Autosomal Dominant Acute Necrotizing Encephelopathy
This genetic disease runs only in the Udell family. There are well over 30 cases in this family. The basic thing that happens is that a child will become ill with a common cold or the like, and when the fever spikes up to 103 or higher, the child becomes more lethargic and non-responsive fairly quickly. It affects the brain with swelling. What generally would happen in our cases is that we would take the child to the hospital and the hospital would tell us that the child has the flu, to take them home, they will be fine. This, in our case, is very far from the truth.
This disease can be very deadly. A lot of Udell’s have lost their life or become mentally ill because of this illness. The fever is a primary thing in every case. As far as I understand, all children that have this has had their 1rst bout was before age 4, and their second bout was before age 6. No one that we know of has had it more than twice except our son Andy. Please take time to read through this. It’s worth your time.
Let me tell you about my twin boys Andrew & Corey
and there 5 bouts with this illness.
Let me start by saying the boys were growing and learning like ever other child, they were almost 4 years old when they were hit with their first bout with this evil illness. They were completely normal in society’s eyes. (Typical 3 year olds) They knew their letters, how to write their name, etc. We have videos of them playing together, and running around and laughing. I would love to add some video clips to the website if I can figure out how to.
Bout 1 & 2: Both boys hospitalized
This all changed in March of 2000. Both boys were hospitalized due to unresponsive behavior, and they could not sit up on there own anymore. Andy had a 9 minute seizure. After 5 days of coma type status they started to respond to us a tiny bit everyday. They took a muscle biopsy from Corey’s leg to try to narrow down the cause. The boys now have to learn things all over again such as sitting, walking, eating. Others have told us it is much like someone that has had a stroke. After 30 days, Andy begins to speak again. His speak is very drawn out, and he had remembered a familiar book I was reciting to him, and he would finish the sentence. I realized at that moment, he remembers.
There was point that we weren’t even sure the boys knew who we were at all. Then Andy says “M…o…m”, and I began to cry with joy, I said, “Oh Andy, I love you!” He comes out with “L…u..f…. You”. I hugged him so tight with excitement. That was the beginning to a very fast recovery for both of them. They remembered everything; they just couldn’t verbalize it as well as they could. We left the hospital after 30 days, we walked the boys out by holding their hand. Within 6 months literally, they were back to their old selves again. Those that didn’t know us would have never guessed they went through this the first place. All was well for a year and a half.
Bout # 3 – Andy gets knocked down again
October 2001: Andy then had his second episode. Same things happen, non-responsive and lethargic, here we go again. We again spend 30 days in the hospital, although this time A.D.A.N.E. has taken a bigger toll on him. We leave the hospital carrying him out with a feeding tube in. At this point, he is unable to walk, eat or sit up on his own at all. At this time, all he could do is lie down, and look around. Not many facial expressions until he saw our ceiling in our house and he began smiling for the first time in 30 days. We worked with him extensively to get him to sit up, and speak. He had to be in a wheelchair to sit up, and still no speech. Now is around Christmas, and he is sitting, standing and trying to walk on his own. Very unstable, and he had plenty of bumps and bruises trying. He made us a nervous wreck.
The doctors told us if he does not start to eat on his own, we will have to put in a permanent feeding tube in his stomach. We realized we had to do something soon. We went back to the basics. We fed our son with a baby bottle, until he learned how to drink/swallow again. We took him off of the feeding tube except once a day to transition him. He did learn, and we graduated to the “sippy” cup.
He gradually learned to walk again and in April of 2002, and begins to speak again. Whew… you never know how much you miss your kids talking until they are unable to speak at all. He continues to have speech delays and a lot of people still misunderstand what he is saying. But He can talk, walk, run, and jump. Delays in the physical, but doing great!
Bout #4 –Corey gets knocked down again too
March 2003: While in the ER for being lethargic and a little responsive, he has little twitches like dozed off and them jumps a little like he is frightened. The life flight folks are there now, and they ask if Corey has ever had seizure. I said “no” and it wasn’t even minutes later and he was in a full blown seizure with his whole body jerking. He was sent to Cleveland Ohio, where after three days of being in a coma, he is given a 12 hour dose of sodium in a 6 hour period in the middle of the night, and it herniated his brain.
He died on March 14th. Yes we have forgiven, and no, we did not press charges. We left it all in God’s hands. He can take care of it better than we ever could. He loves us so much.
Bout #5 – Andy’s most recent episode
March 2005: This is the same day we buried his twin brother just two years prior to this day. What’s up with March anyway?! He is acting a little more tired than usual on this day, and has had some kind of cold, and we are trying to keep his fevers under control but he was not eating well at all. We went to bed, and 3am we are awakened by a gasp from Andy, his feet and arms sticking straight up in the air and he is in a seizure.
His eyes are rolled back in his head and his mouth is bleeding, because his bit his tongue. At the ER the seizure lasts for approximately one to two hours, although it seemed like forever. After being sent to Cleveland again, Andy is responding very well. They took him off of the breathing machine they had on him for the flight. Only 5 days later, they want to send us home because Andy is doing great. He did have to learn to balance again while walking.
He became fearless once again and thought he could do all the things he could before he was in the hospital. He jumped out of the bed, and almost fell on his face. Thank God for quick thinking on my part. He was off and running down the hall after his dad. He got better very quickly. He is back to baseline where he was after his second bout. He lives today with delays in speech, and rarely uses his left hand unless he is forced to. His arms are shaky, and he can eat on his own, he has a hard time with fine motor skills, and gets better everyday thanks to God! He is unable to write his name clearly, but he tries real hard. 2006: He is now 10 years old and in second grade again for his second year.
He knows some words, but is unable to read a book yet. He is an audible learner, and can memorize scripture of the Bible. He loves to play with smaller children, and cognitively, he is probably in the range of age 5-7. He loves to laugh, sing, and dance. He loves others unconditionally, and he always tells us that he loves everyone in the world. He misses his twin brother so much, and talks about him almost every single day.
2008 Update:
We thank God for the wonderful miracles our children are. Due to the biopsy taken from Corey’s leg, the doctors working on this case have narrowed it down, and they can now test others to see if they carry the gene that causes this, so they will be able to know ahead of time, to decide if they want to still have children. I know if I would have known ahead of time about this illness… I would have chosen to have children.
I would not change it for the world.
God Bless you all. Sincerely, Melissa Udell
