Share each other's burdens, and in this way obey the law of Christ. Galatians 6:2

      It doesn't seem fair.

I've been following 3 month old baby Chaia's story. Today they did a PET scan, and it showed her heart is more damaged than they originally figured.

I want to ask "Why God?" But her parents have such a heavenly perspective. They count it as joy to go through these trials.

 I remember God's grace carrying us through during those early months of Riley's diagnosis.

But we weren't looking at such a serious, dire prognosis. 

My mama's heart is in pieces for this family. I cry throughout the day and fall to my knees begging God to work a miracle here. 

It's all too close for comfort. Watching their video's describing their days at the Cleveland Clinic filled with up's and down's, bring rushing back all the emotions that flooded me during our stay.

 The scariest days of my life.

Less than 10 months ago,

but they seem like years ago.

So much has happened since then!

God has been very good to us.

 But He's a good God, and no matter His plans for us, he will always be good and loving. 

Ben ( Chaia's dad) said something in one of his video clips about believing in the character of God.

 It has stuck with me.

I just pray that God will choose to let her live a full life and receive glory through the medical miracle she can be.

If there is one thing that I HATE in this world, it is children suffering.

I see God using Chaia's story to draw me closer to Him though. He is a rock. The only One you can count on in a world gone mad.

              God brought this verse to mind tonight

concerning Chaia...

"Now unto him that is able to do EXCEEDING ABUNDANTLY above all that we ask or think, according to the power that worketh in us,".. 

[Ephesians 3:20]

Count your blessings. I'll be counting mine...

                                           Goodnight

You can click on this one to stay up-to-date on Chaia!!!

http://mustardrevolution.blogspot.com/2011_10_01_archive.html

For God hath not given us the spirit of fear, but of power, and of love and of a sound mind.

Well, you know what they say.."no news is good news." But i have missed blogging. I think i'm going to wean myself off Facebook and spend more quality time writing and recording my life between here and my journal. What do you think?

It's 2am but my mind is full and my heart is heavy. I was introduced via blog to another little family with a very sick 3 month old daughter, Chaia. She has a very, very rare genetic disease with only 108 (?) known cases ever! She is staying at the Cleveland Clinic, 1 floor below the one they had us on in January and then in March. She is adorable! The disease has affected different areas of her body but the biggest concern is her heart. They will be doing a PET scan tomorrow (Thursday) at 12:30pm to determine if she'll need a heart transplant or if the cells will rejuvenate themselves and make her heart stronger. It is pretty serious..thus heartwrenching. But I can testify of their amazingly strong faith. I love how when God is putting you through trials of fire His grace is overwhelming. I don't know how else we could get through it. Anyways, PLEASE, PLEASE pray for this angelic sweetie tomorrow particularly at 12:30pm. We don't even want to entertain the thought of what could happen, and we know without a doubt that God is more than capable of healing and restoring. Pray with me please. You can follow the dad's blog at mustardrevolution.blogspot.com

   I'm not sure what it is. Perhaps it's hitting the one year mark; reminiscing about that time of year when 9 months of waiting and loving this fluttering, kicking, hiccuping snuggle-bug actually came to an end and she was born. The first time I laid my eyes on her and the moment I finally got to hold her. Nursing her, our eyes locked on each other. Without a doubt, thee best day of my whole life. Or maybe it's because we're getting closer to the winter months, that time of year where I had the worst day of my life. The day the dr's informed us of Riley's diagnosis with TSC. The overwhelming fear of the unknown and something harming my baby. Me feeling completely helpless to protect her. Here's the bottom line. I'm scared. I feel like it's been too calm for too long. I know that's a waste of time, worrying; fearing something that I don't have to deal with yet, but i can't help it. That's what i hate about TSC. There is no cure. There is no remission. Tumors could be forming as I write.

   Riley IS doing pretty well actually. She never did get those feeding tubes. The next visit, she made the leap into the 15 lbs and Galen and I felt as long as she's gaining, we're not doing an invasive surgery. She hasn't been weighed at a dr's office for awhile. But last time she was 16 lbs. Most girls are 20 lbs by 1 year. Not our Riley. I weighed her on the scale with me today and it looked like 16.5lbs. I'm avoiding her pediatrician because he is gung-ho feeding tubes. She is skinny. There's no doubt about that. But i don't think she looks unhealthy. Her new nickname is "skinny-minnie" :). People are still shocked to hear how old she is when looking at her. :(

  Now here's some super-duper news! Riley is STILL seizure-free. 6 months, baby!! We are tapering Keppra. But after that is done she will only be on one med, Topamax. I wish it didn't affect her appetite so much... :-/ I honestly think, no, know how good we've had it these past months and I'm afraid I've gotten so accustomed to this norm. Sick kid? What? Where? The other day, i just thought back to what her seizures looked like. The pain, the discomfort, the confusion which quickly led to a loss of the few skills she had at the time such as smiling, cooing, and waving her arms. My baby has come soooo far! She is 12 months old and wearing 12 months clothes [major growth spurt UP]. I still have to use a safety pin to keep her pants up. she is walking along the furniture...al-most walk-ing! She smiles her face off...seriously! The sweetest, most affectious giggles. {Peek-a-boo is the bomb!} She still has not said anything. She makes noises enough but not what they'd expect at her age. There's some concern in this area because of where one of the tubors is located. It may be affecting her speech. She has until 18 months before they'll introduce speech therapy. Her physical therapist is a darling-dear! Riley loves her. She now comes every other week because Riley is not really considered behind anymore. She's not advanced by any means but nothing concerning.

    Well that's about it folks! Galen's still working like the hard-working man he is and I am loving fall and the slower pace that comes with it! We will be remodeling a couple rooms upstairs and rearranging our house a little before winter. My interior decorating side is cyked about that! Oh, and I will post pics of Riley's 1st birthday party...hopefully tomorrow. Ok, must retire now. Remember to pray for Chaia please! Love you!

(Rachel, I owe you big time girl! Have not forgotten about you!)

The day we decided to get Riley a MIC-KEY Button

I'm not okay. I'm not okay. I can't smile this away. I can't be strong when every muscle in me feels weak. Don't get me wrong, I will be. I'm a mom. I have to be. But in this moment, all by myself with the laptop, closed off in our room, I will be weak and that's okay.
  This morning we drove our hour and a half drive to Cleveland to see a pediatric gastroenterology specialist to evaluate Riley. After months of pushing this visit off and doing my absolute best to plump up my baby on my own, we succumbed to the inevitable. Our name was finally called, and as we undressed her for her weight check that evil hope resurfaced. She felt heavier, right? Her face looked rounder, right? "No, you fool!" The scale mocked me. Riley weighs 14.8 lbs. She lost 5oz in two weeks. Nothing new there.
  The GI specialist came in a little while later and she didn't waste any time in discussing feeding tubes. The MIC-KEY button is our best option for Riley. She started crawling a couple weeks ago and since then, there's been no stopping her. She's into everything! Having the tubes stick out of her nose would just be asking for trouble. She had some more blood work today just to make sure there wouldn't be any other issues affecting her weight gain. We have a couple follow up visits before the surgery. We are putting it off until the end of August. Maybe the beginning of September. It's not a big emergency and we have a lot planned for August. It would be best if we were home and settled before she gets the button. You never know what might happen with something new like this.
 I should say that both Galen and I went in there pretty determined to hear them out, but not to budge and give in to the feeding tubes. But after the specialist showed us Riley's growth chart and how now not only was her weight being affected, but also her height and head circumference, we felt their alarm too. Galen and I asked one question right after the other, and although it will be a challenge and heart wrenching, we want what's best for our Riley. The Dr also stressed how important it is for children with neuro issues to have the best nutrition in the first two years of life. Riley hasn't been getting that. On average she eats 15 to 20 oz in a 24 hr period and that's just not enough for a growing, now crawling (almost walking!) baby.
 We will be seeing Riley's cardiologist this month on the 28th to have a follow up on the three tumors on her heart. While there, we will also be asking his clearance for this procedure considering she will be put under anesthesia. Riley will be getting her weight checked that same day.
 I'd like to leave you with a few happy reports. Let's see. Well, I'm not sure this is a good thing but it's sweet for me. Riley has turned into this big mama's girl! Now that she crawls, she follows me everywhere! When I took a bath tonight, she sat outside the door! She is still doing great at her physical therapy each week. Cathy (PT) is so impressed. Riley is standing near the couch and it looks like walking is her main goal right now! I will admit, she has accumulated far too many bumps and bruises for my liking with all this "trial and error" moving! But how else is she to learn and she wasn't meant to be in a plastic bubble! She's on the verge of talking, but nothing I can really call a word yet!
  I forgot to mention that after the GI specialist, we were seen by a dietitian to help us until she gets her tube. She gave us some great ideas to fatten her up including ice cream, pudding, cheese wiz on her puffs or frosting, extra butter in her veggies, extra sugar in her fruits, fruit pie filling, and avocados! She also increased her formula concentration a tad.
 Alright, major headache from crying, so I need to drink some water and get some sleep. I don't want to sound needy, but I could really use some more love and support through all this. Those closest to me know how badly I didn't want this. But I know I did my best and now we have to accept the best help they can give her to prevent other, more major issues. I can't help but feel that sometimes people like to hear the gory details, but once they get them, they're off sharing them instead of being there till the next post. Again, I'm strong because I need to be and I know for the most part I look like I've got it together but there are plenty of days where I'm overwhelmed all alone. Many of you are far away and I know you care. Your love and prayers ARE priceless. Again, I'm not trying to sound needy, I just know it's going to get harder from here on out and I would rather bare my heart than wallow in depression all alone. As always, much love from Galen, Riley and I!

As depression sets in and my desire to do anything productive fades away, I am left sitting here at the computer desk battling feelings of numbness and waves of emotion. At Riley's 9 month check up today, one of my worst fears I tried so hard to fight stared me in the face. As I laid my baby on the weight scale, the nurse asked what my guess was. I responded that I was hoping for 16 pounds but would be happy with 15 anything. She announced 14.13 and my heart dropped. I knew her pediatrician would not be pleased with this and I beat myself up for not trying harder to get her to eat more. As I put a fresh diaper on my underweight baby in our small exam room, I wondered what our next step would be.

A FEW DAYS LATER...

The appointment I was speaking of above was on Monday. This is Friday. So I am not in a state of shock anymore. In fact, today when the nurse called to inform me of the appointment they scheduled for us with the GI folks in Cleveland, I canceled it on the spot. After a lot of  consideration, we decided it was not an urgent need. Riley is thin, yes. But so far it has not impaired her development or her growth. She is a very tall little girl and her head circumference is growing like normal. Galen and I are going to be very pro-active with feeding Riley and expect to see a difference. Sometimes, it's hard when you have such an easy-going baby because she hardly fusses. Plus, her primary seizure med, Topamax, decreases hunger. So that's not doing us any favors in the weight gain area! We feel there are clear explanations to why she's not gaining weight. (Medications, genetics,etc.) Galen and I both see where we can step it up a notch and help her. The pediatrician is trying to head off problems before they happen, and I appreciate that, but they're not here yet. Riley is still seizure free {YAY!!!} and is crawling. The PT says she really is not behind developmentally. She's just not advanced. Anyways, there are more details but it is late (?) or super early depending how you look at 12:25am. Bottom line, I'm tired and need to get some zzz's.  I'll try to blog more! :(  I won't feel so overwhelmed and you will be filled in more. Even if some details are trite. Good night!



Riley and Mommy at Daddy's first truck pull!



Riley in her new sleeping position!



Two Perfectly Normals

Quick post because I love you guys and want to keep you informed..and I have dishes waiting for me in the kitchen! We went to the Cleveland Clinic Main Campus today (1.5 hour drive) for Riley's appointment with her Opthamologist (eye dr), Dr. Paul Rychwalski. We were instructed to have this follow up vision check up since Riley no longer takes Sabril (Vigabatrin) to see if her eye sight was affected. We were very happy to hear that her vision is perfect, like her first check up before starting the drug. Which ofcourse was a relief and what we had hoped especially because of her short time on it (less than 3 months.) So glad that's behind us and not something else we have to worry about at this time.
  Also, wanted to share with you the results of my genetic testing. I called today since it had been a couple weeks past their expected deadline to have the results and be able to tell me. I was so glad to hear that I am NOT a carrier of TSC! My blood work showed I was in "the normal range" whatever that means!. We're leaning to not getting Galen tested because of the rarity it would be for him and his sister to not show any signs of the disease. 33% of TSC patients get it from a parent while 66% is new genetic mutation. So it would appear that somehow while her genetic makeup was forming, the TSC 2 gene was mutated. After considering this, we've decided to have more children in the future and make more cute babies like Riley! Adoption is always an option though! Alright, well we'll see how her weight check goes tomorrow. Not too excited about it. I started her on fresh goats milk today. I want us to exhaust our options before feeding tubes! You'll here from me soon! Again, prayer's appreciated!

Discouraged, but not Defeated.

 I hate this disease. I HATE Tuberous Sclerosis Complex. I want to sit here and write a string of curse words to describe my frustrated feelings about this awful, merciless disease. But I won't. It will do no good. So I'll share with you; my amazing, always loving and encouraging friends, the latest on my sweet, little trooper of a daughter, Riley.
  Yesterday (Tues. May 10th) was Riley's weight check-up at her pediatrician's office. First, I need to tell you that Riley has been eating sooo well these last two weeks! Three jars of stage 2 baby food, plus three 4 oz bottles of the richer formula a day. I thought this was good. Galen and I both thought we saw a weight gain in her face. So imagine my SHOCK when I lay her butt-naked on the scale in that small dr's office room, watching the number's fluctuate for a few seconds, only to land on 14 lbs exactly!!! (She lost 1.5 oz's) "What?!"..I'm thinking. I was so tempted to ask the nurse if she was sure the scale wasn't broken or off. But instead I picked Riley up, put back on her pamper and took a seat in disbelief...and fear. The nurse cataloged the pathetic number in their computer and left me in a quandary. What was I doing wrong?! This was the most I had seen her ever eat. I was sure the baby food had more calories than the milk and she really was enjoying it. Hmm. Well, our very attentive pediatrician, finally made an appearance 20 minutes later. As he took a seat, he informed me that he had taken the time to talk to the visiting pediatric neurologist that visited the office that day. Asking him if there was anything related to TSC that could affect Riley's weight gain. He did say that if one of her tumors was close to her hypothalamus (the part of the brain that controls growth and development) it may be a cause for her lack of real growth. So after viewing her number's and seeing that not only had she not gained, or even stayed the same but LOST weight, he proceeded to tell me my limited options. She can come in at night and get a feeding tube put down her nose and into her stomach and get those extra calories throughout the night. Or she can have a surgery where they insert a feeding tube into her stomach. Um, NO WAY JOSE! Not a freakin' chance!!! So yes, I have one week to get her to gain something; anything! Even an ounce would be promising! Or else we will need to try one of these more drastic options.
  So yup, that's about it. Oh, and Dr. Strong (pediatrician) did some research and amazing, crazy math skills and configured that Riley's calorie intake in a day is only coming out at 400 something calories when ideally she needs to be consuming 900 something. So baby food is out of the picture until we get her to the weight needed for surgery. The way he has me prepare her formula is almost double the regular dosing, so it makes it so much more nutritious and fattening. I need to go from 12 oz's a day to 28! Big jump. It doesn't help that she won't take more than 4 oz's at a sitting, and boy have I tried! So we are aiming for six 5oz bottles in a 24 hour period. It is almost 8pm and she has had 13 today. So if she doesn't consume our goal 28oz during the day this mama is setting an alarm for night time feedings. I'm going to do everything I can. Then if it comes down to those nightmare options, I'll know I did all I could do.
  Next Tuesday (May 17th) is our deadline. It's not long at all, so please join me in praying for our peanut! That brain surgery HAS to happen, and July is right around the corner. FYI, Riley weighed more at the end of January when she was first diagnosed with TSC at the Cleveland Clinic. At 14.8 lbs. I won't lie to you, this is getting harder. On both Galen and I. Last night we confessed our arising fear. Losing her. We never would have imagined we'd be facing this issue with her health, and that's what I hate about TSC. You  just never know. I'm trying to have faith and trust God. I know He would never allow us to bear too much. But I fear He's going to push me to my limits. But alas, He is a loving God, and that is one thing that I've never taken into question through all this. I love Him. How could I not when He's given me this darling little miracle who gives me so much joy and astounds all of us with her fast learning capabilities. (Totally impressing her physical therapist, Audra, these last couple weeks!) Again, loved ones, we CAN NOT thank you enough for all the prayers you send up and love you give us. We love you! I'll keep you updated.   

Comments!

I just quickly wanted to inform everyone that I changed my comment settings. Anyone can leave a comment now and you won't have to jump through hoops! So now, I expect to see some comments!!!

UP's and down's....

I am so embarrassed!!! As much as I do love to write, it is apparent blogging is NOT my strong suit! Forgive me followers! I started this blog soon before Riley's orignal surgery date. I wanted to make sure I had a nice, comforting outlet for sharing my heart and keeping loved ones updated on Riley's status. Riley hasn't had any major medical changes so I haven't needed this outlet very much yet...and if I'm honest, there are many days I don't want to be reminded what we're faced with, with Riley's health.
  But it has gone long enough without an update, so here I am! Galen is hard at work..at work! and Riley is taking a much needed nap snuggled under her baby quilt her Auntie Rachel made her (her eyes close so fast when the silky side touches her chubby cheeks!) So yes, MOMMY TIME!!! 
  After our visit with Riley's Cleveland Clinic surgeon, Dr. Bingamen, on April 5th, at which time her brain surgery was post-poned, we have enjoyed the somewhat laid back, normal lives we lead. That weekend Riley and I came down with a nasty head cold. Mine lasted a week but hers held on longer and a small raspiness is still lodged in her throat. We did end up taking her to a pediatrician just because we weren't sure if it could be something more and she was having trouble eating.
Continued Friday, April 29, 2011
 Sorry about that interruption! Galen came home and whisked baby and I away for a little bit! I'm going to try my best to get a post up tonight (as long as hubby helps like he said! :) Ok, so where was I? Her pediatrician appointment when she was sick. So we left the dr's office relieved to hear that Riley's lungs were completely clear and it was just a typical, pesky cold she would have to naturally get over. Which she did sometime last weekend! It's nice to have her "healthy" again! One less thing we have to worry about! 
 While there, Dr. Strong checked Riley's growth chart. He was quite upset to see that Riley hadn't gained weight in almost 3 months. He instructed us on how to make her feedings more fattening. We add an extra half a scoop per scoop of formula. So for a 2 oz bottle where you would add 1 scoop of formula, we now add 1.5; for a 4oz bottle, 3 scoops and so on! Then we were instructed to return in two weeks for a weight check. That appointment was this past Tuesday. Not a good day, I can tell you! Galen asked if I could handle it alone. I assured him it wouldn't be a problem since it was just a little pediatrician appt. Ugh, I don't like the unexpected! Especially when I'm alone! ( I'll post what I shared on my Facebook that day..I'm sorry if this is "cheating" as a blogger but I really don't have a lot of extra time!)
....

And we go from bad to worse!

by Kamzin Faith Hostetler on Tuesday, April 26, 2011 at 2:13pm

Riley's follow-up pediatrician appointment, this afternoon, did not go well. Her dr is monitoring her weight and wanted to see her today after changing Riley's diet around a little, two weeks ago. We were both very disappointed when the scale weighed her in at 14lbs 1oz. She lost 8oz since our last visit. Granted she's been sick but he said he at least wanted her to remain the same weight even if she didn't gain any. It doesn't sound like that big of a deal, right? WRONG! She is going on her fourth month of not gaining. She has been in the 14 pounds for three months. Now her pediatrician is very concerned. There could be a few problems he's checking into, none of which I remember their fancy medical term. One has to do with her kidneys not functioning properly. They could be washing everything out including proteins to help her gain fat. There is a medication for that. Her thyroid might not be working well either. So poor, lil Riley had to have a catheter put in so they could draw some urine and then she had blood work drawn at the lab. We will know the results of those today. Ok here's the term (I looked it up)- Renal Tubular Acidosis. So by the end of today we will know if she has this condition and she'll get prescribed a medication for it. If the tests do not come back pointing towards this, we'll have to check her thyroid. Then towards the end he said two words I never thought I'd here- "feeding tube". I just cried. How much worse can this get?! I can't even honestly answer that, because I know this is just the beginning and it scares me to death. I feel like with each dr's appointment filled with negatives prognosises my worst fear is coming too close for comfort....losing her for good! Please pray for us, especially that Riley will EAT and gain lots of weight. You see, we're coming to a standstill. She NEEDS the brain surgery but they can't operate on her till she gains weight so something needs to change and we don't have a lot of time to stand around hoping they will on their own. My mama's heart is overwhelmed. It is soooo painful to watch your baby who you love more than anything, go through this.

That was my morning. Early that evening I did receive the call from her pediatrician's office with good news that Riley does NOT have RTA! YAY! We have another weight check in two weeks; May 10th. Come on fat rolls!!! Riley and I have the opposite problem with weight. I would gladly help her out by giving her some of mine, but, well, that's just not possible! So we'll work hard on helping her to gain while I do my best to shed my winter (just go with me here!) flubber!  I am happy to report that her appetite has increased the last couple days. Our goal is to see at least 15 lbs on the scale at our next appointment! Please pray with us!
 I would like to thank you all for your faithful prayers and support for Riley and our family. I'm telling you, they are felt and seen in our everyday lives! I stop sometimes when I feel we have a victory with Riley or I have that extra patience and endurance in that trying moment, and just KNOW God is answering your prayers. I love not feeling alone. But I have this huge amount of gratefulness to this one particular friend, her family and her church! Every time I talk to my dearest friend Rachel, she asks about our Riley, shares how they're praying and also tells me about her amazing church who continually ask for updates and who really, really cares for our Sweetie! I cannot tell you how this blesses me. Sometimes, it's hard for me to understand how folks who don't even know us could care so much about our trials. But I am overwhelmed with your love and thank God for you! So thank you so very much Rachel's pastor and church!!!!! 
  Riley ended Sabril (Vigabatrin) on Monday (April 25th) after 3 months of taking it. We are glad to see this seizure medication in particular go because of the dangers of sight impairment. It has only been FDA approved for one or two years, I believe, because of these dangers. There are also only a select few special pharmacies in the US that carry it, so it had to be mailed to our house each month. The cost was over a whopping two grand for one month's supply! Anyways, one's gone but another has taken it's place. Riley is now on the very strong seizure med, Topamax. (So Riley is taking three seizure meds at the time.) Thankfully we haven't seen any crazy side effects with this new one. But we are still increasing it, and will continue until the full dosing in a couple weeks. Ugh! I guess this is our life..in this life anyway. I must be honest. Having a child with an incurable disease makes sin and the consequences very real. I've thought back to Adam and Eve a lot lately. That's when sin was introduced and all the ugly and evil of life with it. It's not just snakes and lamb-eating lions, but sickness and disease and thus suffering. I have a much more heavenly focused mindset now. This life stinks. I'm glad to be alive, yes, but it's like a pot-hole full of stagnant water. (That's the analogy that came to mind...and yes, our post winter roads need help bad!)  So, bottom line, having a sick child with a future unknown makes me look heavenward and focus on His promises.
 I'm going to skiddadle, head to the kitchen, snap my fingers and try to make the dirty supper dishes disappear!  Hostetler love to you all!   ~ Kam


Our Pretty Spring Flower!



Riley's Surgical Change of Plans (taken from my Facebook page)

 Well, I arrived home an hour ago from being out-and-about all day. After coming in the door, I immediately sat down at the computer. I typed up half my update but while trying to copy it, I hit a wrong button and POOF! It was gone! Grrrr! I believe that's happened to everyone one time or another, so I don't have to tell you how aggravating it is!

  Ok. So we arrived at our appointment scheduled for 2pm. They lead us to a room where the nurse practitioner working with the surgeon, filled us in on what they had planned for our Riley. We had made this decision without knowing too many details. Just that we didn't have many options out there and it was necessary if we wanted Riley to develop normally in these vital first years of her life. We were also given the green light by our very trusted pediatric neurologist. Saying that it was a good idea and the sooner the better. So anyways, I had set a date to have this consultation with her surgical team a couple weeks ago. While we were on the phone, I hastily set a date for surgery because there was a spot open and we didn't know how long the waiting list was. Now you can see how little we knew and why we were so shocked to hear what they planned for our peanut!

  Back to the nurse practitioner in the small exam room..so we're sitting there and she fills us in that they are planning a hemispherectomy! That is where they remove half the brain!!!! Yes, my jaw dropped too! Here's a link http://www.epilepsy.com/epilepsy/hemispherectomy  which explains it in one paragraph pretty closely to how it was explained to us. They did specify cutting the connections to the other side of her brain which is supposed to eliminate the seizures. The success rate is 80 to 90%. So after hearing how they were planning on doing this (which is, by the way, the most severe of all brain surgeries or so the NP said) all my fear and anxiety came back. After talking to a brain surgery veteran at church on Sunday, I was so calm and at peace. After this little talk? Not so much!

 Ok it is seriously late, and my little bunny just went to sleep so I have to wrap this up and do the same. Bottom line, she (NP) explained and answered our overabundance of questions as best she could. (Riley would be in the NICU 3 to 4 days. Then to a monitoring unit for another 7. She would lose her peripheral vision..maybe more eyesight and most likely never drive. Her left hand would always be a "helper hand".) She left the room, and Galen and I just looked at each other and sighed. A little while later the surgeon himself came in. He was nice enough. Asked a lot of questions and then shared something we didn't know. Riley is 14lbs 8oz. She has at most 450 cc's of blood coursing through her body which is about half a Pepsi bottle. He went on to explain, he would easily lose all that blood during the surgery. He's had 0 fatalities on his operating table and he wants to keep it that way..(yeah we're 200% with you there, Doc!) So in conclusion, she needs to weigh at LEAST 20 lbs. The more weight the better. They fear blood loss, but also a major temperature drop. There was no needing to convince us! We will live with seizures for now over not having her living at all. Major duh! But I was soooo thankful for their attentiveness and honestly! That's one big reason I love Cleveland Clinic. They give you the pros and cons, their opinion and ask you yours, but in the end it's not about numbers or money, it's about the patient!

 I also have not shared with you that Riley has been seizure free for 6 days!!! That's huge! Her last time was coming out of the hospital after her first stay in Jan. She was SF for 9 days. So lets see if we can beat that! They did just change her meds up a week ago which is why we're having this reprieve. But I'm going to be positive and relish it!!!
  So our plan with her surgical team is to hold off if posiible till July. Then instead of the hemispherectomy, start with something smaller. Simply removing that lesion on her right brain and see if that takes care of them for awhile. There's always the possibilty of more surgeries down the road. But due to Riley's age and weight we need to proceed with the utmost caution. So that about sums it up. If her seizures get out of control like January then we'll have no choice but to schedule a surgery and pray for the best. But we can thank God because at the time, they're not life-threatening and she doesn't spend a lot of time in the hospital.

 Alright this mama's signing off. I have that feeling I forgot something. Oh well ..ask questions if you have them, maybe I'll remember. So bottom line, it's not a "no" for surgery, just a "not now''. July will be here before we know it but in the meantime I will enjoy my daughter's huge smiles, giggles, and growing hair!!! Love, Love, LOVE you my dearies! Good night and as always THANK YOU!

A History Lesson

                             The Birth of our Pink Blessing
   It only seems fitting, since I'm starting something new with writing this blog about my daughter, her battle with TSC, and my heart as her mama, that I should start at the very beginning when this crazy, unplanned journey began. My beautiful daughter, Riley Elizabeth, was born September 24, 2010 at 10:24am after a long, complicated labor ending in a cesarean. My very first thought when I saw my baby girl was "Wow. She is BEAUTIFUL!" Not just because she was mine but because she actually was a very pretty baby and I was expecting the "wrinkled old man" look my dad had warned me about! My second thought and question was "Is she ok?" Meaning is she breathing? Does she have all ten fingers and toes? Is she fully developed? With my husband's  reassuring "Yes! She's perfect!!" response I allowed myself to relax and just bask in the pure joy of having my precious angel delivered safe and sound. Little did I know, as I laid there being stitched up while they whisked my baby away with my husband following, that she was far from ok. There, being hidden under vernix-covered baby skin,were three tumors on her heart and three tumors in her brain.                           

                                                What Was That?!

 One evening, as I was dressing Riley in her pajamas, she did this weird thing. As she was laying flat on our bed, she threw her arms out to her side, drew up her knees to her chest, tucked her chin and grunted as she stayed there for a few seconds. It happened again...and again. I took a video and sent it to my older sister asking her what she thought. She mentioned vertigo; which by definition means dizziness. It looked like she thought she was going to fall. We saw this strange behavior continued over the weeks. I remember being with my family in Connecticut over New Years, and watching her do this perplexing body movement while my mom was holding her. I remember my mom being concerned, saying she had never seen that before. Everyone I asked for an opinion told me not to worry; that she was still little and her nervous system was still developing.
                                        
                                  Our Wasted Night in the ER

    It finally came to a head at the end of January. She had just had her four month well child visit with her pediatrician. We mentioned it and even showed him a video. He showed no concern. The next day was Sunday, January 23rd. A day I will never forget. My poor Button cried the whole day and towards the end it was bloody screaming! Interspersed were these "convulsions" as I could only think to call them. Galen (my husband) and I were at our breaking point. We were snapping at each other and doing everything we could think of to calm her if only for a few minutes of reprieve. Finally I couldn't take it anymore and called her pediatrician even though we had just seen him the previous day. I spoke with the on-call nurse and she strongly suggested we go to an Emergency Room immediately. We didn't need any convincing and left for the ER. We waited maybe 45 minutes but for this poor mama ,who walked the corridor with her screaming infant, it seemed to be decade. Finally a nurse saw us...the check in nurse. Long, horrible, wasted evening short, our nurse told us she thought it was Colic and we should give her Colic Calm and close her in her room to cry. But the Dr's diagnosis was even more frustrating. After he watched the video we brought  along, he thought it to be acid reflux and the screaming? Well that's simple. "You don't feed her enough", he stated. Since I was breastfeeding and not able to give him the exact ounces she consumed, he figured she was still hungry. So after trying my best to express my concern and my unwavering mama's intuition that SOMETHING was wrong with my baby, with no give or real concern on his part, we went home. We left the hospital 5 hours later the same way we came in; with a screaming baby girl.

                                Trust Your Mama Gut!!!
   As we arrived home that night, I couldn't rest. I knew there was something wrong with Riley. I wanted to be wrong, but I couldn't shake this feeling in my gut. I sat at our computer and googled "infant spasms". I clicked on the YouTube link. As I watched the first video, I saw a little baby boy do the EXACT same thing Riley was. As i read the comments, I learned that it is called Infantile Spasms. I felt three things. #1 Relief to finally have a name for it and #2 Dread for the unknown ahead, feeling I had only uncovered the tip of the iceberg. And #3 Pride knowing that mama's do know best!
 
                           First Visit at Cleveland Clinic
The following day I made an appointment with one of the best pediatric neurologists in the world. We were able to see him the next day which still didn't feel soon enough, but at least help was around the corner.
Tuesday we met with Dr. Lacchwani and both my husband and I liked him immediately. He was soft spoken, attentive, gentle and very sweet to Riley. He asked us questions, gave his opinion, asked us ours and made us feel like we were not alone in this fear-filled journey anymore. What a relief!!! He watched our video, observed Riley and looked over her body with a Wood's Lamp which would show any lighter patches of skin which would indicate brain damage.She had one small patch on one leg and two on the other. At the end, he commended me saying "you were right on the money".  He then told us Riley was definitely having  Infantile Spasms but that lots more tests would need to be done to figure out why she was having them. She was immediately admitted into the Cleveland Clinic Hospital where we spent the next four days. They immediately put EEG leads on her head and monitored her through video surveillance 24 hours every day we were there. An MRI, EKG, Heart Echo, Kidney and Lung ultrasound were all done. After a misdiagnosis on Wednesday (from another Dr), we were given the hardest news of our lives on Thursday. We were informed she has TSC or Tuberous Sclerosis Complex. It is an incurable, genetic disease that causes tumors to grow on major organs like the brain, heart, lungs, kidneys, and skin. As I stated earlier they found three tumors on her brain. We were able to see the dark areas on her MRI scan indicating the tumors. Her Heart Echo revealed 3 tumors which they classify as Rhabdomyoma.. Which is a noncancerous tumor. Their normal MO is to shrink and disappear on their own. They said they were able to see signs that they have shrunk since birth. (Now it's pretty clear why our Punkin's heart rate kept dropping during labor! It couldn't take anymore strain!) So far all her other organs are clear. But that's what is so depressing about this disease. It likes to play peek-a-boo, so you're never really in the clear; there's no remission.
 We went home in shock. I struggled with depression while trying to wrap my head around all the info that had just been dumped on us. Talk about information overload! They started Riley on two strong seizure meds and she was seizure free for 9 whole days.
                                          
                                                  Here and Now
   It has been a little over two months since our first stay at Cleveland Clinic and we had one more in the beginning of March when they added a medication. But, unfortunately, our sweetie's body accepts the meds and makes the changes it's supposed to but only for a little while. Then her body becomes immune and we're back at square one. Which is where we are today. So that is why Riley will be having brain surgery in less than three weeks on April 21st. They will remove the biggest tumor where they have seen most of her seizure activity coming from. Then hopefully she will be seizure free. At least for awhile.
 Wow! I guess that sums up most everything. Those are the facts anyways. If I had shared all my emotions through these trying months, well, lets just say you would be sitting reading this super long post a little longer and you might feel the need to sit in front of a  shrink after! So there! If my faithful followers wanted a refresher or missed some of the history and if my new followers wanted to know what they were following, here you go! One of my friend's son also has TSC and I love this quote she shared the other day! This is how I feel, and the concept that has helped me get through this far!

If and when a horror turns up, you will be given grace to help you. I don't think one is usually given it in advance. "Give us our daily bread" (not an annuity for life) applies to spiritual gifts too; the daily support for the daily trial. Life has to be taken day by day, hour by hour.

-CS Lewis

Short, Sweet and off to Sleep

It is 1:30am. It only seemed fitting after a loooong day of getting this blog up and running that I should follow with my first post. It will be short and sweet because this mama is EXHAUSTED! My darling daughter had a rough day. Thus subsequently, I did too! Tuesday we were given instructions on changing her three seizure medication doses. Unfortunately, the one that makes her irritable was increased. So she was a fuss muffin all day. (It's amazing the funny pet names we parents come up with!) I tried e v e r y t h i n g! I'm trying not to get frusterated with her because I know it's not her fault. Regardless, it's still hard not to pull the hair out of your head when all you here all day is that cranky cry. The words "This too shall pass" helped me put the day in perspective. Alright, I will impress you with my lengthy writing skills some other night. Must get some shut-eye!