After bringing her newborn twins home from hospital, Casey Hurst laid them down to sleep. When she heard a disturbing noise emanating from their room, though, Casey investigated and found that her babies’ breathing had become labored. And after she subsequently took them to the doctors, it was revealed that her boys were not as healthy as medical staff had previously believed.
Bryce and Baen Hurst had arrived into the world via cesarean section on March 3, 2011. Mom Casey knew to expect twins when the sonographer detected two separate heartbeats at the nine-week scan; after that measure, though, she would have no rigorous fetal testing.
“I didn’t want to know if there were problems before they came out,” Casey told The Chronicle in December 2011. “There were no problems [with the pregnancy] and all signs pointed to healthy babies.” In fact, the twins didn’t even spend time in the neonatal ICU at the EMH Medical Center in Elyria, Ohio.
After she had taken her sons home, though, Casey made that fateful discovery about their breathing. Still, doctors only believed that this was due to the hernias both boys had been born with. As a result, surgery was performed on the two-week-old babies, after which specialists had asserted that their health would improve.
Of course, Casey and her husband, Brian, kept a close eye on the boys during their first few weeks of life – not least because they had a feeling that their children’s problems weren’t over yet. “We didn’t know what it was,” Brian told The Chronicle. “We just knew that something wasn’t right. They were also slowly getting behind with their milestones.”
And unfortunately, things did indeed seem to gradually get worse. When Bryce was four weeks old, for example, Casey found blood in his diaper. One specialist believed, however, that it was simply due to an allergy to cow’s milk. And even more disturbingly, at the age of just six weeks, Baen suddenly began to suffocate. As Casey explained to The Chronicle, “He just turned blue on me.”
So, as the medical problems became more and more serious, Casey and Brian were referred to specialists at the Cleveland Clinic. They, however, suggested that Bryce and Baen may have a serious condition. Dr. Vickie Zurcher, the boys’ pediatric geneticist said, “[We] recognized that the Hurst brothers had a possible storage disorder at their first appointment, primarily based on the twins’ facial features.”
Although the babies appeared healthy, they had coarse features, with thicker-than-normal gums and arched pallets. These, combined with the already-detected symptoms and numerous physical problems exhibited in the boys, indicated to doctors one possible cause: Hurler syndrome.
On November 1, 2011, that diagnosis was confirmed – and the prognosis for the pair was not good. Some Hurler patients don’t make it to age five, while many more won’t see their tenth birthday, usually due to cardiac arrest or constricted airways. There is also currently no known cure for the condition.
However, Hurler syndrome can only develop if both parents carry the genetic defect which causes it. Neither Casey nor Brian would have been aware they were carriers, though, when they married in 2003 after meeting in high school. And they wouldn’t have known from their older children, either, as neither of them have the condition.
Dr. Zurcher explained, “When two carriers of Hurler syndrome have a child, there is a one-in-four chance that the child will be neither affected, nor a carrier; a half-chance the child will be a carrier; and a fourth-chance that the child will be affected with Hurler syndrome.”
It is therefore a lottery as to which children develop the condition and which ones don’t, meaning that Casey and Brian’s elder sons, Beau, 12, and Brady, 10, are very lucky indeed. Bryce and Baen, on the other hand, are the first recorded identical twins to develop the disease.
And University of Minnesota medical director Dr. Paul Orchard explained just how extraordinary the Hurst twins are while speaking to The Chronicle. He said, “Incidents of Hurler’s are one in 100,000. It’s a very rare… disease and even more rare [for the Hurst twins] because their parents got together [and they both carry the defective gene].”
As Dr. Orchard, the boys’ main physician, has explained, the twins lack the alpha-L-iduronidase enzyme, which is vital for breaking down molecules of sugar. If these molecules remain partially intact, though, they attach themselves to cells in the body. And that’s when the damage starts.
“The storage material builds up in all sorts of areas – the bones, heart, liver, lungs and in the brain,” Dr. Orchard said. “Once it’s in the brain, the patients start to lose ground.” What’s more, the disease is usually only detected after a period of declining health – so in the twins’ case, the early onset of the syndrome may have saved their lives.
For the Ginger Twins – as they are known, thanks to their shocks of red hair – their early diagnosis allowed for treatments to prolong their life expectancy. As well as weekly enzyme replacement injections into their spines, the then one-year-old Baen and Bryce received bone-marrow transplants.
Tragically, though, the pair had altogether different reactions to the bone-marrow surgery. Bryce had coped well with both the operation and the preparation for it; his twin brother, however, suffered a stroke. That had come after a series of seizures over the course of 24 hours. And even more worryingly, one of his lungs would collapse several days after the procedure.
However, the transplants eventually proved to have been a success. And against the odds, the twins turned five in 2016 and were attending school. Shortly after reaching that milestone, they both had surgery to treat their claw hands and carpal tunnel syndrome. And although they are both often in pain, they attend physical, aquatic and speech therapy to help them.
Meanwhile, on December 18, 2015, the Hursts welcomed their seventh family member. Casey knew the risks of her unplanned pregnancy, yet nonetheless proceeded. It wasn’t long after Brock was born, though, that the mom recognized the signs. Indeed, on January 28, 2016, tests confirmed that he, too, had Hurler syndrome.
And while no one can predict what’s ahead for Bryce, Baen and Brock, what is more certain is that dealing with their condition has brought the family together. Casey recalled, “Someone [said], ‘I can’t tell you why this is happening.’ But our friend said it’s a good thing they were born into a family like ours. These are our kids. What else is there to do? We can’t fathom not doing this.”