Albany family raising awareness on Chiari

John Cooper, 5, stands behind a wheelchair occupied by his brother, Thomas Cooper, 6. Thomas suffers from Arnold Chiari Malformation, a condition that results in overcrowding of the brain. (Jennifer Parks)

John Cooper, 5, stands behind a wheelchair occupied by his brother, Thomas Cooper, 6. Thomas suffers from Arnold Chiari Malformation, a condition that results in overcrowding of the brain. (Jennifer Parks)

ALBANY — While still a relatively unknown condition, Arnold Chiari Malformation is a condition that — thanks to increased use of diagnostic imaging — is being seen more often.

After learning that the hard way, the family of a 6-year-old boy in Albany is working to ramp up awareness efforts on the condition to make life easier for the next child who is diagnosed.

The National Institute of Neurological Disorders and Stroke describes Chiari malformations as structural defects in the cerebellum. They often develop when the bony space is smaller than normal, causing the cerebellum and brain stem to be pushed downward into the foramen magnum and into the upper spinal canal — which could result in pressure that affects functions controlled by those areas as well as a block in the flow of cerebrospinal fluid.

For roughly two years, Thomas Cooper has suffered from chronic recurring severe headaches. He had speech and coordination problems, and failed to achieve the developmental milestones his peers were reaching. He also had a tendency to be “very irritable,” and was sensitive to light and sound.

“He still wasn’t talking at age 3,” his grandmother, Phoebe Cooper-Wright, said. “… There were signs when he was younger that were not recognized. They thought because he was a preemie that he was slow. He was born a couple of months early, but we felt it couldn’t just be because of that.”

Now under the guardianship of his grandmother and great-aunt, Thomas was sent from physician to physician for months before receiving the magnetic resonance imaging (MRI) scan that ultimately confirmed the diagnosis in March 2012. He was eventually sent to Emory University Hospital where he received his first surgery on June 26, 2012, at which time part of his skull was removed as well as part of the first two vertebrae in his neck.

The results of that surgery worked out fine for him until the following October when he started having symptoms again. He went back to a pediatric neurologist, and following a trip to the Chiari Institute in Great Neck, N.Y., his family was told he would need three more surgeries. He was later taken to Augusta, where he had another surgery in June. During that procedure, they opened up the same incision site as before and opened the covering of the brain, cauterized the tonsils of the brain, released tissue holding the brain in place and covered it back up with an artificial mesh graft and placed a titanium plate over the exposed spot.

The testing alone prior to the surgeries was tiring for the family.

“He had to be put to sleep,” his grandmother said. “You won’t get a child his age to sit still for two hours to do an MRI.”

Thomas’ body soon began rejecting the foreign materials, causing infections in his cerebrospinal fluid as well as his blood. On Aug. 21, after the infection levels had gone down, he underwent surgery again in Augusta — during which time he was given a shunt to relieve the pressure on his brain and spine, and tissue was retrieved to replace the mesh graft and plate.

In addition to Chiari, he also has attention deficit hyperactivity disorder (ADHD) and traces of autism. In all, he is on more than a dozen prescription medications, and from the time frame from June 6-Aug. 18 of this year, he received a total of seven lumbar punctures, said his great-aunt, Kim Dean.

“Before the surgeries (the Chiari) affected his arms and legs,” Dean said. “He tends to fall, so they are talking about putting braces on his legs. He gags sometimes, and he has lost control of his bladder and bowel. It is a horrible, horrible disease. With the surgeries and all, his immune system (has been significantly weakened).”

As a result of his Chiari, Thomas’ family says he is restricted from doing a number of activities such as attending movies and riding bikes. Due to his illness, he has yet to finish pre-kindergarten. He was to attend a cyber school this year, but his doctors have recommended against pushing him.

“Because of being put to sleep so much, his memory has been affected,” Cooper-Wright said. “He no longer remembers the ABC song. By the way he reacts (when he tries to sing it) he knows he doesn’t remember it anymore. He knows his (younger) brother can do things he can’t do, and it bothers him.”

Aside from the knowledge that Thomas will have to endure a lifetime of pain and surgeries, it is not clear yet what his overall prognosis will be. The family is hopeful that it will be awhile before he has to go under the knife again.

“We are hoping the shunt will last until he is in his late teens before it has to be replaced,” Cooper-Wright said.

After spending months advocating to make sure Thomas got the care he needed, his family is now working to ramp up educational efforts on the disease. Thanks to the sponsorships of Conquer Chiari and Procter & Gamble, there is a walk set for Sept. 21 at Riverfront Park on North Front Street in downtown Albany.

Cooper-Wright said that there will be various walking distances available, and participants will be able to walk at their own fitness level. Registration will begin at 8 a.m., with the walk to follow at 9 a.m. Participation is free, but at-will donations will be accepted for Conquer Chiari, she said.

“Our main goal is not so much raising money, but raising awareness.” she said. ” … I don’t care if it’s three cents or $300; it all adds up. The research is needed.”

Pre-registration is still open at www.conquerchiari.org. Goody bags will be available on a first come, first serve basis. There is expected to be family-friendly music, as well as individuals coming in to share stories on their experiences with Chiari.

Cooper-Wright said she is still looking for individuals and businesses to help provide materials for the event, such as discount coupons, gift certificates or water bottles. For more information or to help, Cooper-Wright can be contacted by calling (229) 869-6825, or by emailing phoebecooperwright@gmail.com.