**PLEASE NOTE: THIS UPDATE WAS ACTUALLY WRITTEN ABOUT OUR LAST VISIT IN NOV (THANKSGIVING) AND NOT THE CURRENT ONE (12/19/2014). JUST GETTING AROUND TO EDITING AND POSTING NOW.**
Dr. Niebauer!!! Our angel doctor!
How we love you! It's probably sinful.
Let me first start by saying that when we were initially hospitalized and then released, I was hoping to never have to revisit the hospital. Not because of the staff or St. Joe's in general but because it would mean that she needed to be in the hospital for some reason and that is what I wanted to avoid.
But then I must remember that God's in charge, not me.
Never me, always Him.
Being admitted twice has turned out to be the BEST thing that could have happened to her.
It was His plan all along.
Philippians 4:19 And my God will meet all your needs according to the riches of his glory in Christ Jesus.
Not only has God done that for us since we've been in the hospital, He's gone above and beyond to further my faith in His providence.
I love this description of what providence is in relation to God: Providence, then, is the sovereign, divine superintendence of all things, guiding them toward their divinely predetermined end in a way that is consistent with their created nature, all to the glory and praise of God. This divine, sovereign, and benevolent control of all things by God is the underlying premise of everything that is taught in the Scriptures.
Ever hear about God placing people in your path for His purposes?
I know that every step of our journey has been just that...people placed in our path based on His plan...our us placed in their path for His plan.
Most recently, our paths crossed with a doctor in the PCICU who in no uncertain terms has changed our lives and more importantly, bettered and, I'm positive, prolonged Everly's.
Dr. Julia Niebauer is an angel. A real, doing-works-on-earth, angel.
She is an out-of-the-box-kind of doctor. She didn't just treat Everly for the acute illness. No, ma'am.
She looked over her chart, her labs, her history. Every single detail.
Then she leapt into action. And I mean literally 0-60 in 5 seconds!
To try to give a play by play of the last two weeks, this would turn into a small book. So, instead, I will just cover the highlights.
Everly's Feeds/Weight: Dr. Niebaur inquired about her feeds and the fact that she wasn't consuming enough calories. I was taken aback that she had noticed because I have felt that for the last two months and even went to our GI to address this. She also asked if we had ever tried continuous feeds (feed running continuously for a period of time). I responded no but I was open to any changes, especially when it will help her to gain. So, literally that night she started continuous feeds from 8pm - 8am. Everly's body did well with the change. From there, Dr. Niebaur continued to tweak her feeds, both timing and volume, until currently she is at the optimum schedule. Prior to the changes, Everly would eat every three hours over the course of an hour and fifteen minutes...around the clock. Each time, the process was the same: check placement, residual, heat food, clean food bag if pump feeding, if not, then process was longer because we would feed her with syringes (bolus feed) by hand over that hour time frame. Now she eats at 11 am, 2 pm, 5 pm over a half hour period and then has continuous slow feeds over a longer period of time (12 hours) all via the pump. Plus, this allows her to receive more volume (=more calories=more weight gain) overnight. Yay! I'm normally not one to embrace change but this is one I'm digging! Oh, AND, Everly gained a whole pound while in the hospital over 2 weeks!! Currently 12 lbs 8 oz!
Everly's Meds: Another positive change that was made...ditching the caffeine that Everly has been getting since NICU! She received it because of her central apnea. However, while we were in the hospital, angel Dr. Niebaur asked why she was taking it. After we told her, she said that they hadn't recorded any apneic spells while there since they monitor her 24/7. She stated that it is possible to outgrow this apnea and would we be game to try to titrate her off the caffeine. You betcha! The least amount of meds, the better, I say! So, that we began the process the very next morning. Within days, Everly was completely off of the caffeine...had to go slow due to side effect of possible headaches (you know how you feel during a caffeine withdrawal!). Praise God for this step!
Everly's NG Tube: Another concern, angel Dr. Niebaur had was of Everly's NG tube. She at first was worried that she may have been silently aspirating and that was part of the reason she had pneumonia from continued build up in her lungs. She consulted with Dr. Pham and he came to see us and together we came up with a plan. We would take her to nuclear medicine for a silent aspiration test to see if she was or was not having this issue. If she was, then step one would be to replace her NG tube that runs to her stomach with a longer one that would go lower and not allow the silent aspiration. The problem with this solution for us is that the tube can only be reinserted in the hospital not at home how we'd been doing it. Also, when it came time for a G tube surgery, she would need an additional procedure and a different kind of tube set up which is not what we felt was ideal. However, following the test, we found that she in fact does NOT silently aspirate so the NG can stay as is and there would not be any other kind of G tube needed. Another praise report!
Everly's Immune System: Well, we already knew she was medically fragile and ridiculously susceptible to illness. While inpatient, we also learned just HOW fragile her immune system truly is at this point. Dr. Bash, our hospice doctor, had her system tested to see what her antibody levels were overall. Found that she has something called Hypogammaglobulinemia which means that she has an abnormally low level of immunoglobulins (antibodies also called proteins that help fight infections). The thought is that she has more specifically Transient Hypogammaglobulinemia of Infancy which means she would grow out of this between the ages of 2-5 normally. Basically what this means is that to protect herself she requires an IV infusion of antibodies in the hospital or immunology office every 3-4 weeks. So, the poor baby has to get an IV each time and the infusion lasts between 2 to 6 hours, depending on how she can handle it. She received her first infusion inpatient at St. Joe's the night the blood test came back. They wanted to protect her right away.
What does this mean for us as a family? Well, that means that we are going to be the Germ Gestapo now as if we weren't before. So, in a nutshell, our home will become (like it almost is) our safety shell for her. Our outings with her during this Sept-Mar yuck season will be severely limited when it involves crowds, groups, etc. We will stick to our hikes, park outings, walks and the like. We'll play lots of games, build lots of puzzles and finally finish the last seasons of Little House on the Prairie! We will divide and conquer for errands and such...one will stay home with Everly (like we already do actually) while the other goes with the boys and/or by self. After April, we should be able to open the bubble a little more. I know that some are able to take their fragile T18 babies out and about but after now at this sitting 3 hospital admissions, 2 ambulance rides and upwards of a month sleeping with worries and fear--I'm happy to remain close to home where my girl is safest. Please understand how crucial these precautions are to her ability to live...you see how fragile she is. One common cold has done all of this to her. And the third admission is even AFTER the IV infusion so that's not a magic pill by any means. Friends, you have done an amazing job helping us keep her safe for 10 months and we just respectfully ask that you continue to walk with us on this journey. I've never been around anyone so fragile with their immune system and the doctors have even told us that it is surprising (interpret as a MIRACLE) that she hasn't visited the hospital or at least had one sniffle before 9 months! So, something we are all doing has worked, right??!! **My boys really would like to get out and hang with friends and now that we really can't have friends over to our house, they would love to have some invites. Yes, that was shameless begging maybe. This is a HUGE transition now even more for them as our circle has shrunk EVEN MORE. Thank you for your love for all of us :-)
Everly's Heart: Her sweet, tiny heart. Well, where to begin really. I have posted before that she has a very large VSD and that her lung pressures have been balanced since birth. She's had pulmonary hypertension since birth and since it's a progressive disease, it will only get worse with age. Once she got this Rhinovirus, it seemingly has altered that balance ever so slightly. The doctors felt like this was the right time to test her reactivity to a vasodilator like nitric oxide. "Vasodilators are medications that open (dilate) blood vessels. They work directly on the muscles in the walls of your arteries, preventing the muscles from tightening and the walls from narrowing. As a result, blood flows more easily through your arteries, your heart doesn't have to pump as hard and your blood pressure is reduced." If her body had not reacted--her SAT's quickly rising to mid-90's--then we would know her body had passed the window of opportunity to use these medications. The pulmonary hypertension would have been considered irreversible at that point. Note: if a person's body is found to be reactive still and they use the vasodilators, it does not mean that the PH will never come back just that it can be managed. Well, the good and exciting news for us it that her body was still reactive to the nitric oxide! And immediately! So, cardio started her on Sildenafil, liquid Viagra, and the oral version of nitric oxide. FYI: Viagra was first used and made for this purpose. The "other" was found to be a side effect and marketed to that population after the fact. This dosing of Sildenafil will help to manage this PH until further plans are made to investigate further the option of heart repair.
One of the most exciting things to happen during our stay was a pow pow that occurred among a small group of physicians regarding Everly's future heart repair plans. I told them as we began our stay that our goal is to repair her heart to the extent that it is a feasible and logical option for Everly, not just because a surgeon can do it. Following that meeting, our cardio--the one we've had since NICU--came in and after a long day for her in clinic and then a heavy, medicalese-laden conversation, told us that she would do the heart catherization needed to see if open-heart surgery (repair) was an option.
Why is this important?
Simply this: Cardio had told us from day 1 that a heart catherization was not an option for patients with Trisomy 18. Bigger than that, without this procedure that measures all sorts of complicated vitals heart-related, heart surgery is not even on the table.
This, my friends, is A BIG DEAL!!!
FYI: The date was Dec.1 so it's now a recorded date and a family holiday! lol
So, here's the part that is important to remember: just because a procedure--like open heart surgery--can be done, doesn't always mean it should. For example: trying to repair her heart with the balance that she's had over her life would most likely kill her. Why? Well, it's complicated but trust me, not an option.
Herein lies the necessity of this heart catherization. "It is a surgical procedure used to evaluate heart function and diagnose cardiovascular conditions. During cardiac catheterization, a long narrow tube called a catheter is inserted in an artery or vein in your groin, neck, or arm. This catheter is threaded through your blood vessel until it reaches your heart. A dye can be injected through the catheter to allow the doctor to look at the vessels and chambers of the heart with the use of a special x-ray machine. Once the catheter is in place, doctors can use it to run diagnostic tests." Basically, this can help the cardiologist to determine the value and benefit of heart surgery.
Our nighttime gal pal, Laura! Always a smile and even Everly is amused!
She's on it and even thinks of things we don't!
The heart is complicated and one change affects something else. So, for example, the concern that we have for Everly still needing O2 is that because of it being a vasodilator, it will hasten the change in balance she currently has. If she is preparing to go into surgery for her heart repair, then this is what is needed and the protocol would be for her to be on continuous 02 and a med like Sildenafil. However, if she is not heading into that scenario, the concern would be altering an otherwise "stable" balance that is allowing her to have the kind of life she has without aggressive intervention.
So the next BIG news...is...are you ready???
There is an amazing surgeon who has agreed to do Everly's heart repair here at St. Joe's if it comes to that!!! God put the right people in place at the right time with the right heart. That is all I can say. This is AMAZING! Even if heart repair is not an option physiology wise, I will always know that someone deemed MY child's life AS VALUABLE AS ANYONE ELSE. I am humbled and so grateful. God is SO good.
NEXT STEPS: As for our next steps, the plan is to schedule the g tube surgery as she's approaching the year mark. Next, depending on we feel with the response her body has to anesthesia and the like, we will then schedule the heart catherization. Following that, we will have to wait and see the results from the heart cath to see what should be the appropriate next steps. By the way, we are not going to have the malrotation of the intestines corrected at this time. People can survive without that fix for quite some time; you just need to be aware of the danger signs. **Note: I believe due to the nasty germ season we are currenly in, we may wait until spring to schedule the surgery. Right now, germies are everywhere and are dangerous, especially in the hospital so if we can avoid it during this time, all the better!
Love Kathy! She's always cheerful and wanting to know how she can help you! A doll!!
Other: Everly is just so special to all of us! She has these piercing eyes that look as if she has something prophetic to say. She has clear likes and dislikes. For example, she loves to touch hands, fingers, facial hair, or just about anything else you keep close to her. She enjoys lots of stimulation and can get easily bored when there's not much going on around her. She recognizes faces and people and will smile to let you know. She loves her brothers greeting her in the morning and LOVES to watch them play together. She's extremely content lying next to them just attentive. She is so joyful and rarely cries and then it's for a specific reason, to be held, moved, changed or something like that.
Pediatrician: Most of you know that our beloved pediatrician is moving to another type job right after the new year and we will need to find another. Just to update you: my search is turning up a lot of No's. Meaning they upon learning of her diagnosis have felt like she'd be better cared for by someone else...that's me putting it in a nice way because, honestly, my daughter has every specialist under the sun, all she needs is a ped who can see her for shots, monthly growth charting, yeast infections, whatever. Can't a regular ped do that for ANY child, regardless of special need? Please, please pray for us to find THE one that was called to care for Everly because mama is discouraged. DISCOURAGED. Maybe I should send them a video of her smiling and interacting and being very TYPICAL??!! FYI: Taking names, suggestions and if you HAPPEN to be a pediatrician reading this and you know how deserving she is of quality and equal care like any other kid, pretty please contact me?!?! SHE IS WORTH THE EXTRA TIME A VISIT MIGHT TAKE!!! crystaldmm@earthlink.net
One Last Thing: As you can all see from our experiences as of late, it doesn't take much to take down a baby with Trisomy 18. So, please keep our whole community of kiddos in your prayers this germ season. Our friend, Elizabeth Maxine in Austin, and all our other little friends need your prayers. Please pray that Everly has had her LAST hospital stay until her g tube surgery in the spring. St. Joe peeps--we love you better from afar! lol
The Nitric Oxide set up. Dum dum dummm.
See why it's taken me SO long to write this up?? It's A LOT of information and we were slammed with lots over the course of two weeks. But now you are caught up to date with all of Everly's medical stuff for the most part. There's always more but that's another message on another day.
Thank you everyone for lifting us up in prayer and specifically for lifting Everly's name up for healing. This has been a very difficult time on the whole family. Here's to looking at 2015 with good health for all! Before I forget, please pray for our whole family that we don't get sick because then we would have to stay away and that would be hard, too.
With love and appreciation,
Crystal
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