Bump in the Road

Sadly, I saw signs of the spasms today. It is extremely faint - her arms and legs barely tense up, and she looses her focus. It is not bothering her - but I am assuming it will continue to get worse.

She is still crossing her eyes too (this started a few days ago). Sometimes it is really upsetting to her, and other times not. She has done it when she is tired, but also after she wakes up from a nap. Our Dr. said it could be tied to the Infantile Spasms. I emailed him today, so hopefully will hear something soon about our next steps. I'm guessing we are going to have another EEG done and maybe move forward with the ACTH treatment. We'll see.

I'm just bummed because she has been soo happy and seemed really normal this past week. I almost forgot we were in the middle of a crisis! Just kidding, I didn't forget. Actually, we got her signed up with this great therapy program and she is being assessed by the Westside Regional Center this weekend. They offer all kinds of help and it is state funded. Also, my friend Crystal (who is a social worker in ND) had someone she knows send us a ton of helpful information about organizations that offer assistance in CA.

Anyway, thanks for keeping us in your thoughts! I'll update this once I know what is happeneing.

September Plan

Marleigh - right after the electrodes were attached.


Now that we are home the plan is to keep her on the zonegran and B6. Hopefully, she won't have any more seizures - but I guess they do tend to come back in a large percentage of patients. And because her EEG was not normal, even after the seizures stopped, they are still considering the ACTH treatment or possibly surgery. We are working with the doctors to do what is best for Marleigh in the long-term. If these drugs stop the seizures but she will still have a lot of problems later in life we will want to consider the other options.



Keith and Marleigh hanging out in our hospital room.

The plan right now is:
1. Do a follow-up appointment in 3 or 4 weeks for a "Snap-shot EEG" which is a shorter brain scan, so they won't have to glue on the electrodes. If her brain waves are still showing Infantile Spasm diagnosis, even if no seizures are present, then we'll start the ACTH therapy.

2. If she continues to be seizure free and the brain scan looks good but she is falling behind developmentally, then we'll do the ACTH. Because the Zonegran may be slowing her down, due to the drowsiness side-effect.

3. If the seizures come back at any time, we'll start the ACTH therapy.

4. If ACTH therapy does not work, then we are looking at brain surgery. It sounds like they have the best results when the child is close to 1 year old. This is part of the reason that they want to do the ACTH sooner rather than later - I guess it is about a 3 months process, so they would want to have time to see if it was effective and still be able to do surgery before she is 1. Surgery may involve us getting a second opinion at John's Hopkins - so prepping for it could take months.

In the meantime, we are thrilled because we are seeing a lot of smiles from her!!

Zonegran Working?

So we checked into th hospital on Monday (9/15). The plan was to start the ACTH therapy but it turns out her seizures have stopped. We think it is because of the zonegran, becuase we increased the dose last Tuesday to 100mg per day and over the last week we have seen some of the seizures become less severe and shorter, mixed with some of the normal more intense seizures. But the last serious one she had was on Sunday at 11:30am (there were other smaller ones till 1am on Monday morning). Which is really great news!


Our doctor decided to have her hooked up to the EEG with video monitoring again to really make sure that there aren't seizures that are not visible or that happen when we are asleep, etc... She was a real trooper this time and didn't make much of a fuss while Andrea attached the electrodes (it is a long and smelly process - the glue has Ether in it). And when the glue dries it feels tight on her head. So we didn't get much sleep last night and she's more than a little crabby today.

The good news is her EEG showed no seizures. Sometimes there are seizures happening that don't show up physically, so it is good to know that she is not having that either. They are keeping us another day just to make sure. If she does have a seizure we'll be here longer to start the ACTH therapy. If not, then they'll send us home in the morning

Starting New Treatment

So I found out today that we are starting the next treatment option sooner than we thought. This means we'll be admitted to the hospital on Monday.

The current drug (zonegran) is only making Marleigh sleepy and there has been very little change in her seizures. I'm a little surprised we are moving to the ACTH on Monday because I thought we were going to let the zonegran run for two more weeks first.

On one hand it is good because, I have heard that the longer the seizures continue the more likely there is imparment in her learning abilities. ACTH has been a drug for use in Infantile Spasms since the 50's, so it isn't some new drug that Doctors are experimenting with. While I don't know how long her course of treatment will be, it won't be longer than 12 weeks.

On the other hand, while it is generally pretty successful it does have some very serious potential side effects. Like heart failure for one. I'll post all of the ACTH info from EpilepsyFoundation.org in a different post.

So we are going to enjoy the weekend and hope for the best on Monday (9/15). I think she'll have an EEG again and be generally wired up to keep track of all of her vitals. I should be talking to the Dr. tomorrow so I expect to have more information then.

ACTH Treatment Info

ACTH®

generic name: adrenocorticotropic hormone -- HP Acthar Gel

Manufacturer: Questcor Pharmaceuticals

Uses: Treatment of Infantile spasms. ACTH has been used since the late 1950’s to treat infantile spasms although this use has not been submitted to the U.S. Food and Drug Administration for approval.

How it works: It is not understood why or how the ACTH works to control the infantile spasms.

How given: ACTH is given by intramuscular injections. Parents and/or caregivers are taught how to do this by medical/nursing personnel. It requires a prescription for the ACTH and equipment such as syringes and needles. Dosing amounts and schedules will be determined by the prescribing physician.

Duration: Unlike other medications, ACTH is used only temporarily for periods ranging from 2 – 12 weeks depending upon the seizure response.

Side Effects: Most children will gain weight and retain extra fluid during the time they receive ACTH. This weight may be seen with facial puffiness. The weight will disappear once the ACTH stops. The appetite increases and the children crave more food. Irritability with increased crying and decreased sleeping are common side effects. High blood pressure, heart failure, calcifications of kidney and pancreas, increased risk of infections, increased blood glucose are other possible side effects. Children receiving ACTH should have blood pressure, weight and serum electrolytes monitored frequently during the time they are receiving the ACTH.

Care during ACTH treatment: Do not receive vaccinations. Avoid exposure to those who have received recent polio vaccine. Avoid known exposure to varicella (chickenpox) and measles. If exposed, contact your treating physician. If surgery is required during the period of ACTH therapy or within the first 12 months after stopping treatment, be sure to let your physician (surgeon) know as supplementation with a steroid may be required at the time of the surgery.

Possible drug interactions: some heart medicines, acetazolamide, amphotericin B, nonsteroidal antiinflammatory drugs (e.g. ibuprofen), aspirin, barbiturate preparations, growth hormone, phenytoin, potassium salts, rifampin, water pills.

Possible Contraindications to Use of ACTH:

Diabetes

Acute or chronic bacterial or fungal infections

High blood pressure

Heart failure

Renal failure with fluid retention

Not everyone experiences side effects. There may be other side effects not appearing above. For a complete list, consult your doctor, nurse, or pharmacist. More detailed sources of information on side effects include the drug's prescribing information sheet, the Physician's Desk Reference, or pharmaceutical company which produces the drug.

9 Weeks

Well, I cannot believe we have been doing the seizure meds for more than two weeks already. I have been communicating with our Dr. via email to let him know how many seizures she's having each day. I'm writing them down and tracking how long they go for. It is still usually about 10 minutes, but sometimes they are more or less intense. I may not be catching every one of them but it does seem like there are a couple less each day. (She was having up to 11 a day and now it is more like 7 or 8.) I asked the Doc if that was the medicine and he said it is hard to say what is medicine and what is the seizure changing on its own.

The plan continues to be to keep up with the B6 and increase the Zonegran each week until we are at 150mg a day, so 4 more weeks - if at that point she is still having seizures then we will look at the ACTH therapy. It would be really great for Marleigh for this medicine to work, so send positive thoughts.

Some other things of note. She is actually doing good physically and has not technically fallen behind on any developmental milestones. I'll be keeping a close eye on that to make sure there isn't something I can do to help her. She is sleeping a little more so that is really nice!

Our family & friends have been super supportive, sending well wishes and helping us out in ways we never dreamed of! Thank you very much to everyone for your prayers, phone calls and happy thoughts. It has been really nice reconnecting with everyone.
Hugs,
Erin

Apointment with Neruologist

Well, we met with our neurologist, Dr. Kornblum on Tuesday. I had a huge list of questions which he did his best to answer. And we left feeling more than a little down.

Basically, because there are so few cases of this a lot is not known. And I hadn't considered the possibility that maybe nothing, not even surgery would stop her from seizing. I did have it in my head that maybe we should skip to surgery, since I know it can have a very high success rate in eliminating seizures entirely - but my expectations were brought back down to earth. The Dr. said he would not want to operate until she was a bit older, like 6 or 12 months old. And that was only if, at that time she was a strong candidate for the surgery, which would involve another hospital stay, in depth testing and possibly getting a second opinion (in depth testing included) at another hospital (he suggested John's Hopkins or Harvard Medical School).

It is really hard to sit here and watch her have an episode, especially when she screams every time she has the spasm (which can be 20-40 times in 8minutes). Researchers beleive that these spasms aren't harmful, the problem is they prevent the brain from it's regular learning which can slow developement, and after a seizure people tend to be confused for a bit and then want to sleep. Dr. Kornblum says that it isn't the spasm that can cause autism, epilepsy or development problems, it is the thing that is causing the spasms.

I emailed out the link to this blog to my family and friends and almost right away my cousin Julie got back to me. It turns out someone she knows has a child with a very similar diagnosis. So I'm looking forward to talking with her. On her blog about her son Ty, she had this link about surgery as a cure for Epilepsy. http://www.msnbc.msn.com/id/18786765/from/ET/ I thought you might find it interesting. Of course, we are still hoping that the medicine she is taking will help. Cross your fingers. The Dr. just doubled the dose, so hopefully there will be some improvement.

As far as the chiropractor goes, the neurologist is strongly against it. He said it won't help and it could hurt. Acupuncture is the other thing we were looking into, and he supported that although, he feels the needles would be really uncomfortable for Marleigh and that you always have to be careful not to expose her to infection. I think there is also a kind of pressure point massage they can do, and I like the spiritual aspect of it. We are going to avoid herbal treatments for the time being, aside from the vitamin B6 that is. As for me, I'm still going to make a chiro appointment ;)

Keith is doing good. I think it is hard for him to be back to work with all this going on. But he has enough vacation that he can take days off when she has an appointment.

Dylan is also well. He has a lot of fun at his preschool and enjoys being a Big Brother. He was pretty worried when we were at the hospital, asking a lot of questions about the wires on her head and the IV. But now he seems better. He likes to lay on the blanket with her under the play gym.

And finally, Marleigh is actually doing great. When she is not having a spasm she seems totally normal. And today, for the first time, she smiled back at me. She had been smiling, but this time I know it was in response to my smile. That gives me some hope that she'll be OK.