Month in Review

Since my last blog post on April 21, life has been crazy! Blogging is something I have failed to squeeze in. So I will attempt to recap the last 30 days.

Emma came home from the hospital on Sunday April 22. I now possess a talent that few out of the medical field will ever have. I can successfully place a feeding tube. Over the last month I have had to replace it several times and I've done quite well, if I do say so myself.

Emma is doing OK. OK meaning she is doing well considering all the horrible things that are happening to this sweet little girl. Considering she has a hole in her heart, which is causing her heart to enlarge and that is causing the enlargement of her lungs and liver as well, she continues to hang in there. She definitely has good and bad days. She throws up a lot! I rarely make it through a whole day with the same outfit. I can't pinpoint what is causing it all the time. It think she looses her feedings for different reasons. A lot of it has to do with the feeding tube. She has such a horrible gag reflex that if anything makes her gag, she looses it all. Sometimes she is fed to fast, sometimes it's the way the tube is placed and irritates her and sometimes I don't know what to think. 

But despite loosing a lot of food, she is finally gaining weight. As of Tuesday May 15, she is finally back on the chart as far as her weight. She is now a whopping 8lbs! And that places her in the 1 %tile amongst other babies her age. However, her height places her in the 56%tile and head size is 87%tile. She is very long and skinny. 15 lbs is the ideal weight they want her to be. Still seems so much to gain in just 4 months.

Within the last 2 weeks, Emma is now gaining the minimum amount they want her to. 20 grams a day is her minimum. She did just that. We are doing some things right!!

Emma is such a strong spirit. If she wasn't a fighter she might not even be here. Despite all of this, she is so active. She loves to kick and move around. She is a pro and holding her head up now. It stresses me out to see her move. I just want her to lay still and gain weight, not burn calories and ease the stress on her poor heart.

Developmentally she is right on track so far, except for cooing. She doesn't coo. She barely makes any noise, ever. Which from what I have researched online many children with Rec 8 have speech problems. This worries me to no end. She will occasionally cry if something really makes her mad ie shots, being in her car seat for too long and the last time we were at her cardiologists, for some reason the stethoscope made her cry. Getting her blood drawn however does not. I think she's had so many of those pokes she's used to it now.

So for now we are staying the course. She's gaining weight and her symptoms have stabilized, which makes her doctors happy. We see her pediatrician every other week and we alternate the off weeks with her cardiologist. In the last couple weeks we have also started seeing Kids on the Move which will give us all the tools to help Emma stay on track. Early intervention is the key. The greatest part is they come to our house! So far we have a occupational therapist coming to work on feedings and to make sure we don't create an oral aversion for Emma. We don't want her to loose the will to eat. We will most likely start seeing physical therapists and developmental therapists over the next several months. It's nice to know that she will have the help that she needs when she needs it. Most of these things will start after her surgery.

We have our appointment with the geneticist on Wednesday.  I have a lot of mixed feelings about this appointment. I'm interested to see which way it goes. I've heard good and bad about geneticists. The geneticists office has already called 3 times in the last 5 days to confirm the appointment. They must have a lot of no shows, which surprises me. If you have to see a geneticist you would think people would want to make sure they were at that appointment. It's not just your average physical.

My other children and crazy, but fantastic. They tend to act out, a lot, whenever I feed Emma. I understand this, but I don't appreciate it. I try to find something cool they don't normally do or schedule their meals while Emma eats, but in my zombie like state it doesn't always allow things to happen that way. It takes about an hour to do a feeding, at which time my children have learned I can do nothing to them. They take advantage of that far too often.

I am only getting about 4-5 hours of sleep a night. And that is broken into 2 segments, because I have to wake up to feed Emma. I'm hoping at her doctor's appointment on Tuesday they will give her a pump that will continuously feed her throughout the night so I don't have to wake up to do it. She rarely wakes up for the middle of the night feeding. I have to set an alarm to wake up to make sure she's getting the intake she needs, so I sit there and wonder why I am awake holding a tube of formula while she sleeps. Especially when I just found out a machine could be doing it for me! I would love to be myself again instead of the hollow shell of a person this lack of sleep has created.

Other events of the last month:
* We finally got everything out of our old place. Randy did an amazing job cleaning it out all by himself. With all the events of our life, it really did take us 30 days to get out and clean. It was nice to have 2 places for the month of April. And amazingly we got all of our deposit back. I'm still in a state of shock.

*Beck finished soccer. Loved it. Now we wait for T-ball to start in the middle of June.

*We officially moved all the stuff out of my parents house they have been storing. This may be more exciting for them than it is for us. Their home looks oddly empty now.
 
*Randy finished spring ball and has had most of May off. So I've been putting him to work. We have a lot of furniture that has the potential to be nice. Yesterday he went and bought a power sander and started getting some of it ready to paint. In all, we plan on refinishing the boys' dresser, Emma's crib and dresser, our kitchen table and chairs, a hutch, a desk, shoe rack and a toy box. And that's just what is currently sitting in my garage. I'm sure more will be added as time goes on.

One day we will finish moving everything into our new place. Probably months from now.

Randy will be coaching the summer camps at Utah in June and then thankfully he has July off to finish all these crazy projects before football season sweeps him away.

And now some pictures of my adorable children from the last month:

Pediatrics has a really cool playroom for patients and their families. My boys miss it terribly.

The boys flock to Emma, like a moth to a flame whenever she's out of her bed.

Love <3

My Grandma Pat came down for a visit

Aunt Wendy visiting. My boys have been talking about their visit for weeks.

So handsome. This is why we keep them around

Beck loved soccer. Randy loved buying gear for soccer. Just wait for T-ball, there's even more gear.

I couldn't find Trey the other day. He was asleep on the chair.

Emma's favorite thing to do is watch her brothers. She is eager to be apart of the action

Beck helping Randy sand his dresser.

Jinx

I don't allow myself to believe in jinxes or any superstitions for that matter, but every time I put on Facebook that Emma is coming home it never happens. Although when I came to the hospital today and learned we weren't leaving "jinx" was the first word that popped into my head.

They want to give me another day to learn how to do the NG feedings. Which I am grateful for. I want to know that I can in fact put the tube in her stomach, and not her lungs, successfully. Half the battle is getting her to hold still. Emma is quite the fighter.

Emma's feedings have taken a big hit since the feeding tube was placed. They want her to eat 55 milliliters every 3 hours. She would have a couple feedings that she would eat all 55 mils before the feeding tube, but at least half of her feedings would be in the 35-45 mil range. Since the tube has been in place she ate 40 mils on her first feeding. 20 mils on her second and then through the night she at 15 mils every feeding. This morning she had another 40 mils and then back to 15.

I'm hoping she is just adjusting to the tube. I know it irritates her, but I was hoping for better results this morning. It worries me that she won't want to eat at all soon. No eating = surgery now.

On another note, I mentioned yesterday she had another echocardiogram. I got the results last night as I was leaving. Her VSD has never shown resistance to the blood flowing through the hole. Holes that show resistance create more pressure, which could be a sign that the hole is starting to close. Yesterday Emma's hole had resistance. I'm told it is just the smallest amount of resistance there can possibly be, but there is resistance! I just want to stand on the roof and shout it to the world! Resistance is a great thing. Emma's echos have never come back with good news. It's usually the opposite. I can't help but hope that we might continue to see positive results. Sure maybe the hole won't close, but it gives me hope that the chance is still there. 

Step 5

We now have a feeding tube. Which I learned how to put in and administer food through. Oh how under qualified I am to do this! I do not feel comfortable putting a tube down my daughter's nose into her stomach.

I have yet to actually place it myself. I just learned the way to measure and then stick it down, followed by how to put formula in it by watching. It's just a matter of time until she learns how to rip that thing out and then I have to step up to the plate. Tate and Trey got very good at taking those out. No matter how much we taped them down, those tiny fingers would always find a way to slip under the tube. However, the wonderful nurses that took care of them replaced them. This is all new.

Emma will eat as much as she can. Once she can't take anymore I then feed her the difference through the NG tube. If we get to a point that she cannot take any formula in on her own they will operate.

Emma hates the NG tube. When she first tried eating she would have a horrible look on her face every time she swallowed. It is causing her to gag. A lot. Which makes my heart race. A lot. I think I'm figuring out ways to lessen her gagging. What a learning experience this is.

If she tolerates her feedings well for the next 24 hours with the tube in place it looks like we are going home. Her breathing is in an area we can tolerate. Her medications seem to be balancing things OK. Now she needs to gain weight. They are also setting us up with Home Health & Hospice. They will come and help if we can't get the tube in on our own. They will also make sure we have all the medical equipment we need.

We have steadily marched through all the steps to prevent surgery. #5 is a place I hoped to never see. As long as we can keep her gaining weight we can get closer to the six month mark they so desperately want to hit.

Today I've decided I'm not going to think about 5 months from now. It's too overwhelming. My new goal is to make it to the 26th. One week. Everyday will be triumphant if we survive once we get home.  I was scared to go home with oxygen and a heart monitor. I can use those with ease now. I am hoping the same will happen with this.

Having a feeding tube will be so much easier in many ways. She won't be connected to the wall. She can move as she pleases, or as I please.

This picture was taken a couple minutes after the tube was put in. Her expression encompasses the situation well I think. She keeps looking at me wide eyed trying to figure out what it is we are doing to her now.

Emma had another x-ray this morning. It showed no change. Her doctors are satisfied with how she is breathing now. Things might not change until the hole is closed. Emma creates her own normal. Her blood work came back looking much better than before. She is taking her new medication well. She also had an echocardiogram. I hate how I sit in the room with the tech that I know can tell me if things are better or worse, but they aren't allowed to tell you. I haven't heard back from her cardiologist yet on what he saw. I'm not expecting much to change. I'm always hoping it will, but realistically I'm just hoping she isn't getting worse.

Another Day

Emma is still doing the same. Same is better than worse. I'm grateful she isn't doing worse. They started her on a different medication today. The diuril wasn't working the way we anticipated. It was causing some problems. She started throwing up the last two days and I'm convinced that's why. She hasn't even spit up once while in the hospital.

Her new medication is called aldactone. Which I just learned cannot be picked up at just any pharmacy. They actually have to make the drug, or compound it, was their term. Thankfully the pharmacist here went and found a pharmacy in Saratoga Springs that is right by us, that can make it.

Her doctors are now starting to wonder if there is another reason behind why she is breathing so fast. They are wondering if she has a very narrow airway that is not supported well. That could also cause rapid breathing. There are tests they could do, but they are quite invasive and involve sticking a lot of tubes down her nose to her throat. At this point they aren't interested in doing those, especially because it is something she will most likely grow out of as she gets bigger. So we are just keeping that in mind for now.

When Emma was first born the murmur was really hard to hear. Many a doctor did not ever hear the murmur. Then after it was caught, most nurses could not hear it and doctors would really have to search for it and could only hear it in one spot on her back. The last 2 days I have had multiple people tell me how loud her murmur was. These were people who had never seen Emma before. I didn't know what to make of this.

Today when her cardiologist rounded on her, he pointed out that the murmur was louder. This can either mean something awesome, or something not awesome. As holes in the heart close they get louder, because the pressure rises in that one area. That is the awesome part. Or it could mean that there is just a lot more blood flowing through that hole. That part is not awesome. Her hole won't get bigger. It is what it is. But the amount of blood flowing through can change constantly. I'm really holding out hope that the hole is closing. Oh how I will be praying that is the case.

They are going to do a follow up x-ray tomorrow to see if we are making any headway in the fluid in her heart and lungs. In the next couple days they are going to do another echocardiogram. That will be able to tell us if her hole is closing or if there is more blood flowing. So fingers crossed!

Puzzles

I've tried all my life to like puzzles. My mother loves them, I just don't have the patience for them. I am now living the puzzle Emma has put before us. I think we've got all the border pieces, but filling in the middle is proving difficult and frustrating.

So Emma's first medication, lasix, is not working as we found out yesterday. It is not removing the fluid in her heart and kidneys. They added the second medication. They did blood work today, which they have been watching while she has been on her medication. They check the sodium levels to make sure her kidneys are processing all the extra sodium they are receiving from her medications. In the blood work it also checks the function of the kidneys. It came back today showing that they kidneys are not receiving enough fluid. Which makes us thing we are drying her out.

This is where everyone started scratching their heads. We have been closely monitoring Emma's input vs output. Babies should put out the around the same amount they are taking in. In Emma's case, because we have her on all these water pills, she should put out more than she is taking in, because the fluid in her heart and lungs should be leaving her body.

For the last week Emma has been positive, meaning she is taking in more than she is putting out. By a lot. That is another reason they started the second medication. They don't understand why her test would come back showing her kidneys aren't getting enough fluid. If anything they should be getting too much. The pieces aren't fitting.

They decided to take her off her second medication for 12 hours to see what she does. If her output is positive 100cc's+ they are going to put her back on it and try again. 

Emma was not eating very well today. Her doctor has her on a high calorie formula, so she can take in less volume and still get the same calories. Our goal was to help make feeding easier. Her doctors want her to drink around 50 milliliters a feeding (every 3 to 4 hours) When I left her last night she drank 70 mil. I was elated! It was the most she has ever taken in. When I came in today I found out 45 mil was her highest through the night. The last feeding I did today was 35 mil. Emma was eating every three hours consistently. Today she was going four. We are getting worse.

Her breathing has plateaued. She's definitely better than when she was admitted, but not improving.

They haven't been successful in removing the fluid with medication. At this point I can't see a time when we will come home before her surgery.

When it rains it pours

I hate rainy days. Bad things always happen when it rains. My boys act up more when it's raining. I hate when the bottoms of my pants are wet when I take my shoes off.  The whole world just feels depressed. Me and rain... we don't get along.

Whenever a doctor has told us "We think you'll go home on _____ day" something always happens shortly after that shatters our hopes of ever leaving this place. I can't help but get my hopes up every time they say it. I just want to take my princess home with me so badly! As I put in my last post, they were going to move her to oral medication today. When I showed up at the hospital this morning I noticed she was still connected to her IV. Instantly I knew tomorrow wasn't our discharge day.

Throughout the night she was spending most of the time breathing 90-100 times a minute. They gave her an extra dose of her medication, but it didn't help much. Because of her problems while breathing they never took her off of the IV. They ordered an x-ray to see if her heart is still enlarged. If her heart is still enlarged they will add another medication to her mix. She has already maxed out the one she is currently on.

Getting her x-ray

The x-ray came back showing "slight" improvement. So her heart is still enlarged just not as much. Her doctors are adding the second medication twice a day.

I think I've said before that they don't want to operate on Emma until she is six months. However, at some point the risk of surgery is less than the symptoms she is having. How I understand it is there are 5 steps we will take to try to get her to that 6 month mark.
1. Put her on lasix. Done and done.
2. Up her dosage on the lasix. Check
3. Add another diuretic called diuril. The lasix is also a diuretic, but seeing as how we've maxed out the amount she is allowed to have, we have to add another into the mix. Which we are starting today.
4. Up the dosage of the second diuretic.
5. Add a feeding tube. It's so hard for her to eat while working so hard to breathe. We would still let her try and eat, once she gets tired then I would supplement the difference in what she needs through the tube.

Once she is unable to eat at all on her own, the surgery becomes the next step regardless of how old she is. So we are already on number 3 of 5, with 5 months left to go.

I can't help but feel very discouraged today along with the mountain of emotions I'm constantly feeling. Part of me just wants to have the surgery now so it's over with. I hate seeing her this way, but she is so tiny. I can't imagine someone doing open heart surgery on her. Then again I don't ever want to think about that happening ever. Regardless of when they do it I will never be ready for her to go through that. 

However, there are some positive points of the day. Emma has been oxygen free for over 24 hours!! She hasn't dipped below 80% oxygen once.

I love being able to see her beautiful face without anything blocking it, I'm sure Emma is more excited about it than I am.

Our second great thing of the days is I got a call from her geneticist. Our appointment has been moved from November 13 to May 23! It will be nice to see if they can give us more insight into Emma's disorder. I'm grateful for Emma's doctors that wrote letters on her behalf to help her get in earlier.

I know that trials make us stronger and we are blessed for them. Thankfully the Lord has been sprinkling us with blessings throughout this difficult time.

April 16

I honestly spent more time trying to come up with a title for today's blog than I did typing the blog entry. So today's date is all I could come up with.

Emma is doing better today. I think we are finally getting a head of all the fluid in her heart and lungs, finally! It's been a roller coaster of ups and downs this last week, but she has been oxygen free for about 8 hours with no problems. What this means is the fluid is clearing out of her lungs and it isn't as difficult for her to breathe. She still isn't breathing the way a "normal" infant should, but she won't be able to do that until the hole is closed.

Her doctors are hopeful that we will come home on Wednesday. They are going to give her medication to her orally starting tomorrow. If on Wednesday her heart x-ray comes back normal we get to leave. If we are truly lucky we will avoid the hospital for the next five months.