Part Three

After a long day last Wednesday, we somehow managed to have an even longer day on Thursday.  Edward started with another EUA (exam under anesthesia) at Wills Eye early that morning.  He is such a tough little boy, but he always wakes up pretty cranky from anesthesia (understandably so).  The exam part of the EUA only takes 20-30 minutes, but the whole process is a several hour ordeal.   Similar to his prior exams, this EUA confirmed the Group B tumor in his right eye and a Group C tumor in his left eye.  Unlike his prior exams, he actually received some local cryotherapy to the blood vessels supplying his retina during this EUA.  Anatomically, there are a few places in the body that are considered 'sterile' and are separated from the rest of body with a special layer of cells connected with 'tight junctions'.  The most well known of these spaces in the brain which is surrounded by the blood-brain barrier which, for example, helps protect against meningitis.  Similarly, the blood-retinal barrier exists and helps keep infections out of the eye as orbital infections can quickly lead to vision loss and are considered ocular emergencies.  Anyway, the cryotherapy that Edward received temporarily disrupted this blood-retinal barrier and actually allows the chemotherapy to penetrate into the eye more effectively and in higher concentrations (at least in animal models - poor rabbits). 

Thus, immediately following our appointment at Wills, we crossed the Schuylkill (obviously pronounced /sku-kel/) River in downtown Philly to reach the Children's Hospital of Philadelphia (CHOP), one of the best pediatric hospitals in the country.  There, we met a new pediatric oncologist who is also considered one of the experts in the field of retinoblastoma.  We again discussed his diagnosis, its implications and treatment options.  After hearing all of the risks and benefits of the proposed chemotherapy regimen of vincristine, etoposide and carboplatin, Katie and I quickly found ourselves signing consent to deliver these agents.  These drugs are given over 2 day period roughly every 4 weeks for a minimum of 6 cycles: the first day consists of all three drugs and the second day is only a 1 hour infusion of the etoposide.

After a quick tour of the facilities, we were brought to the Day Hospital - a place where children can receive their chemo infusions as outpatients instead of inpatients.  He still had his IV from the EUA at Wills earlier that day and he was first pre-medicated with dexamethasone and zofran - two agents that can help with chemo-associated nausea.  Then came a quick infusion of vincristine over 20 minutes or so, which was followed by an hour long infusion of the etoposide.  At this point in the day (around 6pm), Edward was essentially beside himself.   He had undergone so much that day already - an early morning commute to Wills Eye with no morning bottle, the EUA, cryotherapy, anesthesia, oncology appointment, IVs and now chemo.  The actual infusion of the chemotherapy is not that bad, but keeping a 1 year old happy and calm while tethered to an IV pole for a few hours is pretty tough.  Regardless, we were close to the end, with only 1 hour of carboplatin left.  The infusion ended a little after 7pm and we figured we could be home by 8:30pm or so for a well deserved warm bottle for Edward and a couple cold bottles for Katie and me.

CHOP is planning to manage his chemotherapy without a central line and instead use peripheral IVs.  The central line prevents repeated needle sticks, but is a significant nidus of infection, especially as one's immune system is depleted by the chemo.  Thus, we were trying to 'save' Edward's peripheral IV that night to use the following morning for one more infusion of etoposide the next day.  The nurses were showing me how to flush the IV with heparin and saline the next morning to maintain its patency while Katie was packing up our bags, when Edward decided that he had had enough.  He started to cry and fuss, pulling his foot away from the flushing of the IV.  He then let out a ear-splitting wail, but then went eerily silent - the dreaded breathhold!  Tears were still streaming down his cheeks and his facial expression was locked into one of complete agony, but there were no audible cries and more importantly, there was no breathing.  The nurses started coaxing Edward, a hint of urgency in their voice and Katie started blowing in his face, a well-known trick to abort the breathhold.  But Edward would not relent.  He started to turn a little dusky around the mouth at this point, so I scooped him up, firmly rubbing the center of his chest and now screaming his name.  Still nothing.  His color was now a dark shade of purple and he actually went completely limp.  The nurses screamed for assistance and fortunately, an attending physician was literally a few feet from our door and was bedside immediately.  I laid Edward down on the hospital bed and still nothing - purple seemed to be fading into gray.  The attending physican calmly, yet purposefully began her assessement, immediately performing a chin thrust to open up his airway while the accompanying nurses instinctively set-up 'blow-by' oxygen to supplement his oxygen intake.  I looked at Katie who was a different shade of gray herself with tears filling both her eyes - could this really be happening both of us were thinking? 

By the grace of God, with the simple chin thrust, Edward finally took a breath and his colored instantaneously improved.  His hands were up by his face, locked in fists.  Despite his breathing, he was still not moving.  The nurses and doctors hooked him up to multiple monitors, checking his heart rate, blood pressure and oxygen levels.  Finally, after what seemed like hours, but was likely on the order of 2-3 minutes, Edward began to stir and even opened his eyes.  He let out a defeated whimper that still breaks my heart when I think about it.  But he seemed to be 'back' - Katie and I finally took our own deep breaths and the weight of the world seemed to move slightly off our shoulders.  The doctor began explaining everything to us and told us that clearly we needed to be admitted for observation that night.  They tried to directly admit us to the hospital, but there were no beds immediately available.  So instead, we were re-rerouted to the Emergency Department.  Four long hours later, we eventually arrived in our room, exhausted and hungry, but happy to have our sweet little boy with us and seemingly OK.   

This post is already entirely too long and fortunately, the rest of our night was uneventful.  Edward was able to receive his second infusion of etoposide without any difficulty and we were finally discharged home late Friday afternoon.  We spent the weekend with Katie's immediate and extended family who spoiled all three of us rotten.  It was a long, but successful week - I just hope to never go through all of that again.