Thursday, May 6, 2010

New Diagnosis: Infantile Spasms

After a month of contacting my neuro here in Fort Wayne, seeing a neuro at Riley Children's Hospital, and still not getting Myra's 'possible' seizures under control, I was getting quite frustrated. After a bad reaction to a new drug this past weekend, I called both neuros Monday morning. Dr. Calm at Riley has moved to another hospital, so I needed to find a new neuro at Riley. As part of God's intricate plan, I got in touch with the "epilepsy specialist" at Riley, Dr. Z. After hearing a summary of Myra's history and current condition, she felt I needed to go to Riley's ER as soon as possible, be admitted into the hospital, have a repeat EEG and whatever other tests to figure out what is going on with her. It seemed a little extreme at first, but Mr. Prince and I just are wanting answers and figured this was the best way to get them at this point. So, I head down to Riley Monday evening (5/3/10). By God's grace Myra did not cry on the way, even though she had been crying almost the entire day.

I was in the ER from 11:30pm to 4:30am (which was filled with around 6 doctors/nurses/residents waking Myra every 30 min or so and her subsequently crying herself back to sleep after every one) and it was 5:45am by the time we were admitted and settled into our room, which we shared with another baby who was up and crying. Myra's white noise machine was our life saver while we were there and we fell asleep quickly. At 7:15 we were awaken by the nurses saying we had an EEG immediately. 1.5 hours of sleep was not adequate for either of us and the EEG did not go well. I'm guessing the results were inconclusive because we started a 24hr video EEG around noon that same day.
Myra after IV, not happy.
Sleeping in between doctors bugging her.

They glued a bunch of electrodes to Myra's head and I hit a button each time Myra had an 'episode' and they recorded her on camera, too. I could see the EEG running right next to another little girl's. It didn't look normal, but that was all my expertise could tell. My mom came to help for the night. It was a long night, but we got a few hours of sleep. In the morning they transferred us back to the infant unit in the hospital while we waited for results for 6 hours.

Video EEG

Dr. Z came in and said they have diagnosed her with what were called "Infantile Spasms." I had heard of these before and researched them myself and knew it wasn't a good diagnosis. Four doctors, including two pediatric neurologists, had seen these 'episodes' and did not think they were Infantile Spasms (IS). She has atypical EEG findings and atypical physical symptoms of IS.

Infantile Spasms are a catastrophic form of epilepsy that usually starts around 4-6 months lasting until around 2. They are abnormal brain waves which cause clusters of 'spasms' or specific jerky movements that each only last a few seconds or less. Children can lose developmental milestones at the onset of spasms and prognosis is better the quicker doctors get the spasms to stop. The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms. Spasms usually resolve by mid-childhood, but more than half of the children with IS will develop other types of seizures.

Myra's spasms have increased since they started on March 21st. They started with just one or two clusters a day of 1-3 spasms. They increased on week 3 to 4-5 clusters a day of 20-25 spasms. Week 4 she had 5-7 clusters a day of 50-60 spasms. She was given Keppra on week 4, and they decreased the spasms to 5-7 clusters of 20-25 spasms a day.

During the video EEG she was recorded to have over 10-15 clusters of 20-60 spasms (5/4/10).

Today (5/6/10) from 7am to 2:30pm she has already had 6 clusters totaling over 700 spasms.

Choices for treatment are few and all have the risks of some horrible side effects.

Choice A is Topamax.
This is an anti-seizure medication that has a small likelihood of helping control IS. It would be a month before determination could be made whether it was helping control the spasms. Side effects of Topamax are kidney stones, UTIs, decreased sweating, suppression of cognitive abilities, decrease in appetite.

Continuing spasms for another month would carry it's own side effects.

Choice B is Vigabatrin.
This is not FDA approved and only available in Canada. Side effects are changes in white matter brain tissue after MRI scan when treatment is stopped (inconclusive whether damage is done because most children already have brain damage and it's unknown what limitations they might already have), and significant peripheral vision loss in 1 in 3 children.

With cortical visual impairment, Myra may only have her peripheral vision in tact, so we are not willing to subject her to more damage.

Choice C is ACTH (adrenocorticotrophic hormone).
ACTH is supposedly the most effective drug for IS (60% will stop having spasms after treatment). But many children have a recurrence of spasms after 2-12 months. ACTH is given through injections twice daily. Side effects are serious and potentially life-threatening. Side effects are increased appetite and excessive weight gain in the face and trunk, high blood pressure, electrolyte imbalances, high blood sugar, changes in mental status because of high blood pressure, and bleeding from the gastrointestinal system. Weekly blood pressure checks and blood draws help monitor effects. Mortality rates are around 4%.

So, choices are bad, worse and horrible! I feel like we just got kicked in the gut while we were already down...

We have contact Dr. Z to start ACTH as soon as possible, given Myra's exponential increase in spasms in the last day. It will probably take a week to start treatment.

Please, please, please pray for Myra. Pray these spasms stop before we even start ACTH! I know God has used Myra's miracles in the past to grow many of His children and I'm praying He uses this opportunity as well.


Taz's Mama said...

oh gosh. i read through this cringing over and over. i know this is really bad news. i'm not going to placate it to make you feel better. it's bad. and i'm so sorry. i don't really have anything better to say. just that people are praying and you never know what can happen. be prepared for the worst but hope for the best. i will pray for poor myra. and i'll pray for you and paul and the rest of the family as you deal with this and figure out how to move forward. i know this is so much stress on you and your kids. keep us updated.

Mo said...

Hang in there, girl. You're sweet baby is the same baby today as she was before the IS dx. You will come up with the best plan as you can and take it all in stride. It stinks, but hang in there.

Shanna said...

Oh, sweet one, praying now!

ANewKindOfPerfect said...

I just found your blog from another and wanted to comment. Your daughter has sure been through a lot in her short time. My daughter also has microcephaly, cerebral palsy, and epilepsy, although it's not IS. I just wanted to say that I will be following your blog and hoping for the best for your little one!

Anonymous said...

Go for ACTH or Vigabatrin. Dont even waste time on Topamax (not really for IS)

Join the infantilespasms mailing list on yahoogroups

-C (father to a child who has IS)