Tag Archives: ERG

Relieved after talking to the neurologist.

Feeling much better this morning after an appointment with Connor’s neurologist. We were dismayed to hear about changes on the ERG, but I really freaked out yesterday when his medical assistant mentioned in an e-mail that he wanted to discuss the ketogenic diet. The ketogenic diet is no joke–read here–it involves hospitalization to start it, measuring everything he eats and it’s more extreme than Atkins. I have never wanted to do the keto diet and have always hoped it would never even be a consideration.  Don’t get me wrong, it’s not off the table and I understand why people do it, and some have great success, but it just seems like a nightmare to me. I hope to find seizure control another way.

I was really freaked thinking we were going to be told Connor MUST come off the vigabatrin immediately and that the keto diet would be on the immediate horizon. Thankfully, not the case. While a change on the ERG is reason for concern and close monitoring, our doctor said to also take it with a grain of salt. Many parts of the country, people aren’t even doing these because it’s somewhat unreliable at this age. It can’t tell you how much vision is being affected, if at all, only that the retina isn’t responding quite the same way with one of the wavelengths or something–honestly I don’t understand it well enough to explain it. Sometimes, later tests go back to normal and it was just an anomaly, and yes, sometimes the peripheral vision is affected, but the positives of the meds outweigh the negatives. In his experience, most of the time a kid has gone off due to changes on the ERG, they ended up back on it because it was simply the most effective med for them. Unfortunately, keeping the seizures at bay must take precedence over perfect vision. If it was an issue of blindness, I’d be much for freaked out, but hopefully, if he does suffer any loss to peripheral vision, he will learn to compensate.

We do have to decide if we go back for another ERG in a month, or wait the standard three months. He didn’t seem to think it was worth the sedation to go back in a month, though. Obviously, we could have to face decisions over again if we continue to see changes, but hopefully that won’t be the case.

As far as the keto diet goes, he brought that up more as a possibility, and he felt the modified Atkins diet was equally good (and less insane–my words, not his). We will revisit that in a few weeks after we see what happens with the increases in Onfi. We are also finally weaning Keppra- the drug that has been the unchanging constant through all this. We’re not sure it’s really doing anything, so we’ll monitor and see what happens.

He also said down the road, there are other drugs we haven’t tried, though he can’t vouch for them when so many have failed. And we can also revisit surgical options as well as VNS. VNS doesn’t thrill me, as I haven’t seen as much success with that online as I would like for such a major surgical/implant intervention. What is VNS? Read here. That being said, like any treatment, one person’s fail is another’s miracle.

Please cross your fingers for the vigabatrin/Onfi combo and no further change on the ERG!

Last night, we attended a meeting with a Met Life rep regarding financial planning for special needs. The truth is, we don’t know where TSC will have Connor as an adult. Maybe he’ll be doing great and self-supporting, but we just don’t know at this point. So it was time to look into how to plan for the possibility that he may not live entirely independently, possibly because of cognitive issues, or maybe because of health/epilepsy issues. Thank goodness, we did! It’s pretty easy to complicate your child’s situation and have no idea you are doing so. If you have a child receiving any government benefits, such as Katie Beckett or if they are over 18 on SSI, if they inherit anything adding up to over $2,000 they can lose their benefits! Who came up with that number as a cutoff? Seriously? And can so easily happen by accident–savings bonds purchased for them by a relative, naming them directly on a will, putting them as your life insurance beneficiary, or just not having a will and they automatically inherit. Once those assets are spent, they can reapply, but those of us who have applied for such things know how nightmarish it can be. And losing them even temporarily can wreak havoc. The presenter shared a story of a woman in her 50s or 60s living in a group home supported by Medicaid. She had been there for a long time. When her parents passed, they had no will and she inherited what they had, which wasn’t much. She had to move out of the home, and within a few months the assets were spent. She could then reapply, but her spot had been taken. She lost her parents and her home.

We will have to have a special needs lawyer help us with a special needs trust that will protect him from anything like that. Anything that he inherits must go to the trust. That way, if he’s receiving any benefits they won’t be affected. Don’t worry, the government gets theirs. This kind of trust is taxed at one of the highest rates, in case you were worried they’d run out of barricades for our national monuments.

 

 

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Join us for a meeting with new Atlanta TSC clinic director, Dr. Wolf!
Sunday, November 10, 2013
2 – 4 p.m.
Mount Vernon Baptist Church
850 Mt. Vernon Hwy NW
Sandy Springs, GA 30327

Dr. David Wolf will be spending his afternoon meeting the local TSC individuals and families of the TS Alliance of Atlanta/North Georgia. Join us for this valuable opportunity to meet both Dr. Wolf and other local TSC individuals and families!
There will be light refreshments available.

RSVP to Becky pin.the.map@gmail.com

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Vigabatrin may be causing changes on the ERG.

I got a really aggravating phone call yesterday. It was the opthalmologist’s office after they reviewed Connor’s ERG from last Friday. Connor is supposed to undergo a sedated ERG every three months (description here) because he is on vigabatrin- a powerful seizure medication with the potential side effect of loss of peripheral vision. He was on it for nine months previously for infantile spasms. We weaned him off and began to see increasing seizure activity–complex partials, which vigabatrin is also used for. So we went back on it. Connor had two ERGs the first time around. Being that it is sedated and requires an IV, it is no simple procedure. It is handled by day surgery. So we didn’t adhere to the every three months suggestion. We chose not to have an exit ERG when we stopped because the first two ERGs showed no change or issues. We figured at that point, why put him through that, when it won’t change anything? Even if there had been some changes to his vision, he wasn’t having issues and it can’t be corrected. We had no reason to think there were any issues anyway. While there are definitely some stories of kids with some loss of peripheral vision, the stories of no issues seem to outnumber them. That is purely anecdotal by the way–not evidence based–just my experience online. So we don’t know if this occurred last time we were on it, or in the short amount of time this round.

I was so sure we would never have an issue. But the eye doctor reports “minor changes” likely related to the vigababtrin. The neurologist’s office referred to them as “early signs” that the vigabatrin is causing changes. That’s as much as I know. Connor’s primary neurologist is out of the office until Thursday, but we actually already had a followup scheduled Friday morning anyway, so at that point we will have to discuss our options.

Connor is not going blind, to be clear, but nobody wants to see any damage to their child’s vision. But I’m so frustrated. We’re seeing improved seizure control since restarting vigabatrin. Control we couldn’t get with Keppra or adding Onfi, and Trileptal was a total fail. Phenobarbital was used early on, but needed other meds added and is not the drug I want him on anyway. He was on Dilantin for a bit, but when his levels were checked they were nothing, like his body just ate it up (He’s currently on Keppra, Onfi and vigabatrin).

The issue was a no-brainer when we were dealing with infantile spasms. I wouldn’t have even considered taking him off, even with vision loss, because of how dangerous those are. But now that it’s complex-partials, I don’t know what to do. I mean if nothing else will control it, we need to stay on it and hope there are no further changes. He can’t have seizures all day long. But can there be something else that will work?

I just hope his neurologist has a strong idea of what direction to take, because to be honest, I don’t feel equipped to make the call.

It always has to be something. Suck it, TSC.

Fall explorations.
Fall explorations.

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20 20 24 hours to go…I wanna be sedated!

One 45 minute test equals 4.5 hours in the hospital.

Ah, the electroretinography (ERG). a routine test you are supposed to have done every three months while taking Sabril (vigabatrin). You are also required to have an eye exam every three months because this particular drug carries a risk of loss of peripheral vision. Sabril has only been approved by the FDA for a few years. Before that you had to order it from Canada. But then it received approval here, the cost skyrocketed, it became heavily regulated, and it’s only available through specialty mail-order pharmacies. Many people resent being treated as if we’re too stupid to understand and take on the risks.

Given the risks, should we keep a close eye on the vision of our children? Certainly. But the intrusive nature of the ERG makes many families angry. It’s no simple test. We took Connor in for his second one yesterday (mmm hmmm, we’re behind schedule). We had to be at the hospital by 9 and get admitted through day surgery for a test that won’t start until 11. Yup, I told you it’s not a simple test. Then there is lots of information gathering, weighing, measuring, and finding a vein for the IV. Connor likes to make things difficult by hiding his veins. He always requires the IV team, rather than a regular nurse. Then we take him down to the room where they will place contacts in his eyes that test his reactions to stimuli. But he won’t be awake. He must be sedated with propofol (yup, Michael Jackson propofol). Once it’s over, we must wait for him to wake up and drink before they will pull out the IV and we are allowed to leave.

These tests and the frequency with which we are expected to undergo them frustrate a lot of people. We know there are risks to the peripheral vision. But we didn’t put our kids on this drug just for the heck of it. If someone is taking it, it’s for pretty dire reasons. The primary reasons I know of for its use are infantile spasms (why Connor is taking it) and frequent compex partial seizures that have not responded to other medications. Most people (based on my interactions on message boards) wouldn’t take their kids off this drug, even if they were told vision was being affected. Infantile spasms, left untreated, can cause major brain damage. What good is fantastic vision if your brain is fried? And the people taking it for other seizures have likely tried every cocktail in the book and are having so many seizures that their lives are being adversely affected to the extreme. So is being sedated every three months a bit much? You decide.

I’m not really freaked out by Connor being sedated, I guess because I’ve seen so much in his 10 months. I also know he’s being closely monitored. It’s not like Michael Jackson, who was abusing it with the help of a shady doctor. I just think the overregulation is an American agency assuming we are idiots. Should we keep an eye on vision? Yes. Every three months? Some people are on this drug for years. What hell. Especially if you are getting it through Accredo Pharmacy, specialty pharmacy of Medco. If so, you are probably already going through the monthly hell of securing your refill from this incompetent place.

Modeling high hospital fashion.
Modeling high hospital fashion.

ERG 2

Heat packs to bring out the veins.
Heat packs to bring out the veins.
Headed home with giant pupils.
Headed home with giant pupils.

 

 

 

 

 

 

 

 

 

 

 

We recently increased Connor’s dose of vigabatrin again in hopes of putting a dent in these eye rolling seizures. We are seeing some improvement. We actually had a day recently where we didn’t see any, which hadn’t happened in a while. We are also having more days in which we only see 1-2 clusters of eye rolling. But other days we see up to 4 or 5. Then again, for a while we were frequently seeing 4-6, so it’s definitely an improvement. His other seizure med is keppra. His neurologist is considering adding Onfi, and if it seems to help, we will wean him off the keppra.

Any TSC families reading this, I will be going to DC at the end of the month with volunteers from all over the country to meet with members of Congress regarding TSC research funding. I’m in need of personal stories and letters to your congress people. If you can help, e-mail me at pin.the.map@gmail.com. I can give you more details.

Thanks to everyone who has been voting for me to the right by clicking on the Top Mommy Blogs button.