Okay, so this going to be a heavy one. Sorry in advance. September is both Childhood Cancer Awareness Month AND Histiocytosis Awareness Month. Most likely, you have never heard of Histiocytosis, and that’s cause it’s a REALLY rare cancer that mostly hits small children, usually infants and toddlers, and can show up with symptoms that mimic everything from pneumonia to eczema. It is Satan Incarnate in the form of cells.
I’m going to try to approach this with some humor, since in all honesty, even after nearly 8 years of facing this stuff down, I can either laugh about it or cry uncontrollably. No, I’m not unstable at all. ~shifty eyed look~
Okay, so background. My oldest, Sophia (Temperance/Tempee/The Divine Madame Sophie, Grand Empress Megadeath…. she chose that title, I am just the bringer of the good news that She has arrived.) was born in October of 2007. 6lbs 8oz, screaming, hairless, wrinkled little old woman with dark grey eyes and one HELL of a temper (no idea where that came from). She snuggled and loved her daddy instantly… me she took her time on. She had cradle cap nearly immediately and eczema we treated with lotions and cortisone. After 2 months, she started having blood in her diaper. From birth she barely ate, and vomited her breastmilk/formula constantly… even the $39 per teeny little container ubber hypoallergenic formula crap. ER visit after ER visit, we kept getting the same diagnoses: Milk Allergy/Anal Fissure/There’s nothing wrong with her.
We moved from NC to FL when she was 5 months old and the skin issues and bloody stools (sorry for those who are squeemish) kept going, getting worse. We went to numerous pediatricians for answers until finally, on August 1, 2008, I took her to this one total @$$hat that had the nerve to look me in the face and give me the diagnosis of “First Time Mother Syndrome.” You can imagine my response to that.
Okay maybe you can’t. I should have slapped him. But I was broken by then. I KNEW something was wrong with my baby girl. I sat in the parking lot and sobbed. No one was hearing me. They thought I was exaggerating her symptoms. I was told I was “being dramatic,” that “babies just do that.” No… They don’t. I googled Tallahassee Pediatricians and found another pediatrician that hadn’t told me I was insane. Dialing the number, the nurse told me that they didn’t want to see her until she had a well-baby check so they had a baseline to compare her to. My response: “She won’t be a well baby if someone doesn’t figure out what is wrong!” I think I yelled it. I was still sort of hiccuping. I’m sure she thought I was nuts. Happily she decided to let the crazy woman hear that she was crazy from the doctor the next morning at 7:30.
August 2, 2008: We got there at 7:25 a.m. The doctor came in and started to palpate Sophie’s tummy around 7:45. She got REAL silent. Then she said she’d be right back. Came back in with another pediatrician who did the same thing. They were both real quiet. They left the room AGAIN. At this point I’m panicking. The first pediatrician came back in the room about 10 minutes later to tell me that I needed to get my little girl to the Shands hospital 2 1/2 hours away from Tallahassee, by 1:30 that afternoon, to the liver clinic. That her liver was enlarged, as was her spleen, and that the clinic team at Shands wanted to see her. We drove to Shands, went to the clinic, and the Doctor palpated her tummy and again did the same thing. Leaving the room, getting another doctor…. what the hell is that? Do they take a “how to be dramatic when palpating a stomach” course? I mean… come on. All I got was “We need to run tests. We want to admit her today.”
We stayed in a long skinny room with a tiny window and a tiny metal crib for 9 days while they did biopsies, endoscopies, CTs, MRIs, everything you can think of. The long and short of it, by August 11 was that she was in liver failure and no one had any idea why. Her liver biopsy showed little whirls of white blood cells, so maybe it was a new strain of autoimmune hepatitis? Maybe it was some other autoimmune process? No one knew. BUT they put my little 9 month old baby on prednisone and numerous other liver support drugs in the hopes that something would stop whatever was going on. She was turning yellow from the bilirubin in her blood stream. We were told to pull her from daycare cause the dose of prednisone made her immunosuppressed, so we did. We gave her the meds, and continued to see horrible labs. In September we went for liver clinic and were told that they were scheduling her liver transplant evaluation. She needed a new liver. This whole time everything is a blur to me. Sometime in September, before her first birthday, they did the evaluation, CTs with contrast, ultrasounds, more biopsies, more endoscopies, visits with the family psychologist and a financial advisor and social workers and all sorts of people. Sometime in mid-November we got the call that she’d been put on the list. I can’t even remember what her PELD score (Where she was on the list) was. This was all just a huge blur of medications and clinic visits and hospital stays and I’m honestly surprised I managed to keep it together to get to all the appointments.
During all this, I’m working full time at the Department of Health, 4 ten-hour days a week, and Daniel is staying home with our kiddo. He had to give up working full time at his job at Envision Credit Union (who actually put him on as a zero-hour employee, allowing him to get what hours he could to help us afford life, so he worked on the days I took Sophie to the doctors and during the weekend). He gave up a dream for our baby girl. Everything is one huge terrifying blur of loss and pain and financial hell and not knowing what to do or who can help. SSI turned us down, despite Dan having to stop working cause of our daughter’s disability. I’m still wondering why I’m paying taxes for disability after that.
November, 23, 2008, the day before Thanksgiving, I took Sophie to liver clinic. On the way home, the clinic attending physician called me. They had results from a smear they ran on her liver biopsy from back in August. On a whim, some doctor that had never looked at Sophie before said “Hey, could it be histiocytosis?” during one of their meetings. BAM, the smear said yes. She had Multisystem Langhans Cell Histiocytosis. (say that five times fast.) Or LCH. She was going to need chemo, but no, it wasn’t cancer. (back in 2008 it wasn’t yet classified as a cancer, despite the fact that it was jacked up cells trying to kill her body, and the fact that she needs the same chemo that kills cancer and lots of other stuff… stupid if you ask me, but they were all like “the cells don’t look like cancer” and I’m the mom over here like “you just put my kid on 21 months of chemo to kill something that might kill her. What you wanna call it? a cold? Dumb@$$es.”) Well, because at the time it wasn’t cancer, we didn’t have access to any of the services that pediatric cancer patients get access to. Yeah… I have so much anger from this time period still. You wonder why I listen to metal? This is why I listen to metal. Anyways, I was driving as the doctor was telling me her diagnosis. I couldn’t even pull over on the highway with my baby in the back seat to write it down, so my brain clung to the one thing it heard. “histio.”
I called my mom once I got off the phone.
Me: “Mom, they have a diagnosis.”
Mom: “GREAT! What is it?”
Me: “Histio something. I didn’t get the whole thing. She said it was whirled white blood cells. Can you look it up and tell me what it is?
~clicking in the background~ Mom: “It’s okay. It’s a white blood cell disorder. It looks like 6 weeks of a type of chemo and they have a good chance of killing it off.”
LCH is where the histiocytes, a type of white blood cells, goes wacko and starts to group up and form lesions on the skin or large tumors or lesions in the organs/bones/brain. Sounds like cancer, huh? We didn’t know what it really was. Just the preliminary stuff. Most kids with Histio get it on the skin, and it looks like eczema (Sound familiar?) Prednisone might make it go away if it’s just skin involvement. Vinblastine and a few other lighter chemos for 6-8 weeks will usually kill the skin LCH that prednisone doesn’t kill. But not for multisystem LCH. Multisystem means it’s more than just on your skin… it’s in your bones, your organs, your brain, ear, everywhere. Well Sophie had it in her organs. She had multisystem LCH.
And the normal treatment doesn’t usually work on multisystem LCH. They try the normal stuff, but more likely than not, multisystem LCH won’t respond and the doctors will classify those who don’t respond as “relapses” or “resistant.” Well no shit it’s resistant. THIS kind of LCH isn’t going to respond to your little water pistol, gentlemen. Get out the big guns. And in my opinion, get that gun out first, cause your water pistol is just wasting my baby’s time and putting harsh chemicals through her already struggling liver. (long story, long argument, I didn’t win this argument… they did the water pistol anyways).
The next day, on @&*%#&$#ing Thanksgiving, the liver team called us to tell us they wouldn’t transplant her liver cause she had an “active malignancy.” AKA, if her liver didn’t survive the chemo and the cancer until a year post remission, too bad, so sad… But it’s if you talk to the oncology team, it’s not cancer, folks. It’s an “active malignancy” that is “not cancer.” Someone explain that to me? I just wanted to call bull$hi+ in the loudest loudspeaker made at the building. It was clear the liver guys weren’t talking to the cancer guys at that hospital. Which, by the way, is the story of the last 7 years of my life. Liver guys? Talk to oncology? Not without a gun to their heads. And the oncology guys act like they aren’t even aware the liver guys are alive. It’s very reassuring to deal with these people.
We were ordered back to Shands on December 2, 2008 for an inpatient stay to insert a port and start chemo and get all the information they needed. We get there, my baby girl is in her little long sleeve, long pant, cherry covered sleeper with her little puffy-prednisone-moon-face and her big eyes and terrified. I’m terrified. Daniel is holding her and being strong, but he’s pretty much stopped talking to anyone about anything substantive since August.
On one day, they put her into surgery, got her port under her skin, leaving a permanent scar on her chest the length of my thumb, did a contrast CT of her entire body and a bone scan. End result? She has it in her liver, spleen, GI tract, and pituitary gland (in her brain). Yes, all those places. They started the chemo that day. It was LCH III protocol. Vinblastine with a cocktail of other lower dose chemo and prednisone. The water pistol of chemo for multisystem LCH. The side effects suck: nausea, headaches, weight gain, water retention, nothing tastes good anymore, neuropathy, weak bones, etc…. And this was the water pistol chemo, ladies and gentlemen. This was the stuff that DIDN’T WORK. By the end of the month, we did another scan, labs, etc, and they didn’t even wait for the full 6 weeks pass for them to tell me it wasn’t working, she had a “poor prognosis.”
Let me go into that a bit more. Since November 23, I had been researching like a PhD student desperately trying to finish that dissertation. I have dug into LCH. I know what the studies say about multisystem babies. I know that if she doesn’t respond to LCH III protocol, she’s in the “poor prognosis” group. And at that time, that poor prognosis group had an 18% survival rate cause they didn’t really have another chemo protocol to deal with those who didn’t respond. There was no $$ to research chemos to figure out how to stop it cause LCH and all other histios are what’s called an “orphan disease.” Meaning it’s got no funding. So my kid might die because the idiots in charge said it’s not cancer so it gets $0.00 for research from any cancer foundation.
Top THAT off, our normal oncologist, who is a saint and going to be canonized upon his death, wasn’t there for that oncology clinic to deliver that particular “poor prognosis” blow. Instead we got the heartless oncologist who specialized in some other pediatric cancer. She has NO idea who Sophie is, doesn’t know LCH at all, and just dropped the “poor prognosis” bomb and walks out of the room, leaving me clutching my baby and sobbing and rocking. This whole thing, though, just had Sophie confused as she played her fingers in my hair. That’s a moment that will forever be etched into my soul. That woman needs about 800000000 hours in bedside manner classes. ~growl~
Sooooooo, we yet again go home, thinking my baby girl is dying. They ordered us back on January 2, 2009, for another chemo…. something experimental. What they were calling the “salvage protocol,” which gave me so much confidence about what they were doing, gotta say. Like they were trying to salvage anything they could out of the treatment. Bad name. She was #37 in the world put on it for LCH (per our oncologist).
January 2 was when we moved into the hospital for two months. The next two months are a blur of chemo and morphine drips and arguments with attending doctors about washed blood and chemo doses and when she can eat something and scans. Thank God for her primary liver specialist and oncologist. If it wasn’t for them, no doctor in that building would have heard a word I said. The nurses were incredible, but I have grown a generally horrible opinion about the whole M.D. profession except for a few who had to EARN my respect. The chemo killed her bone marrow… 0 white blood cells for 19 days. That means no immune system for 19 days, people. Nothing to fight off so much as a hang nail.
Her liver took such a hit her bilirubin hit 19.9 (it’s supposed to be below 1) and she was the color of a banana boat suntan lotion model or a basketball… depending upon how generous I was feeling that day. I got sleep in 1 hour spurts between vitals and doctor visits. She got infections and was so sick. She had bloody stools and bleeding gums and so much vomit. She had such bone pain they put her into a morphine induced coma to deal with the pain cause she couldn’t take it. She lost her hair and weight and her skin looked grey. There were nights she cried for hours straight as I rocked her. Daniel was working full time in Tallahassee during the week at this point to try to get extra $$ so we could afford things like electricity and trash service at the house. He came down to Gainesville on the weekends to be with us. One of my coworkers went around my office asking for donated leave for me to keep getting paid. People came to visit, but I tended to not let anyone in her room cause she had no immune system. I cleaned the place myself, sanitized everything, found my inner OCD b*tch and fed her neurotic self with bleach wipes and antiseptic floor cleaner and all sorts of other fun.
There were days that were great and she played. One day I went into the en suite restroom and came out to find out that my 1 year old baby figured out how to unclip the alligator clip holding the fluids to her port access, causing blood to back up and out of the port access…. aka I came out of the bathroom to find my one year old finger painting in her own blood. I screamed so loud the nurses heard me on the next unit, but it wasn’t much and she was fine, and after suppressing their laughter, the nurses were able to peel me off of the ceiling and get her cleaned up. I think they were wondering whether I was going to end up on the “special care” floor (where they send those with mental breaks). Cause Dayum, I was close.
Sophie will never live down painting in her own blood. Sick sick child. I have no idea where she gets her sick and twistedness from. ~innocent look~
On February 24, 2009, we got a clean scan. It was all gone. Her liver was still shot, and the liver team wouldn’t transplant her for a year post “remission,” which our oncologist listed as the date of the clean scan, so we had to make it a year before she could be listed for that liver she needed so badly, but the CANCER WAS GONE! Because resistant multisystem LCH has an 86% relapse rate, they put her on step down chemos for the next 20 months. two months high dose 2cda, 6 months, one huge pile of oral chemo cocktail, 14 months another, lower dose of a huge pule of oral chemo cocktail. Her liver numbers slowly dropped down and she slowly recovered. Her liver was still huge. She still couldn’t go to school. Dan still stayed home with her (we tried the full time at home nanny thing… what a frickin’ nightmare.)
In July of 2009 she had a black poop in her diaper. We knew from the liver guys that she could have an upper GI bleed as a result of her liver disease (long explanation that boils down to varices in her esophagus. You don’t want details… or if you do, google it. I’m not going down that road in detail right now, I can’t think about it anymore). I took her to the pediatrician the second I saw the bloody poop, knowing what it could be, and it tested positive for blood, but the labs showed she was in the okay range with her red blood cells, so they didn’t put her inpatient. Until that night. We ran to her room to her screaming, gagging on what looked like grape jelly that was spewing out of her mouth in huge clumps. She was bleeding internally.
I grabbed her, blood and stomach acid and all, and we ran to the car, put her in (my hands were shaking so much Dan had to buckle her in). 90 seconds down Miccosukee Rd going 90 miles an hour with the blinkers on to the ER. Grab her and run in the ER screaming that she is a Shands cancer patient with extensive liver damage and she just vomited a ton of blood. We were rushed to the back. I don’t even remember most of this. There was blood, she was given a transfusion I think, labs kept being taken, she just laid there and I was trying to not cry cause she was watching me. Dan was pacing a hole through the floor. I can’t even recall if we called anyone. They came in and said they had to fly her to Shands, that the helicopter was too small for us to go with her, we’d have to follow behind her. She had a 45 minute flight, we had a two and a half hour drive, and I swear I could have sobbed when that helicopter nurse called and said she landed and was safe and in the hands of the pediatric liver team. The rest of the drive was just balled up fear. Never want to do that again.
That was her first bleed. Endoscopy led to banding of huge varices and we were back there every 4 weeks for months for the same thing. After that first bleed, there were permanent burns in the surface of our hardwood floor around her crib where the acidic blood burned through the wood. I’m so glad we sold that house. I hated those marks.
So one of those follow up endoscopies that we had scheduled didn’t turn out to be a standard scheduled outpatient procedure where they found a number of varices and banded them without complications.
Funny thing about endoscopies, the patient has to be NPO, meaning no fluids or food from midnight. Normally she was the first patient handled the next morning. But this one time she was bumped. Well we were old pros at this NPO thing by then given all her previous surgeries and endoscopies and biopsies; we knew that Sophie on NPO means she’d pop a fever when dehydrated, but if they just got her port hooked up and fluids flowing, her fever would instantly drop.
Usually fever in an oncology patient is an automatic admission and hospital stay, but the oncology team was used to this with Sophie by now, so they didn’t even bat an eye. The liver team, or the head of the liver team at the time, however, REFUSED TO LISTEN TO ME. (When will these guys learn??) So this one endoscopy Sophie is bumped from the first in line for an emergency. No problem. I asked them to just hook her up to fluids, but no one would do it without the doctor okaying it, and he was the king of the egomaniacs. I kept telling them she’d get feverish without fluids. But nooo… that’s not normal. I couldn’t be right about what I was saying. So no fluids.
By the time they get to Sophie, she has a low grade fever and since she’s an oncology patient, Dr. Egomaniac refuses to touch her, instead canceling the endoscopy to band off the varices, despite her bleed history, and sends us over to oncology. At oncology, they listen to me like they can’t believe what I’m saying, hook up her port with fluids, get her temperature down, and call the liver guys to see if they can do that scheduld endoscopy since now someone listened to us. But by then they don’t have an opening in an OR for the rest of the day. That liver doctor had the NERVE to look me straight in the eye and tell me she wouldn’t have a bleed between that day and when they rescheduled the endoscopy.
She had a bleed two days later. @$$ hat nearly killed my baby. He hasn’t touched her since, and if I have my way, it will stay that way. I’ve told the hospital to put on her records that he is not permitted to touch her. Ever.
Anyways, loooooong story short, over the next 3 years, the liver team (not Dr. Egomanic) got her varices under control with constant scheduled endoscopies. Her liver lab numbers started to look good, she started to grow, she ate and her blood counts recovered (though not all the way. She’s likely to always be immunosuppressed… the chemo did a number on her bone marrow). Her liver is stable and not needing a transplant right now. The damage is there and permanent and she has portal hypertension and can’t ever do contact sports, drink alcohol, and has to be careful with medications for her life. Her spleen is huge, and always a rupture risk, but we keep her from anything where she can get hit. Her platelets are low. And we still don’t know if the damage is so extensive that her liver can’t keep up with her growth during puberty, but we will cross that bridge when it comes. If it was too damaged, she’ll probably have to be transplanted then, but I’m optimistic. Her labs look so good that I just can’t see that the liver is going to fail in the future.
Here we are. Sophie is 7. Going to be 8 in October. And she survived. She was lucky. I should say we were all lucky. I still have my baby. I can’t believe, looking back, that we were that lucky. It was like walking through the caverns of hell holding your child and wishing you could throw yourself in between her and the flames that have you surrounded. And sometime in 2014, the “experts” decided it was cancer. Finally.
I am a member of an LCH support group (Hey there Histio Moms, Dads, and Warriors!! ~waving~) and a Pediatric Liver Disease support group (Hey there Liver Families Peeps! ~Waving~), and I have watched as many little ones weren’t so lucky. Histio has taken so many innocent lives. Liver Disease as well. It’s not okay. It’s not funny. It’s just plain wrong.
And what are we, as a society, doing about it? 96% of all cancer research funding goes to adult cancer research. ADULTS. Did you know that most adults that get cancer are over the age of 60?? So Grandma and Grandpa are getting the $$ for research. But my baby and countless other babies… tough luck. So sad. Let’s feel bad as we look at the pictures of the little pediatric cancer patients, but do we do anything about it??? Grandma and Grandpa have LIVED. My baby nearly didn’t. So many other babies don’t. No mother should have to bury their child. None. It needs to be FIXED.
I remember sitting in the hospital next to Sophie’s crib while she was in the morphine induced coma for her bone pain… listening to the machines beeping her heart rate and respiration rate or something. Holding the front door key to my house. Sophie had picked it out at home depot before we went inpatient. It had a huge Tinkerbell on it. I hated Tinkerbell. No one is that damn chipper. But that key became sacred to me because it might have been one of the only things she ever chose for herself. I sat there and wondered if it would be one of the few things I had left of my baby, cause I didn’t know if I’d leave that hospital with her. Walk in the door with my amazing, sweet, loving daughter, walk out empty handed. That’s the visual I kept having. No one should have to go through that.
So when you think of cancer awareness or histio awareness, think of the babies that didn’t get to start kindergarten. Think of the ones that will never graduate 5th grade. Would they have their mothers blonde curls? Would they have laughed like their daddy? Would they have grandma’s little eyebrow movements? They will never have their first dance or graduate high school or have prom. Daddy will never walk them down the aisle. College or technical school won’t be something they have to figure out. Having their own babies? No. Because of cancer. Because of tiny little cells that we have to figure out how to stop.
Sorry, got heavy there. It’s a heavy topic. I tried to be funny, but I think, honestly, I’m still so angry and sad and hurt by the whole thing… I don’t think I can have humor about it. Sorry. I tried. Stay tuned… I plan on having ice cream tonight and tucking this all back into that horrible little black box I keep it in. Tomorrow I will likely be the snarky smart@$$ that you have all grown to kinda like a smidge.
Update: I was asked to post where donations to charities for the research of histio could go. Originally Histio Heros Research Fund was the place I sent people, but they merged with histiocure.org back in 2013, and they have donated $1 million to research on histio in the last few years (since it is magically considered cancer now). This money goes to Dr. Ken McClain at Texas Children’s Research Hospital. Dr. McClain was the oncologist that consulted on Sophie’s case with our oncologist at Shands. If it wasn’t for McClain’s research and participation, Sophie wouldn’t be with us today. So please, go to http://www.histiocure.org and give freely to keep this from happening again. Please go and help research proceed that will stop this horrible disease. Thank you for everything you do to help spread awareness and find a cure.
Update May 2018: Sophie now demands to be called Tempee. She’s nearly as tall as me at 10 years old and is gorgeous and funny and witty. We home school her because the school system here couldp’t show us that she was safe enough at their schools to go with her low platelets and enlarged spleen. Her liver numbers look great, even with her growth spurts. We are checking her liver and spleen each year to make sure that the portal vein is okay. And we are still so super lucky. It’s hard to look at that little picture of her for her first biopsy and not remember the sound of the machines. But I can look up and see her today as such a beautiful young woman and I’m thrilled.