The Best Month in '06!

I'd like to start out this blog by saying Jordyn has been seizure-free for one whole month! AMAZING! We started her on a new medicine called Depakene. It has a few bad "possible" side-effects to a couple of organs, but we are taking the added precautions to make sure her levels are good and that she's fine. The only discomfort she's been experiencing is teething. Her molars are reeking havoc on her gums and Oragel makes her vomit, so she's not been a happy camper OR a night-sleeper for the last week or so. Her seizure medicines as of today are the new Depakene, the life-long pal Phenobarbitol, and the nasty-tasting puke-inducing Zonegran.

On another note, for those who don't already know, John and I are expecting another kiddo in July! I am a couple of months pregnant now and VERY excited! Of course, we are also very nervous. My OBGYN doc is great and takes my worries seriously. I will be doing a series of tests just to make sure the baby is healthy and fine. Of course, if we had done the same tests with Jordyn, Aicardi Syndrome wouldn't have shown up in a million years due to it's crazy rarity (word?) but this time, we just want to be more prepared. The odds are on my side with this one and I just pray to God every day to bless Jordyn with a happy, healthy little brother or sister to play with and help her flourish.

Although Jordyn is doing well with her seizure-control, I still went ahead and scheduled her for an evaluation in January to see if she'd be a candidate for surgery. It's January 22-24 in Dallas, and the only test they'll be running that I know of is just a 48-hour EEG. She's had tons of these EEG's, but this one will be super-long and the neurologist there will determine if the seizures start and stay in that left-hand side of her brain. If they meander to the right side, than surgery's a No-Go. We'll see.

So, Jordyn's doing great and I'm pregnant! Big news!
I hope this finds everyone well also, and we wish everyone a very safe and happy Holiday season!
Kristen, John, and Jordyn

Next Step, Brain Surgery...

Ever since my last post, things around here have been so hectic. After our decision to take Jordyn off of the Ketogenic Diet, we also had to make a hard decision to take her off of the "Canadian Medicine" Vigabatrin, also. Vigabatrin had been doing "OK" with her seizure control as long as it was paired up with Phenobarb and Zonegran, too. But, we were going to have to increase it which meant we were going to have to buy a whole lot more of it, more frequently. It's a very expensive drug that's not FDA approved and we wanted to substitute it with an insurance-covered, FDA-approved medicine that we wouldn't have to worry about so much. The HUGE problem, though, ended up being that we weaned her off of the Vigabatrin too quickly and she had a horrible reaction to it. Our funds were tapped from the Diet, and from missing work due to the last hospital visit, and FedEx is breathing down my neck, so we just couldn't swing another shipment of Vigabatrin.
We would have not paid a few other bills and called Canada for one more box had we known the extent of Jordyn's withdrawal seizures from the rapid wean we did:
On Monday, October 16th, John called me at work and told me to get home because Jordyn was having very strange, continuous seizures where her mouth and hands were twitching and she had excessive drooling. I raced home and called an ambulance. No seizure has ever been this scary. Usually, we'd give her a rectal medicine that stopped seizures quick, but it hadn't worked. The team came and we flew to the Children's Hospital where they tried 3 times to get this seizure spell stopped. After an hour, Jordyn finally fell asleep and her little hands stopped twitching. They kept her in the ICU for a night to monitor her close since she's never had a seizure this long. She was doing better, so the next couple of nights we were in a regular room. Both the doctor and John said it wasn't our fault that she was doing so badly, and that she could've had these bad seizures for any reason, but I still feel horrible.

We started her on a drug called Lamictal to be paired up with the Phenobarb and Zonegran so hopefully it will kick in and help. Lamictal cannot be given to her in large amounts yet, or she'll have a bad skin rash reaction, so we'll have to slowly increase it week by week until it's at it's full potential.

We hate these stupid drugs!! She's been having some subtle spasms where her right arm will raise up and her neck will tighten. I gave her some meds that the hospital uses, but they just won't go away until I give her a large enough amount to make her so drowsy that she'll fall asleep.

I have been trying to get things in order for her Brain Surgery Evaluation in Dallas. They will keep her for a few nights and run all sorts of tests to see if she's eligible for the surgery.(Called a Temporal Lobectomy) If their tests show that the seizures are started only on the left side of her brain, then she'll be a good candidate. If not, then back to the drawing board.
Aside from having to deal with different Dallas Hospital people on the phone, trying to schedule this evaluation, (The doctor is doing the evaluation even though they don't take Jordyn's insurance, and every one I've talked to keeps trying to blow me off because they don't know about her case) we are also trying to figure out how we are going to be able to afford this trip with our part-time schedules. Missing work, gas to and from, food... that's about $400 that is not in our beloved little budget.
I'm trying not to worry about it so much, but I may have to host a very chilly "Aicardi Car Wash" (any helpers? lol) or something soon and try to raise the funds.
I just hope that Jordyn is a good candidate so we can stop the meds and start working full force on her development and just Jordyn herself. She is such a happy kid when she's not "drugged" up. We may start the Ketogenic Diet again, but I want to make sure that we get the right info on what to do the second time around.

So, right now we are trying to organize this Dallas trip while also seeing how this new Lamictal drug is going to work with her other two meds. I'm sorry I didn't make this long story short, but I've been just so full of emotion. It feels so good to "get it all out" and share this with you, my family and friends. Thank you everyone for reading her updates and being such wonderful people! We love you all.

Kristen,
John, and Jordyn

So Far, So BAD!!

There will always be speedbumps in your plans that will slow you down. It's the pot holes that mess up everything. The Ketogenic Diet should be a pretty smooth transition: You give the child certain measured foods, certain approved medicines, you check their ketone levels in the urine, and you give them vitamin supplements that the food lacks. No biggie. Ever since Day 1, Jordyn has had issues with almost every aspect of the diet. First, she vomited in the hospital because she is allergic to both Soy and Milk. After we got home, she was vomiting up a certain approved drink because it's first ingredient is Citric Acid and she's apparantly intolerant to that. Then, she started spitting up and vomiting because the vitamin supplement was way too "heavy" on her tummy. It's been a little under a month and the poor child has been sick for more than half of it.

Not only that, but with all the vomiting she hasn't been getting the full effect of her seizure medicines. It's the medicines that have been controlling her seizures this whole time, not the diet. She hasn't even had ANY ketones since the 3rd day into the diet. And THAT was only because she wasn't eating well because of all the vomiting. {grrr}

Pot holes in the plan. But that doesn't mean it's botched. It just means we'll start over with more knowledge on what to expect. For now, though, John and I have decided to take a break and let Jordyn have her regular formula and Gerber foods and get well. The only foods that were "coating" her tummy were Micro-Lipids and tiny amounts of beef and applejuice, so this break is necessary for her to regain her strength and give her belly a break. When I told her Neurologist about our decision to take her off the diet for a while, he replied "You have been right about everything so far, so I trust your decision". I loved hearing that, of course! :)
There's a chance that even though she wasn't in ketosis, the modified diet could have still had an affect on her seizures. She hasn't had any since we put her back on Phenobarb, but there's always a "Honeymoon Period" where the great no-seizure time wears off. If she starts having seizures again, we'll start the diet again. If they don't stop still, then the Honeymoon Period was in full effect, and the diet doesn't work for Jordyn.
At least then, we'll be able to have a smoother start with the diet now that we know where all those pot holes are. :) I have high hopes for it still.

We started her back on Nutramigen formula and baby foods on the 9th, so we'll see what happens next. Hopefully, the medicine concoction (Phenobarb, Zonegran, and Vigabatrin) prevails... I'm still working out the kinks in Plan B, which is surgery Just in Case.

Thanks for reading!

Happy 30th Wedding Anniversary to Debbie and Terry Kuns
and
Happy Birthday to Annie Gomez!
Both special days were on October 8th.

Kristen, John, and Jordyn (16 months old)

So far, so good! :: Ketogenic Diet

Hello, everyone!
We got back from the hospital late Friday afternoon. It was an extremely long and tiring week! The process of starting Jordyn on the Ketogenic Diet on Monday should have been a fairly easy transition, but the first 2 days were horrible. It turns out that she is allergic to not only Soy products, but Milk products, too. For a whole month prior to the diet, I tested her allergies to milk, and she didn't show any signs of sickness, so this was a tiny surprise to me. The main menu item on her diet was a formula that is soy-based and cream. After the first evening of gulping down the two ingredients, she was not happy to say the least. She had terrible tummy problems ( I don't want to elaborate on them too much- just know they were BAD) and her seizures came back full force. She wouldn't take down her medicine, and her blood sugar levels were extremely low. John and I were very scared and we didn't know what to do with ourselves. It was very hard to see her so miserable and not be able to do anything about it.

By the end of the 3rd day, she had the color back in her face and she was eating well. What we did was take the soy and milk completely out of the equation. It wasn't necesary to have the formula anyway- it was just going to be a little bit easier to have it around for trips and late-night feedings.

We were discharged on Friday with a clean bill of health. Jordyn is loving her diet which consists of meals like Gerber Beef & Gravy, Gerber Pears, and Micro-Lipids (a fat solution that takes the place of cream) and other fun foods like diet Kool-Aid and avacados. Everything has to be weighed to the tenth of a gram and served at specific times of the day, but I've got the hang of it. It's not a hard diet, but it is tedious.

Jordyn has not had any seizures or spasms, and has actually seemed to be in a better mood. She'll track objects a little longer and she'll even look straight into your eyes for more than a few seconds. The doctor says we can start weaning her off of her seizure medicines within the next few weeks! I know her sweet, smiling face will emerge shortly after that begins!

We still have to check her blood sugar levels here at the house with a One-Touch Ultra, and we check her Ketone (the body's natural enzyme that will let us know she's still ketonic) levels to make sure they are good and high.

All in all, after the first couple of days, this diet is going really well! I will post another update in a few weeks and I will let you know.

Kristen, John, and Jordyn

Ketogenic Diet - next treatment!

Jordyn has been on quite a few medicines (not to mention one that involves a shot in her thigh daily) and none have really stopped or decreased the seizures/spasms to our liking. Some people don't mind their child having one or two seizures a day as long as the child is happy and flourishing, and believe me, that'd be GREAT for us. But in Jordyn's case, she is having about 3-5 spasms a day WITH heavy lethargy and a scewed sleep schedule because of the current medicines. She has been on a new medicine from Canada, but it has only helped a tiny bit, even though it is also paired up with another medicine, Zonegran, which has worked in the past but not anymore.
When children have hard-to-manage seizures, the next mainstream step to take after medicines have failed is Brain Surgery. We were going to go that route until John and I saw something so interesting on TV late one night.
We saw a little boy having a Grand Mal seizure on a movie while flipping channels. Those are the kind that throw your body into harsh jerks (also called Drop Seizures). The boy tried every medicine available at that time to no prevail. Then the mom (played by Meryl Streep) went to the library (pre-internet era LOL) and schooled herself on this special diet that had been around for decades, made specifically for seizures. (The movie is called First, Do No Harm)... The doc's didn't want them to try this treatment because "Gee, no money to the hospital OR medicine companies" But she took her son to Johns-Hopkins Hospital where they first did the diet and lo and behold, the seizures shortly STOPPED and the medicines were STOPPED.
The Ketogenic Diet involves foods and liquids that are high in FAT, low in proteins and carbs. Jodyn will ingest about 4x as many fat grams as protein and carbs combined. She won't gain weight because her body will use that fat and not store it. Her body will produce little enzymes called Ketones that we'll check everyday that let us know her levels of glucose and such are normal. One tiny mistake on this diet could throw everything off and EVERYTHING has to be measured on a gram scale to the tenth decimal place, and EVERYTING has to be strict as far as what kind of food, at what gram, and what time of day. WHEW! (we've been through more tedious things... )
Here's the incredible part, though- She will only be on the diet for 2 years, then if the diet has shown great improvement, she will be able to eat regular foods and be medicine and seizure free for the rest of her LIFE. Sounds too good to be true, but it is not unheard of or atypical. The success rate of this diet is incredible. Even if it just decreases her seizures by 50%, we can take her off the medicines so her quality of life is higher.
Also, she is young enough that she won't sneak off to the pantry and eat a cookie or take candy from a friend. We will be in complete control. There's also a special made formula for the diet called Keto-Cal. Since Jordyn has not fully mastered the art of chewing-and-swallowing, we will keep her on this formula for a good year or so.

Jordyn is scheduled to start the diet at Brackenridge Children's Hospital on September 11th. She will stay a few days so they can moniter her levels and make sure she doesn't get dehydrated. They used to have to starve the kids first, to get their Ketones up and running, but they do NOT do that anymore at this hospital. Jordyn will ease into the high fatty stuff for an easier, less invasive transition.
It will take about a month after we start the diet to fine-tune everything to make sure her intake of the foods is comparible to her level of activity and weight. We hope that by mid October, we will be pretty comfortable with the change, and that her seizures will be decreased (or stopped!)

We are so optimistic about this new venture, but as always, we are "Preparing for the worst, but hoping for the best"

Visit the link above to read a little more about this diet. I have a few more links below:

Matthew's Friends:
http://www.matthewsfriends.org/intro.asp

our-kids:
http://www.our-kids.org/Archives/Ketogenic_diet_FAQ.html

Jammin' For Jordyn

John, himself, put together a Benefit Show for Jordyn that the Corpus Christi Caller Times newspaper dubbed "Jammin' for Jordyn". It was such a fun show! John is good friends with some talented performers who were gracious enough to come and perform in honor of Jordyn. We raised enough money to start her on her new treatments. We are very greatful to have such great family and friends to share the moment with us, and/or donate. We appreciate everyone's help, prayers, and thoughts.

The performers included Steve Trevino (featured on Comedy Central's Mind of Mencia), Eddie Ojeda (Twisted Sister's lead guitarist), comedian/rapper Chingo Bling, and of course, John's band Sonido Azul (comprised of Grammy award-winners from The Chris Perez Band). The event was MC'd by C101's Rex Gabriel.

John will try to put together an Austin benefit show when everyone isn't on national tours. I'll keep you posted!






Info table, with "Jordyn's Dreams" donation box, made by Kristen's dad


Kristen and Steve Trevino


John drumming


Eddie Ojeda and band, rocking out


John and Steve Trevino

  





Corpus Christi Caller Times newspaper full length article about the Benefit
http://www.caller.com/ccct/entertainment/article/
0,1641,CCCT_874_4725900,00.html

The Beginning to Now :: Written by MOM

My pregnancy was completely normal. Great, even. The birth wasn’t too bad, either although it seemed bad at the time, LOL. Jordyn Terra Gomez was born June 16th, 2005 at 5 lbs., 15 oz. and 19.5 inches long. She was even a whole week early, the sweetheart! The first features we noticed were her huge brown eyes and her funny little birthmark on her chest.
It wasn’t until we brought her home when we noticed the strange, small arm-jerking movements she’d have every once in a while. I called the hospital nurses and expressed my concern, but they told me it was “just hiccups” and to not worry so much. I even brought the weird movements up to her (now Ex-) pediatrician who also wrote it off as “nothing”. He said he’d schedule her an EEG test (aka Brain-Wave-a-gram) for my piece of mind, but he dropped the ball and never scheduled it with the hospital for that week like he said he would.
When Jordyn was 12 days old, we visited my sister Dena’s house. By this time, her “hiccups” had increased from one a day to six. After Dena saw her have an episode, she told us we should go to the Children’s Hospital ER, so we did. We were not prepared at all for what was about to unfold.
Once at the Hospital, the doctors immediately rushed her to a room and took her stats and blood levels. We were quickly admitted and over the next 24 hours, they ran a whirl-wind of tests, including the long-awaited EEG, MRI, Lumbar Puncture, X-Rays, everything they could. Jordyn’s room was top-priority for the nurses for the next week and a half.
Our newly appointed Neurologist, Dr. Kane, gave us the horrible, complicated news about our 2 week old daughter:
“Jordyn may have a rare neurological disorder called Aicardi Syndrome. She has Agenesis of the Corpus Callosum (“ACC”; the absence of the bridge that connects the left and right hemispheres of the brain), Cysts in the left temporal lobe, and Infantile Spasms (seizures). We are having an Ophthalmologist come in to confirm it, because you have to have retinal lesions to be fully diagnosed with it.”
You can imagine our disbelief and worry. As if that’s bad enough news, we also learned there’s only 500 cases WORLDWIDE and the disorder only occurs in females. There’s also a poor life expectancy.
The eye doctor didn’t find any lesions in her eyes (just bad vision), but there are a few other cases of Aicardi patients that involve no lesions. Jordyn is the rarest of the rare.
Fast forward to now:
As far as life expectancy, her case is more promising than the typical one. Most Aicardi girls need feeding tubes and are extremely non-responsive to anything. Jordyn was a great eater, whether it was breast, bottle, or spoon. Her development is severely delayed, but we think it’s mainly because of all the harsh medicines she’s had to have over the past year. She has 6 doctors, 4 therapists (OT, PT, ST, and vision teacher), and 2 very devoted parents who’ll do anything to help our Shabootie-Toots. In fact, John recently produced and directed a Benefit Show in her honor to raise money for her new medicine. (Read the blog about it!) The show really helped us start out the new treatment. It is not FDA approved, so it's pretty costly to order it from Canada. It’s worth her not having seizures! So far, so good! I’ll keep you updated.
Thank you so much for your thought and prayers!