Santa Baby

Well I won't bury the story on this one: We had our UVA followup yesterday and because Molly has been gaining weight and eating better, they want to HOLD OFF on listing her for heart transplant. This is actually pretty huge because UVA has been adamant that heart transplant was her only option left. We had already done all the medical tests and social work screenings and the application was sitting at our insurance company waiting for final approval. We appreciate that we have doctors who are willing to reevaluate.

As with any good news about Molly, it may only be temporary. Her health is still volatile and her heart function has not really improved any—she is still considered to be in moderate heart failure due to dilated cardiomyopathy. It seems like overnight she just started eating better and gaining weight faster even though nothing about what we were doing had changed. Of course, we're hoping it's a sign of better things to come. But as always we're scared that she could just as easily regress as she has progressed. But for now, were taking it as a Christmas miracle and birthday present for Brian (today!).

We knew she was doing better going into the appointment (though still has that dang feeding tube), so Brian and I were hoping for news that her heart function had improved. He said it was the same. When I pushed he said it was maybe a "smidge" improved. But also that he does not expect her heart function to really get any better. The damage is done. I asked what this meant long term...are we only delaying the inevitable transplant? Could she live a life in moderate heart failure? He seemed to say yes and that he was following a teenager right now in even worse heart failure than Molly who is able to go about her life. Frankly, our ultimate goal is to get Molly out of the woods and be able to keep her heart (heart transplants do not last as long as you think they might...average 15 year life span on a new heart and 2nd transplants don't last as long). I truly believe that if we can do this, we can wait for new medical procedures to help her that will avoid ultimate transplant all together. Even right now there are trials for a band that fits around the heart that squeezes the ventricle for you (heart failure is basically that your ventricle doesn't squeeze as it should) and another study about injecting heart stem cells (not the embryo kind) back into the heart. It has already shown to improve heart failure in adult patients.

Anyhoo, that's our hope, but it's very far off. We're still one day at a time and hoping to get through the winter without any hospitalizations.

Also during our appointment we witnessed a first. They were completely giddy because Molly is finally on the growth chart!! After being diagnosed with failure to thrive and having a pretty volatile up and down growth she is finally on the chart. In fact, her growth "curve" for the last month has actually been more of a 45 degree angle. Steep gains. She is officially at 6.62% (the nurse tried to say 6.6% but we worked our ass off for that .02 and we're taking it :). Last month, she was well below 0%. Her height also made it on the chart entering at a whopping 8 1/2%! 

Some new stats on Molly since she is now almost 11 months!

Master of:

- waving

- hugging (animate and inanimate objects)

- showing how big she is (sooooo big!)

- peek-a-boo

- standing with support

- high five-ing

- making her parents get her things she can get herself

She may or may not have:

- newfound separation issues as witnessed by suddenly waking up in the middle of the night, every night screaming unless we pick her up (which we try very hard not to...sooooo hard)

- said "hi" to mom

- chosen a favorite cat

We're staying home for Christmas this year to avoid inevitable travel sickness. But right when we thought it was going to be just us, Brian's family booked a hotel to come for Christmas! Yay! So we'll have a big 'ol Coffey Christmas. Hope all is well with all of you. Thanks for all of the continuing prayers and support. It's working!

We're working on uploading some videos to YouTube and will post when they are up!

It's been a while

So I got tired of writing everything down, I guess. Not having written since July and lots has happened. So here is a quick review:

Home:
We went home and everything was progressing great. Her heart function was showing minor improvement every echo. The docs were happy, we were happy and Molly was happy. She started gaining weight normally too, which was the first time EVER. So all yay. Then at the end of August...

Hospitalization #4
Molly stopped eating suddenly one day and just seemed off all around. We took her to the pediatrician (we hoped that one of these times it would be something normal) who noticed her breathing harder than normal. So he sent us to her local cardiologist who did an echo and saw that things were the same. He sent the echo to UVA just incase. We then got a call from UVA to come in for an appointment in 2 days. Uh oh... So they found that her heart function started decreasing and decided they needed to adjust her medications. We were at UVA for 8 days so they could make sure she tolerated the medicine fine (which she did). This was when we were told that she was "accidentally" taken off a pretty important medication. Great! I wonder why her heart was getting worse. Fuck.

And this is the trip that marked a big change in how the doctors are viewing Molly's condition. More on that to come.

Home:
We went back to life at home with no changes except Molly got back to eating well and she just wasn't growing as much anymore. In fact her weight gain started slowing before the hospitalization. My hunch is that she was slowly declining since being taken off the medication. Her weight gain reflected this. The docs wanted to do a cardiac catheterization to measure pressures in her heart. With her last blip, they wanted to make sure her heart wasn't worse than they thought it was. It should have been an outpatient procedure, but...

Hospitalization #5
Her second hospitalization was just 1 day. I like to think we're getting shorter and shorter with longer and longer in between, but we'll see. She had a cardiac catheterization to measure pressures in her heart and had a bad reaction to the anesthesia. Her blood pressure dropped really low. But she recovered immediately after the stuff wore off (with some help from dopamine in the meantime). But because she's Molly they sent her right to the ICU for observation. Chelsea, the infant educator, came by to say hi. She said that she saw Molly's name written on the board to go to the general pediatric floor (which was already reserved for Molly before the cath even started for observation). She also said when she saw her name was erased from that board that she would have thought any other kid would have gone home. But Molly? She knew to come to the ICU to find us. Haha?

Home:
So we're back home again. The catheterization numbers showed mildly elevated levels, which was all to be expected given her poorly functioning heart. So phew. But remember how I said above that the docs shifted their views on us? Well, that shift is that they are recommending we list Molly for heart transplant. We had an appointment mid-August and the docs were literally doing little dances because her echo and all looked good. Then when she took the step backwards (2 weeks later), all of a sudden they shifted to Molly being completely fragile and needing a heart transplant. Their reasons are because she took this step backwards (which they admitted was most likely a medication screw up on their part, but suddenly they no longer took that into consideration/were behind their initial statement) AND because she wasn't growing as well anymore (again...medications AND nutrition...more on that later).

Brian and I think they made up their minds about transplant when her function went backwards (but then went back to normal within hours of being given the right medications, again they don't really care about this now). Dr Schneider said he would talk to his friend who runs the UCLA Heart Transplant to get her opinion and also float it to the docs at UVA. All said they agreed with listing her status 2 for transplant. Status 2 means we get to stay home and she is on the list but not as a priority. Status 1 means she has to be hospitalized while waiting and she would be priority.

I asked Dr S what would change his mind about transplant and he said of everything the largest factor was that she is not growing as she should. OK given. She is tiny. She's 8 months and still only 12lbs. However in the midst of all this I realized the second big mistake, which was UVA nutrition telling us to actually feed her less than we should have been. To be honest, I should have known. I should have done the math instead of just following their instructions. But  didn't and molly wasn't getting what she needed. So we instituted a more agressive nutrition plan. The feeding tube is back in because Molly just cant eat all that she needs to compensate for her heart function. So she eats during the day and we supplement with more calories through the tube at night.

Dr S said if she would start gaining .5 to 1 oz avg per day, he would change his mind about transplant. Well guess what? Molly is now getting the calories her body needs and she's gaining about 1 oz a day. Surprise, fucking surprise!

So we just went back to UVA for a follow-up. They got the news about her weight gain, and didn't really care. Yup. Didn't care. In their minds...full speed ahead for transplant. They said it didn't count because she can't eat all that she needs on her own (aka needs the tube).

So now we're in the process of getting second opinions and trying to find someone who thinks transplant now is premature (like we do). We're going to be getting opinions from CHOP (Philadelphia) and NYU (where the leading heart block researcher is). Hopefully they can give us some hope. In the meantime, we just need to make sure Molly doesn't take any more steps backwards. Which brings us to:

Soooooo, this is the part where we break up with you. Yes, you. It's not you. It's us. Well, it's Molly. One thing that the doctors have been hammering into us is that Molly is so fragile that any illness could be detrimental to her. Her heart is so weak that it just might not be able to withstand something like the flu or another infection/virus. Well going into winter, this means that we decided to take Molly out of daycare (our beloved Kathy is on hiatus) and I had to give up my classes this and next semester to stay at home with her. So the break up...we also have to limit her contact with people (aka germ carriers). So we're on a break from visitors and visiting until the sick season is over (Molly says, "See you in May!"). Skype will be alive and kicking, though, and we'd love to see you there :)

Over the next few weeks, we'll be getting all these opinions and I'll be better about more regular updates.

Warm feet

Well we did indeed get spring from UVA last Thursday. While we still love our UVA family, it was time to GO! Our biggest test we had to pass before going was learning how to put the feeding tube in. My second try at putting in the ng tube went like this:

ME: ok I'll try again.
NP Carol: You'll do...
ME: I'm done!
NP CAROL:...fine.

Second time is a charm it turns out, for me. Brian was a pro from the beginning as we all expected. And because we found a smaller width tube, Molly doesn't really mind it at all. Phew. So instead of putting it in and taking it out every night, we just leave it in. Saves all of us some stress.

So we got our kit of parts for the ng tube (pole, pump, stethoscope and more supplies than you'll ever need) and it's quite easy to set up and use. Frankly it's a lovely backup net to have and we're all a lot less stressed because of it. With that said, MOLLY HASN'T EVEN NEEDED IT since day two home!! They say that they eat better at home, but I didn't really believe them because Molly has never followed conventional wisdom. It goes to show that when I'm all into the tube, Molly decides she doesn't want it anymore. And she's even been eating a bit over her mar. So go eater! Brian and I have spent the last few days just dumbfounded at how easy it is to feed a healthy(ier) baby. They just eat!!? Within a half hour!!? Of course she has the typical feeding distractions and gas and such. But geez. This. Is. Easy.

We also have a home nurse coming in a few days a week to make sure she's doing well and most important...growing. So far so good. From Friday to Monday, Molly gained 5 oz! As a comparison, Molly gained 6oz all of last month. Yes, month. She's coming back tomorrow so we can confirm that she wasn't just needing to have a 5oz poop or something.

And most of all this is a major indicator of her health improving. It turns out a healthy baby eats. At least a healthy Molly does. Right after surgery when we went up to see her I remember feeling her feet and they were warm. The doctors said it's an indication that her heart is actually pumping blood now to where it needs to be. A few days later I actually asked the doctors to check her out to see if her feet were too warm (I'm crazy town). I remember the doc looked at me and said, "Well, I guess you'll have to get used to what a baby should be like." It took us about 2.5 seconds this time to adjust to her eating well.

Go Molly go!
Oh and hospital pics to come soon on Facebook!
Baptism this weekend too so cute dress pictures to follow.

Going home

Most likely today, and if not...definitely tomorrow. Woo!

We're back on the general pediatrics floor here at UVA. It made us nervous to leave the PICU since they monitor much more closely (and would have caught the whole kidney failure thing that the floor docs neglected to catch), but things have been rolling fine. We asked to stay in the PICU and they agreed if they didn't need the bed space. Well this was on the 4th of July. And guess what happens on the 4th of July? People blow their hands off with firecrackers. And not just people, but kids. So they had multiple traumas come in and we were bumped. Oh well. We got bumped back to a private room, so we can't complain.

Molly is still eating better than she ever has. But it just hasn't been as much as the nutritionists want her to eat. So we're going home with an ng tube (feeding tube through her nose and down into her stomach). We'll be bottle feeding her during the day (count those fucking calories!) and whatever she doesn't take will get put down the tube at night. We hope this is only temporary as Molly has been eating 80-90% of what they want her to eat. We're hoping that last 10-20% will come once she's back home and less stressed out by general hospital antics. We hear this is often the case. But in the meantime we don't have to worry about her not getting enough nutrition to heal and play catch-up growth. She's still only 8 1/2 pounds, so every bit helps.

If we go home today or tomorrow depends on how fast we learn how to put the ng tube in. I tried yesterday and it's not as easy as you would think. Turns out that hole at the back of the nose is hard to locate. Round 2, though, is in a few minutes. It's sad because she clearly hates it, but it's not as sad as when we had to give her the lovanox shots for her blood clot. So the methods that UVA sends us home with to torture our daughter are getting less and less severe.

Otherwise she's still doing great. Smiles at everyone who comes in (until they try to touch her), has discovered her feet and has no major organs failing anymore besides her heart (though the surgery we're here for seems to show promise for that too!). Win? We'll take it.

Who needs the beach and picnics and fireworks anyway?

Well this marks the 3rd holiday that we'll be spending here at UVA. Valentine's Day and St Patty's Day being the first two (hey at least we got Easter out). We're working on 3 months in and 2 months out of the hospital for Miss Molly girl. A sad percentage, but one we hope to fix really soon.

We were working on discharge either Sunday or Monday. For those of you counting that's today or tomorrow. Well Molly had other plans. So we got moved to the general pediatrics area of the hospital to work on feeding. The docs also wanted a few more days to tweak her medications before going home. Molly was going strong on feedings, then about 3 days ago hit a wall. And the wall hit her back. She went from eating 80 to 90% of what they wanted her to to 25%. It was really sudden and getting her to eat was a huge struggle. She kept this on for about 36 hours. We began having discussions about putting the tube back in, which was depressing because she was doing soooo well.

Brian and I began thinking up every reason in the book why this could have happened. At the same time we noticed her heart rate had dropped across the board about 20 points. We weren't sure if it was the pacer modifications or what. We thought the eating issues could be teething, thrush, sore throat...

Finally the morning came (Saturday) that the cardiologist came in. This was one we hardly deal with being the weekend and all. When I started giving him my list of reasons why she might not be eating, he cut me off and said she was just a crappy eater and that we were going home with the tube. Not only did he tell me that teething was "a myth", but he also said if something was wrong with her heart beat being lower, she would look sick. Which she didn't. Smiling away as usual.

So the nurse brought in the tube and we were prepared to just deal with it. Literally 5 minutes before the tube was going to be put in, one of the docs comes in to say that they need to recheck a blood level because it came back really high and they were sure it was a mistake but needed to recheck for their records. Then about an hour later, the same cardiologist came in and told us the level was not a mistake. It turns out Molly's potassium levels skyrocketed from dehydration and as a result her kidneys were failing. They moved us back to the PICU and immediately began giving her fluids and stuff to flush potassium. Fortunately she started responding right away. After a day of this, we're happy to report that her potassium is down (now too low and they are giving her more potassium...seriously) and her kidneys are functioning again. And guess what? Her heart rate has gone back to normal AND she's eating again. And not just eating, but ravenously eating. Full feeds eating. So suck it doctor who blew me off and told me she is just a bad eater.

Of course the inevitable "will they make her too wet again" is an issue. And one we have been vocal about. We're hoping they just let us cut and run before something else happens.

In the midst of all this craziness, they stopped one of Molly's meds that is a bit hard on the kidneys. Last night they discussed putting her back on Milrnone (aka fucking Milrinone) if she couldn't go back on the enalipril. So they did bloodwork this morning to check her BNP to make the call about enalipril or not. This led to our best news of the day...to recap, BNP is a marker of heart failure. The higher the number, the worse the failure. While the numbers themselves don't mean anything specific, in general zero is best and the farther you get from zero is not good. Molly's was 3200 on admit. 15,000 post-surgery. 2500 a week ago. And now? 135! Yup. Not a typo. 135. We're not sure what this means as far as her function yet because they haven't done an echo in over a week, but it's a really good sign. Hopefully they'll do an echo tomorrow and we can see what's going on in there.

So the ups and downs and ups continue. I think Brian and I are the only parents who are happy to go back to the PICU from the general floor. Molly is now doing great AND we get our much comfy in-room bed back.

So Happy 4th. We hear that we'll have a good view of fireworks from our hospital room. Molly does like looking at lights so we consider it a win.

The D Word

So the yang has been going well. After my talk with Dr J, I decided to just release all my expectations and go with the flow. I was ready for however long we needed to be here and do whatever the docs say we needed to. The second you give something up, it seems to come to you...

You know when doctors start throwing out the word "discharge", you have about a week or so left barring any unforeseen things. They started talking about it on Monday, so keep your fingers crossed.

It has been a big few days for Molly and mostly all for the good. We got an awesome new attending physician in the PICU who moves fast. His first mission was to get Molly off the Milrinone and her central line out asap. Yesterday cardiology came by to say they were going to slowly wean her off the milrinone and possibly she'd be off by Friday. 10 minutes later I'm in rounds and Dr Willson (my fav new attending) is directing them to stop it immediately and pull the central line. I thought cards changed their mind, but when I mentioned it later, they were surprised (but fine with it). Yay! Dr Willson came by later to remove her central line, thus making Molly wireless once more. He said his reasons for the quick move was risk of infection. He said they had a kid the day before who was supposed to get their line out the next day and got an infection. Now they are stuck here for 2 more weeks on antibiotics.

The EP has been in the do some tweaking on Molly's pacer. We've been noticing that sometimes her heart rate suddenly drops to the 100s for no reason. When he did the check he saw that her atrial lead (the one that senses her atrial rate to tell the ventricle what to pace at) was picking up some reverberations from the ventricles pace and trying to pace that. So the machine was switching modes and doing this drop thing. It doesn't hurt her in any way, but they want to tweak it so it doesn't happen. Yesterday they made the lead less sensitive and she's had way less spells of this. Again, these pacers are made for old people, so retrofitting them to work for a baby takes time and adjustments.

So Molly is off all her iv meds and back on all her oral meds (with a few more added for bonus). We've officially crossed over into only being here for feeding. She is still not eating the volume that they need her to eat in order to get all she needs. Cardiology said they would be fine sending us home with an ng tube (feeding tube) so we can supplement whatever she doesn't take by mouth. We're fine with that too because she needs the nutrition. However, Dr Willson (in another awesome move) let us pull the feeding tube to see how she would fly on her own. It's been 24 hours and Molly is eating well. At home she ate about 375 ml a day. They want her to eat 560 ml a day. In the last 24 hours she ate 475. HUGE improvement after just one day. So we're working with speech therapy (yes, they actually work on feeding issues with babies) to help her get more. Working through some reflux issues and today trying to add some rice cereal to the bottle. Apparently adding some rice cereal allows their stomach to empty quicker. They have labelled Molly "quick to satiety" meaning she eats and once she feels the food in her stomach, she's done eating. We're hoping the rice cereal empties her stomach a bit more so she has more appetite. Thank freaking god we have specialists helping us.

Otherwise Marie and Hannah are back! And so is Brian!

So once Molly perfects to F word, we'll be D worded. If she just doesn't get there, a feeding tube it is. Whatever needs to happen to get us back home!

Wean, baby, wean.

It turns out, I am a medical conspiracy theorist. When Molly responds poorly to something I come up with about 8 different explanations for what could be going on. Then I systematically go about disproving each theory. It helps me deal with things, but I think it drives the nurses and docs a wee bit nutty.

Today my theory is that Molly hates her feeding tube. She randomly gags. When we feed, she's hungry but as soon as she goes for the bottle and starts to swallow she freaks out. Hates it. But she's still hungry. I think she's a bit of a prima donna about this, but it's annoying because we need the tube still as she recovers. So she's a horrible eater. I'm not sure how to resolve this. I guess we'll wait until she's more stable and then pull the tube and see if it makes a difference.

There are just so many other small things that we have to worry about on a daily basis that it's tiring to write about. Potassium, heparin, sodium, calcium, levels, levels, levels of everything! And her pacer is pulling shenanigans that I still don't understand (but according to cardiology is fine).

They have begun the milrinone wean so hopefully this is the beginning of the end for it and her central line (the iv access). The longer she has in her central line the higher the risk of infection. So everyone wants it out as soon as possible.