17 weeks

They went down on Grace's flow today, back down to 2 liters on a blender with the Nitric still at 20. Sildenafil  has been being given for over 24 hours now, such a low dose but so far so good. I dont know when they are going to get an ECHO I don;'t know when they are going to wean Nitric.Grace has been ok. We napped together for a bit today, one of the sweetest things about having a baby is falling asleep with him/her on your chest, I'm glad that's one thing we didn't completely miss out on with her.
she likes having kisses on her cheeks and chin, she sort of smiles for it. I might take a purposeful blogging break, to be honest my hearts not really in it right now. day to day  nothihng really changes, pphn stays the same, stomach stays the same, her mood stays the same her reflux stays the same. Im sort of quite discouraged by the lack of progress lately, and just feel like taking a break, so I suppose no news can be considered good news or exactly the same news. if anything astronomical happens, I'll come back.

5 am phone calls

Are never any fun especially when they are from your childs surgeon. Grace had another huge desat epidode this morning but I guess it was worse than ever and they put her back on high flow and also back on Nitric Oxide and gave her a blood transfusion and are giving her extra diuretics. They are going to try slidenafil again and see if maybe she can tolerate it yet. PPHN is what is holding her back from home, though it is much more controlled than it was, it's still not controlled enough for her to be off of Flolan or to not desat like she has been doing. At some point she will outgrow the PPHN as she gets bigger but I'd kind of like her home before then. There are 4 more weeks left before school starts and honestly I really thought we'd have all 4 of our kids home for a bit before then, but its not going to happen.

No change

Grace had her ECHO today, no change. So frustrating. Waiting for word from Cardiology as to where they want to go from here.

The weekend.(and the first half of the week, since Ive been writing this for 4 days)

For the most part, Grace had a good weekend. On Saturday she had a cranial ultrasound to check on the fluid levels in her venticles because Friday night I guess she was just very fussy. Her nurse described her as "her pre shunt self" but the ultrasound was fine and she went on to be fine. So on Saturday she got to play wiht her big brothers for a bit, this was the first time they had been up to see her since the shunt. She was a little fussy at first, but then she ended up not minding them and she liked when they held her hands. The boys were so excited and happy because we let them hold her. She lounged in their arms and was pretty happy.However, then something scary and unusual for her now happened. She started to fuss just a small amount so I started changing her diaper, she wasn't even screaming or that upset. Then she started to desat. Big time. She went down to 31%. For a number that should be as close to 100 as possible, that's not good. She was very pale, limp, quiet, and blue. She recovered with a few minutes of extra oxygen and then was fine, back to satting 100. They did a chest xray and it looked fine, we have no idea what happened. It was pretty scary but she has been fine since.
On Sunday I got to take her for a walk around the 4th floor wiith a nurse. Grace really liked being in the stroller just strolling around, we only walked for about 10 minutes.
On Monday we gave our first try at nursing. It didnt last long and though she didnt hate it, she didnt love it either, and didn't really know what to do. I'll spare the details but will share if you are interested. Tuesday I tried again and it was an immediate fail/freakout so I'm not sure I should try again. She obviously wouldn't be doing it for nutrtion as she is fed bypassing her stomach but maybe for comfort and practice using her mouth.
This morning I was told that by her nurse (who is one of her primiaries, so she knows her well) that Grace got madder than she's ever seen her and she desatted very low. The monitor read 12 but she was kicking and moving so she's not sure if 12 was accurate because you would think at that low she would be passing out, but she was dusky and her lips were blue so she was very low nonetheless.
Tomorrow is ECHO day, we'll see if upping her oral med and switching to jtube for meds has made any difference in the pphn. I really hope so.
I'll try to update again tomorrow, I know I havent been keeping up on it as much as I'd like, most days I don't get home from the hospital until after midnight and if I am home before then theres entirely too many things to do around here. Please continue to pray that she might turn around on the pphn front. It's basically the one thing thats keeping her there, but unfortunately its a very very big thing.

16 weeks?

Sunday marks Grace's 16th week of life, 16th week of NICU visists, 16 weeks of this beautiful little girl fighting each day to live. Grace is so alert and so much more like a "normal" baby these days, everyone says "she likes her shunt" and it is so amazing to look at her and remember where she has been the last 16 weeks. I never thought it would be true but looking at her, it is seriously hard to ever picture her as the same baby on ECMO with her intestines in a silo. I found a picture on my phone that Adam had texted me months ago, of the vent showing she had .8 lung volume, how excited we were, how far we've come!
Grace got her PICC line out on Monday, so exciting. The PICC had delivered all of her conintuous drips, she is off of all of her IV sedation/painkiller meds and is weaning the oral Methadone and Ativan. The PICC could also give any other meds she might need, but she doesn't really need anything extra these days and it could be used to give blood, but aside from a transfusion after her shunt surgery she hasn't needed blood in a really long time, and the PICC was being used for drawing blood gases, which we don't really need anymore either! It's very exciting that she doesn't need that port! Now Grace is down to two pumps on her IV pole, 2, there was a time when she had the double pole, plus the one on her isolette AND pumps running off ECMO. Now she only has her Flolan and her feeding pump. That's IT. She is still connected to the monitor, one wire running from her leg and 3 leads on her chest/belly and she also has her g tube to drain into a bag so she is not completely "wireless" but so so much more than she used to be!
Grace has been smiling, it is the most amazing thing ever. I just sat and cried the first time she did, I couldn't help it, I am just SO happy. She has this mobile that she used to love and for awhile just didn't even give it the time of day, but we kept it, thoping one day she would like it again and she does! She LOVES to watch her bugs go around and sit in her vibrating bouncy seat. She loves to sit in the door of her room and watch all the people in the main room, she's a nosy little girl. Sometimes she just complains, she is perfectly fine and satting great and nothing is going on but she is wide eyed and yelling. She has quite a story to tell and wants to make sure everyone is aware of her presence. Last night when she got her breathing treatment (she gets them 2x a day) it was so cute, because she was screaming but she wasn't actually crying and she kept looking out of the corner of her eye at the RT, as if to say, "she's still here? Go away!"
Though she is doing better than ever, there are still a few things we could really use some prayer for. The Lord is amazing and has worked in wonderful ways in Grace's life, she's such a miracle I know we can overcome these next few things that we need to!
1. Grace really needs her pphn to improve more, her ECHO's have been unchanged for quite some time now, obviously great that it hasnt gotten worse but we need them to improve so that she can start weaning FLolan. Flolan is a 24/7 coninuous drip through the Broviac in her chest that is to help her pphn. The ideal thing is that she goes home off of it, and her pphn is controlled by oral meds. She is at the max dose of it. But until her pressures lower more she cannot wean Flolan.
She is on a med called Bosentan which is oral and her dose is being highered every other day on that to see if it helps and they can wean Flolan. If her ECHO remains unchanged after the higher dose of Bosentan then they are goign to see if they might be able to try Sildenafil one more time to see if it helps. If not then she will go home on Flolan and in time hopefully her lungs will grow and develop better and she will be able to wean it. They have switched from giving her meds through the G tube, into her J tube thinking that perhaps her stomach isn't completely absorbing the Bosentan which is maybe why her ECHO hasn't changed.
2. Grace's abdominal incision is having trouble healing, most of it looks great and in fact is so light it's already hard to see. One spot however has always been a little bit of a troublemaker. It had healed up ok and is now broken open and has even started to bleed. Please pray that this wound can heal quickly.
3. Please pray that her stomach would just start to work. Pray that she could begin moving things through, right now, she is putitng out so much bile into her drainage bag, and they aren't sure she was absorbing the meds, please pray that her stomach will just wake up soon and begin working like it should so that we can start feeding through the stomach like normal or even just that she would tolerate moving her own digestive juices through and we could clamp it and not have to have a huge drainage bag.
Grace also has awful reflux, please pray that it might get better.
Even aside from all of those things, she is doing amazingly well. She is so so gorgeous, I just cannot belive how great she looks after the shunt. I am so glad. We are so incredibly blessed to have our sweet girl, she beat some incredible odds. 
Please also pray for a family we met in the NICU who's own sweet girl went to be with the Lord over the weekend, our hearts ache for the Martin family. As close as she sometimes came to joining the 50% who do not survive, I cannot even imagine it actually happening, and how hard it has to be.
I heard a song on the radio recently that I am in love with, by Stephen Curtis Chapman and it says "and tell the story of grace with every move that you make and every little thing you do" Grace has certainly lived up to her name, each day of her life has been telling the story of Grace, what an amazing little blessing she has been, not only to us but to everyone else who knows her.

The surgery, recovery and self extubation......

So I mentioned that Grace was doing well after the surgery, and each day seems to be different. When she came back, it was around 10:30 pm and it was pretty hard to see her intubated, sedated, and bandaged again, she was also orange from all the betadine that they clean her with to keep her sterile. She stayed sedated the night mostly, she opened her eyes a bit but was very out of it. They got a blood gas and the RT's felt she might even be a little over ventilated. The next morning they made one change to the vent and said they were nervous to extubate too quickly given her history. They wanted to wean slowly. All day Thursday was spent trying to keep her from pulling her tube. IT was great though because you could watch her look at the tube and then grab it, she never really had much hand eye coordination before and now she does. She tolerated being intubated alot better than I thought she would. Yesterday she wasn't as eager to pull it, she behaved herself a little more. I held her yesterday and she kept moving her head and pushing with her feet, trying to get it out though. She finally got the MRI, it kept getting pushed back until around 10 pm! She only needed morphine and ativan, much better than her first one where she required general anesthesia! They did a sprint overnight and while she did good, she got tired and her gas was ok but not great. So they wanted to sprint her again a few times and a gas tomorrow morning. The sprint during the day went well and then after lunch I held her. While I was holding her she needed to be suctioned and she hates that, I hate it too. After she was suctioned she started vomitting and after that, she started moving her head like crazy and i was trying to stabilize it. All the while I was watching her tube and it was taped. then I realized that though the tape was all intact, the tube was moving in and out of the tape. I yelled for her nurse (Amy, one of our wonderful Primaries) and she came in and looked and said well she's not vocalizing and she wasn't desatting so we thought it was still in, she checked it out and turns out the end of the tube was just inside Grace's mouth. She extubated herself! They got some nasal cannulas and put her on 3 liters off the wall and she is fine! She did not want to wait for the surgeons to call it, she said she was ready! An hour later they got a gas and it was fantastic! So though I am obviously not happy she needs a shunt I am glad she has it now because she really seems to be doing better now.She is focusing her eyes on you now and she is trying to reach for things and she is bringing her hands to her face, doing so many things she wasnt! I pray this is the last thing we have to overcome before home. One of the things that is worrisome though is how her abdomen is going to respond to the extra fluid being shunted in, so pray that this all works as it should and she can finally be on the road to recovery.

Doing well

Sorry I have not updated since the surgery. Grace was not down in surgery for as long as we had feared because they were able to go ahead and place the tubing into her stomach rather than have to figure out how to get it through her heart. SO overall it only took her about an hour. The neurosurgeon said it went as well as he would have hoped on his end, however sedation was an issue as always with Grace. She is still intubated, which really stinks. SHe hates it still, but is much more tolerant of it than she was a month ago. They are being very cautious of extubating too quickly because of her PPHN. She is awaiting an MRI hopefully later today and then if she tolerates that well, then we might be able to continue weaning her vent today and hopefully extubate tomorrow. I will post a more detailed update later, but she is doing well right now. It seems she is much less irritated, perhaps she did just have a headache her entire life.

Surgery again.

This morning when I came in, Grace had had the cranial ultrasound and her nurse was waiting for the final results. She paged the PA for Grace's surgeons and she came down and talked to me. She said they got the report back and Grace did have fluid accumulation around her brain. Hydrocephalous. Neurosurgery came down and checked her out and talked with me. They said based on how her head was bigger than it should have been, and her ultrasound and the fact that her eyes were downshifting and she was very irritated but in a different way than normal, were all indicative of Hydrocephalous. So the solution is to place a shunt in her skull. She will have this rubbery plastic piece that sits on her brain with tubing through and down to her stomach to drain the Cerebral Spinal Fluid from her brain to her stomach, where her body will hopefully reabsorb it. The shunt will be permanent, and she could possibly need numerous additional surgeries. They know some people do not need more than this one, some need just another and some need up to 10 more just depending on different factors. With Grace having had her CDH repair, then having it opened again to place the GJ her stomach is compromised so they may not be able to place it there, if they cannot place it there then they will need to use her venous system. Which in Grace is tricky since she was on ECMO she does not have use of her right jugular and Carotid artery, so they would try to go on the left but she has a Broviac in her right subclavion so then there are challenges finding where to thread it through. So hopefully they can go to her stomach.
Grace will intubated for her surgery and will come back from surgery intubated and it will be very hard for me to see her like that again. Hopefully she will be able to extubate soon and not have any issues coming off the vent, she was doing so well before. She is down in surgery right now, and it's unkown how long the surgery will take.
Please keep thinking of her and keep her in your prayers. I really hope this is the last hurdle she has to jump.

Grace is having a cranial ultrasound this morning to check for fluid in her brain. Please pray they do not find anything, as it could be a scary situation. I will post a more detailed update hopefully later but wanted to get the prayer request out.

Alot of the same.

Grace has an ECHO tomorrow to assess her PPHN and see how well the Bosentan is working and then hopefully we can develop a plan for weaning Flolan. Please pray her ECHO is awesome and we can finally start the wean and that her PPHN stays controlled without it. I am so so anxious to just get moving. I just want her home, to have our family whole again.
So please pray for more improvements for Grace, though she has come so so far, she still has so so far to go.