Addison: The End

When Dr. Parkham told me they had given Addison an ommaya reservoir, I was in complete shock.

I stared at him with wide-eyed incredulity.

"No! They didn't!"

He looked at the ground ruefully and shook his head. "I know... believe me, you're not the only one who thought it was a little crazy."

How could they? How could anyone in their right mind drill a hole in the head of a little girl dying of cancer and pour chemotherapy in!?

Especially with what happened next. One of our newer doctors told me the story of how he was called to witness Addison's first intrathecal chemo administration so that he would know how to operate the ommaya afterward. He said that while the fluid flowed into the space around her brain, Addison turned blue and stopped breathing. Although she recovered shortly after, that doctor flatly refused to do the next "treatment."

Unfortunately, even the spinal fluid chemotherapy did not help; it was beyond human intervention. Sadly, Addison died from her tumor a few days later.

So heartbreaking.

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Here is my take, and probably most of the rest of the world's, on palliative care: when death is inevitable, the goal becomes COMFORT. If you do anything that does not make the patient more comfortable, you are really breaking the Golden Rule of medicine, which we all know is: Do no harm.

Doing no harm is usually taken to mean one of two things: curing disease, and/or relieving pain. Therefore causing more pain to extend the life of a suffering person is harm.

I think this is why Addison's story has always bothered me so much. Its been over a year but I really couldn't write anyone else's story out until I had written hers. I feel in her case, we gave in to a parent's wishes and violated the Hippocratic oath.

And its something I've had a hard time forgiving us for.

Addison: Part VII

Relapsed, widely metastatic ATRT is fatal.

It just is.

Not immediately, of course; but there isn't much to do, although I guess you could argue for some palliative radiation. The chemotherapy she had continued to receive throughout the last few months of her life- the best therapy available, to our knowledge- obviously hadn't worked. In the end, with such an aggressive tumor, you sometimes have to accept fate.

I wish that Addison's parents had understood that. Sometimes I wish the internet didn't really exist; not for sleep deprived people desperately trying to fact search for a miracle drug in the wee hours of the morning, anyway.

Not that any such drug existed, or popped up in a google search. But desperate parents are, well... desperate.  And when it comes to death and dying, I've learned this lesson: you absolutely cannot make a parent who is not ready accept death for their child. No words exist in the English language that can get across the truth to a person who is not ready to hear it. In fact, many of our parents who have children with poor prognoses are conditioned by support groups or their own inner vows to adamantly reject "giving up." Some of them simply cannot do it and fight vigorously against "accepting" death. Patients themselves are often the same and refuse to hear their own CT results or discuss end of life care.

In Addison's case, her parents were not ready to accept death. Neither was her primary oncologist, who offered them further therapy. Since systemic chemotherapy hadn't worked, her doctor researched giving chemotherapy into the fluid surrounding her brain and spinal cord. It had been done a few times with some anecdotal success in kids with ATRT.

This is called "intrathecal" chemotherapy. We do it routinely for children with leukemia to treat any cancerous cells in their spinal fluid, as that fluid can serve as a reservoir for the disease. The normal method for giving spinal chemotherapy is through lumbar punctures- "spinal taps." These are very common on the oncology service for so-called "liquid" tumors.

However, this is not routine for solid brain and spinal cord tumors. And since Addison's tumor was growing around the bottom of her spinal cord, it was not even possible to do a lumbar puncture to inject  chemotherapy.

So something more drastic was done instead.

A hole was drilled in Addy's skull (called an "ommaya reservoir") to be used as a kind of permanent "brain" tap for chemotherapy.

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.

Addison, Part II

Addison did well for awhile. Around 6 months, actually. After her tumor re-grew so quickly after the initial resection, she received emergency high dose spinal radiation. That halted the tumor's progress in its tracks. Long enough to get in some chemo.

Addison then received approximately six months of very high dose chemotherapy. She was frequently inpatient in the hospital, receiving some of our most intensive IV therapy. This was a very trying time for Addison's mother; since her daughter's diagnosis and for weeks before, Addison had experienced non-stop, intense nerve pain.

Nerve pain is unlike other kinds of pain in that it is usually sharp, shooting, and often sudden. Not the kind of pain you  would want to have to manage in a three year old.

However, sometime in January (about four months after radiation) Addison's pain had resided, and her neurologic function had improved enough that with aggressive physical therapy and a walker, she was actually able to walk on her own again. Which again was a relief for her mother, who had frequently been carrying around a large three year old.

Amazing, what a kid's spinal cord can do. Seems like adults lose function and never can regain it. (This, in my opinion, is only one of the many things that make children more like amphibians than people.) Really pretty astounding.

Addison then enjoyed a few months of improved function, bowel and urinary continence, and a relatively normal life. She even smiled and talked with me during one of her admissions- the first time that had happened since I'd met her.

In that respect, Addison was fairly normal. Some toddler and preschool aged children do continue to interact normally after a diagnosis of cancer; however, there are definitely an equal or higher number of children that regress. Whether from fear, anger, grief, or an inability to understand and cope with their diagnoses, many children in this age group simply stop talking to anyone other than their families. A few stop talking at all.

Addison had been in the former group; until her condition improved, and she emerged from her shell. It was then I saw the Addison none of us had ever known.

After all, living a pain-free, relatively normal life had been out of her reach for over 4 months. It was a glimmer of hope in a a desert for a devastated family.

A glimmer that vanished just eight weeks later.

Addison, Part I

The first time I met Addison, I have to admit, she freaked me out a little.

And I'm going to be honest with you: if you're looking for something cheery... this is a long story, that doesn't end well. Look elsewhere now for a good old fashioned American ending, because this story is sad. And real, honest-to-goodness non-fiction.

Addison was almost three years old when she was diagnosed with the rarest of the rare- a very rare childhood brain tumor, that- unusually- was growing at the bottom of Addison's spine, instead of in her brain.

The unusual thing about ATRT (atypical teratoid rhaboid tumor), is how aggressive it can be. Addison lost the feeling in her legs first, then her ability to walk within days of diagnosis; one week after her tumor had been completely surgically resected, it had regrown completely, back to its original size.

The other thing about spinal cord tumors, is their destructiveness to a person's functionality. Imagine how many functions depend on the nerves going down and out the bottom of your spinal cord. Leg muscles, sensation, reproductive functionality, bowel and urinary continence. Everything below the waist, really.

And when I saw her for the first time, she was the first toddler I'd ever seen in a wheelchair, paralyzed by her cancer. 

Cancer can be such a bastard.

Our Culture's Unwitting Acceptance of Machine-Controlled Lives

I may be getting a little paranoid lately... but tell me if you think this is true. In our world today, almost everything is controlled by machines. And these machines are feeding us ridiculously large amounts of information daily that influence our thinking, morals, beliefs, and, in some cases, are isolating us from the most important things in life.

In other words, mind-influencing; if you are aware of the messages these machines are feeding you. If you aren't, it might just be mind control.

Thinking I might have gone a little wacko while taking a rather long break from my blog? Well, you'd be wrong. I've been thinking deeply on this subject for all of seventeen minutes this evening, and have come to the following realizations:

People are obsessed with machines in our culture. Absolutely obsessed. Cars, phones, computers, televisions, iPads- you name it, people will do anything to get their hands on it. Machines.

For starters.. what kind of car you drive, our culture says, tells something about the person. Thus the machine you drive has some control over your social status.

And with commutes getting longer and longer as people have to move farther and farther away from the city (to cram their McMansion into some treeless suburb that is a safe distance from the inner city but close to a nice mall), people spend more and more time (alone) inside of their driving machines.

And what do they listen to in these driving machines? Music playing machines or broadcasting machines. Machines broadcasting lyrics and information into our heads.

I have spent a lot of time in airports and on airplanes in my life. And I can tell you that you used to see people talking to each other in airports- finding out where the person in the seat next to you was from and where they were going. The last time I was in an airport (this last May) I had never seen so little social interaction between strangers. Most people are staring into their machines- iPads, iPhones, laptops. Keeping them from having to even make eye contact with a person; omitting even the chance for a friendly smile, much less a conversation.

And what are these machines "telling" the person? Well, if you are on facebook, your machine might be telling you what everyone else in your culture is doing. And therefore leading you toward certain thought lines about what you need to do to fit into your culture.


"Skydiving!"


"Our accomodations for the night {insert picture of Scottish castle}"

The interesting thing is that you have no actual interaction with a person, although you are using a machine to ascertain what people are doing.

Going on... I will skip the 8 hour day staring at machines that most of us have. Because most of the staring into machines at work we do is inputing information, and not necessarily being influenced by it. And a given work day may include a lot of social interaction.

However, when the average person comes home from work, what do they do?

Turn on their staring machine. The big machine on the wall they stare at and soak up advertisements and cultural messages in the form of reality TV shows or sitcoms showing what "everyone" in the culture is doing. These TV people are flawless and have perfect houses... hard to live up to in real life. And these are machine people; they are not real, they don't exist. And they are not interacting with you.

That's the sad part about machines; they can be so isolating. Most people commute in their car alone. You can't find out someone's hopes and dreams by watching TV with them. You can't meet friends from other countries in an airport by playing Angry Birds for an hour.

So, after most of us are done being influenced by our brainless home machines for an hour or three after work, what do we do then?

Get on our small handheld or desktop machine again to check our electronic machine mail or facebook again to see if anyone sent us a message with absolutely no human element of interaction. Because only our grandparents handwrite letters anymore.

The real kicker for me tonight was when I was watching my house's machine on the wall and saw an iPhone commercial. Some actor whose name I can't remember but whose face I recognized was sitting in a beautiful apartment, dressed in an expensive suit, utterly alone. And this man was having a conversation and entertaining himself solely with Siri, the robot on his iPhone machine. She tells him a joke and the commercial ends with him chuckling as if he is interacting in some normal, human way.

And it just hit me... what an empty, horrible life. To have everything you need materially and to have access to all this "information" but to have such a poverty of real humanity. Like handing your wife a hand picked wildflower on the side of a lake with the wind on your face as you watch the smile in her eyes when she smells it.

Now don't get me wrong... most people's experience with Siri more likely includes "Sorry, I don't understand" in that robotic voice. But the way I interpreted that commercial's message was: Information-feeding machines can provide everything you need.

Sure... maybe the machine can give me directions to the lake to an enable an experience I might otherwise not have had. In those cases they are useful; when you control them.

But when the majority of "information" these machines are feeding me is so unrealistic (i.e., perfect houses, perfect bodies, flawless faces, exciting adventures, fantastic vacations) how else can I interpret it but that these machines are evil and give people unrealistic expectations of themselves and their culture?

FURTHER, (sorry getting on some kind of tangent now), machines may be productive (work, email, etc) but.... really. Farmville? Facebook? Desperate Housewives? These machines are sucking away hours of our time with no results, nothing done. AHHHH! Its so evil!

Having started a garden this year, I have a very small concept of the kind of hard work people used to do (and in some parts of the world, still do) to survive. It was not only outside in the sunshine- in nature; it was often collective, and most importantly it was productive. How awesome, to see that the tiny seeds you planted months ago have now yielded a tangible harvest that engages your senses- smells good, tastes good! One that you can enjoy with your family, that you can feast on together to fill one of your most basic needs. But our culture has lost a lot of that now.

Count me out. I hope to never own an iPhone or be addicted to the machine on my wall or in my hand.

The real question is, are you controlling yourself and your own mind? Or are machines controlling you?

Back from Hiatus...

Hello again! My nine loyal friends who read this blog. ;) And to clarify, that was in the past tense.

Back, indeed- after a long hiatus, is more like it.

I'll skip everything in between and put it simply.

I'm going to start writing again.