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...



6 comments:

likeschocolate said...

So happy for your family and for Miss E!

Lilly said...

i have a wide world grin on my face, what a way to start my day! HURRAY for emmie, she is the best trooper!

Anonymous said...

Brave sweet girl.You are a wonderful mom to your beautiful girls.
Renate

Matt Jamie Dailey said...

We will be doing this next month with Belle. Thanks for the blow by blow.
So glad Emmie's turned out so good!

Anonymous said...

I'm so glad for you and for little Emmie! Congratulations!!!

Marika

Karla Baca said...

SO happy to hear Emmie's Scan was so good!
Our Daughter( age 14) is on Exjade and Ferriprox and her liver scan cam back a 9 this year...last year it was 6...the year before a 4, the year before a 3. Josy developed an allergy to desferol and cannot take it anymore. Hydrea did not have the hoped for results ( being able to space out her transfusions further than every 4 weeks) Exjade has been tricky with her kidneys...higher doses lead to fanconi syndrome. Thalassemia Major has been a difficult road for our family with 2 adopted from India. We don't know anyone else, and Sanjay died 3 years ago at age 10 during a BMT from a freak brain bleed. Thank you for your posts about Thal treatments...they do give me hope for my daughters future.