Subject: Re: Seizure control

Hi Jeanne:

Can you or anyone else help? Rene (21 months) had his first bout of seisures 2 days ago. His neuro had him on telemetry at the hospital for 24 hours and now wants to put him on TEGRETOL. What is your experience with this drug??? if any??? We have not been able to locate a good nuero with AS experiece. The one we have now has only seen 2 other AS children and only for a short period of time. Any information is greatly appreciated.

Thanks Rene & Betty Guim Miami, FL

Subject: Re: Seizure control

Rene and Betty,

We had the same experience with Tegretol as Kevin related...Emily was put on it after her first seizure and less than a year later, the seizures returned and were totally out of control until we put her on Klonopin -- the doctor first tried depakane but with no success; however, I understand that a lot of kids are successful with it. Our neurologist chose Klonopin because Emily responded so well to Adavan or Valium to stop episodes of multiple seizures...Klonopin is in the same drug family. Sorry to hear about the seizures...in most cases, they become infrequent as the children get older.

Marianne Mosley (mom to Emily, 6, +)

Subject: Re: Seizure control

Hi Rene & Betty,

Sorry to hear of Rene's seizures, although it's to be expected with AS. I know it is frightening to watch, but after seeing as many of them as we AS parents have seen, you become an expert on your child and know just what to expect and how long they will last. We could usually tell when she was going to have a seizure -- not the exact time, but usually sometime that day.

Tegratol is the drug that our daughter, Olivia, was on shortly after her onset of seizures at one year. It seemed to work fine for about 12 months, with occasional breakthrough days. Then all of a sudden seizures returned with a vengence. When our nuero added Dilantin to the Tegretol, she went into status and was almost comatose. I then read a note in the AS newsletter that Tegratol may actual increase the frequency of some types of seizures in AS kids! I got online with this mailing list, and everyone advised us to try depakote. Our neurologist was happy to act on the information, and she has been seizure free ever since (9 months ago!)

Depakote has one serious side effect in children under two. In a rare number of cases the depakote attacks the liver and does rapid and serious damage. Olivia takes a drug called "carnitor" to buffer against this reaction.

Kevin Ditamore

Subject: re: Re: Seizure control

Rene & Betty,

So sorry to hear about the seizure trouble. Our son Ben (9) did not have a good experience with Tegretol. In fact, it helped to increase his seizures. I have heard other Angels have had this same experience. Dr. Terry Hutchinson, a neurologist who sits on the ASF board, also advised against Tegretol.

The drugs of choice for AS children, according to Dr. Joseph Wagstaff (Children's Hospital, pediatrician and geneticist involved with AS) and Dr. Hutchinson are: Depekene or Depokote sprinkles (Valproic Acid) and Klonopin (Clonazapaem). Other children are taking a few other drugs, but I'm not familiar with them.

Hope this helps you...

Best wishes,

Jeanne

Subject: Re: Seizure control

Liselotte's seizures started at 3 months, we didn't know she had a.s. at that time. She was prescribed tegretol as well, but immediately her seizures got worse, and when I say immediately I mean the same hour she took her first medication. We then switched to depakene which worked fine, but after a few months we had to switch to phenobarbital because her seizures were coming back and the pediatrician was afraid to raise the dose of depakene. What I understand from the postings to this list and from the ASF newsletter is that tegretol is the worst anti-epileptic drug to give to a.s. kids, try to convince your neurologist that tegretol is not the drug to try, if he is obstinate I would find another neurologist right away.

Frank van Hof, father of Liselotte, 9 months, del+.

Subject: Re: Seizure control

We want to thank each and everyone that posted information regarding Tegretol. We got the info just in time before Rene was released from the hospital this afternoon. Needless to say we are desperatly looking for a new neuro that has more experience with AS. We gave copies of your e-mail's to his pediatrician and he instantly changed the prescription to Depekene. He is doing a liver function test tomorrow to monitor any potential side effects. Thanks again to all that responded so fast. We are very greatfull for your concern and support.

Regards,

Rene & Betty Guim (Rene 21 months)

Subject: Re: Seizure control

Hi Rene and Betty,

I'm wondering how your son is doing after several days on the medication. I'm sure there are others on the list who are also concerned. The initial onset of seizures is about the most sressful, anxious time of being an AS parent. Hang in there!

Kevin Ditamore

Subject: Re: Seizure control

Hi Kevin: Thanks for asking. Little Rene is doing well on the medication. Thank God he has not had any more seizures. It took until today for him to get back to his old self. He was hardly smiling and had a very sad look all the time. Today he started smiling again. His sleeping has become a problem. Can't get him to sleep until 4 am. He used to fall asleep around 11:00 PM until 7:30 am each night. We are working on this new problem with sleep. Thanks again for checking on us.

Regards, Rene & Betty Miami,Fl

Subject: seizures

Hi!!

Our daughter Melanie (3 del +) is on Phenobarbitol 15 mg a.m 30 mg p.m and 250 mg of depakote sprinkes am & pm. Since she has been on depakote her seizures and starring episodes have increased. We went to the neoro on 7/19/96 and they increased the depakote to the above level and dropped the pheno level. The seizures have again increased and she is not sleeping!!!! They had us give the pheno about three hours before the depakote and this helped her sleep through the night but the seizures are not controlled. Any suggestions??

Thanks for your help!

Lisa Koch

Subject: Re: seizures

From talking to our neurologist, taking multiple seizure medications can really mess each other up. It's best to find one that works if possible. If you started out with Pheno and added depekote, you probably don't have a good indication of how effectively depekote works on you rdaughter.

Very few AS kids are on pheno from what I understand.

Kevin Ditamore

Subject: Re: seizures

Lisa, My daughter Taylor (4 1/2, del+) also takes both phenobarb and depakene. We tried to wean her from the pheno to the depakene last October when we decided that pheno might be causing further delays. She did fine for about a week then started having so many seizures that she had to be hospitalized. She was put back on the pheno (20mg/bid) and continued to take the depakene (300mg/tid). She hasn't has a seizure since December. We hate all of the medication but prefer it over the seizures.

Good luck with Melanie, Robbin Clark

Subject: Depakene

Lisa and Rick,

Our daughter, Shannon (3.5, del-) didn't have a very positive experience with Depakene either. She did alright for a few months and then ended up in the hospital with seizures three times in three months. Shannon had weekly blood tests for a while and the results were never the same even though the amount of Depakene she took didn't change. Her system obviously didn't take to this drug very well.

Shannon was switched to Frisium (Clobazam) in January and hadn't had any tonic clonic seizures or myclonic jerks since. She started having drop seizures in May and began taking another drug, Lamictal (Lamotrigine), to help with those. We are still in the process of introducing the drug to her (she had the chicken pox and we had to hold off for a while) so we haven't noticed a big change yet, the drop seizures seem to come and go. These medications were prescribed by a neurologist at Children's Hospital, in Vancouver, B.C., who has had some experience with AS children.

Let us know what your neuro's plans are for you daughter. Good Luck!

Kathy

Subject: Re: behavior problems (and Hospitals)

I have several suggestions I feel qualified to make for the following reasons: I'm a long time nurse, and grandmother to Katie (age 7, del+) and her primary caregiver. First: Seek out someone who will teach you about the various types of seizures, so that you can recognize them and be able to describe them accurately. Second: Have that person teach you the appropriate responses to take when seizures occur, which is often nothing. But do learn CPR just in case. Third: Find a pediatric neurologist with whom you can develop a good rapport and who is accessible by phone or beeper at all times. Fourth: Unless your current docs can tell you unqualifiedly that your son is in a life-threatening condition, get him home!!!!!!!! It has been my long experience that children and the elderly have a bad reaction to being placed in an unfamiliar environment. These reactions are exhibited mostly by cognitive and behavioural changes, but these changes can also affect their physical functioning, too.

We went through several bad months with our child and seizures last year, and I truly believe that she could have been hospitalized most of that time, but decided with our neurologist to handle the situation at home unless it became truly life-threatening, which it did not. I feel that this decision averted many bad disruptions of both Katie's life and ours at a time when we all felt very threatened. Deanna

Suggested resource: the Epilepsy Foundation

Subject: Re: Hospitals-Deanna's disclaimer

I need to make some things VERY clear: I am not advocating trying to treat one's child oneself! I am not advocating that one ignore their doctor's orders or instructions! I am not advocating that anyone ignore any seizure activity that one of the children might be having. In fact, I recommend keeping a log of seizure activity. What I do advocate is that the parents of children who have seizures EDUCATE themselves in every way possible so that they can be an active participant in decisions concerning their children! So that they become comfortable in their ability to decide when to call an ambulance or doctor. So their child does not have to be hospitalized with every bout of seizure activity, because the environment there is so detrimental for the child. So they know when to call the doc, if their child is not responding to a drug as expected and tell him if they don't think the drug is right for their child! I completely agree with both Alice and Jeanne that these are important persona l decisions. I also feel that we have an obligation as parents to seek out any education available that will enable us to make the right decision for our child and not to have to rely solely on someone else's judgement.

Deanna

Subject: Hospitals

Alice,

What did your neurologist say about when it was appropriate to go to the hospital for seizures? Our neurologist is not much help in this regard...he says that seizures aren't serious and I'd never even heard the word "status" until I read it in an Angelman newsletter or parent letter. I didn't even know you could die from seizures -- our neurologist downplays it so much.

Marianne (mom to Emily, 6, del+)

Subject: Re: behavior problems

Too bad they didn't suggest an age when he might start to outgrow the seizures. Susan has not had seizures since into puberty---her late teens. Each year seemed to bring on different types---and we had many hospitalizations. Depakote helped wonders. Good luck to you. There are new medications coming out all the time. Perhaps something will come out of the Calgary conference. Keep in touch. This week I'm going back to the Epilepsy center at NIH and Doctor who treated Susan last in 1989. At that time AS was not diagnosed. Perhaps we can get some studies going. Susan is maintained on Dilantin---(I note that no one else has mentioned that) but still has petit mal all the time. Thank heavens the status epiliptics (sp.) has stopped. I felt klonopin made Susan very active --- that was before Depakote was on the market. We quickly took her off it and went on a combination of Depakote and dilantin which worked well---however there were still break through periods usually associated with times of high stress, viruses or seasons of the year. Susan spent every May and October in the hospital---our muggy, moldy D.C. weather---I always felt there was a connection but no one would verify it. We "trained" many a new Emergency room physician. May 10 and Oct. 31 were always the dates give or take a few. Seemed worst when pollen counts were very high. "Mother" always felt these were times when it was more difficult to breathe but, of course, Susan didn't show any symptoms. Keep up you spirits and don't hesitate to take him to the hospital for added medications----we kept thinking the seizures would stop but they never did.

Anne (Mom to Susan--24 UPD)

Subject: Re: behavior problems (and Hospitals)

Deanna-

Thank you for your note! I couldn't agree with you more!! I have often felt that the "trauma" Whitney endured as she was undergoing all the "obligatory tests" in a very strange environment was very detrimental to her recovery. (We often left with absolutely no conclusions about what triggered her seizures.) After 15 long years of experience... and a neurologist who detailed when it might be necessary to head for the ER...we now feel quite confident. However, I can understand the apprehensions of our "younger parents"... Everyone must make his/her own decisions...

Subject: Re: Hospitals

Marianne--

Susan's seizures would start mildly, hours apart and continue until they came very close together---almost continuously. We kept thinking they would stop, but they didn't and of course became impossible to give her more medication. That is when we headed to the hospital for IV. Last time, we called Dr. immediately and didn't wait for them to "go away"--he prescribed adivan. and they stopped. What a miracle drug as far as I'm concerned. Only needed to use it once.

Am sure someone can explain this to you better. The most frightening is not knowing. This might never happen to you---I wish I had known more at the time. Good luck in getting good explanations.

Subject: Re: behavior problems (and Hospitals)

Deanna,

I've been surprised (and somewhat alarmed) to read about all these seizure episodes in older AS kids who had been seizure-free. Emily's worst episode was a period of 4-5 months when she was three, but she's had very few seizures since they were brought under control at that time. (she'll be 7 in October) Are there more types of seizures than what Dr. Hutchinson described at the Seattle conference? (I believe they were tonic clonic, absence, myclonic, complex-partial.) Emily's last seizure a year or so ago was a doozy -- a tonic clonic that lasted six minutes. I got in a panic because it had been so long since a seizure and called 911 -- I was afraid she had a severe bacterial or viral infection.THere was no explanation for that seizure -- no fever or infection. I agree, by the way, about avoiding the hospital, but sometimes it's hard to make the decision if you're not a medical person. When Emily would have several seizures in a row (back when she was 3), she would get a shot of Adavan and it would work. Can Adavan be given orally? Thanks for your input -- it helps having people knowledgable about both medicine and Angelman syndrome on-line. Marianne (mom to EMily, 6, del+)

Subject: Re: Hospitals

Marianne- Our neurologist recommended that we head for the ER (it's 5 minutes away) or call "911" if a seizure episode becomes prolonged-lasting 5 minutes or longer.

I have spoken to Dr. Hutch about this, too, and he said about the same thing. Perhaps Deanne or another medical professional can give more info...Is the danger possible oxygen deprivation?... or???

Our neurologist-or an associate on call- is quite accessible and I feel confident that if we are having any "doubts"- we can describe what is going on over the phone (and aren't we parents good at being thorough??... I've been asked where I went to med school!!) and the doctor can give us advice... We know that he will be cautious and error on the safe side.

I want to make it very clear that everyone must feel comfortable in their decision-making and trust their instincts... I certainly don't advocate recklessness or irresponsibility!

I do want to mention little Lauren Fenzan who passed away last August. Someone else might give more accurate details... but from what I understand... she was having seizures and was taken to the hospital. Later, she was discharged (despite her parents' fears, I believe) and passed away at home. Please correct me if this isn't accurate. Anyway... we all need to keep on our toes and keep well-informed!

Alice Evans

Subject: re: Ativan for seizures

Marianne, Ativan can be given orally, but if your child is actively seizing, that's not very safe because of the risk of aspiration. It can also be given rectally with a small syringe, which is a much safer method and is fast acting.

Jane in Portland

Subject: Re: Ativan for seizures

Also, Dr. Hutchinson said the best way to give ativan in a pill form is to put it between the cheek and gum and let it dissolve. I wasn't told this when I got the Rx and it took forever to work. The pills are so small that they dissolve quickly.

Robbin

Subject: Seizures & Danger signals

Alice wrote in a previous message, and I am also interested in the answer to the oxygen question:

I have spoken to Dr. Hutch about this, too, and he said about the same thing. Perhaps Deanne or another medical professional can give more info...Is the danger possible oxygen deprivation?... or???

In our experience with our son Ben (9) who has experienced status twice, valium via an IV was the only thing that got everything under control. We were misinformed the first time when Ben started to go into status: our neurologist at the time did not return our calls but once in which he said, "the seizures won't hurt him." We learned later that this was not true. From our current neurologist (who is always available via a pager or someone covering for him) we learned that the important thing to do when you see ANY seizure activity is to call him right away to discuss. He said it is important to get them under control, but seizures will usually beget more seizures if they are happening pretty close together.

At one of the Florida AS conferences we went to I thought I remembered Dr. Hutchinson saying something about Lorazepam (not sure of spelling) suppositories to keep on hand in case you are in an emergency seizure situation. (Does anyone recall this?) I asked my neurologist about this, but Ben has been doing so well the past 5 years he didn't think it necessary...and he is just a phone call away, which is the best reassurance in my book -- especially after our first horrid experience.

Best regards,

Jeanne

Subject: Re: behavior problems (and Hospitals)

Seizures: There are only subcatagories of the types mentioned. That's not important, what is, is to be able to accurately describe any and all of the seizure symptoms that you see to the doc in an understandable fashion. That's why I mentioned that. I believe someone had put the seizure article by Dr. H. into the mailbag a while ago, and though it is in medical terminology, there are very good descriptions of the various seizure types in there along with frequency that occurs in AS kids. A medical friend or perhaps the Epilepsy Foundation could define the terms for you. The type of seizure is important because the Neuro docs do try the drugs most effective for a specific type of seizure first. Older Kids and Seizures: I've noticed what seems to be a common thread among the reports of this. It seems that when they've been seizure free for long periods, the attempt is then made to reduce, change or eliminate the drug(s). Then the kids start having seizure activity again, sometimes more severely. A lot of the drugs used for seizure control are either barbiturates (phenobarb), Benzodiazapines (Ativan, Valium, Tegretol, Zarontin, etc), or are other categories of central nervous system depressants. Many of these drugs list the precaution of withdrawing them very slowly, or the withdrawal can produce "adverse" reactions. I would think that the longer a child has been on a particular drug, the more slowly it must be withdrawn. In my past personal experience I have seen physicians who either ignored or seemed unaware of such precautions-so be careful! Calling 911: When in any doubt, do it!!!!!! The reason I suggested learning CPR is that "airway management" is taught there. These children do have large(?), protruding tongues which can obstruct their airway when unconscious. There are very simple tools to evaluate breathing (or "a patent airway") and to open the airway, if it is obstructed. When I say "tools", I mean simple skills that anyone can learn. No real tools needed. I personally have called 911 for Katie twice. In one instance, her breathing was so shallow as to be ineffective, and in the other, she was not breathing and I was unable, due to seizure spasticity to open her airway. In both instances she was losing her color and turning blue. Unless someone sits at a bedside 100% of the time, whether at home or in hospital, the risks would probably be better for one of our kids at home. My favorite saying about Katie is that she requires about 97% of my attention, 100% of the time I'm with her--even if I'm doing something in the next room. BTW, Katie's brother, Matthew was only 4 when he called 911 the first time for me! Ativan: Yes, it can be give orally, IM, IV, rectally, and buccally(in the cheek or under the tongue). I personally don't like it for daily use, because Katie has had bad experiences with the benzodiazapines when we were trying different drugs to get her seizures under control last year! They made her semi-comatose and one of them even made her seizures worse. I hope this answers your questions.

Deanna

Subject: RE: Seizures & Danger signals

In our experience with our son Ben (9) who has experienced status twice,

What does "status" mean exactly???

Chris

Subject: re: RE: Seizures & Danger signals

Chris,

Status refers to when the seizures are recurring one after another. In Ben's case, he was having tonic clonic seizures every 10 minutes which progressed to every 5 minutes. It is a life-threatening condition, can cause brain damage (this I only learned later from Dr. Hutchinson), and in rare circumstances, death.

You want to avoid this situation at all costs!!!!

Best regards,

Jeanne

Subject: Re: Seizures & Danger signals-"Status"

From Taber's Medical Dictionary: " a long, sustained abnormal or pathological condition" also: "s. epilepticus: rapid succession of epileptic attacks without regaining consciousness during the intervals." The term "status" is simply a Latin term that means, "remaining in the state of", and can apply to many medical conditions. Deanna

Subject: Re: Seizures & Danger signals

Jeanne, Lorazepam is the generic name for Ativan. A generic name for a drug is the name given to it by it's developer. It usually is made up of syllables that give some indication of the family of drugs it belongs to or to it's chemical makeup. A drug can be then be given a proprietary name (eg: Ativan, Loraz) by any company that chooses to buy the rights to manufacture and sell that drug. The reason some of the families have been told to give it buccally, is that it will work faster that way. A suppository will take about 20 minutes to dissolve, while in the cheek, a small tablet is immediately dissolved by the saliva and starts to absorb right away. The rectal route is probably a little safer (no danger of aspiration). BTW, any seizure can be dangerous. It is advisable to notify the neurologist of any changes right away, if only to make him aware that something is going on. This way, it is more likely that he will make any associates that might be covering for him aware of your child's case. No guarantees, of course, but really concerned docs often do that sort of thing.

Deanna

Subject: Treating Seizures at Home

When our daughter Olivia was battling seizures (prior to control with depekote) she would have multiple generalized seizures spaced about 40 minutes apart from each other. When she would have a really bad day, we would take her to the emergency room and they used valium (administered rectically) to knock the seizures down. This usually knocked her out, and when she awoke she would no longer have seizures (until the next bad day in a week or two). This process got to be very expensive as our health insurance plan charges $250 for an emergency room co-pay. We developed a good relationship with Olivia's neurologist and he prescribed the same valium for us to administer, complete with a kit and a nurse's instructions to do the same thing at home that was costing so much money for us to do at the hospital.

Kevin Ditamore

Subject: Seizures/Depakote

Shannon has just come off "Status" in June. She has been putting her hands over her eyes and is now doing it more frequently. Her Depekene levels the last time they were taken about a month ago were 137 and the Doc said that it was high but kept her on the same dosage of Depakote. Does anyone know the symptoms of too much Depakene in the system. Would appreciate any experience in this matter. Dr Zorre said he thought it was an "Aura" effect and was the start of a seizure that did not reach seizure????????

Dede & Art Anderson

Subject: Re: Seizures/Depakote

To Dede and Art, Lab methods and values can vary, so I can't say what a normal depakene level should be-certainly your doc is to be trusted on that. Side effects, common, are: sedation, nausea, vomiting, increased appetite and weight gain or anorexia, elevated liver enzymes, elevated serum ammonia. Life threatening side effects are: pancreatitis, toxic hepatitis (usu. in very young children). Drug Interactions are: antacids and aspirin-may cause toxicity. Phenobarb-increased phenobarb levels. Phenytoin (dilantin)-may increase or decrease phenytoin levels. These last two, pheno and dilantin, may actually be a desired effect in some cases, whereby the depakene level could be increased without actually having to increase the dosage. I need to explain about side effects further. These are effects produced by a drug, other than those for which it is prescribed. Experiencing a side effect is not necessarily an indication for discontinuing it. They may wear off after a while. (Example: when Katie was first put on phenobarb, she was v ery drowsy; that stopped being a problem after about 10 days.) Side effects need to be reported to the prescribing physician. Synptoms of pancreatitis and/or hepatitis may include: poor appetite, nausea, vomiting, weakness, lassitude, abdominal discomfort or pain (in pancreatitis), bleeding tendencies (in hepatitis), jaundice (yellow skin and eyeball color). Nowhere do I see photosensitivity listed as a side effect of depakote or depakene. An "aura" is a sensory phenomenom experienced just before the onset of a seizure. They are most usually visual, but can involve other senses, including smell and hearing. Your child may well be experiencing "failed seizures" that are being controlled by the drug(s). Hope this info helps, Deanna

Subject: re: Seizures/Depakote

Art & Dede,

When my son was on Depekene (liquid version of Depakote) our neurologist monitored his levels very carefully, saying that too high a level could also cause seizures. Another possible rare side effect of Depakote/Depekene in general is pancreatitis, which Ben did get.

Best wishes,

Jeanne

Subject: Re: Seizures/Depakote

We have had a couple of experiences when they tried to increase Kelsey's Depakote with poor consequences. Once, she started to become very shakey and jittery (almost like BEFORE she was on the Depakote). They checked her levels, backed her off and she was as good as new.

The other time, she became very, very lethargic. In fact, when she almost fell asleep on the exam table with her pediatrician, he ordered blood tests immediately. Her levels were really high (over 140).

Now that she has been on Depakote and we are more regulated, we have not seen any bad side effects. But I get a little jumpy whenever they mention increasing her levels. Hope this helps.

Kathy V