Honestly, I truly did NOT think we'd go into August and if we happened to, it would be a few days only. Folks we'll go into at least September.
The last week has been very very frustrating and the last 48 hours even moreso. Two weeks ago, we thought we'd be home within the month. Yes she was still on Flolan, but there was an awful lot of talk of her going home on it and being fine. Suddenly she's back on high flow and Nitric but we thought just for a short period of time. NOW, she is doing awful. There were even mentions of having to intubate again (her surgeon is NOT going to at this time but if she does'nt start improving, it's not out of the question) Grace has not tolerated at all the weaning of the Nitric, she desatted and became frantic each time it was tried. Sildenafil was started and she was tolerating it and the increase of it. Two days ago, she spiked a fever again and her white count is slightly eleveated, her heartrate is sky high and her sats are not great, she is requiring alot more oxygen and is working very very hard to breathe.Her belly, where her repair inciscion is, is awful, it's oozing and red and open. She once again is having a whole septic workup and was given blood, her flow was turned higher. The fits she's throwing are crazy, I was holding her and feared I might drop her because she was flailing around so much. She was frantic. I held her until 4 am yesterday because that's what she wanted. Today she finally calmed down after she was given some blood and cooled down and got some more flow. We had a family meeting today and the original point was to be able to iron out a plan for going home and what needed to be done before hand etc. but turned into how are we going to get Grace out of this downward spiral she appears to have fallen into. She could be reacting to the Sildenafil again, which is an easy fix, to stop giving it to her but then obviously she needs something more than the flolan and bosentan that she was getting because she started up with these desatting episodes. Then we might have to start looking at whehter or not she has some other issue going on the cause the PPHN rather than just CDH. If she does then it's a whole other ballgame which will be much trickier and harder. She could also just have an infection that they could treat, right now she's on IV antibiotics. Please pray hard that htey figure this out because she is not doing so great. If it's the Sildenafil then that's so upseeting because she needs some major help with her PPHN. We fond out that she does have pyloric Stenosis after all but right no w nohting will be done to fix it. She is not in a position at all where she could get a repair for it. FOr a baby who does not have CDH who's only issue is pyloric stenosis, its a 12 miute laproscopic procedure, but for Grace it would be a much more lengthy and difficult procedure. So right now the plan is to hope she will grow out of it in the next ten months or so, and if she does not then they will re evaluate and perhaps she will be more stable for an operation but right now since she is fed post pyloricly it is just not worth the risk of intubation, anesthesia and opening her stomach up again. I completely agree that she's not in a postition for a surgery right now but how I hate this because she has begun loving haveing breastmilk given by mouth just a tiny bit, and I think as she gets older she wouldn't be against eating actual food, which she will not be able to do until the PS is fixed. But at least she can have breastmilk/formula. Please pray that she will outgrow the pyloric stenosis much sooner than later.
So right now we are between a rock and hard place again. If Grace has an infection in one of her lines or her shunt or her patch then that's not good. But at least it's not the viagra and we can get her pphn under control still. If it is the viagra then she cannot have it, and we are back to the drawing board with how to deal with her pphn and how do we get it under control and get her home? The options all kind of suck.
I struggle daily with just being so thankful that she is alive and taht she made it, I am SO happy she is here, the numbers don't lie and HALF of all CDH babies born will not survive and yet she did, as sick as she was/is. At the exact same time it is so hard and frustrating that she is seemingly "stuck" where she's at. She is not going to live a "normal" life by any means, she will be j tube fed for a very long time with the G to drainage ( not sure if I ever explained this one. She has a tube that drains all her stomach acid into a bag) she will very likely e connected to flolan for quite s ome time and will be dependant upon oxygen for awhile, as it is right now, her requirements are high and if i t is accidentially disconnected, she is blue and struggling almost immediately. But yet she is alive, so I know we are very lucky. We lost another NICU friend this week, please keep the Pepper family in your prayers. /Also, please keep a new CDH fighter in your prayers, his name is Aiden and he's in the hands of Grace's amazing team so already he's got alot going for him!
I had one of Grace's surgeons share with me the other day that as much as he believes in the medicine because that's the field he chose and that's what he is forced to know, he also know's there is something more to Grace, that she is not here just because of science. I know this without a doubt. Prayers worked to keep her here, now lets amp up the prayers to get her through these challenges and get her HOME. She is 4 months old, she will not be coming home before she is 5 months and possibly not before 6 months. It's basically hard to say at all but she needs to come home soon. She needs to for her and we need her to for our family. Our kids need both parents around, Grace always gets one, and the boys get one. Nobody gets two at the same time. We've almost depleted our savings in the gas alone it costs to drive the 2 hour round trip each day. School is starting soon, which creates a bigger challenge of how I am going to manage school pickup and drop off as well as spending time with Grace. Grace knows and feels better when I am around, I didn't believe in the begining that she knew who I was or that I was there, the nurses would say " oh she knows youre her mommy" when she was on ECMO and I would tell myself the same thing but truly didn't believe it. How could she know? But now? She knows, she knows and she loves me and she is happier when I am there and it makes it so so SO hard to leave her every night and to know that when school starts I will not be able to spend anymore than a few hours each evening with her. I will not be able to be there during the day and it hurts me so much to know that she will not have me. She does have awesome primaries who love her too, and all the other nurses do too, but there is no replacement for mommy.
Please pray that
she could come off Nitric and back to 2 liters
wisdom for her Dr's (who have already done an amazing job with her) to be able to find what works for Grace to get her home. My favorite Dr said yesterday "we will get her through this, we've all invested far too much physically and emotionally in Grace to not"
Her pyloric stenosis to resolve soon
her pphn to start to change for the better
she has a very hard time avoiding diaper rash, pray for a clear bottom!
pray for our family to figure out how to navigate this for the next few months.
As very much as we still have to overcome, Grace is truly truly a miracle. Jesus has carried this baby from the begining and has not let her go. Grace shouldn't have survived either of her transports and she did, she wasn't expected to make it off ECMO and she did, she is fighting like a champ each day. CDH babies who are diagnosed prenatally and delivered in tertiary facilities with everyone ready for them and with a plan do not survive sometimes for more than a few hours. Babies who were given excellent odds before birth, do not survive and yet she has. She has beaten every odd stacked against her and she is absolutely amazing. I have no doubt that she can get through these huge hurdles, but have no idea how long or hard it's going to be. Thank you for your contiued prayers and support.