Spirits brightened
Pictou County girl who lit hospital tree battling rare genetic illness
NEW GLASGOW, N.S. — On July 15, 2019, Sharayah Wartak’s worse nightmare came true.
Her six-year-old daughter Tirza had been complaining of stomach pain for several days. At first, she and her husband Mark passed it off as a stomach bug, but when it didn’t go away, Sharayah took Tirza into the emergency department at the Aberdeen Hospital.
She had a nagging fear of what it might be, but prayed she was wrong.
The doctor on duty said the little girl’s vitals were all stable, so he wasn’t worried. He suggested giving her pain medication and sending her home. Or, if Sharayah wanted, he would have some bloodwork done.
She opted for the bloodwork, suspecting the worst — that her daughter might have the same gene mutation that Sharayah, her father and two siblings have.
“An hour later I got the crushing news that I was right,” Sharayah said. “My daughter’s lipase were through the roof and she was in the middle of a pancreatitis attack.”
The gene mutation is referred to as PRSS1 and is tied to chronic pancreatitis. The pancreas is supposed to release juices that aid in the digestion of food. For a person with pancreatitis, the pancreas doesn’t always work properly, and the digestive juices end up eating away at the pancreas itself. For those with the PRSS1 gene, the attacks generally worsen over time as the pancreas becomes more and more damaged. It results in chronic pain, diabetes and eventually pancreatic cancer.
Pancreatitis is diagnosed through bloodwork that shows elevated lipase levels. Genetic testing is needed to confirm the PRSS1 gene.
“I knew each of my kids had a 50 per cent chance of carrying my disease, but still I prayed to be wrong,” Sharayah said.
MOTHER’S DIAGNOSIS
Sharayah was well acquainted with pancreatitis at the time of her daughter’s diagnosis, but was shocked Tirza developed it so young.
Sharayah’s own pancreatitis didn’t show up until after she suffered abdominal injury from a horseback riding accident at the age of 18.
Her father had it, however, and had to have a stint put in. Then, two of her brothers were diagnosed. Suddenly what she thought was trauma-induced pancreatitis seemed more likely to be an inherited illness. Genetic testing confirmed it.
After living with chronic pain for more than a decade, Sharayah finally sought relief through a relatively new and innovative surgery that one of her brothers had last April. It involves removing the pancreas and several smaller organs in the abdomen and realigning the remaining organs to function without them. As part of the procedure, doctors perform an islet cell transplant, which involves taking cells from the pancreas and implanting them into the liver. The goal is to allow the liver to act as a pancreas and prevent the person from becoming a Type 1 diabetic.
Sharayah had the surgery on Aug. 13 in Edmonton. It was performed by Dr. James Shapiro, who is considered a leader in the field of islet cell transplant. In fact, the procedure he developed is referred to as the Edmonton Protocol and is now used throughout the world.
The surgery is intense, and more than three months later Sharayah is still recovering. She’s told it can take up to a year to fully heal.
“I woke up and the pain was completely different. The sharp stabbing pain I had been living with every moment of every day was gone. I had a new pain and I still have pain, but it’s a different pain. It just comes in waves now.”
Unfortunately, her pancreas had been so damaged from previous attacks that there were not enough healthy islet cells to be transplanted. As a result, she is now a diabetic. But she knows that it’s better than the alternative. She says Shapiro told her that if she hadn’t had the surgery, it was only a matter of time before she would have developed pancreatic cancer.
“He said there was just a matter of time before cancer would start. It was not an if, but when.”
ANOTHER DIAGNOSIS
Last fall, the Wartaks got more devastating news. Their twoyear-old daughter Ellie was also diagnosed with a pancreas attack. Then in November, Tirza had her second attack and spent a week in hospital.
Already, the Wartaks have been communicating with Dr. Shapiro in hopes of intervening early enough for the girls, so their pancreas doesn’t become as damaged as Sharayah’s was.
But there is a lot to be weighed. On one hand, the younger they have the surgery, the more likely they’ll be to get healthy islet cells to transplant, which could prevent diabetes. They’d also likely recover easier. On the other, it’s a lot for a kid to go through.
“It is a very serious massive surgery,” Sharayah said.
Through it all, she’s been thankful for the staff at the Aberdeen Hospital who have showed love and care for her daughters.
Tirza has said she wants to be a nurse now.
Friday, Dec. 6, Tirza had the special honour of turning the switch on the NS Power Christmas tree that’s put up in front of the hospital. NS Power gave the girl gifts in thanks of helping to light the tree.
“It was a positive experience,” Sharayah said.
She hopes there will be more of them in the year to come.