About

Our 26 month old son, Charlie “Crash” Pageler, was diagnosed with Pleuropulmonary Blastoma (PPB) cancer on June 5, 2019 at Yale New Haven Children’s Hospital in Connecticut.

This is the story of how we got to diagnosis:

Towards the end of May, Ami ventured up to Boston along with the boys Crash (2) and Jack (3 months) for her niece’s graduation. During that weekend, Crash got sick with a fever which resulted in a facial rash of sorts called petechiae.

The fever soon broke and Ami returned home for Jack’s 3 month pediatric check-up but scheduled Crash in as well because the facial rash seemed odd and Crash - usually a very good eater - was not really eating much and seemed much more tired than usual. It was determined that it was likely a virus that had played itself out but that we should keep an eye on him. A few days later we traveled to Seattle for Memorial Day and while Crash was his usual annoying self on the plane he actually seemed better than he’d been with me in the past. When we arrived in Seattle Ami found herself coming down with a short bout of viral conjunctivitis, which seemed to confirm the theory of Charlie previously having a quick viral infection.

We proceeded through the Memorial Day weekend with Charlie still not much interested in food (though milk was ok) and being much more tired than usual - but we had time zones to consider, a very active social life, and other factors that didn’t cause us to have alarm. That began to change when we left our weekend event and headed back to Seattle. He began to complain of pain when we picked him up, he was a bit feverish and he just seemed “off” to Ami. I was fairly unsympathetic but Ami was insistent and we’d never heard him complain of pain before (frankly, I didn’t know that he had the words for it). When we got back to Seattle, his fever spiked - 104* and Ami and my father took Crash up to the urgent care center at Seattle Children’s Hospital - one of the best pediatric facilities in the country. After a quick check up and a listen to the lungs, the Dr suggested Tylenol and monitoring but cleared us to fly back home to the East Coast the following morning.

Crash slept for most of the flight but Ami still felt something was off so when we landed that evening Ami secured an appointment for Crash at his pediatrician’s first thing the next morning. That night, I filmed how Crash was breathing as it seemed fairly rapid and labored. I don’t often join for his pediatric visits but this somehow seemed a bit different. The check up was fairly routine until our brilliant pediatrician brought out her stethoscope. It was clear that she was concerned. After an especially long listening exam she turned and said something to one of her nurses who quickly ran off and the entire atmosphere at the center changed in that moment. Our pediatrician then explained to us that we should remain calm but that we needed to go to the local hospital and that despite its being less than a mile away that we were going to have to travel by ambulance. In the meantime they need to put Crash on oxygen. WTF!! Crash was fine and had just walked in under his own power - complete with playing with the elevator buttons - 20 minutes ago !!

So Crash gets his first ambulance ride and I get to ride in the back with him, albeit for a total of three minutes (though I am looking forward to seeing what that three minutes will cost). We were brought straight into the emergency room and Crash was up and functional and playing with all the equipment so the staff - including the attendings - just sort of looked at us like - um, what are you doing here and why all the fuss. They did a routine exam and were like yeah, he’s breathing a bit fast and his blood oxygen levels are a bit low but really (?!?), bringing him here to the emergency room, in an ambulance no less. And we didn’t necessarily disagree - he seemed perfectly fine.

Well, that was before our pediatrician finally got the Drs on the phone and browbeat them into giving Crash a chest X-Ray. The image came back fairly quickly and showed a substantial white area where his right lung would be.

This was exciting - though not concerning - to the emergency room physicians. So much so that they insisted we come over and take a look at it and even take a picture of it with our phones - as a fun sort of keepsake earmarking this large pneumonia we had. “Not to worry, we will give him some IV antibiotics, maybe even hold him overnight, and all will be fine.”

So we move from emergency up to pediatrics and we are just starting to get squared away when the hospital pediatrician comes in and gives us a download. Seems like a pneumonia, but a fairly impressive one and given where we’ve been (Pacific Northwest), how he is presenting (pretty much normal function though now very labored breathing) and our brief history (viral infection followed by now this fairly rapidly developing bacterial infection) it might be best to send us up to Yale New Haven Children’s hospital - by ambulance. Uh, really?!?

By this point we’d talked (and shared our keepsake photo of the x-ray) to several of our friends in the medical community and were starting to feel that being at a Children’s Hospital was perhaps more appropriate than being at our local community hospital. And there was something that the pediatrician told us that was sticking with us. She relayed to us the conversation she had with the radiologist who gave the read of Crash’s X-Ray. The radiologist indicated that it was an impressive (3rd time we’d heard that word impressive so far) scan and that he’d like to have someone follow up with him to let him know how our case turned out. Ummm, ok…

So we now head up to Yale - me in the front of the ambulance, Crash in the back no doubt wondering WTF is going on, and mom following in a car probably panicked out of her head. Her cousin, an infectious disease specialist out of John Hopkins, was frantically researching rare diseases of the Pacific Northwest.

Once at Yale Children’s Hospital, we had basically the same experience we had at our local hospital. Kid seems fine… labored breathing and diminished air passing through his right lung but other than that presents fairly well. We started to get concerned that they may even release us until Ami asked if they had seen the X-Ray. They had not as they were having trouble uploading the CD, so Ami offered them her phone with the image. Again, things changed fairly dramatically at that point.

We were immediately scheduled for a chest X-Ray and they quickly ordered up a concentrated set of IV antibiotics. After the confirming X-Ray which indicated a lot of fluid in the outside lining of the lung, the working diagnosis was an aggressive bacterial pneumonia that had followed his earlier viral infection (which had weakened his immune response). It all made logical sense but there were still some things that seemed odd. For example, if he had such a bad pneumonia why was he not presenting as much more ill.

This was a dark time for us. Sleepless nights were spent nervously watching the vitals monitor to ensure his blood ox didn’t drop below 90 and that his heart rate didn’t go above 170 or that his breathing, which was erratic and very labored at this point didn’t exceed one breath a second. In looking through our photos I realized that we only took pictures of the good screens.

Given the increasingly labored breathing and lowering blood oxygen levels we were quickly scheduled to have a procedure in which a tube would be put into the outside lining of the lung (the pleural) in order to drain the infected fluid and relieve some of the pressure on the lung. This fluid would be tested to see if we could identify what sort of bacteria was causing the infection though considering that he had already been on antibiotics it was unclear as to whether we’d be able to grow any in culture - though if it were a bacteria that we weren’t treating for presumably that would have shown up.

They drained a lot of fluid out of him and left a tube in him to continue the draining. When the Dr who performed the procedure called to let us know the Crash was being transferred to the ICU I had learned enough to ask him a few questions - specifically, how much fluid had he gotten and whether or not it looked infected. He reluctantly let me know that there was a lot and more importantly that it was bloodier than he would have expected and that it didn’t present as infectious fluid often does.

It became clear that this was a bit more info than the Drs overseeing the totality of our care were ready to share with us but it was also clear that Crash’s case was drawing interest from more and more specialists across the hospital, as evidenced by the sheer numbers of visits he was receiving from an ever diversifying group of medical staff. The buzz in the air slightly annoyed Ami but to me it meant that there was collective brainpower trying to figure this out.

Still, the working assumption was a particularly aggressive bacterial pneumonia - and the balance of the facts still pointed to that - but more tests were needed. Crash meanwhile is being a trooper and is charming the pants off of the hospital staff. As he is being poked, prodded, and otherwise tortured, he always managed so say please - even after screaming for the staff to stop it! His “Stop it, please” became a constant refrain among the nursing staff and his “thank you” when they would tell him they were done hurting him was a big hit as well. And his activity level was charming as well, whether dunking a basketball in the hospital playroom as we wheel the IV pole and the pleural drain bag around after him, or happily painting in his hospital bed.

Given the lack of response to the anti-bacterial as well as the presence of blood in the pleural, an MRI is ordered. The results are fairly obvious. He has a mass estimated to be 8cm X 10cm X 12cm occupying at least the lower lobe of his right lung (the right lung has three lobes, the left only two - one less to make room for the heart). We weren’t told the dimensions at this point (because it is huge) but we were told that they needed a biopsy to determine whether it was cancerous. They biopsied the next day and promptly came back with a cancer diagnosis. Specifically, Pleuralpulmonary Blastoma, or PPB.

While not the worst of the potential diagnoses - there was a time where we were not sure we’d ever get to bring Crash back home - this was fairly devastating news. This happy, healthy, active, engaging little two year old boy who was just learning to express himself with vocabulary he’d clearly been storing up in his brain for many months had cancer. A cancer so rare that Yale has only ever seen four and fewer than 1000 cases have ever been documented. Initial internet scans were quite dire but further investigation gives us great hope. Treatment has come a long way in the past decade and the treatment protocol can be quite successful. And there is not much - if any - debate on what that protocol should be so it thankfully takes the having to make a choice out of the equation (at least for now).

Once it was determined that it was likely PPB, they quickly did scans of his brain, abdomen, heart, and bones to see if it had spread. Thankfully it had not, though in cases of PPB it seems that it seldom does. So diagnosis comes in on a Wednesday night and chemo starts on Friday. Things are moving fast and there is a ton of attention being paid to Crash. We are moved to the oncology ward, which was sad to us because we loved the nursing staff in the pediatrics ward, but they were close by and came frequently to visit. Crash’s “pleases” and “thank-yous” were becoming less frequent now as he was clearly over this place, tired of seeing the concerned looks on mommy and daddy’s faces, and all too done with being relentlessly poked, prodded, drugged, etc. However, the plan was set - 4 rounds of intensive 3-day inpatient chemo every 3 weeks accompanied by weekly single dose outpatient visits with occasional blood count days thrown in. After the first 2-4 rounds a decision will be made as to whether the tumor has shrunk enough to extract it and then after a successful extraction another 32 weeks of a slightly less severe chemo regime. But the good news was that after a very long 12 days in the hospital we were finally going to be able to go home, albeit with a PICC line dragging off of his arm.

Ah, but home. In our own beds, with our own toys, eating our own food. What a great thing to look forward to. Just get through this fairly intensive first round of chemo and we can get out of here. And while the chemo was fairly intense (one of the drugs called doxo requires you to wear gloves while changing diapers every two hours in order to avoid burning your skin), Charlie only got sick once and was generally only slightly off during the treatment.

Driving home was a bit surreal, the whirlwind of the past 12 days (and the reality of the next 11 months if not much, much longer) was finally starting to sink in. So it was with much emotion that we walked into a small surprise welcome home party that our neighbors threw for us as Charlie walked into our home. We weren’t very successful at holding back the tears.

So that is the story of how we got to diagnosis. Which given the rarity of the disease and how Charlie presented through all of it, seems to us to have been done in record time (essentially 6 days) - in large part due to the awesomeness and attentiveness of our local pediatrician who no matter what we will always owe a tremendous debt to. (There are awesome sidebar stories of her tracking down Yale Oncologists at conferences demanding updates and advocating for us). And despite some tongue in cheek references to our stint at our local hospital, I am entirely satisfied with what happened there - and frankly at every turn in our health care experience thus far - with the possible exception of the Seattle Children’s Hospital urgent care doc missing the fact that there was no air passing through Charlie’s lower right lung. But even that I think is understandable and certainly not something I am not angry about. And the care we got at Yale - particularly by the nursing staff and other support functions - was beyond any expectation we could have had.

And on that front, the amount of support, well wishes, genuine offers of help, etc that we have gotten from so many has been overwhelming. Truly quite humbling. So thank you to all of you. Crash may have a long road ahead of him, but he will get through this and it will be in no small part due to the community of support he has.