — Nov. 2012 — Ezra Bessey, 5 months, with his father, Ethan; his mother, Emily; and his sister, Daisy. | Photo by Jeff Dachowski

Last Sunday, she said her family talked about the prior year’s Super Bowl game. Something that night was so funny to Ezra — perhaps the lights flashing on TV — that he couldn’t stop laughing. It was the last time they really heard him laugh. The next day he was worse — vomiting and with a fever. He slept a lot and wasn’t smiling. He had been sick like that before, Emily said, but something felt different.

The next two days dragged on as they watched their baby die. Before he passed away in Emily’s arms, he opened his eyes and locked eyes with his mother. “It was the most powerful gift of love he could have given me,” she said. Then he looked over at his father, closed his eyes and was gone.

FOR MORE INFORMATION

Want to help? From noon to 4 p.m. Sunday, March 9, attend the second annual Hearts for Ezra auction and fundraiser, at Hall-Dale High School in Farmingdale, with music by Rick Charette and magic by Dana Perkins. There will also be a silent auction, kids activities and a bake sale.

Want to learn more about Hearts for Ezra? Check out www.heartsforezra.org.

Want to volunteer? Email heartsforezra@gmail.com.

Want to read more? Check out Emily Bessey’s blog at ezradavid.wordpress.com.

Erin Rhoda

About Erin Rhoda

Erin Rhoda is a writer and storyteller. As editorial page editor of the Bangor Daily News, she writes the newspaper's opinion on matters from Kittery to Fort Kent.