Wednesday, June 17, 2020


Running Update:
5/18/2020
Run

5.0 mi
47:17
5/20/2020
Run

5.0 mi
47:43
5/22/2020
Run

7.6 mi
1:15:18
5/23/2020
Run

5.0 mi
48:06
5/25/2020
Run

5.0 mi
48:25
5/27/2020
Run

7.5 mi
1:14:58

Even though it’s June 17, May 27 is my most recent running entry because on that day I went and broke my heel!  It wasn’t even a running injury—I was foolishly standing on a chair to open a window and lost my balance.  It turns out I cracked my calcaneus and I’ve been in a boot ever since.  It looks like it’s going to be a while before I’m running again :(  My back-up sport is swimming, but since the pools and beaches here are still not fully open, I can’t swim right now, either.  I confess I am an unhappy camper!  The perfect running weather we’ve been having lately isn’t helping.  I’m trying to keep in running shape with some weight and strength training so I can jump back into training as soon as I am able.  I am thankful I don’t have any races scheduled for the near future (and who knows what will even happen with races this fall?)

Every year I try to run 1000 miles to fund raise for NF, so by each July 1 I like to have completed 500 miles.  On May 27 I was at 442.8 miles for 2020, so I thought I was golden—not anymore!  I might have to find an alternate goal this year…


NF Update:
There’s been so many more important things going on in the world that I hadn’t posted a follow up to our discussions with NIH and from NF Awareness Month.

Since my last post we had a very helpful and informative phone conversation with Dr Prashant Chittiboina, the neurosurgeon from NIH who will be following Jane’s abdominal tumor.  He is still recommending that the tumor be removed but he felt that this surgery would be considered "elective" at the moment, and suggested the following time frame:  repeat abdominal MRI in September at our next NIH visit.  If the tumor was stable, consider following it with imaging and then plan for surgery next summer (or over some other school break) so that Jane wouldn't have to miss school and to allow for the pandemic to be under better control.

The next step in this process is to consult with the team’s Urologist at NIH to see his thoughts on surgical removal of the tumor, specifically to see if it could be removed robotically or if it would require an open surgery.  The plan is to meet them both surgeons in person at our visit to NIH in September. 

~~~

Next I have a few videos to share from the final days of NF Awareness Month in May.  First is the broadcast of Home Is Where the Heart Is, a Zoomathon held on May 17 (NF Awareness Day) to benefit the Children’s Tumor Foundation.




You can see Jane’s interview starting right at the 10 minute mark!

And remember all the gorgeous Make NF Visible portraits I posted last month?  Here is a montage of interviews with the subjects of those portraits, including Jane.



~~~

Finally, even though it was approved by the FDA almost two months ago, I got a thrill seeing Koselugo listed on our hospital formulary.  That makes it seem even more real.



Jane (and family) Update:
We have two big news items from our family this month.  The first is that we got a dog!
Meet Henri:





Yes, we have a Pandemic Puppy, and we are all in love—even me, who had been the most reluctant to take the plunge <3





The second is that Helen graduates from high school this week!  As everyone knows, the Class of 2020 everywhere has had its senior year celebrations curtailed, and Helen’s class is no different.  Still, our school system has tried its best to host some special senior events to honor them.  They’ve had a couple of drive-in activities and the local movie theater displayed all the graduates’ names on its marquee. 



Graduation itself will also be drive-in: each family is allowed one car per graduate. After the usual student and faculty speeches, the students will be able to walk across the stage to receive their diplomas.  The students were even each given special a black mask with a tiger paw (the school colors and mascot) with their cap and gown!  The event will be livestreamed for out-of-town friends and family, and after the ceremony there will be Class of 2020 vehicle parade to the town green.   No handshakes or cap tossing this year, but we are thankful we will be able to celebrate Helen and her classmates together.

Congratulations, Class of 2020!

Saturday, May 16, 2020

Running Update:
4/29/2020Run 9.1 mi1:30:41
5/1/2020Run 7.6 mi1:14:23
5/4/2020Run 5.0 mi48:14
5/6/2020Run 5.0 mi48:35
5/8/2020Run 8.0 mi1:18:44
5/9/2020Run 3.2 mi30:18
5/11/2020Run 5.0 mi47:44
5/13/2020Run 9.1 mi1:30:01
5/15/2020Run 5.0 mi
48:09
Still maintaining...


Jane Update:
Jane had her MRI earlier this week at the Yale's Trumbull facility (the only place locally they're doing pediatric MRIs). For the first time ever I was more nervous about bringing her into a medical facility than about what the MRI might show.
 
It was quite a novel experience.  Jane hadn't been out in public since school closed 3/13!  She knows what's been going on with the pandemic and with social distancing, but it was a different matter to actually experience needing to wear a mask and staying 6 feet apart from people.  I think she was a little shocked by the process.  I was a bit overwhelmed myself!  I've been working in the COVID call center, but I haven't had direct patient care since March, either, so even I had some culture shock with having our temperature taken at the entrance to the facility.  We both wore masks and old clothes so we could throw them in the laundry as soon as we got home.
 
The staff at the MRI center could not have been more welcoming, professional, and reassuring.  Jane tolerated an hour and a half in the scanner, complete with IV and contrast.  She's a rockstar.


I got a disc with the MR images and uploaded them to NIH the next morning, and our team reviewed them at their NF Clinic later in the week.  I am happy to report the abdominal nodule is very stable in volume and has no change in its appearance, so both of these things are reassuring.  However, our team continues to think that removing the nodule is reasonable, not because they have concerns for malignancy, but because of its location and potential to affect Jane’s kidney in the future.  Therefore, we’ll be meeting remotely sometime soon with the team’s neurosurgeon and urologic surgeon to discuss the implications of a surgery.  We are still waiting on the volumetric analysis of Jane’s facial plexiform neurofibroma but the team doesn’t have any concerns of obvious growth, which is a great news, between hold on Jane's selumetinib this past year and lowering the dose.
 
While we're waiting to talk to the surgeons and for the plexiform volumes we'll continue on Jane's current dose of medication and plan a return trip and repeat MRIs in the fall.
 

NF Update:
Meanwhile, NF Awareness Month going strong!  The Children's Tumor Foundation really outdid themselves with their campaign this year.  Their theme all this month is "Make NF Visible", and they have done an outstanding job bringing it all together, especially during a pandemic.  My favorite parts (so far) are the Make NF Visible Portraits and the upcoming NF Zoomathon scheduled for May 17 (World NF Awareness Day).


For the portraits, they partnered with award-winning photographer Craig Warga, whose son also has NF, to look at the different ways NF can affect a person’s life, even when no one else can see.  They paired people with NF who have manifestations that can be seen with those whose affectations can’t be seen, as a way to highlight the visible and invisible ways NF makes itself palpable in a person’s life. They comment, "Life with NF can mean living with things like brain tumors and cafĂ© au lait spots, neurofibromas and learning difficulties, loss of hearing and excruciating pain, successful surgeries, failed clinical trials, physical scars, emotional wounds, cross-country networks, unspoken understanding, and unbroken bonds."  You can view them below or at the CTF website:  https://www.ctf.org/understanding-nf/make-nf-visible


Jane’s quote hit me in the heart: "I think it is important for people to know about NF because you don't know what someone might be going through if they have NF and I feel it is easier to talk or comfort people if they know what I am talking about."


MY HEART <3












The Zoomathon to benefit the Children's Tumor Foundation is being hosted by actor/producer Jonathan Sadowski and will bring together well-known actors, musicians, athletes, comedians, chefs, magicians, celebrities, and NF Heroes (including JANE!) all combining forces to make sure the world knows about neurofibromatosis and TO MAKE NF VISIBLE!  It will be live streamed at www.ctf.org/heart, with links provided to view on Facebook, YouTube and closed-captioned on Zoom. You can watch a preview here!  Please tune in between 7-9pm (ET) on Sunday--it's going to be so exciting!









Tuesday, April 28, 2020


Running Update
4/6/2020
Run

7.6 mi
1:14:51
4/8/2020
Run

5.0 mi
48:10
4/10/2020
Run

9.4 mi
1:33:40
4/13/2020
Run

7.6 mi
1:15:06
4/15/2020
Run

5.2 mi
51:38
4/17/2020
Run

9.1 mi
1:32:38
4/20/2020
Run

5.0 mi
48:17
4/22/2020
Run

7.6 mi
1:15:02
4/24/2020
Run

9.1 mi
1:31:32
4/27/2020
Run

5.0 mi
49:09

Still running while staying safe during this pandemic.  I’m registered for the New Haven Half-Marathon on Labor Day and the New York City Marathon on November 1st, but there’s some skepticism in the running community that any of the big fall races will go on as planned.  (Dumb Runner author Mark Remy puts it this way:  “Adorable Child Still Believes in Tooth Fairy, Unicorns, 2020 Fall Marathons”)  For now I’m just enjoying my runs and holding off on training until the summer.


NF Update
This big news this month, of course, was FDA approval of selumetinib, the study drug Jane has been taking for the past six years!  We are thrilled, especially after all Jane has been through to get to this point.  We got a lot of news and social media coverage, starting with this announcement from the Children’sTumor Foundation:  www.ctf.org/first-drug-approved-for-nf.  It featured a video of Jane and me and our comments about the approval


Our picture even made it onto Cancer.gov!



Selumetinib’s brand name will be Koselugo (pronounced co-sell-you-go), manufactured by AstraZeneca (www.koselugo.com). As my friend Simon Vukelj at the Children’s Tumor Foundation told me, “You'll recognize the 'selu' from 'selumetinib' in 'koselugo'.”

You might say that there’s been a “selu-bration” in the NF community this month :)

The words that meant the most to me were from another NF Mom:  I am so grateful to all who made this bold and courageous decision to enroll their child in the clinical trial for selumetinib; the kind of choice that a parent should never have to make, but that they are grateful to have available when there is nothing else. 

We also received this letter from our team at NIH.



Jane Update
Jane is staying healthy, busy enough with distance learning from her school but enjoying extra time for reading and watching movies and taking walks with the family.  So far her local MRI is still scheduled for mid-May—we’re hopeful it will go as planned.

Keep an eye out for lots more pictures of Jane during the month of May--NF Awareness Month!

~~~

Finally, I’d like to dedicate this blog post to Jane’s Grandpa Bob, who passed away this month.  Today would have been his 91 st birthday.  He was a true gentleman-- kind, considerate, and unassuming.  Our lives won’t be the same without him.  He could always be heard whistling happily, and I won’t ever be able to listen to this song without thinking of him.

Three generations of Robert Constable


Friday, April 3, 2020


Running Update:
3/18/2020
Run

7.6 mi
1:15:44
3/20/2020
Run

5.0 mi
44:49
3/23/2020
Run

7.6 mi
1:17:21
3/25/2020
Run

5.0 mi
49:30
3/27/2020
Run

10.2 mi
1:42:20
3/30/2020
Run

7.5 mi
1:13:47
4/1/2020
Run

5.0 mi
48:03
4/3/2020
Run

5.0 mi
50:01

Just before my last post the NYC Half Marathon had been canceled due to COVID-19.  Not surprisingly, the Vermont City Marathon scheduled for May has now also been canceled :(
So as of this moment, I don’t have any races planned until the New Haven Road Race on Labor Day.  I’ll continue to train, and if the pandemic abates sooner than expected, maybe I’ll find a summer race to register for.  But for now, I’m just going to maintain my miles.

In other pandemic/running news, it turns out that Dr Anthony Fauci, Director of the National Institute of Allergy and Infectious Disease, is an avid runner.  (As if we needed a reason to love him more!)  At age 79 he still runs 3.5 miles a day, even when working 19-hour days during the current coronavirus pandemic!  Read about his running here.


NF Update:
More good news regarding selumetinib, Jane’s study medication.  Phase II of the NIH clinical trial of selumetinib for plexiform neurofibromas was published in the New England Journal of Medicine on March 18.



Jane started on the drug in Phase I, the results of which were published in the NEJM back on 12/29/16.  I wrote about it here: nfemom.blogspot.com/2017/01/.

When the National Cancer Institutes tweeted about it, they used a picture of Jane and me!



The Director of NIH, Dr Francis Collins, wrote an article reflecting on the study.  In it he gives some background about NF and describes his personal connection to NF patients:


Jane Update:
As anticipated, our 6-month return visit to NIH this week was canceled due to COVID-19.  This is the email we received from our team last week:

Our team has reviewed J in detail during our NF Clinic this morning, as our study sponsor said that our team could decide which evaluations would be necessary to complete, either at home or NIH and either now or in the future, for J to continue on MEK/Selumetinib.  Based on that, J does NOT need to come to NIH for any evaluations.  J’s next visit to NIH will be for her pre-cycle 83 visit.  I’m including a list of each evaluation that J would have had completed below, with our thoughts on each:
  • H&P with Anne: We will do this “virtually” over FaceTime through our phones.
  • Vital Signs: Not necessary at this time and will be deferred
  • Measurements (Height, Weight, & Body Surface Area): Not necessary at this time and will be deferred
  • Labs: Not necessary at this time and will be deferred
  • Urine: Not necessary at this time and will be deferred
  • MRI: We would recommend having an MRI done at home, when it is safe to do so, given the current COVID-19 restrictions.  Our first priority would be a clinical MRI of J’s abdomen, and if J was going in for the abdominal MRI, to also get a face/sinus/ENT MRI (what we use for the MEK re-staging), since we did reduce her dose of MEK in October.
  • Echocardiogram: Not necessary at this time and will be deferred
  • Eye Exam: Not necessary at this time and will be deferred
  • EKG: Not necessary at this time and will be deferred
  • Additional Consults:
    • ENT: Not necessary at this time and will be deferred
    • Dermatology: Continue management with dermatologist at home.
    • Neurosurgery: Depending on when the MRI will take place, would determine when we proceed with the Neurosurgery consult.

As of this moment Jane is scheduled for her MRI at home in mid-May, the first available date since all non-urgent medical appointments in our area are on hold until after April 30.  It might be that this date will get pushed back as well depending on what happens with the pandemic, but we’ll take one day at a time.

A FaceTime visit with Anne, the Nurse Practitioner on our team.

In happier news, we celebrated Jane’s 13th birthday this week!  We joked that she is now a “quaran-teen”.  She won’t be able to celebrate with her friends in person, but we had a family celebration and I even managed to procure an ice cream cake, Jane’s favorite.

Singing for Jane!



Thirteen presents for a thirteen-year-old!


Lego Orthanc!  More exciting than the books :)