The Amateur EMT
~by Brian Latimer
Alex was born at home on September 2, 2007. While the pregnancy and delivery had progressed normally, we saw right away that Alex was different. The midwife at first noted his clubbed feet and that his fingers were unusually long for a baby. He also kept his hands clenched into fists and at the midwife’s insistence we took Alex to Markham Stouffville Hospital where our medical journey began.
Stepping deep into the medical world would at first prove to be a stressful and demanding experience. Fortunately, what was at first strange and foreign soon gained our trust and admiration.
After genetic testing revealed a mutation in Alex’s DNA, Jacqui and I were also tested. The tests revealed that Alex’s condition – now known as Loey’s Deitz Syndrome – was in his case, a random mutation. We were then referred to Dr. Tim Bradley at Sick Kids Hospital in Toronto where he specialized in the study of LDS and told us that our son, at the time, was the youngest person ever diagnosed with the condition.
Imaging soon revealed that Alex had been born with a dilated aortic root. Dr. Bradley had been closely monitoring the situation and told us right away he would need surgery “sooner rather than later”. So at 18 months of age, Alex had his first successful aortic repair surgery. I remember being with Alex in the waiting room watching him play with the toys, oblivious to what was about to happen. A week later, we were home again and moving on with our lives, scheduling in the regular appointments at Sick Kids to manage the many other complexities of LDS.
We continued this way for 9 years until one January evening as we were sitting down for evening prayers, Alex started crying telling me his shoulder hurt; then his jaw hurt; then it was his back. He immediately broke into a sweat and we knew something was seriously wrong. In the back of my mind there was a story I’d read in the GADA Newsletter describing the symptoms of an aortic dissection and the intense, non-localized pain that resulted from it. We rushed Alex to Bowmanville Hospital where he was kept under observation until an Ambulance could arrive to take us to Sick Kids.
At Sick Kids there was more monitoring, some pain management and a CT scan. Finally around 4:00 a.m., I was informed that Alex’s aorta had dissected from the site of the original graft operation all the way down into his thorax and possibly further. Emergency surgery was immediately scheduled to replace the aortic arch and most of the descending aorta. Alex came through with flying colours and we were home again after a week, but we knew we weren’t done yet. While the surgeons did their best, it wasn’t possible for them to completely replace the dissected parts of Alex’s aorta. They told us to hope for the best outcome and that a further surgery would be needed soon.
We spent three weeks at home praying every day for Alex’s health and walking on eggshells knowing that the remainder of his aorta could re-dissect at any time. We had also been told his aorta could rupture and would most certainly be fatal. Fortunately the worst did not occur and while his aorta did dissect again, we had plenty of time to get him down to Sick Kids and back under close observation in the Cardiac I.C.U. The nurses there are some of the most amazing people I’ve ever met. We stayed the night in the I.C.U. as I grew increasingly certain Alex would not be going home for a while.
A team of surgeons had met to discuss Alex’s case and it was their feeling that his aorta was not stable enough to be left alone. Another surgery was needed. I broke the news to Alex, who was less than pleased at the prospect. Jacqui and I were nervous, of course, but also relieved. The waiting game was ending and the worst had been avoided. By this time we had enlisted an extensive mailing list of people and I was sending out regular updates and asking for continued prayers.
We met with Alex’s surgeon, Dr. Maral Ouzounian, just before she went in to the O.R. She left us feeling reassured and ready for the long day ahead. I remember she turned back and waved to us just before she walked through the doors. It was a small courtesy on her part, but it made us feel much better.
The Thoracotomy she was performing was considered high risk, very invasive and required a long incision. The recovery from this type of procedure is long and very painful. I spent hours sitting by Alex’s bedside as he drifted in and out of a morphine-induced slumber. We went through many ups and downs during Alex’s recovery. We worried about him developing a morphine dependency (not uncommon on long recoveries), about his bowels, his lower back, and the nerves that led to his legs. We worried about a collapsed lung and fluid build up in the pleural space behind the lungs. There was also a brief but terrifying allergic reaction to a routine Vitamin K shot. He was on a ventilator for four days after that and had to return to the I.C.U. because of some bleeding in his stomach, possibly caused by an ulcer after being so long without solid food.
At this point I had been by Alex’s side for almost three weeks non-stop. Jacqui would come and relieve me for a day here and there, but with six other kids at home and myself being unemployed, it was pretty obvious who should stay and who should go. I joked with Alex that I actually had a job and my title was “Amateur EMT Technician”. I drove our “ambulance”, saw to his comfort, fetched drinks and smoothies at all hours of the day or night, changed his sheets and clothing, met regularly with teams of doctors, surgeons, and nurses, and just generally advocated for the best care and attention I could get for him.
And then, one day, after Stockholm syndrome had completely overtaken me and I was convinced that I would never leave this place and maybe didn’t even want to, it was suddenly time to go home. Wait, did they say “Go Home”? Why would I want to do that? This is where I belong! But the news did eventually sink in and I came to believe it would be possible to return to life outside the hospital.
Until then, the most exposure I had to the world outside the hospital were my semi-regular night-time walks along Yonge St. after Alex was safely asleep. It was a strange thing to be out on the street and see all those people going about their business, as oblivious to what my son and I were going through, as I was to whatever path they might be on. I realized that the grumpy person you sometimes run into could be someone like me; someone with a loved one in pain or in danger; a loved one who was dying. There, but for the grace of God, go I.
By March 10th, 2016 we were back home once more. Alex was thin and frail and did not breathe very well for the first few weeks as the fluid in his left lung cleared up.
I am still in wonder sometimes as I watch him running around, playing and play fighting with his brother Matthew – a solid and athletic boy – who is the perfect foil for Alex; a spur in his side, driving him to compete as only a brother can.
Today, we are seven months post-op. Alex is still recovering, but to look at him, you’d never guess what he’d been through. I know now, the body heals faster than the emotions. I struggle every day to let him go a little more and stop worrying. Although he’d never have the words to describe it, Alex struggled for a long time with anxiety – worrying that something would happen that would take him back to the hospital for another surgery. The path to recovery is long, but it’s going steadily upward at last.