Annabelle is handling things 10x better than any of the rest of us likely would. She is still quite sore and is very cautious when doing things. She is terrified to situp from a laying down position.. I keep reminding her to roll over to her side instead of using her tummy to sit. When she climbs to our table, she forgets about her tubby when getting down and always snags the tube on the chair - which results in screaming. The natural instinct for a child with a tube hanging out their tummy and it hurts them, is to pull it out - and this is Annabelle's reaction, every single time. She used to acknowledge her NG Tube easily, she would point it out, show people and talk about it. It was present, there was no hiding it and even if it hurt her, it was in the open. The G-Tube has dramatically changed Annabelle's tubey perspective. She is very nervous about it, she does NOT like anyone to look at it, she doesn't want to talk about it and if you grab the first piece of supplies, she cries hysterically and begging "Noooo! Please!!" The whole process is still so new & the soreness is very present. We will find our groove soon and the pain will start to subside.
Our current routine is.... freaking nuts.
Belle is sleeping in the bed with me, and AK is sleeping on the couch. She is having a hard time at night and staying comfy without hurting her belly. She needs to be vented more often at night for some reason, and the Farrell bags just aren't doing the trick. In a great world, we would be racking 24/7 bc that seemed to be the best method but it's also the most stationary and messiest method.
During the day, we are feeding by pump every three hours, for one hour total at 100ml x hour. This process looks a lot like:
- Gather supply
- Now calm Annabelle down because she just realized what you were doing.
- Make a new concoction of formula.
- Prime and prepare the pump
- Calm Annabelle down and avoid a tantrum & trip hazard around your ankles.
- Vent Annabelle with 60cc syringe
- Change dressing around gtube
- Clean surface area around stoma (site) and remove all crusty, goopey, gross stuff.
- Calm Annabelle down again bc this hurts her like HELL
- Re-tape her extension tubing to her tummy.
- Re-apply dressing around gtube. Tape again.
- Again, calm her down because this also hurts like hell.
- Finish venting. Place contents back into her tummy.
- Hook up pump
- Administer all necessary meds.
- Flush tubing from meds to clean med port.
- Begin feed.
During feeds, sometimes Annabelle's tummy fills again with gas/air that cannot escape, so we pause the feed to vent her manually vs using the Farrell bag that should be doing that for her.. but don't. Sometimes the feed rate is too high for her tummy at that time and it causes pain, so it's hard to gauge what is going on or what needs to be addressed. Sometimes the feed makes her nauseated and she vomits. Most the time, feeds make her very sleepy, which makes her irritated that she cannot get comfy bc of the tube, which turns into a fighting-sleep-in-pain-ticked-two-year-old.
Up Side Of Things
The new formula regimen in finally fully in place and on the outside seems to really be working better than before. If you want to try to comprehend what we've done, here it is:
- 40 Scoops BCAD1 (180gm)
- 4 Scoops Anamix (20gm)
- add water to make 40oz formula (24 calories / oz).
This is much more than she was previously getting. Before, we were making her formula at 15 scoops BCAD1 to 20oz water.
This diet now provides Annabelle with 976 calories / 32 gm protein x day.
Down Side Of Things
The consultation that was scheduled for today did not lead us with many answers. I spent quite a while on the phone with our doctors and they were disappointed to not gain much new advice from the consulted team. The intentions were all well, but they simply have never heard or experienced what we are with Annabelle. They all decided we will call this the Metabolic Annabelle Disease. I agree it's cute, but Google doesn't give me information on how to treat this Annabelle Disease, and that ultimately is what we need.
For right now, we have a band-aid of a treatment plan. We have absolutely no explanation for why what were doing is working, but it is.... kinda.. almost.. well..... it's working until we have to react to the next swing of irregular labs and make alterations again.
We need a diagnosis. We need to figure out what specifically is wrong with Belle so we can safely treat this disease. The one good part that was delivered from today, is that the consulted team agreed with 100% certainty, that whatever this is, is definitely Metabolic. So there, we at least have something semi narrowed down. It's related around Amnio Acids, possibly Organic Acids, its potentially an Inborn Error of Metabolism of some sort, we just have no idea which one. Something is not being absorbed, created or working properly within her system therefore everything is falling short.. each time we try to place a bandaid over what we believe is the biggest / loudest / most pronuonced issue, we seem to launch a new set of problems in reaction. We keep getting a little closer with treatment, but haven't hit the nail on the head yet. With every treatment plan we *think* is logical, we are still shooting in the dark, because we have no idea what is truly the issue.
Our doctor purchased a new computer over the weekend.. this now allows her to speak with physicians in other countries and translate between languages, I chuckled at her determination but I couldn't thank her enough for her efforts. Thank the heavens for our doctor! Thank you!
I myself, am looking and fast and as hard as I can within the United States to find Research Centers/Hospitals that have a focus in Metabolics and these diseases. I do not want a darn good Doctor, I want someone that is willing to investigate this on a research level. If Annabelle had something that were already printed in a textbook, we wouldn't even be having this conversation. Clearly this disease isn't recognized or big enough yet, that is our brick wall.
So we are trying our hardest, working our fastest and trying to move thru life.. thru December.. thru every hurdle life continues to throw our way so we can find a diagnosis and answers for sweet Annabelle. She deserves nothing less. She deserves to begin the New Year with fresh hope. It's a stretch goal, but as a parent, that is ultimately my goal. I want answers.... yesterday!
How are we? We're tired. But I do think you all deserve a tiny insight on our lack of being involved with friends and family lately. We're so detached from the world, that at times during the chaos, we feel lonely. We have a flood of people around us I know, but I pray you all do understand why we just suck as people during these last several months. You deserve better of us as friends and family... we;re trying, I promise. We're just so tired. Our days are crazy and finding a nurse is like finding a needle in a haystack. That's ONE of the many current battles: Annabelle gets 16hr x day of in-home nursing care.. but the trick is finding a company with a nurse (we like) that can do the job, wants to do the job, can travel and be here for the little pay we and the company can afford, but a company that also participates with our insurances. AK and I both work full-time and making that happen and keeping our jobs is going to darn near kill us.
Our schedule is insane too.. here is the VERY high level of what is going on in our home during the days, this doesn't include therapy sessions for Speech Therapy and OT, this doesnt include nursing visits for post-op, doctors visits, consults, labs every week or every other week, life in general, happy times, sad times, extra bath times because a tube has leaked/exploded on everything, grocery store run or just life in general.
7am - Stop nighttime feeds. Manually vent. Administer morning medications. 8:45am - assemble supply
9am - Change dressing and begin bolus feed 5oz over the course of 1 hour.
10a-10:10a unhook Belle from feed.
10:30a Manually Vent
11am Manually Vent
11:45am assemble supply
12pm - Administer noon medications and begin bolus feed 5oz over the course of 1 hour.
1p-1:10p unhook Belle from feed. Get her ready for a nap
1:30p Manually Vent while she's asleep
2pm Manually Vent while she's aslep
2:45pm assemble supply like a ninja to be sure not to wake her
3pm - Change dressing and begin bolus feed 5oz
4p-4:10p unhook Belle from feed.
4:30p Manually Vent
5pm Manually Vent
5:30pm Take a bath/shower. Administer nighttime medications
5:45pm assemble supply
6pm - Apply new nighttime dressing and begin bolus feed of 5oz
7p-7:10p unhook Belle from feed.
7:30p Manually Vent and get ready for bed.
8pm Manually Vent and PRAY she's asleep and you do not wake her.
8:45pm assemble supply
9pm - Begin continuous nighttime feed of 20oz over the course of the next 10hours.
12am - Pause pump, manually vent, begin feeds again (do NOT wake the baby!)
3am - Pause pump, manually vent, change diaper, begin feeds.
I am not complaining by any means, please, whoever you are, do not take it that way. This is our life, and by any means is it easy or welcomed. But we know it could be 62049610x worse. We are so very blessed to have these routines, family and friends that love us, each other, a home and lights that *somehow* are staying on. We are very busy though, we are stretched thin but want you to know that we are so very grateful to have each and every one of you. I promise, my heart is worn on my sleeve and every person that has reached out to our family deserves a heartfelt, hand written note to share with you how blessed and humbled we are by your generosity of kind words, prayers and support. That is the way we carry our family, I would love to have the time to individually thank every one of you but please know that in our hearts I am pouring our love your way just as much as you're pouring yours towards us. One day soon, I hope to be able to do something for Annabelle and bring everyone together, I would love to host an event where I can hug everyone as tightly as I have wanted over this last year, and you do the same for Belle. She LOVES a big "SQUEEEZEEE" from anyone willing to give her hugs and kisses. She is a sweetheart and I know would love a fun day to spend with every one of you. In the meantime, please keep praying for us and understand just how much we love all of you but are so very busy at the same time.
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