Addison, Part VI

It wasn't until I returned from my honeymoon that I found out the truth about Addison's mysterious "enhancement."

Somehow (miraculously), I hardly thought about Addison during the two weeks we spent vacationing as newlyweds in Florida and the Bahamas. It wasn't until lunch, a few days after I returned to work in late May, that it suddenly dawned on me that she was no longer on the hospital service. And that I had no idea what had happened to her. I quickly inquired of my colleagues, with much interest, as to the answer. 

Addison only worsened the week I'd left. Her pain became so uncontrollable that yet another MRI was done, which this time finally confirmed what I felt like we had all really known for weeks: the ATRT had spread everywhere, all over Addy's spinal cord and even up into her brain. Ever the optimist, our brain tumor doctor insisted it be biopsied to make a definitive diagnosis. The surgical biopsy was definitive. 

And this is the sad part, the ugly part, the part I have such a hard time sharing: what came next.

Most of the time, I think pediatric oncology is really good at recognizing when to offer families palliative care. If there is even a 10-20% chance of survival, we usually offer them something- chemotherapy, surgery, what have you- in order to prolong the amount of time they feel good, even if we know there is only a small chance it is actually going to cure their cancer. Hey, most people take a 10% chance over nothing. Especially if the side effects are not significant and the time they can spend making good memories with family and friends is prolonged.

However, somewhere in there, when the chances of cure keep going down, a gray area is entered. This is the case for anyone with an aggressive or advanced form of cancer, particularly in relapse. A decision has to be made about whether to continue therapy that may or may not prolong life, especially if there is a chance that therapy could decrease the patient's quality of life. Nausea, vomiting, and body aches may no longer be worth the trade if the tumor is not responding.

And in these sometimes murky waters, infrequently doctors can cause more harm than good.

Sadly, it happened to Addison.

Addison: Part V

You see, spinal cord tumor relapses and radiation necrosis are treated very differently.

In the case of radiation necrosis, you would give a patient oral or IV steroids to decrease inflammation (hopefully decreasing compression around the spinal cord) and give symptomatic support. 

But for tumor relapses, well- the only options are chemotherapy, surgical resection and/or more radiation. 

So for radiation necrosis, you obviously wouldn't re-irradiate. But you wouldn't give steroids for a growing tumor, either. 

It saddened my heart so much that April, knowing that with the information we had, we did not know for sure. Looking back, it still makes me sad that we didn't figure it out. And as more time passed, sadly, Addison lost more and more function. By the end of April, she was completely paralyzed below the waist again and was unable to have anyone touch or move her legs without excruciating pain. She had also lost complete control of all her bowel and bladder faculties. 

Taking care of Addison daily while watching her slowly lose most of the neurologic function below her waist was unlike any experience I have ever had. It was unspeakable, really. I cannot describe how horrible it was- for Addison, for her parents, and for everyone who loved her.  There was such pain in my heart every morning, going in to see her- knowing that something else very real and precious might be gone. And upon arrival, often was.

It was then, the beginning of May, that some of us taking care of Addy began to really argue for tumor relapse. Treating her for necrosis obviously had not worked; at the very least, a few docs argued, we should try to biopsy a piece of the "enhancing" area to get the truth. 

But we were overruled; and a few days later, on Tuesday, I said goodbye to Addison. 

By Saturday I was married and had left on my honeymoon.

Addison: Part IV

Frustrating. It was simply frustrating.

We continued to care for Addison through much of the beginning of April, when her one-sided weakness progressed to an inability to move her right leg. And the pain continued; no matter how many pain killers we added, Addy's pain was refractory to treatment. I don't remember seeing her do much besides grimace and shout irritable words at her parents during much of that month.

And no wonder... an adult in real pain is just as irritable. A three year old is the worst. I felt deeply sorry for Addison's mom and dad during this time. No matter how hard they tried, or what they did, nothing could really make her comfortable. Sometimes, for short periods of time she was able to rest or sleep; but these were just tiny pauses in a very frustrating life for them.

Sadly, as the middle of April approached, Addison began having difficulty with urination and defecation. Ah... this broke my heart. It is almost universally a sign of spinal cord compression, and so yet another MRI was done. Unfortunately, this one continued to show worsening "enhancement."

Now, at that time I had not taken care of very many children with spinal cord tumors. Addison was really the first. However, even I at that point began to get suspicious. It seemed almost irrelevant what some medical scan showed, when we couldn't actually interpret it- wasn't the fact that Addison seemed to be losing neurologic function weekly proof enough that her tumor might be spreading?

Several other doctors agreed; however, the radiation oncologist and the brain tumor doctor both still believed that the cause was post-radiation necrosis. I guess sometimes, there are things we never know for sure...

Even when it might make a huge difference.

Intermission...

Just an intermission for a short update for our friends.

Sitting here typing up Addison's story, I am reminded that it is much more difficult to write these stories almost a year and a half after they occurred. There are so many amazing ongoing stories right now that deserve to be written but I'm torn between finishing a sad story that was touched me so deeply and sharing the amazing epilogue to Lily's story that has happened in the meantime! And all the other stories that are ongoing right now and are so much fresher in my mind....

So I will try to show some restraint and finish Addy before going on. However, teaser: there is more, MUCH more, to Lily's life story that I absolutely cannot wait to put into words! Look for "Lily: The Epilogue"... soon.

I had promised myself I was going to write more this summer. I guess its not so bad to break a promise to yourself, as long as there aren't really any consequences. But one of the reasons was that writing some of these out is so therapeutic for me. These stories happen in real life so much faster than I can write them down, that I rarely have the chance to think on them or really process them very deeply. But these children are so precious, and the stories of their lives so invaluable- they are beyond words. I can't usually sum them up in a few sentences in response to a passing "How is your job going?" Therefore, I am going to start making a real effort to keep up with this, more for myself and the kids than anyone else.

In that light, in the last week, I had the great privilege of taking care of and being involved in the stories of two children who passed from this life. And at church this morning, I just sort of had an epiphany. I have never thought of my job as particularly spiritual. Mostly, in my mind its very logical and scientific; I mean, its medicine. But it really stuck on my heart that part of my job is extremely unique- the part where the science doesn't work, and we don't "win," and I do help children pass on from this life to the next.

And you know, then I thought, what a cool job.

Addison, Part III

Addison was re-admitted in March with pain. That sharp, shooting pain had returned in one of her legs. Uggg... never a good thing. Of course, the first thing we all assumed was that she had relapsed.

But Addison's MRI was not definitive. It didn't have any major new areas of tumor, and just showed a kind of diffuse "enhancement" everywhere. The neuroradiologist told us that could be several different things- relapse, radiation necrosis, infection, etc. 

Addison's doctor, the brain tumor doctor, went with radiation necrosis. This is a process that happens sometime in the window 6-9 months after tumor irradiation when you can start to see some of the normal cells in the radiation field die. This "necrosis" really just means that although the radiation killed the tumor, it took awhile longer, but it also killed parts of Addison's spinal cord.

But, no one could definitively say whether this was really the case. We all just kind of took our best guess.

Pain meds were increased. Addison was discharged and fairly comfortable. 

But in April, she came back to the hospital again. 

This time with one-sided weakness; her right leg was becoming weaker and weaker.

And guess what the MRI showed again?

Enhancement. More enhancement.