Scott City toddler receives second bone-marrow transplant in a year to counter rare syndrome

The Blankenship family knows deja vu.

In 2002, their daughter, Kristan, was diagnosed with hemophagocytic lymphohistiocytosis, a rare syndrome that causes the bone marrow to produce an excess of cells, which causes inflammation and eventually damages organs. In 2007, their son, Ethan Lee, was diagnosed with the same disease.

Kristan, now 7, had a successful bone marrow transplant in 2002. Ethan's transplant in December proved unsuccessful. Doctors tried other procedures but eventually decided Ethan, who was 7 months old when he was diagnosed, would have to have another transplant.

Now 18 months old, Ethan received his second bone marrow transplant Nov. 17 at St. Louis Children's Hospital.

Hemophagocytic lymphohistiocytosis affects one in 1.2 million people and can be fatal without a bone-marrow transplant.

KIT DOYLE ~ kdoyle@semissourian.comLee Ann and Dennis Blankenship must constantly keep watch of their son Ethan, whose immune system is weak, making sure he stays on his bed or is held Nov. 21 at St. Louis Children's Hospital The family was photographed through a window from an adjoining room, due to possible germs. Ethan must be kept off the floor for safety, which is difficult with a lively 18-month-old who will also pull out his monitoring tubes if left unattended.

Lee Ann Blankenship, Ethan's mother, said, "His physical therapist says he's a little miracle." Unlike Kristan, who didn't walk until she was 21 months old, Ethan took his first steps shortly after his first birthday, at 14 months.

"The steroids they have to be on make their bones brittle, so you have to watch," Lee Ann said. "Kristan broke her leg when she was learning to walk."

Bleeding is also concern because Ethan's blood platelet count is 20, when a normal count is 150 to 450.

Ethan and his parents left their home in Scott City for St. Louis Children's Hospital on Nov. 11 for the transplant.

The family of seven was too large to stay at the Ronald McDonald House, and the cost to stay in a hotel was not feasible. At midday Ethan and his parents said goodbye to Morgan, 12, Logan, 10, Kristan, 7 and Addison, 4, who stayed with their grandmother in Scott City.

"It was one of the worst days ever," said Lee Ann when she thought of leaving behind the remainder of the family and knowing that seeing them would be limited because of the concern for infection and the cost of traveling. "Last time the other children were just two miles away in a hotel that the hospital helped to pay for."

Lee Ann talks to the children once or twice a day. "The littler ones, Addison and Kristan, don't always talk on the phone because it hurts too much. They miss their mommy and daddy. We've seen our kids only once in two weeks, and we won't get to see them again till we get out -- possibly the first week in December -- if there's no fever. Anything can delay that, even the sniffles."

Kristan, diagnosed six years ago, understands what's going on.

"She realizes there's someone else in the family who has HLH. She knows she's a survivor and expects him to be one too," Lee Ann said.

When visitors come, they are separated by a glass barrier to avoid infection because of Ethan's weakened immune system.

The first 100 days are critical, but it takes one to two years to have a normal working immune system, Lee Ann said.

"One of the biggest complications that can happen is infection from having no immune system," Lee Ann said. "The other risk is graft-versus-host disease, a common side effect."

In that scenario, the immune cells from the donated bone marrow attack the body of the transplant patient. Graft-versus-host can range from mild to life-threatening.

Ethan's donor is a 26-year-old woman from outside the U.S. The donated bone marrow was divided into three bags, two of which were frozen for future use. Ethan was given fresh bone marrow in his first transplant in December 2007. The second transplant was from the donor's frozen bone marrow.

"There's really no difference between fresh or frozen bone marrow," said Amy Chadkhtzian, bone marrow transplant coordinator at St. Louis Children's Hospital. "The stem cells are frozen in a preservative so we give premedication to counteract any reaction to the preservative or stem cells."

Ethan's medical condition has not caused the family to lose hope. To raise a little money the family began making beaded bracelets to offset medical costs. They're called Hope bracelets because it's the name the family gave to Ethan's donor since her identity is confidential.

Ethan has visitors to Caring Bridge (a free, personalized website for anyone facing critical illness), and the congregation of St. Joseph's Catholic Church in Scott City and friends and family are praying for him.

"He's looking pretty good so far," Lee Ann said in a telephone interview more than a week after the transplant.

Ethan's sister, Kristan, had a bone-marrow transplant six years ago at a time when the syndrome was not known to be genetic. Her brother, Logan, then 4, donated the bone marrow to his sister.

"It's scarier this time around because we know what could happen. We've had so many years of this we're just tired," said Lee Ann, who feels blessed that her other children are unaffected by HLH. "We have so much hope with a survivor in our house."

There is no test for HLH. Although there are handful of genetic markers that could be evaluated to determine the likelihood of the Blankenship siblings contracting the disease, Lee Ann said they've chosen not to do it. She believes that since her two children with HLH developed it when they were babies, most likely the remaining three will not.

cpagano@semissourian.com

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WANT TO HELP?

Donations may be sent to Ethan's Hope, P.O. Box 4221, Scott City, Mo. 63780. Order Hope bracelets at dlblank@showme.net or get updates on Ethan's progress at www.caringbridge.org/visit/ethanlee.