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!

Busy Week

Sorry, I been the worst blogger ever this week. Things have been SO busy around here I haven't even had a second to breath much less anything else. But busy in a good way I suppose... No major issues more of cleaning up all the mess of issues that happened or had to due with us being gone for weeks and then coming home and having my whole Friday lost to Socializing with DCF, ha ha!

Liam didn't have surgery on Tuesday. The surgery center called and set it all up on Monday only to call later that afternoon and change there minds and say they weren't comfortable handling Liam and he needed to be done in a hospital. Great now you decide this! Anyway after a lot of screaming between the Dr's office and the surgery center. His office called to say his next hospital day is Wednesday at Wuestoff... That aught to be a blast! A whole new hospital and a whole new set of issues. Whatever, at least it's close enough I could walk there. That's definitely one good thing.

Nothing else really huge or pressing right now just trying to get a lot of things ironed out and having a lot of meetings with people. I'll update on all that later. Not really anything extremely medical.

Riley has been very happy to be home as always! This extreme heat has really been taking a toll on both kids. She started having some autonomic issues start sneaking up last night. Boo hoo, everything had really been good otherwise. I noticed her pee smelled horrible last night when I was changing her at midnight. She had had plenty of fluids, she is still quite puffy and pale though... Today her autonomic stuff was a little worse, but both kids had a runny nose first thing this am. I was hoping they had just caught a cold but it kind of went away and neither kid seemed to have any more cold issues. I did cath her before bed and she had small leukocytes which sucks since she has been totally negative on the strips for the last few months. So who knows if she's still acting funny tomorrow we will take in a UA and culture to the Dr. She will let us know she always does! lol

I will give a better update tomorrow, I promise. I just wanted to make sure everyone knew we didn't fall off the face of the earth!

Constantly Compeating

Yes, that would be my children! Always trying outshine one another with who can cause me to stress out the most that week. Liam didn't used to be so bad but he is definetly making his presence known now a days!

So todays fun adventure was waking up today and bribing Wil that if he would dress Liam I would take him to school. So, he did and then Wil came back in the bedroom and I was getting dressed for the day. When I came out of my room Liam was no were to be found. I asked the nurse who was in the baby's room with her if she had seen him and she said no. She had just returned from taking the baby for a walk... She had thought he was in our room with us. When she left with the baby he was sitting on the front porch and she thought we were coming right out. Well once we realize this like I said it was 10min. tops so I look out the front and back door and see nothing and no one. I ran in to get Wil and we each went in different directions. After about 5min. of looking I turn around and see Wil walking downthe side walk in front of the apts holding him and there is a cop about 10ft behind him and the cop car driving down the street....! I live in a complex that is basically a big oval. Each building has like five townhouses attatched to each other. The main entrance is about 100'ish ft off Wickham Rd.(a major 4 lane road) and we are almost all the way in the back of the complex too. When the police found Liam he had gone all the way across Wickham Rd.

All I could do was sit there and go OMG!!! How could this have happened?! We have taught him about roads and he knows never to wonder away. My god we have NEVER even let him play outside with one of his friends unless we are out there. And he's not allowed to go out of our sight even then! I was just in utter shock and he was completely non phased by it all. Even when the cop was lecturing him he just said, yes sir... He told them he went across the street looking for his sissy. This is so unlike him! Idk, what I'm going to do with these children! lol Some days you can't do anything but laugh, that and be greatful once again for nothing happening to him.

So, needless to say DCF was knocking on my door within an hour... Doing a full investigation. Just what I felt like doing today. They went everywhere and talked to everyone to try to find out who to blame for the child neglectfull unsupervision of a minor and child endangerment, Seriously!!?? Omg, what a joke. Thankfully, after talking to us she saw it was just no ones fault and said not much should come of it. Still... Then within minutes of her leaving one of our caseworkers from Yellow Umbrella came by and we had a 3 or 4hr meeting. She has been wonderful and it's so nice of these agencies to reach out and help us even if we aren't the exact client they are ment to help. We still need someone to help us. So, after everything was said and done we have a lot of things to think about and choices to be made. We also have a lot to work on.

Riley has been fine today I'm assuming, lol. Things were so busy I didn't have a lot of time with her. She had a wonderful nurse today and i know she would have told me about anything important. I took her temp before bed and it was only 100.4 so that was awesome! Way better than yesterday. I have work in the morning so I better get to bed. Here's to really hoping we have a nice quiet and uneventful weekend.

Finally Home! Thankful....

Only had to spend 11 days in this trip so all in all not bad considering. We have at least managed to bring her once almost routine month long stays to a much more do able 2wk average. Although I must say this was definetly one of the more 'intense'(for lack of better word) stays given the time fame... But it truly was one time things could have gone very different and VERY wrong for many reasons but thankfully I know my baby must have someone watching out for her! It was truly touching in the mitst of everything to see SO MANY Dr.'s and other hospital staff so worried, upset, and determined to help her as I did the other day. All because it was happening to "Riley". Of course we all know they would have sprung into action if it would have been any child. But in such a large childrens hospital it is so amazing to see how much they truly care about her and how special she is to everyone when they see a hundred children just like her every day. We are very blessed to be right where we are!

I just wanted to give a quick update since it is late. Her temp were better today. Still having some autonomic issues but to me they weren't as persistent as yesterday. She did go up to 101.2 before bed tonight but we will see how it goes. She was ready and HAPPY to come home as were all of us. Given everything she has been through I am glad they were more causious. Now is not the time to wait and see... I remembered she needed her TPN labs drawn today too so we pulled them off when her TPN was done. I was glad I thought of it with all the temp issues. When I looked at it her Hemaglobin was 8.1, well hello! Hopefully this explains the autonomic issues! She doesn't handle being that anemic well so if it is only 8.1 now, 48hrs after surgery, I can only imagine what it was a few days ago. Especially considering they purposly drew an H&H the night before surgery and I was shocked and thrilled to hear she was 10.5 then... That's a little on the higher side of her norm. I should have know since she has dried up blood all matted in the back of her hair in PACU and it was all over her body. I had been hoping what was on her was bedadine but it actually washed off a little to easy the other day. I have no idea what they did that caused her toloose so much blood since no one spoke a word about it to me. It doesn't matter now though, I am just happy that hopefully this is what is stressing her body out and causing the autonomic issues rather than an infection.

Liam is being a trouble maker to now. He must have heard about Riley's escapaid the other day and desided he needed to try to get some more attention to. Wil took him for his follow up to the ENT's office today. Apparently something happened to his Left ear tube and it flipped all the way out and around and has almost fallen into his ear drum, Only my children! Of course I didn't even know it was possible but it needs to be fixed now. So, they have schedualed him to go back to surgery on Tuesday to have a new one put in. Hopefully it will be as simple as it sounds. Otherwise they said everything looks good. There is still a small amount of packing in his nose and they said he should be able to breath much better once it all comes out. So, that was good to heat. It's hisown fault since he wont use the saline spray it takes longer to fall out and heal as well.

But all in all I'm just glad to be home and a little tired, LOL :) What can you do but be thankful as I said things could easily be very different right now...

Day 10: Shato APH

Yes, still here... We had the best of hopes for going home today. Everything was going according to plans. But I noticed she was feeling pretty warm early and sure enough her temp was 100.6 and we desided to just watch it for awhile since we had to wait until after 4pm for the TPN to be delivered. It didn't seem to want to budge and held pretty steady around 100.6 and 100.7 the lowest it went all day was 100.2. It was 101.1 before bed. Whithin a few hours of going to sleep though it was down to 97.7R. We are all kind of scratching our heads...

She looked great as usual all morning definetly not showing the effects of the day she had yesterday. Riley is so amazing, she never lets anything stop her. I love that girls spirit and hope to have half as much strength as she has one day!

The Resident was all ready to send us out the door and wrote up the paper work. The nurse however had called the attending to tell him about her temp and he was much more nervous and asked what I wanted to do. I said lets see how she does over the afternoon and we'll go from there. We have a long history with this resident. She took care of Riley for almost the entire month she was here the first time as a baby. She has also been on with us occasionally through out and has a LONG history with her. I can guarentee you Riley is one of those patients she will never forget and changed the way she is as a doctor... This paticular attending has also seen Riley before, he took care of her when she had the urosepsis. He was the one who told Dr. Ulrich he didn't think she really even needed to be admitted going off his initial exam and "how good she looked" he was trying to send us home the next morning. I thought he was going to fall over when he saw her blood culture growing out 2 different organisms and it showing she was bacteremic. Needless to say I think he knows better this time than to "blow off" one of her fevers. The resident was telling me later she wanted us to stay until 8pm or so to watch her but he wanted to watch her overnight. She said, if I pitched a total fit he would let us go but... Of course staying til 8 would't work anyway since Wil had a class at 6 and Liam had a 9am Dr. appt. So, we all comprimised and had them go home and agreed to have him come back in the morning to get us after Liam's appt.

Truthfully I have no idea what to do... I asked Wil what he though he said he doesn't know either. I don't think anyone does. She has had a very stressful few day and her autonomic issues could be acting up due to that. BUT the main issue is she had had that femoral line in for over 8 days. This is not good. After about 5 days your risk of infection gets extremely high and goes up everyday there after. So, there is also just as good of a chance that this could be a massive infection brewing and I do not want her new line compromised! So, we are all at a loss. Even at this very moment I am unsure what to do tomorrow. Another major issue if we go home is it's almost Friday and Riley ALWAYS likes to act up on Friday nights. It's murphy's law! To top it off her pediatrician is on vacation so we are totally on our own and would have to bring her back to the ER here if we had any issues.

So, just say a prayer for us that we will be able to know what the right thing to do is without compromising her health. I'll update tomorrow when we make a desicion...

Day 9- Getting to Comfortable

Sorry I was unable to post the update last night the hospitals wireless was down.

The everyday, everyone has certain things or activities in their daily life that just become so normal and even routine you don't even give it a second thought. Some things are no big deal like brushing our teeth, doing the laundry, cooking dinner. However we forget some everyday things we do can pose major risks due to us becoming so desensitized by doing it everyday. Like driving to work everyday, your chances of being killed or seriously injured in a car accident are ridiculously high but how many of us seriously think about that risk every time we get in a car.....? You don't, you don't think about it until it happens to you or someone you love. Then you can't forget the reality of it anytime soon.

I was very quickly knock back down to our reality today with three little words "Open Heart Surgery". Riley has had over 15 broviac placements over the last year, she has also gone under anesthesia or sedation at least twice that many times. My daughter going to surgery, as sad as this sounds, is just an everyday thing.... We truly forget the risks and just always think everything will be fine because it always is. I woke up just like everyday with the doctor in the room trying to talk to my half asleep self. We talk about this and that and how we will be back quickly and then get everything wrapped up to go home the next morning. After he left, I ran downstairs as fast as possible to quickly get the coffee I desperately needed before they came to take her to pre-op. As I came back through the doors they tell me they called for her. I quickly change her clothes and we were off. So much for my shower... :( Downstairs it's business as usual everyone ohhs and ahhhs over Riley and says how big she's getting. Surgeons are running behind as usual so we had a last minute switch and one of the other surgeons was going to do it. Whatever....they all know her. I give her a big kiss and off they take her to the OR around 10am, finally... I head off to the waiting room and make myself comfy and get on my laptop. It had been about 30 or 45min and I knew it was a little longer than usual but nothing much, didn't think much of it and was just expecting the surgeon to come out any time. Next thing you know the doors open and I see him motion to me to come with him, rather than him coming over to me like they normally do and tell you how it went. So, I quickly grabbed my stuff and walked though the doors not thinking much of it. He then begins to tell me he was able to get it in the left but kept bumping into the stent. At that point he tried to get in the right and was able to get the guide wire in but once he threaded in the catheter it pushed the stent into her heart. That's when it started to feel like a dream, this couldn't be happening.... He then proceeds to tell me she's still in OR and completely under, he called the IR(Interventional Radiology) Cardiologist but he was in his office seeing patients, he was coming over as quickly as possible, he was going to try to "fish it out" going in doing angioplasty again, if he couldn't which he really didn't know if he would be able to, that meant she would have to be opened up and we would have to place her on bypass while they went into her heart to get it.... This is at which point I went numb, this could not really be happening, this was just a broviac placement.... and now my baby, who had a no cariac issues a few weeks ago, was going to have her chest opened and her heart cut into! Not to mention this stent is metal it could easily nick her heart or an artery when bouncing around and she would quickly bleed out. It felt like someone had just slapped me into reality. This Dr. mind you has always been my least favorite because while he is actually a quite competent surgeon he has NO bedside manner and he's just a complete butt head most of the time. He was almost in tears while telling me this, he felt horrible. After all this was RILEY. I was the one telling him it's ok, it was a complication and it is a risk we knew was there and it was nothing that was done wrong. We can't always be lucky. He said he would let me know when David (Cardio IR) showed up. I was in utter shock when I walked back out. I could barely manage to get my thoughts organized enough to tell the receptionist I would be right back since I needed to find her dad. I think it was the shear fact of everything catching me so off guard. This might be one of the worst times I have been through. Mind you Riley had had much worse happen many times over but NOTHING has ever blindsided me like that.

Yes, this was our morning and it was crazy. David ended up showing up and coming to tell me that once he looked at it under floroscopy real quick it had moved into the pulmonary system already. He was hoping to take her to cath lab and maneuver it into the pulmonary artery. It would be able to sit and stay there for awhile without doing any harm. Problem was with stents in a child this small as they grow the stents will need to be re-sized. Our hope was at least in the pulmonary artery we can forget about it for a year or two. The other issue was some one was in the middle of a procedure in the cath lab already. It would be at least an hour before they were done and the room was ready. So, she had to be kept in the OR under general and on the vent until they could move her over. David finally came to find me around 2:45pm to tell me the stent was safely sitting in the Left PA and this was probably the best outcome we could have hope for. We are hoping the stent being in the SVC for a few days was able to stretch it enough that for now she will be ok along with starting the blood thinners.It will have to be for now anyway since we are kind of back at square one and can't replace the stent in her SVC while her Broviac is in....

She made it to PACU by about 3pm. So she was kept under anesthesia for over 5hrs. Then we had the lovely task of keeping her laying flat for 6hrs this time! Yes, all that fun I was hoping to NEVER repeat again after Friday. We made it back to our room about 12hrs after we had left. But yet so much more thankful to be "home" than normal! Speaking of home they are still planning on letting us take her to our real home today!!! They still have to pull the femoral line since it was forgotten about with all the chaos yesterday. You know it's crazy, this has been a very eventful stay even for us and even with everything that happened yesterday, today still just feels like every other normal day in our lives. This is now our world, OUR norm, we face life threatening issues everyday and someone walking by us in the grocery store would never have a clue what are life is REALLY like. Just be mindful the next time some one cuts you off in traffic or gives you an attitude at the grocery store for no reason. You never know what someones life is really like and what they are dealing with everyday just by looking at them.Just another day..... but ever so grateful to have another day!

Day 8....

Where do I even begin...? Sorry it's been a few days since I've updated. I ended up going to work on Sunday so between getting up at 4:30 in the morning and not getting back until 10pm I was a little tired, lol. Things around here seem to be going along as planned. The best news is we are hoping to go home on Wednesday!!!! We can't wait!

Riley started receiving the new Lovenox shots(blood thinner) on Saturday. We had to give her a higher dose for treatment therapy for a few days to make sure all the clots she had were gone. This worked great and her blood draw for levels last night showed her therapeutic range was great. So, now we had the all clear to stop her shots for the 24hrs that are necessary before she can have surgery. She is scheduled to have her broviac reinserted tomorrow at 9:30 so that is great news. Then we wait 12hrs and she can re-start her Lovenox injections but at a lower prophylactic dose. She will have to continue these twice a day for an indefinite period of time. That is what we are being told at least... The hematologist most likely wont return but we are supposed to follow up with them within a few weeks of discharge. So, we will see what that brings and I believe we will see Cardiology in 6 wks to follow up on the stent placement. It does kind of suck that we will now go from seeing cardio every 6mo. to a year to now having a lot closer follow ups. Not to mention adding the hematologist to our list of people we have to visit regularly. As if we didn't already have enough specialists! She now has over 15 specialists that CLOSELY follow her. Sigggghhhhh... I guess we'd be a lot worse off without them though.

I am still hoping they will get into contact with her geneticist tomorrow and he will stop by and see her. He hasn't been able to see her since December due to him being on medical leave and then we had to miss our appt. a few weeks ago. We shall see... I am also hoping if all goes according to our schedule we I might be able to get them to let me "sneak" Riley out to her Nephrology appt. that is at 2:30 tomorrow. Since it's only a few blocks I can walk over and it will save me a whole trip over here and besides she needs to have her follow up anyway.

We did get one piece of good news today. Our favorite nurse of Riley's in the whole wide world is ready to come off of maternity leave. She will be returning to us on June 25th. Thank god we missed her! And the best part is she works every weekend! No more worries about me being able to work. We'll just pretend there is no bad news and continue living in denial as I am now!

Please say a prayer that this line tomorrow goes in easily and without incidence. The Cardiac IR the other day told us Riley's right subclavian vein has officially become to narrow from scarring and he was unable to use it. So we just need nothing to happens to hinder what we have left. God knows too we have to get this line in in order to get the femoral out and also because Riley can't take anymore of these peripheral labs/ She has bruises and needle marks covering her entire body. Sh freaks out anytime two nurses walk in the room together since she assumes they are here for her. I'm getting tired. I'll try to update tomorrow after her surgery. Thank you to everyone who have sent up all the many thoughts and prayers for my baby!

Evening of Day 5- Long Day

Sometimes when things get quiet I will catch myself for literally a split second going, omg how did this happen? When did this happen? Last I knew I was pregnant and expecting this wonderfully healthy baby girl.... Then BAHM like a ton of bricks reality sets in. Sometimes I wish I didn't know almost everyone in this entire hospital. That I could walk through the lobby and not see at least 10 people who know us every single time. Don't get me wrong, I couldn't have made it this far without the amazing and supportive "family" we have made here. Everyone has truly embraced us and cares about Riley and our family as a whole. But they are all in love with Riley... Some days though you just wish you weren't that 'Family" some days you want to be the the one down the hall who is just here because "Sarah" had to have her appendix removed and then you go home and life goes on. To us, our world has stopped and everything around us outside "our world" continues. Some days you are to exhausted mentally and emotionally more so than even physically.

But one good thing I try to always remember is something a nurse told me in special care when Riley was just 4wks old. We were still in shock over everything that was happening. After all how could OUR BABY be sick. I kind of took it as an insult at first like she was belittling our situation, but the more time has past it is so true and it's probably the most helpful thing anyone has ever said to me during all this. She said, One thing about being around here is someone is ALWAYS having a worse day than you, someones child is ALWAYS sicker than your, and someone ALWAYS has it worse than you.... It is so true though! Right now, today as hard of a day as we had someone watched there child die in this hospital today, someone is having to make a choice that they know most likely will kill there child but it's there only hope, and someone found out there child will die within days or most likely not live through a surgery. Someone is ALWAYS worse, so I always am reminded of that when faced with a hard day. At least living in this world will keep you grounded and make you thankful for EVERY SINGLE DAY! That is something I am forever grateful for. We still have HOPE!

Riley looks much better now she is quite hyper and having a hard time sleeping actually and is busy over there giggling and dancing. Probably because Wil and Liam are sleeping over here. Liam has been screaming all night for me so I finally let him stay. They are nice and don't really say anything anymore to us since they know we don't do it often. All three of us sleeping in this tiny cot is fun... Anyway, once I got to PACU I found out they did put a special pressure dressing on her leg but that she wasn't able to sit up or move her legs for 4hrs... Yeah I looked at the lady and told she better go find me some drugs for her then and when she looked and found nothing ordered I then told her she better get out the soft restraints. She laughed but I was so not joking! It was a nightmare I literally had to stand next to her crib for 4hrs and lay on top of her and at times get slapped in the face and my hair pulled out when she was sick of it. Finally at 5pm we were able to let some of the pressure out and she was able to move around. She was such a mess though she wouldn't put weight on the leg they did it on but her other leg has her femoral line in it, her pulse ox on her foot and a peripheral IV in that foot. poor child.

So, now that this has been resolved we still have two big issues to handle. She still needs to get her IVIG and she needs to have her broviac replaced. Other things that will have to be addressed are what's going on with hematology and there plan once she's discharged. Also I was trying to get them to contact her geneticist/mito doc to get his blessing on everything that has been going on. Since we were gone all day and it is , of course, Friday! Everyone was gone for the day by the time we made it back to the floor around 4:30pm. And everyone knows nothing happens around here over the weekend not to mention they never sent her TPN orders to the pharmacy. So that means at the very best the earliest we can leave is Tuesday evening. Blah............. and that's if everything goes exactly right. I'm not counting on anything at the moment yet. This is ridiculously long and rambaly already and I'm exhausted so I'm going to sleep on my tiny little section of the cot. LOL I post more as soon as I know more!


Riley in PACU before they told me her legs had to stay flat too!

She is so swollen now! You can see the special

pressure dressing on her left thigh, it was actually pretty cool.

And of course being fiesty as usual and taking her EKG leads off to play with them.....

Afternoon of Day 5

I'm sorry for not updating sooner it has just been very busy around here and there was no time. Between dealing with Riley and the multiple doctors and such plus Liam and Wil came back yesterday evening.

At first yesterday they were really planning on sending her home like this but I kind of had a fit... There plan was to give her IVIG today and then go home. As long as the line was working to run TPN deal with it basically was the attitude. This was definitely not cool with me and I knew it was not the right thing for her. Things kind of inched along all day and we didn't really get much done, until ID came in that is. They gave her the all clear that we are confident she doesn't presently have an infection. So, she was more than happy to tell cardiology this news. Who in a little of a whirlwind agrees to call the interventional radiology cardiologist and find out when he wanted to cath her. We figured it would be Monday or something but about 30min later I walked out to the front and the Hospitalist caught me to tell me they wanted to take her first thing in the morning. They also said however her old broviac had to be removed first in order for them to cath her properly. By this point it was 6pm and we would need an Echo again in the am to confirm it hadn't moved or anything with the removal of the broviac.

So, we were able to get a hold of the surgeon who also happens to be our surgeon so he said he would make sure it got done for us at some point that night. He came by before doing it and said see I knew this needed to be addressed, that's why I wouldn't pull the line the other day until this was looked into. He said, him and I both know there are only so many places she has that can be accessed and if we screw around and loose an access point the price could be unthinkable. At this point, much to every ones dismay, If she didn't have access she would die. That's just the way it is, so we HAVE to be careful with what we have. Just because hem/onc kids use lines that are clotted doesn't me she can. They might need there lines but they don't depend on them everyday to live. So he ran off saying he would be back tonight to do it and would remove it in the treatment room. He kept his word and showed up about 1:30 in the morning. This is why we had a long night of waiting. They gave her a local and a dose of morphine and wrapped her in the papoose. It was in good but we managed and got back to bed around 3am. It had proceeded to gush some blood right after he removed it ( He let me stay and help) this was most likely due to the pressure from the clot. So, we had to put a pressure dressing on her. Which she didn't like and kept waking up all night to pick at it. So she woke up in a lovely mood this morning, sarcasm intended!

They were in early bombarding us with new drs. and people. They had her down to cath lab by 10:00am and had her IV versed ready for her! LOL They then preformed a sedated echo in the cath lab and removed the clot and placed the stent. She did great and apparently it was actually a significant clot. It was a 9mm clot.... So hopefully now that the blockage has been removed she will feel better and the stent will help us. The IR was awesome and very helpful. Another new friend, LOL. She is still in recovery and they just called me to be with her. There are still many unresolved issues. I will try to post again tonight when things are more resolved. Hopefully tonight will be a little calmer. *Crossing our Fingers*

Day 3 APH

So, after a long and eventful day. We still kind of didn't get far. We do know she has no clots in her neck or surrounding veins which is good. Functionally her heart looks good right now. Apparently the clot is smaller than then they expected however still of significant enough size to be officially partially blocking her SVC as we expected. The clot looks like it is embedded in the tip of her broviac and that is why we were having the issues with it. Hematology is sitting on all of this for the time being and waiting until all her testing comes back to decide what to do. The consensus is she will probably need to be started on some form of a daily maintenance blood thinner like Lovenox injections. But we will address that more later. And if one more Dr. tell me how we really need to think hard about getting her off of TPN I'm going to scream! Like they think we have just thought it would be a fun idea to leave her on it for over a year. Ummmm NO, it's a total PIA and one I would LOVE to be without but am grateful for because without it Riley wouldn't be alive right now.

So the plan for today was to TPA the line and see if that would clear it enough to get blood return. After two doses and letting the second one dwell for 2hrs. still nothing BUT it flushes great still....

From ID's standpoint given that her fevers have resolved they are happy. So, this most likely means there is no infection thank god! I think her autonomic issues were just out of control when we were using the line and it caused the temperature elevations. Now we just have to convince cardiology of this! They are not sold on it... We still need to do her IVIG at some point too since it was due today.

I was out at the back nurses station talking to one of the GI's, since they are all like family to us, and this is her first ever admission not to have them taking care of her. The hospitalist saw me and said ohh good I wanted to talk to you and told the GI she had a question for her too. She actually asked her, "can she be sent home and run her TPN through the line even with the clot?". What!!!! SERIOUSLY? I was not happy at that point and as we are walking back to the room she tells me she was thinking if the TPA didn't work(it was dwelling at this point) she would run the IVIG tomorrow and then send us home! I was like NO, we can't go home with a halfway functioning line and this clot has to be dealt with! So, once I talked some sense into her she is going to try to get ID to convince cardio she doesn't have an infection and see if they can to the angiogram to remove it. So we shall see how that goes tomorrow. But I'm not leaving here without a fully working line or risking loosing this one completely if it can be saved...

The other issue is her swelling and elevated BP's. I brought it up to her yesterday and this am she had thought they were fine but I told her about how they were elevated all day and she was mad no one called her. She ordered Riley's renal ultrasound for in the morning since it was time for it anyway. Of course the nephro I don't like is on call this week so maybe we can wait til Friday and see if her Nephrologist is on then. She is wonderful and will take care of this issue for us. I also happened to run into her Pulmonoligist later who is another very close "friend" of ours. Riley hasn't been able to follow up with him in forever due to her constantly being in the hospital. I told him how she was still on her caffeine and how her hypoventilation seems to have gotten and gets bad when she's sick or to fatigued. He said he would stop by for an official visit tomorrow. We mine as well get everything done at once, less appointments later for me!

The only other thing of note today was her CPK level came back elevated quite a bit. Everyone is kind of scratching there heads about this one. She was just kind of pale and really tired all day but overall in good spirits. I'm assuming it was from everything yesterday. They called to tell the hospitalist the TPA didn't work and they said we'll deal with it tomorrow. So for now Riley just fell asleep and Wil and Liam went home for the night because Liam really wanted to sleep at home for a night. For tonight we are tired but can't ask for more. It's just like I told Liam the other night when he was crying to go home and sleep in his bed, but he wanted me and sissy there too. It doesn't matter where you are home is where your family is whether that's in a tiny hospital room, at the RMH, or at your house. As long as we have each other we will make it through this!

Here are some pictures of the kids from the last few days:

Liam sleeping after his surgery on Friday

Playing on the bed yesterday morning

You can see how puffy her face is in this one

I love how she just sits there like a big girl

with her legs crossed

Getting our 2hr Echo, I was desperate to find

anything to entertain her!

In the PICU right after getting her Femoral line

Completely Snowed....