Friday, June 27, 2014

A Good Hospital Day

We spend a lot of time at Boston Children's Hospital.  We just do.  I'm not complaining about it, I'm just stating it as a fact.  There are lots of people who spend WAY more time than we do, but there are also plenty who spend less.

Hospital days are long, stressful, tedious, and emotional for us.  I stress about pre-transfusion hemoglobin, how many sticks it's going to take to get the girls' lines in, hives, ferritin trends, palpable spleens, antibody screens, getting a urine sample, labs, labs, and more labs...I just stress myself into a stressed tizzy.  (Just ask my mother who I always call, nearly hysterical over something.)  Hospital days are often 9+ hour days for us...starting in morning traffic and leaving in rush hour and Fenway crowds.  By the time we get home, I am totally wasted.

I try as hard as I can to make hospital days easier and more pleasant for the girls.  I pack lots of stuff to keep them happy, order their favorite foods, get them plenty of snacks, keep the DVD player loaded with a favorite video.  I hold them and cuddle them and rub them and love them through the whole thing.  I try to wait on the girls the best I can, only leaving their side to use the bathroom.

This is where our BCH village comes in.  We are so blessed to have an amazing child life specialist who thinks of every little thing you could ever imagine to make things easier for the girls-and for me.  We have a great nursing staff, including our favorite nurse who--somehow--has the magic touch getting Emmie's IV in with one stick.  We have an NP who comes by to check on us, and an AMAZING hematologist who (as incredibly busy as he is) happily comes down whenever I ask for any reason-big or small.  The volunteers, clinical assistants, admins, and even facilities staff all know us well and make us feel loved.

A few weeks ago, I walked into our infusion unit nearly hysterical.  We were super late, both of the girls were covered in vomit--one from car sickness and one from a stomach virus.  Everyone was upset and crying and frazzled and miserable.  I just stood there, threw up my hands, and said to pretty much everyone at the nurse's station, "I NEED HELP.  I AM OVERWHELMED."  Everyone jumped up grabbing throw-up bins and towels and johnny's and ice water.  I was nearly in tears worrying about dehydration from the vomiting creating impossible-to-find-veins and before I knew it there was a cranberry juice in my hand and everyone was reassuring me to calm down, that it would all be OK.  And it was OK.  Not an easy day by any stretch, but it was OK.

That was a crappy day.  Today was a good day.  No drama.  Rosie was one stick and getting her blood and eating her 4 turkey sausages in no time.  A new volunteer came by so I could take a quick breather and a walk for some hospital coffee.  The clowns visited after lunch and we all laughed and laughed.  The girls got clown noses and had the whole unit in stitches.  The art therapist stopped by and before I knew it, an entire hour had passed with the girls working on art projects and having a great time.  She even said that the girls got the "prize" for the best attention span of anyone she'd visited in a week.  Then we were done.  The girls gave hugs and high-fives and grabbed plenty of snacks and stickers for the road and we were all smiling.  As we were leaving, I said to our dear child life specialist, "You know...this was a GOOD day at the hospital.  A really, really good day."

People are always commenting on how busy we are doing fun things with the girls every minute we can.  And, we do, because we want them to remember their childhood as lots of fun stuff, not lots of medical stuff.  But, if we have to spend a 90 degree summer day at the hospital instead of at the beach, we are lucky to spend it at Boston Children's with skilled nurses, a caring staff, and circus clowns!

Hospital Day....

Emmie's New Blood Day.  Rosie is pretty psyched she's just along
for the ride.

Emmie's pumped up to be getting pumped up.  Nothing like squeezing
all of us together in a hospital gurney!
Beach Day....

Having a blast in Rockport, MA.

Emmie said, "Mama, we're REALLY beach girls!"

A late, late afternoon snack before the long ride home.  We always
seem to be the last to leave the beach!


Hospital Day...

Rosie's New Blood Day.  Emmie's turn to clown around!

After the clowns dropped by, they left clown noses for the girls.
Unfortunately, the girls' noses are too tiny for them to stay on!

An awesome visit from the art therapist.  The girls had so much fun
passing the time making arts and crafts.

We were having so much fun, it was like we forgot where we were!

Next Beach Day coming soon...you get the idea!

Saturday, May 10, 2014

Mama's Day

"Mama, can you make one of those pretty bows like the ones for our hair?  And can you make a box?  Do you make boxes?  And can you help me wrap the box?  I want to put one of my toys in it.  Then I'll hide it.  For Mother's Day!"

A friend asked me today what I'd be doing for Mother's Day this year.  Apparently, per my 4-year-old's request, I'll be making bows and boxes and wrapping...So I can get a recycled toy from my daughters.  How green of them.

Later on, another friend implored me to enjoy these times, because, in her words, "...when they are sixteen they'll act like they don't even KNOW you.  They won't even CARE that it's Mother's Day.  Trust me!"  Wow.  So I'm going from wrapping my own used puzzle with half the pieces missing to being completely ignored.  That's Mother's Day, huh?

No.

Mother's Day to me is a special day because it makes me slow down and think about what it means to be a mother.  Yes, I'll honestly admit that I hope to get a few extra minutes of sleep, a scribbly handmade card, and an extra-delicious cappuccino.  But my day isn't going to be spent drinking mimosas or at a spa or wearing heels at some fancy restaurant.  It's going to be spent with the two little beauties who made me a Mama.  Thinking about the amazing love we have between us, and how it came to be.  Questioning how I could ever be so fortunate to have the most rewarding job in the world...a mother.  Emmie and Rosie's mother.  I'll also be thinking about Emmie's two mothers in China, and Rosie's one, and how their birthmothers made the ultimate sacrifice.  I'll be wondering how in the world I'd ever be able to navigate this minefield that is motherhood without my own precious mother to help me every step of the way.  Remembering my Nana who raised two children, and opened her loving heart and her home to countless foster children.  Thinking about my other Nana who raised five children, and wondering how she did it.

Truthfully, no matter how we celebrate the day tomorrow, I'm pretty certain my present came tonight.  I was knocked down with some stomach bug out of the blue, and the heartfelt tenderness and love my girls showed me was the sweetest thing to witness.  They brought all of their favorite dolls to lay on the couch with me.  They made me about 10 cards.  They covered me with stickers.  They "read" stories to me.  They decorated the room I was relaxing in.  They kissed my belly over and over to make it feel better.  They even did a musical performance for me.  (Goodness, it's just a stomach bug that will hopefully be over in 24 hours!)  Watching them work as a team and snap to action as soon as I said I felt crummy, hatching plans about how to make me feel better and what surprises to bring me made my heart nearly explode.  This is what being a mother is about.  This is my reward and my present for taking care of these girls the very best I can day in and day out.  And no present wrapped up in a box, no champagne brunch, is better than this.

Getting serenaded by the dollies.

A performance in plain clothes just isn't a performance.  So they
had a costume change and really belted out "Mary Had a Little Lamb."  The
extended remix version.

"We're taking care of you, " Rosie said over and over.  Jeez, gals,
it's just a stomach bug that will be gone tomorrow!

So tomorrow we'll enjoy some time as a family, probably doing some of the girls' favorite things as well as mine.  I know I'll get all teary-eyed watching their excitement as I help the girls wrap and then unwrap my "gift."  I'll look at this beautiful family that my husband and I worked so hard to make and feel like the luckiest woman alive.  I'll tell my own mom for the zillionth time how I finally get it.  I really, truly understand and appreciate everything she did for us.  All of the days spent caring for us.  All of the nights spent worrying about us.  And, most importantly, the complete joy we have brought to her life.  Because there is nothing at all in the world like being a mother.  Nothing even comes close.

Moments of sweet perfection...


It doesn't get better than this.

Tuesday, May 6, 2014

One Year with our Rosie

Every time someone meets Rosie for the first time, they say, "She's TINY!"  Which, of course, is true.  But for a teeny, tiny little girl, she has a big personality and an even bigger heart.  She lights up any room with pure joy.

One year ago today, we met our little spitfire in a crowded, loud room in Guangzhou, China.  She was sick and timid, wearing a cute pink sweatsuit and slippers.  When I picked up my baby for the first time, I couldn't believe how small she was.  I felt like her little body might slip through my arms.

In those first few days in China, there was probably nothing that Rosie wanted more than to slip through my arms.  She wasn't super happy with any of us, but she definitely liked me the least.  It broke my Mama heart to be shunned by the little person I had yearned for and dreamed about for months.  Whenever Baba left the room or was out of her sight, she would look at me with sad eyes and say something over and over that we later found out was "Baba come back."  Even though I knew in my brain that she didn't know any of us at all, I still felt hurt and like a failure.  I'd be lying if I said I didn't take it personally.

Rosie threw some tantrums that were so wild that we feared our little sweetheart would hurt herself.  She was confused, sad, and unable to communicate with us.  As a result, she would throw herself on the floor and do what we nicknamed the "crab" where she would crawl on her back wildly to get away from us. During these tantrums, she seemed to have no regard for her own safety, nearly "crab crawling" into the walls.  We ended up strategically placing pillows all over the floor in preparation for these tantrums.  She liked none of us during these times.

Unlike our first days with Emmie, there were certainly moments of laughter and smiles.  Rosie ate and played and was very responsive to us.  Her little personality as a jokester began shining through very early on.  Although Emmie was struggling with having to share her Mama, Rosie benefited greatly from Emmie's presence.  This was a time when Emmie's amazing heart really shined through.

During the past year, we have watched Rosie blossom into an amazing little girl with a huge personality.  Her language has come along much quicker than we imagined it would.  She is as smart as a whip, and doesn't miss a trick.  She loves to dance and sing.  Her favorite outfit is jeans and a monkey shirt.  The little girl who was terrified of grass and sand and the ocean loves feeling it between her toes and splashing in the waves.  She has a very adventurous spirit.  She is goofy and funny and silly.  She is definitely the sidekick to Emmie.  The color commentator.  The Ed McMahon to Emmie's Johnny Carson.  She loves to make people laugh, play jokes, and act goofy.  She has a contagious laugh, and the little dimple under her right eye is a perfect sign of her happiness.

When we met Rosie, she didn't care about hugs or kissing boo boos or any of that.  She'd fall, get up, brush herself off, and move on.  We had to teach her that kind of love and tenderness.  Now, Rosie can show us that she has an amazingly tender heart.  If someone coughs--anyone--she asks, "Are you OK?"  She loves kissing boo boos, and will pat you on the back, saying, "It's OK, honey" if you are sad.  Today she walked by me in the kitchen and said, "Ooh! I forgot something!" and wrapped her little body around my leg and kissed me.  "There you go!  Perfect!" she exclaimed.

One year after we struggled in China, Rosie is a Mama's girl.  Well, and a Baba's girl.  And an Emmie's girl.  She loves us fiercely, and tells us all the time...right out of the blue.  She gives kisses and hugs freely and often.  For a little person, her hugs are some of the tightest, most heartfelt hugs I have ever experienced.  When I kiss her goodnight, she often pulls my head to her chest and asks me to lay it there.  She takes her tiny little hand and strokes my face with a tenderness I only dreamed I'd experience from her.  "You my Mama," she says over and over, with a look of perfect contentment on her face.

This little girl has come such a long way in just a year.  She's had an awful lot of medical "stuff" to contend with, in addition to basically learning a new life, and she's making it look easy.  And I, of course, know it is not easy.  She is strong and brave and someone we could all look up to.  And she's my daughter.  Our daughter.  Emmie's sister.  Grafted into our lives, into our family, into every fiber of my being.  Just when I think my life can't get any better, that my heart can't get any fuller, it does.

Thank you, Rosie, for letting us love you, for accepting us--with all of our imperfections--as your family.  We are blessed and honored and forever grateful.  Happy Rosie Day!

Those first few moments....

Our first glimpse of tiny Rosie.

Not too sure about Emmie yet.

An hour after we met.
Rosie looks (understandably) dazed.  My hand is on Emmie's head
because she was devastated I was holding her new little sister.
One year later....

Just chillin' it in a patch of dandelions.  She even makes
weeds look pretty.


From arch rivals to best friends.


Happy.

Our loves.



What better way to celebrate Rosie Day than with Chinese food.
Rosie (the kid who never eats) ate her weight in fried rice and wings.

Eating Chinese food, just like we did one year ago today half way
around the world...

...except here you end Chinese food meals with Jell-O.  Didn't
see a lot of that in China


Monday, April 28, 2014

Thyroglossal Duct Cyst - Sistrunk Procedure

As promised, this is a detailed post about my experience with a thyroglossal duct cyst and the Sistrunk procedure.  If you do not like medical stuff or gory pics, stop reading.  This is for people who are going through the same thing and want to hear about someone else's real life experience.

My thyroglossal duct cyst (TGDC) first reared it's ugly (and, yes, I mean ugly) head in August.  It came on after a upper respiratory infection as a sore, firm lump around the midline of my neck, above my thyroid.  I went to my PCP who said she suspected TGDC, but referred me to an ENT.  The ENT also said he suspected TGDC and sent me for an ultrasound.  He also put me on 10 days of antibiotics, in the hopes that it would clear things up.  He made it clear that if it was a TGDC that surgery would not be indicated unless I wanted it, or the cyst got repeatedly infected.

When we got back the results of the ultrasound we were shocked and scared.  The ultrasound showed that it was in fact a solid, vascular mass and NOT a cyst.  Obviously, this is NOT what we wanted to hear.  Since it showed up solid, the ENT sent me for an MRI for more information.  When I asked him what it could be, he said he didn't want to guess and he told me, "anything I can say will only make it worse."  The wait for that MRI felt like an eternity, and the wait for the results was even worse.  It ended up that the results of the MRI did show that it was a remnant of a TGDC.  We were thrilled and since the cyst had disappeared, we decided to wait and see if it got infected again before doing anything.

Sure enough, a couple of months later, it came back with a vengeance.  I made an appointment with the surgeon who is also the chief of ORL and the minute he saw it he said it had to go.  It had been infected several times, it looked bad, and my lack of spleen had him concerned.  He also noted that the tract went up very far to the base of my tongue.  He put me on more antibiotics and said it needed to come out once the infection was a bit under control, but ASAP.  Not what I wanted to hear a couple of weeks before Christmas, but at least it didn't give me too much time to worry about the surgery.

Because of my complicated medical history with thalassemia, I had a detailed pre-op visit, despite what the surgeon's admin wanted--she insisted because I'm "young" and "healthy" that I could just do it over the phone.  I knew this didn't sound right.  I contacted my hematologist who was really irritated by this and concurred that I really needed the full workup.  I had a transfusion a few days prior to the surgery to--as my nurse put it--get me all "shined up" and in tip-top shape for the surgery.  I also had a CT scan as the surgeon felt this would give a better picture of the cyst and duct than the MRI could.  It was really no big deal, and I didn't feel any weird sensations or have any metallic taste in my mouth.

The night before the surgery I had to stop eating at midnight, not that this was difficult as I was a total nervous wreck.  Fortunately my surgery was scheduled for the morning so I didn't have too much time to work myself into a frenzy.  I went up to the OR floor by myself and got changed, and then my husband was able to come up and sit with me.  I talked to a zillion people and answered many of the same questions over and over, which was fine with me.  The anesthesiologist was very nice.  He placed my line, using lidocaine first, although I told him that I was really a pro at getting IVs.  He also told me that they were going to intubate me through my nose to keep my oral cavity open because the surgeon wanted to do some exploration through my mouth, and might possibly have to do some of the procedure that way as well.  I had to squirt something akin to Afrin in my nose several times to help with this and to also help prevent trauma to my nose.  The surgeon came in to chat with me.  He was a lot more chipper than I was.  That's when I got really scared and started to cry.  Pretty soon after that I said goodbye to my husband and was wheeled into the OR.  The anesthesiologist put a mask on me and said it was oxygen to really fill my lungs, and after that I remember nothing else.

The next thing I knew I was being wheeled down the hall and the surgeon was joking about it being like a spa day and I got to relax.  I tried to talk and weakly asked him if he painted my toes and we laughed.  I was in and out of sleep in post-op.  At first they asked me to rate my pain and I was like, "what pain!?" but not long after that my neck and throat felt really uncomfortable so I was given some pain medicine in my IV.  I slept on and off.  I kept asking if I could go to the bathroom but they didn't want me to get up yet and I refused a bedpan so I waited maybe an hour and they let me go.  In the bathroom I saw a pretty huge bandage and the drain coming out of one end.  I was a little surprised at how big it was.

I stayed overnight in the hospital's 24-hour observation unit.  The bedspaces are just separated by curtains but it ended up being a nice quiet unit, at least on the night I was there.  My husband and sister came to visit so that was a nice distraction, but the pain was building so I was keeping on a 4 hour schedule of liquid oxycodone.  I ordered just about every soft food on the menu and was sure I'd at least be able to eat chicken soup, but even the soft noodles wouldn't go down.  Really, nothing would.  I generally felt OK while my visitors were there, but as soon as they left I started to feel absolutely awful.  The pain in my throat was terrible and I kept complaining to the nurse and was literally counting the minutes for my oxycodone.  She said that they would not give pain medicine on a schedule, that the patient needed to be awake and asking for it, but I knew there would be no problem because there was no way I'd be able to sleep.  I kept asking her if there was anything at all they had that would make my throat feel better and it took her 3 hours for it to dawn on her to offer me popsicles!  They ended up being a lifesaver that entire first night.

The next morning the resident came in before 5am to take a look at me and he said he'd be back to remove the drain.  The drain was grossing me out, but the thought of getting it pulled out was even grosser.  The drain filled up with a quick 30cc right after the surgery, but after that it slowed down quite a bit and probably only did another 30cc through the entire night and morning.  Again I ordered lots of soft food and liquids but the only thing I could get down was the Dunkin' Donuts coffee my sister brought me.  The resident came back to remove the drain.  He clipped one stitch and then just pulled this long tube out of my neck.  It went across the entire front of my neck and I could feel it being pulled out.  It was gross, but it did not hurt.  I was released to go home before noon.  The hole where the drain had been was simply covered with a piece of gauze, and the incision was covered with steri-strips that I was told would fall off in a few days (they did not).

I came home with the usual, "I'll be able to go back to normal!  I don't need any pain meds!" feeling that people get when they are sprung free from the hospital.  By the time I got home, I was totally exhausted and my neck and throat were killing me.  Since I wasn't really able to swallow, we got all of my meds in liquid form:  Oxycodone, Tylenol, and antibiotics.  One tasted grosser than the next, so my stomach felt quite queasy.  Swallowing really hurt, and I often had the sensation that I was choking on my saliva.  My mom made chicken soup and I was able to drink the broth.  I know that some people say they are eating sandwiches the day of their surgery, but there is no way in the world I would have been able to do that.  That night I had to sleep sitting up both because of the pain in my neck, and because of the choking sensation I kept feeling.  If I had not taken the Oxycodone, there would have been no way I could sleep.  I actually slept upright for over a week after the procedure.

The next few days were much of the same.  Laying on the couch, taking medicine, and only eating soft foods and liquids.  I was able to wean myself off the Oxycodone after just 2 days, but continued to use the Tylenol.  It continued to hurt to swallow for about a week.  For the first few days I was not able to eat anything very solid or dry.  The first time I tried to eat chicken I choked.  By the end of the week I was able to eat small pieces of solid food if they were moist.  I would say in two weeks time I was eating normally.  I did not drive or do any heavy lifting (ie, pick up my kids) for a week.

At one week I had a post-op appointment.  The surgeon took off the steri-strips.  There were no stitches to remove as they were all internal and dissolvable.  He said that everything looked good, and as expected.  He discussed the pathology report with me which proved that the cyst was totally benign.  He also gave me the operative report.  He noted that since a fair amount of underlying tissue needed to be removed from my neck in order to remove the entire cyst, that he had to implant some AlloDerm to make it look right again.  This explained the rectangular firm thing I could feel in the front of my neck.  (It got softer over time.)  I saw him again in another month and he thought that things looked good.  He noted that my neck was still swollen, but that it was to be expected.  He said that if the scar did not look better at our 6-month visit that he could do something in the office to "correct" it.  I'm not sure what that means.

As far as my neck is concerned, it started off very swollen.  The swelling was from my chin to under the scar.  For a long time I thought I looked like a linebacker and I was worried I would always have a fat neck.  It actually took months, but it went completely back to normal.  My tongue was almost completely numb from something that happened during the surgery (maybe I bit my own tongue, maybe from a tool they put in my mouth, maybe from a temporary stitch they put in my tongue to pull it out to look at the back of it-I'm not going to ever know).  At first, it even effected the way I ate and spoke.  I was terrified that it would not go back to normal, but it gradually did over about 2 months.  A large portion of the front of my neck from my chin to below the incision was numb, also.  The surgeon said it was from all of the tiny nerves being torn when my skin was pulled back to do the surgery.  He said that it would take several months to go back to normal.  At almost five months, the sensation in the front of my neck is improving, but far from normal.  The scar is a straight-ish line, longer than I expected it would be at about 7cm.  It is on one of the folds in my neck but I personally think that it is still quite visible.  It has flattened out and gotten less red over time, but at almost five months still doesn't look great.  I was told it can take a year for a scar of this nature to look good and be barely noticeable.

So, here's what I learned from this yucky experience:
1.  If you have an ultrasound and it says you have some awful horrible solid mass in your body, it might not be.  Get more imaging done.
2.  If you don't have a spleen or you are super anemic or if you have some other issue that puts you at higher risk than a "typical" person, go ahead and get the full-on pre-op done.  The extra visit is inconvenient, but probably worth it.
3.  If you are having the Sistrunk procedure, make sure to get all your meds in liquid form.  I don't care what anyone says, swallowing probably isn't going to be very easy or pleasant at first.
4.  If the nurse is too dumb to offer you popsicles, ask for them.  They are SO soothing, and if you are like me, probably the only thing you'll be able to eat that first day.
5.  Hopefully you have a great family like mine and they'll stock you up with lots of soft, yummy foods for when you feel like eating.
6.  Stay on the pain meds for the first day or two and don't fall behind.
7.  If you have that gagging feeling after the surgery, sleep sitting up or at least partially upright.
8.  Be patient.  (I'm still in this stage.)  I'm sure it depends, but if you have a large incision like mine, it's probably going to look crappy to you for awhile.  It does start to get better.  And, if you lost sensation anywhere (tongue, neck) be patient about that, too.  Zillions of nerves regenerating take a long time.
9.  If you hate the way your neck looks before or after the surgery, buy yourself some nice scarves.  They are very fashionable, anyways.
10.  You might be terrified.  I was terrified.  And, in my opinion, that is OK.  They are cutting your neck open and rooting around near some pretty important stuff.  So, it's OK to be scared.  Before you know it, it will be behind you and you'll wonder how you walked around with that yucky looking thing on your neck.

So, since a picture says a thousand words...here are some pictures.  Again, if you are not here to read about the Sistrunk procedure or thyroglossal duct cyst, stop looking now.  I'm being very brave sharing these as they aren't exactly, ahem, flattering...

This is the end of October 2013.  The bump had gone away and then came back
with a vengeance.  The next week I saw the surgeon and he said it had to come right out.


This is when it was at it's grossest.  This is two days before my surgery and I was
very worried because it looked so angry.

One day before surgery.  It looked like it was going to pop out of my neck.  For this reason,
the surgeon had to cut away part of the skin that the cyst was attached to on the outside.

This is a few hours post-op.  You can see at this time, it wasn't really even
swollen yet.  If you look carefully, you can see the drain on the left side
of my neck.  I'm smiling because I was drugged and laughing with visitors...the worst
was yet to come.  The pain that first night was really bad.

Day after the surgery.  You can see the swelling.  It got worse before
it got better.

Just over one week post-op after seeing the surgeon and having the
steri-strips removed.  I thought it looked horrible.

Just over one month post-op.  My neck was still definitely swollen.

Almost 5 months post-op.  Much less swollen and flatter, but I think the scar still
looks pretty noticeable and crummy.

Monday, April 21, 2014

Ferriscan...Redux

Well, this is where the rubber meets the road.  Emmie's first Ferriscan was scary because, well, I find general anesthesia to be scary and having your child "go under" is a million times scary.  But we sort of knew that the results would be less than stellar because she hadn't started chelation yet.  And, as promised, the results were in fact less than stellar.  (For you Ferriscan experts out there, her cardiac T2* was 21 and her Ferriscan liver iron was 25.  For you non-experts, that's pretty low for cardiac T2*, and way too high for liver iron.)

This year was scary times 10.  I was still terrified about the general anesthesia even though I knew better what to expect, but we were also really anxious about the results.  At the time of her 2nd Ferriscan, Emmie had been on Exjade for just about 12 months.  As you might have read here, we fought about it, cried about it, gagged over it, and struggled a LOT to find a way that was palatable for her to take the medicine.  In the end, Emmie took every dose of Exjade in yogurt or drinkable yogurt.  As I stated in my post about Exjade, I felt pretty good about this as it was recommended by our hematologist (one of the leaders in the field) and because there is one study and lots of anecdotal evidence to suggest that Exjade works in food despite the fact that it is not on the label.  However, now and again I have heard other parents saying that their doc doesn't recommend using Exjade in food, or that Exjade doesn't work well in dairy products, and so on.  Still, my stance has been that having Emmie take the Exjade in food seems preferable to her not taking it at all.  And, if it's not working, there's always Desferal....

So, the Ferriscan.  Overall, our experience was better.  The hospital allowed us to do pre-op for both girls on the same day, which was helpful.  Our experience in pre-op was NOTHING like our experience last year.  Last year there were literally patients everywhere, even flowing out into the hallways waiting to be seen. This year we were the only patients there, and we were in and out in an hour.  It would have been less if I didn't make a habit of asking the same questions over and over.

We got the call the day before the scan that Emmie would be one of the first scans of the day, arriving at 7:15 am.  We were so relieved to hear this, as it's less time for her to be fasting, and it's less time for me to be hysterical waiting.  We were taken back pretty quickly into what I'd like to call the "Scary Anesthesia Room."  This time, I wasn't the only one who was scared.  (You might remember last year Emmie was mesmerized by the TV, not noticing anything else in the room.)  Emmie's eyes were as big as pie plates as she watched the nurse fill syringe after syringe, laying them all out on the table in full view.  It seemed everywhere she looked there were more syringes or tubes or face masks or you name it.  It was so obvious to me how being just one year older made it so much scarier for her.  She knew something "bad" was coming, and there was NO distracting her.  Well, except for the Versed.  So we very quickly had the Versed/mask discussion with the anesthesiology team and we all agreed that since everything went well last time that we'd just repeat that.  (Of course we got the disclaimer that from one time to the next children can have different reactions to the same drugs, so we weren't totally in the clear.)  This time I let the nurse give Emmie the gross-tasting Versed in the syringe and she cried and gagged a bit, but then it was almost as if the drug started to take effect immediately.  I'd say in 5 minutes she seemed six sheets to the wind, slurring her speech, cuddling me, and calmer.  We laid her on the bed and put Curious George on and she kept asking if the people were "real" or in the TV.  At 8:20 it was time for us to leave the room, and even though Emmie was pretty well drugged, she cried such a sad cry that really broke my heart because last year she did not cry when I left.  We took comfort in knowing that the Versed not only relaxes you, but it also gives you amnesia, so she has no memory of us leaving her.  I could see through a crack in the window as we left that the nurse had the mask on her face and that she seemed peaceful.

As promised, we got a call at 9:15 saying that everything was going well and that she had about 20 minutes left.  They told me that she was given "laughing gas" at the beginning, and then had an IV inserted in her right hand, and then she received the general anesthetic.  They said she was comfortable and doing great, and that they were getting great pictures.

At 9:40 we were called back to recovery.  Emmie was extubated in the procedure room and woke up enough to breathe on her own, and then went back to sleep, likely from the remaining effects of the Versed in her system.  She seemed peaceful and after about 15 minutes she woke up normally and gracefully.  She was happy to see us, and right away asked me to get in bed with her so she could lay on me.  Her voice was a bit scratchy.  She asked for a popsicle and was looking for her Leappad to watch Caillou, both signs that she was feeling pretty good.  Three popsicles later she was doing great and given permission to leave.  That was probably before 10:30.  Emmie was chatty, hungry, and very clingy on the ride home.  She wanted me to somehow navigate the curves of Storrow Drive while reaching into the back seat to hold her hand.  Which, I was happy to do.

After that it was a pretty normal day.  She had a great lunch at Nana and Grampy's, played, and took a nap.  She woke up asking for spaghetti, so I made a tuna pasta puttanesca-style that she ate two huge bowls of.

 The only thing she really gave me any flack about all day was taking her Exjade before dinner.  How yucky it tastes, how the stuff at the bottom of the glass is gross, how she needed an M&M to get it down.  And, as we fought about it, I had this sinking feeling in my stomach.  What if, after all of this Exjade stuff for a whole year, she's one of the 30% of patients who are non-responders to the drug?  What if those moms were right and Exjade doesn't work well with dairy products?  What if we haven't been crushing it enough?  What if the sprinkles and Dora yogurt and daily Exjade Happy Dance and now splitting the dose so we do it all TWICE a day...was all for nothing.

But it wasn't.

The NP called the next morning with literally some of the best news I've ever heard.  Emmie's liver Ferriscan was down to 5 and her cardiac T2* was up to 35!  I cried and cried and nearly dropped the phone as I asked her to repeat those numbers again and again while I waited for the report to show up in my inbox.  Yes, Emmie's ferritin had started to come down slowly, particularly in the last two months.  But I never, ever thought that we would see results like this--actually, some of the BEST results I have EVER heard of.  With her latest ferritin still at 2200, even KNOWING what I know about the lack of correlation between ferritin and total body iron, I never expected her to have a liver iron in the normal range.  And when the NP said to me, "Do you remember the Exjade non-responder study we worked on?  Well, I would classify Emmie as a responder."  To which I replied, "Yeah, you freakin' think?!?!?"  Very scientific of me.

So, she did it.  Well, being honest here...we all did it.  While the bulk of the burden was on Emmie to take the Exjade, I'd be lying if I didn't say that it was a team effort.  So we hugged her tighter than ever and went to The Cheesecake Factory to celebrate.  And when we asked her why we were having a family celebration, she said, "Because I did a good job with my medicine, and because my belly pictures were great!  And I don't even remember them putting the IV in!"  Spoken from a real trooper, a great role model, and our inspiration.

Relaxing while the Versed was taking effect.  Emmie, not Mama.
I was totally hysterical and could have used some myself.

This is just a few minutes after she woke up!  Lookin' great!

I was feeling SO SO SO relieved when this picture was taken.

Nothing like having my baby back in my arms.

Exactly one week later, we'll be back, doing it all over again for Rosie...



Monday, March 24, 2014

Maine Maple Weekend

Being a huge Maine lover and a huge maple lover, I have always wanted to participate in Maine Maple Weekend.  For whatever reason, we've never made it happen until this year.  What a super time!

I did a lot of research (typical for me, I OVER-research everything) before we went, looking for the perfect farm to visit.  My goal was to balance visiting an authentic sugar shack with finding some fun things to keep the girls happy.  Chase Farms was the perfect place for our family to visit!

Chase Farms is an easy ride from the Boston area, and near everything we love on Maine's beautiful southern coast.  The farm stand itself and sugar shack are pristine and very visually pleasing.  I felt good about eating food from their operation.

Given the super cold winter, we were very excited to see that they were boiling sap for maple syrup.  We each had a "shot" of warm maple syrup right from the evaporator.  Well, actually, Emmie ended up with 4 shots of warm maple syrup right from the evaporator.  She LOVES her maple syrup!  We also had samples of maple cream which was delectable, and free servings of freshly popped popcorn.

Warm maple syrup.
The website indicated that there would be hay rides, although we did not see any happening during our visit.  Which was actually fine with us because we felt that there were plenty of other interesting things going on for the girls.  Both girls participated in the free face painting, but the place where we actually spent most of our time was in the barn.

Being the "city folk" that we are, of course the girls walked into the barn and yelled, "PEEE-YOU!" about 10 times, embarrassingly loud.  In their defense, it didn't exactly melt my mascara, but it was rather stinky.  You get used to it, especially as you see the super cool animals and adorable baby pigs living in the barn.  There were workhorses, cows, and pigs.  I kept saying to Patrick, "Is it me, or are these animals HUGE!?!??!"  They were really quite impressive and kept the girls interested for a very long time.  Of course the baby pigs were the favorite of everyone.  All of the workers were friendly and patient, especially when Emmie was DYING to pet the horse, Bill, but was still a little shy about it.

Their "PEEEE-YOUS" turned to smiles once they saw all of the animals.

Rosie checking out the baby pigs.  She kept saying, "Oink, oink!"


Emmie petting Bill the horse.  She said next time she wants to ride him.
We told her it might be better if she started off on a slightly smaller horse...
They look like little ranch hands.

After all of the barn fun we warmed up in the sugar house and had some amazing maple syrup cotton candy.  It was being freshly made and was a HUGE serving.  Turns out, Emmie loves cotton candy.  Rosie, not so much.

We bought some delicious maple goods to remind us of our great visit to this super cool farm and were on our way.  We left smiling and super happy that the sun had come out (it was snowing at the beginning of our visit) and that we visited this great family farm.  All in all, we would highly recommend visiting this farm, whether it be for Maine Maple Weekend or any weekend.  The people were nice, their treats were delicious, and it was a beautifully kept place.






As we headed to Ogunquit, we noticed that Chase Farms actually has a farm stand right on Route 1.  We've probably driven by it about 1,000 times and never stopped...this summer, we definitely will be sure to support this local business.

After our great time "down on the farm" (yes, we love country music), we headed to one of our favorite places on the coast of Maine, Ogunquit and Perkins Cove.  It was so lovely out (ie, 45 degrees...I guess we are true New Englanders) that we were able to get some delicious coffee at one of our favorite spots, Breaking New Grounds Coffee, and sit outside on their adirondack chairs.  The house blend was perfect, and the view of the cold Atlantic waves crashing on the rocky Maine coast was beautiful.

Perkins Cove coffee shop.

Perkins Cove.


Perkins Cove footbridge.

Our final stop on our perfect Maine daytrip was to Bread and Roses Bakery on Maine street in Ogunquit.  We had a huge, warm chocolate chip cookie and a whoopie pie.  While the whoopie pie might not have been quite as fresh as during the busier summer months, the cookie was soft and gooey and sweet.  You can just tell they used great ingredients.  Now, even though I didn't think the whoopie pie was as good as they are in July, that's not to say that we didn't eat every last morsel of it.

We sat outside and enjoyed the last of the "warm" weather for I guess another week, chatting up some locals and eating our treats.  It was a perfect end to a sugary, smelly, happy, and amazingly memorable day with the girls.  Well, isn't every day a sugary, smelly, happy, and amazingly memorable day with the girls?  Yes, I think so.