Monday, March 18, 2013

Can you hear me now?!

Well, Daddy went to go get the hearing aides with Grandma Brenda while I worked on the 14th of March!  I was kind of a mess that day at work because I so wanted to be there, in case there was a huge reaction.  I had actually sort of planned to be off at 3 that day as my aunt and uncle were also coming into town for a night and I hadn't seen them in years!!  I planned dinner with them and my parents and was so excited that they would get to see the hearing aides and everything too!  Well, the day didn't go as planned (as usually happens) and I ended up cancelling dinner and hoping I'd make it the next morning to see my family.

I worked until almost 8pm that day and when I heard from Daddy about Harmony's hearing aides, he said it wasn't that great.  He said the earmolds didn't fit and he would show me everything they got at the appointment when I got home.  I got home just in time to put Big Sister Liberty to bed and then Daddy showed me everything!  They gave us a little pack of all the hearing aide "stuff" we needed.  Cleaner, batteries, some paperwork.  They even gave us a book and puppet to explain to other kids what the hearing aides were for.  We ordered some trendy zebra striped hearing aides with bright pink molds.  We ended up getting clear light pink molds because the bright pink doesn't come as little as she is.  But that's ok.


Well, she was recasted for her molds and this time we will get them sent directly to us in about a week and a half.  Hopefully they won't be too small again.  Right now we only use them when she's laying flat and we know they won't fall out or get lost somewhere.  If I can figure this out, here is a video of Harmony trying out her hearing aides at home.



We did end up getting to see my family members the next morning and showing the hearing aides off a little! 



We also got to use them at a birthday party we went to this past weekend and she seemed to really like it!! 

 
It's weird, we haven't had a doctor's appointment since then, and I have been off 4 days in a row (I go back tomorrow morning!)!  This has been a great weekend!!  We have an ultrasound coming up this Friday to make sure there was no scarring from the UTI that Harmony had about 3 weeks ago.  We don't go back to GI for about 2 weeks!!  Hopefully everything continues to go well! 

Until we have more info...thanks for keeping up with Harmony's Smiles!! 

Friday, March 8, 2013

Testing....Testing....

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!!! 



 

Thursday, March 7, 2013

Where to start???

Introductions (for those who don't know us!):

I'm Nicole:  the Mommy of the group and an LPN for a nursing home near us.  I also sell Living Lockets for Origami Owl, because they tell a story and mean a lot to people (and myself).  http://fergusonfamily.origamiowl.com if you want to look at the products!  Bryan: the Daddy and an awesome Real Estate Agent for Keller Williams.  We have 4 wonderful kids.  Cassidy is 5 and Zachary is 4, they are from my previous marriage.  Liberty is 20 months and Harmony is going on 5 months.


Harmony's Beginning:

In February of 2012, the whole family got sick.  We got this awful stomach bug that was "going around" and everyone got it one right after another.  The pattern was, a little sick the first day, a lot sick the next and then no sick the day after.  Well, this pattern stayed the same until I kept getting sick....for almost a week.  I guess it was time to take a pregnancy test.  I took one and it was positive!  There were a lot of mixed emotions, we weren't prepared for this, we just had Liberty 8 months ago!  And, I was on birth control, called the "mini-pill" which my dr. gave me because I was breastfeeding.  Well, all at the same time, Liberty started refusing my milk, we were all sick, and now I'm pregnant!! 

I went to the dr. in early March and he was as surprised as me that we were pregnant again!  He said "I honestly can't believe you got pregnant using the "mini-pill". So we did a regular ultrasound at the same time to check for dates.  We were due October 13th!  7 months away!!  Our kids were only going to be 15 months apart...not quite Irish twins...but close! :)


 As a concerned Dr., he also wanted to set me up for a Level II ultrasound at 20 weeks, "just in case".  Now we had to wonder, what was "just in case", what did he see that we didn't?  What wasn't he telling us?  So, worried, but hopeful, we went to the Level II ultrasound near the end of May 2012.


It's a GIRL!!!  YAY!!!  We love girls...and our boy...but little girl clothes and bows and tutus are just SO CUTE!!! I got to see a Dr. I used to work with and she was pregnant also, so it was fun to talk about our babies and have her explain, in detail, everything they were looking at on the ultrasounds.  Obviously, we found out the gender and she looked at her brain, heart and other organs.  She said everything looks great, she just wanted to see us about every 8 weeks to keep checking on her.  We scheduled our next appointment and got to go home with some great pictures of our new little baby girl! 

The next appointment was mid-July 2012.  We came in and were told that our Dr. was on maternity leave and we will see someone else.  They checked the same things, the brain, heart and organs, the bone structures...everything looked great!


Everything was so great, in fact, that the dr. didn't see any reason why we need to come again in 8 weeks for another ultrasound.  We, again, got to go home with some great pictures of our developing baby!

The next 12 weeks were uneventful.  They dragged on, as most 3rd trimesters do.  We were just excited to meet the new little girl and have her brothers and sisters meet her as well!  She moved and kicked and we just kept building up to meet her!!  In October, she still hadn't decided to come on her own, so the dr and I came up with a plan to induce on 10/12/2012. (We were hoping she would come on her own on 10/11/2012....10-11-12 would be such a fun birthdate to have!  and, especially knowing that we had "scheduled" her to come the next day!)  She was stubborn, though, and decided to wait for us to begin the process without her.

We were to report to the hospital at 5am (!!) for the induction.  Of course, we couldn't sleep the night before because we were so excited...so we show up exhausted!!  The IV and medication started at like 6 or 6:30am.  Everything was going well, and started to go fast around 11am.  I got my epidural and I was comfortable enough to take a nap.  Then, my dr. told them to "hold off" for a couple hours as he was scheduled for a procedure in the OR at 1pm.  Ok, so everything then slowed down, and they really hoped that the meds wouldn't be needed anymore at this point, and I would just keep progressing.  That didn't happen, Harmony was still not ready to do this on her own!  So, around 2:30, the medication started again and things started to go well.  I was still comfortable and we were getting impatient to meet our new baby girl!!  About 4:00, the nurse walked out and I started to feel like it was time.  So, we immediately called her back in, she checked and then called the dr.  It was time!!! 

Harmony arrived at 4:25pm on 10/12/2012! 



She was a loud little one!!  She came out screaming!  Bryan says the dr. jumped out of the way of the fluid that followed Harmony upon her arrival.  Bryan describes it as "gallons".  (TMI, I know).  She was 6 lbs. 14 oz.  My smallest baby...and the loudest!!  She stayed in our room all evening and she was loved on and cuddled a lot!  She got her first bath and hated it....she was swaddled and held and loved it!!  I breastfed her a few times and everything went great.  She did start to gag a little after her feeds, but we didn't think anything of it and the nurses didn't seem worried either.  Looking back, that was our first little insight into what we would be dealing with later...acid reflux issues.  We had them take Harmony for a couple hours overnight so we could get a little sleep.  The next day, the pediatrician came and said she looked fine but that they did the hearing screen and she failed, so they will try again tomorrow because sometimes it just says "refer" for no reason, and my dr. came to see me to see if I was ok.  We were concerned about the hearing, but other than that, we were just happy to have her in our family and couldn't wait to share her with our family!! We had visitors and everyone loved her and held her.  She seemed happiest being held and swaddled.  She hated being alone in her hospital crib. 

We spent another night at the hospital and the next day they did the hearing screen again and then referred us to Audiology for further evaluation, as the test they did didn't gather enough information on her hearing.  We were, again, concerned, but the nurses said not to worry as "only about 3% of babies really have a hearing issue after getting a "refer" on the hospital test.".  Ok...well, we hoped she could hear and we didn't want there to be any issues with our perfect little one that just arrived in our world!!  We were also told that we could go home, but that Harmony had lost about 10 oz. while in the hospital.  This was not concerning because it wasn't 10% of her total weight, but it was close.  We were to follow-up with our pediatrician in about 3 days and schedule the audiology appointment as soon as we could. 

We got to leave...finally!!

Back at home, we were busy!!  We scheduled our appointments and settled into our home with our new little one!  When we fed her, she would still gag, so we elevated her a little in her bassinet.  She didn't want to be alone so she ended up sleeping in our bed the first couple nights, while being breastfed nearly all night.  During the day, she really liked her little vibrating bouncy seat and being swaddled.  She slept a lot and cried a lot.  Life with a new baby. 

We went to her first dr's visit, and found that she had lost 2 more ounces.  How could this be??  She was eating ALL the time and spitting up a bit, but we still didn't think aything of it.  She got a heel stick for her bilirubin level and were told to come back in a week for a weight check.  That week, her spitting up got a little worse and she seemed to be either crying or eating and sometimes crying while eating.  We got a call that her bilirubin was pretty close to normal, but to supplement her with a couple bottles of formula anyway, to get the level to come down a little more.  So, that's what we did.  At her weight check a week later, she was down 3 ounces.  Now, we were stunned and confused and the pediatrician was too.  What could be causing this?  The dr. told us to come back again in 3 days and if she was still dropping, we would have to be admitted to the hospital.  We certainly didn't want that, so we tried to feed her all the time, and take a diary so we knew when she was eating, how long she was eating for and then her diapers (I'm a nurse, so it was like doing our I's and O's - Inputs and Outputs).  We tried to be vigilant so that we knew that she was going to gain SOMETHING!!  Well, 3 days later, and she was down again, just one ounce, but enough that the dr. was concerned, she was now 5 lbs 14 oz.  He told us to go straight to the hospital... 

We weren't ready...we weren't packed...we had unpacking still to do from being in the hospital to bring her into the world...but we off we went to Admitting....

To be continued....