Saturday, June 13, 2015

NIH Visit June 2015—Summary

Another NIH visit done!  Here’s an overview of the last 3 days:

The Wednesday afternoon we arrived the Children’s Inn had some special activities in store for us.  Jane got to participate in a play performed by Only Make Believe.  Only Make Believe is a non-profit organization that creates and performs interactive theatre for children in hospitals and care facilities.  We had seen them perform last summer when were at NIH, and this show proved equally entertaining!

Later on we made home-made play dough and indoor snow.  Jane also became fast friends with a little 9 year old girl from Austin who was visiting NIH for evaluation of a rare skin disorder.


Jane at her photography appointment.
On Thursday—the first day of our clinical appointments—we checked into the hospital 8:45am and out of the hospital 3:45pm. Whew!  Jane handled it with grace and was in good spirits.  We showed our sonographer Jane's diorama of the Lego ultrasound and we showed our MR radiologist Jane’s Lego MRI suite—both were duly impressed.

We shared and learned some new jokes.  Jane had fun tricking various people with, “Mississippi is a long word.  How do you spell it?”  [Answer:  “I-T!”]  Jane was tricked in turn by a nurse who thought she looked bored.  He said, “I can prove you’re excited!  Knock-knock!”
“Who’s there?”
“Woo who?”
“See!  I told you you were excited!”  (Jane liked that.) 
We also shared a joke our sitter, Brittany, had taught us:  “How many tickles does it take to make an octopus laugh?”  [Answer:  “Ten-tickles!”]

When we arrived back at the Inn after our long day I was puzzling over how I could go about getting Jane's medication diaries back to the hospital (I forgot them that morning) without dragging Jane along with me, when two of the Children's Inn staff offered to watch kids in the playroom in order to give parents an hour's break.  I love that place sometimes.


Friday morning I did not handle things as gracefully as Jane did.  I did a full-on Momma Bear when the anesthesia team mistakenly thought that Jane was having a 3 hour full-body MRI (instead of the 90 minute face and orbits scan that was ordered—there’s a big difference between a 90 minute scan and a 3 hour scan when you're under anesthesia) and didn’t listen to me when I told them I didn’t think it was right.  I was so angry!  I felt like I was watching one of those movies hospitals show their staff illustrating the importance of communication between members of the care team in order to avoid medical mistakes.  I can be ugly when it comes to health care providers who don’t listen (especially when I haven’t had coffee yet!)  This is why we do pre-procedure time-outs, people!!

We checked into hospital 7:25am and out of hospital 2:55pm with only a few bumps along the way, but the upshot of the day—a STABLE tumor!

We met with Jane’s neuro-oncologist, Dr. Brigitte Widemann, who was pleased with the results.  She said in her patients taking AZD6244 it has been very typical to see a plateau of tumor volume after about a 25% decrease (Jane’s was 24%).  The fact that Jane's tumor is still responding in this typical fashion, despite Jane being on a lower dose of the medication, is even more encouraging.  Dr. Widemann recommended staying at the lower dose, and reassessing again in 6 months.  I was disappointed at first that we hadn't seen any further decrease in the size of Jane's tumor, but I am relieved that Dr. Widemann was so pleased with the results.

I’m going to show one more set of pictures—it’s a bit confusing at first if you are not used to looking at MRIs, but what follows is a side-by-side images of the same slice of Jane’s tumor from 2/2014 compared with now.  Tumor is the big white blob on the left of the image.  You can see in some spots where it appears to have just melted!  (You can also see from these images why her tumor can’t be surgically removed—it wraps around everything.)

Friday afternoon we spent back at the Inn chillin' with some DanDTM videos (Minecraft :/) and with Jane drinking an Oreo milkshake.  (Mmmm!)  She was still feeling sleepy from her anesthesia, but said, "I'm just going to drink my shake to get my energy back."  Over the course of three days we burned through several volumes of Jane’s new favorite chapter book series—The Littles.  (One of my childhood favorites, too.)


Here we are at the airport, awaiting our flight home.  I'm looking pretty worn--definitely think these trips age me!

Tuesday, June 9, 2015

Running Update:
5.0 mi
5.0 mi
7.6 mi
Just maintenance right now.  Training for the Hartford Marathon doesn’t start for another couple of weeks.

Jane Update:
Jane and I return to NIH tomorrow for the next four days for her restaging.  This means lots of testing to make sure she is not having any adverse effects from the study medication, and another MRI to check to status of her tumor.  (I went back and counted recently—this will be Jane’s seventeenth MRI.)

Here is our schedule for the visit:
Wed 6/10
Arrive at NIH
Thu 6/11

9am – Photography, Room 1N230, 2nd Floor
9:30am – EKG, Radiology, 1st Floor
9:45am – Vital Signs (3 Hts & 3 Wts), Pediatric Clinic, 1st Floor
10am – ECHO, 5NE, 5th Floor
11am – Physical Exam, Pediatric Clinic, 1st Floor
12:30pm – Pre-Anesthesia, Room 2C523, 2nd Floor
2:00pm – Eye Clinic, OP Clinic, 10th Floor

Fri 6/12

7:30am – Labs, 1NW Day Hospital, 1st Floor
8:45am – Sedated MRI, Radiology, 1st Floor
1:30pm – Family Meeting, 1NW Day Hospital

Sat 6/13

Looong days for an 8 year old!  I always hope I’ve packed just the right items in my backpack to keep Jane entertained through these visits.

Speaking of schedules…  Every family’s schedule is hectic this time of year, what with end-of-year projects, recitals, field trips, exams, and final games.  We’re adding in Todd’s busiest few travel weeks for work and our trip to NIH—I’m maxed out!  I decided to post below the email I sent out a few days ago to my Mom, my husband, Todd, and our awesome sitter, just so someday I can reflect back on the craziness of it all (perhaps fondly, perhaps with relief!)

Day 1
[sitter], FYI.  H has tutoring at home as usual.
J does NOT have piano (lessons over for the summer).  Also different this week:  T and A have a soccer game in Meriden (instead of practice), though they will probably still leave home at about the same time,  I anticipate being home about the usual time.

Day 2
J and I leave for Washington about 10am.
Grandma:  will you pick A up from school as usual and then bring him to piano lessons?
Todd:  will you plan on being home in reasonable time so as to relieve Grandma?
H, A, and T:  the cleaners come on Thursday, so remember to put away anything that you don’t want moved.

Day 3
[sitter]: usual pick-ups and then violin lesson for H.  Would you also stay long enough to bring H to soccer practice?
Todd:  Will you please remind H to bring her violin and music home today.
Grandma: would you bring H home from soccer practice?

Day 4
Todd:  A has a field trip with his class for the school day.  He needs to wear comfortable clothes and sneakers, and he needs to wear BLUE.  He needs to bring water and a bagged lunch without glass bottles.  He should put on sunscreen and bug spray before school (and bring a hat if necessary).
[sitter]:  usual pick-ups for Friday, no afterschool activities.  Maybe froyo?
Grandma:  T will bring the kids over after dinner for sleepover.

Day 5 (weekend)
Todd leaves at 5am
[sitter]:  Can you pick the kids up at my Mom’s this morning?
It will be a long day for you!  J’s and my flight gets into Hartford, which means we won’t be back in Madison until a little over an hour later.  Any activity you think might be fun for you and H and A that morning?  (Or just chill at home…)  On the schedule:  H has a soccer game, so will need to be there about a half-hour early.  J and I will then meet you and A at home in time for us to go to A’s piano recital.  Could you then collect H from her game and bring her home?  We’ll all reconvene at our house after the recital.  Whew!

Day 6 (weekend)
[sitter]:  If you don’t think you will be too worn out from the day before, would you mind coming over for a couple of hours Sunday morning so I could run?
Grandma:  Would you please watch H and J while I bring A to his soccer game in Cheshire?  I would leave with him about an hour before the game and possibly drop the girls off with you at that time.

Day 7
Grandma: will you come over to the house to get J ready for school and on the bus?

Day 8
[sitter]:  regular after school pick-ups.  The only activity is A has soccer practice.  If you can get him there I will ask his coach to bring him home.

Day 9
Grandma:  THIS IS A NEW REQUEST.  I have had to add a surgery in for this morning.  Could you come over to the house to get J ready for school and on the bus this day as well?
FYI:  H’s recital is this evening.

Day 10
Grandma: will you come over to the house to get J ready for school and on the bus?  And once again, could you bring H home from soccer practice?
[sitter]:  like the previous weeks, can you stay long enough to bring H to soccer practice?

Day 11
[sitter]:  I don’t remember if we discussed this—this is the kids’ last day of school and they have an early dismissal.  My last patient is at 11:30am, but I may not make it out in time to pick up A and J.  Are you available early that day?

Day 12 (weekend)
[sitter]:  I also don’t recall if we discussed this weekend!  It turns out Todd doesn’t arrive home until Saturday afternoon, but both H and A are in a soccer tournament in Cheshire this weekend!  I don’t yet have the schedule—I am hoping that they will have games at similar times so that J and I could just go and hang out at the Cheshire soccer fields and watch all the games.  If the schedule ends up with games at wildly different times, would you be available to either watch J and/or give rides to H or A?  Let me know.

OMG.  I have been feeling a bit nauseated just wondering whether we can pull it off…

On a lighter note, Jane had her end-of-year piano recital this weekend :)

Before the performance.

NF Update:
Exciting news regarding Jane’s study medication.  This week at the NF Conference, the largest gathering of the world’s NF experts in neurofibromatosis, Jane’s doctor, Dr. Brigitte Widemann, presented the results of the Phase I trial of AZD6244.  As readers of this blog recall, Jane was one of only 24 children enrolled in the study to test this new, experimental treatment for NF.  For reference, the tumor on Jane’s jaw and neck is called a plexiform neurofibroma, an inoperable type in her case.

Some key information from the text:
“Plexiform neurofibromas exhibit the most rapid growth in young children, and therefore early intervention in children with growing PNs may result in the greatest clinical benefit. Consistent volume decreases of large PNs has not been reported in the past, and today’s announcement indicates a promising future for the development of effective medical therapies for NF1 related PNs.

"16 of 24 patients treated with the MEK inhibitor selumetinib (AZD6244 hydrogen sulfate) saw a tumor volume decrease of ≥ 20%".

(See the full text of the article here.)

It was very frightening to be one of only 24 patients trying this new drug, but we have been so lucky to be one of the responders!!  I desperately hope it continues to work well!

PS  This is your donation dollars at work!  Thank you thank you thank you!
PPS  You can always donate here :)

Finally, one more light-hearted note:  Todd brought home a souvenir from his MR meeting last week—an actual toy MRI!  What a perfect gift for Jane!