Child struck by rare brain disease

"Pray for Eddy," reads a sign in front of the school's main entrance.

"We love you, Eddy," could be read 100 times over on a poster inside the school made by East Side students.

Eddy Ramirez is a first grade student at East Side, suffering from an extremely rare brain disorder called Moyamoya disease. (T-G Photo by Sadie Fowler) [Click to enlarge] [Order this photo]

Eddy, a student in Benita Spain's first grade class, has been diagnosed with Moyamoya disease, an extremely rare disorder, which leads to irreversible blockage of arteries that deliver blood to the brain.

Eddy is at Vanderbilt Children's Hospital in Nashville -- and he is fighting for his life.

Today, from 11 a.m. to 2 p.m., there is a benefit car wash being held for Eddy at Tractor Supply Co. on North Main Street.

"Eddy is a very easy child to love," said Spain, explaining that he has worked extremely hard to do well in school. "He always has a ready smile, has wonderful manners, and very much wants to please. The children in Eddy's class talk about him all the time."

Just two weeks ago, after enjoying a normal day at school, Eddy went home, ate a quick snack and began playing with his two siblings. Minutes later, he got fiercely ill, suffering from severe head pain and vomiting.

His parents immediately took him to Heritage Medical Center, where he was flown to Vanderbilt by LifeFlight helicopter. It was determined that Eddy had suffered from a brain aneurysm, and later determined that he had Moyamoya disease, which caused the aneurysm.

About moyamoya

According to a web site recommend for viewing by Spain, www.brain-aneurysm.com, "Moyamoya disease is a condition where there is progressive blockage or occlusion of the ends of the internal carotid arteries and their major 'terminal' branches in the brain."

Moyamoya was discovered in Japan and affects children and, occasionally, adults.

On a cerebral angiogram, the arterial blockage leads to a "puff of smoke" appearance.

"The 'puff of smoke' is actually a myriad of tiny vessels that form in response to the blockage, in order to allow blood to continue flowing to the brain through this alternate tiny vessel network or pathway. The word 'moya' is Japanese, and is taken to mean 'tiny,' referring to the tiny vessel network that forms," the web site says.

A tough two weeks

Early last week, a tube was placed in the top of Eddy's head to drain blood and reduce the brain swelling.

"On Monday, Eddy looked better than I had seen him," Spain said. "His color was good. He did not look as swollen and was a little responsive."

The following day, however, when the tube was replaced with a new tube, complications developed.

"Apparently this caused more swelling and more bleeding," Spain said. "His condition deteriorated from that time."

Spain, who has been to visit Eddy nearly every day, said there is only one cure for Moyamoya, and that is rerouting the blood flow into the brain. Before doctors can even think about that procedure, however, they need to focus on reducing the bleeding and swelling in the brain, and stabilizing Eddy.

More complications

On Thursday, doctors performed surgery in an attempt to stop the bleeding and remove a blood clot in Eddy's brain.

"(The chief surgeon) stopped to tell the Ramirez family that Eddy was a very sick boy and that the surgery was very serious," Spain said.

Six hours later, doctors told the family they had "clipped the aneurysm that was causing the bleeding" and had removed a blood clot the size of a child's fist.

In doing the surgery, doctors had to remove a large piece of cranial bone on the child's head. The bone will be stored in a freezer for an estimated two months, when doctors hope swelling will no longer be an issue. At that time, the bone will be replaced.

Also while doing the surgery, doctors had to severe Eddy's temporal artery in order to get into the brain.

Moyamoya surgery

Spain said that doctors, at the present time, do not think they will be able to perform the Moyamoya surgery, the only cure for the condition, where blood flow is rerouted to the brain through a different artery.

"(The doctor said) that it probably would not be done as he had severed the artery that would have been used and (it) was no longer an option," Spain said. "He told of another surgery where an artery taken from the arm or leg and attached to the head on the outside of the skull might be an option but he doubted that because it was very difficult."

Currently, doctors' first priority is keeping the swelling down. They are also considering putting a shunt into his brain so blood can drain more easily.

"We want to get Eddy out of danger right now and then look for solutions for the Moyamoya," said Spain, who also said she has been in touch with other specialists in California and Boston who are also evaluating Eddy's records.

Strong faith

Over the last two weeks, Spain said, Eddy's family is surviving as a result of their faith in God.

"The Ramirez are a strong Christian family who very much believe in the guidance that prayer gives," Spain said. "They have only asked for prayers and nothing else but are very much grateful for all that everyone has done and is doing."

Mercedes, Eddy's mother, has only left the hospital one time since Eddy was admitted two weeks ago.

"Both Eddy's mother and father are struggling with their little one having to go through all of this but they know that God has His plan," Spain said.

What you can do

East Side Elementary, 421 Elliott St., has set up an account for Eddy. The school asks contributions be brought or mailed to the school. Also, there will be a benefit car wash for Eddy today, from 11 a.m. to 2 p.m. at Tractor Supply Co., 1714 N. Main St.