Tuesday, May 22, 2012


One of the challenges of diagnosing retinoblastoma early centers around the fact that it causes very few symptoms and can often go undiagnosed for some period of time.  The most common presenting sign of retinoblastoma is leukocoria, or the white pupillary reflex.  This is most often noticed on a photo of the child.  Instead of the normal black or 'red' pupil, the pupil looks strangely white or opaque.  The second most common sign is strabismus - a malalignment of the eyes which prohibit them from precisely focusing on the same point in space.  Making things even more complicated is the fact that strabismus is actually pretty common and only in very rare instances does it represent something serious.

Edward suffered from retinopathy of prematurity (ROP) and as such he had to have regular eye exams.  He had a little strabismus last fall, but his eye exam at that time did not reveal any evidence of tumors.  His strabismus continued and maybe got a little worse, but otherwise, he was his happy, normal self. Of course, his next eye exam in March had much different results, revealing bilateral rb tumors.  After soaking in the diagnosis, we frantically searched through all of our photos of the little man to see if we missed leukocoria.  After sifting through the hundreds of photos of Edward (his life has been well documented to say the least), there was one photo that maybe showed a white pupil.  However, it was subtle at best and hindsight is always 20/20, so we did not feel that we had missed anything obvious.  However, a couple weeks after his diagnosis, Katie got a new phone with a slightly different flash and out of nowhere, we saw it. 

The photo made us stop in our tracks.  We again looked through every old photo of Edward, but still could not find any obvious warning sign.  So, if any of you happen to notice a dramatically white pupil in any picture of a child, definitely do not ignore it.  Of course, there are several things that can cause a white pupil and not all of them are cancer, but a white pupil deserves and requires a thorough opthalmologic evaluation and you might even save the child's life.

Thursday, May 17, 2012

World Rb Week

Edward had his weekly CBC today to monitor his cell lines and most importantly his neutrophil count.  We are two weeks out from the last cycle and this is typically the time when we see his neutrophil count really fall.   Right on schedule, his neutrophil count is down to 30....yep, 30.  As a quick reminder, anything over 1500 is considered normal for the most part.  Severe neutropenia (and the associated risk of infection) starts at anything below 500.  The lowest measured value we saw for the first two cycles was in the mid 200s, so 30 is a new low for us.  Needless to say, Katie and I are both a little more nervous than usual.   But, he is still a happy, rambunctious 16 month old, filled with too much energy for us to be too sad or worried.

World Rb Awareness continues on and we have two videos.  The first is Dr. Carol Shields (Edward's ocular oncologist) at Wills Eye in Philadelphia discussing a brief overview of retinoblastoma.  The second video is a link (I could not figure out a way to embed it) to Dr. Shields discussing the management of retinoblastoma.  This video is a bit more technical, but has a lot of good information. 

Management of Retinoblastoma

Wednesday, May 16, 2012

World Rb Awareness Week

This week marks World Retinoblastoma Awareness Week, an effort spearheaded by Daisy's Eye Cancer Fund, an organization dedicated raising awareness for pediatric eye cancers and providing resources to those affected.

Needless to say, Katie and I are excited to participate in raising awareness to this issue in any way we can.  To this end, we plan to have several posts this week dedicated to discussing Rb in addition to our regular updates on Edward.  We also plan to slightly change the structure of the blog in the next few days, adding a FAQ section on retinoblastoma.

Today's video post follows an adorable little boy from Greenville, South Carolina who makes monthly trips up to Philadelphia to see Dr. Shields at Wills Eye and Dr. Leahey at CHOP.  The story sounds strangely familiar.... Enjoy!

Sunday, May 13, 2012

Mother's Day WEEKEND

I kept reminding Michael that we were celebrating Mother's Day WEEKEND, as if Sunday was not enough, we had to emphasize my special day for two whole days :)  I was just nagging him and being a brat, but we really did have a nice WEEKEND together, which is a rarity caused by Michael's resident work schedule.  This month Michael is on an outpatient month (dermatology) which only requires him to work 8:00 am to 5:00pm (dare a say it.....normal hours?) and he has weekends OFF!  This means that I get extra help with Edward's bedtime routine and weekends are actually more relaxing, for the BOTH of us.  So thanks to the science dedicated to skin and its diseases, we had a family weekend! Woo hoo!  Michael tried to say that it was Mother's Day everyday, but, nice try Mike..........  We had a picnic, saw lots of friends, went for a few walks, spent many hours outdoors, ate frozen yogurt, grilled out, and napped..... all of us!  Happy Mother's Day WEEKEND to all of the hardworking mommas out there, keep up the good work!

          Edward and I, just after we devoured our  vanilla yogurt!
My most special gift from Daddy and Eddie, secretly made.  Since we are not supposed to have fresh flowers because of Eddie and chemotherapy, Daddy remembered that I love, love, love yellow tulips, and Edward completed his 1st finger painting session for a nice touch!
Wearing his new outfit from Aunt Kelly! Stylish as always :) I swear, I have yet to purchase this child an outfit of my own.  We keep getting presents in the mail!  Edward does not yet know how loved he is :)
Trying to eat cheerios off of the ground, while I have a whole container in my hand.
                                                                      Loving his car!
                             Michael and Eddie, my two favorite gentlemen.  I am one lucky lady!

Tuesday, May 8, 2012

Cycle 3....Complete!

Edward had a CBC on Thursday morning and his neutrophil count was a robust 1064, over 300 above the 750 required to receive his next cycle.  He was subsequently admitted to the pediatric heme-onc floor and received his half of cycle three.  Having the PICC line makes the delivery of the chemo a little easier as we do not have to worry with peripheral IVs.  However, he is tethered to an IV pole regardless of his venous access and keeping a 15 month old whose only goal is explore every nook and cranny of whatever room in which he may be, remains a challenge during these admissions.

To distract Edward, we snagged a toy 'car' that he could sit in and we could push around the halls of the hospital.  We unplugged his IV pole and off we went!  Edward absolutely loved it and had a big smile on his face the entire time, charming each passerby with a devilish grin or enthusiastic whole arm wave.  Needless to say, we repeated these walks around the halls as often as we could to pass the time.

Edward tolerated the first day of infusions well and he spent the night in the hospital with Katie.  The following morning, he received his second infusion of etoposide which he also tolerated well.  After an hour of observation, he was discharged back home!  The first thing Grammy Schuster did when we got home was buy him one of those cars!  Edward 'helped' me put it together, had a great time playing with the box in which it came and eventually realized that we now had a car of our own and started pushing it around our living room.  Since Friday, our walks in the stroller have been replaced with walks in the blue car.  The car is tricked out with a working horn, a front hood compartment for his toys and a cup-holder that does not quite fit a sippy cup, but is great for storing cheerios.

As previously mentioned in the last post, Katie's little sister Nicole had a fundraising party last week in Boston and it was a raging success on all fronts.  She actually raised over $7,000 for Edward's treatment in just one night!  Thank you Nicole and thank you Boston College!!  From the looks of the pictures, it seems that everyone seem to have a great time in honor of Edward.

Wednesday, May 2, 2012

Cycle 3!......in the works.

Katie and Edward made the trip up to Philadelphia this week and had their appointment today at Wills Eye for an eye exam under anesthesia (EUA).  These trips are starting to become routine to some degree, though still can be challenging.  Edward cannot have any formula after midnight on these days and often wakes up pretty ravenous.  Luckily, Katie has that magic motherly touch that somehow consoles a hungry baby without actually giving him a bottle.

Today's EUA revealed that both tumors regressed again!!  The changes were not dramatic, but any amount of regression is absolutely fantastic news.  Dr. Shields, our ocular oncologist at Shields, delivered 'thermotherapy' to Edward's tumors - a type of local therapy that helps control the growth of the tumors.  Edward, Katie and Grammy Schuster then made a furious trek back to Virginia so we can get chemotherapy tomorrow at UVA.  We first have to get a CBC to check his neutrophil count.  Hopefully, we will above 750 and can keep moving forward!

We have to give a big shout out to Nicole, Katie's little sister and her Boston College colleagues.  Nicole is organizing a fundraiser event tomorrow evening to help defray the costs of Edward's treatment.  Nicole, a star athlete on the BC hockey team (all ACC!) has put together quite the party.  Over 300 tickets have been sold for her event.  In fact, her fundraiser was featured on a local Boston College blog!  So if any of you are in Boston tomorrow night, please join Nicole and her friends at The Place on Broad Street.  Thank you in advance to all the support and generosity of the BC family.  I am not sure if Nicole told any of them that Katie and I are ND grads, but we both agreed that we could be Superfans, at least for a short while.  Go Eagles!

Here are some pictures of Edward with an awesome cape that Edward's Aunt Lisa and Uncle Patrick made for him!

Tuesday, May 1, 2012


" Life moves pretty fast. If you don't stop and look around once in a while, you could miss it."
                                                                  - F.B.

Two months ago to the day, Katie and I received the news of Edward's most recent challenge and the past 60 days have been a rollercoaster of emotions and an absolute whirlwind of events.  However, with the help and support of all of our family and friends (and an occasional cold beer at the end of the day), we have seemed to weathered the first part of the storm.

Since the completion of his second cycle, we have been quite busy to say the least.   Edward has continued to amaze us with his strength and resiliency.  Despite the chemo, Edward keeps charging forward both literally and figuratively.  He loves to stand, crawl, play and explore; he is also very close to walking on his own.  With all of this adventure, he seems to acquire a new bump or bruise every few days, though he seems unfazed by it all. 

Edward continues to have his weekly CBC drawn to monitor his blood counts, most specifically his neutrophil count.  We need 750 neutrophils to move forward with each cycle.  He just barely made counts for his second cycle and we have been quite nervous about making counts for this next one.  The effects of the chemotherapy on the bone marrow can be cumulative as he continues to receive his treatments, thus potentially leading to a more profound suppression of his cell lines.

One aspect of Edward's care that has proved somewhat challenging has been insurance.  We were referred to both Wills Eye and CHOP because they are two of the best centers in the world in treating retinoblastoma.  We knew that Wills Eye did not take our insurance, but we under the impression that CHOP did.  Unfortunately, during cycle 2, CHOP informed us that they actually did not take our insurance.  Bummer.

After much prayer, consideration and multiple discussions with both of our oncologists and ocular oncologist, we elected to continue to receive care at Wills Eye, but transition our chemotherapy back to UVA.  Both the UVA peds-oncology and ophthalmology departments are excellent, but they just do not see a lot of retinoblastoma due to its rarity.  But the treatment regimen of vincristine/etoposide/carboplatin is the same at both locations, so we both felt very comfortable getting those infusions here as did our oncologists. 

One of the bigger differences between CHOP and UVA is that UVA prefers to establish 'central access' in their chemotherapy patients - i.e. they like to place a permanent type of IV that allows them to deliver the chemo to the larger veins in Edward's circulation.  Thus, Edward had to have a PICC line placed (PICC = peripherally inserted central catheter).  The PICC line is a small thin rubber tube that enters the inside aspect of his right bicep and then courses up his arm and into his chest.  He yet again had to go under general anesthesia to have this placed as keeping a 1-year-old still for this type of procedure is impossible. 

The PICC line has its advantages and disadvantages for sure.  The good news is that as long as it is working, Edward will not have to have any needle sticks for blood draws or infusions.  Considering how many IVs he has already had and how much he hates his weekly lab draws, this is a huge plus.  On the other hand, keeping an external catheter clean, dry and intact with a curious, mile-a-minute 1-year-old is a unique challenge in and of itself.  Additionally, we have to 'flush' the line everyday with saline (salt water) and heparin (an anticoagulant) to help keep the line patent.  Again, not exactly a cake-walk with a squirmy baby.   But the worst part is bath time.  Previously, a highlight of the day for the whole Johnson family, bath time is now a quick angst-ridden 3-4 minutes of trying to clean Edward while keeping a saran-wrapped-around-a-wash-cloth-over-the-PICC line dry!  Whew. 

Another disadvantage to the PICC line is its associated risk of infection.  Anytime anything breaks the barrier of the skin and enters your bloodstream, you are at risk of developing a fairly serious infection.  Throw in the fact that Edward is neutropenic (immunosuppressed) and the risk is quite high (though thankfully, still unlikely).  Meticulous attention to keeping the PICC line clean and using good hand hygiene on our part will minimize Edward's risk, but certainly not bring it to zero.  Should he develop a fever, he would likely need to have the PICC line pulled and another inserted. 

On the bright side, Edward got to visit with the Johnson side of the family last week, as my little sister got married.  He loved all seeing his grandparents, all of his aunts and uncles and meeting many new cousins.   He certainly does not shy away from attention at this point in his life!

So, how are those neutrophils?  Well, last week we had our weekly lab draw and unfortunately, his neutrophil counts was even lower than the previous week - all the way down to just above 200.  We were scheduled to have his eye exam tomorrow at Wills Eye under anesthesia followed by a furious trip back to Virginia to get chemotherapy the following day, but we were afraid we were going to have to delay by a whole week based on his low neutrophil count.  Our oncologist decided to check another CBC yesterday just to see if by chance they were above 750 and we could keep moving forward.

No luck.  650.

But they were higher....much higher!  In fact, after some deliberation, they allowed Katie, Edward and her mom to head back up to Philly to have his treatment tomorrow with the hopes that by Thursday, his counts will be above 750.  Considering that his counts almost tripled in three days, we are hopeful that we can gather a measly 100 more neutrophils by Thursday.  We will let you know how it goes!