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Hydranencephaly Resources in caring for a Child with Hydranencephaly Physical Care of a Child with Hydranencephaly Difficult Times
Pt. 1: Taking Care of You Book: Caring for Your Child With Hydranencephaly
Printed Materials |
Epilepsy (Seizures) Seizures are quite common in children with severe neurological damage. However, according to one of Kayda's pediatricians her diagnosis would indicate that she doesn't have enough brain tissue to have seizure activity. Since the age of 8 though she has had very mild Focal seizures (staring spells). For some of the children I know of with Hydran... seizures are a major problem. There are many different types of seizures and treatments for each type. Over the last year as the number of families that we’re in touch with has grown so too has our knowledge about the type of seizures many of the children have. Finally, after years of no explanations and being told that Kayda does not have seizures according to her EEG, it was explained to me that most of the children with Hydranencephaly are having brain stem seizures. EEGs are designed to detect seizures that originate in the cortex. As our children have no or very little cortex, it’s no wonder that their seizures aren’t picked up. Also, pretty well all the medications that are currently available are for cortical seizures. That is why medications often don’t work as well for our children. I have been unable to find much information on brain stem seizures on the internet. In the meantime, as the children’s seizures often fit the patterns of cortical seizures I am including information about them in the hopes of helping families have a bit of a basic understanding of seizures or epilepsy and the treatments available. Many of the children do respond to the typical seizure meds at least to some extent. I have also included a section with information about the Ketogenic diet. This diet has had good results for a couple of our children. Seizures can be broadly categorized into generalized seizures which involve both cerebral hemispheres, and partial seizures, which originate from a specific area of the cerebral cortex (focal origin). Generalized seizures always involve an abrupt loss of consciousness at seizure onset; a simple partial seizure doesn't impair consciousness. However, any partial seizure can evolve into a generalized tonic-clonic seizure. Here’s how to distinguish seizure types:
Seizures and Epilepsy:
Status
Epilepticus
Lennox-Gastaut Syndrome Drug Therapy For
Seizures
Unless otherwise noted the following information is from the Epilepsy Ontario web site. Carbamazepine
Clobazam
Clonazepam
Clorazepate Ethosuximide
Gabapentin
Lamotrigine
Levetiracetam
Nitrazepam
Oxcarbazepine
Phenobarbital
Phenytoin Sodium
Primidone
Topiramate Valproic Acid
Vigabatrin The following three medications are most often used to bring someone out of a prolonged seizure (called status epilepticus). They can also be used as sedation. Diazepam
Lorazepam
Paraldehyde As there was no real information on Paraldehyde on the Epilepsy Ontario site the following information is from: http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202440.html
Ketogenic Diet This option is being used by a number of children with Hydranencephaly Information For Parents Of Children
On Ketogenic Diet
Vagal
Nerve Stimulation The following is from Epilepsy Ontario so is based on the Canadian Medical system. Introduction
to Vagus Nerve Stimulation
The following are links to the information sheets that are part of the Patient Education Pamphlets from the Cincinnati Children's Hospital, which also appear in other sections of the book. I thought that these might be useful if your child will be having one of these tests. Sedation for a Radiological Procedure
Electroencephalogram (EEG)
CT Scan
MRI Brennan, age 3: Brennan began to have absence-type seizures (petit-mal) a couple of months after he turned two. He would just stare off, his lips would turn bluish-purple and he would start to drool, and sometimes one of his eyes would twitch with it. It usually lasted for 15-20 seconds. I would try to snap him out of it by talking to him or caressing his face and kissing his cheeks. he was prescribed Phenobarbital elixir at first and then we switched to tablet form. The elixir was just too nasty and after awhile Brennan wouldn't even open his mouth to take it. I think he maybe had 1 or 2 little breakthrough seizures, about a month or two after starting the med, but has had none since then. Rachel, age 11: Her seizures are not troublesome unless she is getting unwell. Rachel has different types of seizures although I'm unsure of the correct name for them. She can have a small jerk that finishes with a big smile, or staring spells, or a seizure where her face becomes all twisted. She will cry and become pale and sleepy. Noah, age 3 1/2: Noah has experienced a LOT of seizures since birth. At first we didn't know he was actually having seizures but unfortunately we have become experts in what is a seizure. Noah's first type of seizure was a "yawning seizure" where he would yawn and then scream afterwards. This would happen many times a day. Once he was diagnosed with Hydranencephaly at 9 weeks old, he started on Phenobarbital and his seizures stopped and he became a very happy and settled boy. After a couple of months a new type of seizure started: myoclonic jerks. He would suddenly seem like he was startled by something and have a little "jump". This would happen many times a day but didn't seem to be related to anything startling him. Our Dr. confirmed that it was a new type of seizure. Another type of seizure he
would have was similar, but would happen when he was tired or straight after he
would wake up. His whole body would jerk and this would happen up to 30 times in
a row. We did attempt the
Ketogenic diet twice but unfortunately Noah couldn't tolerate the high fat
formula and at the time didn't have a Fundoplication so he just kept on vomiting
and it became very dangerous as he was swallowing this back down. We may try it
again in the future, but there is not a lot of knowledge about it here in
Tasmania. Kayda (12/2/88-6/23/00): When Kayda came to live with me at the age of 4 ½ she was on Phenobarb, & Nitrazepam (Mogadon) to control seizures. But when the pediatrician and several other doctors saw what had been called seizures (we think they were muscle spasms) they said they weren’t. So we worked hard on getting her off her seizure meds. As far as I know she was seizure free until when she was 7 or so. She started having what looked like shivers. They were very short; almost gone before you knew what they were. After a couple months of having these dr put her on Sabril (Vigabatrin). These stopped her seizures but caused her to have excess saliva which caused respiratory problems. She went on to Robinul to control her excess saliva. Shortly before she turned 9 she started having these horrible startling spells. It would take only a tiny noise and her arms would fly up (her sign of distress) and her breathing would get all labored. The only thing that would bring her out of these episodes was for me to pick her up. They would last for an hour or more if I wasn’t there. I remember one day when she’d had one as she got on the bus for school. They called me when she got to school as she was still in it. I went in and picked her up and held her and I remember her picking her head up off my shoulder and going “uh” as if to say “See I told you my mom would take care of it.” I took her to the dr numerous times for these but all the drs we saw said they weren’t seizures. The school kept calling them seizures and it caused a lot of problems when the school nurse contacted Kayda’s GP behind my back telling her I sent Kayda to school with continuous seizures; the same letter stated I was sending Kayda to school sick. If the nurse had spoken with me she’d have known that the pediatrician had said Kayda wasn’t sick, she simply needed an increase in her Robinul. One day while at the dr to check out a startling spell, Kayda suddenly stared off into space for a few seconds and then came back. She did it twice while the Dr & I watched. Over the next few weeks she started having these incidences several times a week. And, that started a nightmare that actually had us ending up with a new wonderful pediatrician. The dr first put Kayda on clobazam but didn’t start it gradually and she suddenly was so sedated she couldn’t even clear her airway. Then the dr put her on Tegretol. That started her vomiting and ended up with her in the hospital for 3 weeks over Christmas and New Years with aspiration pneumonia and vomiting. We tried everything to stop the vomiting. Then one day the pediatrician got out the Drug reference book and looked up Tegretol. One of the most common side effects of Tegretol is vomiting. As soon as we stopped the Tegretol she stopped throwing up and got better. Then she was put on Lamictal. That’s the medication that worked the best for the longest period of time. When she was 10 she started having her period. Her tiny few second staring spell seizures went from 3 or 4 a day to many hundreds each day and were especially bad during her menstrual cycle. When she was younger she’d had a habit of going into comas that would last for about 36 hours. Doctors always said she was shutting down and that she was dying. But then one doctor suggested they could be a non-convulsive seizure. Once her health improved these stopped; but they came back when she started her period. They lasted exactly 16 hours. We had to wait 9 months to see the neurologist so just had to live with all of these seizures. When we finally saw the neurologist she put Kayda on Topomax. A couple of weeks after starting that med, she started having a new kind of seizure. This only happened if she’d been asleep for a few hours (the first one was in the afternoon). But they were more of a full body seizure (not quite tonic clonic) and they lasted until she woke up-it could be hours. Because the seizures lasted so long the neurologist wanted me to make sure I was awake in case she had one so I could give her Ativan to stop the seizure. Aitvan was about as effective as giving her a glass of water to stop the seizure. Finally after a month of staying up until 4 am every night the neurologist decided I didn’t have to stay up and it was ok to have her maybe have a seizure that wasn’t noticed. They’d wanted me to bring Kayda to the children’s hospital if her seizure didn’t stop with Ativan. There was no way I was putting her in the van which would wake her up and stop the seizure and drive an hour and a half into the city with no sleep. I finally convinced the neurologist that the Topomax was causing the new seizures. She didn’t believe me but took her off of it. Kayda had her last nighttime seizure 1 week after stopping the Topomax. Throughout her life she had many EEGs. Not one seizure was ever picked up on it. But her doctors watched several of her seizures and were sure that they were indeed seizures. By the time she came off the Topomax she was having thousands of small 10-20 second seizures every day. I always thought they weren’t that bad until I realized how much time several thousand times 20 seconds actually was. During our nightmare with the Topomax the neurologist commented to me that someone who had that many seizures wasn’t going to live much longer. Unfortunately she was right. We decided to try the Ketogenic diet but by the time we’d taken care of some other issues that needed to be worked out before trying the diet (fluid intake & reflux were the main ones) it was clear that Kayda was dying and I said no to any other treatments. It was too late. I was puzzled that no seizures ever showed up on the EEG yet she was clearly having them. It wasn’t until she started going to Canuck Place during the Topomax nightmare that the Dr there told me she was having brain stem seizures. And, that EEGs are designed to pick up cortical activity. Well, Kayda didn’t have a cortex. The only medications that she could tolerate were Lamictal & Nitrazepam. Everything else caused way more problems than they solved. Other pages in this section: |
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August 16, 2001- January 12, 2005 This website is funded in loving memory of Jason S. by his mother Kammy The information on this site is provided by families, caregivers, and professionals who are or have been caring for a child with Hydranencephaly. Please report any broken links or missing photos to angelbearmom@shaw.ca
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