Every night, Milledge Estes is hooked up to an infusion line through his leg for 12 hours to receive medicine and make sure his iron levels stay normal. He has also gone through more than 100 blood transfusions since he was born.
He’s been through all this and he’s only 6 years old.
Estes has a rare, life threatening bone marrow failure disorder called Diamond Blackfan Anemia (DBA), which causes his body to not make red blood cells, giving him low hemoglobin.
“He was symptomatic at birth in the form of really low hemoglobin,” said Melanie Sisemore, his mother. “He had a blood transfusion while I was still in recovery from my C-section. When he was 6 weeks old, because his hemoglobin didn’t bounce back, he was formally diagnosed.”
There are fewer than 700 known cases of DBA in North America and there is no cure.
“When you’re diagnosed, you’re going to first be put on a blood transfusion protocol,” Sisemore said. “You’ll go in every two to four weeks, depending on how long you can go without a blood transfusion.”
Due to the small numbers afflicted with the disease, which is casused by a genetic mutation and is sometimes hereditary, the closest families with someone who has DBA live in Canada, Ohio and Colorado.
There are some treatment options, including corticosteroids. It involves taking a prescription steroid of Prednisone or Prednisolone to force the patient’s bone marrow to create red blood cells.
The goal is to ensure those with DBA maintain a hemoglobin level of 9 gm/dl or higher.
“That didn’t work for (my son), though,” Sisemore said. “We tried steroids a few times and each time it didn’t work. He’s what we call ‘transfusion dependent.’ He has to be kept alive with transfusions.”
With all the transfusions he has to undergo, a big side effect is a build-up of iron and, because Estes’ body doesn’t know how to use the iron, it can eventually lead to organ damage and organ failure.
“He gets hooked up to a pump every night that delivers an infusion of medicine over 12 hours that removes the iron and keeps him from going into organ failure,” Sisemore said. “A little needle sticks into a little fat in his leg and then while he is sleeping, hopefully, he gets the medicine.”
All-in-all, Estes is doing really well, given the hand he was dealt with since birth.
“He probably has a more severe case than most because he has such a high need for blood,” Sisemore said. “He’s lived this life since he was born. He has a real positive spirit about it and is always eager to share with people what he goes through.”
In order to raise awareness and funding for research, Sisemore decided to hold Mighty Milledge, a charity work out and bar-b-que at Crossfit Kennett Square.
“I’ve been going to (there) since it pretty much opened two years ago,” Sisemore said. “I was looking for a way to keep in shape and relieve stress. It’s a great community of people. We all work out together. If people don’t show up, people call you to find out where you’ve been.”
The close bond among the members has led them all to care about how Milledge is doing and they are always asking for updates on him.
They were even asking about ways they could help, which is what brought about the idea of the Mighty Milledge fundraiser.
“The owners of Crossfit Kennett Square opened their arms and offered their facility,” Sisemore said. “It was just a really casual day of burpees and a bar-b-que.”
The event took place June 28, from 11:30 a.m. to 5:30 p.m., and for just $30, anyone could complete a fun, optional workout, enjoy some food and be entered in a raffle.
People were constantly coming and going throughout the day and by the end of it all, they raised $5,000 for the Diamond Blackfan Anemia Foundation for research.
“It was a really great time,” Sisemore said. “We all came together, did a really tough workout, but had lots of fun. Milledge cheered everyone on.”
Sisemore never expected to see that amount of support from the community.
“One of the advantages of living in southern Chester County is that you know everyone and they also know you and they want to support you,” she said. “I was just struck by the spirit of the community that did this – to rally behind something that will, realistically and statistically, never affect their children.”