I have a little friend named Jeremy. I have known him since he was 4 months old when he and his family moved in next door. His Daddy Paul is in the military and has served 4 tours in both Iraq and Afghanistan totaling more than 4 years away from home and family. I have already written about his mom and my good friend Amanda, here.
Jeremy
Jeremy has a very rare condition called Sturge Weber Syndrome (SWS). For a brief explanation go Here. Among many other things it causes seizures. These seizures are not like normal seizures people have, each one he has can result in Jeremy having a stroke. He has taken seizure meds all of his life.
When he was very little and Paul was deployed in Iraq, Amanda came running over. Jeremy had been seizing for more than 5 minutes, a dangerous situation where medication is given and if ineffective, emergency medical care must be called. She called 911 and he was transported and admitted to the army hospital. The next day that child had more than 60 seizures as we stood by his crib. It was so hard to watch. He was so little and his Daddy was so far away. Amanda knew what he needed and the staff was not listening. After a little talk and after they actually googled Sturge Weber (because they'd never heard of it) they were on board and began to listen to Mom. Amanda was now a veteran medical mom.
Jeremy has endured many, many painful laser surgeries to try and lessen the vascular complications of the port wine stain on his face, head and ear. It extends into his brain which is why there are cognitive and motor difficulties as well as seizures. Each time he is put under a general anesthesia there is the danger of more seizures and stroke.
Jeremy after a laser treatment. Pretty painful stuff but he's a trooper!
After Amanda's boys, four in all, grew a bit older she began a quest to educate, raise awareness, money and to help train a service dog for her son. It has been quite the journey to watch, in fact it's called Jeremy's Journey and you can follow it here.
It has gone world wide and my friend would tell you it's no big deal but it is. She helps others and they help her. She goes to conferences and she helps others get what they need.
Jeremy needs his service dog Balto for several reasons. He is going blind in one eye due to glaucoma. This is one of the many complications due to Sturge Weber. This makes navigating stairs, curbs, anything requiring depth perception impossible for him. He also has balance issues due to neurological problems. Balto will be trained for seizure alert. Seizures are the reason for this post.
Most people have never heard of SUDEP. I know I never had until Amanda told me about it. It is Sudden Unexpected Death in Epilepsy. It is grossly under reported by coroners but there are two states New Jersey and Illinois I believe, that just past bills requiring epilepsy to be considered during an autopsy and if SUDEP is considered it must be registered in a national registry.
Due to a lack of large-scale, population-based studies and since SUDEP often goes unrecognized by physicians and medical examiners, the incidence of SUDEP is probably underestimated at 1 per 1000 people. This may be because of inconsistencies in the investigations and recordings of these deaths. In people with frequent epileptic convulsions that are poorly controlled with medications, the rate may approach 1 in 100 per year.
The following is from the Chelsea Hutchinson Foundation:
"The cause of SUDEP is unknown. It usually occurs at night or during sleep, and this makes it hard to find out exactly what happened during the last moments of life. There is often, but not always, evidence of a seizure before death. A seizure at the time of death is not needed for diagnosis of SUDEP.
It is unlikely that a single cause will explain all SUDEP deaths. Advances in research are identifying critical risk factors and a number of potential mechanisms for SUDEP. Researchers are investigating problems with breathing, heart rhythm and brain function as possible causes of SUDEP. During seizures, there is often a change in breathing and heart rhythm. In most people with epilepsy, this is not dangerous. Sometimes, a more serious drop in blood oxygen levels or changes in heart rhythm may occur. Seizures may also affect the brain’s control of breathing and heart function. In addition, researchers are exploring genetic links between seizures and heart rhythm abnormalities. Further research is urgently needed to reveal these possible causes of death and to help people with epilepsy understand how to lower their risk of SUDEP."
Jeremy's service dog Balto will be able to alert Jeremy's parents or caregivers in the event Jeremy has a seizure. There are also machines that can detect movement and alert in the middle of the night with an alarm for those with movement seizures.
Lest you feel sorry for Jeremy.....don't. He is smart as a whip in some areas but deficient in others as is common in those with SWS. He smiles at everyone, loves trains, school and his Ommie and Pawpaw. He loves to ask Mr. Ed about the Post Office and his mail truck and we love to have him over to play trains. Our boys have always liked to have him around even though they don't talk much. They build elaborate train tracks and cities together.
When they still lived next door we were describing Jeremy to someone once and said something about his birthmark. One of our little boys said, "What birthmark?". I thought that was funny, they don't even see it because they have known Jeremy since they were all babies. It is not, however, that easy for him in public. He is stared at and pointed at and it hurts. He has a younger brother who is ready to punch anyone who gets near him! He's well protected....always has been. But it isn't easy. Amanda tells me adults are the worst. They don't mind people asking questions or if you want to look then look, but smile then look away. It's just a colorful face and a sweet one at that.
~Blessings~
Lisa
Beautiful! Thank you for telling Jeremy's story. I had the pleasure of meeting both Jeremy and Amanda last month when they were at a SWS Conference!~
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