It’s April
break and we spent a few days hiking in Acadia National Park, Maine, so I’ve
had a little time to catch up on this blog.
 |
| Jane and me on the South Bubble peak in Acadia National Park |
Running Update:
|
3/20/2018
|
Swim
|
|
1500.0 yd
|
|
|
3/21/2018
|
Run
|
|
5.0 mi
|
48:47
|
|
3/23/2018
|
Run
|
|
5.0 mi
|
46:31
|
|
3/25/2018
|
Run
|
|
10.2 mi
|
1:40:46
|
|
3/30/2018
|
Run
|
|
4.9 mi
|
47:20
|
|
4/1/2018
|
Run
|
|
14.0 mi
|
2:19:51
|
|
4/2/2018
|
Swim
|
|
2050.0 yd
|
|
|
4/4/2018
|
Run
|
|
7.6 mi
|
1:16:32
|
|
4/6/2018
|
Run
|
|
5.0 mi
|
48:45
|
|
4/7/2018
|
Swim
|
|
2000.0 yd
|
|
|
4/8/2018
|
Run
|
|
18.1 mi
|
3:01:28
|
|
4/11/2018
|
Run
|
|
5.0 mi
|
48:26
|
|
4/13/2018
|
Run
|
|
7.6 mi
|
1:13:59
|
|
4/14/2018
|
Swim
|
|
2200.0 yd
|
|
|
4/15/2018
|
Run
|
|
10.2 mi
|
1:42:12
|
|
4/17/2018
|
Run
|
|
5.1 mi
|
50:12
|
|
4/18/2018
|
Run
|
|
7.6 mi
|
1:12:44
|
|
4/20/2018
|
Run
|
|
3.4 mi
|
38:35
|
|
4/22/2018
|
Run
|
|
20.7 mi
|
3:32:14
|
Five weeks
until the Vermont City Marathon! It
comes right at the end of May, which is NF Awareness Month. You are welcome to donate to our race anytime!
NF Update:
When we left
NIH earlier this month we did not have the findings of Jane’s MRI, nor a plan
for her treatment going forward. We
thought it was likely that Jane’s medication dose would be increased, given
that she has grown so much in the past 6 months. Over the last couple of weeks, we’ve learned
the results and have had several discussions with Jane’s care team about where
to go from here.
 |
| Jane's MRI volumetric analysis plot |
Dr Widemann, Jane’s pediatric neuro-oncologist, reviewed this with Dr Dombi, the pediatric radiologist on our team, and both agree that her tumor is overall stable.
On Jane’s lip and lower face there is a distinct
subcutaneous nodule, which may be a separate plexiform neurofibroma (PN)
or may be part of Jane’s larger tumor, that has “minimally increased” over the past
few scans. It seems to be limited to the
skin, and Dr Dombi reassured me that it exhibited no other concerning signs. In her words, there is “zero worry of
malignancy” in this nodule, despite its recent change in size.
 |
| Sagittal (top) and coronal (bottom) views comparing 2015, 2016, 2017, and 2018 |
Because Jane’s tumor is stable overall, Dr. Widemann considered maintaining Jane’s current dose of selumetinib. She did not want to risk side effects since Jane is tolerating the drug so well. The problem with this approach is that right now Jane taking 30mg of selumetinib daily, which translates to about 20mg/m2 daily (the medication dose is calculated based on the patient’s body surface area, or BSA, in square meters), which is only about half of the dose she could be taking in this study.
Of course, we are very happy that Jane's tumor is
stable. It's true, when I look at the plot, the volume hasn't really
changed since May 2016. But it makes me—and
our team—nervous to see the trend upwards in the last two scans. My brain
tells me it isn't necessarily a real trend, but my heart wishes it would turn
downwards again! I am also happy that
Jane has not had significant side effects and I want it to stay that way, but
in the back of my mind is always the concern that she won't benefit from the
drug if the dose is too low.
Ultimately, it may be that Jane’s tumor could stabilize on
its own, without medication. Natural history
studies of plexiform neurofibromas have shown that some tumors spontaneously
stop growing after puberty, the median age being “adolescent to young adult”
according to Dr Widemann. However, given
that Jane is only 11 years old, Dr Widemann does not favor stopping her medication
yet.
We decided to gradually increase Jane’s dose of selumetinib. We are limited somewhat by the dose of pills
that are currently available (10mg and 25mg), and by the fact that our team
does not want to increase the dose more that 30% at a time. The plan is to go from 30mg daily to a total
of 40mg daily (20mg twice a day) for the next three months. If Jane tolerates this change, we’ll jump to
50mg daily (25mg twice a day) for three months, until Jane’s next MRI. Fingers crossed.
Jane Update:
Earlier this
year I mentioned how Jane is the only one of our kids to play basketball and to
play in the school band (instead of the orchestra). She also is the only one of our kids to try acting! Below is a clip of Jane’s monologue from the play her theater class put on.
(It’s only one minute long.) The
theme was the Greek Gods, and Jane played Demeter, Goddess of the Harvest.
Here she is confronting Hades, God of the Underworld, after he kidnapped
Persephone, Demeter’s daughter :)
