Pictures

The blog, the blog, the blog!

If I had a little cartoon bubble of dialog above my head over the past weeks, it would have read "I need to update the blog!" I don't know if the end-of-winter blahs got to me, if there was not much to report, or if I was too busy being a mom to write about being a mom! Probably a combination of all three, now that I think about it. It can be challenging to find new and diverting entertainment as winter drags on and on, and reporting the intricacies of doing one more puzzle (while pulling Annie off the top of the kitchen table yet again) doesn't seem to be a fun thing to do, either.

Now, however, the snow is gone, the sun is shining, we are newly back from a soul-refreshing vacation (thank you K. and T. -- a million times over!), and I even have a bit of news to report. It's likely to be a long one, sorry.

Here's the lowdown on the medical side of things:
1. Annie saw her cardiologist here in New Hampshire last month. He continues to be pleased with how Annie is doing and feels that her appointments in Boston every three months should be sufficient now. So, instead of seeing him between our Boston visits, we won't need to check in with him again for a YEAR. I know he was saying that because Annie still is followed closely in Boston, but still. It's a step in the right direction!
2. Dr. Johnson had the report from our last visit in Boston (in January). We didn't get the final numbers while we were there (and I suspiciously and pessimistically thought that Dr. Smoot did not bring in her usual bevy of charts because the numbers had dipped a bit and she didn't want to show us), so I was interested to hear. Despite my pessimism, it turns out that Annie's Ejection Fraction was up just a smidge more -- from 56% to 58%. It's barely mentionable in terms of actual change (especially given the extreme squiggliness of the patient!), but it was good to hear and still in the "low normal" range.
3. Scheduling Annie's sedated echo/bronchoscopy in Boston has been a struggle. It's the first time I have had to really hound folks to get things done. I wasn't anxious to go through with the procedure, so I was happy to play phone tag ... until exactly April 1. It suddenly hit me that it was April, that we hadn't had an echo since January, and that we didn't have one on the books anywhere yet. Plus, Annie seemed more tired and took an ultra-early nap that day. Add several stories on the cardiomyopathy foundation's list serve lately surrounding kids who had been doing well and had taken sudden, drastic turns for the worse and that was all it took! It's nerve-wracking not getting a regular look at that heart, even barring any onset of physical symptoms. (And a too-early nap? Symptom? Doubtless not, but the mind quickly races from point A to point E, believe me.) I kicked into high gear and told the various doctors' assistants that I had just gone to Defcon Five. Long story short (honestly, this is the short version), Annie is scheduled for her procedure on April 30. Now, someone tell me how the heck we're going to keep an IV in this kid!!!
4. We have our last PT session with Kelly from Easter Seals tomorrow. (Sniff, sniff.) Annie's services will be managed by a Speech Therapist from now on. (Annie continues to lack upper body/shoulder/torso strength, but you wouldn't know it unless you were a physical therapist. She compensates for it very well, and it makes her little walk too funny. I keep trying to get it on video so I can get it up here.) We'll continue to do things on our own to help Annie develop strength (gym class at the Y, all the wonderful suggestions from Kelly, etc.), but the biggest focus of concern has shifted onto her eating skills and her speech. She is still not speaking for all intents and purposes (a vague approximation of "uh oh" and "mama" and that's about it), and she is a mess. Literally. A MESS. She is really testing my stain-removing-Supermon capabilities and my ability to keep up with laundry ... since every solid food she eats gets mashed, made gooey and disgusting, and then comes back out. LOVELY. Sometimes she even stops as she is walking along and bends over at the waist to spit it out on the ground (so fun to explain -- you'd think she was stopping to lift her leg and pee on a prize orchid by the looks on people's faces sometimes), but more often than not the masticated glob blob gets evicted unceremoniously onto her clothes. Or my clothes. It's no wonder the kid goes through several outfits each day! And no wonder I pretty much dread giving her solids ... ugh. She demands them, though! She opens the pantry, points to whatever she wants -- goldfish crackers elicit a particular groan since they turn into a cheddar-y, paste-like goo that drives me crazy -- and even closes the pantry door after I retrieve the oh-so-politely (HAH!) requested item.
5. Weight seems to be chugging along -- last time I checked she had rocketed to the 12th percentile in weight (and a whopping, unheard-of 49th percentile in height). She doesn't look quite as peanut-y for her age these days!

I think that is about all of the medical news around here. In other news ...
1. Jamie turned 5!!! I never want to see pink or purple tulle again, but it was a princess-y good time. :-) In honor of her fifth birthday, Jamie has picked up a new habit. She actually rolled her eyes at something I said the other day. She's FIVE!!! Good grief! (Is she my kid or what?!? Wait, do you hear that? It's my parents trying to stifle their gleeful chuckles ... or not stifle them, actually. Guffaw away, you two. Go ahead. You earned it!) When asked where she learned to roll her eyes, she proudly told me that she "just learned it all by myself!" She also asked me today if I was proud of the sun. "For what?" I asked. "For not making anymore snow!" she responded, obviously in heaven to be spending the entire afternoon outside. She is swimming like a fish (cannonballs into the deep end!), especially after our week in Florida with all-day every-day swimming. More than anything, she is SO excited about her first-ever sleepover and tells anyone and everyone about it. ("When my baby sister has a little operation, I get to sleepover at my best friend Hannah's house!!" I'm sure Annie takes no offense to the fact that Jamie is looking forward to her sedated echo!)
2. I am threatening to take Annie to a baby psychologist. (No, not really .... But somedays I would take her to a medium or baby whisperer or tarot card reader or phrenologist or ....) She is doing our heads in! It's a long story, but she has become a brute. Without any notice, she will grab at our faces or arms or anything she can reach; whack the dogs over the head; or head-butt Jamie. I am going to write the list serve folks and ask them about the side effects they have seen with beta blockers (it has been mentioned before, but I am REALLY interested in the details right now). We have no idea if she is agitated by the meds, or if she is frustrated at her inability to communicate effectively, or ...??? No idea. Being beaten up by your innocent-looking 17 month old makes for some really long and emotionally draining days, though. (We are doing sign language with her as we did with Jamie, and she has 20 or more signs, but she still gets very frustrated when we don't get her meaning right away.) (And let's be honest ... knowing how to sign gorilla and dinosaur and duck doesn't do a whole lot in your everyday life, unless you happen to be visiting a zoo or have a rather eclectic set of bathtub toys. And I think she finds pointing and screaming like a pint-sized inhabitant of a kung-fu movie much more effective than signing "more, please" ... aye aye aye. She's either going to be a wildly successful CEO -- although I can guess how her employees will feel about her leadership style --or a comedian.) I won't even get started on her must-do-everything-that-Jamie-does attitude, because I'm sure everyone else with more than one kid has gone through it, too, at one time or another. It's just odd to realize that there just weren't markers out and floating around all over the house when Jamie was Annie's age (or Play-Doh, or Polly Pockets, or anything else less than 100% infant-approved, pediatrician-recommended -- but hey, the kid can't swallow solids, right? Silver lining: low chance of choking, right? Right?!?). Annie is adamant (a la the aforementioned kung-fu movie rapid-fire burst of loud, unintelligible syllables) about getting her hands on any type of writing utensil. As a consequence, she ends most days looking like some sort of Rohrschach test by way of Picasso. (Which will win? My expanding stain-fighting skills ... or my growing desire to overlook all manner of mess on a poor, neglected youngest child? Hmmmm .....)

Gee, can you tell the little one has decided that she had to step things up a notch? We laugh regularly about our early assessment of Annie's temperament ("mellow, easygoing -- just like her dad!!"). I guess she just needed a little more blood pumping around her body so she could let her true colors fly. And speaking of flying ... I have spent the past few months retrieving Annie from the top of the island or the top of the kitchen table regularly throughout the day. Today, as we started our spring clean-up in the backyard and spent the afternoon playing, I realized a whole new world of possibilities has opened up: namely, the swingset/play structure. Good times ahead. Maybe it's my heart that will be having problems soon, as I imagine Annie tumbling hourly from the top of the slide!

Phew, quite enough! Sorry for the extra, extra long post. I'll not make this any longer just now, but I'll post some photos separately. Thanks for checking in on us, especially since I haven't been good about keeping the news flowing lately. Like spring, I feel ready to start anew now that the long, snowy winter is behind us. :-) Hope everyone is enjoying the weather -- I hear spring sprung in the rest of the country weeks and weeks ago.

Hannah!

If you are in the New Hampshire area and have access to WMUR Channel 12, our very own sweet, smart, artistic, fabulous Hannah is going to be featured on the 5 o'clock news either tomorrow (Wed.) or Thursday. Hannah and Jamie have been best friends for just about their entire lives, and I consider myself lucky a million times over to have Hannah's mom, Nikki, in my life. Nikki has been incredible for me through our journey with Annie ... and in the middle of it all, her amazing Hannah was diagnosed with Juvenile Rheumatoid Arthritis. Hannah and her family now visit Children's in Boston regularly, and we have even managed to pass through on the same day at one point.

We are so, so hopeful -- along with everyone who loves Hannah -- that the newest medication is making the difference in turning Hannah's disease around. It has been a tough road for all of them, ESPECIALLY since the latest med has to be injected weekly by Nikki. Nikki's strength and fortitude as a mother amaze me constantly! (Nikki and I comment often upon the craziness that life has dealt us -- that we have become such incredible friends and we both have kids who are ... giving us a run for our money, shall we say?) Nikki often prefaces some news about Hannah with some nonsense about it "not being a heart problem" or "not what you have faced with Annie." This experience has taught me another thing -- and that is that there is no degree to worrying about your children. Worry is worry when it comes to our little guys, and there is no sliding scale. We deal with what we are dealt; but, more importantly, we are all stronger than we give ourselves credit for being.

We will be joining the Thompson family in supporting Hannah and arthritis research at the Arthritis Walk on May 10 (https://www.kintera.org/faf/donorReg/donorPledge.asp?ievent=260885&lis=1&kntae260885=89AC9D7042B64A49B61AD7055A5DB392&supId=204154983). You can read Hannah's story on their blog at http://www.hannahhugs.blogspot.com/. Hannah's dad, Michael, heard from the arthritis folks that they were having a hard time getting the message out about arthritis and the walk. He promptly contacted the local news, and the news story that will run this week is a result of his efforts. (Go, Michael! And being the marketing person that I am, I have to help in whatever small way I can!) So Tivo, DVR, set the VCR, whatever, but catch beautiful Hannah and her amazing family sharing their story on the news!

Happy Heart Day!

I don't have much new to report, but it is a "no news is good news" kind-of thing. I write many blogs in my head about this or that, but I don't seem to sit down and get them written in reality.

Annie seems to be doing well as she franken-walks around with her toddler belly sticking out. (We weighed her tonight and she is 20.4 pounds -- that's great!) Her other issues have come to the forefront right now, as she is not talking and still not swallowing solids. Her Physical Therapist is looking to transition her from PT to speech therapy sometime early spring -- a move that we expected. The speech therapist will work with swallowing/feeding issues as well, and Annie seems to be doing really well strength-wise. (We joke around here about Annie being "our hypotonic daughter" as she crawls out of her seat and WALKS across our island or dining room table, climbs ladders, or carries the step stool with her around the house so she can see and do anything she wants! The hypotonia diagnosis seems to be a distant memory, but the PT does still see some weakness in her arms and upper body. If you saw Annie in swim or gym class, though, you would never guess she has any strength issues! Strong-mindedness can make up for a lot, I think!)

As for the not talking part ... well, I don't know what to think about that. She has missed the dreaded developmental milestone of 3-5 words by 15 months. The dentist that saw Annie for the first time last week didn't see any structural reason Annie can't talk, so the ball is firmly in the speech therapist's court. Meanwhile, Annie has NO PROBLEM getting her message across. Believe me! Even the dogs know what she means sometimes (as she babbles loudly, holding a pretzel or cheerio in one hand, and clearly telling the dogs to leave her alone). She also has told me firmly to let her do things by herself, to help her up into a chair, give her my swim goggles, and many other things; all without uttering an inteligible word. Just a typical second child? We don't know at this point, but we're working on figuring it out.

Annie's sedated echo/bronchoscopy procedure has not been scheduled yet, and March is becoming crowded. I won't be sad if we have to push it out until April, but I know I can't put it off forever .... Lately there has been some talk on the children's cardiomyopathy list serve about the effectiveness of beta blockers in children. I hate seeing research like this come out. It's just so frustrating! I want to think that all of the work to get meds into Annie is paying off in increased heart function, but I think the reality is that nobody knows with this disease. As always, most people comment on "how great" Annie looks and that "you'd never know" she was sick at all. That's another common theme with families on the list serve, because these kids usually DON'T have any external symptoms of their disease. It's hard -- in many, many ways -- to keep telling people that we know she looks fine, but she could be on the transplant list after her next echo or hospitalized at any moment or ...???? (Truth be told, it's hard for me to remind myself of that, too, as she laughs and plays with her big sister. I have seen so many stories about cardiomyopathy, though, that reality is never far from my mind.)

This sounds like I am being or feeling negative and that is not the case at all. So let me reiterate that Annie seems to be doing really well right now! She is eating, gaining, taking her meds happily, and just a crazy, happy presence in our lives. Annie is definitely one-of-a-kind, in many ways! I have to admit that I have been in a bit of a funk lately, as we just passed the one year anniversary of Annie's cardiomyopathy diagnosis (Feb. 9). Last Valentine's Day, we were on the cardiac floor at Children's and our lives were upside-down. Seeing these pictures makes me happy, though, and helps me focus on TODAY. Today, we are doing well and managing Annie's diagnosis.

Last year versus this year:

Just a Photo or Two

After being weighed (19 lbs, 9 oz., thank you very much!), waiting for the echo at Children's. This was the first time Annie was given a hospital gown to wear, and she was not a fan. Within 30 seconds of this photo, she was running around in just her diaper, socks, and red shoes.

Trying to get a photo for Aunt Beth in an outfit she (and Christian!) gave to Jamie four years ago. I just love this Cover Girl shot! (And I love all of the outfits from Aunt Beth -- either to my girls or to my sister Lori's girls that have eventually made it to us, too -- they remind me of her every time I see them. That's the best part about gifts, if you ask me.

And this is what happens when I push my luck trying to get aforementioned photos .... Notice Britain's long-suffering look as well. (Tasse, my 13 year old girl, is deaf now ... happily, I think.) Oh, and Scott just looked over and asked about the socks. Annie has a sock fetish. These are some of Jamie's Little Mermaid socks on her feet -- ON TOP of Annie's own socks AND her Robeez (shoes, sort-of). It looks like she has gigantic feet, but she really doesn't. So far, only Jamie inherited that ... from me, sadly. (Ah, genetics.)

Night!

Quick Boston Update

We had a very squirmy-moving-target echo in Boston yesterday. The short version is that there hasn't been much change. The echo numbers weren't finalized while we were there, so Dr. Smoot wasn't able to give us actual figures. In general, she said that the condition is telling us to keep paying attention and keep doing what we're doing. So, Annie's meds have been increased to keep up with her weight and we'll just keep everything else the same.

Since Annie's heart didn't show another marked improvement (and she was so darn squirmy), Dr. Smoot would like to get a detailed echo. That means sedating her, so the question about the bronchoscopy became moot. We'll be looking at doing the sedated echo with the bronchoscopy procedure together sometime in March. The CT/MRI business regarding Annie's dimple is still on the back burner for now.

Obviously, we were disappointed that we didn't see another great leap in Annie's numbers but we are grateful -- honestly grateful -- that she is holding steady where she is. It could be a lot, lot worse.

Sorry for not updating sooner! It's been one of those where-did-the-week-go kinda weeks.

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!