So, we arrived at the hospital Admitting office and they didn't even know we were coming. They didn't have any idea why we were there. So, we waited...
...and waited.....
....and waited.....
Almost an hour and a half later, we were registered and got sent up to our room. We arrived in a room that had a child's hospital bed in it, so that was moved and a crib brought in, and it was a corner room so it had 2 "recliner" chairs for us to sit/sleep in, no couch or anything. We weren't told what was going to happen, or even how long we were to be there. We really hoped it wouldn't be long! Thank goodness Grandma Brenda was able to watch our dear Liberty while we were here.
The first night was ok, we were told that I had to pump and give bottles to Harmony, so we could track how much she was getting, and we were "watched" during feeding times and diapers were weighed, vitals were checked and we uncomfortably slept while trying not to worry about our little baby in the hospital crib next to us.
We got some take-out delivered from the local chinese place, had Liberty and Grandma Brenda visit and then tried to go to sleep.
The next morning the nurses came and weighed her, and she gained a little. So, we were on the right track, hopefully they would see that she's gaining and we can go home!! We got some blood tests ordered and drawn and were also watched again and diapers were weighed again. Most of the nurses didn't even know why we were there and kept saying that no one has really told them the plan either. Well, good...I'm glad we aren't the only ones.
We asked about more comfortable accomodations on the 2nd day because we had seen couches in other rooms and if we were going to be here a while, we'd like to be able to stretch out on something at least a little bit. We were moved to another room, ordered pizza for dinner and tried to sleep again.
The next day was more of the same, although a bit more relaxed being that it was a Saturday. Harmony was weighed and she had gained and the doctors came to visit and told us that one more day of gain and we can go home. YAY!! They started her on Zantac for the spitting up and we hoped that would help the reflux problems. We hoped that this would help her gain weight and we would all be fine after this.
At this point, we kind of felt like nothing was accomplished, besides a lot of watching us be her parents. Were we being accused?? Had we done something wrong in their head that we needed watched?? We FEED her ALL THE TIME!!! She spits up, I don't know what else to say...that's all I can contribute her weight loss to.
The next day was the same, a little gain and the doctors came to visit. They made good on their word and we were able to go home!! They started the discharge paperwork and we packed up Harmony and the rest of the stuff we gathered while we were there and waited to go home.
Upon discharge, we were told to go for another weight check at our pediatrician's office in a week. We were given a hospital-grade pump for rent so we could continue to pump and feed her at home.
We celebrated Halloween and went trick-or-treating! How fun! Harmony was Minnie-Mouse and Liberty was Rapunzel (underneath her winter coat)!
We fed her, gave her medicine and kept track of her intake and output while at home. We went for her next weight check and she had gained a little. See again in a week for a weight check.
The next week, her weight went down a little and I was in tears. This was ridiculous, we just wanted her to gain so that we didn't have to go back to the hospital and be away from our family again. The doctor consulted GI (since they had seen us in the hospital) and decided that in another week, if she was still down, that we would have to talk about being admitted again.
The next week was the week of Thanksgiving. We were planning for the dishes we were going to make and bring to all the holiday happenings. We were excited and hoped that "she looked fatter, so she must have gained". We went to our appointment and she had lost. Not a lot, but enough. GI was consulted and they suggested re-admitting her. Both the pediatrician and I were dumbfounded and didn't know what was going on. When he said we have to get re-admitted, we said no. We wanted to celebrate Thanksgiving with our families...nothing was going to happen in the hospital until the holiday was over anyway, so just let us have our holiday and we will report to Admitting again on Monday. The pediatrician and GI both decided that was an ok plan. So, we went to celebrate Thanksgiving.
We spent some time at my parents' and some time at Bryan's Aunt's house. We have such great family and we were happy to get to spend the holiday with them!!
Monday after Thanksgiving came too quick! We arrived at the hospital in the morning and were told that now Liberty was not allowed to come up to the rooms because of flu season. We didn't have anyone to keep her for however long we were going to be there this time, and we didn't want to keep interrupting her schedule because we were gone. So, our plan was to alternate. Mommy stayed with Harmony while she was admitted and vitals and everything again. A pump was brought to get milk to feed her and her weight checked so we could get a baseline. The rest of the day, nothing happened. Harmony and I just hung out all day. Daddy came to spend the night and I went home to see Liberty.
We woke up around 9am the next day and called Daddy to find out what was going to happen. He said that they are setting her up for a swallow-study so that they can see if her swallowing was an issue and to see how much of a problem the acid reflux was. They gave her some barium and did the test. Nothing looked abnormal besides the acid reflux and there was a lot of that, but nothing else they could see. Harmony had lost a little at this point because they kept her NPO (nothing by mouth) for a while for this test so that was to be expected. We would hope to see a gain the next day. Daddy went home with Libety for the evening/night and I stayed with Harmony for the night.
The next day there was a gain and we were happy. Nothing else really happened besides maybe a few more blood samples for more blood work and Daddy figured out that he could stay with Harmony and I that night because his mom (Grandma Brenda) could take Liberty from Wednesday night until Friday morning. Great!!
The next day was Thursday and we were hopeful for another gain, which there was! The doctors visited and told us that they want to try to feed her with a tube that goes straight from her nose to her belly (Naso-Gastric tube, or NG tube) and feed her with a pump. We weren't happy about this, but we figured whatever helps! I went to go get us lunch downtown while Daddy stayed with Harmony and they placed the tube. There was some controversy over the tube because they wanted it when she was losing weight but since she was gaining, they weren't sure. They said yes, then no, then all of a sudden yes again. So, when I arrived back at the hospital with our food (finally...after fighting downtown traffic and issues with parking), the tube had been placed and my beautiful little girl had medical tape all over her face!! :( I just had to keep reminding myself that this was what was best for her....and she's still beautiful anyway!!!
We were then scheduled for a "class" to go over placing the NG tube ourselves when we're home (once a month and as needed if she pulls it out). We scheduled this early the next day, hoping we would be getting discharged at the same time. We also got a visit from a nurse that taught us how to use the pump and got all of our supplies for going home. We saw an end in sight...we also had to pick up Libby around 1pm the next day! We were going to be busy!!
The next day, she gained again, the pump and NG tube feeding were going well and we were told we'd get to go home!! It was now December and we were so ready to get her gaining well so she can stop being bothered and poked and prodded all the time! We went to our class, we packed our stuff up, along with all the stuff we had now accumulated in our week there, and we were off. We picked up Libby and got home, hopefully to settle down and have things go well!!
We had scheduled a GI appointment and we were monitored outpatient. We had some adjustment to medications, as Harmony was starting to have issues in her Liver Profile tests and we felt that she was "getting used to" the Zantac as she was starting to spit-up entire feeds again. We were also told to stop breastfeeding altogether, and go straight to the formula Alimentum (which we had tried for a week or so mixed with breastmilk). We also got Harmony's first set of shots and her first Audiology evaluation in December. We found out that she cannot hear normal speech sounds, and that she has moderate-to-severe hearing loss in both ears. The test showed that she could hear very low sounds and if you were close to her, she could hear you, but that was it. At this point, we were told about hearing aides and further testing and evaluation. The test could not distinguish whether it was complete sensorineural hearing loss or a mix between the sensorineural and conducive hearing loss. We were told that there could be some fluid behind the eardrum and we could get tubes to drain that to see if there would be an improvement. We were set up for an ENT/Hearing Clinic appointment and were sent on our way.
We got the girls' picture with Santa and we think we did a pretty good job of hiding her NG tube!!
We had our family celebrations and talked about the tube since our families had never seen one. We had a great time, even when Harmony pulled out her tube on Christmas Eve at like, 1am after we had just gotten home from Christmas Eve at Bryan's Aunt's house. It was so nice to relax with family and have no worries for the time-being.
Shortly after Christmas was our ENT/Hearing Clinic appointment. We talked about putting tubes in her ears and we planned on it for January. January came and we had her surgery. Everything went well and the doctor said he drained a lot of fluid from both of her ears. The tubes were in, we were sent home with instructions for ear drops and had to set up another Audiology appointment. We made this appointment for February. We also set up an appointment to see Genetics. This appointment was in January and Bryan took Harmony since I had gone back to work and was sleeping between night shifts or napping before my first night back. The Genetic doctor didn't mention anything that he could think of right away and ordered some general testing. Harmony had developed quite a diaper-rash at this point, so Bryan took her to Urgent Care right after the Genetics appointment so he could get the rash taken care of. We got some cream and were told that her acid reflux was causing a lot of acid to come out the other end and that was burning her skin. The general testing came back for Genetics and found a small mutation, but nothing that we should really be worried about, as it was in a "non-coding" region. At some point in January, we also had an abdominal ultrasound to make sure there were no blockages and no abnormalities. Everything was normal. Finally...a little good news.
One of our GI appointments after the Genetic evaluation, I went to with Harmony alone. I was told that the genetic doctor had mentioned a disorder called CDG or Congenital Disorder of Glycosylation. There was no mention of this at the appointment, but I'm guessing after our visit, research was done and the clinical presentation of her symptoms so far seemed to fit. GI seemed to also be concerned about possibly Cystic Fibrosis...so a sweat test was ordered and came back negative/inconclusive (as 2 samples were supposed to be sent, but only one could be collected). We weren't told to retest though, so we moved on.
While December and January were relatively uneventful with no hospital stays and no major gains or losses in her weight, we went to our next appointment for GI at the beginning of February and were told that her weight had dropped and she wasn't gaining enough for their standards so we have to re-admit her. We laid down the ground rules, it would only be for 2 days, and both days better be busy in terms of getting testing done. We didn't want any real downtime and if we stay over the weekend, when nothing really happens, we will start making calls to go home. The doctors made calls and we were admitted at 6am the next day. We had an MRI of her brain at 9am and saw Ophthalmology in the afternoon. Ophthalmology said that her eyes look normal and they want to see her when she is 6 months old. Her food wasn't started until almost 5pm that day(!!). Needless to say, we were a little upset about that. She had been starving since 2am so she could have her MRI!!! It was now almost 16 hours later....and you want her to GAIN weight with this??!! I went home to be with Libby overnight and so that Bryan could get the MRI results without me. I'm a little bit of a wreck about all of this, and I just couldn't handle anymore bad news.
The next day, she did gain weight and everything was good. The MRI came back that all structures look normal, besides the cerebellum being undersized. It was explained that this is the growth center and that is why she isn't growing as expected. This also fits with the CDG that they were thinking about. After more research, this is also the movement and balance center so there is a possibility that she may not be able to walk on her own...but that's all in the future. There was some blood drawn for the transferrin (Tf) test to confirm CDG and we were discharged. I went to the hospital to pick up Bryan and Harmony with Liberty. We were just glad to get to go home.
We were then seeing GI every 2 weeks and getting things adjusted as her weight improved. They tried to place her on a new recipe for a thicker formula and that didn't work. We were seeing gains in small amounts but no more admits. We got adjusted back to a formula recipe that had worked in the past and placed her on continuous feeds at a slower rate. We got a call the same week that confirmed that she has CDG and now we need to get a type. We received the paperwork, and have yet to get the blood drawn. While being confirmed, we were also told to start Harmony on D-Mannose to supplement her formula. We started this right away. After about 3 days, her spitting-up had decreased significantly and after about 5 days, she was more alert during the day and she liked to be held, talked to, and moved around a bit. We don't know for sure if it is because of the slower feeds at a more consistent rate or if it's the D-Mannose, but whatever it is, we'll take it!! We love our happy little baby!
She had her next Audiology exam and we were told her hearing had improved by 15db and the hearing aids were ordered and her earmolds were done. The process took a couple hours and we were told that we would get her hearing aides in 3 weeks!! How exciting!! At the time of this post, she will be getting the hearing aides in about 6 days!!!!!!!
At the last GI visit, she had gained almost 5 oz!! This has been the biggest change in weight so far and we are so pleased!! The GI doctor also said that things seem to be going well enough that we don't have to go back for a month!! YAY for good news!!!
We have also had home-visits with our local Help-Me-Grow Program and our state insurance program, just to check and see if we need anything right now. We will probably be starting physical and occupational therapy soon and GI is talking about a G-Tube and Mic-Key button. These things can only happen after a few other specialty visits, though (Hematology for blood clotting issues that are common in CDG and Cardiology to make sure she doesn't have any heart issues before surgery). We have also had some great support in our church and have had a special prayer circle after service one day. Thanks to all that came to support us and pray for Harmony.
We hope that our little Harmony starts getting bigger and we start having more good news come our way, until then...we just hope for the best and take it one day at a time!
Special thanks to our family for being there for us during our hospital stays and doctor appointments!!! We thank everyone also for your prayers, support, and mental <<HUGS>> .
Also wanted to post a special thanks to my ex-husband/his family, who has helped us with Cassidy and Zachary when we've needed them to. As much as we don't get along, it is really nice to be able to have his or his family's help since this whole hospital stays and many many specialist appointments is just so new to us...
We will update more as we know more!!!
