Rhabdoid Cancer

I have not posted a lot of details about Harry's particular type of cancer. But I thought I would today.

When we first got the diagnosis of a 'primary rhabdoid tumor of the liver' on March 6th, 2008, we had no idea what were were up against. Initially, Harry's doctors thought he had an hepatoblastoma - one of the more common types of paediatric liver cancers. Complete testing of his biopsy sample proved otherwise, and resulted in the diagnosis of rhabdoid cancer instead.

In truth, during Harry's whole journey with cancer, I don't think I really knew what we were up against. If he had been diagnosed with a Stage 4 Hepatoblastoma he would have had about a 20% chance of survival. When we were given the Rhabdoid diagnosis, I recall asking his doctor, "Okay, so what are his chances now?" I guess the doctor's couldn't face telling us, "Somewhere between 0-1%" so instead they said, "No better than his odds were with Hepatoblastoma".

One of my first nights home from the hospital I recall doing a google search on 'rhabdoid cancer'. All that came up were links to obscure journal papers and the Rhabdoid Kids site. You would think, having just finished a PhD, I would have been all about digging into the research side of this disease. But my instincts told me otherwise. I read one journal paper and just couldn't digest any of the information. I switched to the Rhabdoid Kids site, read about a few Rhabdoid kids, but could not bring myself to even open the Rhabdoid Angels page. I just didn't have the energy to read about other kids, I could only muster the energy to focus on Harry.

My intuition where Harry is concerned has been uncanny, every since he was first conceived. And I so clearly recall every ounce of my body saying, "Stop, stop looking at web sites. Don't focus on the science of this disease. That is not your job this time. You need to focus on healing Harry, taking care of Harry, loving Harry. You are not meant to focus on the technical side of this disease".

I listened to that internal voice and I didn't do any research or reading about Rhabdoid cancer again till after Harry had passed over in August.

Once Harry had died and I had hours and hours of empty days to fill I started to obsess over reading everything I could about Rhabdoid Cancer.

I think I am glad I didn't know just how cruel this disease is while Harry was alive. I don't think I could have had the courage to fight and maintain my hope and optimism had I know just how poor his chances really were.

Rhabdoid cancer is known as the rarest and most aggressive of all childhood cancers.

It was first discovered in the kidneys in 1978. In 1991, doctors realized that a class of brain tumors was actually the same cancer and so classified the brain tumor version, AT/RT or Atypical Teratoid / Rhabdoid Tumor. Basically, now the cancer is classified as either AT/RT or non-CNS MRT (Malignant Rhabdoid Tumor). Renal (kidney) and AT/RT are the most common variants of this cancer. 'Officially' Harry's cancer was an extra-Renal, non-CNS MRT of the liver.

A presentation I found on the web, put together by a doctor in St. Louis in 2007, listed a table of all of the published cases of primary Rhabdoid liver cancer that the doctor could find.

There were 25 cases listed.

Yes, that is it. 25 cases documented by 2007 in the entire english language academic literature. Of those 25 cases, three had survived, 2 girls and one boy. For some reason, girls have a slightly higher survival rate. So, since not all kids who have rhabdoid liver cancer will have a paper written about them, Harry choose a very exclusive club - there have been maybe 50 kids ever in the world with this type of cancer.

Go figure. My baby doesn't just get cancer, no, he has to get the rarest and most aggressive variant of the rarest and most aggressive childhood cancer of all.

The only path to long term survival is to remove all of the cancer and that means surgery. Chemotherapy alone is not enough, the primary tumor HAS to be surgically removed for survival. Rhabdoid cancer is treated with the strongest chemotherapy they can give a person. Most adults given the chemo Harry had are unable to move out of bed for days, they are so sick. For those kids who live long enough to start chemotherapy, the usual pattern is that the cancer responds well at first, but it is so damn smart, that it pretty soon figures out the chemo and relapses. Most children die with weeks of their first relapse.

Because the cancer grows so quickly, almost all kids exhibit very few symptoms until a few weeks before diagnosis and then, like Harry, present only with mild flu-like symptoms. Most kids are diagnosed at Stage 3 or 4. Many kids don't even survive long enough to start chemotherapy.

Given how extensive Harry's tumors were when he was diagnosed, by all rights, he should have died that first week in hospital.

So if you ever wonder whether we managed a miracle with Harry, the answer is an unqualified YES. That Harry survived to actually start chemotherapy, that he responded so well, that he smiled and laughed and bounced through each brutal round, that he got to come home, that he got to celebrate a first birthday, that he got to experience one summer as a little boy was all a miracle.

Strangely, I take some comfort in Harry having Rhabdoid cancer. In a strange way, it would been more difficult, I think, had he died of a more common cancer, one that most kids survive. Then we would have been left asking, "what did we miss, what did we do wrong". With Rhabdoid cancer, I know we really did everything we possibly could do for Harry. We gave him the very, very best shot we could.

But I sense that Harry choose Rhabdoid cancer, if it is possible to say such a thing, because he knew he had to come and give us all this love, but then go back to God. He had to choose a cancer we couldn't possibly have beaten.

I always said that whatever child followed Lydia was going to have to do something 'big' to not be caught in her shadow. Well, this wasn't quite what I had in mind. But Harry sure did do something big with his short life, of that there is no question.

Peace,
Cynthia