‘He died in my arms’: Hamilton mom loses two children to rare Schwartz-Jampel syndrome

Jodi Davies is so grateful for the time she had with them. That helps her face the time without. Soon, she hopes, she can get back to work, which she had to give up for many years.

There was a time when Teah and Caleb weren't part of Jodi Davies' life, before they were born. But it was a time so pushed back into the shadows by the enormity of their day-to-day needs and joys that it must sometimes have not seemed real to her, their mother.

That time has returned, with a terrible force, but one that Jodi had been preparing for. They will always be, of course, a part of her ongoing life. But within the space of 14 months, both Teah and Caleb died; Teah (she was 21) just before 2020 started, and Caleb, just after it ended, this February past (he was 18).

Jodi's world has in some ways become unrecognizable to her. Teah and Caleb filled so much space in her days and in her living. In turn, she's almost unrecognizable without them, people were so used to seeing them together. She would do everything with them, including carry them up and down the stairs of their house, when they became physically unable to manage the climb. They seemed somehow attached."

I want people to know they're gone," says Jodi. It's hard for her to keep repeating. They see me and keep expecting the wheelchairs to come up behind."

Jodi is adapting. She has to. And there's a grandchild on the way. Her daughter, Haven, 20, is due any day.

I've written about Teah and Caleb before, their mother, Jodi, and brother and sister, Justin and Haven. Teah and Caleb were both diagnosed with something called Schwartz-Jampel syndrome. It's a rare genetic disorder that can distort bone growth and cause the skeleton to crowd development and placement of internal organs, resulting in breathing difficulties, heart trouble and fluid buildup.

When I wrote about the family between 2016 and 2018, they'd been through so much. Caleb, barely a teenager, had to choose between life and death. Whether to undergo a tracheotomy - it meant losing his voice, being confined to home; an altered life. If he didn't get it, he would likely die. Jodi let him decide. He chose the tracheotomy, but it made life harder. The care, the cleaning. He had to give up school.

Around that time, Teah was hit by a car while in her specialized wheelchair. Amazingly, she wasn't hurt but the chair, very expensive, was destroyed. There was much hardship, darkness. But there was also deep and abiding closeness, an exuberant and lively sense of fun and enjoyment.

Jodi made sure of that. She would go fishing with Caleb, find opportunities for them and encourage Teah in her studies to become an early childhood educator. They were bright, social, spirited young people.

All through it, there were many trips to the emergency, usually because of fluid buildup and severe respiratory distress, even congestive heart failure in Caleb's case.

Teah ended up in hospital during Christmas 2017. She pulled through. But often she was close to not making it.

I was always optimistic," says Jodi. Still, she also knew the realities. And she was always on call.

In late December 2019, they found themselves once again in hospital. Teah.

The day before she was hospitalized, she was Christmas shopping at Lime Ridge Mall, on the 23rd. She came home and got Haven to go the store to get her a blue freezie. She loved blue freezies. But the next day her oxygen levels went really low.

Her one lung was not opening at all and the other was only at 40 per cent and full of pneumonia."

She was not conscious the whole day of Dec. 28, and then she was gone.

Teah's death scared Caleb. So, needless to say, 2020 was a hard year, harder than most, and COVID-19 didn't help, as it made them dread hospital visits even more than normal.

Caleb, Jodi and the family spent as much time together as they could, doing things Caleb loved to do - fishing, video games, practice shooting, driving his remote control car.

The winter months were tough. He'd always say, When's it going to be nice out again?' so he could go fishing."

Then, early this year, the family noticed Caleb seemed lethargic. He was sleeping more, and yet constantly waking up every couple of hours needing his trach to be cleared

Every time he lay down, he'd need suction. I slept three hours in eight days," says Jodi of the week before Caleb died. He was always apologizing. I was happy to do it."

One day, he got to the point at which he was needing 10 litres of oxygen. Then he couldn't breathe on his on.

They went to the hospital. He didn't seem himself, says Jodi. At 9:36 a.m., he was on the gurney and texting her as she was still outside the hospital. She rushed in, caught up to him. At 9:58, he was gone.

He died in my arms," says Jodi.

Now, says Jodi, she makes a conscious effort every day to think positively. She has Justin, Haven and the grandchildren (Justin's sons Connor, 7, and Lennox, 1). And, a new French bulldog puppy.

I didn't know how long I'd have them for," Jodi says of Teah and Caleb. Caleb got five extra amazing years (because of the tracheotomy) - fishing, boating. Quality of life. We were a team. They were the boss. I never tried to talk them out of anything."

They received great care, she adds. Doctors, nurses, other caregivers came to adore them and were devastated when they went.

I was blessed to be called their mother," says Jodi. Every day they'd fight, without complaint. Now they're free of pain."

Jodi asks that people support NORD (National Organization for Rare Disorders) at rarediseases.org

(Since the writing of this article, Haven has given birth to a boy, Clayton, and all are doing well.)

Jeff Mahoney is a Hamilton-based reporter and columnist covering culture and lifestyle stories, commentary and humour for The Spectator. Reach him via email: jmahoney@thespec.com

A confusing genetic picture for Davies family

Teah and Caleb and the family will always be remembered for their outstanding characters under great duress and also for helping raise awareness around SJS and generally for those among us, especially young people, with similar challenges.

Teah and Caleb were diagnosed with SJS years ago but in a confusing development, they were both found to be nonconclusive for it in genetic tests, says Jodi.

SJS does have a genetic component but Jodi says there is no history of anything like it on either parental side.

Still, Jodi worries about her grandchildren. Justin's sons don't have any signs. She hopes the same holds true for Haven's.