Hemophilia is a blood disorder that keeps blood from clotting properly. It’s inherited, and there’s no cure — but it is treatable by regularly injecting a protein that helps the blood clot.
A program at the Phoenix Children’s Hospital called "Infusion Boot Camp" is teaching kids with hemophilia how to do these injections all on their own. Wednesday’s meeting was the last one for this round of the boot camp.
“Before we start are you able to tell me all the supplies that you have to get?” a nurse asked one child.
The four boys who participated are ages 8 to 13, and they were being tested on what they’ve learned over the past month.
“Syringe, needle, gauze ...” one boy listed.
They laid out the supplies for an infusion, including the most important item.
“And what’s the name of your factor? Do you guys remember that?” a nurse asked the group.
“Factor eight!” one boy responded.
That’s the clotting protein. Today, the boys are going to inject it themselves, with the nurses’ help.
It takes bravery to put the needle in, but it’s important to learn. They have to do this multiple times a week to keep their factor levels up.
“You’re doing awesome, just go for it,” a nurse encouraged, as Clayton Cooper took a deep breath.
It takes a few tries on both arms for him to get the right spot.
“Well, my veins are really small, so I can’t really get them all the time,” Clayton Cooper said.
“Watching 8-year-olds stick themselves with needles, that’s impressive. And it’s scary,” said Becky Garcia, the nurse coordinator for the Phoenix Children’s Hemophilia Center.
It’s one of two centers in the state. Working with these patients, Garcia found the most helpful thing she could do was teach rapid treatment — meaning if something happens, infuse first, ask questions later.
“For a family to be able to — and a child to be able to — say, ‘Hey I know I’m starting to get a bleed in my knee, I’m going to treat now,’ it causes a dramatic difference in the quality and the outcome of that bleed,” Garcia said.
Because getting that protein into the blood means it will clot and not cause bigger issues. Some kids learn how to do it themselves at special summer camps. However, Garcia said they often stop doing it shortly after.
“And then come camp again they’re kind of starting from square one,” Garcia said.
So she decided to start a more involved program that brings the parents in, too. According to the Centers for Disease Control and Prevention, there are 146 hemophilia centers around the country, but none have a program like this, Garcia said. She knows because she looked.
“I didn’t want to reinvent the wheel,” she said. “But I wasn’t able to find any kind of a similar program where it was on a consistent basis over a long period of time.”
One explanation for why this didn’t exist before might be that hemophilia is a rare disease. An estimated 20,000 people in the United States have it. That’s good for the population as a whole, but for those who are affected, that means they get less attention on the research front.
“We knew nothing,” said Christine Cooper, Clayton’s mom. “This was a spontaneous mutation. We don’t have any family history of this.”
So when the Coopers learned their 3-day-old son had hemophilia, they thought they were looking at a boy who wouldn’t be physically active, “that would never be able to run at school, that will probably always be on crutches,” Cooper said.
Not the case, thanks to this treatment, which Garcia said is fairly new. It’s still very expensive, starting at $6,000 for one week’s supply of the protein. But in the past few decades, it’s allowed people with hemophilia to lead normal lives, which is what the Coopers want for their son.
“He’s going to be playing club soccer; he’s going to be on the top team; he’s going to be slide tackling. He’s going to be kicking some serious butt,” Christine Cooper said.
At the end of the meeting it was time for ice cream and awards. Garcia handed out cloth patches for each skill.
“This is that you know your factor product, your dose and your frequency,” she said, holding up one patch.
The boys will use these skills for the rest of their lives.
They have a disorder that not a lot of people understand, but is always present. So the best advocate for each of these boys is themselves.
