July 6, 2018- Henley Jane may only be a month old, but her story started a decade ago, with the birth of her big brother Ethan.
Henley’s parents, Tina and Darren McKown, said Ethan, their firstborn, smiled all the time. He smiled no matter what type of day he had. No matter how long he had to be at the hospital, or what kind of tests the doctors ran, Ethan smiled. In some ways, his happiness made his illness easier to bear, his parents said.
“When I would come home from work, he had this way of reaching out and hugging me,” Tina said, grabbing her own arms and squeezing, with a smile as she remembered her son’s embraces. “He gave the best hugs. Darren would hand him to me, and he would just snuggle up to me.”
Medical concerns existed with Ethan from the beginning. He struggled to gain weight. He often caught respiratory illnesses. After months of testing, Ethan received a diagnosis of Gaucher Disease when he was around 8 months old.
GAUCHER DISEASE DIAGNOSIS
Gaucher Disease causes fatty lipid deposits to build up on certain organs and bones. A genetic condition, experts separate it into three types. Doctors diagnose up to 1 in 40,000 live births in the general population with Gaucher Disease, the majority with type one, which may not have any symptoms at all. The more rare type two involves brain stem abnormalities causing it to be fatal, usually within the first two years of life. Type three has a severity between types I and II, according to the National Gaucher Foundation, and while patients typically have a shortened lifespan, some can live into their 50s with treatment.
Initially, doctors diagnosed Ethan with type three, so they began treatment. Soon after, however, doctors changed his diagnosis from type three to the more severe type two.
Their time with Ethan would be much shorter than they initially thought.
The diagnosis devastated them, but they remember his laugh, which they described as simply the best. The placement of his tracheostomy tube silenced him though. They no longer heard that sweet sound. Still, no matter what, Ethan smiled.
“His sense of resilience—anyone who ever saw him talked about it, about how he was always smiling,” Darren said.
Darren said the treatment probably extended Ethan’s life. It gave him an extra six to eight months with his parents. But once he was re-diagnosed with type 2, insurance would no longer pay for the treatment. As treatment stopped, Ethan’s symptoms became more pronounced. Soon after, he died at the age of 2 years and 3 months.
SHARING THE GRIEF
The death of their child devastated the McKowns, but they turned to each other. Often, following the death of a child, couples face a higher rate of divorce. The McKowns, however, leaned on each other. No one else could understand what they went through. Other parents know the horrible pain of losing a child, Darren said, but only he and his wife know the very specific pain of losing Ethan James.
“For me, there was no other choice,” Tina said.
She said she relied on her husband for support. Acknowledging they were both experiencing the same pain, at the same time, she gave him her support in return.
Grief for their firstborn will be lifelong. The couple needed time to recover before even considering having another baby. Darren said they simply escaped for a while. Furthermore, because both Darren and Tina are carriers of Gaucher Disease, they decided to do in vitro fertilization treatments with embryos testing free of Gaucher Disease. When both parents carry Gaucher Disease, their children have a 25 percent chance of being born with Gaucher.
So, eight years after losing their smiling boy, Darren and Tina made the decision to have another baby. They began in January of 2017. The first round of IVF didn’t take, but the second did. Eight months later, the couple held their little girl in their arms in a moment Darren described as surreal.
Tina said Henley’s facial features remind her so much of Ethan. When she smiles, they see both her big brother in her expressions and her own emergent self, too. Tina said she’s curious to see if her mannerisms will be anything like her big brother’s, as well. Darren said they love Henley with everything they have, but Ethan, and his loss, which Darren described simply as a hole, will always be with them. They love Henley for Henley’s sake, and not as a replacement for her big brother Ethan.
Moments catch Tina’s breath in their bittersweetness. Although Henley is their second child, they also experience many firsts, as well. Since Ethan came early, Tina said once she got past 33 weeks, when Ethan came, she would find herself realizing, “This is the most pregnant I’ve ever been.” They know, too, that Henley will develop in ways Ethan could not – such as crawling and eating solid foods. She looks forward to those developments.
Both McKowns said finding other parents whose children had the same diagnosis help them. With a rare disease like their son’s, they have had to go online to make those important connections. Tina said it’s often moms who are online, but that it’s important for dads to reach out, too. Darren said he’s made close friendships with a few other fathers who have made the same journey, and it’s helpful to have someone with whom he can talk freely since they understand.
Being Ethan’s parents for those two years and three months shaped both Darren and Tina. Darren said he used to keep emotions locked in tight, but now, he no longer worries about keeping that armor up. Instead, he lets it all out, he said. For Tina, Ethan taught her to appreciate every moment—even the little ones that seem like not much can be everything.
“Ethan changed everything,” Darren said. “He changed us all.”
Certainly, he shaped the beginning of the small sister who will grow up hearing the stories of her blue-eyed brother’s sweet laugh and being raised by parents who know the value of each precious moment.
The McKowns encourage people to learn more about Gaucher Disease. Neither of them knew anything about it a decade ago, Tina said. Now, fortunately, it is included in the typical newborn screening given to babies at birth, and more treatments have become available. To learn more about Gaucher Disease, visit the National Gaucher Foundation online at gaucherdisease.org.