Bug in Theratogs

Well, while we are anxiously awaiting the test I will fill my time by writing a little blog about Bug's new TheraTogs. Bug has been wearing them for about a month now. Once on her, they look like some sort of spandex bodysuit. The TheraTogs catch-phrase is "Therapy You Wear". Bug's physical therapist at Jim Thorpe Rehab recommended them for Rayleigh Bug. She said it would help with Bug's low muscle tone and her sensory issues.

Here are some lines from around the TheraTogs website:

For patients with biomechanical or neuromotor issues

TheraTogs work by allowing the clinician to set the patient’s body in proper functional alignment, and letting them re-train their body through the ‘practice’ of daily activities. By applying prolonged, gentle forces during activity, TheraTogs can actually change the muscle and connective tissue – and for infants and young children, bone and joint development as well – in favor of better function and better alignment.

For patients with sensory and sensorimotor issues

TheraTogs work by applying consistent, gentle compression and proprioceptive input across the entire trunk, and by offering stability and support in a dynamic system that moves with the patient.

For sensory integration/sensory processing applications...A snugly-fit TheraTogs system provides the wearer with gentle, consistent proprioceptive and deep pressure input - a 'wearable hug' worn discreetly under clothing.

Here's there prochure with some more great bullet-points and information about TheraTogs for children http://theratogs.com/Doc/pdfs/TheraTogs_Corporate_Brochure.pdf

And if you're interested in the Research and Studies that have been done on Theratogs you can visit http://theratogs.com/Research.aspx.


In a nutshell, for Bug, Theratogs are meant to help support her low muscle tone because they wrap around tightly and hold her snug and give her deep input. Like it says above, they provide a wearable hug. They release the same feel-good hormones as a long hug or petting an animal which helps with her sensory issues.

She wears them every day, all day, and takes them off for sleeping. They are hand-washable, and I wash them about every other day. They stretch out but after washing them they form back to their shape.

These are all the pieces that wrap around her to form the TheraTogs

This is how they go together - it's a puzzle

The inside, that touches her skin, is like a soft leathery foam

This is what the basic suit looks like:

The white tabs are plastic and have velcro-like teeth on one side that adhere to the gold fabric

Here they are under her clothes

And then we've added this back strap which helps tremendously with her back arching:

And we just recently added these 3 straps across the belly, one straight across and the other 2 form an "x" across her tummy. These are supposed to help her stay forward in the sitting position but I haven't seen much difference between adding the back strap and then adding these.

At first, when it was just the suit, I didn't think these would be something we would need to stick with for long because we weren't seeing effects but after adding the strappings we can all tell that they help!


This is something new I'll be including at the end of each blog, Rayleigh's Stats:

Medicine: Ketogenic Diet, 4:1 ratio; Clobazam, 1 10mg pill 3 times a day; Vimpat, 50mg pill 1 in morning & 1 1/2 at night; Lamictal, 1 5mg pill twice a day.

Seizure Control: This week she has only been having 1-2 seizures a day. They are lasting about 10-25 seconds. She locks up, labored breathing, eyes look left and then she relaxes and breaths normal and goes back to sleep - they have all been during sleep as of this week.

Success and A Test

I'll get straight to the point on this post - We have reached our goal in raising the out-of-pocket expense for the EIEE test that Rayleigh needs done!! We have had the blood drawn and will get the results (and the bill) within the next 3-4 weeks!

A huge THANK YOU to each and every person that helped either by donating items for the garage sale or money to the fund, or with prayers, love, support and spreading the word! We could not have done this without you!

“We can’t help everyone, but everyone can help someone.”
-Ronald Reagan

So, again, thank you for choosing to help Bug and I will keep you posted on the results and anything that may be interesting enough to post about before then.

Pediatric Epileptologist Visit

Today Rayleigh had her first visit with our state's only pediatric epileptologist, Dr. Ng. We started out with about 20 minutes of her history: full term birth, no complications, home at 2 days old, when we noticed seizures, when we got diagnosis, that they were originally tonic-clonic seizures, that they've changed just about every single time we've changed medicines, what medicines she's been on, that she's now no longer having infantile spasms but she is back to having partial seizures, that she's on the Keto Diet, that she's taking Clobozam and Vimpat and we've got planned for the future as far as upcoming MRIs and such.

Wow.

Dr. Ng is better at explaining things than we expected him to be. He's also very good at having a long-term plan as well as a start-right-now plan. He is more aggressive than Bug's pediatric neurologist but we were told to expect that. He's also good at giving facts, statistics and percentages which we like.

It's been 2 weeks since we raised the Vimpat levels last time and we haven't really noticed much of a change. Dr. Ng prescribed going up on the Vimpat to 1 1/2 pills twice a day starting tomorrow morning (we were at 1 morning, 1 1/2 night), this will be as high on the Vimpat as she can go.

If in 2 weeks we do not see a significant decrease in seizures, he prescribed Lamictal. We have to work our way up to the full dose of Lamictal very slowly for Bug. Lamictal has only one major side-effect that happens to about 1 in 10,000 patients: a possibly fatal rash. We can decrease the chances of the rash even more than that by starting her off very small on the dose and working her way, over 3 months, up to the full dose. He said that it shouldn't cause drowsiness, irritability or many other side effects that are associated with anti-convulsants and it is a very successful drug in seizure control.

Dr. Ng's office is scheduling an EEG through their hospital for him to have a fresh look at her brain activity and he will add his name to see the results of her upcoming EEG that will be scheduled through her neuro. Other than that, he had us go down to labs to check gluclose levels and that's it for today. We see him again in 3 months along with a geneticist in his department.

Garage Sale to Earn Money

Continuing our efforts to raise money for Rayleigh's Early Infantile Epileptic Encephalopathy test, we are going to start gathering items for a garage sale. If you have any items that you were just going to take to a secondhand store or give away, please consider donating them to us for the garage sale. All money from the sale will be going towards Rayleigh Bug's EIEE test.

To learn more about this genetic test that Rayleigh needs please read this blog post.

We are definitely not afraid to work for the money that Rayleigh needs!  We have already been going through our own home finding items all over to sell. If you have anything that you would like to donate for our garage sale it would be a very appreciated contribution! We'll take anything! Clothes, picture frames, baby items, books, jewelry, kitchen items, electronics or anything you're willing to donate to the cause! Anything unsold can either be donated to a Goodwill or given back to you - whichever you prefer!

We're more than willing to come get the item(s) from you if you live in or around Oklahoma City. If you are out of town or state and would like to contribute items for the sale to raise money for this test you can mail them, contact me for information.

Early Infantile Epileptic Encephalopathy Test

Rayleigh's next test that is needed is called the Early Infantile Epileptic Encephalopathy (EIEE). This will test for these rare genetic disorders, each on 2 different levels: CDKL5, ARX, and STXBP1.

Information on this test provided by Athena Diagnostics Quick Guide:

The Early Infantile Epileptic Encephalopaties (EIEE), including Ohtahara Syndrome and early myoclonic epilepsy, are a group of severe epilepsy syndromes characterized by intractable early-onset seizures (generally in the first months of life) and a highly abnormal "burst-suppression" EEG pattern.

EIEE typically presents with treatment-resistant tonic spasms, focal seizures, and rarely, myoclonus. Over time, 40-60% of EIEE patients evolve to West syndrome, characterized by infantile spasms and a highly disorganized "hypsarrhythmia" EEG pattern. In general, many of these patients evolve to Lennox-Gastaut syndrome (often, but not always, between ages 3-6), which presents with mixed seizure types. Testing is indicated for individuals presenting with the above symptoms (tonic seizures, abnormal EEG, and myoclonus).

Reasons to test for EIEE:
Genetic testing can:

• Provide a confirmatory diagnosis
• Genetic testing for STXBP1, ARX, CDKL5 was rated "very useful" and "highly accurate in correct clinical context" by International League Against Epilepsy (ILAE) guidelines
• STXBP1 is mutated in 36% of patients with Ohtahara Syndrome.
• Provide important inheritance and genetic counseling implications
• ARX is an X-linked disorder that is seldom inherited, providing important inheritance implications
• Determine appropriate treatment options
• STXBP1-associated seizures in EIEE may respond better to Vigabatrin

This test is over $10,000. Insurance is willing to pay for some of the cost but we will still owe $2,100 ourselves. We are now on a mission to raise enough money so that Rayleigh can get tested as soon as possible.

Below is the link to a fundraiser I have set up to collect some of the costs needed, please share it with anyone who may be able to help Rayleigh Bug!

If that doesn't work, try this direct link to the Fundrazr: http://fnd.us/c/eKEyc

Catch Up Post

Wow. I cannot believe it has been over a month since I've posted anything. Not many new things have been happening for me to post about, I suppose.

Well, that's not entirely true. We've made some changes. Bug is still on a 4:1 ratio on the Ketogenic Diet and trying new foods all of the time! She's still such a good eater! Bug is also still on the same dose of Clobozam, 1 pill 3 times a day. The change is that we added Vimpat about a month ago and then raised the dose of it about 2 weeks after starting it and that's when we started seeing improvements. The head drops decreased. But she has been having partial seizures.

A partial seizure happens in a specific side of the brain (left side for Bug), the body generally tenses up, arms will extend with clutched hands, legs will extend with curled toes and face will redden. Bug breaths well the entire time for most of these seizures, she also looks off to the left side with her eyes and occasionally smacks her lips throughout the seizure. Hers only last 15-45 seconds, and sometimes it's less than 10 seconds.

While a partial seizure isn't as harmful on the brain as infantile or epileptic spasms, it is still scary and definitely something we want to fully control. Infantile or epileptic spasms can be worse because they cause a lot spike and slowing activity in the background of the brain along with spike activity during the actual spasm. With partial seizures, her brain activity is more normal between seizures allowing for more development.

That being said, we still have not made many new milestones. She can now take the sippy cup to her mouth from the tray on the highchair if we put her hands on it. And she will take it to her mouth when you put the cup in her mouth while she's propped up or being held every time. She's still arching and resisting the sitting position, we're getting Theratogs that might help (that will surely be it's own post). And she doesn't have the same muscle tone in her legs that she used to. Bug outgrew her Jumperoo and her walker over a month ago and the Lecky Squiggles stander that we ordered is still not in. So the only standing she's getting done is when we hold her up and at 27.6 lbs we can't hold her very long but we're working on it!

We just increased her Vimpat again on Wednesday because she was still having 2-4 partial seizures a day. Before raising the levels (a week before, actually) we took her up to get her blood drawn so labs could check her Vimpat levels.

Her level was low + continued partial seizures = raising the dose.

So, the worse part about raising Vimpat is this: Every time we raise the dose we go through a rough couple of weeks. There are side effects with all medicines that affect children differently so this is just our experience.

Week 1 of the raised dose comes with insomnia. Insomnia comes with crankiness and both of those lead to more seizures. It's bizarre that she has more seizures when we raise a medicine that is meant to control seizures.

Week 2 of the raised dose involves drowsiness and irritability. The drowsiness only really becomes a problem for her physical and occupational therapies, all she wants to do that week is sleep through them all. The irritability is pretty bad though, she acts like a full on 16 year old girl and it seems like nothing I do makes her happy. But the week after those 2 are over things really settle down and we get the chance to see how the new dose is acting. So give me 2 weeks before I report any change on that :) Hopefully I'll have good news to share!

Just a Quick Note

So I don't have a lot of time to post, Bug is getting antsy and Sesame Street is almost over!

Bug had an EEG a couple Fridays ago and then a follow-up appointment with her neuro the following Wednesday. He said that there is some improvement! Woo-hoo!

It was funny, when he told us the news, neither my husband and I were expecting to hear that so we both were like "Really?!?" "Are you sure?" We weren't trying to imply that he didn't know what he was talking about, we were just shocked!

See, the thing is, since we weaned her off the Sabril medicine and started the Vimpat medicine (still on Clobazam and the Ketogenic Diet) she started having less headdrops but more small partial-type seizures.

So her background activity has normalized a bit, meaning that there are not as many spikes and slowing as there were on the previous 2 EEGs. She's still having spikes but between the new medicine and getting her ketones as high as possible, we've controlled some of the seizure and epileptic spasm activity.

This is great because the less spasm activity there is, the more room for development improvement we have!