Frustrating Morning!! No News Yet

We had quite a morning. Apparently, scheduling glitches happen at Children's, too. After going down this morning around 7:20, we found out at 8:30 that the orthopedic surgeon had been scheduled for this afternoon, NOT this morning. So we sat and sat (and paced and paced), trying to keep Annie asleep and not thinking about her empty belly. (She really did great, snoozing most of the time and only fussing when they flushed her IV and when she couldn't get her fist with the IV in her mouth.) Dr. Smoot finally came up at 9:40 and made the decision to go ahead with Annie's cath and just skip the skeletal muscle biopsy. That was what we wanted at that point because I was not willing to fast Annie for four hours during the day and go through the whole process again. Dr. Smoot is comfortable with skipping the skeletal muscle biopsy at this point because of all of the metabolic testing that has been done already.

They'll be redoing her IV in the cath lab (under some sedation because I was a mother on the edge at that point and ready to pull the plug on the whole thing over a non-functioning IV) and giving her the B12 shot as well. Dr. Smoot said to expect an hour and a half for the procedure, start to finish. So here we sit, waiting. The room seems awfully empty without Annie in it, even if she is only a little bug.

Somehow, I missed the message that Dr. Smoot doesn't do the cath! It's Dr. Lock, Chief of Cardiology here, and another cardiologist performing the cath alone -- Dr. Smoot isn't in there at all. We hadn't met Dr. Lock, which I thought was strange. It would be one thing if it were me and I would then meet the doctor performing the procedure when I went back to the lab or OR or whatever ... but to hand over Annie and not meet the doctor who was about to work on her seemed strange to me. The nurses called Dr. Lock's office and had him come up and meet us before he went back to work on Annie, which was great. She may be their patient, but she's MY baby. I want to look at the person who is about to work on her so invasively!

More as soon as we know anything or get back up to our room after recovery.

P.S. Not a great photo, but this was Annie this morning as we tried to keep her entertained before heading down to the cath lab.

Back at Children's

We're here and settled in. We've signed consent forms, heard about all of the nasty possibilities and been briefed on what to expect tomorrow. Annie will be the first case of the morning, with Dr. Smoot, Dr. Lock (Chief of Cardiology here at Children's) and another cardiologist whose name escapes me working on her. There will also be an anesthesiologist present, which isn't always the case with these procedures. (Sometimes the cardiologists do the anesthesia themselves, but there was concern about Annie's jaw formation so they called in the anesthesiology team. The doc who saw her didn't even mention it, so I brought it up. He said not to worry -- it didn't look bad to him at all.) There also will be a liaison nurse who will report Annie's progress and condition to us throughout the procedure.

I believe they are still planning on giving Annie supplemental B12. I think they only do that through a very painful shot, so we've asked if they will do the shot while she's sedated. Hey, why not? After just going through the IV, I'm glad we asked! Annie usually is happy as can be through blood draws and pokes and prods (smiled through her chest x-rays tonight as well), but this IV didn't go so well tonight. Ugh. If only I could do all of this in her place! To top it off, Annie has been chowing on her left fist and fingers lately ... but the IV is in that hand and wrapped up like crazy. She got very fussy when she tried to get her hand into her mouth. She is just NEVER fussy! I haven't learned what to do for her when she's like that because I've never had to. Nursing always worked for Jamie, but Annie doesn't nurse. Sigh.

The good news is that the resident assigned to us this visit seemed happy with Annie's muscle tone. Apparently the hypotonia (floppiness or low muscle tone) can progress with some metabolic issues, but Annie's definitely has not progressed. She did quite well tonight for the resident, as a matter of fact! I was impressed and glad to see it. Maybe the hypotonia ISN'T related to the cardiomyopathy ...?!?? She's definitely not where she should be for her age, but she is doing things that she wasn't doing at all just a week or two ago. We'll take it!

We didn't see the genetics/metabolism fellow so we don't know much more than that right now. Dr. Harris will be in tomorrow, so I'm hoping to have a good long chat with him and clarify all of the methylmalonic acid/fatty acid oxidation/etc stuff.

We'll post during the cath as we get updates, if possible. Otherwise, it will have to wait until Annie is out of recovery and we're back in our room. 'Night

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!

Hooray, hooray, hooray!

We met with the geneticist today and it was all good news! He didn't feel there was anything to worry about genetically and didn't even order any blood tests (the main thing I was dreading after our last experience). He actually said that Annie does NOT have Pierre-Robin because, strictly speaking, she would have to have three characteristics -- not just the small jaw but also a cleft palate and another thing with her tongue (long medical word I can't remember). That's even more good news! His opinion is that Annie's small jaw is a result of restricted growth in-utero. (Warning: overshare of information ... but I have a uterine anomaly that can restrict growth. That's why I have multiple ultrasounds during pregnancy. Dr. Moeschler tied the growth restriction to that anomaly as a probable cause.) He feels that with the restriction removed (i.e. being born), her jaw will grow normally and "catch up" with her upper jaw over time. He said he can't say that with 100% certainty, but it's his opinion.

We went from the geneticist's office to Annie's regular two month check-up. Again, all good news. She is a whopping 9 pounds, 2 ounces now (finally surpassing her big sister's BIRTH weight) and made it to the 10th percentile. She is -- hooray, hooray, hooray -- doing great! She is smiling up a storm -- huge, gummy smiles -- and cooing back and forth with me all the time in little mommy/baby conversations. She has great head control and seems very strong for her advanced age of two months. (Ha ha.) Her eyes are as blue as Jamie's were at this point so it really looks like brown-eyed Scott and I will have two blue eyed girls, believe it or not. She still is not able to suck, but we have gotten into a routine with everything and we're hanging in there. Ninety to ninety-five percent of her nutrition is still breastmilk, so I'm happy with that.

Unfortunately, she's being uncharacteristically fussy right now so I'm not able to get some new pics uploaded. I want to get this post up right away, so I'll have to add the pics later. I keep trying to catch her smiling, but so far no luck ...!

One last hurdle to come -- her second echo is scheduled for Feb. 9th, one month from tomorrow.

Three Specialists in a Week!

I am sorry to have been so slow in getting another update posted. I think life would be hectic enough with the addition of another kid (it's hard to get used to saying "kids" or "the girls"!), but this time of year has made it even more hectic. Thank goodness for on-line shopping, that's all I can say! Anyway ....

Several weeks ago, we met with a pediatric neurologist. He said that he sees many babies who are referred due to suck/swallow or feeding issues. He checked Annie out and felt that her muscle tone was good and that she looked good otherwise (strength, etc.). Before the appointment, I had become convinced that Annie's feeding issues were solely anatomical with her lower jaw formation, but he didn't agree. He felt that Annie is having trouble coordinating everything as well, but it's not anything that warrants an MRI at this point. He felt that the MRI would, in all likelihood, come back normal so it isn't worth the risk of sedation and all of that. (Fine by us.) The other option -- if we did find something -- wouldn't change how we're handling Annie at this point (working with the special bottle to feed her, etc.) so, again, it's not worth doing anything else at this point. I gathered from him that it's a wait-and-see game for him. We didn't make a follow-up appointment now, but we'll wait to see how things develop with Annie over the next couple of months.

The ENT guy seemed to be concerned mostly with making sure that Annie's airway is not compromised by her lower jaw formation. He said that her breathing is good and there is no reason to be concerned about that. He found fluid in her ears, so he wants to check on that in 6 weeks. She didn't have an ear infection; rather, he felt that it was a drainage issue that could be tied to her anatomy/jaw positioning.

The lactation consultant in Concord recommended the appointment with the ENT doctor as a means of getting to a speech pathologist. Through both of them, we met up with a speech pathologist who works for the state. Her specialty is suck/swallow issues, especially with preemies and newborns. She came out to our house a couple of days after we met with the ENT and she was awesome. She definitely was the person with whom we needed to connect! She worked in a NICU for 10 years and just seemed very knowledgable and competent. She, too, talked about Annie's airway, saying that she was doing a great job "protecting" her airway when she eats. Overall, everything she had to say was positive as far as how Annie is coping with her anatomical challenges. We talked about the coordination issue and she seemed to think that it was a tough call -- would Annie have the coordination issue if she didn't have the anatomical issues to deal with? No one can know that just yet.

The speech pathologist did put a name to this and said that Annie has Pierre-Robin Sequence. The sequence, as far as I understand it, is just the lower jaw formation. (At least that's about all it is for Annie. Some babies with PRS have cleft palates and require either feeding tubes or tracheotomies to help them breathe or eat.) Interestingly, in some cases PRS has been linked to issues or disruptions in the first trimester of the pregnancy. INTERESTING since we know that whatever happened with Annie's heart happened in the first trimester. In fact, the speech pathologist was glad to hear that Annie's heart had already been checked out -- I guess there can be some link there to the development that occurs in the first trimester. (I get in over my head on the science very quickly -- my apologies to our doctor and medical friends!)

The thing with PRS is that it is linked to other issues in about 40% of cases. So ... you guessed it ... we're on to another specialist. We'll be seeing a medical geneticist sometime in the near future so that full genetic testing can be done. The most common syndrome linked to PRS just doesn't fit for us -- I think we would have seen it elsewhere in our family by now and the symptoms just don't add up for Annie -- so I am cautiously optimistic that the genetic testing won't reveal anything too exciting. That could be denial at this point though.

Meanwhile, Annie's chromosome analysis and other blood tests have all come back normal so far. She continues to gain weight, albeit slowly. She was six weeks old a few days ago and we weighed her at a whopping 7 lbs. 12 oz. Still, she is doing everything she should be doing (except nursing!) and seems alert and happy. She's very calm and only cries when she's really hungry, and that's not very often. We still have to wake her up or feed her by a schedule, because she rarely lets us know she's hungry.

Jamie (since a few people have asked) is still in love with being a big sister!! She thanks me just about every day for having her. Out of the blue she'll say "oh, thank you, Momma, for Annie!" (Seriously.) She's having a hard time not having her every whim and desire met at the drop of a hat, but mostly she blames Scott and I for that. Annie still can do no wrong as far as Jamie is concerned, she just wishes she wasn't sleeping and eating all the time so that she could 'play' with her more.

The photos are from Annie at one month (on the red blanket with hearts from my sister Lori) and on the plane on our way to St. Louis to surprise my father for his 70th birthday. Jamie's pic is from a few weeks ago. She decided to 'pretend' to be sleeping, which lasted about one minute before she was really asleep (of course).

Bahama Mama & Other News

I said life had been busy, but it was news for another post. As it turns out, we've had a bit of a domino effect and still need good thoughts for our sweet new girl.

It started with jaundice, which landed us in the other hospital across town for a night. (We referred to Annie as Bahama Mama whenever we put her 'sungear' back on and put her back under the lights. She looked quite relaxed in there, as if she was just waiting for her margarita to appear.) Her bili count was quite high (22) and we were told initially that we would have to be in the hospital for 2 -3 days. At the last minute (and right before my friend Nikki showed up with treats and a stack of magazines for me, in anticipation of a long haul in a boring hospital room) we were told we could go home just a day after we had been admitted. Annie's bili count came down to 13 and we were sent home with a "Wallaby" light blanket to continue getting her bili count under control.

We spent Tuesday night (11/7) in the hospital, and got home late Wednesday night. In the hoopla of getting settled back at home and getting Annie wrapped up in the Wallaby, Jamie informed me that something hurt ... that she had "Spicy pee! It's SPICY!" Well, that's about the most accurate description of a UTI as any I've ever heard, so I knew immediately that Jamie would need to see her doctor on Thursday when we took Annie in for another blood test. By that Thursday morning, I realized I had a sinus infection, too. Luckily, we all have the same doctor so it wasn't too convoluted to have our sad trio seen. When it rains, it pours! Antibiotics for two of us and a stable bili for Annie and we seemed to be headed in the right direction ....

But ... it's still been a bit of a rough road with Annie. We thought she was tired from the jaundice (a side effect) so we had to work incredibly hard to get any food into her. Nursing didn't seem to be going anywhere. To make a long story as short as possible, we thought she had come out of it last week (the week of 11/13), but a weight check that Friday showed she had lost four ounces over the course of the week. Our little peanut was down to 6 lbs. 3 oz., a full pound less than what she weighed at birth.

She obviously was not nursing effectively, raising all kinds of questions as to why. This past Monday, we had blood drawn (from her arm -- much worse than the 8 heel sticks for all of her bili tests!) for genetic testing and chromosome analysis. We expect the results sometime mid to late next week. We have also made an appointment for her to be assessed by a pediatric neurologist, as ineffective sucking can be a sign of neurological problems.

In the meantime, I went up to Concord to meet with a highly recommended lactation consultant. She immediately switched us to a special bottle that helps Annie eat. The major concern is her jaw construction. I joked on my way out of the OR that Annie's second chin had swallowed her first one, but it turns out that it may be something more serious. Her lower jaw is substantially smaller than her upper jaw, something that can be a sign of genetic issues or larger syndromes. (I have mentioned the jaw thing to just about everyone who has looked at Annie. The only one who really commented was one of the pediatricians during our second hospital stay. She agreed that it could be a sign of something larger, but usually it was seen in conjunction with other symptoms or physical abnormalities, none of which Annie has.) The lactation consultant and another nurse who took a look at Annie suggested we see an ENT specialist to evaluate her jaw and suck/swallow issues. We now have that appointment arranged for Wednesday of next week.


The concern now is getting Annie to eat, gain weight, and get her strength up. The new bottle seems to be doing wonders, as she has gained six ounces by our scale in the last four days. Meanwhile, we are back to worrying about our girl and wondering what -- if anything -- is going on over and above the physical formation of her jaw. The research seems to say that a lot of children born with small lower jaws/recessed chins grow out of it all on our own. We are hoping that will be the case with Annie, but we're glad to be doing the bloodwork and extra doctor visits at this point. It's better to know what's going on sooner rather than later.

This girlie is not going to let us rest just yet ... so please keep her in your good thoughts and prayers. Scott and I are doing our best to manage her feeding schedule around the clock (Scott is awesome with her and keeps track of everything so my overtired brain doesn't have to!) while still having energy and patience for our rambunctious Jamie. (Jamie still loves being a big sister three weeks into it, but she definitely is aware that life has changed! She has learned, too, that there are times when Momma can't jump up and run after her and is taking a wee bit advantage of that.) Thanks to my mom and dad being here for a few weeks, my friends helping out, and the Thanksgiving holiday, I have had a lot of help. Next week will be a big one, though, with Scott at work all week and me on my own ... outnumbered by the little ones!

I Wasn't Crazy After All

Thanks to everyone who e-mailed to let me know they had seen my mysterious disappearing post! Apparently, it posted that night but then disappeared at some point in the next couple of days. (As I mentioned before, I didn't even get a chance to look at it for several days, so I don't know when it disappeared or, much less, HOW it disappeared!) But thanks to the kind folks who wanted to assure me I wasn't that doped up in the hospital and didn't hallucinate the fact that I posted the echo results late that Friday night!

Sorry again to anyone who was waiting for news. We appreciate the concern and certainly didn't mean to make anyone wait for the good news!

~Ross

Mysterious Missing Post -- Echo Results

Mysterious is a big word in our house right now, thanks to Jamie's favorite episode of "The Backyardigans." No more toys in our doctor's office is "so mysterious" as is anything she can make into a mystery! So I suppose it's appropriate that I have a bit of a blog mystery. Ok, so it was 3:30 in the morning (11/3) while I was still in the hospital and on serious pain medication ... but I still can't account for the update I worked on and THOUGHT had been posted. Of course, I can't even begin to recreate it or even remember most of what I said. I remember it being a bit schmaltzy at the end -- tired and doped up as I was -- with Annie, Jamie, and Scott all sleeping soundly in the room around me.

In any event, the gist of the post was the all-important results of the echo. Just a little something we've been waiting on for months ... hopefully we have gotten the word out via e-mail to just about everyone by now. For anyone who has been waiting on news -- I am so sorry! I honestly thought I had gotten a post done that night, but things have been a bit busy (a story for another post) and I haven't been back to the blog since then. I have no idea what I did, but somehow the blog didn't post that night -- the most important post of all!

In any event .... We had a bit of drama actually getting the echo done, but eventually the pediatric cardiologist came over to our hospital after a full day at his clinic across town. I told him it was terrible, but I really did hope we were wasting his time and taking him away from his family for no good reason! And that turned out to be the case, after a very quiet echo. (I know I was holding my breath, but I can't speak for Scott.) Dr. Johnson went through every part of Annie's little heart methodically, eventually granting us the best word I've ever heard in the English language: normal. NORMAL! Annie's heart appeared normal, normal, normal! There is no visible thickening left in the walls of her left ventricle; if anything, there was a tiny bit evident in the septum but nothing to worry about. There is a leak in her mitral valve, but he's not concerned about that. It's common in newborns from what I gather and he said he wouldn't even worry about another echo if it weren't for Annie's dramatic history. So, one more echo in two months to check on her mitral valve ... but still, all glorious good news.

It turns out you can feel when the weight of the world lifts off your shoulders! I slept better that Friday night than I had in months, that's for sure. Thank you to everyone who has hoped for and prayed for this wonderful outcome along with us. Our sweet Annie is here and healthy!!!!!!!!

Announcing Annelise

Annelise was born at 8:02 this morning with loud cries to let us know she was out and in the world. She weighed in at 7lbs, 3 oz. and is 19 3/4" long. The pediatrician and nurse proclaimed her perfect, with absolutely no concerns at birth. She was able to ride out of the OR on my chest and met her big sister, Mam, and Pap back in our room. She is pink and looks great!!

This afternoon the nurse detected a heart murmur, so we're waiting to hear more about that. We're hopeful that it's just a normal newborn murmur, but obviously we'll be able to discuss that with the pediatric cardiologist tomorrow. Not sure when the echo will take place, but I'll do my best to post as soon as possible ... with all good news!

Thanks to everyone who has called to check in. Sorry it took so long to get this posted! Things went well this morning, but I didn't sleep much last night and it's been a bit of a long day. :-) But as I sit here typing, Annie is sleeping peacefully in her Mam's lap. Hooray, hooray, hooray!

Tomorrow's the Big Day!

We're on for tomorrow at 7:30am! We'll post as soon as we can after Annie is safely, finally, happily in our arms.

Thank you to all of you who have expressed your best wishes and thoughts for tomorrow morning! We appreciate it and can't wait to share our good news with all of you.

Scott, Ross, and Jamison

Delivery Decision

I feel like a huge weight is off my shoulders! We are going to deliver Annie here in Manchester!!!

I had a growth ultrasound at my regular OB's office on Thursday and saw the doc on Friday. It has been 2 1/2 months since the last ultrasound in this office and wow -- what a difference. We couldn't see any bright spots in Annie's heart at all. Last time, her left ventricle was a complete, bright white circle. Everything else looks good, although she is much smaller than Jamie was at this point. They are estimating that she is about 4 lbs. 15 oz. right now, putting her in the 41st percentile for size. Jamie was never below 85th percentile, I think! If Annie gains the expected 1/2 lb. per week, she will be just under 7 1/2 pounds at birth. We'll see!

In any event, talking to my regular OB was the last hurdle in deciding where to deliver. She has seen the latest report from Boston and contacted the pediatric cardiologists here in town. (They have been receiving copies of the reports from Boston as well.) The cardiologists are willing to come over to Catholic Medical Center to echo Annie the day after she's born! That was the big question -- whether or not we could get a newborn echo done at CMC. With that piece in place, the next big question was about the worst case. IF something were to happen (as the cardiologist said in Boston "if this baby hiccoughs funny, they'll put her in an ambulance and send her here"), would I have to be separated from Annie? The answer is no. Dr. Johnson said she would either discharge me early so I could go to Boston with Annie or she would transfer me down to Brigham & Women's at the same time Annie is transferred. Everyone -- including Dr. Levine in Boston -- just doesn't think that's going to happen, but it's good to know that the worst case scenario does not mean being separated from Annie.

Soooo ... Catholic Medical Center it is! We are on for November 2nd: a mere four weeks, five days from now. The OB was happy to see another ultrasound at her office and ecstatic about how Annie is doing. We'll do one more ultrasound at the end of October, but we would have done these growth ultrasounds anyway because of other issues. Meanwhile, it was a bit of a jolt to be talking about delivery with Dr. Johnson and going over all of the precautions they're going to take to try and keep me from contracting a post-op infection (as I did with Jamie -- and I guess once you're colonized, you're colonized). It felt like a major shifting of the gears to be discussing that instead of heart stuff! Worrying about a little (ha, ha -- I know Mom is rolling her eyes at that one) infection seems silly after all of what we've been through the past few months. But I'll take the shifting of the gears any day, thank you very much!

Posting those Annie photos and closing out this blog is closer than ever .... Thank you to EVERYONE for the comments, inquiries, concern, prayers, and good thoughts. We are so grateful to everyone who has shown interest and concern and checked in on us. We feel very fortunate to have you in our lives. ~ Scott, Ross & Jamison

Non-stress test

I am falling down on my job as blogger! I got a few questions, so I knew I had better update the blog (thank you everyone for your concern!). The non-stress test last week (last Wed.) was 'perfect' according to the OB. Her baseline heart rate stayed around 120, with typical spikes when she was moving. The only time it slowed at all was right after I talked to her, in an attempt to wake her up!

Five weeks and counting .... Yes, counting trips to the bathroom at night, counting how many Tums I've had in one day, counting the times each day I think about napping ... it's all about counting!

Third Echo in Boston

We just got home from our third visit to Children's Hospital in Boston. We are happy to report more good news! Dr. Levine deemed the thickening of Annie's heart to now be 'virtually insignificant' and said that her heart function continues to look perfect. Once again (it's really a running joke at this point) Annie was not in the best position for Dr. Levine to get a good look at her heart, but she was able to see enough to feel comfortable in telling us how encouraged she is. (And when she made a comment about Annie not being in the best position, she was rewarded with not one but two STRONG kicks right where the echo tool was located on my belly.) In fact ... she doesn't feel the need to see us again before Annie is born! That has to be a good sign, right?!?

On a side note ... for most of the echo, Annie's heart rate was much slower than we have ever seen it before. It was still within the 'acceptable' range, but definitely on the lower end and much lower than they have ever seen it before. I just heard from my OB's office and they are going to go ahead and bring me in tomorrow to monitor her heart rate for a block of time, just to make sure nothing is going on there.

Now we really have to figure out where to deliver. The cardiologist is comfortable with us delivering here in Manchester. She was careful to point out that if anything does seem the tiniest bit out-of-the-0rdinary when Annie is born, though, they probably won't hesitate to send her immediately to Children's via ambulance. That would mean being separated from her until I was ready to be released from the hospital. Dr. Levine feels like the chance of something like that happening is very minimal, but wants us to be aware of it. So, we have to decide if we want to risk it for the comfort of delivering up here or not. All in all, I'm so glad this is the 'tough' decision we are faced with at this point! I'll take this dilemma over what we were offered two months ago (transplant, etc.) any day.

Overdue Update

Sorry it's been so long since I've posted anything. I didn't realize it had been so long until I got a few e-mails asking what was going on. We've been crazy busy with the end of summer and getting back into a schedule (Jamie now goes to preschool 3 mornings per week). And ... we haven't had much to report. I have been back to my OB here in town a couple of times and they have checked Annie's heartbeat. Her heart rhythm has remained strong and stable. Meanwhile, she is active and feisty and reminds me constantly that she is in residence!

The blood tests came back from Boston on the second titer for the coxsackie virus. I'm not sure about the biology behind this, but they did tell me that my titers were slightly higher than the original test. Apparently they weren't high enough to be considered a full-on infection, but I'm not sure if that means that I don't have the virus currently or what. I thought the question was whether or not I had the virus early in the pregnancy, but I'm not sure if these tests tell them that or not. I'll have to wait for my next OB appointment to ask.

We are back to Boston this Tuesday, 9/19. We will only be meeting with the pediatric cardiologist at Children's this time. (The perinatologist is going to wait to discuss the latest echo findings with the cardiologist to determine our next step; mainly, determining if I should deliver in Boston or up here.) Obviously, we are hopeful that we will see a huge improvement in Annie's heart on Tuesday! It has been four weeks since our last echo -- the longest span between any ultrasounds or echoes since June -- and last time we saw such an improvement after 2 1/2 weeks that it's hard not to be hopeful. Please keep us in your thoughts on Tuesday and we will post any news as soon as we get home.

Hope everyone is enjoying a wonderful fall! Thank you again for all of your e-mails and expressions of support, prayer, and love. -- Scott, Ross & Big Sister Jamie

Good day in Boston!

We had a good day in Boston today, thank goodness! We began, again, with an ultrasound at Brigham & Women's. As soon as the tech put a close-up of Annie's heart on the screen, I got Scott's attention (he was keeping Jamie entertained) because it looked so different to me from the last time. It was definitely less bright (the brighter it is, the more dense the tissue is) to our untrained eyes. You could still see some brightness down the heart's septum, but it definitely seemed less obvious than our last look.

We had a new radiologist this time, and he asked us to tell him what had been diagnosed and what the cardiologists had to say at the last visit. He was a character, but had good things to tell us. He said that if he were seeing us at B&W for the first time with no history of any trouble, he would not be "impressed" by this presentation. I told him we were more than happy not to impress him! We discussed the fact that it looked much less bright and less comprehensive (not involving the entire ventricle in a complete circle of bright white/thickened tissue) to us. He deferred to the echo we had scheduled later in the day, but said that he really wouldn't be concerned about what he was seeing. He also said that from his point of view, the heart function looked great. They also checked for growth and development in general, estimating Annie to be about 2 lbs. 6 oz. right now.

We went straight from B&W to Children's and met with the cardiologist, Dr. Levine. We told her the news from the morning so she was anxious to get a look at Annie herself. On the way to the echo room, she told us that she had just seen the 'baby' that was the only case she had ever had with anything like Annie's presentation. She had wanted to look up his echoes, but had been unable to remember his name. Lo and behold, he showed up in the clinic for his annual appointment a week after she met us! He's not a baby anymore -- he's five years old -- something I had been wondering about. (We had no idea how long ago this case had been, so we had no idea how long the child had been followed and deemed healthy.) Dr. Levine said that his heart does not show any thickening at all at this point -- and continues to function perfectly -- so that was even more good news.

The echo was quick this time since Dr. Levine had just seen us 2 1/2 weeks ago. Annie was in a completely different position, but still had a hand in front of her heart. Still, she has grown so much that the size alone helped out. Dr. Levine agreed that the thickening appeared much less comprehensive than it had at the last echo. She was able to confirm her suspicion that the thickening does not appear to be in the endocardium but more in the outer lining of the heart, so she's still saying it's not EFE. The thickening is definitely most evident in the septum, but the diminished nature of it in general made her even more positive about Annie's prognosis. She told us that she knew she had told us she felt 50/50 like this would turn out to be nothing to worry about, and today she said she would raise that to 70/30. YEAH!!!!!!

We talked a little bit about the positive blood test for coxsackie virus as well. She said that the retest they did this morning at B&W would help them determine if I had been exposed to the virus during my pregnancy. They know that the virus can cause cardiac abnormalities in fetal hearts, so if I have been exposed she could make a reasonable assumption that the virus caused something to happen with the development of Annie's heart. We'll never know for sure, of course, but it's the most likely culprit at this point. (She even told me that the virus can cause so much damage in children's hearts that they require transplants. I had no idea the virus could be that severe.)

So, we're headed in the right direction. We're happy the radiologist this morning wasn't impressed by anything he saw (!!), and we were definitely given reason to think positively by the cardiologist this afternoon. It was a good day! Thank you, everyone, for keeping us in your thoughts and prayers today. Hopefully, we'll be able to continue delivering good news and one day close the blog out with Annie's first photos!

Change to Boston Appointment

I received a call from the perinatologist at Brigham & Women's yesterday (Monday). Apparently, one of the blood tests they ran came back positive for the coxsackie virus. That virus is linked to EFE, but we're not operating under that diagnosis anymore. Still, it sounds like they don't know what to think. When Dr. Dunn called the pediatric cardiologist to discuss the results, they both decided they would rather see me (ok, Annie really) again sooner rather than later. So, we're back to Boston on Friday for a repeat of everything we did a couple of weeks ago: another ultrasound at Brigham, appointment with the perinatologist and a second blood draw, and another echo at Children's with Dr. Levine.

Annie continues to be active ... so active that it is hard for me to imagine that anything has gotten worse. Needless to say, I hope that we are not surprised unpleasantly tomorrow. Either way, I'm glad to have the echo moved up. Hopefully, Annie will be in a better position -- and goodness knows she has been growing -- and they will be able to get a better look at her heart. It will be the second study on the same equipment with the same settings, also making it easier for them to discern any difference from two weeks ago.

Right now I have to pack for the beach ... we are going to stay with a friend overnight tomorrow night and Jamie is asking if we're going to the beach NOW about every 10 minutes (really). :-) I will update the blog as soon as we get home on Friday to share any news.

Thanks!

Next Appointment & Jamie's Heart

We're headed back to Boston on September 5th for our next echo with Dr. Levine. It will be about 4 1/2 weeks since the last echo at that point, but we do have another ultrasound scheduled up here before then (8/22). I see my regular OB up here tomorrow, but I doubt there will be anything to report. It's just the waiting game right now ....

Meanwhile, Annie has been doing a pretty good job of making her presence known throughout the day. She hardly gives me a chance to start worrying about her! I had gotten a feeling that she was a calm baby (ok, maybe it was wishful thinking -- thinking I was due a calm one after my exuberant Jamie!), but I think I'm changing my mind. Annie can be pretty feisty, especially once I've crawled into bed at night. Uh, oh. Another night owl, maybe?!?

And today I took Jamie into see her pediatrician. I initially made the appointment the day after Annie's first echo, thinking I wanted to hear Jamie's doc tell me her heart is perfect just for my peace of mind. EFE has a slight genetic factor, but diagnosis is usually made between the ages of 2 and 12 months. We would have known by now if Jamie had EFE, without a doubt. And now that the doctor doesn't think it's EFE anymore and more likely some event within this pregnancy ... well, maybe I should have cancelled the appointment. I didn't though -- I just wanted to have her doctor listen and check everything out. Happy news, of course -- he said she's perfect. Her pulses are all great and there isn't the slightest hint of any sort of murmur or other heart problem. And goodness knows she doesn't suffer from any growth issues. She seems to be leapfrogging over size 4s and jumping right into size 5s.

Thank you, everyone, for your continued support and words of encouragement and friendship. They truly are appreciated!

Annie Photos

I realized suddenly during the first ultrasound in Boston that we were out of the sticks (no offense to NH -- I love living here and appreciated it even more after a day of Boston traffic!) and they might have the cool 3D ultrasound. The tech switched to it just to show us, but said that it was still early to get the kind of images you see advertising the technology. Scott says it's still a frightening image, but I think it's cool. (It's a look at her face straight on, with her hand up by her face on the left side of the pic.) I hope I can convince them to give us another peek later on. The second image is a regular ultrasound image of Annie's profile. Just wanted to share ....