Monday, Snowy Monday (And a Blog Surprise!)

Annie is doing really well. Thanks to everyone who e-mailed and asked about both girls after our last post! (Jamie never got sick again outside of the ER and was fine the next day ... go figure.) Today Annie is just about up to where she usually is on food (and it has been easy to keep track since we have been snowed in all day!). In fact, our little mimic had a ball feeding herself with a spoon for the first time. She thought it was HILARIOUS. Enjoy our girl in action (it starts out slow, but give it a second -- it's worth it!).

ER Tonight

Fun-filled four hours in the ER tonight! We decided we needed to make sure Annie wasn't getting too dehydrated after three nights of upchucking and four days of refusing food. (The million-dollar question from everyone was whether or not she was still getting fluids. Um ... you mean the thin liquids she is not supposed to have? How do we deal with that one?? She needs fluids (!!), but we don't want her aspirating ....)

Well, we headed to the ER about 6:30 tonight after going back and forth all afternoon about it. We found out that the cardiac playing card does get you some panache, as we were ushered through the hallowed double doors immediately. You tell them "cardiac kid" (or some version thereof) and it seems as though the doors open magically before you. I also discovered that they take you very seriously as a parent. One of our nurses commented on the "parents of kids with medical histories like Annie's" and the fact that we know when to raise the red flag. (Well, sort-of ... I'm not sure we really needed to be there tonight ... but Scott is sleeping soundly tonight and that counts for a lot. He was getting pretty worried!)

So here is the really, really fun life-keeps-you-laughing thing: we were ushered into triage with a nurse immediately after our arrival. While in triage, we told the nurse all about Annie (her meds, diagnosis, history, etc.). In the middle of our Annie story, Jamie suddenly started throwing up. Funny, right? Well, it's funny now at any rate! Really, we seemed to be falling apart at the seams before their very eyes. ("No, we're not here for her. We're here for the little one -- the one laughing and playing peek-a-boo with you.")

Long story kinda short, but we spent about four hours in the ER. Annie's blood and urine were tested and she had a chest x-ray (all tests that were so much less fun than when she was younger -- she was not happy about any of them this time -- ugh). All of her tests came back normal, including her electrolytes -- so she didn't even need IV fluids. (No, really, I'm glad Scott is a bit of an alarmist. If I had vetoed going when we did, Murphy's Law says that we would have ended up going at 2am or something.) They figure it is just a virus that we need to keep an eye on at this point. (Jamie continued to not feel well while we were there -- and that's a sanitized description -- and they asked us if we wanted them to start a chart on her, too. We declined ... here's hoping that doesn't jinx us. So far, she seems to be sleeping just fine ....)

Just another day in the world of parenting adventures! I'm happy that tonight was only our second experience with ER visits. I know that many cardiomyopathy kids on the list serve are frequent flyers! Still, they had all of our info in the computer already .... Jamie has never been to that hospital or ER, but Annie's practically a regular. Sigh. And that officially begins my butterflies for the next echo, 1/24. Here we go again ....

P.S. I have some pics of the girls in the ER (enjoying popsicles together on the gurney and also Annie kissing on her sister and daddy), but I'm too tired tonight to get them downloaded. I'll get to it ASAP, I promise.

No Echo Yesterday

Just a quick update to let everyone know that we did not go to Boston yesterday. Annie became sick on Sunday (fevers/lethargy) and still is under the weather. Plus, Dr. Smoot was not able to meet with us because of a schedule conflict. All in all, it was best to keep Annie home and visit our nurse practitioner.

The biggest problem is that Annie is refusing milk almost entirely. We have been lucky to get 4-5 ounces into her per day. She won't even let us sneak it in on her while she's sound asleep! She is also fighting her meds like she never has before (usually she sucks them right down out the syringes). Coughing fits in the middle of the night spur throwing up ... well, you get the general picture. So far she is staying hydrated, but, just the same, panic hit me this morning. I put a call into our cardiologist up here, and I hope that they will bring us in ASAP for an echo just to rule out any correlation between what's going on and decreased heart function. Ironically, Wednesdays our doc spends down at Children's in Boston, so tomorrow will be the earliest we'd be able to see him anyway. (Ugh.)

So that's where we are! I have officially cancelled the bronchoscopy at this point. I am going to wait until we speak with Dr. Smoot about it in person, and our visit with her has been rescheduled for late this month. If we have an echo up here before then, though, we may have to reschedule the Boston appointment AGAIN ... and so it goes. (Yes, I do feel like I'm chasing my own tail sometimes!)

Anyway, thanks anyone who was checking in for an echo report today. Wish I had some more good news to report on that front (oh, how I wish!), but we've got a little bit of a bump to get through right now.

It's Going to be a Long One!

No, I'm not referring to the winter in this blog's title, although that would be an accurate statement! I mean it's going to be a long blog because I was lazy over the holidays and only wrote blogs in my head -- never managing to get anything posted. I never got around to writing a separate post about the bronchoscopy (as advertised in the last post). So beware, this will probably be a long one, if the kids cooperate and let me get it done before demanding some breakfast (they're so gosh-darn needy, silly kids -- I have to feed them and everything, ha ha ha).

I'll start with the medical stuff so you don't have to hang in for the long haul: First of all, we started the new ACE-inhibitor drug for Annie that I mentioned in the last post. It has made an incredible difference in our lives! Annie's meds are now only at 7am (beta blocker, ACE-inhibitor, Digoxin, Lasix, and her reflux med) and 7pm (just beta blocker, Digoxin, and reflux med). Honestly, the freedom this change has given us has made a world of difference. No more late night dose, no more midday dose (the hardest one to remember!), and no refrigerated meds. I won't go on and on about it, but if you have gone through it with your kids you know what I'm talking about. If you haven't gone through it yourself, you have no idea! (All the same, I found myself wanting our Christmas vacation in Missouri to be a vacation from meds, too. It doesn't work that way ... but thanks to Scott I did get a bit of a vacation from them. He did all but a couple of doses the entire time we were gone. He's the best!)

I continue to be indecisive about Annie's scheduled procedures for her swallowing issues. As it stands, we will be going to her pre-op visit this Tuesday, Jan. 8. We now have an echo and visit with Dr. Smoot scheduled for the same day, so there is no way I want to cancel that! Still, I'm not sure we'll be going through with the bronchoscopy on the 16th. I think my latest decision has been to wait until we talk to Dr. Smoot personally. If she feels that she would not want Annie to go through a laryngeal cleft repair surgery, I really can't see the point of putting Annie through the bronchoscopy (which will determine if she has the cleft). If it's not going to change what we're doing right now as far as feeding Annie, I can't imagine why we would go through sedation, a hospital stay, an IV, etc. As for the CT scan and MRI currently scheduled for March, Dr. Smoot was leaning away from that when I spoke to her about it on the phone last month. Again, I am looking forward to speaking with her in person about all of this. So stay tuned for an update next Tuesday!

Other big Annie news: she's a chunk! Scott fed her and fed her and fed her over the holidays, stuffing her full every time he could get her to swallow (mostly when she was asleep!). She put on some weight -- about a pound and a half! -- in about 10 days. She is noticeably pudgier, with round cheeks and her toddler belly leading her way as she franken-walks around the house. It's wonderful to see her so chunky, but it also was a lesson learned. Between Annie's b-day party and the holidays, life was very busy around here the last few months. Scott's full-time feeding of Annie and the obvious results reminded me to slow life down a bit and focus on Annie more. Too often, I stop feeding her as soon as she falls asleep so that I can hop up and attack the ever-exciting, neverending "to do" list. I learned from Scott that I need to keep feeding her until she absolutely refuses another swallow. (Many times Scott brings her to me, telling me that "Bug is a stuffed tick!") So we'll be doing a few less playdates and generally trying to keep life a little less hectic ... easier said than done as school gets back into swing and life just rolls along, but I'm going to try!

So what else is going on? Well, thankfully winter "officially" started right before Christmas. Thank goodness! I don't know how we would have known it was time for Christmas otherwise.

Exhibit A (The view out my front door):

Exhibit B (Scott snowblowing a path for our elderly dog-kids in the backyard so they wouldn't have to mountain-goat-pee on the wall of snow immediately outside our sliders):

Yep, it's a snowy one! Snow affected our travel to and from Missouri for the holidays, as well. We had cancelled flights both ways, but we knew ahead of time in both cases so it was best-case scenarios: we were never stuck in the airport (or on the tarmac, for that matter!) and even got an extra night with Mam and Pap! Our poor dogsitter (thank you, Auntie Val! Nikki and Sandy, too!) had some adventures taking care of the pups, but she was a trooper. The dogs had a field trip to Val's house for the last (snowiest) night, and I hear they even made a friend!

In other news, we said goodbye to part of last year's struggle with Annie; a sad goodbye. Sheila, the wonderful friend and nanny who came to the rescue at just the right time (our need for help coincided with my friend Beth's maternity leave, so we were able to have the most fabulous Sheila for several months last year), headed home to Canada and the teaching career she was so clearly born for. We'll miss you so, so much Sheila! We look forward to hearing about all of your teaching adventures and we know exactly how lucky your future students will be to have you.

A couple of housekeeping things, too: We had birthday gift trauma at Annie's party in November. In the cleanup at Old Town Hall (in the pouring rain! -- thank you everyone who got us cleaned up and packed up in under an hour!!), gifts and cards became jumbled. I am so, so, so sorry! If you have not received a thank you note from me, it is because I don't know what you gave to Annie. Please, please let us know so I can thank you properly! (And please don't turn me into the etiquette police!) Also, if you haven't received a Christmas card ... well, I ran out! Our Christmas list has grown since last year, thanks to all of the wonderful folks who have become a part of our lives lately. Apparently, I didn't adjust my card quantity accordingly! I didn't have time to order another batch before heading out to Missouri for the holidays, and it was just too darn late by the time we got back. If I missed you ... um ... Merry Christmas! Happy New Year! (The photo at the top of the blog is the photo from the card, by the way.)

I know have more little things I have been meaning to write about, but time is up. Jamie just came in and said "I'm hungry. Will you please make me something?" Needy, needy. :-) Annie is awake next to me now, too. Morning has broken!

'Tis the Season

Long time no blog! I suppose it IS the season ... although we have had plenty of snow days this year already. I think I should have taken advantage of them and gotten a blog written, but I probably did some crazy winter-y craft with Jamie instead (or cleaned up after eight snow-ridden doggie paws and two kids -- just as likely!).

In any event, we haven't had too much to report. On the heart side, we have met with Annie's cardiologist here in NH. He was pleased with Annie's progress and patiently listened to me question/vent/explain about the upcoming procedures scheduled for Annie on the swallow side of things. (Thanks, Dr. J -- you said exactly what I needed to hear: "I think those are legitimate questions to ask.") The really big news at this appointment involved Annie's meds. I must have said something about the Captopril (but I don't remember what exactly). Dr. Johnson responded with a "why don't we switch her to Enalapril?" He went on to say that it would be just a 1/4 tablet twice a day. I couldn't believe it! That would be LIFE CHANGING for us. Then, after he checked his references, he came back in the exam room to say that it actually would be just 1/4 tablet once a day. Wow.

Let me backtrack here and say that meds rule our lives in every way. I have a friend who has a child who requires medications like Annie's (if not more) everyday. I am the first one to tell you that I had no idea what that meant for her family before we began our journey with Annie. As an outsider, you think -- or at least I did -- that it's just medication. You don't think about what that really means to a family. Now I know (and I have commiserated with my friend Teresa, who I am so grateful to have for chats like this! Thank you!). Meds for kids are a big deal, and I have to say -- with all humility -- that unless you have traveled this path you can not understand what it means. Who is going to get up for the first doses of the day? Who will stay up late for the last dose of the day? (My friend Teresa even has a 1am dose each and every night!) Will we be out running errands so long that we should pack her meds? (And don't even get me started on the planning for an extended trip!)

So, back to Captopril. Captopril has been one of the biggies for us in that it is three doses per day, meaning every 8 hours. That means an early morning dose, a late afternoon dose (the hardest to remember -- I just got the hang of having my Blackberry nearby so I can utilize the alarm for this dose), and a late night dose. In addition, Annie's Captopril is custom compounded for us and must be refrigerated. If I am going to be out around 3pm any given day, I have to load up a syringe and put it on ice to take with us. I don't tell you guys any of this for sympathy or anything -- it's more that I am shocked at how little I realized medications with children impact a family before I experienced it first-hand. It's all part of the routine for us now ... it's just a routine that takes planning, forethought, and effort ... all of which I never thought about before Annie.

So ... the Enalapril would be 1/4 tablet ((in applesauce or Annie's milk) once per day, would not need to be refrigerated, and would eliminate our late afternoon dose AND our late night dose. LIFE CHANGING, as in a whole new world!! I could have kissed Dr. Johnson!! We are waiting to hear from Dr. Smoot and make sure everyone is on the same page ... and we'll have to make sure that Annie is able to handle the once-per-day dose of this med without her blood pressure responding negatively ... but here's hoping! This switch would leave us with 7am meds and 7pm meds and that's it! Hooray!!! I don't want to get my hopes up too much in case Dr. Smoot says she'd rather wait until Annie is a bit older, but I would be happy just to know that this change is possible at some point. (This is the med -- an ACE inhibitor -- that Dr. Smoot said "we may well send Annie to middle school" still taking. I take it that it is one of the last meds to be withdrawn once a cardiomyopathy kid is considered to be resolved, so even more reason to be happy with one dose per day.)

This post is getting too long entirely ... I should post more often! I'll make the news about the bronchoscopy and the other stuff a separate post in order to give everyone a breather. :-) I have to add some pics, though, to make it interesting. (Annie waiting for Dr. Johnson, Annie on the Polar Express, and Jamie with the first snowman of the year.) Annie is franken-walking (as we call it) EVERYWHERE! If it is a cabinet or drawer under the two-foot mark, it is emptied daily by Annie. She is signing up a storm ("more," "water," "Mama," "Daddy," and "milk" so far) and attempting to say a few words. And the rest I'll save for another post ....

Sorry for not Updating

Sorry for not updating sooner ... I really haven't had the energy or the words. (Sorry if that sounds whiny, it's just what it is.) The ENT follow-up was what we expected in some ways, but not what we expected in others. Be careful what you assume! I assumed they would be able to do the throat-probing at the same time they did the CT scan (i.e. with the same sedation) and that's not the case. I also hoped that they would be able to "fix" any problem they found in Annie's throat at the same time ... again, that's not the case.

As it stands right now, Annie is scheduled for a bronchoscopy (the aforementioned "throat probing") to check for a laryngeal cleft on January 16. The procedure itself should only take about 10 minutes, but we were told to assume we will spend the night at Children's after the procedure. Any sedation for Annie is a risk, so they plan on her spending the night to make sure she is ok after the procedure. Plus, her risk of aspiration means that an anesthesiologist will have to be present. (For her cardiac catheterization, the nurses were able to manage a minimal level of sedation for Annie. Now that her aspiration issues are known, an anesthesiologist will have to manage her sedation -- no matter what the level -- to monitor her and make sure she's ok throughout the procedure.) Dr. Smoot will schedule an echo for our pre-op visit the week before the procedure, just to confirm Annie's heart is still headed in the same (positive) direction.

If they find a cleft, we were told it is a complicated procedure to repair -- something they would do at a later date. In talking with Annie's cardiologist, we're not certain that we would go for a lengthy procedure at this point in time. So I guess the bronchoscopy will tell us whether or not it is a structural issue, but we may or may not move forward with repairing it. Annie is handling thickened liquids well according to her swallow studies, so we may just keep doing what we're doing one way or another. (The kid is addicted to water, though. She is obsessed with every water bottle she sees. I swear she knows she isn't supposed to have it! Any other kid and parents would be begging them to drink more water. This one we can hardly keep away from it! The water bubbler in the middle of the kitchen is a HUGE source of stress -- she knows water comes out of it and she WANTS SOME!)

The plan right now includes going back to Children's on March 10 for Annie's CT scan (which may also involve an MRI, depending upon how the CT scan goes). I'm not 100% sure that we're going to go through with the CT at this point. The ENT wants to confirm whether or not Annie's dimple in her nose is a dermoid. At this point, I just can't imagine putting her under (with another IV, another night in the hospital, etc.) for the outside chance that Annie could have a dermoid. I don't mean to second-guess the doc's expertise, but I am not convinced that it is in Annie's best interest to sedate her again for this procedure. We're still figuring out what we're going to do on this one, and luckily we have some time to work it all out.

Meanwhile ... I'm trying to get used to the idea of Annie going through another procedure. I keep telling myself -- and everyone who asks -- that it's not a heart transplant. But sill ... the idea of going through an IV and the risk of sedation with Annie at this age is just ... yuck. It has its own issues, just as going through the cath when Annie was so tiny had its own issues back in March. I just can't imagine it right now, but I know I couldn't imagine going through the cath with a 10lb. baby, either. We'll get through it. I know we will. That doesn't mean it won't make me nauseous between now and January 16, that's all.

The Party!

Just getting started ....

No kids' party is complete without face painting!
Heart Bean Bag Toss
Pin the Heart on the Bug!

Mike Morris entertaining the kids Most of the kids listening to (and singing along with!) Mike

Thank you, Bev, for the AMAZING cake and GORGEOUS flowers!!!!

Cake time brought all the kids at once!

Ups and Downs

If something wasn't going to be good news, I'm glad it's the swallow stuff and not the heart stuff.

We had Annie's second swallow study done in Boston on Monday. She has sounded great lately (no "wet" sounds after eating) and really seemed to be doing well when eating, so we were hopeful that the swallow study would be normal (or nearly there). Unfortunately, it was not better than the last study -- if anything, it was worse. This time, Annie did aspirate clearly at least twice (whereas last time she didn't clearly aspirate, but was said to be at high risk for aspirating). So, thin liquids (normal liquids for the rest of us) are still not safe for her to ingest. We can continue feeding her the thickened milk that we are giving her, thankfully, since she handled thickened liquids well.

The downside is that the ENT told us that he would take Annie into the OR to probe the back of her throat if this study wasn't normal ... which clearly, it wasn't. (We don't need to see the report to know that!) We don't have the follow-up with the ENT until the middle of November, but luckily we were allowed to go forward with what we have been doing (thickening her milk) and weren't sent into panic mode or G-tube mode.

It was a letdown to hear the news, but like I said ... if one thing has to go south, I'd much rather it be the swallow stuff than the heart stuff. Still, we hoped for better news and hoped not to have an OR visit in the near future. I thought I had prepared myself for the news, telling everyone that Annie had to be sedated one way or another (for the CT scan of her nose) so it was no big deal if she had to do the OR visit at the same time. Secretly, I was sure the news was going to be good and that we'd be fine. I was bummed ... but then Annie's wonderful physical therapist snapped me out of it and put it in perspective. She is the one that pointed out that we were home and not admitted to Children's in preparation for the placement of a feeding tube. Oh, yeah. Be grateful that we've gotten as far as we have ... and, my new mantra: it's not a heart transplant. It's not a heart transplant.

Meanwhile, it is difficult to remain bummed anyway, since New Hampshire has put on the dog and is gorgeous beyond belief right now. Every fall it strikes me that I live in NEW ENGLAND now (going on eight years now, unbelievably). The colors are simply amazing! And thank you, Scott, for patiently taking me leaf peeping every year ... despite the fact that you are totally red/green color blind and see little of the beauty! I only hope some of the color remains through next Tuesday, when my parents arrive. Almost eight years here and my parents never have been able to experience a beautiful New England fall. (Last year they missed out because they had to come at the very end of the color ... but just in time for Annie's debut. Really? Is she almost 1? In some ways it seems like it has been years since she was born ....)

Oh, and I almost forgot! Annie decided to take about six consecutive steps in the radiology waiting room before the swallow study! She first rolled over when she was a patient at Children's last February, so I guess she has decided to mark milestones there .... Whatever the case, there was a time when we weren't certain that she would walk anywhere near her first birthday. Go, Annie! You amaze me, Bug.

P.S. All you have to see in the last post (Annie's EF Graph) is that the latest echo, Echo #4, fell between the two lines representing the normal range. Sorry I didn't clarify that before!

Annie's EF Graph

I've looked at this about 100 times in the past couple of days!

GREAT NEWS TODAY!

It was a terrific day in Boston ... so terrific that I virtually am at a loss of words. Thank you to everyone for support, prayers, hugs, and encouragement that have gotten us through this week! (And extra special thanks to Jennie and Emma for taking on Jamie all day so we could focus on Annie while we were in Boston. Thank you so much, Jennie!) We are happy to have good news to report back to everyone.

Normal. Annie's Ejection Fraction (percentage of blood in the left ventricle that is pumped out to the body with each compression of the muscle) has entered the normal range. Her EF has been as low as 28%, but today it was measured at FIFTY-SIX PERCENT!!!! It was INCREDIBLE news since the normal range for EF at Annie's age is anywhere from approx. 50% - 70%. This is the first time any of the heart measurements they take (size of the heart, ejection fraction, shortening fraction, etc.) has fallen into the normal range. (The doctor reviewing and finalizing her echo report actually paged Dr. Smoot while we were still in with her going over the preliminary reports. He wanted to know what Dr. Smoot's secret for Annie's drastic improvement was! His input also meant that we could consider the numbers as finalized and not just preliminary.)

All of the other measurements that they took during Annie's echo also improved and continued to trend towards the normal range. Dr. Smoot was very happy all the way around and moved us officially from "cautiously optimistic" to "justifiably optimistic." Kids have trended this way in the past, only to lose some function later -- so we are by no means out of the woods. However, Dr. Smoot is encouraged by what she has seen over the past several months and told us that Annie's age is further reason to feel optimistic. She said that many dynamic things happen with children's hearts in the first year, and they can do amazing things. The fact that Annie has trended upward and gone through all of this in the first year of her life is actually a positive sign in the world of cardiomyopathy!

More great words to hear: Annie's diagnosis will no longer include "Failure to Thrive" because it no longer applies to her. (Dr. Smoot kept calling her 'Fatso' today and oohing over her big belly. Who would have ever thought we would see that day?!?) Dr. Smoot also said that medically speaking, nothing can be 'blamed' on her cardiac function at this point. Her heart function is NORMAL, so it won't be considered the basis for anything else that goes on with her (but let's just assume nothing else is going to go on with her, thank you very much).

Annie was a charmer throughout, acting as official greeter in Children's lobby by waving at each and every person who walked past us for an hour or so. She was squiggly for the echo, but they were still able to get all of the measurements they needed as well as the images necessary for the study Annie is enrolled in. She was all smiles for Dr. Smoot, as Dr. Smoot tried to chase her around the examination table to examine her. Not even the news that we needed to do a blood draw could phase us today! Annie was a little put out with having her arm restrained, but forgave everyone as soon as a sticker was proffered.

And now I am going to collapse and sleep the sleep of the VERY RELIEVED MAMA, snuggled between my two girls. Thank you again, everyone who has asked about today and offered their words of support. It is wonderful to be able to share some good news. Happy, happy weekend, everyone! And, oh what a party we are going to have on November 3!!!!

Just Some Pics

The echo is this Friday, and that is occupying all of my brain power right now. I'll post as soon as we can Friday night, since we'll more than likely get stuck in Boston traffic on the way home. Meanwhile, I just wanted to share some new photos. Oh, and we're looking forward to seeing so many of you at Annie's birthday party! Thanks so much.

Doesn't Seem Abstract ... Seems Pretty Clear

Just when you think it's ok to do some more research on-line .... Here's the abstract to an article I saw last night (I'm trying to get the full article):

"Heart transplantation is usually utilized in pediatric patients with dilated or restrictive cardiomyopathies, or in patients with hypertrophic cardiomyopathies with the hemodynamic characteristics of dilated or restrictive cardiomyopathies. Pediatric Cardiomyopathy Registry (PCMR) data suggests that transplantation is utilized in patients with idiopathic, familial, or myocarditis dilated cardiomyopathies to a greater extent than in dilated cardiomyopathies observed in malformation syndromes, inborn errors of metabolism or neuromuscular disease. Single and multicenter studies suggest that lower left ventricular ejection fraction at presentation is associated with a greater likelihood and a diagnosis of myocarditis to a decreased likelihood of heart transplantation respectively. International Society for Heart and Lung Transplantation (ISHLT) data demonstrates cardiomyopathy is making up an increasing proportion of heart transplantation in infants.

PCMR data suggest that the overall freedom from death or transplantation in patients with pediatric dilated cardiomyopathy has changed little in the past 15 years. However, survival after pediatric heart transplantation has improved over the same time period. Two single center studies have found that while survival with pediatric dilated cardiomyopathy has improved, the freedom from death or transplantation is similar to survival in pediatric dilated cardiomyopathy prior to the routine use of heart transplantation. Furthermore, these studies could find no impact from new medical therapies on transplant-free survival. These findings suggest that heart transplantation may be the most effective therapy for improved survival in pediatric dilated cardiomyopathy. " [Bold mine.]

Ugh. On the positive side, Annie was young at diagnosis. On the negative side, hers (so far) has fallen into the idiopathic (no known cause) category.

On the positive side, Annie is rolling all over the dog bed as I sit writing this, laughing and giggling like a loon. She's practically doing somersaults!

October 12 and the first echo since starting beta blockers can't come soon enough.

ENT Excitement

Annie had an appointment with a pediatric ENT at Children's this week. After a wonderful, leisurely, relaxing drive down to Boston in the rain (ha ha ha!), we met with the doc's assistant. She took Annie's history and then warned us that the doctor probably would want to put a small camera down Annie's nose to take a look. "Kids usually cry," she said. "But that's good. It opens everything up and the doctor can see things better."



"We'll see," I thought to myself. They said the same thing before the chest x-ray several months ago, and our sweet, easygoing girl didn't let out a peep. I didn't say what I was thinking out loud though, thinking that maybe I'd be wrong and this time she'd find the test objectionable. She's older now, after all. She has definitely found her voice ... like when you take something away that she wants to play with!


The doc was nice. Soft spoken and thorough. (We were told by the swallow specialist up here that he was the best. I think she was impressed that we got in to see him so quickly. Thanks, Dr. Lightdale!) As reported, he wanted to take a look at Annie's throat with a small camera inserted in her nose. They had me hold Annie on my lap, facing the doc, and holding her arms down. (Oh, boy.) Our little trooper didn't let out a peep -- not when the camera went down the first nostril and not when it went down the second! When I turned her back to me (after it was done), her eyes were all watery but that was it. What a girl! And you should have seen the smile she bestowed on the doc when he handed her a sticker! You could tell all was forgiven.


Long story short ...

1. Annie needs to have a CT scan of her nose/head at some point. There's no rush on this, but it needs to be done. It turns out that the dimple in her nose could be indicative of a problematic condition. Neither Scott nor I can remember the name, but it involves skin being in the nose as opposed to just cartilage. It can be seen as a dimple in the nose or in other ways. (The questions for which prompted me to tell the doctor that the dimple in Annie's nose didn't represent an unborn, parasitic twin!) A CT scan will need to be done at some point to rule out any complications from her ... yes, from her DIMPLE. :-) Bad news is that a CT scan will require sedation. Sigh.


2. We'll repeat the swallow study in Boston next month, mostly because we believe Annie's swallowing skills are headed in the right direction. The doc is going to give her a chance to prove it by doing another swallow study and comparing it to the one we did in July. If this study is normal, we go on our happy way. If it's not, we head to the OR (and sedation again) for the doc to take a better look at Annie's mouth/throat/vocal chord construction.


She really sounds better ... so here's hoping the swallow study next month will reflect that! Meanwhile, the countdown to the last echo of the year has begun. (Translation: I can feel the butterflies in my stomach already.) Less than one month until that day .... I hope it will contribute to a BIG celebration at her birthday party on Nov. 3!!

Cardiology Appointment 8/30

Just a quick note since I mentioned we had a cardiology appointment in the last blog entry. We saw Dr. Johnson here in NH on Thursday and he checked Annie out. Dr. Johnson hadn't seen a copy of the report from our last visit and echo in Boston, so we updated him on the latest echo numbers. He thought Annie looked great and was glad to hear we started her beta blocker and she seems to have handled the additional med relatively well. (She's spitting up again, which we learned can happen with beta blockers. The GI increased her reflux med a tiny bit to see if it helps; otherwise, we may have to try another beta blocker. We don't want to see her lose ground after the past few weeks -- when she seems to have gained weight more easily than she has in the past. Not exactly quickly, but at least more easily.) He opted to increase her doses on the other meds to keep up with her weight as well. She was 16 pounds, 14.4 ounces and 27.75 inches by their measurements.

Dr. Johnson won't see us again until December -- and they weren't even able to schedule the appointment that far out. That was a first ... a happy first! We're back to Boston on Sept. 11 to see the ENT and then on Oct. 12 to see Dr. Smoot again. As it stands now, October will be Annie's only other echo for the rest of the year.

Oh, and she's ten months old today! Can't forget that. She had a great time both days at the beach this past week. My mother's response to this photo, though, was "please don't feed my grandbaby sand!" We don't feed it to her, Mom. Quite the opposite. I can't keep her away from the stuff!

Happy holiday weekend, everyone!

Cruisin' Along

Annie is cruising like crazy! She really looks strong and great. I weighed her a couple of days ago here at home and she was just about 16 pounds, 13 ounces. Wow! We're headed for 17 pounds in what seems like the blink of an eye. (I say she was "just about" because she is pretty squiggly for the scale here at home. It's a decent baby scale, but she just doesn't sit still for a second these days.)

More good news today from Annie's physical therapist and the swallow specialist. We were meeting with them today to follow-up on everything, especially after the alarm felt by the GI a couple of weeks ago. Annie had an audience once again as she ate ... but this time, we weren't hearing any congestion or "wetness" after she ate! That's great news! I feel like I haven't heard her sound junky after she has eaten for the past several days, and it was great to have the experts say they didn't hear it today either. The physical therapist even said Annie sounded better just in the past week. She also continues to improve her spoon feeding capabilities and continues to try new foods, so it's all good news.

Tomorrow we have an office visit and EKG with the cardiologist here in NH. We hope (hope, hope, hope) to get more positive news tomorrow, even if it's not an echo. Then we're headed to the beach for the second time this week, trying to soak up the last gasps of summer up here in New England. (Meanwhile, I'm happily busy planning the big birthday party! Mike Morris will be performing for the kids (but he's great fun for the adults, too). Check him out at http://www.mikemorris.us/. )

Photos are from a summer visit with my nieces, Ella and Evie (Lori's girls). Annie took a break in the middle of our visit to the park and the girls had fun decorating our driveway. Annie certainly was enthralled with all the activity while the girls were visiting. It was a cousin-fest of girls!

Happy end of summer and start of school, everyone!

SAVE THE DATE! November 3, 2007

You (yes, you if you are reading this blog!) are invited to help us celebrate Annie's first birthday. It will not only be a celebration of Annie's first year, but also a celebration of the friends, family, nurses, doctors, co-workers, friends-of-friends and everyone else who has played a role in Annie's life and our life. It will be one big shindig to say thank you to everyone for the support, love, meals, shoulders, gifts, and encouragement that has been shared with us as we have ridden the diagnosis roller coaster with our little Bug.

So, please mark your calendars for Saturday, Novemberr 3 from 2:00 - 5:30. Details to follow. We hope to see you ALL then!

No Time to Write

I have news, but not enough time to write all about the dramatic day we had today. The bottom line is I think it all has worked out for now. We'll be visiting a pediatric ENT at Children's sometime in the next few weeks as a result of several conversations today (GI specialist, Annie's primary doctor, physical therapist, swallow specialist and cardiologist). Stay tuned ... her heart may be headed in the right direction, but we've been thrown a bit of a curve ball on her feeding issues. Again, I think it has all been worked out for right now and we're just going to keep doing what we've been doing (thickening her milk with rice cereal while trying to get her some practice with spoon feeding). It was no fun having talk of an NG- or G-tube again today, though!

In lieu of a more detailed post, here are some new pics! (Check out all the new teeth in the first photo from the beach.)

We're Here, We're Here

Ahh, summer. So much to do ... and then you get hit with the below-the-belt hit: a summer cold. It's so not fair! Why can't they stay confined to the nine months of winter when you don't mind staying inside by the fireplace? Yes, Scott and Annie have been under the weather a bit. One of the patients I can tote to the doctor and have checked out; the other not so much. (He did say that he "might" call the doctor tomorrow now that he's certain he has a sinus infection. We'll see if that really happens! And mind you, I took Annie to the doctor at his insistence, really. Our fabulous, marvelous, wonderful nurse practitioner said she doesn't mind seeing Annie anytime but thought we might be able to manage to listen to her lungs ourselves once in awhile. LOL Vicki, I'll buy a stethoscope, I promise!) Annie, unlike Scott, does seem to be on the mend without any real fuss (thankfully).

These days we are doing really well. All of the specialists (swallow, nutrition, PT, GI, etc.) have decided that Annie's food is safe for her once it has been thickened with rice or oatmeal cereal. It's working with her special bottle, so it's all good. The reflux medication seems to have helped with her spitting up, and her appetite -- even with the additional calories per ounce that the cereal adds -- seems to have increased a bit. (It seems like we teeter on the precipice of each pound marker. This month it's the 16 pound mark that we are awaiting eagerly. I weighed her this morning and she was 15 pounds, 15.5 ounces.) Meanwhile, we're working on feeding her by spoon as often as we can, so that we can get her the practice and move towards spoon feeding her everything. She is safer the thicker her food is, apparently. (The GI in Boston said things like "I don't know if it's safe to let you leave the hospital with her today" ... ugh! Luckily, things seem to have calmed down with the involvement of all the specialists.)

Oh, and the other thing we are doing these days is baby proofing. Annie is into everything! She has a determined crawl when she spots something new and exciting (still crawling army style, but is using both arms almost equally now). She loves emptying the bottom shelf of books and then eating their dust jackets. Alas, my Harry Potter books will never be collectors' items now. Other favorites include the tupperware cabinet, the bag of plastic recycling, and the ever-popular houseplant. She is pulling herself up on practically everything, including the couch, chairs, ottomans, toys, and cabinets. She has started to cruise a tiny bit and will even walk a few steps holding onto our fingers. She's so tiny that it looks comical, if you ask me. (Think Chucky.)

Jamie and Annie have begun to hang out in the living room together, which is fun. Jamie asks for me to put Annie in the living room with her first thing every morning so they can play or put on their "big kid talent show." I'm not close in age to my siblings, so the closeness of Annie and Jamie is a revelation to me. I can't help but hope that they will be close as they grow up (or at least after they both survive their teen years ....). So far, Jamie truly has been a model big sister. She loves Annie and wants to be with her "sista" ALL THE TIME. The only rough spot has been overexuberant love, especially now that Annie is on the go. I catch Jamie "helping" Annie to stand or moving her from one place to another. She thinks Annie is all set for playing and doesn't realize that she's still tiny and shouldn't be manhandled by a four-year-old! If Annie starts to cry at all, the first thing I hear if Jamie is innocent is "she just bumped her head! It wasn't me!" If Jamie is complicit ... well, silence on her end speaks volumes.

We'll be headed to see the cardiologist here in NH in a few weeks and Annie has her nine month check-up tomorrow. Other than that, we are blissfully free of doctors' appointments and happy to enjoy the summer again, now that Annie's little cold seems to be on the way out. I wanted to write and update because I had a few minutes. Thanks to everyone who wrote asking about the GI appointment!

It's a bit bittersweet, but the first photo I put on this blog was a photo of Jamie at soccer camp last year. Here's one from this year's camp ... wow, what a year. July 18 marked one year since our first fetal echo and the initial diagnosis of EFE. We wondered if we would ever even hold Annie in our arms and now she is a laughing, giggling, fabulous addition to our family. I'm grateful she's here every day, no matter what came with her. We can't imagine life without her, that's for sure. She is our happy, sweet, easygoing baby who wakes us up each morning with a big smile on her face. Now it's time to start planning that first birthday party!

(Other photos: We tried to get a good picture of Jamie & Annie with the blueberries we picked, but Annie was just too interested in grabbing those lovely blueberries!

Also, Annie hanging out at soccer camp. and Annie and Jamie playing in the living room.)

We'll Take It!

Long day in Boston and two kids need attention, so this is just a quick post to let folks know that Annie's echo today was good news. Boston got the same numbers that we saw at the last echo here in NH -- shortening fraction in the 21 - 24% range -- and they show a decent improvement in both the size of Annie's heart and the function. Overall, Dr. Smoot thought Annie looked wonderful and was pleased with everything she saw. (We hadn't seen Dr. Smoot since mid-April, so there was a huge change in Annie --teeth, crawling, squirming, etc.) The only change to her meds will be the long-awaited addition of a beta blocker.

Annie was super squirmy for the echo, but we were relieved to hear that Dr. Smoot doesn't want to sedate her in the future. Since the heart is getting better, she doesn't feel like we need to sedate her to have ultra-precise images. In general, they'll take the images they can get in between Annie's squirms. (At our last echo in Boston, they saved over 100 images throughout the echo. More than normal because Annie is in enrolled in a study surrounding echo techniques; still, they were doing the study again today and only managed to save around 40 images. Big difference in the squirmy girl, trying to eat the cord attached to the wand and kicking the heck out of the techs.)

Our GI appointment last week was another story, turning Annie's feeding upside down a bit (and adding a new med for reflux). Everything is still up in the air until we meet with the nutritionist and swallow specialist again next week, but for now we are trying to get Annie fed with milk that has been thickened with rice cereal. It can be tricky with her special bottle, so that adds an additional challenge. So far it hasn't been too bad and Annie has adjusted. And hey, it adds more calories per ounce!

Thanks everyone who kept Annie in their thoughts today! We feel like we can take a big deep breath after today ....

Swallow Study & Other News

Annie had a swallow study done yesterday at Concord Hospital. The nutritionist, physical therapist, and swallow specialist who met with us a couple of weeks ago had recommended we do the study based upon Annie's history and the way she sounds after she eats. Most of the time Annie sounds pretty junky and congested after she has eaten, so we weren't terribly surprised about the suggestion to do a swallow study.

The swallow study was very cool. (This is Annie sitting in the seat as we waited for the radiologist to come in and start the study.) It's a video x-ray, basically. They mixed barium into formula (they said they couldn't stand the thought of using Annie's breast milk and ruining it with the barium!) first, and we all watched on the video as Annie ate. Sure enough, you could see some of the formula starting to go towards her vocal cords and not straight down her esophagus. She did much better when they moved on to some thicker things -- a thickened cranberry juice and sweet potato puree that we fed to her by spoon.

We won't have the final report for awhile, but the preliminary report will be sent to the GI we are seeing in Boston this Friday. There is a decent level of concern about her eating now (yes, just as things were starting to go so well -- oy!) because of the risk that she could be aspirating milk into her vocal cords and lungs. I'm not sure what all of it means for the short term or for the long term, but I know we'll have something to talk to the GI about on Friday now!

Meanwhile, the swallow specialist has asked us to try thickening Annie's milk with pureed bananas or rice cereal or something and see how much we can get into her via spoon feeding. We'll give that a shot today or tomorrow for a 6 - 8 hour window. The question, of course, is how we can continue to maximize her caloric intake while making eating as safe as possible for her. Milk is the best thing for her, calorie-wise and nutrition-wise, so we'll have to work out if we can thicken it and spoon feed it to her efficiently. She has tried a few foods via spoon on a few different occasions, but she isn't used to spoon feeding. So, we have to see if we can get her to be more efficient with spoon feeding if we can get her more practice at it.

The only other news is that the next echo in Boston has been postponed from this Friday (when we had it scheduled to coincide with the GI appointment) until next Wednesday. Lucky us -- two trips to Boston within a few days. C'est la vie. I'm not complaining! We'll combine Friday's visit with a trip to Boston Children's Museum for Jamie ... we're still so darn grateful to be living close to Boston!

Otherwise, Annie is still doing GREAT. Her third tooth has popped through, she is able to push herself into a sitting position now (and launch herself back onto her belly to crawl wherever she wants to go), and she is still pulling herself to a standing position in the c0-sleeper (constantly) and pack-n-play. Her physical therapist said that if Annie were evaulated right now she probably wouldn't qualify for services, other than her feeding issues. She is really looking great and we are just so, so, so hopeful that her echo will reflect some positive changes, too. She was just a smidge under 15 pounds last time we weighed her, so her weight gain seems to be following her own little curve, too. (This photo is from the fireworks last night in Manchester.)

Thank you to everyone who has continued to follow Annie's story. I get nice little e-mails or notes from folks saying they continue to check the blog, and it is hard to describe how that makes me feel. It's as if we can feel everyone's support and encouragement, wrapping our little Annie in best wishes, prayers, and hopes. I knew there were some pretty amazing people in our lives, but the past year -- and it has been a year this month since Annie's in utero diagnosis of EFE -- has shown me that I really had no idea how amazing people can be! It has been a long year, to say the least. But there have been really incredible things that have come out in the past year, too. Family, friends, co-workers at BAE, co-workers at BYPC, everyone at Willow Bend, neighbors, and friends-of-friends top that list. Thank you.