One Year Ago Today.....

One year ago today life was uncertain.

One year ago today we were scared of what was to come.

One year ago today it seemed never ending. 

One year ago today we were given a gift....that gift was hope.

One year ago today we thought our world was ending only to realize new beginnings.

One year later look at how amazingly far we've come.

This was Riley just over a year ago after a heart cath.

One year ago today what I had always prayed for, although most days thought was only a dream, became a reality..... Riley was no longer depended on her central line (large IV in her chest) and it was removed. She had been off TPN (IV nutrition) for around a week at that point. Although scarred to death of what was to come the joy of knowing my baby had done it was unlike anything I can describe. The complications from being TPN dependent for her were to great, it was something that HAD to happen. Being one year TPN free is absolutely amazing!! I want to celebrate it as if it was a birthday.... In a sense it was a new beginning.

Although this came about through unbelievable and unthinkable circumstances that should have never happened in the first place hindsight is 20/20. I can't be angry, or I'd never be able to move forward. I have to be thankful for the gift we were given. These "circumstances" I am speaking of I will talk about more another time. Even after a year it's still very hard to come to terms with and understand it all.

I miss my blogging though.... it has been over a year since I have done it. I want to start again. As always there's lots of things going on with the kids. I want for everyone who loves and cares for our family to know how we are. It is Mitochondrial Disease Awareness Week. This is something I felt I could do to bring more awareness to a disease that most people have never heard of even though it is as common as childhood cancer. To put our story out there helps to educate people. Slowly over time I will fill in the missing peices of the last year for everyone.

Liam is now in kindergarten!!! He is so excited and such a social butterfly, I truly love it. He wants so badly to impress everyone and tries so hard! He has been doing very well health wise. When we moved to SC last Dec. I kept him home with me until it was time for kindergarten. That helped him tremendously with not constantly catching everything and anything the preschoolers were passing around. Adjusting to school has been a little hard on him. He got a cold after the first week of school which lead to an exacerbation of his gastroparesis (the stomach emptying to slowly). The doctors always thought he probably had it to some degree due to his reflux ht. still  being such an issue and based on his testing. It has never been a major issue though. We are thinking it is just a flare-up though and we are waiting for it to pass. He has been very tired and is usually asleep by 5:30 on school nights but I know to some extent that is normal and hoping he will adapt to the new schedule. Over the summer he was diagnosed with ADHD. I don't think anyone who knows Liam was shocked by this though.  He is in OT now and I'm very excited that they are finally working on his sensory issues especially when it comes to eating. That battle is getting old! He is also playing soccer on Saturday mornings! He wasn't able to play the first few weeks because of feeling bad but has been able to participate the last 2wks. He has really been getting the hang of it and loves playing with the other boys his age. 

Riley is well Riley, truly amazing! She never lets anything get her down or keep her from doing things! But considering were she was and is now we feel truly blessed every single day. She still see's a lot of specialists. Her stomach was doing quite well since we did away with formula. She was coasting along with mostly "manageable" issues and we had even been able to only have to use her G-tube a few times until the beginning of July. The heat and not drinking enough made her stomach angry and it decided to go on  vacation for almost 7wks. So, to make a long story short we are having to use her G-tube to ensure she is staying hydrated and is able to eat during these episodes. She has a lot of issues with fatigue when she isn't getting her nutrition so it is something we have to be mindful of. She has had to restart her IVIG (an IV infusion she gets once a month) since her immune system had not started doing what it was supposed to on it's own yet like we'd hoped. Her heart is doing good! We have to have echo's yearly to make sure everything is ok and to watch the stent. She could have issues with the stent in her pulmonary artery as she grows but her new cardiologist is hopeful we will be able to catch anything that comes up early and deal with it. Developmentally she is doing well. She is extremely smart.... sometimes to smart, lol. Her speech is still a work in progress. When she turned  2 in March she was only saying a handful of words. She is making great progress although talking is still a lot of work for her. Her speech therapist sent her for a swallow study since she was still choking when drinking from an open cup and when her stomach is acting up she has periods where she wont swallow.anything. It showed mild dysphagia during the pharyngeal phase of swallowing with all things and there was some penetration when using an open cup. Luckily as long as we just keep using a sippy cup for and make her drinks really cold it isn't a big deal. So anyway, she goes to speech twice a week and during one session she receives OT as well. She also gets PT once a week due to her issues with falling a lot and balance and has developmental therapy with her early interventionist once a week at home. I'm sure there's more but I think that's all the big stuff.. 

Life is crazy and busy but it is our life. We are thankful everyday for what we have. I love my children more than anything in life! 

 

This is Today 

 

For Mitochondrial Disease Awareness Week educate yourself on this disease. Mito is called the "invisible disease" for a reason. Don't be ignorant to it and add to the problems these families face everyday.... Go  to MitoAction and educate yourself today!

Yes, I know it's been forever! We have had so much going on it's been unbelievable and things have been changing by the hour most days, lol.

A few things first... as most of you probably know Riley was in the hospital shortly after we came back from NC. She had yet another line infection. E-coli this time and it hit hard and fast. Thankfully it responded very well to the abx we gave and all was well. We were able to come home after only a week in the hospital. Thank God...

We did finally hear back from NC. Dr. Fortunado was able to call me personally to tell me the results. Bottom line functionally her stomach looks ok. Everything is there that needs to be for it to function basically. He thinks her whole gut dysfunction is due to her autonomic dysfunctions.... Which explains why she dumps one minute and then her gut completely shuts down the next. Any and every little stress on her system or even body in general causes her GI tract to go crazy. He said her autonomic testing that was done was abnormal and this was done when she was not having any "issues"... The only real issue I can't get a straight ensure on that doesn't really make sense is her formula intolerance. That's not an autonomic thing and no one really knows what to make of it or do about it as far as I can tell. Dr. F wants her to have the separate J but Dr. M(her GI here) doesn't. Dr. M wants something done with her stomach to help her be able to eat by mouth better and Dr. F doesn't think it will help her thinks we just need to pursue J feeds. Even then though we still have the huge issue of What To Feed Her! Which really is the MAIN problem. Until we figure that out none of the rest matters....from my point of view anyway. Dr. M and Dr. F need to talk and between both of their horrible schedules they have been unable to do so since we were there last month. This has caused us to still be waiting for "the plan"!

Although they haven't talked, Dr. M and I both feel strongly that we need to try something... He wanted me to try to start giving her small boluses of pedialyte every 4 to 6 hrs. After her IV abx were finished last week and she finally quit dumping she actually started drinking some on her own and doing great! So, on Saturday I tried to start some half strength RCF since this is the only thing she has ever tolerated before and this went along with our "lets do something" plan. Mind you don't forget all she has at the moment is a G-tube. I started giving her 60 cc's of the half strength formula 3x's a day. She didn't vomit or anything so that was good. By Sunday morning my Ry Ry had disappeared and this screaming child appeared. She was a hysterical mess all day. All she did was scream and throw herself on the floor all day. She was completely unable to sleep and even if she did she would wake up screaming... She also stopped pooping as soon as she was given the formula on Saturday. After the 2nd bolus on Sunday I told her nurse not to give her anymore. We let her belly rest all day Monday and even last night she was up screaming and the one goldfish she had eaten was still in her stomach 8hrs later. Today was the first day she was back to her smiley self. Although her autonomic issues were horrible last night. Her temp was in the 101's before bed and all night while sleeping her heart rate kept dropping in the 50's. So, I called to let the GI know today and we'll see what he says about it tomorrow. This was obviously a huge disappointment. But then again we just went down this road and spent 6mo. trying formula's and different feedings and that's what got us to NC.... So, this is why we NEED our "new plan"!!!

Liam is doing good. Everything finally healed from his sinus surgery. He is definitely able to breath better and can actually blow his nose!! So, we are happy we did it. He has still had 2 sinus infections that needed abx in the last 6+ wks but he has actually gone 2wks now without being sick so I'm thrilled and keeping my fingers crossed that it was all a fluke. He has also started OT now once a week and they recommended he start seeing PT too. So now he is getting both during school thankfully, each week. He also started VPK last week. My baby is a big boy now. I miss him.... They always grow up so fast! We shout heat soon about his IEP eval that is being scheduled.

There has been so many more things happen but this is enough for tonight... The rest I will get into slowly. Some of it I still can't even talk about yet. But I will when the time is right. As always thanks to everyone who has been there for us and who always pray for us!

At the End of the Day... Thankful

It's amazing how from the second you realize your eyes are actually open and it's a new day, the balls can already be coming at you at full speed and if you don't duck... well lets just say, You Better Duck! This is the kind of day it was here today. One thing being thrown at you after the next and you don't even have time to sit down and rationalize what just happened since it is yet again time to duck. You know I think that might be a blessing in disguise though. If you actually had to stop and think about each thing you probably wouldn't make it to the end of the day.

Somehow though at the end of the day (the very end...) there is peace, it is all ok. To know that there really are good and caring people in the world is what makes us continue on. Some days it feels hopeless, you don't know how you will make it through, but yet you ALWAYS do. For that I'm thankful....

I guess what I'm alluding to is all the drama that you deal with when having chronically ill children and leading the life we do. And the thing is it's not even the medical problems it's all the other daily drama you still have to face, only it's usually 10x worse because of your situation. But yet it's then even harder to deal with. This probably makes no sense and people who don't live in Holland probably don't understand, but it is what it is....

This day is just one example of why I feel my life is a blessing not a curse. At the end of our day, you are so tired. Even though today was a bad day, you have no fight left, but yet somehow in the end it there is peace. How is that possible??? I guess because to me, and my family, the things that used to be important just aren't anymore. All that matters is that we have each other, that we will remain strong, that we will continue to fight the fight even when everyone else deems it hopeless we will show them we are strong! We are not like every other family! We will win our fight and never let anyone tell us otherwise! I am thankful for everyone who has told us what we do is impossible because it makes us fight that much harder. To everyone who tells us we can't, watch out!

I am also so grateful for our amazingly relentless support for our community agencies without them we could not do this! So THANK YOU from the bottom of our hearts to CARES, Yellow Umbrella, & Life Paths. Without you we would not be the family we are today. We will finish the fight and not only win but better for it in the end!

Patience is a Virtue

Or so my mom would always tell me as a child. I wasn't patient then and I'm not much better now, lol. It's all over and now we wait...

Well we made it home thanks to all the wonderful people at Children's Flight of Hope! Our flight was wonderful and I have to say I have dealt with more agencies, charities, and organizations than I can begin to count since having Riley and I have never met a nicer group of people! They all went out of there way to help us and made sure every detail was taken care of so that I didn't have to stress over it. I think this is the first time I have ever traveled and not been completely exhausted just from dealing with all the hastles of the airports. Its just really not a place for Riley to be anyway... These pilots go out of there way to give there "free time" to give us this gift, and we truly appreciate it. Without them and many others we never would have been able to get Riley the care we are hoping and praying will save her life. Thank you to everyone out there who gives back...


She was able to complete her mystery testing today which we now know was a heart rate variability test. It's basically where they place a special monitor on you(3 lead), blood pressure cuff, and what looks like the equivalent of a weird pulse ox probe and you stay hooked up for five minutes meanwhile it's all going into a computer with a very special program that records specific things about your parasympathetic and sympathetic nervous system that will later be analyzed by the special "guy" who does the testing. Sounds easy right??? Well you try keeping a 16mo old wild girl still for 5min, and I mean almost completely... Especially a girl who is fascinated with any and all that is medical and we basically gave her new toys to play with. Let me just say we did it, even though it took all morning and we were finished 10min before we had to be down stairs to meet our ride to the airport. Thankfully "the guy" was extremely understanding and very willing to work around our issues and be accommodating. He said he will be spending all day today and tomorrow analyzing everything and will make sure Dr. Fortunato has the numbers on Monday. The neat thing about this test is they have never done it on a child Riley's age as I said yesterday. Come to find out the next to youngest they did was a 4 year old. So, the only bad thing is they have no real reference ranges per se since the normal ranges are based on what an 8 year old would be. It certainly seems like they are trying to establish this testing and I could easily see it becoming a huge thing! It reminds me of an EEG in a way, since it's like I told "the guy" I wish we would have had more time because we could have easily induced her autonomic issues by sleep deprivation and then they could have really gotten some good results. There's always next time...


Unfortunately, I was kind of disappointed we didn't see Dr. Fortunato again before we left. We didn't really see anyone after the test was taken off yesterday. I guess he will be getting with her GI Dr.'s in Orlando to come up with a plan. Which brings me to another infuriating issue... I called to make Riley an appt. with GI for when we get home to discuss some of the issue's we've been having and the trip since she hasn't seen them since May and she's supposed to be seen every 2wks. Come to find out her GI, D. Mehta is gone until Aug. 20, ok I did know that. But, our back up guy who is the ONLY other person I trust the management of her GI issues to is gone too! He wont be back until after July 29... WTH is that! How can they seriously both be out for so long... They said they could go ask if someone else would see her if she needed to be seen sooner but that is pointless to me since I don't want anyone else besides them managing her. So, I guess I'll deal with that issue on Monday. Oh yes and her pediatrician will be out again next week too. I really wish these dr.'s would get how important they are to us and that they can't just up and take off on us. Ohhh well, I'll deal with that on Monday.

We went and got Liam as soon as we got home. He was so happy to see us and we missed him so much. Thank you Denise!!! I love you for loving my wild man so much! He had a great week and was spoiled rotten as always by everyone there. So, now the easy part is over (HaHa) and now we just patiently wait, Or impatiently depending on how you look at it. We have a busy week but hopefully the weekend will be somewhat quiet even though we don't have a nurse due to circumstances beyond her control. Ohh, and that's another fun issues to deal with Monday the fact that they are wanting to drop ALL our nursing hours at the end of the month. I haven't even decided if it's worth fighting over yet or not.... Some times you just get tired of fighting. But I'm just going to enjoy my restful day tomorrow with my cute and wild children and try to be thankful for every single wonderful day I have with them!!!

At the Airport

Waiting to take off... Not sure what's up yet

Being Silly!

We're Home!!!

Thank You to Our Wonderful Pilots Drew & John!

Same Story, Different View.....

I started to write this out last night but then Riley decided to keep me busy until all hours of the night.... I'll get to that in a minute though. First off, We made it to NC!!!!!! Thanks to everyone who worked so hard with us to make this trip a reality. We couldn't have done it without you!

Our flight up was great. Children's Flight of Hope has been wonderful to us and we were able to quickly and easily get up here with no hassle what so ever. Let me tell you, small planes definitely aren't as bad as everyone makes them out to be. Having all the leg room you want and not being squeezed between two fat people is the best! What's priceless though is not having to drag our entire hospital and pharmacy we carry everywhere with us through security! I'm sure you can imagine how fun that is. I got to take anything I wanted and no special packing.Woo Hoo It's the little things in life that make me happy now a days.


The hotel we stayed in the first night was nice and Riley enjoyed roaming around somewhere new. The next day it was off to our business bright and early. We met Dr. Fortunato at his office for our initial appt. at 9am. He was everything we expected and more. He was very nice and patient and had a wonderful bedside manner. He listened to everything and had some good ideas. We then went to have all her Momometry testing started. They took her to the OR to do an endoscopy with biopsies and place the probe for the mamometry. Since she has a stoma they were able to use that to put the probe thru they also placed a small foley catheter beside it to use as a G-tube. Riley did great although he had some difficulty getting it to stay down straight. He laughed about it being his OCD, and something about sending it off (the probe) to have it fixed if it wasn't better next time. Little did he know.... LOL


The only problem we encountered was that the probe was not secured in place by anything except the tape we had used. Due to a lot of pressure in her belly she kept leaking out around the stoma and popping the cap out of the end of the foley. The machine was next to the bed and hooked to the probe that was inserted in her belly. It reminded me of a EEG machine. It had like 6 different lines with wave forms on them. About an hour being hooked up her dressing was soaked and fell off before we noticed the probe managed to slip out some.Not to bad though. Around midnight it happened again but only worse. It seemed to slip out quite a bit this time.So, Dr. F got woke up at midnight..... That's when we got the whole, "I've never had this happen before...." thing. The other weird part was the stuff pouring out of her stomach was really gross, unlike anything we had seen before. Everyone seemed to agree that it really looked like poop, although we don't actually know what it was. It was thick and brown though. So, he had the resident push it back in and we hooked her g-tube up to drain. This helped to reduce all the pressure she had and we were able to keep the probe in for the rest of the study although it wasn't exactly in the right place but, we do think we have enough data to make this all worth while. We were also able to give her the meds to stimulate the gi tract and watch it and we got to feed her some at the very end and it captured everything.


Bottom line is.... We wont know anything officially until he reads the entire study since they do a lot of editing and that type of thing. Anytime she was moving it would jumble the test but they have ways to pick out the important pieces of info. Just from the prelimenary glance he took he was happy since he saw some movement in her stomach so AT LEAST it's work in some fashion. The problem is we think it's just not working together properly and this is what we are hoping to determine from this test, and why we came. Just from what he has seen so far, the history he has read on her, and what we have told him he did say, he is definitely AGAINST THE SURGERY! Omg, just hearing him say this made this entire trip worth coming for! It confirmed everything Wil and I knew was right and her GI at home too for that matter. Everything happens for a reason! If Riley wouldn't have gotten that horrible staph infection in May she would have had surgery two days after. Not to mention she also had the huge blood clot in her heart and I'm sure we can all imagine how bad that could have been. He confirmed though, he thinks her pylorus is just very dysfunctional, she has muscle/nerve issues and in his experience the more you cut the more you further damage the muscles and nerves. Not to mention the chances of making her dumping issues worse are quite significant and outweigh any possible benefits.


He DOES feel she should have a separate J-tube and we need to pursue J-feeds again as he feels this is her only hope of getting off TPN. The problem there in lies what are we going to feed her! I explained this was one of the biggest problems, she has never tolerated any of the 20+ formula's we have tried. So, ultimately this will be for him and Dr. Mehta to hash out. He will get all his testing results together and then track down Dr. M and together they will come up with our plan for managing her. I can not imagine any two doctors I would ever trust more than them handling her most precious issues though! I am very happy with our new "team" !!!


She is scheduled to have her last test tomorrow at 9am. It will be having to do with autonomic issues. This is the whole reason we had to wait a month to come! I don't know a lot about the test but I know it's amazingly luck that Autonomic issues are Dr. F's area of interest just like Dr. M's thing is formula intolerance's involving sugars. HaHa, everything happens for a reason. I will update everyone tomorrow after the testing.


We are to be at the airport tomorrow at 2pm for our flight. Then we will be home!!! Hopefully we will have some time to kill tomorrow before we leave and I can update more then. Until then enjoy these pictures!



Before take off....

....30min after take off!

Welcome to Brenner's Children's

That's MY GIRL!!! Already a Wifi addict!!!!



Riley's Stomach while doing jumping jacks. Check out
the computer screen,





Dancing in the Window after a much need bath!

Working very hard!

My ANGEL!!!! I LOVE YOU!

Going or Not Going.....???

Well, I know everyone has been going crazy asking me about the NC trip and if we are going or not. Well it looks like we ARE GOING!!! Ugghhh, this has really been a lot of work. I was surprised.... I mean taking Riley any where is never easy or quick much less 3 states away but we haven't even got to that part yet! The hospital in NC called to get all of her registration done over the phone. Apparently, they say there is no issues...??? I realized though they are just under the same impression we are that we are just waiting for final approval and normally Medicaid always back pays at least a month so I guess that's what they are going on. Bottom line we are going and if the "Medicaid Miracle" doesn't happen by Wed at 9am. All of the Dr.'s and Hospital bills will be my responsibility. But you know what I COULD CARE LESS! As long as my baby gets the care she needs the rest can always be worked out later. We really need this done, and it's not like the pyloraplasty will be her "cure all" by any stretch of the imagination. I'm really starting to get excited about the trip now that we know it's finally going to happen! And Thank you to Flights of HOPE for taking us there!!!!


Riley is continuing to swell up like Sponge Bob taking a bath... Every day you can see it get worse. Even over the last two days. As of Thursday she had gained 3lbs in less than 3 weeks. I'm almost scared to know what she's at now and just PRAYING we don't have any major issues pop up before we leave. It's getting to be around "that time" again, siggghhhh. We will be coming home Friday night and that next Tuesday we have an appt. with the surgeon to discuss everything that happened in NC and to schedule surgery. That means no rest in between basically since this surgery is going to be rough on her and not quick and easy by any means. But at least we are having things start to move forward once again. It was very frustrating to sit around and not really be trying to do anything.... Anyways, now I'm just rambling.


On other fronts Liam definitely seems to his happy wild self again thank goodness, I wasn't sure if I would survive all of that, LoL. So now on to packing and getting things together although I'm not sure exactly when since I'm working all weekend. Ohhh, well I'll find the time. Who needs sleep anyways :) Not Me!



Liam and I on his 4th Birthday





Trouble Maker! Trying to take apart her pulse-ox machine...

My Little Mermaid! She LOVES the water

Let Me Off the Rollercoaster Already....

You know those really annoying kiddy rides at the fair that you are forced to ride with your kids...? The one's that go around and around and up and down.... It feels like you've been stuck on there for an hour and leaves you thinking, "ohh great, they're being nice to my kid or something and giving them like 3 turns"... But in reality you've only been on for 5min. Ya, that's how my week feels. Endless up's and downs and there's no end in site.

I literally DRAGGED myself out of bed at 7am today after another fun filled night with Liam itching and screaming things at us. Thankfully the nurse was here and we managed to make it out the door and arrive only slightly late for her Doppler study of the right subclavian vein (the vein in the top of the chest that her broviac goes through) and her SVC. After having to wait to long I knew something was up... They didn't want to do it. They were scarred of her age and medical issues blah blah blah... same thing as always. Bottom line a heart is a heart, Big or small! After finally convincing him to do it, he finally told me he didn't like the pics he got of the SVC and knew something was there but couldn't tell what it was meaning broviac vs. clot type of thing. He didn't even want to use the pics since he didn't want it to get blown off and something missed. So we were scooted on our way but having wasted enough time to have missed Liam's 9:15 appt, Grrrr!

One home I was actually able to get some cleaning, laundry, and phone calls done since Liam and Wil were still fast asleep. Everything has been going along nicely since the other day for our trip. That is until my phone rings this afternoon and the kids CMS nurse was on the other end (some days I need to learn to quit answering that thing) to tell me he had canceled my appt in NC but that everything was submitted to Medicaid. Long angry story short, they aren't submitting for Medicaid to pay for our trip on the 14th per se they are just asking if we can go basically. They will pay for anything on of after the date they stamp the approval, which can take up to 6wks, no back paying. So, hoping it can be done in 4 days is a joke... So, I was beyond frustrated. They can bill us for the appt in his office most likely but I think the chances of them then wanting to run all these tests if they now insurance wont pay is slim to none. Medicaid doesn't pay much but it's a lot better than none.

Liam slept until 1:15pm today... Without waking once. I don't think he has ever done that in his entire life! Actually I know, this kid is awake between 6-7am everyday, no matter what! He did need it though. His fevers finally seem gone today! And the rash finally seems to be fading and not itching quite as bad. Thank God!!!! This was a rough one.

Riley's appt. with the pediatrician was this afternoon, her usual weekly appt. I was more disappointed over all the hard work everyone put into this trip being all for nothing than anything. As soon as we sat down she mentioned something about going. I had then had to tell her the bad news. Without missing a beat the first words out of her mouth were, "Well who can we call at medicaid, is it in Tallahassee?" "I'll start making phone calls and we can AT LEAST try to get this done". I truly don't know what I would do without her. Some days I feel like I am constantly trying to paddle a boat upstream. It feels hopeless at times and you just want to give up but she is always there to lend a hand in winning our race and I am SOOOO grateful for everything she has done for Riley. So, she was working on it before we even left the office and I told her to call me if she found anything out. So, now we wait....

On the Riley health front. She has gained 13oz. since last Thursday and her IJ's (neck veins) are slightly distended but not horrible. The right more so than the left. BUT otherwise she looks great! She is totally back to Riley this whole week constantly happy as can be and running around like crazy. That's been really nice. Her B/p was actually quite good today too better than normal. I had called the office this morning to tell her the Doppler was useless basically. So, she called the IR cardio and the cardio who both said call the other one and have them handle it. Basically, I know where they are coming from, they are both great Dr's but both know are options suck and are essentially to put her through the same thing as last time. Everyone knows how that turned out. So, for know we closely watch and wait and pray nothing bad happens while hoping it gets better. We have set her next IVIG up for Monday we have scooted it in after her cardiologists appt. where they will be checking an echo on her and her weight and such again. Monday is a BUSY day and we have to pack.

The only other thing for today was Liam's new behavior/play therapist came out tonight for the first time. She was really wonderful and energetic. She has A LOT of helpful resources and is the absolute perfect person for our "team". She seems to really understand the whole situation and thinks she can really help him without placing unrealistic expectations on the family given our situation. So, that is exciting for everyone since this has been a huge concern and she will even be helping us get other services coordinated for him and helping get through IEP and starting a new IEP type of plan for him at school.

Well I'm exhausted and the time has gone by to quick tonight. I'm hoping to get some sleep now that Liam seems to be getting a little back to his norm. Hopefully the ride has ended here, at least for tonight anyways! LOL

Finally, A Little Good News! I Think....

So, after many frustrating hours on the phone with multiple different people and the help of two truly wonderful Dr.'s (to whom I am yet again forever grateful for!) WE ARE GOING TO NC!!!! I am so happy! I would have just so hated to have waited all this time and made a ton of plans all for nothing. Just start praying now something useful comes of all this! Since as of this morning we thought the trip was off we have a lot of unfinished plans to hurry up and get together. Thankfully, most of the big thing were already mostly arranged like flights and someone to watch Liam (I love you Denise!!!) so now it's a matter of everything else like packing for Riley... No small task.

On to Liam. He woke up with a temp of 105 today. That was fun.... but I was finally able to get that down around noon and surprisingly they have actually been under control today. It wasn't until around 5pm he started going up and I just headed it off with meds. He was up the ENTIRE night last night however itching. His rash has just all run together now and all you see is a white streak here or there everything else is just red and swollen from the rash. Literally, it's even under his hair! I was talking to the pediatrician this afternoon about both the kids and when I told her about his itching and the max doses of benadryl still not even letting him be able to sleep, she quickly called in some Atarax syrup for him. I think all the meds together had him all wired and he missed his "window" earlier when he was sleepy and is still rolling around and now itching again next to me at 1am. He has yet to go to sleep... Thank goodness it's time for more meds!

I did hear from GI finally after spending the majority of my day trying to get a call back from their office or even someone to answer the phone! But after much debate we decided Riley could wait to see GI until we get back from NC. I also heard back from Hematology, they want a Doppler of her Right Subclavian and the SVC. This will help to check for clots so I'm just happy someone is doing something! Well since Cardio decided to take the wait and see approach anyway. She is still doing good and being exceptionally good and happy even though she does probably have a sinus infection. But I'm glad she's happy.

Liam is out there FREAKING out so I better get to him! I'll update again soon....

What a Week....

So to start the day off, Liam woke up like this today... We knew he had started getting a fever last night but this is how our day started. Not fun after we were up all night because of his 104 temp. I swear it's literally always something or somebody around here! The Dr. thinks it's a reaction to the Septra he was on or of course a virus is always a possibility. I'm much more inclined to think it's a sulfa reaction since I had a severe reaction to sulfa as a child and my mom also has a bad allergy to it. That is why I have never let them give it to Riley. We will see how he is tomorrow. She changed his abx since his throat is very red and we have had strep running crazy through the house. Along with some other sinus thing that Ry and I have. Hopefully he will start to look slightly better by tomorrow. His fever was over 101 and usually 102 even with Tylenol and Motrin.

Anyway, we have had a lot of appt. and things since the last time I updated. Riley saw the hematologist, pulmonologist, opthamologist, and of course the pediatrician last week. The eye doctor said she looked great and he doesn't need to see her for 6mo. !!!! Wooo Hooo, always nice to have good news. The hematologist appt. wasn't very informative except we realized Riley has gained 1 1/2 lbs. in about a week and a half. This is worrisome due to the fact that it's mostly swelling and we knew the first sign of any issues with her SVC would be swelling in the top half of her body. He couldn't find any of her records from the visit for some reason and since he wasn't the Dr. who saw her in the hospital he didn't know any details except what I told him. He was going to try to find out if there was any testing we could do to check this but I never heard back from them and this was last Tuesday. We had a good appt with the pulminologist. He said she has had no apnea on her monitor in awhile so we can start trying to wean her caffeine again. He did agree with me that the apnea alarms she had been having were probably just due to hypoventilation rather than true apnea. So, once we have the caffeine weaned in a 2 or 3mo. we will repeat her sleep study. The pediatrician appt went good we checked her Lovenox level although I haven't heard yet how it was and did a blood cx just to be safe since she had a small temp in the office. We are sure though it is just from the obvious cold she has although you always have to be safe. She was also concerned with the weight gain. She has been getting the same amount of fluids and the same TPN formula for several months now so it's most likely not do to that. To make a long and very aggravating story short the final word from the cardiologist was that there is really nothing they can do right now unless they sedate her for an echo which even then isn't always accurate as we now know, so the only way of knowing for sure would be to do another cardiac cath which no one wants to do so soon. So, basically we have to wait until it's bad enough to be causing obvious major issues again.... GREAT! Especially since those like to pop up at the best times.

I also got a call out of the blue from Dr. Mehta who was very unhappy. He heard that we were now having issues with getting the insurance approval to go to NC next week. If we don't get to go next week he is absolutely not willing to wait any longer and wants her to have surgery immediately. Due to the major life-threatening issues she has had over the time we have spent waiting for this appt. He is no longer willing to wait even one more day. I had to agree with him though. I also heard CMS is trying to say she doesn't even need to go and they don't see the point. They think she just needs to have the pyloraplasty done and there is no need to go there since we would still do the surgery once we come home anyway. So, basically they are the one's who have made it so we will not be able to go. Or at the very least it's going to take a miracle for sure! This is also the same agency who had ABSOLUTELY no idea what all of Riley's medical problems really are and have no updated records since she was maybe 3mo. old. Lets just say they thought she only saw 3 specialists...... Trust me you don't even want to get me started on them!

Liam finally had his surgery on Thursday after all the time we waited. Everything went very well. He was able to remove the tube and patch the ear and re-tube it. I think we later found out the problem, He could have sworn he remembered telling us not to use drops in that ear after the first surgery since it was patched but I don't remember him saying that. It obviously isn't the point at least now we know why it probably happened. The ear drops they have you use will essentially "wash" the patch away before it has had time to adequately adhere to the ear canal. So, NO swimming for him until he is re-checked, lol. His nose looked good though, at least the Septra did something good for us! Everything was still open except for one of his maxillary sinuses so he re-opened it and then cleaned everything out. He did say there was still quite a bit of swelling in there so that is probably why he's having so many issues breathing still. Hopefully that will go down with some time.

Onto other things, my mom was able to come down for Liam's birthday which was on Saturday. She got in Thursday morning and the kids had a great time seeing her and we loved having her here too! Liam was able to have a wonderful b-day this year even with all the craziness. He loved thinking all the fireworks were for his b-day, lol! He LOVED all the attention and got spoiled rotten despite our poor nurse being sick that day and unable to come, Riley being sick, and the fact that it was to hot to go out and do anything. My mom left early on the 4th but I had to be at work anyway and besides it rained most of the day anyway so we weren't able to do much that night either.

For this week there is of course to many things to remember as usual. We are supposed to go to TPN clinic tomorrow since Dr. Mehta wanted her seen once I told him about the weight gain issues. The nurse never has called me back with an appt. though despite calling her and leaving a message at least once a day since I talked to him! We were also supposed to go and take pictures with Flashes of Hope since they were going to be at APH tomorrow. I guess we wont be getting to do that now since Liam is sick :( I am really upset about this, we had to cancel our spot last time they were here in March due to the kids having RSV... Guess we'll have to wait until they come next time. Liam has his 4yo check-up on Thursday and he'll also have his appt in the evening with his new behavior/play therapist. She is supposed to be wonderful and was very nice on the phone. I am really hoping she will be able to help us with some of the issues we have had lately. But I am just praying we have a nice quite week that involves no hospital visits. I think we all need it...

Here are a few pics from last week. My mom has all the pics from Liam's birthday and stuff. Once she sends them to me I'll post some of them.

Riley waiting patiently in pre-op,with one shoe on, for them to take

Liam. I think she was just glad it wasn't her, lol

Liam happily "drunk" on his Versed and Tylenol. At least

this time he didn't tell any nurses to take off their clothes!

Riley running around and being her wild cute self in the OR waiting room.

I wasn't exactly planning on doing this since I really wasn't in the mood but I need to... Everything is just starting to take it's toll on everybody around here. It's been a very long and hard 3weeks. Riley of course is the only one who is obliviously happy since she doesn't know any different, lol. If only we could all be so blissfully happy...

I can't even remember if I knew the date yet when I last blogged but Liam's surgery is scheduled for Wednesday. He will have the dysfunctional ear tube replaced and we are just all praying it hasn't managed to fall behind his eardrum in the last 2weeks. We have been worried Liam had a sinus infection since before we left the hospital. The green stuff coming put seemed to be getting worse but it was hard to tell since he still had a little packing and scabs in there and stuff. On Friday morning we noticed a rash on Liam's face that didn't seem like any "normal" allergic rash he gets. When nothing was helping I had Wil take him in to the pediatrician on Saturday morning. When I told her about it when I saw her that morning we had both been thinking viral.... NOPE she thought it was probably Staph once she saw it. Ugghhhh! It was in his nose and when I talked to his ENT today he said he's probably already had it colonized in his nose so this is no surprise. What a surprise, one of my kids has yet another complication! We started oral and topical antibiotics. Within 24hrs there was a huge difference so that's good at least. His ENT today said too, that while he's in on Wed. he'll scope his nose and do a really good washout and get all the junk out. He can also take a look and ease my mind that everything looks as it should in there. This was just a huge disappointment although I don't know why I didn't see it coming since Liam ALWAYS gets something big after any major "stress" on his body. He's always had some major bounce back issue after every procedure he's had.

Riley is actually doing very good for the second except for some pretty severe insomnia she has been having. Idk what all that is about but hopefully she's just getting a new tooth and that's all. The truth will show itself eventually. Always does...

We have an insane week coming up that is jam packed with excitement and a million appt and things to do. I'm tired just thinking about all the errands I have to run before 9am. Riley will also see the hematologist and pulminologist this week so we'll see what they have to say. We have a big CARES meeting on Wed and all of our other coordinating agencies will be hopefully coming. I am definitely hoping we make some good head way there. But I'll try and update each day this week since we have a lot going on and I'm already tired just thinking about it!