Arden: Part I

Arden Smith was diagnosed with neuroblastoma in August, and in fact, was the sickest new oncology diagnosis I had ever seen. (Neuroblastoma is a tumor of the nervous system that usually originates in the adrenal gland, a small piece of tissue that sits on top of the kidney and makes all kinds of important hormones.) 

Arden had quite the journey: First, her pediatrician in the tiny Oklahoma town where she lived told her parents that the mass growing in her belly all summer long was "no big deal" and probably just a big spleen. Fortunately, they sought a second opinion in the nick of time, which saved her life. A CT scan showed a gigantic mass filling her belly, even invading some of her other organs. When she finally got to us at the Children's Hospital, Arden looked pregnant- except the bulge was too irregular and nodular to be a pregnancy, and resided mostly on the left side of her abdomen. She also just looked ill. 

One thing all doctors learn to do: look at a person and judge how close to death they might be. You'd be surprised how quickly that skill is learned. 

Arden then went to the OR to have the mass biopsied and almost bled to death on the operating table as her surgeon, Dr. Puffinbarger, tried to get a piece. Puffinbarger saw what we saw in Arden: "This baby needs chemo!" She almost shouted when she saw me in the OR hallway afterward. "She's on the edge. When are you guys going to start some and save her life?!"

She was right. Arden was on the way out, and fast.

Intermission & Announcement

First, I just want to say what a pleasure it was to write out Lily's story. After everything that's happened this last year, and all the different children we've treated, it was really fun to go back and remember all the way through one entire story. On a day to day basis, its easy to forget the big picture truly is bigger than any single day's joys or sorrows. I am looking forward to continuing this with others of these little people as time goes along- if for no other reason than making sure I don't forget them either. Thanks for reading and remembering these precious children with me.

Second, I wanted to announce that... 

Larry asked me to marry him on Christmas Day. We're engaged!

Lily: The End

Don't you know, Lily not only survived her fungus-removing thoracotomy that fateful Friday, but also the ensuing weekend ICU stay; then, the little fighter survived her next big procedure, another chest-opening OR visit to remove more aspergillus on Monday (which ended up this time as a mere thoroscopy).

Praise the Lord, Lily lived through all that awful torment. And the torment accomplished its only necessary task, and saved her life.

When she came back to us this time (in early September), Lily was exhausted, deconditioned, and in almost constant, intense pain from the foot-long surgical wound that included twelve severed ribs, all healing inside. All this, and she still had to keep fighting the remaining fungus. All this, and her constant pain persisted, despite morphine drips, for the next three months. All this, and she still had to undergo the biggest unknown of all: a risky bone marrow transplant. All this, and we still weren't even sure that Lily was in remission from her leukemia.

All this, and the girl still had joy. Simply astounding. Although she was often in and out, drowsy from the pain medications, and often depressed secondary to her pain: she never gave up, and often managed a weak smile when I'd sneak in personal visits. During this time of pain and healing, her will to live persisted and... blossomed, even. Lily became my hero.

Lily was then given one more bone marrow test that showed she still had 6% leukemic cells in her marrow. Because so much had already been done to save her life, the transplant team went ahead with their plans and Lily received a bone marrow transplant in October, 2010. Her transplant was relatively uneventful, and she engrafted within the month. With new donor white cells present to fight the fungus, Lily's energy was no longer drained on a daily basis and her body began to truly heal from her chemotherapy, transplant, and surgery. Just before Thanksgiving, her intense pain had finally resolved and she was alert and eating on a daily basis, able to get up without excruciating pain.

At the end of November, we released Lily from the hospital. Her room was decorated; Sam Bradford called her on the phone to wish her a happy break out: and Bob Stoops came up to the hospital and wheeled Lily through streamers and out of her suffocating little hospital room for the very last time. He then took her down the elevator and out of the building to the limosine that was waiting outside to take her away.

Lily, with tears running down her face, saw the glorious light of day and breathed the free air from the outside without having to fear death for the first time in 113 days. Just like that, the nightmare was over.

Writing this, a song fills my head, with one name- Jesus- interchanged for our girl:

Hallelujah! Lily is alive!
Death has lost its victory,
and the grave has been denied!
Lily lives today,
She's alive, she's alive!

"Then the end will come, when Christ hands over the kingdom to God the Father after he has destroyed all dominion, authority and power. For he must reign until he has put all his enemies under his feet. The last enemy to be destroyed is death." -1 Co 15:24-26

Little victories like these are truly a little piece of heaven on earth.

Though none can say how long Lily will remain in remission, or indeed how long any of us will manage to stave off the enemy death, in the short term at least- victory is sweet! Shout with me!

"Where, O death, is your victory?
And where, O death, is your sting?"

Triumph to those who have overcome with life- to you, Lily, bravo!

And to the Lord, highest of praise for the short and sweet victory of this life!

Lily, Part V

I don't remember exactly when Lily's plight became so close to my heart. To tell the truth, most of our kids don't die. Definitely not within the first year of therapy, and that's all the longer I'd been working at the Children's Hospital; the thought of losing this precious girl, soon, had been weighing on my heart since the fungus was diagnosed. 

But the realization that she might die that day had suddenly come too close for comfort. 

Please, Lord. Save Lily's life. Save her life. Please save her life.  Over and over and over.

Dr. Rooms and I had made an arrangement- she was going down to the OR to watch the surgery and would text me to come down when they opened Lily's chest. After all, even for those of us who work on the oncology unit- this was something really unusual. We all wanted a first row seat. 

Lily's thoracotomy did not disappoint. The surgeons had decided that a sternotomy was too risky, and didn't think she could survive it. They wanted to do the surgery laproscopically, but there were just too many fungus balls. And so they decided to open up each side of her chest separately. 

Two thoracotomies. Ouch. 

Dr. Room's text came just as I was finishing a few things on the floor; it was a picture text. Of the first fungus ball. I raced down to the OR and threw on some paper scrubs over my clothes. Entering the OR, I was greeted by the sight of a room filled with people, all surrounding the table where Lily's body lay carefully positioned, her left lung visible through the gaping hole made by the rib spreader. 

(Rib spreader.)

The surgeon reached his hands in and began cutting away at something. Looking over at the instrument table, I saw several little pink and black chunks sitting, waiting to go to pathology. 

"They're almost done!" Dr. Rooms exclaimed as I walked in. 

"But you just texted... I thought they were doing both sides?"

"She's losing too much blood. They're giving her more but she's not very stable; definitely not enough to open the right side. They're going to send her to the ICU for the weekend and try to finish the other side with the laparoscope on Monday."

That's right; it was Friday. Friday when the surgeons nearly killed her, trying to save her life. Friday that she went to the ICU for the second time. Friday that she survived her first thoracotomy. 

Lily, we realized at this point, was not going to give up without a fight. 

And the true fighter that lived within her was only beginning to arise. 

Lily, Part IV

Thoracotomy: an incision into the chest wall through the ribs, to enter the chest cavity.

I have seen three thoracotomies in my life; two were actually sternotomies (cutting open the sternum, rather than the ribs) for heart transplants from my cardiology rotation- one of those was recorded in detail on this blog. The third was an emergency thoracotomy I saw at 4:00am one morning in the ER, on a man dying after a traumatic motor vehicle accident. As Dr. Lovelace said: When you can’t figure out what’s wrong after imaging someone over and over, and they keep dying, all you can do is cut them open and take a look. Once we’d used the rib spreader to get that look, it was obvious that the man’s diaphragm had been severed and he was bleeding heavily from a laceration to his lungs. He passed just minutes after his thoractomy.

Point being: a thoracotomy is a big deal. Doctors don’t cut into someone’s chest unless they absolutely have to; its only done in the most extreme circumstances, and then only to save a life. Needless to say, cutting open Lily’s chest to remove the fungus was exactly that kind of heroic, life-saving measure; and those procedures are always the riskiest. Even though it wasn’t an emergency, it would still be incredibly painful and would also take her months to recover.

If she survived.

When Dr. Rooms approached Lily’s parents with the options, I don’t know whether or not they fully realized the gravity of the situation; but regardless, when everything had been explained… they decided to take their chances, and go with thoracotomy.

I don’t have children, and I always think about how unimaginable it would be to make the kinds of life altering decisions our parents are often being forced to make. But one thing I’ve learned from them is that anything is better than letting your child die, and then wondering later if they might have been saved. That is the unbearable thought that trumps every other medical risk or procedure, no matter how horrifying; and rightly so.

At ten a.m. the next morning, I stood in the hallway of the bone marrow unit and watched as the transporter arrived to take Lily to the OR. She looked much too ill to be undergoing this kind of surgery; her mom was waiting to get in the elevator to go down to the OR waiting room. She caught my eye and so I walked over and tried to reassure her with a hug and a smile, and let her know I was praying for her and for Lily.

But inside I wondered if she would ever see her daughter alive again.

Lily, Part III

And so, Lily came back to us septic. In fact, she went almost straight to the ICU. I think she lasted a few days on our unit before she ended up there with low blood pressures, being kept alive by medicine drips while the antibiotics worked on killing all the bacteria in her blood.

I still remember the song that was on repeat in my head the morning I came into work, sat down at my desk, and saw that Lily's name and room number had changed to an ICU bed.

Rise! And shine! And give God, the glory glory!
Rise, and shine, and give God, the glory glory!
Rise! And shine! And! Give God, the glory glory, children of the Lord.

A sort of sick feeling entered the pit of my stomach. Not this girl. This was our sweetest girl, one I loved more than a lot of the others I didn't see as often. I didn't really believe Lily was going to die in the ICU, but you never know for sure, until they survive.

To make a long story short: Lily survived. She fought off the infection and came back to our floor, weakened but alive. At this point, it was still such a joy to see her every day; sick to her stomach from her second round of chemotherapy, she still managed to conjure up a smile for us on rounds each morning. Pictures and Bible verses went up all over her walls and outside her room in the bone marrow transplant unit. She survived her second course... and we all began to think she would be ready for her transplant soon.

Until one day, Lily started coughing. It was a harmless cough at first, but then the fevers started; the antibiotics restarted; and within a few days, after imaging her chest, we found out more bad news: Lily was so immune suppressed that big balls of fungus were growing in her lungs. Lots of them.

Ordinarily, no one goes through chemotherapy without getting low white blood cell counts. This is to be expected. However, we also give patients antifungal medicines when we know they will have no white cells to fight infection for an extended period of time. So, when Lily began growing fungus balls while on antifungals~ we started saying serious silent prayers. I did, anyway.

Here's the point: you can get a bone marrow transplant and have a 50% survival if you are in great condition. But start knocking out major organs, like your lungs, and your survival from that kind of transplant starts to approach zero. Fungus just before transplant, like secondary AML, is a real killer.

We gave Lily antifungals... and more antifungals. And then increased the dose of her antifungals. The girl might as well have been taking baths in voriconazole, chugging kegs of ambisome; caspofungin was approaching high enough concentrations to start spouting out of her eyeballs. But despite all this- Lily didn't clear the fungus. She didn't come anywhere close, in fact; upon repeat CT scan, the fungus balls were still there. Smaller, but just barely. There was just too much lung fungus for her weakened immune system to clear prior to transplant, even with the help of drugs.

It was at this point that Dr. Rooms, Lily's bone marrow transplant doctor, had a big decision to make.

Do I offer hospice to Lily's family, and let her go home to die?

Or do we send her to the surgeons to cut her chest open, spread her ribs, and dissect out the fungus balls, one by one?

Lily, Part II

We were all totally devastated.

Secondary AML, the disease Lily had just been diagnosed with, has about a 5-10% 5 year event free survival. That means, more than 90% of kids... don't really survive. Secondary AML is a killer.

Secondary AML? Secondary AML?? You have got to be kidding me! How can someone with such promise get a death sentence at 14 years of age? And handle it so well? Lily herself barely reacted; the shock itself was enough to numb her for days.

Lily entered the hospital for her first course of AML therapy in April 2010. This she handled like a champ; because of the high risk of a certain bacterial blood infection following the specific chemotherapy she was given, Lily was resigned to wait in the hospital until her blood counts fell to zero and rose back to acceptable levels. This period of time, in general, is about four to five weeks from admission to discharge. Because we give the next round of chemotherapy almost immediately after the blood counts have risen back up again, AML patients are usually in the hospital for around 6 months while they complete 5 rounds of chemo.

In secondary AML, however- because of the awful prognosis- we simply get the patients into remission and then go directly to bone marrow transplant. BMT: a procedure that, by itself, has a 50% mortality rate. Not a promising outlook. However, getting into remission usually takes- *we hope*- just one or two courses of chemotherapy.

Well, like I said- Lily got through the first round like a champ. Very few problems... our girl breezed right through it. We then checked her marrow.. not yet in remission. Darn. Because we knew she'd be in the hospital for such a long period of time, Lily was allowed to go home for a few days in between chemotherapy when she returned for her second course. It must have been a nice few days at home- but unfortunately she came back to us very ill, with a serious blood infection.

That was the last time she'd leave the hospital for months.

Lily, Part I

Nearly two years ago, 14 year old Lily was diagnosed with bone cancer. Not the "bone metastases" that old people get from other primary cancers, but an actual cancer of her bone cells- the kind that usually only happens in children. Lily first underwent a few cycles of intense chemotherapy to shrink the size of the tumor, which worked fairly well. The next stage of her "roadmap," as we call it- the path of things that has to happen to lead to a cure- was surgery.

In the old days of osteosarcoma, up until around 10 years ago, children had the entire limb affected by a bone cancer amputated. That was the safest way to ensure a cure, to know that you had gotten all the cancer. In more recent times, and luckily for Lily, orthopedic oncologists have devised a limb salvage surgery, in which the entire cancerous bone and any surrounding involved muscles are removed. The bone is then replaced with an allograft, or fake bone made of a strong, metallic alloy, and muscle and skin grafts from other places are reconstructed to ensure continued function of the salvaged limb. These kinds of limb salvage resections are so extensive that they take much longer to recover from than most other kinds of surgeries.

Lily had her limb salvage, and it was extensive. The first time I met her in early 2010, she had a long, two and a half foot, well-healed scar running down the shaft of her left leg. Even with the help of physical therapy, she was still struggling to recover the entire use of her leg. At that appointment, I distinctly remember feeling deeply sorry for her- 15 years old, and already disfigured. At that time I had no idea that the scarred leg was totally insignificant, and the worst was only waiting to come.

Following her limb salvage surgery, Lily had about six months of remaining chemotherapy. This was to ensure any possible microscopic cancer cells left anywhere in her body would be totally destroyed. Included in this list of life saving poisons was Etoposide, also known as VP-16. Etoposide has very few side effects, at the time it is given; nausea, vomiting, maybe some electrolyte abnormalities.

If you were to look at the chemotherapy chart found in our 10th floor work room, you would find Etoposide somewhere around the bottom third. Mechanism of action: Topoisomerase II Inhibitor. Most Likely Side Effects: Nausea, vomiting, etc. Unlikely Side Effects: Electrolyte abnormalities, nephrotoxicity, etc. Rare Side Effects: Small chance of secondary malignancy, acute myelogenous leukemia (AML).

Two percent. Everyone who gets Etoposide has a two percent chance, after being totally cured from their first cancer, of getting a SECOND cancer, leukemia. When we explain to parents that the chance of avoiding this second cancer is 98% percent, they almost always sign the consent to give it anyway. Lily's family would have been crazy not to. After all, two percent almost never happens.

But three months after she finished therapy for her first bone cancer, Lily walked into our clinic with low platelets, and walked out with a diagnosis of leukemia. Acute myelogenous leukemia, or AML.

To be continued...

Changing Course

So, I have been considering for some time how to share with all of you the stories of the children I laugh with, cry with, love and adore on a daily basis who have cancer and/or other blood diseases. Larry is the only person in my life, currently, who knows what goes on in my professional life and has the faintest idea about the 90% of my thought life that is devoted to these children and their struggles. Well, 90% is an overstatement... but its a lot.

I finally came to the conclusion that I'd like to give the kids pseudonyms, and tell you all their stories point blank on this blog. As I haven't used it for much else in the last three months, I can't think of anything better to do with this space than share the true stories, raw and completely unedited, of the hilarious, brave, pitiful, wise, scared, beautiful, unpredictable little people who have changed my life over the last year. Their stories might otherwise never be told.

Alongside this, I'm going to change the name of this blog- its original name, Sturey Mushe (pronounced stir-eye-mush-ay) is the Pashto greeting, "Don't be tired," and has now been outlived. Simbelmynë is its replacement: the flower that grew on the graves of the dead in Rohan, which to me represents that life and beauty endure beyond and indeed, in the place of, death. "How fair are the bright eyes in the grass! Evermind they are called, simbelmynë in this land of Men, for they blossom in all the seasons of the year, and grow where dead men rest."

Also, the title of each post will be the pseudonym given to the child whose story is going to be told below, otherwise unchanged.

More than anything, I want to tell these stories because they deserve to be told.

*Warning: some of these children live, and some of these children die.

Triumph to those who have overcome with life!

And all hail the victorious dead!