Katie lived

This is my blog's first guest post since SuperGirlfriend did one a few months back, and it pretty much speaks for itself, so I'm just going to say, here is my good friend and frequent commenter Scott S., he's going to be telling us about his daughter Katie's recently concluded struggle with cancer, it's an amazing story -- and here he is:


"The smell of hospitals in winter
and the feeling
that it's all a lot of oysters
and no pearls."I don't think the smell of hospitals will ever leave me.

Katie was diagnosed August 26, 2003. She was nearly 3 and a half years old. She'll turn 6 on April 22nd.

She was sent to the hospital for blood tests on the advice of our family doctor, as a result of some odd bruising that had appeared on her back and torso.

The blood tests showed high white blood cell counts and led to further tests. An x-ray showed that Katie's liver and spleen were enlarged. Both of these are classic symptoms of leukemia.

Leukemia means, literally, "white blood". There are three types of blood cells that all people have - red blood cells that carry oxygen to the body, platelets which help with blood clotting, and white blood cells, which defend the body from infection and disease. All these cells are grown in the bone marrow, and are pushed into the blood stream as necessary. What happens with leukemia is that a white blood cell (called a lymphoblast,or blast, for short), instead of developing into a useful disease or bacteria fighting cell, mutates. This cancer cell does nothing useful; it simply multiplies, and multiplies... and multiplies. These blasts fill up the bone marrow, so that the marrow can no longer produce red blood cells, or platelets, or even useful white blood cells. The immune system becomes suppressed, and the body becomes more susceptible to infection, and with lowered platelet counts, bruising and internal bleeding become risks as well (those weird bruises, called patichia).

Eventually, they leak out into the blood stream and multiply again, hence -"white blood".

The liver and spleen become enlarged trying to cope with these mutant cells. Elevated amounts of white blood cells show up in a blood test.

Of course, a virus will also cause elevated white cell counts, and some viruses even eat platelets. An enlarged liver and spleen can be caused by mono. The only conclusive test for leukemia is a bone marrow aspiration (BMA), in which a needle is inserted into a marrow rich bone (usually the hip bone), and some marrow withdrawn.

Katie was admitted to the hospital that night. The mono test came back negative, so she was scheduled for a BMA the next day.

The BMA showed a marrow that was 99% white blood cells - a confirmation that the cause was leukemia.

They also performed a lumbar puncture (LP) - basically a spinal tap - in which they insert a needle into the base of Katie's spine and draw out some spinal fluid, to check for the presence of blasts in the CNS. Thankfully, this test came back clear, which meant no radiation therapy.

The official diagnosis was Acute Lymphoblastic Leukemia, or ALL Leukemia is the most common type of childhood cancer, with around 4000 children diagnosed in the U.S every year. Ironically, because it is so common, it is also the cancer with the highest long term survival rates - around 80% overall, and more than 90% for some types.

The specific type of ALL depends on the kind of white cell that was mutated. There are leukemic cell lines - B and T. The treatment regime, or protocol is determined by the type of cell, along with other factors present at diagnosis. The three types of ALL are precursor B (pre-B), B, and T. Pre-B has the highest survival rate, T the lowest. Other risk (risk meaning risk of relapse) factors include age (infants have a very poor prognosis, as do children older than about 8 or 9), gender (girls have overall better survival rates than boys), the number of white blood cells present at diagnosis, and the specific genetic properties of the cancer cell in question. The technical diagnosis was pre-B ALL.

Katie's age, gender, and the genetic makeup of the cancer cells put her in what was considered the "low risk" category.

So basically, if she had to get cancer, she got the best kind.

At the time of Katie's diagnosis, there were at least two different organizations that had documented treatment protocols for leukemia - POG (Pediatric Oncology Group) and CCG (Children's Cancer Group). Whether you got a POG protocol or a CCG protocol depended in large part on where you lived and who your Oncologist was. Both types of protocols use the same drugs, for the most part, just in different combinations and at different times. However, the CCG protocols do not have a "low risk" category - a child is either "standard risk" or "high risk".

CHEO uses the POG protocols (or rather *used* the POG protocols - the two organizations have since merged their protocols into one agreed upon set), so Katie was put on a low risk protocol.

The low risk protocol is divided into three stages - induction, consolidation, and maintenance. Treatment starts immediately upon being diagnosed.

The induction phase is the most intensive of the three stages. The goal of induction is to wipe out as much of the cancer as possible, and so Katie was bombarded with at least 5 different drugs. The treatment started immediately. The drugs she got during this first phase included a ridiculously high dose of a steroid called dexamethasone, vincristine, L-asparaginase, cytarabine, methotrexate and daunorubicin. Most of these were were either injected via needle or put in through an IV.

Most chemotherapy drugs work by either killing off fast-reproducing cells, or disrupting their ability to reproduce. A side effect of virtually all of them is nausea and vomiting. Unfortunately, that is not the only side effect.

For Katie, the worst drugs in terms of side effects were the dex and the vincristine. These, she had regularly. The other drugs, with the exception of the methotrexate, were one-time doses during the first month.

The steroid caused extreme emotional volatility ('roid rage...in a three year old), fatigue, hugely increased appetite, muscle soreness, and water retention. She had to take three doses of this every day for a month. The effect of vincristine has been described as something like having a bomb go off inside your body. Its side effects include severe muscle and bone pain, foot drop, constipation and hair loss. She got an injection of this once a week for a month.

Three days after she was admitted, she had an operation to insert a catheter into an artery just above her heart (called a port-a-cath). The catheter has a little valve in it just under the skin, which allows easy access by a needle. Once the port-a-cath is inserted, most of the drugs were administered by inserting a needle into the valve and injecting the drugs this way. Because the catheter is tapping a major artery, the drugs are distributed throughout the body much more quickly than otherwise.

(The surgery was postponed twice due to lack of OR space. Not a big deal, except that she was not allowed to eat for 8 hours prior to the surgery. Which kept getting bumped by a day - while she is taking huge doses of steroids which make her ravenously hungry. She ate like two meals in three days waiting for that damned surgery.)

She was also subjected to daily blood draws - usually from her arm - which she *hated* more than anything else. She had several blood and platelet transfusions, one of which caused a severe allergic reaction that very nearly killed her. She also got a line infection (in her newly implanted catheter) and had a 2 week dose of nasty antibiotics.

At this point, I should explain that throughout treatment, Katie was required to have weekly blood tests. The chemo drugs do not differentiate between 'good' cells and bad 'cells' - they simply kill off anything that reproduces quickly. This includes hair follicle cells (which is why hair loss is a common side effect of chemo), and also fast reproducing cells like white blood cells, platelets and red blood cells.

The weekly blood tests were required to keep an eye on her hemoglobin levels (i.e. red blood cells), platelets, white cells, and particularly, something called neutrophils. Neutrophils are a kind of white blood cell whose function is to fight infection. If the level of neutrophils in the blood (called the absolute neutrophil count, or ANC) gets below a certain level, the body can no longer fight infection.

Anyhow, after the induction phase was over, the oncology team kept an eye on these levels, in particular the ANC, in order to tweak the dosages of the chemo drugs Katie was taking. The idea is to keep the ANC levels in a certain range - not too low, not too high. If the level gets too low, she was considered 'neutropenic' - unable to fight off infection - and most chemo would be suspended until the levels are high enough. Similarly with her platelet count - if it got too low, chemo would be suspended.

During induction, however, they just blast away, keep the patient in isolation, and give blood transfusions when necessary.

Almost everyone becomes neutropenic during the induction phase of treatment.

However, an ANC count in the acceptable range for a patient on chemotherapy is much lower than for a healthy person, and so anyone on chemotherapy has a suppressed immune system, with all the risks that implies.

By the end of the month, our happy little girl was gone. She could not walk, she wouldn't smile, she barely talked, except when she was screaming. She did nothing but eat. She ate a dozen boiled eggs *in one day*. She puffed up like a balloon, and her hair fell out in clumps.

It was unbearably terrifying to watch, and we really didn't know if we were ever going to get her back.

At the end of the one month induction phase, she had another BMA and LP. The BMA came back with less than 1% blasts. The LP came back clear. Katie was declared to be 'in remission'.

In addition to all this, she had other tests and procedures as well. She had an extensive neuropsych exam to benchmark her brain's development, and an MRI.

Eventually, she was able to walk again, and regained much of her former spark.

Once the induction phase was over (and the line infection had been treated), the second phase of treatment began. The second phase involved daily doses of a drug called mercatopurine (or 6mp), plus a one week "pulse" of dex between two injections of vincristine once every 6-8 weeks (these were called 'steroid weeks', they were greatly hated and feared). The pills, the dex in particular, tastes terrible (like gasoline). She also had to take a special antibiotic three days a week to protect her from a particularly lethal pneumonia.

Thankfully, my exceedingly clever girl, with the help of my exceedingly brilliant and capable wife, managed to learn how to swallow pills whole, so we would get the pills from the pharmacy, chop them up and stuff them into gel caps for her to take.

Every three weeks Katie had a three day inpatient stay where she received a 24 hour IV infusion of methotrexate (methotrexate is another nasty drug - when the nurses would come in to start the drip, they wore rubber gloves, masks and gowns. The drug itself comes in a bright yellow bag with large black letters that spell 'BIOHAZARD'. If they spilled it, they make us leave the room while a cleaning crew came in. If all went well, and nothing spilled, they started pushing into Katie's veins.). The methotrexate caused Katie's hands and feet to become bright red, as if they were severely sunburned. The skin would start to peel off. The stuff literally burned her extremeties from the inside.

In addition to all that, she had regular (every 8-10 weeks, I think) LPs, in which this same drug (methotrexate) would be inserted directly into her spinal column.

The consolidation phase lasted for 6 months. She had a second neuropsych exam to track her brain's development and the effects of 6 months of chemo. Thankfully, the tests showed no significant developmental problems.

After consolidation, there were no more planned inpatient visits. Kate was hospitalized once for shingles, and once for pneumonia. The dex/vincristine pulses were spread out a bit more, as were the LPs. She started taking a pill form of methotrexate once a week. The pill form of the drug caused the same kind of burning as the IV form. The vincristine never failed to cause her hair to fall out, and the dex turned her into a raving, ravenous psychotic for about a week.

When she had her last dex/vincristine pulse in the fall, we celebrated.

As I describe all these side effects and procedures, it strikes me that it must sound as if we lived our lives in perpetual shell shock and misery, and really, we didn't. The pill taking became routine, the hospital visits became annoyances, and Katie, amazing little girl that she is, pretty much became used to the side effects of the medication. We gave her an antinausea drug pretty much every day to counteract the chemo, but she occasionally would still get sick, usually first thing in the morning. She hardly ever complained. On LP days she wasn't allowed to eat until after the procedure, which happened sometime between 10am and 1pm - meaning that she would go without food from about 6:30pm the night before, without complaint. After these LPs she would come home and go out and swing on the swing set in our back yard and tell her friends she'd just had a lumbar puncture - as if that were the most natural thing in the world.

Because the pills she took worked best on an empty stomach, she was not allowed any food after 6:30 at night. She only grumbled a bit about this if her brother was eating something and she couldn't.

We became constantly vigilant about infection. If Katie became neutropenic, we took her temperature nightly. We became obsessive about hand washing. Even when she went back to school, she missed large amounts of time either because her counts were low, or because there was some plague going through the school.

All this became 'normal' life for us.

For me, making all of this habit was a way of coping with the treatment without thinking too much about its implications. A relapse during treatment drastically decreases the chances of survival. Over the course of the past 2 years, we met and became friends with several families with children who relapsed - and some who died as a result. It is the fear that all parents live with - what if the cancer comes back? It's a possibility so terrifying that for me the best way to cope is to not think about it. I've likened getting through this treatment to walking on a rickety bridge above a endlessly deep chasm. You have to keep moving. You have to keep your eyes on the other side. If you look down, you'll become paralyzed by fear.

But the bridge could collapse at any moment.

The last month of Katie's treatment was nearly as difficult for her (and us) as the first. Because her ANC counts had been consistently high throughout much of her treatment, the doctors kept increasing the dosages of the medication she was taking. The higher methotrexate dose caused the skin on her feet and hands to crack and bleed. Her lips and mouth became blistered and bloody. Her throat became so sore (more blisters) she could no longer swallow pills. She needed two blood transfusions in three weeks. Eventually, her platelets dropped below acceptable levels and she was pulled off chemo for a week, which allowed her to regain some strength.

On February 28, Katie had her final LP and BMA. Both came back clean.

On March 6, she took her last pills. March 7 was the official end of her treatment. The scars on her hands are already mostly healed.

When this started, I couldn't imagine how we were going to get through 2 and a half years. I got through it by doing what children in general, and Katie in particular, do naturally - take one day at a time, and don't think about the future.

It is impossible to overstate how proud I am of my daughter. She handled a horrific ordeal with grace, dignity, and humor.

She still has her port-a-cath, so she has to go into the hospital monthly to have it flushed (to avoid infection). It'll be removed in about 6 months, sooner if my wife has her way (and I wouldn't bet against her). She still has to take a weekly antibiotic because of the increased risk of pneumonia. She'll have monthly blood draws instead of weekly.

If she is cancer free in two years, she'll be considered a long term survivor. If she's cancer free in 5, she'll be considered "cured" - at no more risk of cancer than the general population. For the time being, we're still walking that bridge, even if it's not so rickety anymore.

As for long term side effects - there are a lot of unknowns. She's at higher risk of osteoporosis, due to the effects of the chemo on her bones, but a lot of that bone mass can be regrown with the right diet. Leukemia patients have a higher risk of obesity, for some reason.

The longer term side effects not yet well understood, because it hasn't been until relatively recently (like the last 20 years) that children have survived leukemia long enough to even worry about side effects. Learning difficulties and emotional issues are a possibility. She'll be tracked for the rest of her life by the medical system to find out.

* * * *

Okay, this is Highlander again, with some closing comments.

First, this story is a tribute to more than just Katie's enormous heroism, and the amazing emotional valor of her parents... although, frankly, I am so utterly in awe of Scott and his wife's strength through this unbelievable ordeal that there are no words to adequately describe how much I admire them, or their child.

More than that, though, this story is a tribute to the Canadian single payer health care system.

Try to imagine going through everything Scott has so admirably detailed above -- while worrying about how you were going to pay for everything, and whether or not your insurance plan was going to cover everything your child needed.

Several prominent lefty blogs have started to agitate lately for the Democrats to seriously push for universal health care in 2006 and beyond. I agree with them -- but most of them are very careful to note that Canada and Britain are NOT good examples of a single payer health care system.

I look at a story like this, and I try to imagine going through all this with a toddler in our current nightmarish health care system here in America, and honestly, I don't know what these people are bitching about.

Congratulations to Scott, his wife, and Katie. And thanks for sharing the story. Nobody ever gets enough good news like this.