Weaning Not What I Expected

I really wish that I could say that things have gone great since we started Raquel's medication wean at the end of June. I had very high hopes, which is why when Dr. Mower showed a bit of hesistance because he was leaving, I was pretty insistent that we begin the process, even if slightly. I've done so much with her diet wise. I've taken her out of school because it was triggering seizures. I so desperately want to see her off these anti-convulsants, but most importantly, I so desperately want to see her seizure free and able to experience things a "normal" child is able to do.

Prior to the initial wean, Raquel was taking 1,650mg/day of meds. Seizures were probably happening maybe once a month and they were the tonic ones, where her arms would go above her head and she'd get stiff. At this point, I felt that the seizures were being triggered by deviations in her diet - eating or drinking a forbidden item in error. Dr. Mower thought it was pretty coincidental as well, since I keep journals and document everything, so the weaning began.

One med was reduced slightly, so her new daily dose was 1,400mg/day. She did really well at first - seemed a lot more alert, focused, did well working with her teacher at home. We found her to be more attentive and she had the ability to stay on task for longer periods of time. Yay!!!

About 2 weeks after this reduction, she began to develop what is called "drop seizures", where she would just randomly fall for no apparent reason. It would happen while walking across the kitchen (she actually cut her cheek on the kitchen counter once), in the bath, playing in her room, sitting in her chair watching tv. It was just a random thing - and not a good one. These types were new to her, so of course I called Dr. Mower, who was in the process of relocating to Northern California.

We tweeked the meds again after the drop seizures started, reducing one medication and increasing another so she was now taking 1,500mg/day. Again, she did pretty well initially, but to see the effects of the med increases/decreases can take anywhere between 2 days and 2 weeks with her. It's just a waiting game - all you can do is hope for the best, document everything, and pray.

Around the first week in August, she continued to have drop seizures, although they became less frequent (maybe 5 a day instead of 10), but she now started developing "absence seizures", which are by far my favorite. With these, Raquel would be sitting and then just briefly tip over and check out for a few seconds. Again, not good when it happens in the bath but for Raquel, these are the least evasive to her and the easiest for me to deal with. I don't freak out. :) These are also referred to as staring spells where it kinda looks like she's daydreaming. Who knows? Maybe she is.

So meds were adjusted again in mid-August, just a week or so before Dr. Mower's last day, and I was pretty stressed at this point. We reduced one med more and increased the other, putting her at 1,100mg/day and just went from there. I did speak with him one more time before he left and asked what his next recommendation would be, new medication choices, etc. I felt I needed to have that conversation with him because he has treated Raquel for so long, ordered up all the tests, analyzed test results, etc. There are 2 additional medications he said he'd suggest being tried, but before doing that, he said I should take the Keto Diet to the next level and instead of keeping her on the Low-Glycemic Index Treatment - the lowest level of the diet - to get with the nutritionist again (GREAT) and to start weighing and calculating meals more.

Towards the end of August, seizures increased to the worst they've ever been. The absence seizures were gone, but the tonic ones came back in full force - daily, even multiple times a day - and she continued to have drop seizures in addition to those. Good times. So, I re-read my journals over and over again, and reviewed the doses and the doses in each wean process and after a lot of thinking and soul searching, decided to put her back where I probably should have left her in the first place - at the original med level of 1,650mg/day - until our new neurologist is assigned and I can get in touch with my forever missing-in-action Keto nutritionist.

She has been back at her full dose for almost 3 weeks. I wish the seizures were gone, but she is starting to have good days and bad days, instead of just really, really bad days. She no longer has absence seizures or drop seizures - the two I seemed to handle the best - but tonics are still occuring a lot more frequent than I'd like to see. They are no longer contributed to her being cold, stressed, tired - they are random now, but are becoming less frequent. We are at a point now where she is even going a couple days seizure free. It may take a while to get her back to where she was. I don't know. Hope and prayer, that's all I can say.

She has also started weekly physical therapy and I've learned a lot. We had her fitted for orthodics to wear in her shoes...






And she's doing a lot of exercises to strengthen her legs and overall low muscle tone, such as kicking blocks...



And stepping on squishy ball things, which not only helps with the leg strength, but also improves balance.





Next week weekly speech therapy should be starting up as well.

Welcome to Holland

This pretty much sums it up for those of you looking for a little insight in my world of special needs.  I think Holland is a challenging but rewarding place.

Welcome to Holland
~ Emily Perl Kingsley, 1987

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel.  It's like this...

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy.  You buy a bunch of guide books and make your wonderful plans. The Coliseum.  The Michelangelo David. The gondolas in Venice.  You may learn some handy phrases in Italian.  It's all very exciting.

After months of eager anticipation, the day finally arrives.  You pack your bags and off you go.  Several hours later, the plane lands.  The stewardess comes in and says, "Welcome to Holland."
"Holland?!?" you say.  "What do you mean Holland?  I signed up for Italy! I'm supposed to be in Italy.  All my life I've dreamed of going to Italy."

But there's been a change in the flight plan.  They've landed in Holland and there you must stay.  The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease.  It's just a different place.

So you must go out and buy new guide books.  And you must learn a whole new language.  And you will meet a whole new group of people you would never have met.  It's just a different place.  It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around... and you begine to notice that Holland has windmills...and Holland has tulips.  Holland even has Rrembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there.  And for the rest of your life, you will say "Yes, that's where I was supposed to go.  That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very, very significant loss.  But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things... about Holland.

So Far, So Good!

It's been over six weeks since we started Raquel's medication wean, and I have to say that we've noticed very encouraging changes.  I love my girl!!! :)

For starters, she's more present, more alert, seems happier, more playful.  No increase in seizures - but no decrease either - pretty much the same in that regard but I'm taking that as a positive sign since we're reducing meds.  Miss Crystal - her teacher - noticed over summer school that she was more attentive and able to stay on task for longer periods of time. Again, more great news!

On the downside ... she is very unstable on her feet.  Her balance at times is so bad that I need to walk behind her to make sure she doesn't get hurt.  But it kind of seems she's more alert and trying to move faster and her little low-tone legs can't keep up and she trips over herself.  I pretty much have to keep an eye on it and see if a pattern develops. We have no replacement for Dr. Mower at this point, so by the end of the month, I plan on talking to him again before he leaves and finding out what his med wean would be if all continues to go well, and what he'd like to do if an increase in seizure activity occurs.  Need to cover all bases here.

I have to say that I love my email support groups.  A lot of posts are anonymous so I don't know who or where they are coming from.  I was so touched the other night by an email post, and I have thought about this man ever since.  I guess it's okay if I copy some of his post since I can't ask him for permission:

"Having a special needs child is probably one of the most difficult and most rewarding things we will ever experience in our lives.  It is especially difficult when our children seem to be developing normally and our problems in life are no different than any other parent with a child/children of similar ages.  And then everything changes. Sometimes suddenly with a grand mal, and sometimes slowly with a hint of a drop, or a night time tremor, or a brief stare into the distance as if asleep and yet awake.  And then the nightmare begins!

Tonight my little girl is sleeping peacefully. My wife is chatting on the phone with a friend and laughing, and I'm typing this because I have a few peaceful moments to share and relate.  Moments like this are rare in our life as they are with most of you.  However, the moments have become a bit more common. Slowly but surely we are enjoying evenings with a little less stress, not quite so many trips to the ER, and not as many sleepless nights dealing with the side effects of the diet, nausea, acidosis, etc.

We all know the difficulties of dealing with a child with a seizure disorder. We all have dealt with the difficulties of dealing with all of the different meds and their side effects and we all are experiencing the side effects of the ketogenic diet. But many of us have or are beginning to see the benefits of the diet. What a miracle it is to see our children coming back to life, re-learning those things that they lost over a period of time due to the effects of frequent seizures and/or side effects of anti-seizure medications that all too often do more harm than good. 

So I'm sitting here tonight and for a moment I am able to count my blessings instead of the hardships. I am recalling the pleasant moments of the last two years and blocking out the frequent hospital visits, EEGs, trips to the labs, etc. etc. And for this brief moment tonight, it is all so clear to me just how much our little girl means to my wife and myself. And tonight I am saying a special prayer for all of our children as well as the parents and caregivers, that we can find the answers that will end the countless hours/days/months/years of pain, misery, and stress that this awful affliction adds to our lives..."

I don't exactly know what struck me so hard about this email, maybe just the pure rawness of it. For him getting peace from his wife being on the phone and laughing with a friend - instead of crying - spoke volumes to me. I don't remember when Mike has heard me laugh with a friend for a long time, and that must be hard for the man of the house dealing with things - like seizures, a special needs child - things that are completely out of their control.  He must feel as helpless to me as I feel to Raquel.  So maybe the next time Mike asks me what he can do to help, instead of saying my usual, "Nothing...", instead I should ask for a hug. :)

Have a great night everyone.  I plan on making the best of it!

6-Month Neuro Follow-Up Complete

Today we had our 6 month neurological follow-up for Raquel.  Dr. Mower was happy with how she was doing, happy about her "home hospital" academics and pending weekly physical therapy sessions.  He also put in the referral for another speech evaluation so we can get that therapy started as well. The more therapy at a young age the better.  I'm a big believer in that.

He also was pleased that the MAD (Modified Adkins Diet) I have her on has seemed to decrease her seizure activity.  I have literally eliminated all of the bad carbs/sugars that the Keto Nutritionist requested I do, as hard as that was and continues to be. But I honestly can say that I feel it has made a huge impact in Raquel's health. The last seizure she had, I truly feel was due to her inadvertently consuming some foods and drinks she wasn't supposed to have.  Dr. Mower felt that it wasn't a coincidence either and wants me to continue what I'm doing diet wise.

Then there was music to my ears:  Dr. Mower said we could start reducing medication.  I am so happy!!!  Of course it will be a gradual thing - only 1/2 a pill a day less right now (she currently takes two anti-convulsants totalling 4 1/2 pills) - and then we will see how she responds. Could be few days or a couple weeks to really tell if she can tolerate the reduced meds but if she can, and I think she can, we will continue the weaning process.  Just being to the point right now where we are even talking about this makes all the diet sacrifices and challenges well worth it.  

I do have one bit of sad news ... our treasured Dr. Mower is moving to Northern California so today was our last visit with him.  So bittersweet.  I can't even begin to describe my emotions.  Raquel has been under his care for three really difficult years. We've had countless visits, ER trips, hospital stays, tests, tests, and  more tests under his care. He has made himself so readily available to me - through phone calls, emails, hospital check ups, etc.  I always felt as though Raquel was his only patient, even though I knew that was never the case.  He is just that great of a doctor.  

He told me to keep being strong, to keep doing what I know is best for Raquel, and to stand up to anyone who thinks different.  By that he meant new doctors, therapists, teachers, school districts, etc., not to cave in and do what they say if I don't feel it's right for her.  I'll continue to make him proud. He's taught me so much, and I'll continue to grow from his guidance.

He also had really kind words to say to Gabby.  You have to realize that Gabby has maybe missed two or three appointments during this process as well, so he knows her more than most and has watched her grow and mature. He praised and praised her for what a great big sister she is, how compassionate she is, and to keep on doing that. She was teary all the way to the parking lot, but so was I.  When we got to the car, she put on her earphones and started listening to music.  After about 20 minutes, she stopped and said:  "Mom, I know we're really sad but it's okay.  We had him for 3 years and now other kids just need him more." And she is absolutely correct.

Introducing Dr. Andrew Mower.  I told him I was doing this so it's okay that I publish his picture.  Hats off to a truly amazing pediatric neurologist.  He will be genuinely missed.

I'm Baaack.....

It's obviously been a long time since my last post so I have lots to report - and pictures to share!!

Raquel continued to have "home hospital" for the remaining part of the school year.  She has made great progress working one-on-one with Miss Crystal, her kindergarten teacher.  She is getting stronger - using her legs more and developing some upper body strength. Her big sister Gabby continues to be a huge support - and often doesn't even give her a break from working.  Here they are at the table, in their pajamas, picking items to work with.  Gabby didn't give Raquel a chance to eat or drink anything before setting the table up this day.  Raquel literally woke up, started to come out of her bedroom, and Gabby redirected her right back in to "start the day out right".  Raquel loves having quality time with her sister and didn't put up a fuss.  This day, that is...



Crystal has been such a wonderful addition to our family.  She has not only helped and encouraged us all, she has also introduced me to a mother that uses the iPad program with her son. We now are playing around with a few of these applications.  On one of them, Gabby has taken pictures of Raquel's favorite items, then records sentences in her voice so when Raquel pushes the button, it will say, for instance, "I want some juice" or "I want to play with my sister."  We will have fun with this over the summer months.  This program is just another way to incorporate PECS, but with the touch screen, the response is immediate.  And I really love that Raquel gets to hear her sister's voice saying what she wishes she could say.

Now that summer is upon us and school is out, we did experience one big milestone:  kindergarten graduation.  I am so happy we were not only invited to the ceremony but also that we were able to attend.  Graduation was a little bittersweet.  I was so proud of Raquel and the accomplishments she's made and how far she's come.  But at the same time, I really wish she was up on that stage able to sing and dance with the others (the special needs classes were to the side). But the school made graduation really special, and I never felt like Raquel was isolated, just kind of there watching the others have fun.

Here is my graduate, in her cap and gown.



Here she is with Miss Crystal.  We are hoping she can continue in-home academics over the summer if the district allows it.  I should know about that this week.  If not, Crystal also teaches swim lessons, which is something I would really like Raquel to take, especially considering how much she loves water.




Another special event was running into our beloved Miss Sara, Raquel's preschool teacher, while shopping at the mall.  I've stayed in touch with her, but it was sure nice to see her in person.  I look forward to spending time at the beach with her and her beautiful family this summer.  She has a daughter Gabby's age, so they always have a lot of fun when we get together.



As far as the Keto Diet goes, well, that's still a work in progress.  I pretty much consider her on a Modified Adkins Diet at this point since her nutritionist continues to be missing in action.  In all honesty, the adjustments I've made to Raquel's diet - some were quite drastic - I feel are really working on the seizure front.  I've definitely noticed a decrease in them since we started this, and am feeling pretty optimistic.  Hopefully come June 27th, Dr. Mower will start a reduction to some degree in the meds so we can see how much of a role that is playing.  Often, Raquel appears to be so overly medicated it breaks my heart.  But at this point, I think I would prefer this to her having daily seizures where she can end up in ER again.

Here's me and my girl.  I love her so much.  I would have to say that 90% of her abusive behaviors towards me (the biting, head butting, scratching, hair pulling, etc.) are gone.  I am able to tell when she's getting aggitated and needs some quiet time, and I think that she is just maturing and outgrowing a lot of these behaviors.





Today we went to Children's for her physical therapy evaluation.  We were supposed to have this a couple months ago but it was rescheduled to today.  I absolutely love "The Castle", their new facility up in North County.  It's a pretty happy place.  As with anything I've experienced with Rady Children's Hospital, everyone was awesome, from the staff greeting you at the front door, to the specialists evaluating you, to the actual facility itself.  I was ready to jump into the ball pit!

Miss Lucy did our initial evaluation and could possibly be Raquel's therapist. I requested weekly sessions and looks like she will be getting that.  Now it's just a matter of them writing up her PT goals and fitting her into the schedule. One of the main goals is increasing her walking so I don't have to carry/lift her as much as I do now. Lucy said this was a huge red flag because she will continue to grow and get heavier, and I will ultimately get hurt trying to do what I've been doing.  Obviously the low-tone has to be addressed because Raquel just can't do all the physical things that people take for granted  but Lucy plans on implementing a few things to help get this going. The day Raquel can climb into the bath unassisted will be a wonderful day!

Here is Lucy explaining to Raquel what they were about to do...



And Raquel standing on top waiting to jump into the ball pit.

Crawling to Lucy.

Then taking a bit of a break. I thought she might fall asleep at this point, actually.  Her medication was starting to kick in about then.

I was very proud of my Rock Star today.  She worked  hard, but remained calm and happy.  I am really looking forward to getting the PT going and building more of her strength and endurance.

Orthopedic Evaluation Complete

Mike did it again - he couldn't stand watching me struggle to comb out Raquel's long mane after her bath. Sometimes she doesn't mind, sometimes she does. It's just hit or miss. This night, she wanted no part of it - at all - so he decided it was time to get out the scissors and do a little "trim". He ended up cutting off about 7 inches of her curly locks, which I, of course, saved and plan to donate.

I have to hand it him - he does a GREAT job cutting her hair!!!



And now she has bangs again. I'm told they are "in" now. :)



Regardless, she looks super cute, and her hair is so much easier to manage. In this house, I really need to choose my battles, and brushing her hair shouldn't be one of them.

Last week, Raquel met with an orthopedic doctor. We've always been concerned about her low muscle tone, and since she hasn't had the best track record at school and has missed the majority of her school therapy, her tone really isn't improving as much as we hoped. I do my best at home, but I'm not a physical therapist and just model what I've seen the PT's do with her over the years and hope it helps.

Besides the low muscle tone, she seems to have flat feet and we were wondering if she may need special shoes or even leg braces, which is why Dr. Mower gave us the ortho referral. He didn't particularly think it was anything urgent, but regardless, submitted the request.

As with all of the specialists we've seen, there wasn't a waiting time, we were immediately taken into the room and saw Dr. Mubarak within 5 minutes. We had to cuff up her pants so he could watch her walk first. Look how big she is!!



Then there was a physical exam.



The doctor was really cool. The specialists we've been fortunate enough to see have such an awesome bedside manner. The seriousness of why we are seeing them is never forgotten, but for us, these amazing doctors have a way of making light of the situation, in a very professional way. And Dr. Mubarak was no exception.

After the physical exam, he said that her spine looked great, but he wanted to do x-rays on her hips and legs. What? Her hips? I never once thought there would be something wrong with her hips, just that we needed to get her leg muscles stronger. Fortunately, the x-rays were done right down the hall, and Dr. Mubarak was waiting for us in the hallway before I could even take off my x-ray protective coat. He went to evaluate the x-rays and came back into our room with two thumbs up, saying everything looked perfect. YAY!!! No leg braces, no special shoes, we just need to keep doing what we are doing, that she has flat feet due to her low muscle tone. What a relief. He did say that muscles typically don't get stronger by having others work them for you, that she needs to use them herself. Slow and steady wins the race.

We have our physical therapy evaluation on Saturday, and I'm really looking forward to that appointment to get more input.

Needing More Clarification

I was hoping to start Raquel on the new meal plans this weekend. I spent the last few days after our follow-up appointment pretty much finishing off the stuff she can no longer have. Didn't want to throw it all out, and didn't have the heart to keep it in her eyesight. I'm happy to report that the vast majority of forbidden foods have been eliminated from our household.

As I sat down to start the meal preparations based on Vanessa's new instructions, I became even more confused. I get the part about her having 3 regular meals plus 2 snacks per day, and that I need to now weigh everything. But the numbers don't make sense to me. For each meal, she is supposed to have 8.25 g carbs, 16.5 g protein, and 25.75 g fat. Each snack should consist of 4.12 g carbs, 8.25 g protein, and 12.8 g fat.

Here is the menu for one of her snack choices:

31 g celery, raw
54 g cucumber, raw
39 g turkey breast, smoked & fat free
23 g cream cheese
5.5 g butter

WHAT? How does that equal what her snack meals should be? Am I reading into this too much?

I'll hopefully be able to reach Vanessa tomorrow for clarification. If not, I'll post the question on my Keto Support Group asking for help. I'll also be reading all the books I've purchased so far to see if they give me any direction. The Keto Cookbook I have does show their recipes listed this way as well, but most of them are based on a 4:1 ratio, not the 1:1 that Raquel is on.

I have such a headache right now...