Our Dev - Forever in Our Hearts
The trauma of losing a child tears some couples apart. Others become even closer. Mandeep and Inder are among the latter. Inder says losing their infant son, Dev, has made them stronger as a couple and as parents, "We don't focus on the little things anymore. They just don't matter."
Mandeep and Inder met in middle school. Despite parting ways for a while, they met again in college, realized they wanted to be together always, and they married. They both knew they wanted to have at least two children. Their first child, Sarvin, was born six years after they married. Mandeep describes being so wretchedly sick throughout the pregnancy that her doctor ordered bi-weekly checkups and ultrasounds; however, the pregnancy resulted in a happy and healthy baby boy. Just ten months after Sarvin was born, the couple was surprised and delighted to learn that they would soon have a second child.
A Textbook Pregnancy, Until It Wasn't
Being pregnant in the height of the COVID-19 pandemic was particularly challenging. Medical restrictions meant Inder wasn't allowed to accompany Mandeep to her prenatal visits. He relied on kind nurses willing to video chat him to see the first ultrasounds of their baby.
Mandeep says this pregnancy went more smoothly than her first. Her only concern was that she felt exhausted all the time, but her doctor considered that normal given she was working from home all while caring for an energetic toddler.
When Mandeep went for her 37th-week checkup, she reiterated how exhausted she felt. "I'm really tired. I'm struggling to walk five feet. Is that normal?" The doctor let her listen to the baby's heartbeat, reassuring her that the heartbeat was strong; he was healthy. “I guess you can say I was naïve then, trusting that a heartbeat equals a healthy pregnancy." Mandeep's doctor sent her home, saying the next time she would see her would be for her scheduled Cesarean section.
Later that same day, Mandeep woke exhausted and thirsty. She trudged to the fridge, grabbed a cold drink, and chugged it. Mandeep says her baby had always been active, especially when she drank something cold. This time, though, there was no response. "And that's when the panic set in. I'm not feeling him move. Actually, I don't remember the last time that I felt him move."
Mandeep called her doctor, who advised her to go to the hospital to get checked out. When she arrived, a nurse handed Mandeep a massive glass of ice water and told her to drink it. Still, her baby didn't move. "There was a bit of reassurance when I heard the heartbeat, but I kept asking why he wasn’t moving? And they couldn't answer that." Then her baby's heart rate started dropping, and she was told she needed an emergency C-section. Mandeep called Inder and told him the baby was ready, and he needed to get to the hospital. "I didn't disclose to him that there was a possibility that something was wrong."
Inder arrived just as the surgery began. As the surgeon removed the baby, a boy, Mandeep felt a sense of relief that quickly turned to fear as she realized she hadn’t heard him cry. A surgical team member standing at her shoulder said, "You have to keep in mind the baby was in a lot of distress. Maybe there's fluid in his lungs. You have to be patient.”
Inder asked the surgeon if anything was wrong, and she answered him cryptically, "He's a little slow to come around.” The surgeon told Inder they were taking the baby away for further examination and monitoring. Someone held the baby up for Mandeep to see before whisking him away. She says, "I felt robbed that I never got to hold him right after he was born. I wonder what that would have felt like."
Anxiously Waiting for Answers
Inder and Mandeep named their son Devraj, but they call him Dev for short. A few hours after he was born Dev almost “coded,” which is a short-hand medical term for cardiac arrest. The medical team decided to transfer Dev to a Toronto hospital that specializes in treating sick children. The surgeon told Inder that Dev's lungs might still have fluid in them, and he required more specialized care. Mandeep was still recovering from the C-section, so Inder followed the ambulance.
When he arrived at the new hospital, Inder says a team of about a dozen medical professionals met their ambulance as it pulled in and immediately began working on Dev. He was not expecting so many people and as he watched his tiny baby get hooked up to tubes and machines, the serious reality sank in.
A couple of hours later, Inder met the lead doctor who told him Dev was stable, and they were taking him off intubation to see how he fared breathing on his own. The next morning, Dev had to be intubated again. The doctor told Inder that Dev's blood pressure was inexplicably high, and they were running tests to determine why.
A Devastating Diagnosis
On the sixth day, the doctors told Mandeep and Inder they wanted to meet with them both in-person to discuss Dev's condition. Due to COVID-19 restrictions, Mandeep still hadn't been to the children's hospital, so she was surprised to see so many doctors when they walked into the meeting. After introductions, the head of the complex care team told them that Dev had a rare genetic disorder called Generalized Arterial Calcification of Infancy, or GACI. The doctor explained that the condition causes calcium buildup in the blood vessels. Mandeep and Inder still recall the exact words the doctor said to them next, "Unfortunately, the condition your son has is incompatible with life."
Hearing these words, Mandeep felt so devastated she couldn’t breathe and needed to rip off her mask to gasp for air. After several minutes, she asked the doctor how much longer Dev had to live. The doctor replied that she didn't know – maybe days, weeks perhaps, but most infants with GACI do not survive beyond six months. Inder says that after hearing this diagnosis, everything else was a blur. They simply could not process any more information.
Later, they read the notes that a social worker took during the meeting and learned that GACI is an autosomal recessive condition. This means that babies with GACI have inherited two defective genes, one from each unaffected carrier parent. Dev had GACI Type 1, also known as ENPP1 Deficiency, caused by a mutation in his ENPP1 gene. This genetic deficiency results in low levels of pyrophosphate, a metabolite that regulates calcification in the body. Without this proper regulation, calcium can build up in the walls of the blood vessels, restricting blood flow and damaging multiple body systems. One thing they saw in the social worker’s notes gave them a glimmer of hope: infants with GACI who make it to six months stand a better chance of survival. So, Mandeep and Inder became laser-focused on getting Dev past that six-month milestone. On the advice of their doctor they connected with GACI Global, an advocacy group, and found support, answers to their questions, connection with other families affected by GACI, and tips for advocating for Dev’s medical care.
The first big test was successfully taking Dev off intubation. The risks were high; it was a very real possibility that Dev would go into cardiac arrest. Dev surprised everyone, though. He survived and responded well to his medical treatments: infusions of sodium bisphosphonate, which reduce calcium buildup. Over the next 15 days, Dev's blood pressure decreased, and he began to eat more and gain weight. Mandeep and Inder could hold and cuddle him now. As Dev grew stronger, doctors allowed one parent at a time to take him for stroller rides around the hospital.
Next, the doctors allowed Dev an extra-special visitor: his older brother, Sarvin, who was 17-months old when he met his brother. There was an immediate connection between the two; it was a beautiful moment that the family was able to capture on film. Weeks later, the family was overjoyed when the lead doctor told them Dev was well enough to go home with them.
Dev arrived home on a Wednesday afternoon. This was the first time their family was finally able to be together, and they were ecstatic. "We lost track of time. We lost track of what day it was because we were so happy." They all sat together, taking turns holding Dev. Mandeep describes his long, silky black hair and huge brown eyes. “He was very alert and loved being held and getting hugs and kisses.”
One morning, Inder woke around 6a.m. Deciding to let Mandeep sleep, he got up to feed Dev. Dev didn't want to eat and clung to Inder, not wanting to be put down. So, Inder held his infant son for the next three hours, savoring the time with him. Later that afternoon, they started to worry when Dev, always a good and consistent eater, still didn't eat. When they noted a slight change in Dev's breathing, like a murmur, they called 911. The paramedics arrived, but, unfamiliar with GACI, they were not overly concerned after examining Dev. Mandeep and Inder insisted the ambulance crew drive Dev to the children's hospital for a checkup anyway.
Back to the Hospital
About ten minutes away from the hospital, Dev's heart stopped beating. The ambulance crew pulled over and administered CPR. It took about two minutes to get Dev's heart going again. Afterward, they rushed him the rest of the way to the hospital. There, Dev's heart stopped again, this time for eight minutes.
When they were able to see Dev, Mandeep says he was hooked up to "more wires and machines than ever before, and he didn't respond to us because he was so sedated." The trauma doctor told them if Dev had a third heart attack, he wouldn't make it. Both parents stayed with Dev and he survived through the night. From then on, it was hour to hour and then day to day. They explored every option, including the possibility of a heart transplant, but they were told there wasn't enough time. Inder described the helplessness he felt in that moment, "Your child is dying right in front of you, and you can't do anything."
Dev took his last breath while lying in Mandeep's loving arms.
After Dev was diagnosed with GACI, Mandeep, Inder, and Sarvin underwent genetic testing. Through testing, they learned they all have mutations in their ENPP1 gene, but they are unaffected carriers and not at risk of developing GACI. Mandeep felt particularly strongly about getting Sarvin tested. In Canada, insurance only covers genetic testing of minors for rare genetic diseases when symptoms are present. The family was able to secure testing for Sarvin by working with one of their doctors to successfully argue that some children with GACI don't develop symptoms until after 18 months of age, and early detection and treatment is critical to improve the chances of survival.
Advocacy and Telling Dev's Story
“We’re only just learning to cope with Dev not being here.” Mandeep explains that along with losing Dev, they’ve lost all the potential milestones that parents dream about: hearing Dev utter his first words, watching him take his first steps, cheering as he graduates high school, and seeing him eventually marry someone who would love him the way she loves Inder.
Mandeep and Inder have found solace in connecting with other families with children born with GACI, who they meet through GACI Global, an advocacy group for patients and families affected by GACI/ARHR2. These families understand Mandeep and Inder’s experience and provide support during this devastating time.
The family has made it their mission to give back to the children's hospital and the doctors and staff who worked so heroically to help Dev.
In telling Dev’s story, Mandeep says they honor his life. "We want people to be able to look up his name and know that he existed, to know that he is loved." They also hope Dev’s story raises awareness about GACI and encourages better access to genetic testing and additional research. "Hopefully one day there's a cure, and no other parent has to go through what we did."