The Easiest Way To Update

I haven't had much time to update the old blog, it's been quite a crazy year since Charlie joined us with many ups and downs. I won't be going into all of those right now and unfortunately my reason for blogging again is not one that I'm really excited about. With everything that's been going on in the past couple of days it's just easier to go back to the old way of updating everyone.

The best place to start is the beginning. Our little Amber has been fighting some nasty on and off viruses and respiratory infections since October (really even before then too). She's been on and off antibiotics, additional breathing treatments, comfort meds and increased O2. Finally right after Christmas she seemed to have finally kicked it but really wasn't back to 100%. New Years Eve she started to have some breathing issues again and her heart rate starting creeping up. We really didn't think much of it since she always has some apnea or other breathing issues after a prolonged illness because her body is so tired. Usually it last a few days to a week once she starts to really recover and gain strength again.
Unfortunately it seemed with each passing day her night time breathing was harder and her heart rate continued to climb. By Thursday night she was really starting to have really bad  nights. Each day and night has been worse with desaturations/apnea even during her nap times (something she has never had before). By Sunday night she was up to 4 liters of O2 (the highest we could go with her cannula) and on the maximum amount of comfort meds we could give at home with no signs of her getting better or giving her any relief.
Last night we made the decision to have her admitted to the hospital. Last night was a very rough night for her. Her heart rate would not come down from the high 150's to low 160's. It has been this high at home but she was having some periods where it would come down into the 120-130 with sleep. They started her on a CPap machine via a very large cannula and that has helped her O2 levels stay where they should, as long as she keeps it in her nose otherwise she plummets into the 70's. They also increased her doses of comfort meds and decreased the time between the doses. She finally settled down around 2am and kind of slept until 7:45. Her morning has been some what better. She has a small chunk of the day where she has decent energy levels and she tries to play and talk to us. Today she was definitely struggling more during this time but she is such a fighter. Unfortunately her heart rate has not been improving with medication today and at the lowest has been 144 awake. She is sleeping comfortable right now and thankfully her heart rate has dropped into the high 130's.
We are still waiting for a cardiology consultation to see how bad her heart is and to get a better idea of what is going on. It is a horrible waiting game. When she is awake she tries so hard but the nights are so rough for her. We could increase her comfort by giving her meds through an I.V. but she would not be able to go home on it. She is doing somewhat better on the CPap but there is no home cannula option she would have to tolerate a mask which she will not. Her poor little body has been working so hard for so long. We are praying that we can keep her comfortable and that she will pull through this.



It's very strange to be sitting here in the hospital again on the same floor we were on almost 3 years ago, in a room that looks exactly the same as the one she was in while she recovered from her hernia surgery. I remember talking about similar things and even watching her struggling then.

She was so so tiny back then!

 It amazes me how much she has gone through and how far she has come. We were so blessed to celebrate her 3rd birthday with her this September, something that at one point looked like we would never be able to do.

"Therefore we do not lose hope. Though outwardly we are wasting away, yet inwardly we are being renewed day by day. For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. So we fix our eyes not on what is seen, but on what is unseen, since what is seen is temporary, but what is unseen is eternal." 2 Corinthians 4:16-18 (NIV)

"For I know the plans I have for you," declares the Lord, "plans to prosper you and not to harm you, plans to give you hope and a future." Jeremiah 29:11 (NIV)

Poor Little Amber

She is still very sick :(

This picture is from last Tuesday on our way home from her appointments.

 She fell asleep while she was trying to eat the tag on her doll. 

This Tuesday she did not want to get out of bed!

The pneumonia Amber was fighting last week is gone but unfortunately over the weekend she came down with a viral infection. For Amber viral infections are bad news. There really is not much we can do for them medically, no antibiotics it just has to run it's course. This one has hit her pretty hard, it didn't help that she caught it at the end of having something else, it's been rather touch and go all week. We have her on the highest flow of O2 she will tolerate. We're keeping up on her medications to help fight secretions, fever and we have her on one of her comfort care meds to help with breathing and heart rate. We've gone to all feeds through her g-tube and feeding her only pedialyte. So far things have been going alright, it's been a struggle for her but we've been managing. 

Last night and today she went back to demanding a bottle and tolerating it pretty well. She seems like she's getting over the viral infection but unfortunately her poor little body is so tired from fighting that now she's having breathing issues. All day today she's been very lethargic and having episodes of her O2 levels dropping and some mild cyanosis. At this point we're concerned that her CO2 levels are on the rise again. There isn't much we can do for this. We could bring her in and put her on a CPAP machine, something we've ruled out before because we don't think she would cooperate with it and could make her more upset. Her levels were elevated last spring but came down on their own once she was feeling better. We're really praying that this is what's going to happen this time around. We'll be keeping an eye on her tomorrow and seeing how things go.

Feeling a little better yesterday, playing with Daddy.

This also means the appointment I made a year ago for her with the augmentative speech clinic will have to be canceled. This is really a bummer to me because I have been fighting since she was 1 for her to get speech help of any kind. I was so excited to get her into the clinic and so excited to see what they would be able to do for her. I can't explain how hard it is to not be able to communicate with your child, especially once you start to see how frustrated they get in their inability to communicate. Hopefully we can get her appointment rescheduled soon and not have to wait another year. 

Hopefully we can just get her feeling better and back to her fully happy self!!

And here is a non sick picture from a few weeks ago. Daddy with his two babies! Amber's telling Charlie some sort of story here, I know she doesn't look too happy but she was!

Amber's Latest Appointments

I'll start Amber's update back to February 18th. That Saturday morning I opened up Amber's pj's to find a very weird, very new bump on her chest. I'm not sure if you can see it in these pictures but it's off of the top left corner of the gauze. There is a slight shadow where it is, it's actually a bruise forming.

 She was not showing any signs of pain but we still brought her in for chest x-rays anyways. The x-rays showed that there was indeed a difference to her right side which wasn't there before but it was inconclusive as to why it was different. You can see it a little better in this picture.

 Fast forward to this past Tuesday. Amber had her chest x-rays repeated and again there is something not quite right in that area but we are still not sure what it is. We have ruled out hernias, it's definitely a bony protrusion and we're thinking it's a slight fracture or possibly a dislocated rib head. She has such a high pain tolerance that it just didn't bother her. We're also having them re-read because they were focused on the new findings in her lungs. It's definitely weird how fast it showed up but thankfully it's gone down in size some.

February 27th both babies had a cardiology appointment.

My two trouble makers, Amber was pulling Charlie's leads off!

 This appointment was kind of a nightmare. Neither of them were happy, they both wanted to eat as soon as we got their and were very uncooperative with what needed to happen! Amber finally got another echo! She had not had one since she was a year old, she used to get them every 6 months or so. The echo showed that she still has the severe mitral valve regurgitation and she now has mild regurgitation in her aortic valve. It was kind of disappointing to hear but I'm not surprised. This explains why her heart rate has been sky rocketing when she's sick. Thankfully she's not showing any physical signs of it being a problem so we won't be changing any medication. She is also at this time not showing any thickening in the valves, something that is common in I-cell kids and we would assume to see in her because of her other heart problems. The other bummer news we got at this appointment is that her liver size has gone from a 3 to a 5. I won't lie I'm not 100% what the number mean. I do know that the liver is bigger. This would also be another indicator that the heart changed. It's a bummer because it gives her even less room in her abdomen for what needs to be going on in there. We're also thinking this is why her stomach contents in leaking out around her g-tube more (it's pretty gross). Amber will go back the end of August for a follow up, I'm hoping they'll do another echo at that time to keep an eye on the new aortic leak.

February 29th Amber had Dental and they both saw Genetics

The dental appointment was quick. Amber has a ton of teeth in now and a ton more coming. It's so much fun! She's very cute with her teeth but she is very unhappy about cutting them. We were told the integrity of her teeth is very good this puts her at less of a risk for infection and the teeth are all coming in where they would expect them to be with her hyperplasia so no issues there! We do need to keep an eye on her tongue because she has a few coming in underneath it so they could rub/cut the tongue and cause ulcers. If this were to happen we would have her teeth shaved down, something that would be quick and be done in the office. Thankfully though they don't think her teeth are too sharp at this point and so far we're not having any issues.

Friday March  2nd Amber had a G.I. appointment

This appointment was ok, it was with a new Dr. They didn't really have any answer as to why her stomach contents have been leaking out around her tube. Sometimes she up to at least an ounce come out. The Dr did say it could be the liver and lungs pushing on the stomach. Amber has also been having really bad constipation issues again. She normally gets a 1/2 tsp of Miralax through her night feeding and this used to keep her regular. Now we're doing up to 10mls of prune juice through her g-tube during the day. We were also giving her additional Miralax during the day but this was making her extremely uncomfortable and she would through very violent tantrums. The Dr suggested giving her a probiotic called Culterelle mixed with water through her g-tube to see if it would keep her regular without the extra Miralax. This stuff has worked like a dream and I would highly recommend it to anyone who has constipation issues!!! We still have to do the prune juice and her night time Miralax but it's keeping her regular and keeping her comfortable! We finally have our happy baby back! It will also be beneficial to her while she's on her antibiotic because that causes her to go too much.  

Back to Tuesday March 6th

One of Amber's favorite toys a fortune cookie. She also got into mommy's root beer and decided it would look better on on her shirt!

As I mentioned before Amber had her chest x-rays this day. She also had a swallow study that was an epic fail. Now usually their really good with her but that day they wanted to do their routine and didn't take into consideration that Amber is a 2 year old child and not a machine. They fed her the baby food first (something that is a treat for her) and then tried to feed her a bottle. So needless to say she refused  to take the bottle. To make it worse they tried to bottle feed her straight barium (yuck) and used a regular nipple. She had to work really hard to get it out and then was really grossed out by what she got. By the time they finally listened to me and tried the barium formula mixed with her "Y" cut nipple she was beyond wanting to eat anything. They did clear her for baby food but I'm extremely skeptical because she did not eat a lot of it and it was way thicker than what we would be giving her at home. 

We get to go back in a month or two and try again. They did say the next time we come they will listen to me and they'll have it set up so that I can hold her and feed her her bottle. I'm still not sure if we'll try again or not.  We already know she has severe aspiration issues and it's very uncommon for things to get better with her disease. we might try the thickened baby food recipe our nutritionist mentioned but we're in no rush since Amber is sick. We also get to see a feeding specialist in a month or two as well. I don't really think our feeding issues are due to how were feeding Amber since her previous study showed that it was how her esophagus closes but I would be open to hearing how we could get her in a better position for the baby food feedings. 

Amber has a few more appointments coming up. This coming Tuesday she has an eye exam and this coming Friday she has her appointment with the augmentative speech clinic, so hopefully she's feeling well enough to be able to make those! Other than that she has a few here and there throughout the rest of the spring and summer. 

I'm going to try and take a nap now! 

I apologize for any repeat information but I have no idea what I even blogged about yesterday :) 

Don't forget to turn your clocks back one hour! 

Busy Bees

Things here have been busy busy busy! Both babies have had a ton of appointments and have a ton of appointments coming up.

Last week they both had cardiology and genetics and Amber had G.I., vision therapy and dental. This week they both had chest x-rays. Amber had a swallow study and Charlie had knee x-rays. We also started Amber's schooling process and Make A Wish process. Next week they both have ophthalmology and Amber has her appointment with the augmentative communication clinic. In the next couple of months Charlie will be seeing orthopedics and have a therapy evaluation. Amber will be having another swallow study, GI follow up and be seeing a feeding specialist. Not to mention our usual weekly early intervention therapy, monthly vision therapy and weekly hospice/nursing visits. Oh and any other additional specialist visits they throw in there.

On top of all of this Amber is unfortunately sick again. She seemed off on Tuesday and was having issues in her car seat. The chest x-ray she had on Tuesday showed some additional atelectasis (meaning the air sacs are not fully inflated) than her usual amount in her left lung. Normally we would assume that it's just a progression of her disease. However since we were/are hearing a lot of "crackling/wet sounds" in that area and she had/has a lot of congestion and a very high fever (up over 103) it was safe to assume she has a respiratory infection (most likely her usual pneumonia). Thankfully we caught it quickly and started her on an antibiotic Wednesday night. I think because we were able to catch it so quick she's not as bad as she was in November (I've only had to use one comfort care med once) but she is still struggling a lot and her heart rate has been really high. We switched her to a stronger medication for her secretions and increased her O2 flow which gave her a better night sleep last night. The lower part of her right lung is now sounding a little wet today but she's in a much better mood so hopefully we're on the road to recovery.

It's been non stop here lately. We unfortunately had to let our night nurse go so we'll start interviewing new ones as of tonight. Luke was in a snowboarding accident last week and got a pretty good concussion. He was laid up for a few days but is doing much better. Amber was seriously unhappy with her lack of  "daddy time".  I'm gearing up for arm surgery at the end of the month. I'll have a plate and screws taken out that have been giving me some trouble. It will be interesting to see how the babies will do with less "mommy time" for a day or two and how mommy will do with less baby time! When it rains it pours! Thankfully Charlie seems to be doing alright and thankfully Amber was not sick last week and should be healthy for my recovery.

I'll definitely have updates about their latest appointments in the next couple of days because I'm righting them up right after this :)

My Lovely Little Valentines

The babies had their Valentine's Day "photo shoot" yesterday and although they looked super cute they weren't too happy about it. This is the winning photo of the day! It took about 5 of us to keep them semi happy!

 Here are a few outtakes:

 This is probably the happiest one of the bunch!

Amber is on the verge of her grumpy face here!

The babies and I had a very good Valentines day. The morning was a little rough, we said good bye to our visiting PA family and Amber did the 3 P's for her nurse. Puke, poop and pee so she ended up in quite a few different outfits! The afternoon was just the three of us, were getting pretty good at it. Charlie had a poop explosion, he wanted to get in on the outfit changes too. The poor guy was not happy about it though and Amber patted his head very nicely for a while trying to calm him down. Well that lasted only for a little bit and she gradually got more frustrated that he was not calming down for her so her patting turned to hitting and the next thing I new he was wailing on him with her binkey! Now normally I wouldn't let it go that far but I was up to my elbows in poop literally! 

It's been a poopy day but it's been a chatty day as well. Amber in response to  one of her Kitty books very excitedly told me "kitty, kitty, kit, kit, YAAY". She also yelled "Amber" at her Daddy and signed for her bottle while he was feeding her. Charlie, not to be left out, has been really trying his hand at "cooing" today. He's also becoming infatuated with his sister, looking for her whenever he's awake and she's talking. 

I have to say I lucked out having two very cute little valentines this year!

The Best Of Friends

Amber is turning into such a great big sister. 

This week Charlie has really started to "wake up". He's more alert, starting to get interested in toys and trying to make cooing noises, well it sounds like "whoo".

Charlie was getting some therapy time in the bean bag yesterday and Amber rolled right up to see him. 

They chatted for a while. Amber talks to him in a "baby voice" like we would talk to a baby and he whoo'd back at her. I think they were deciding who would keep me awake for which part of the night! 

Amber had the first shift, Charlie got the second and they both fussed when Daddy left for work at 4.

 And we all took a well deserved long nap this afternoon!

I'm not sure how much longer they'll both fit on my lap and in my arms!

She loves her "Charlie time", tonight she was helping burp him.

 I hope Charlie grows to love his sister as much as she loves him, he's not always too keen on her "loving"!

Poor Amber looks ready for bed!

This is so dear to me, Amber and Charlie meeting for the first time!

I can't believe how much he's already changed.

So if you think I'm pretty bad at keeping up with blogging I'm even worse at keeping up with my YouTube channel. It really is a crime! I've been hording tons of cute videos of Amber, some of them are almost a year old. I finally sat down the other day with Amber's "help" and uploaded a bunch of videos. Some of them are not the greatest quality, you have to be sneaky to get what you want from her. Some of them I haven't even bothered to re-watch so hopefully what I think is on here are the right ones! 

For Amber's 2nd Birthday we took her to the Franklin Park Zoo. 

They have the coolest exhibit there where you can buy a seed stick and feed the Budgies! 

They come right up to you and will even perch on the stick!

Amber loved it!