It Was Nothing, They Think

Dr. Harris phoned yesterday. Dr. Smoot has been on transplant service for the past couple of weeks so she has been "crazy" according to her assistant. I won't be surprised if we hear from her over the weekend or see her tomorrow when we are admitted, but I wasn't able to connect with her yesterday. Her assistant also mentioned that Dr. Smoot was waiting for a call from our insurance company because they had approved only a 23 hour stay for Annie's cath. Ahhh, insurance.

As best I can gather, subsequent tests -- at least the ones that are back -- indicate that Annie may have something they have seen in one (yes, one) other child: transient methylmalonic acidemia. Translation (I think) is that she may have methylmalonic acid present in her urine sometimes but it doesn't mean anything is wrong. The B12 test isn't back actually, but I guess acylcarnitine tests came back negative. I assume that means whatever was indicated by the methylmalonic acid in her urine was followed up by the acylcarnitine test (related to fatty acid oxidation issues and about 15 inborn metabolic disorders), but when that came back negative it threw them back to the transient methylmalonic acidemia. Meanwhile, they are planning to give Annie B12 just to be on the safe side. They'll also -- surprise, surprise -- run a few more blood tests when we're admitted tomorrow.

The positive -- I think, I think, I think -- is that they haven't found any of the really nasty metabolic issues in all of their digging around. Dr. Harris didn't say that to me exactly and I had hung up before I connected those dots, but I think if all of the metabolic testing has come to a dead end that is, in some ways, a good thing. As many of you know, we have been sweating out several dire metabolic issues for which there is very little, if any, treatment.

Meanwhile, Annie continues to gain weight, averaging a little over an ounce per day. That's TERRIFIC for her! She was 11 lbs. 13. 6 ounces at the doctor's office on Thursday (hi everyone at Willow Bend!). As you can see from the pictures we took for Scott's birthday (previous post), she's starting to look a bit ... well, chunky. It's a great thing.

Thanks to everyone who has sent their good wishes and prayers for Monday. We need 'em! The idea of handing a happy, cooing little baby over for sedation and a test that is going to make her wake up sore and uncomfortable is driving me a bit crazy. The good news is that Annie has no idea and doesn't have to spend time worrying and anticipating it like we do! (I can't even imagine going through it with a child Jamie's age, but I'm sure we'll be doing these regularly with Annie so we'll learn sometime.)

Finally, our special good thoughts go out to Uncle Bill and his family as well as Aunt Beth and her family. We are thinking about you all. Speedy recovery, Beth and Bill!

Happy Birthday, Scott!

You are the best man I have ever known. I would not want to travel down this road with anyone else. We love you and are most, most grateful to have you as husband and father. Happy 36th!

Genetics/Metabolism Follow-Up

We heard from Dr. Harris, the genetics/metabolism specialist from Children's yesterday. One of Annie's tests came back showing an elevated level of methylmalonic acid, which can be an indication of a B12 deficiency. We also spoke to Dr. Smoot later in the day about the results. Neither doc thinks that the B12 deficiency could be the primary cause of Annie's cardiomyopathy, but the deficiency could be indicative of other primary issues.

We went back to Boston this morning to have more bloodwork done, and they're working to have some results by Friday. Dr. Smoot is planning to go ahead with the cath on Monday, but these results may change the need for a skeletal muscle biopsy.

So ... we still don't know much more. Dr. Smoot said she has been fooled by results like this before and they have ended up not meaning much of anything. As Dr. Harris said yesterday, they are learning so much about all of this stuff that anything is wide open right now. Oh, and I've been making a menace of myself by digging up info on all of the tests that were requested today without knowing what the doctors are thinking or where they might be headed. Really, the Internet's a good thing ... !

As I was writing this post, my parents pulled up in the driveway. Hooray!

You'd Never Know

You'd never know this girl is sick, would you? Annie is looking great, or at least we think so. She had the smallest of backslides with the increased Lasix dose, and now is back to putting on weight like a champ. She's almost 11 1/2 pounds now, in time for her four month birthday (yesterday). Friends keep saying that you just wouldn't know her heart isn't doing what it's supposed to be doing.

She is such a happy girl and so easy going! She absolutely loves to laugh these days, and thinks it is the funniest thing ever when you laugh with her. She also loves to watch her big sister playing -- no wonder, as there is always A LOT of action involved in anything Jamie is doing!

We are getting somewhat used to the new demands of life with Annie and her medications, but haven't been able to get enough time with Jamie. (Getting food into Annie is just about a full-time job. It takes her a long time to finish 2-3 ounces, so she ends up eating just about all day long.) We are awaiting "the troops," otherwise known as my parents. They are coming back sometime this week so they can help out and be here for our next hospital stay. Thank you, thank you, thank you! We are so, so fortunate that Mom & Dad are able to put their lives on hold for a little while and come help us. It's the next best thing to having them nearby ("there's still a house for sale on our street"). Jamie has long, long lists of all the things she wants to do with her Mam and Pap -- consider yourselves forewarned, Mom & Dad!

We had an initial meeting with Early Intervention here in NH. They contract with Easter Seals for services, so the Easter Seals folks will be here on Tuesday to evaluate Annie. We filled out a questionnaire about Annie for them, and she seemed okay in most areas until it came to fine motor skills. A heart problem doesn't qualify for automatic services, but if it is determined that Annie has a metabolic disorder it will qualify her automatically. I find that kind-of scary ... automatic means they expect with some degree of certainty that there will be developmental delays. I don't know at this point whether to hope that she does qualify or that she doesn't qualify.

Meanwhile, it seems to us that Annie has been doing a bit better strength-wise. Her head control seems to be better and she seems to be doing better when we hold her in a sitting position. The intake coordinator commented that she was doing well with her head, too, so that's one outside opinion (since it's been four whole days since we've seen a doctor!). I don't know what to think about that. Maybe she's stronger because her heart is working less hard or maybe the hypotonia and the cardiomyopathy are unrelated. (I like the idea that they're unrelated because it would steer us away from a metabolic issue, and we are still sweating out some of the nastier metabolic possibilities.) Who knows? It's nice to see her looking so well, no matter what.

The photos are a four month portrait (on a blanket her Mam made for her while she was in the hospital last month), another attempt to get a good pic of her smiling, and yet another sleeping picture. (I have 10,000 photos of Jamie sleeping when she was a baby. I thought I had learned my lesson and wouldn't snap a photo every time Annie was asleep ... oh, wait, Scott took this one!) And one of Super Jamie ("this baby blanket makes a great cape!").

Cath Scheduled

Finally spoke with Dr. Smoot this afternoon. It looks like we will be headed back to Children's on Sunday, March 11th with Annie's cath and muscle biopsy scheduled for sometime on Monday the 12th.

We're still waiting for word back on most of Annie's tests that were done while we were at Children's earlier this month. One of the initial tests came back with some elevated amino acid levels, so a retest was indicated to see if they get those results again. Another test was actually done already, so it's just a matter of waiting for those re-test results.

I asked Dr. Smoot if she was encouraged that Annie's heart didn't show any progressive dilation at the echo on Monday morning. She's actually happier to hear about Annie's weight gain -- she said that says more to her than anything. Here's hoping! (Annie was over 11 pounds on our scale yesterday morning, but had dipped slightly today to just 11 -- not unexpected since we increased the dose of Lasix. I'm hoping that she'll level off tomorrow or the next day without too much of a backslide.)

All "Good" News Today

Thank you, thank you, thank you all the powers that be -- Jamie's heart is normal and looks perfect. It was a very long weekend of waiting, a sleepless night last night, and an interminable wait for Dr. Johnson to come in after the echo ... but it's all good! She even got a lovely picture of her heart to take to school for show-and-share and was only mildly disappointed that it wasn't "heart shaped" a la Valentine's Day.

Annie's heart, as expected, did not show any improvement but it did not show any progression either. That is the small victory for the day today. And she was 10 lbs. 13.6 oz. on their scale -- up about 21 ounces in 14 days. Dr. Johnson said her weight gain is "spectacular." All the blood work from last week (taken here in NH) to check her electrolytes and some other things came back normal, too.

Last night we noticed Annie's breathing looked off. She seemed to be working more than usual to get a breath. Dr. Johnson noticed it late in our appointment today as I was holding Annie in my lap just wearing her diaper. He said it indicated some fluid in her lungs, so we are increasing her dose of Lasix to see if that will help. It's all a balancing act of the right meds at the right levels to give Annie the best chance to rest her heart.

I will be trying to connect with Dr. Smoot's office today to touch base and see where we are for scheduling the cath. For now, we will be going back to see Dr. Johnson every two weeks as well, with echos each time we go. (We'll probably skip seeing him in two weeks since we'll more than likely be doing the cath in Boston around that time.)

I have jumped in on the list serve for the Children's Cardiomyopathy Foundation (www.childrenscardiomyopathy.org) and introduced myself and Annie to the group. I have asked for some input and have received several e-mails back so far. It seems like a very supportive group! There are some great stories of kids with DCM doing well (seeing improvement in the heart function) after they started taking beta blockers. So, I am going to ask Dr. Smoot about that today, too. When can we expect to add in a beta blocker?!? I spoke to Dr. Johnson about it today and he said that the idea is not to introduce too many meds at once, which makes sense. We'll continue to see how she's doing on the Lasix, Digoxin and Captopril for now. Hopefully, though, she's doing well on these meds and we'll be able to move forward with a beta blocker soon.

Thank you everyone for your positive thoughts and prayers for both our girls today. My worry for Annie is not lessened, but there is a huge measure of relief to know that Jamie's heart is perfect! Have a great week!

Some Pics

Hooray, Annie seems to have more than recovered from the dip last week. She's up another three ounces -- just from yesterday. At this rate, she may be 11 whole pounds by her four month birthday this coming Friday. She's even outgrowing the bathroom sink for her baths! :-)

(Excuse the drool in the pic of her playing on the floor. I just wanted to include it because it is one of the few photos that looks something like her in real life. She really isn't photogenic -- photos just don't look like her.)

Another Lesson to be Learned

I am trying to be grateful for everything that has come our way in the midst of this journey. I am grateful to be so near to Boston, I am grateful for all of the amazing family and friends we have, I am grateful that we had an echo that caught Annie's condition before it had progressed ... honestly, the list is very long. It is full of the things I have learned about people -- close friends, acquaintances, and even people we have not met personally -- and their ability to reach out and support us in this awful time. The strength of family tops that list, but my gratitude for them has been so profound as to defy words or description.

I have learned another lesson, this one a bit more difficult. I am still feeling raw and shaky from this lesson, so please forgive me if I babble or don't make much sense. Not all lessons are positive in their nature, but I will try to take something good from this lesson, too. We are trying so hard to stay positive and not get swallowed up in the overwhelming fear and worry that sits on our shoulders night and day. The fear and worry that I know most people can only imagine -- and trust me, from being on the other side with our healthy Jamie, you can never imagine exactly how overwhelming it is until you are faced with it. Focusing on being grateful for all of the wonderful people that we have in our lives has been my saving grace, without a doubt.

I am sad to say that I have removed the comments from our blog because we received a venomous comment intended only to hurt us and shame us and make us feel bad. This comment came from an immediate family member, believe it or not. I won't give the comment any credence by going into what was said (and it was lengthy), but suffice to say that the intent was to hurt us and make sure everyone knew we had overlooked thanking someone and somehow hadn't conducted ourselves in a way that the commenter felt was appropriate. I woke up early this morning to get Annie fed and get her 7am medications gathered, but checked my e-mail as I was getting everything ready. The comment was sitting in my inbox, waiting to greet me and start off my day not just with Annie's-please-keep-her-from-going-into-cardiac-failure medications, but with the hate and venom of someone who claimed -- in the same comment -- to be there for us.

I have spent a lot of my life being incredibly self-centered and incredibly selfish, full of my own worries, concerns, and grievances ... just buried in my own head so much that I often didn't see (or never saw) things from anyone else's perspective. I have worked very hard to change and can say, honestly, that I work on it every day. I know that I haven't gotten there by any means, but it's a work in progress and I'm proud to at least be aware and working on it. So the lesson from this comment is a good one and one that I am working on being grateful for. Seeing someone else's selfishness in the perspective of what we are going through and what we are dealing with IS the lesson. When I tamp down the anger a bit I can feel sorry for this person and the personal misery and hate that must live in them. I hope, as the anger wears off some, that I can focus on that and realize that this person must be an incredibly unhappy person who is so wrapped up in their own unhappiness that they can only live their life selfishly. I hope I can get to the point where I just wish this person well.

So, that was a long, somewhat cathartic way of explaining why the comments have been removed from public view. If I figure out how to remove that one comment, I'll be happy to get the comments back up so that everyone can see what wonderful, amazing people are thinking about and praying for Annie. PLEASE, PLEASE continue to send comments -- they will still come into my e-mail, and we absolutely love seeing who is catching up on Annie, smiling at her photos, and thinking about us as we go down this road. And thank you, everyone, for your continued support and unselfishness in being there for all of us.

(The photos are of Annie the day we got home from the hospital wearing some awesome shoes from my sister Lori, and Jamie painting the girls' new playroom. I thought they would add something to a message that isn't much fun otherwise!)

Weight & More Thanks

Annie's weight is back on the rise. I think we bottomed out with the Lasix, but she is recovering fast. She's just an ounce shy of what she was last Saturday when we brought her home from the hospital pre-Lasix. We hope that it is a good sign (and her NH cardiologist said as much) that she is able to gain weight at all.

More thanks need to be said to everyone at Scott's office -- BAE's EO Lasers Group. We received three wonderful gift baskets from them wishing us well. Thank you! We were touched that you all thought of us in the midst of busy work weeks, busy schedules, and your own busy family lives! We have received meals from folks that work with Scott as well -- folks that have four children each. Wow! I don't know how you guys do it, but you're inspiring. Thank you.

I am trying to spend the weekend not obsessing about EITHER echo on Monday morning. We are hoping to see Jamie's perfect heart and no progression in Annie's dilation ..... All prayers and positive thoughts in that direction are appreciated greatly!

Willow Bend

The kindness of others continues to amaze me. I think it truly is one of the big lessons I am supposed to take away from this experience.

Many of you may know that I struggled, sometimes comically, to find a pediatrician for Jamie. I started to research vaccines and it turned into a big saga and got us (okay, me) virtually kicked out of the first pediatrician we had chosen. In hindsight, I'm glad we learned the lesson early on that it was not the doctor for us. We landed with a nurse practitioner a couple of towns over who agreed with our plan for Jamie's vaccines, but I never loved the office or got any warm fuzzies for the other folks in the office. (Virtually every time we went, a nurse would offer to get Jamie "caught up" on her vaccines, so the nurse practitioner's beliefs were definitely not shared by everyone in the office.) When we decided to leave that office for our current office, I had a huge hassle that again proved to me that we weren't in the right place.

With that hassle ... we entered Willow Bend Family Practice with a bang. To make a long story short, Jamie was very sick one day in the midst of my changing offices. I had requested that Jamie's records be sent to Willow Bend just a week before, but knew we weren't established as patients with them yet. So, I called the old office to make an appointment and get Jamie seen. They absoutely refused to see her because her records had been sent (or, they had requested their third party office to send them -- they couldn't confirm if they had actually gone out yet or not). Willow Bend had not received them ... yata, yata, yata ... me sobbing on the phone, Jamie curled up in a feverish ball, asleep in the middle of playing on the floor, and an incredibly hostile woman on the phone at the old doctor's office. Willow Bend came to the rescue and more than stepped up to the plate to get Jamie into their office that afternoon with just the bare minimum of information finally, grudgingly faxed from our old office. I couldn't have been more grateful for them that day!

Over the course of my pregnancy with Annie, Willow Bend helped with referrals and didn't even ask "What, AGAIN?!?" when history repeated itself and I found myself doctorless the same way Jamie had been several months before. My records had been sent from my old doctor, but hadn't been received by Willow Bend, just when we needed referrals for the first fetal echo in Boston. Again, they moved heaven and earth to help and do everything they could. I kept them aware of what was going on each time I had to take Jamie in, and they were able to have one of their docs present at Annie's birth just to check her out.

It's been a bumpy road since then, and I cannot begin to explain what it has meant to have Willow Bend -- Dr. Thomson, Victoria, their nurses, AND the office staff -- in our corner. They have listened patiently to every concern, brought Annie in anytime there was any question that needed to be addressed, and facilitated appointments for us all over town. They have followed her ups and downs, asked how much weight she had gained since the last visit, and even familiarized themselves with Pierre-Robin Sequence. I have cried on their shoulders and been amazed as each and every person in the office has stopped us to ask about Annie. She is quite the celebrity there -- we clog up the hallway every time we try to leave -- and it is such a statement to what kind of office it is. Their concern has been genuine, heartfelt, and above and beyond anything I have ever experienced in a doctor's office before.

We went in to see Vicki on Tuesday. The person who checked us in asked about Annie but I didn't know if she knew about what was going on. I mentioned that we had been in Children's the week before and she said "I know" just as another staff member came over to take a peek at Annie. She was the one that told me everyone had been keeping up with the blog, so I knew that the word was out at least. Then we went back into the exam room. Vicki and her nurse came in bearing all of the largesse you see in the picture above. Yes, all of that is from our doctor's office and the caring people there. Not only did they know what was going on, but they had decided to get us a "few" things for our hospital stays. Notebooks, toiletries, Purell, magazines, munchies, gift certificates for the restaurant across the street from Children's, and much more for us, as well as (!!!) a bag full of goodies for Jamie, too.

Many of you probably never thought you would hear me say this: I was speechless.

Thank you, everyone at Willow Bend, not just for the gifts, but for every caring word, expression of concern, sticker for Jamie ("and one for my sister, too"), and hug. You are an amazing group of people who have created a doctor's office like no other. I am so incredibly thankful to have found you guys and to have you as Annie's advocate. You have given this Army brat a sense of what community means. Thank you, thank you, thank you. I know that someday we will all be laughing at her last check-up before she goes off to college, remembering the days when we saw you guys virtually every week!

Update ... not much to report

Other than being glad to be home (while missing the comfort of constant monitoring), there is not much new to report. We're worried because Annie has lost eight ounces since Saturday. She went back on Lasix (a diuretic) Sunday morning, but eight ounces seems excessive ... and it's just a bummer because we were so excited about her weight gain last week. We have touched base with Annie's doctor (more about that in the next post) and they have ordered the bloodwork we were expecting this week. I also spoke to Annie's cardiologist here in NH and caught him up on what went on at Children's. We'll see him next Monday morning, and they may even fit Jamie's echo in at the same time.

We said goodbye to my mother yesterday, as she headed home to Missouri. The reality of all of Annie's meds (the logistics as well as WHY she needs them) and the juggling act of managing Jamie amidst Annie's doctors' appointments has set in, so I selfishly have to admit it has been a rough couple of days. Packing a bag to get out the door is epic now -- milk, ice, special bottle, two types of powders to go into her milk, pills, syringes, bottled water, scissors for cutting capsules in half, cup for mixing the powder from the capsule with water ..... It's like starting all over again when we first had Jamie and had to learn how to pack a diaper bag every time we left the house! I know we'll get the hang of it, but it's a bit overwhelming right now.

I received a welcome packet from the Children's Cardiomyopathy Foundation yesterday. Wow. It was a lot of information and a lot to take in. Reading through everything threw me for a loop (again, selfishly). I'm also on their list serve now, and reading back through old messages has been daunting. There are so many kids with heart transplants! There is also someone else who sings Dr. Smoot's praises and a few families that seem to be going to Children's in Boston. I believe the entire list is 300 or so families worldwide. That's a testament to how rare this is (6 children per million, approx. 10,000 children living with cardiomyopathy in the US I believe is what I read). There is some discussion going on now about new genetic testing for dilated cardiomyopathy. When I think about a genetic link and any possibility that Jamie might be affected, I can't even breathe. Please, please, please let her echo be perfect!

On a happier note, I have not had to give a second thought to getting everyone fed. :-) Thank you, again, to everyone at BYPC for all of the wonderful meals. You'll never know how they are getting us through and keeping us going -- literally.

HOME

We just got home from Children's. Rumors of being discharged by noon turned out to be just that: rumors. We finally got out of Boston around 4, dodged a medication crisis at the local pharmacy, and have just gotten the car unpacked. Thank you, Nikki, for the wonderful "Welcome Home" sign -- what a nice greeting when we walked in the door! Likewise, how incredibly nice to not have to worry about cooking dinner for everyone. Thank you, EVERYONE at BYPC!

Annie's glucose level (a simple heel stick) came back perfect this morning, but we later found out that it isn't a 100% guarantee that she doesn't have the fatty acid breakdown issue. A more involved blood test will confirm with certainty whether or not she has an issue, so the genetics/metabolism specialists have said that we have to feed her every four hours until the test is back. So much for enjoying a baby who sleeps through the night! In any event, it means we're back to Dr. Smoot's Plan A for treatment, not going off in another direction with a possible enzyme issue. Oh, and here's the kicker, none of this enzyme issue has anything to do with her cardiomyopathy; it's just something else they may have found amidst all of the bloodwork they've been doing.

So, we had the dreaded big blood draw today. It's been hanging over our heads since Wednesday (including one failed attempt on Friday), but we finally got it done today. It took both arms, but Annie didn't really get upset until they removed (yes, removed) the needle on the second arm. She slept through most of the blood draw until then! They got plenty of blood for all of the tests the various departments wanted to run, but we'll have to wait up to a couple of weeks for some of them. I suppose it's life now and I have to get used to it, but my heart just sank when they told us she should have more blood drawn this week just to check her electrolytes and potassium and a few other things. Yuck.

Along the lines of life now ... we'll go back to the cardiologist here in NH this week and also have to see her regular doctor on Monday or Tuesday. In addition to getting the catheterization procedure scheduled sometime in the next month, we'll be going back to see the neurologist and geneticist up here -- "just to check in with them." We'll also be following up with neurology, genetics, and metabolism clinics at Children's once the blood tests are all back. For Annie's muscle tone and developmental progress, we'll be contacting early intervention (in our case, Easter Seals) so that she can be evaluated for therapy through them. On the medication side, we have Annie on Captopril at 8am, 4pm, and midnight. She takes Digoxin at 8am and 8pm. We'll begin adding back in a bit of Lasix tomorrow morning, but that will just be once a day now. At some point in the near future, Dr. Smoot will be adding a beta blocker to the list.

Here's a bit of deja vu from last summer shortly after Annie was diagnosed with EFE: Annie's diagnosis has brought a concern about Jamie's heart back to the forefront a bit. The cardiologist in Boston confirmed that Jamie should have an echo done sometime soon. There can be familial links with dilated cardiomyopathy (DCM for short -- the type of cardiomyopathy that Annie has), so she has to be checked out just to be certain she doesn't show any signs of it as well.

I'm ending this post on a positive note ... not only are we home, but we're home with a 10 1/2 pound baby!! We thought she looked bigger in the hospital, but I had no idea she had gained just under a pound since Monday. Wow! The extra calories (and, I hope, the meds that are keeping her heart from working as hard) are making a huge difference. Yeah! We'll take whatever positive news we can get right now, and it's great to see her growing. Three-to-six month clothes are just around the corner! AND, I forgot to mention in yesterday's post that Annie decided to meet a milestone while she was in the hospital -- she rolled over on her own twice yesterday. Hooray for firsts!

I have a few more pics to get up, but it will have to wait. Jamie needs some special attention, as do the pups. :-) At the risk of sounding like a broken record, please know how grateful we are for everyone's interest and support. I haven't been the best at returning e-mails or responding to posts, but please don't think that we haven't read every single one and appreciated them all.

Another Night in the Hospital

We're here in the hospital for at least one more night. Annie is doing really well and tolerating the medicine incredibly well -- they've bumped up the dose twice and her blood pressure is holding. She's also having no trouble with the fortified milk. I know she's put on some weight while we've been here, but I'm not sure how much.

Dr. Smoot came by around 7 last night, but things were still up in the air as everyone tried to decide what labwork to do. The other big question -- when to do the cath and muscle biopsy -- was still being decided as well. Dr. Smoot wanted to contact one particular doc to handle Annie's biopsy, so it's mostly a logistical issue.

I'm trying to figure out how to encapsulate all of the info we've gotten in the last 24 hours and it just seems impossible. The one thing that I am able to say without hesitation is that cardiomyopathy, and especially cardiomyopathy in children under the age of one, is complex. We see the doctors conferring outside our room and know that they are working their hardest to figure out what is going on with Annie. What can be ruled out? What are the other pieces to this puzzle? They are trying to track down everything they can right now so that they can have the best possible chance of understanding what is going on.

We were fairly certain we were going home tomorrow (if not late tonight) until labs from Annie's initial blood draw in NH came back. Something glucose related (having to do with fatty acid breakdown, I believe) came back slightly elevated. That means that Annie needs another draw after fasting at least four hours. Luckily, she sleeps through the night so they'll be able to do the test first thing when she wakes up tomorrow morning. If that test comes back within normal range, we'll be good to go home tomorrow. If it is too low, they will do some more tests and probably keep us longer while they work on tracking down what's going on.

I feel like I keep traveling over the same ground with Dr. Smoot (who is patient and wonderful, and I'm not just saying that since she got the blog address from us tonight!), but it all comes back to the complexity of cardiomyopathy in infants. Even then, I asked her if all of her infant cardiomyopathy cases are as complicated as Annie's and she said no. (In fact, she's not working this weekend but plans to come in if Annie's bloodwork comes back funky.) The in utero thickening of the left ventricle, the small jaw, and the hypotonia all contribute to the complexity.

Annie continues to look great, making it even harder to watch her go through all these tests. Today, she had an ultrasound of her head. Dr. Smoot said it would be good to have because it would be one of the pieces that the heart transplant team would need before moving forward with anything transplant-related. When I commented that it was shocking to be talking about heart transplant, she said it's just her perspective because she works on the transplant team. Then today, I commented that Annie's not on the transplant list yet (as in, one day at a time and at least she's not so sick that she's on the list) and Dr. Smoot said that another institution might already have placed her on the list. Yikes. My attempt at looking on the bright side backfired on me -- that was tough to hear! (In other conversations, Dr. Smoot has commented that she has seen kids with echos like Annie's look as good as Annie and others that were extremely sick, entubated, etc. -- all with the same echo presentation. Yesterday, I jokingly said something about "Look more sick, Annie!" when we were talking about getting a test scheduled for Annie. Dr. Smoot said "Oh, no, I've seen her echo. She has my attention and respect, believe me." Again, yikes.)

So where are we? We're waiting to see how Annie's glucose level comes back tomorrow morning. If it's good, we'll be headed home with Annie's two heart meds and we'll schedule her cath and muscle biopsy sometime in the next 2 - 4 weeks. (We'll also check in with the cardiologist in NH next week, in that case.) If her test is low, I think all bets are off and we'll be in the hospital at least a couple more days. At that point, they may decide to go ahead and schedule the cath while we're here this time. It really is one day at a time ....

P.S. Please help with a nice mystery ... we don't know who is responsible for Scott and Mom and Jamie returning to a plowed driveway yesterday. It's hard to imagine a plow driver got lost and just happened to plow our driveway, especially in light of all of the kindnesses we have received over the past week. Thank you, whoever was responsible!

Still Waiting

We're still here and still waiting. I haven't seen Dr. Smoot yet today, but a couple of neurology residents have been by and said they would be back later with their attending physician. We haven't had the dreaded second blood draw yet, but were told to expect it sometime today. (I think they're waiting to see what comes back from the initial blood draw that was done in NH last Friday.) We have begun fortifying Annie's milk to try and get some more calories into her as well. And, Murphy's Law, I finally sat down to get this update written when the nurse came in to tell me they were ready for us down in ultrasound. We just got back, but the tech said that Annie's kidney function looked normal. (I think they wanted to check because some of her meds could affect renal function, but to be perfectly honest I'm having a hard time remembering why they ordered this particular test. Whatever ... I'm happy to hear something came back normal!)

Still no decision on the catheterization or biopsy, but if anything they are leaning towards sending us home to let Annie grow a bit before risking the sedation of the cath. Annie continues to smile, laugh, and talk up a storm for anyone who comes to see her. She just won over an elevator full of stoic-looking people with her big, gummy smiles as a matter of fact. And I had a dose of family times two -- we decided the roads were too messy last night so Mom, Scott and Jamie stayed the night in Boston. I was happy to have everyone here for so long, but they headed home this morning to plow the driveway and give Jamie a little more play room than we have here at the hospital. Jamie's a trooper and seems to be enjoying having her Mam all to herself. She's very worried about Annie's roommate, too, and made sure to send hugs and kisses back with me last night not only for Annie but also for little Michaela.

The photos are of Annie during her EKG (you can see how bothered she was by it -- she has been a breeze for just about everyone who has poked and prodded her) and asleep this morning. And thank you, thank you, thank you for the continued posts and e-mails. They truly are a lifeline to life outside of the hospital and such a comfort. Our girl has some amazing folks pulling for her!

Chugging Along

It definitely was a busy morning -- I can't believe it's almost 2. We've had another echo, met with the nutritionist, met with the geneticist, had chest x-rays, and met with Dr. Smoot (the doctor in charge of Annie's care). We're waiting on a neurologist, but I think that's about it for today.

The echo was ... ugly. The dilation of her left ventricle is severe, there's no doubt about it. It was easy to see it -- the left ventricle seemed to be as large as the rest of her heart combined. In fact, the size of the left ventricle is compressing her right ventricle. (It turns out that the pressures in her right ventricle are still good, though, so the compression hasn't affected the right side negatively yet.) Dr. Smoot came in to discuss the echo findings and agreed that the dilation is severe, meaning we're stepping up treatment plans. She said we need to get moving on treatment while we await other findings and she said that we need to be a bit aggressive to try and reduce the workload on her heart. For now, that means getting her started on an ACE inhibitor. She's also added an ultrasound of Annie's kidneys to the mix of tests and will be doing another blood draw soon. (They're still deciding which tests take priority with the little amount of blood they can take. I have a comical image of all the specialists arguing over whose test is most important in my head.)

Scott asked Dr. Smoot when we might expect to see an improvement in her heart. She said that she wouldn't expect to see any 'recovery' anytime soon, but would be happy to see no more progression for now. i.e. Halting the dilation where it is would be a victory for now. Again, a lot of the concern comes with how quickly she went from a normal echo (the day after birth) to a severely abnormal echo now at just three months of age. All of her "numbers" are good, so she's coping with it for now, but that doesn't rule out a precipitous decline based upon what they saw in the echo this morning.

We're still not sure about a cardiac catheterization or the skeletal muscle biopsy -- they're talking about maybe sending us home and having us come back in a few weeks for that. I think it is still dependent upon the big picture and how it comes together with the various specialists and their pieces of the puzzle, if any.

We don't know much more than we did before, but I have to admit that my heart sank when I saw her echo today. It was so apparent! Still, I'm glad to be here and moving forward in some form. And the good news is that everyone braved the snow and my sweet Jamie is here along with Scott and my mother. It's WONDERFUL to have the company after spending the night alone (not even a dog asleep on my legs!) and going through all of the tests alone this morning. (They arrived right after the final test for the morning -- the chest x-rays. Annie's legs were strapped down and I had to hold her arms above her head. She calmly watched the machine and let me hold her like that without one single peep. She really is a trooper and it has become evident to me -- hooray -- that she has her father's temperament, not mine!) So I'm off to take a bit of a walk with Jamie and my mom and see something beside the inside of this room!

Thank you again, for all of the e-mails and posts (and even the hospital survival bag -- Teresa, you're amazing!). They really make me feel less alone here.

Our Girl at Children's

It's Going to be a Busy Day

Our morning nurse just came in to introduce herself and let us know what's scheduled for today. The only new addition (on top of the echo and consults I mentioned in the last post) is a chest x-ray.

Annie continues to be happy as a clam. They took blood last night and put in an IV (two tries), but it wasn't horrible. They tried to get a urine culture with a catheter but weren't able to after several attempts. (She squiggled herself across the bed with every attempt, prompting the nurse to comment on how strong she is. When I told Scott the story this morning he said "Muscle tone? I'll show you muscle tone! Don't try that again!") She's laying on the bed next to me now, happy as can be, talking away. We got a little bit of sleep last night, thanks to the staff here being understanding and allowing us to have a bed to share instead of Annie in a crib and me on a pull-out chair/cot/torture device. Annie has never slept a minute in a crib so I didn't hold out much hope for us to get any rest unless we could sleep next to one another like we do at home. Annie slept great. Our poor roommate had a rough night, though, so that was tough.

The snow has started so we're not sure if Jamie will be coming to visit today or not. There is a playroom here that she can use so I think we could keep her entertained, but it's not worth it if the roads are too dicey. I asked her on the phone last night if she could help me out and pack a few toys for Annie to brighten up her hospital room. All I heard after that was her voice getting further and further away as she called "I'll be right back Mama!" Sure enough, she had dropped the phone to run and get a bag and throw some baby toys into it. She was back within a couple of minutes, triumphantly announcing "I did it Mama! I packed some stuff for Annie!" That kid loves to pack ANYTHING, funny girl.

They still can't tell us how long we'll be here, but the latest guess is at least until tomorrow. If they decide to do the cath, that will be extended. Again, I hope to know more after all of the consults today. Thank you everyone for all of the posts and e-mails! It's nice to turn on the computer and see the various comments and know that so many people are pulling for us and checking in on us. Annie is one lucky girl to have so many amazing people thinking about her, praying for her, and sending their love to her. It's tough to have a list of thank you notes you need to write when you're only 3 months old! Hope Miss Manners will extend the one year deadline in her case. I'll be keeping all of the posts and e-mails for her to read someday, that's for sure.

Getting Settled at Children's

We finally got word to head down to Children's around 2:oo this afternoon. They had a bed reserved for us, but the doctor coordinating our admission was trying to get everything lined out for us. It became important to get us here before the snowstorm, too!

We've settled into our room and have started meeting some of the docs who will be working with Annie. The main doctor in charge of Annie's care is Dr. Smoot, a member of Children's cardiomyopathy team. We just met her and she had her first look at Annie -- and Annie put on quite a show of smiling and talking for her. Everyone keeps commenting on how good she looks and that is one reason that Dr. Smoot is saying that a cath may not be a given this visit. With Annie's 'floppiness' (she is hypotonic and does not have the muscle tone that she should have at this age), she is thinking that a skeletal muscle biopsy would actually give them more useful information. I asked her about some of the scarier metabolic diseases and she said that there really aren't any now for which there is absolutely nothing that can be done. That's great news! A heart transplant only sounds scary until you think there is a possibility that even THAT wouldn't be an option ....

Dr. Smoot talked about some of the medications that they will start Annie on, based upon the various test results. She will more than likely start on an ACE inhibitor and may also be started on some beta blockers. They're holding off on giving her anymore lasix right now until the decision is made regarding a catheterization.

In any event, we're settling in and know that nobody knows much of anything just yet. Blood draws will start anytime now (I'm sitting here dreading the next footfalls that come around the curtain in our room, knowing that's probably what is coming next) and we'll have another echo in the morning. We'll also be seeing a geneticist, neurologist, metabolic specialist, and a nutritionist (we'll probably be adding something to Annie's milk to try and get more calories into her -- but on their scale she was over 10 pounds for the first time ever today!). We definitely feel like we are in the best possible hands here. After the long weekend of waiting it's nice to feel like we're actively moving towards some answers. But it's sad to be here too, of course, because there are some very sick babies (including Annie's roommate who does not have the luxury of having any family with her and who just had some crisis while I was writing the blog -- her cries are killing me and I just want to cuddle her along with Annie!).

Speaking of luxury ... we've had several wonderful visitors at home in the last couple of days -- everyone bearing food or gifts and, most importantly, their wonderful thoughts and prayers for Annie. Thank you to everyone for helping see us through this incredibly tough week! I'll post a photo as soon as I can with Annie in her big hospital bed. :-)

Not Much More to Report

We saw the pediatric cardiologist here in town again today. The bloodwork that is back from Friday all looks good -- liver function is good, electrolytes are good, blood count is good, etc. The metabolic tests aren't due back until Wednesday or Thursday, as it turns out. Annie is down a bit in weight over the weekend, but that was expected since she started taking Lasix (a diuretic) on Friday night. The doctor said three different times that Annie "looks like a million bucks" and that is worth something. He assured us she is not a time bomb, but did say that she has deteriorated significantly since she was born and said that if she continued to deteriorate at that rate "clearly she would be on the transplant list in three months." Not a nice thing to hear.

We have opted to admit Annie to Children's Hospital tomorrow morning where we can meet with the cardiomyopathy team, continue running blood tests, and try, try, try to get some answers. I think the length of her stay in the hospital will be dependent upon whether or not they decide to do a cardiac catheterization. The cath, we learned today, would let them confirm that Annie's coronary arteries are structurally normal. Cardiomyopathy can be caused by a misplaced coronary artery (coming off the pulmonary side, if I remember the doc's description correctly), but Dr. Johnson believes he got a good look in the echo and that they're fine. Still, he said, they will make the call in Boston as to whether or not they think the echo is giving them a good enough look. No matter what, we're just hoping to get some more solid answers starting tomorrow. Thank goodness for Children's Hospital! We are incredibly lucky to live just an hour away from all of the world-class specialists there.

We have talked about Annie's prognosis in generalities only. Cardiomyopathy in children is very complex and most cases (some reports say 79%) are never linked to a distinct cause. Dr. Johnson said we could generally think in terms of thirds. One third of children recover, one third of children are managed through medication or other medical interventions including transplant, and one third of children do not make it. That's the best we have right now until we get more tests and rule out some of the scarier metabolic disorders (for which transplant is not an option as the disorder would only sicken the transplanted heart as well).

Meanwhile, I'm faced with leaving Jamie for a few days at the very least. Scott would more than likely bring her down to visit Wednesday afternoon but it is New England and it is February ... our first significant snowfall of the year is due to start tomorrow night and stick around through Wednesday night. I might be on my own for awhile at the hospital! So, I'm trying to cope with being away from Jamie for the longest time ever at the same time I'm so worried about Annie I don't know whether to cry or scream. I know Jamie will be fine -- my mother flew in Saturday night so she could be here for Jamie and for us and Scott will be here at night, but it's still another layer to everything that's going on.

I have once again been humbled by the outpouring of support and comfort. Thank you, everyone, for all of your e-mails, posts, and phone calls. We feel surrounded by your support and by your hopes for our girl. If we took everyone up on their offers, Jamie would have playdates morning, noon, and night for the next few weeks. Thank you! We have been and continue to be well fed by all of the wonderful cooks we know, too. It is a load off my mind to think that Mom won't have to worry about every meal for herself, Scott, and Jamie while I'm gone. (Jamie is enough of a handful ... that's why I don't get a meal on the table every night. That's my story and I'm sticking to it!) Really, there are no words to express our gratitude. Thank you.

I'm off to pack and get us ready for the next step ....
Ross

Bad News Today

I don't have the energy today to write everything, but I know several people were aware of our second echo today and wanted to know how it went. The news was very bad. Although Annie does not have any visible symptoms of heart problems, she indeed has severe cardiomyopathy (weakening of the heart muscle). The doctor doesn't know what is causing it and seemed dumbfounded that we went from a normal echo after birth to a severely abnormal echo today. We started a series of blood tests today (after opting not to admit Annie directly to Children's) that will continue on Monday. We will probably be admitting Annie to Children's on Monday and we were told to expect that she will need a cardiac catheterization sometime next week. The best case scenario would be a treatable metabolic disorder that would resolve with medication. In some cases, the myopathy is idiopathic and runs its course. In other cases, more drastic intervention -- like a transplant -- is a possibility. We just don't know anything at this point. She starts heart medication tonight to help her heart work less hard. We should start to know more on Monday as lab results start coming in -- and they're able to take more blood for more tests. It was all we could do to get through blood draws in both arms tonight ....

Please keep our sweet girl in your thoughts and prayers -- once again -- as we go down this road with her. Thank you!