Rasmussen's is a rare nervous system disorder which causes chronic inflammation of the brain and usually affects only one hemisphere of the brain. Besides the inflammation, other symptoms characterize this rare disease, such as, frequent and severe seizures, loss of motor skills and speech, paralysis on one side of the body and mental deterioration.
Jessie Hall is six years old and she suffers from a rare disease called Rasmussen's Encephalitis which causes her to suffer constant life threatening seizures and unending twitching. To fix this, doctors from John Hopkins will remove half of her brain.
The procedure to help Jessie Hall is known as a hemispherectomy and will be performed at the John Hopkins Children's Center. A hemispherectomy is a procedure where half the brain is removed to relieve frequent severe seizures that resist medicines given to help prevent them.
In a study performed by John Hopkins in 2003, which was reported on by ScienceDaily, shows that 86 percent of the 111 patients that received a hemispherectomy as of conclusion of that study, were either seizure-free or had non-disabling seizures that do not require medication.
It is said that surgery has no apparent effect on personality or memory.
JESSIE HALL'S STORY.
Jessie and her family went out on Lake Texoma, in August of 2007, sailing with a friend and about an hour-and-a-half into the trip where Jessie went from being the chattering little girl her family knew to a little girl falling asleep while eating her sandwich, one that they couldn't awaken. The parents would later find out she was suffering from Complex Partial Seizure.
When she did awaken, her left eye and her head started jerking, she was making grunting noises, her legs started twitching and she was drooling.
The parents called 911, while still on the lake and started their trip back to the marina, where they were met halfway by a game warden who had heard the call and he took them in his speed boat the rest of the way for a faster trip back.
About five minutes out from the marina another speed boat met them with paramedics on board who immediately started working on Jessie, but at that point she was through the worse part of the first seizure.
The ambulance took Jessie to the hospital where the family was told Jessie has low potassium but that blood, urine, chest x-ray, CT, ECG were normal.
Two days later the family met with another doctor at Cook's Children's Hospital and preliminarily it was believed that Jessie had Epilepsy.
The next week was busy with further tests being done, more scheduled and little Jessie started kindergarten.
On September 4, 2007, Jessie had her second seizure while in kindergarten class. The parents say that the teacher and staff were "amazing" in how they handled the situation and got Jessie to the hospital.
By September 18, 2007, Jessie was put on Anti-Convulsant drugs to control any further seizures.
On October 3, 2007, Jessie had her worse seizure yet, which the parents were able to stop using medications they were given but that was only temporary and she started seizing again the same day. Again the parents rushed their little girl to the hospital, still under the impression that it was epilepsy, just a worse form than originally thought.
Jessie was put into the Epilepsy Monitoring Unit where the rooms are all wired with infrared cameras and EEG interface boxes.
Throughout the next couple months, Jessie had seizures or partial seizures and her parents kept their logs of times, dates and all pertinent information for the doctors.
Jessie had developed a 24 hour twitch and had lost some use of her left arm and hand, so the parents decided it was time to get a second opinion from another hospital and another doctor.
On December 10, 2007, the took Jessie to see Dr. Steven Lender at Medical City Dallas, who observed the twitching of Jessie's fingers and hand as well as the loss of dexterity in that hand, asking if the treating doctor had seen that, to which the parents said no because they hadn't had an appointment in a while. He encouraged the parents to get her in to see her original doctor immediately because he suspected Encephalitis, which is inflammation of the brain.
Two days later Jessie was back in the hospital, hooked up to machines and she was diagnosed with Focal Encephalitis in the Right Occipital Lobe and Jessie was put on a 7 week course treatment with Anabolic Steroids.
The doctor had also discussed Rasmussen's Encephalitis with the parents and while that was not diagnosed yet, he told them it couldn't be ruled out.
By this time the parents were in constant fear for their daughter as well as having four other children to care for, but they were hopeful the new medications would work.
Long term effects of the Anabolic Steroids does not allow for them to be used for any extended length of time, but while Jessie was on them, the twitching left, she started regaining the use of her left hand and the family had a wonderful Christmas and New Year. they were optimistic.
As they reduced the doses of the Steroids, the twitching started to return as did the seizures.
By January 2008, Jessie was back in the hospital after another very bad seizure.
By February, Jessie's twitching was back again, she would have periods where she lost sight and would speak gibberish and she was having Simple Partial Seizures about once a day.
This led Jessie's parents to write a letter to the treating doctor:
Dear Dr. Chacon,
In the EMU, on 12/14/2007, Dr. Malik diagnosed Jessie with Focal Encephalitis. She was put on a high-dose steroid cycle, which temporarily ended the continuous twitching in her left hand, returned the function of her left hand, and significantly reduced her Simple Partial Seizures; an indication of reduced inflammation in her brain.
On 02/12/2008, and prior, we reported that the twitching was back and worse, and that she had lost almost all use of her left hand.
1.) Has the diagnosis changed?
2.) If not, why are we experimenting with Anticonvulsants instead of going after the root cause and reducing the inflammation in her brain before she has permanently lost function in her left hand and arm or worse?
3.) Are we waiting for test results? Realistically, how long can we wait before we risk Jessie losing function in her left hand and arm permanently?
As parents, we have an innate feeling that something is dreadfully wrong and that the symptoms (seizures) are being treated, and not the root cause (encephalitis). We can cope with her simple partial seizures (hers are short, and don’t hinder her day), but losing function in her left hand and arm are driving us both toward a nervous breakdown.
Cris and Kristi Hall
More tests, higher doses of Steroids again and Jessie's case was to be presented at a Neurologist Conference on February 20, 2008.
February 20, 2008, a team of neurologists at Cooks Children's Hospital in Fort Worth, Texas, agreed and diagnosed Jessie with Rasmussen's Encephalitis, they believed she was in Stage III.
The team recommended a Right Hemispherectomy (Removal of the entire right half of Jessie's Brain), they also recommended that the Hall's get a second opinion from Johns Hopkins in Baltimore, Maryland, who are the experts in this field.
The next weeks followed and the parents were on pins and needles waiting to hear from John Hopkins and they finally got an appointment date for Monday, March 10th. In earlier calls and communications, the the staff at John Hopkins had suggested the family do a few things in preparation. One of those things was to talk to families that have been through the procedure, which the Hall's did.
Meet Cameron. She is 7 years-old and a real cutie. She is in school and doing well and likes to do things that normal 7-year old girls do, like play dress-up and celebrate birthdays, and hang out with her friends and family. Cameron had a hemispherectomy at Johns Hopkins in June of 2007 (nine months ago). Her story is here http://www.blogger.com/profile/09618184310533248537 .
The public response to the family was more than they expected. Jessie received a letter from the Make-a-wish Foundation, granting her any wish that she wants, A fund was set up to help the family with the costs that would be associated, Midwest Airlines wrote to the family offering to take care of the air travel needs between Dallas Fort Worth and Baltimore for Jessie's surgery, Jessie's school principal declared Friday, April 4th as "Jessie's Day" at school, the media highlighted Jessie's situation, the family had an amazing trip to Give Kids the World, and Cracker Barrel sponsored Jessie's Carnival, details about the carnival here.
Jessie also celebrated her 6th birthday on March 8, 2008.
The mayor of their town, Kit Marshall, declared May 10, 2008, "Jessie's Day" and they send her off to Baltimore in style with everything pink because that is Jessie's favorite color and the Dallas cowboys cheerleaders showered her with attention, and she was the Grand Marshall in a giant pink parade.
Jessie Hall's surgery has been scheduled at John Hopkins Children's Center for June 11, 2008.
The family is now in Baltimore at the Children's House which is special housing for the parents of children with life-threatening illnesses that are being treated at Johns Hopkins.
The parents keep Jessie's blog updated for friends, family and everyone that has shown such caring and interest in Jessie's story, so if you want to leave the family a little note of encouragement, the comment section is open.
More on Rasmussen's Encephalitis can be found here.
More on the Hemispherectomy procedure can be found here.