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The Measure of Muskaan

First her breath quickened. Then she stopped drinking milk. Finally her lips turned blue.

Only 10 days old, Muskaan Grewal had been settling into her new home in Surrey, B.C., with her father, Harman, her mother Sukhjit, and her six-year-old sister, Karina. The initial health she had radiated at birth began to fade. Worried, Muskaan’s parents took her to the family doctor. He suggested they bring her to the emergency room; something was wrong with her heart.

Doctors admitted Muskaan to the intensive care unit, where she underwent hours of testing. Her family gathered in the waiting room, certain that they would be greeted with welcome news. Then the doctor came out.

“Her heart is like a nut and it’s not pumping enough blood,” Harman recalls hearing from the doctor. She suffered from a rare heart condition called dilated cardiomyopathy that affects one in 100,000 live births each year. It was a foregone conclusion: Muskaan would need a new heart.

“There are no words coming out. No sentences. Nothing,” Harman says. “We were just staring at each other and crying and shaking ourselves.”

Life suddenly felt fragile for the Grewel Family. They no longer know how to measure Muskaan’s life. In years, months, weeks or hours?

Every time the phone rings, Dr. Holger Buchholz’s heart rate shoots up.

He’s the go-to-person when all options have been exhausted and a child with a failing heart is too sick to wait for heart transplantation. When he first meets his parents, they’re children in an extreamly fragile condition.

Dr. Buchholz is the director of the Stollery’s pediatric artificial heart program. He studied cardiovascular surgery in Germany and was flown in by the Stollery in 2005 to help implant its first Berlin heart, the world’s first mechanical heart designed for children. Months later, he was asked to lead the Stollery’s ventricular assist device (VAD) program.

A VAD is a mechanical pump that supports a damaged or failing heart by circulating blood throughout the body. Dr. Buchholz oversees about 10 pediatric patients per year, most of whom are treated with a VAD until they are able to receive a heart transplant. This approach acts as a bridge to transplant that ensures their survival while they wait for a donor heart.

Implanting a VAD allows the patient’s organs to grow healthier, meaning less time in the hospital and reduced admission rates. Most importantly, the patients are in better condition when they undergo heart transplantation, shortening their recovery time.

When Dr. Buchholz started at the Stollery, the VADs were restrictive and bulky, connected to controllers as big as refrigerators. The risk of complications for patients was high and their quality of life was reduced because of the restrictive medical equipment. But advantages in medical technology in the past two years have led to a new form of long-term, permanent treatment called destination therapy, meant for patients who aren’t suited for a heart transplant.

That’s largely thanks to the Heart-Ware pump. It’s no bigger than a golf ball and can be implanted in patients that weigh more than 20 kilograms, typically at five or six years of age. In 2011, the Stollery was the first centre in Canada to implant a HeartWare pump in a pediatric patient. The 13-year-oled patient wore the four-pound controller in a carrying case strapped to her back.

Knowing his patients could return home helped alleviate some of Dr. Buchholz’s stress. But the distance brought a new host of problems. Would patients get the help they needed in case of an emergency?

“If something bad happens, it doesn’t happen on Tuesday morning at 11 a.m. It happens on Sunday at 1 a.m.,” he says.

So, it was inevitable that the phone call in August 2012 would come in the middle of the night. A five-year-old patient was being flown in from B.C. Children’s Hospital. She had just suffered a cardiac arrest and needed a device immediately. Her name, Buchholz learned, was Muskaan Grewal.

The doctors told them to prepare for anything, so Harman and Sukhjit learned to live day by day.

Following the news of Muskaan’s condition, they uprooted their lives to Toronto so that doctors could sample medications on Muskaan. She gradually responded to the treatment, and within months, the family returned home.

As she grew, Muskaan’s anxiety was crippling. When doctors approached her, she started to vomit. The smell of food would set her off and she would lock herself in rooms, refusing to come out.

But each passing day marked a better chance at a heart transplant. The wait, however, proved too long. At the age of five, Muskaan suffered a heart attack. Her parents performed CPR and rushed her to B.C. Children’s Hospital. She was already on a 24-hour medicine called Milrinone that helped her heart pump blood. It was the last resort for the transplant. Doctors told the family they would need to fly to Edmonton.

It was there that they met Dr. Buchholz and Selvi Sinnadurai, the Stollery’s VAD program coordinator. (Dr. Buchholz refers to himself as the famous one, but it’s Selvi who does all the work, he jokes.)

Together with Dr. Buchholz, Selvi helped the Grewal family through the extensive consultation process. They narrowed their options down to two devices: the bulkier Berlin Heart and the HeartWare pump. At 18 kilograms, Mushaan feel under HeartWare’s minimum recommended weight.

“The family was nervous about it, but were very strong and optimistic and fully aware of what was gong on,” Selvi says. The survival rate for VAD pediatric patients is more than 90 per cent.

Muskaan underwent surgery that week. Dr. Ivan Rebeyka, the Stollery’s chief pediatric heart surgeon, planted the HeartWare pump in her left side and stitched part of it into the left ventricle of her heart. A small tube ran through the skin of her upper abdomen, which connected to a controller the size of an iPhone.

The whirring motor in Muskaan’s chest sounded like a buzzing bee. The alarms on the device made her anxious and sometimes kept her awake at night, but she knew they were helping to keep her alive. Her family underwent 25 hours of training, learning how to understand the numbers on the monitor and change the battery pack every five or six hours.

Meanwhile, a team of specialists worked round-the-clock on Muskaan. She was soon able to walk from the bed into the hallway, and then out to the patio. “Once she started walking, she never, ever sat back down again,” Herman says.

The biggest feat was eating food again, since Muskaan had long relied on a feeding tube. Dr. Buchholz promised Muskaan that if she was able to eat by Halloween, he would take her trick-or-treating with his kids. She ate little by little every day, growing stronger and taller.

It was bitterly cold that Halloween and Dr. Buchholz had to carry Muskaan, but it didn’t matter to her. She posed for a photo with his two children showing off her toothy smile. The purple butterfly wings she wore hinted at a metamorphosis that was nearly complete.

By December 2012, Muskaan was ready to go home. Selvi flew to Surrey with the family to train Muskaan’s school teachers and draft an emergency plan with the local hospital. In addition to fire drills, Muskaan’s classmates practiced VAD drills. When Selvi flew back to Edmonton, the family maintained daily contact with the Stollery’s VAD team.

Dr. Buchholz walked into the darkened hospital room as Sukhjit was getting Muskaan ready for bed. It had been a long three months since Muskaan had been re-admitted to the Stollery in May 2013. She had suffered a small infection where the rube ran from her abdomen. Her condition was further complicated by a stroke from a blood clot in her brain.

Sukhjit turned on the light as Dr. Buchholz walked in. He asked if Muskaan was okay with getting a new heart right away. She wouldn’t have to worry about plugging her device at night, he told him. “I want it,” Muskaan replied before breaking into tears.

Harman had just flown back to B.C. the day before when he got the phone call at 9:30 p.m. Sukhjit was on the other end. “Muskaan has a heart,” she said. Harman couldn’t help himself – he started jumping with excitement.

The next day, Muskaan underwent heart transplantation over a gripping 10-hour stretch. “It’s scary,” Harman recalls.

Within four days, Muskaan started to walk again. After two weeks, she was discharged from the hospital. But before embarking on their flight back home, the Grewal family learned something remarkable. HeartWare, the company that manufactures the pump, said she was the youngest patient in the word to receive the device.

So, instead of a quiet exit, Muskaan and her family said goodbye in early August to a throng of hospital staff and reporters. As the camera lights flashed, Muskaan clung to her parents who smiled with pride, marveling at the little girl with the big heart.

In December 2013, Muskaan had a heart biopsy to see how her body was coping with the new heart. It registered a level zero, meaning there’s almost no change her body will reject her new heart. She likely won’t have to undergo surgery again until she’s at least 25 unless she becomes very ill.

For the Grewal family, life is getting back to normal.

“I have a normal sister now,” Karina, 13, says. “We hang out, watch TV and play games together. It’s more fun having an energetic little sister than the sick sister I used to have.”

“I like watching TV and playing games,” Muskaan, now seven years old, says shyly. Her favourite show is Spongebob Squarepants. His stuffed version kept her company for years when she slept in the hospital bed.

The Grewal family does miss one part of their past life: the Stollery staff. Last September, Harman and Sukhjit penned a letter to Buchholz and Sinnadurai.

“Dr. Buchholz holds a very special place in Muskaan’s heart,” they wrote. “Through Muskaan’s eyes he is seen as a superhero, who is always to the rescue, has the solution to everything, and can make the impossible become possible.”

Today the Grewal family is able to count Muskaan’s life in decades to come, and to make memories with her.

Like Muskaan riding the ghost train in Stanley Park for the first time. Like Muskaan jumping out of bed early her first morning of Grade 2, despite staying up late the night before to catch up on her reading. Like Muskaan finally getting the one present she had wished for every Christmas: a healthy, working heart.

Published Winter 2014, Stollery Children’s Hospital Foundation