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!

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.

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!

Grace!

" 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!

Rainy Days

Rainy days make us a little stir crazy in this house.  When it rains it means no walks for me, Winston, or Eddie.  Thus, my two darling boys drive each other (and me) crazy!



Solution:  What do you do on a rainy day and you can't swim outside?  You have an indoor pool party of course!  Edward's physical therapist suggested I fill the pool with toys and pillows, so I took her suggestion a bit further....

                   "Mom, you are the coolest!" ( What I imagine he is saying to me)

                                                    Rolling around...... a.k.a. "splashing"

                          Wait, Mom, it is not nap time yet, I just have to get this one thing......

                                          I almost got it, I promise Mom, almost............

                                                                 GOT IT!!!!!!!!

                                                 I just wanted my football Mommy.....

BEST. IDEA. EVER.  Thank you Jen! Our "pool party" kept him busy for a long time.  I was even going to put his swimsuit on, and then I thought, "Okay crazy, calm yourself."  It is bad enough that I put his "friends"/ stuffed animals all around the pool.  Not exactly sure who had more fun yesterday......

Today I took Edward to the hospital for his 9:00 a.m. appointment for his CBC.  Sometimes, Mike can slip away from work and join me for this visit so I was not surprised when Michael's smirk greeted us as the elevator doors opened on the 4th floor only to tell me I was 6 minutes late.  Ahhhhhh. Punctuality..... is for the birds these days.... only 6 minutes late after a total bottle refusal and poopie diaper just as we were walking out the door?  I would call that a raging success.  His neutrophil count ended up being really low, 250.  We still have a week and a half for those darn neutrophils to rise up above 750, but it does mean that he is extremely susceptible to any infection, because the chemotherapy is doing its job but wiping out his immune system as well.

I realized on the way home from the hospital that we were out of coffee, and so I decided that since I already had Edward's entire breakfast and milk packed with me, we were going to Starbuck's for a breakfast date! I had my coffee and muffin and Edward sat happily in his stroller munching on about 100 cheerios.  I started to feed him his yogurt and this weird, creepy older man in his workout clothes/jumpsuit leaned over and said to me, " I wish someone would spoon feed me breakfast."  Ha... ha... ha... I awkwardly responded and looked away...and thought to myself, "Quick, exit stage left, let's get the heck out of here Eddie!" And I laughed all the way home thinking, no matter how serious, how stressful, or how awful life might seem at times, there are ALWAYS times for laughter!  WHAT A CREEP.  Edward and I are NEVER having a breakfast date at Starbuck's again.  Our front porch is just fine :)

Cycle 2 - Complete!

Our resident tough guy, Edward, has successfully completed his second cycle of chemotherapy!  I unfortunately had to work and thus, Katie and Edward headed up to Philadelphia without me to check cycle 2 off the list.  They actually spent the whole week and Easter weekend up in Philadelphia with Katie's family. 

Edward first had his EUA on Wednesday at the Wills Eye Institute to check on the progress of the tumors.  After only the first cycle, we were hopeful that we would see the tumors were the same size and definitely not bigger or with new signs of vitreous or retinal seeding  (which are kind of markers of tumor growth).   Yet again, we had another Easter miracle as both tumors were actually smaller!!!!  Our opthalmalogist was very pleased with what she saw and remains hopeful that we can preserve his vision in his right eye.  The vision in his left eye will almost certainly be compromised no matter how successful our treatments are, so the goal is to avoid enucleation of this eye and preserve whatever vision he may have left in this eye.

Following a long but successful morning at Wills Eye, Katie, Edward and Grammy Schuster made their way to CHOP for chemo.  The first few hours of the infusions went well without any serious breathholding or passing out!  They spent the night and received the second infusion of etoposide Thursday morning.  All in all, a very successful trip.  The only bad news is that we learned during this past visit that CHOP does not take our insurance.  We knew Wills Eye did NOT and planned to appeal the 'out-of-network' rate to our insurance company due to medical necessity.  However, we were first told that CHOP did take our insurance, only to find out this past week that they do NOT!  So if any of you actually won that mega millions a few weeks ago, expect an email from us...haha! In all seriousness, because of this change, we may need to receive the rest of our chemotherapy at UVA.  Decisions, decisions.....

Katie and Edward then spent Easter weekend knee-deep in chocolate bunnies (for Edward) and jelly beans (for Katie).  Enjoy the pictures below!  We are now all back together safely in Charlottesville and have a CBC check planned for this Thursday at UVA.

The Road Ahead

Edward completed his first cycle of chemotherapy during the first week of March and since that time, Katie, Edward and I have essentially been living in blissful (and willful) ignorance.  I have to admit that aside for some thinning hair and a somewhat finicky appetite, Edward seems to be his happy, healthy self as detailed in Katie's post below.  Since the first cycle, our only required follow-up has been a weekly blood draw to check his complete blood count (CBC).  This lab test measures his white blood cell count, hemoglobin/hematocrit and his platelet count.  A well-known side effect of chemotherapy is the lowering of these counts and monitoring these levels is part of the process.

The most important of these lab values is his absolute neutrophil count (abbreviated ANC).  Neutrophils are a type of white blood cell that are key in fighting off infections, particularly bacterial and fungal infections.  When one's neutrophils are low (specifically less than 500), you are considered neutropenic and any infection has the potential to be life-threatening.  For example, if he gets a fever when he is neutropenic, this almost assuredly leads to an immediate admission to the hospital for blood and urine cultures, broad-spectrum antibiotics and close observation.

Edward actually suffered from neutropenia of prematurity while in the NICU.  His bone marrow was too immature to produce enough neutrophils early in his stay.  Fortunately, aside from one infection during week two of life, his neutropenia at that time resolved without any lasting consequence.  Before starting chemotherapy, his baseline blood work revealed an ANC of ~1200, which is technically considered neutropenic.  Normal is anything above 1500, mild neutropenia is 1000-1500, moderate neutropenia is 500-1000 and severe neutropenia is less than 500.  Your neutrophil count can bounce around a little bit even in healthy patients, but it should almost always be above 1000 or so.  His ANC of 1200 before chemotherapy was possibly secondary to a mild cold he had earlier that week - sometimes viral upper respiratory tract infections can drive the neutrophil count a little low.  We had repeat labs at CHOP in Philadelphia and his ANC was up to around 3000, which is great.

In addition to the associated severe infection risk, a neutrophil count of less than 750 prevents him from getting his next cycle of chemotherapy.  We have to allow his neutrophil count to recover before delivering each cycle to avoid unnecessarily prolonged periods of neutropenia.  So each week, we head to the oncologist office for a finger stick to check his counts.  Not surprisingly, Edward does not enjoy these visits all that much.  One week after his first cycle, his count was down to 780 and the following week it was down to 340, landing him in that severe neutropenia category.  We had to be vigilant for any signs of infection, no matter how subtle.  Last Tuesday, we had another blood draw, hoping that his counts were going to start showing signs of recovery as his next cycle of chemotherapy is due on this Wednesday.  But, of course, with our luck, his ANC was even lower at 290.

Through discussions with our oncologists, we decided to repeat the CBC on Friday to see if it was at least trending up.  If still going down, we would likely have to delay his next treatment.  Though it has been nice to have Edward at home with us and doing so well, we also want to get this whole process behind us and are anxious to get cycle two out of the way.  So Friday morning, we returned to recheck his counts.  My parents were in town this weekend and accompanied us to the doctor's office.  They weigh him and check his vital signs at the start of each visit.  This is the point where Edward remembers that last visit and begins to cry and fuss.  We then have an opportunity to calm him down only to have a spring-loaded lancet pierce the pad of one of his poor fingers.  It requires both Katie and I to hold Edward down while the nurse carefully 'milks' the blood into laboratory vial.  Katie and I were each fairly certain that his counts were going to be low again and delaying the upcoming cycle was inevitable - nevertheless, we held out a small ray sliver of hope, just in case.

A couple long hours later, I actually called the lab to get the result......880!!!!  We 'made counts', as they say!  We were all overjoyed and kind of surprised how excited you could get about being able to give your child chemotherapy.  He has a minimum of 6 cycles, each one month apart.  The effect of chemotherapy on the bone marrow can be cumulative, so at some point, we will likely to have to delay a few of the cycles by a week or two, but at least we will be able to get cycle 2 out of the way.  Katie and Edward are headed up to Philadelphia tomorrow (I have to work for this cycle, unfortunately).  He will have another EUA on Wednesday at Wills Eye to check on the progression of the tumors followed by his chemo infusions delivered at CHOP. 

Cycle two, here we come.