I put a post on the Team Pooters Facebook page to see what people would want me to talk about. I got a lot of great answers. Some asking about the medical side of things and some asking about the personal side of things. I decided that I would start with the medical side of things and then do a personal side on a later post. I think the personal stuff is harder to write about, you know? So I’ll go with the medical side of things which is easier to write about and also, because I think it’s important that people know what this disease really entails.
I get asked a lot about how we found out she had cancer. What it boils down to is constipation. She couldn’t poop. She couldn’t bend over and pick up a toy because she hurt so bad. The thing is though, it happened so suddenly. I now know that her tumor had just reached the point that it was pushing on her windpipe and also in her abdomen which was causing her pain. I have been asked how did I not feel she had a tumor, hell I still feel guilt about not finding it sooner. The truth is though, is that her tumor was growing through her ribs from inside her chest and there was never any visible signs of it. Of any of them. The one on her kidney was growing downwards and not out. We had taken her to the doctor -four- times before she was diagnosed…four times. No one could feel it, even after they knew it was there.
The first thing they do after diagnosis and all the initial testing is to start chemotherapy right away. We started the next day even before her bone marrow or biopsy. We were told that the treatment wouldn’t change which seemed very odd to me at first, but now I understand it. Katie-Anne is on COG protocol (Children’s Oncology Group). There are several different protocols in the world and it depends on which hospital or doctor you go to. COG protocol starts with six rounds of different combinations of chemotherapies. For Pooters doxorubicin was the worst. It is known as the red devil and for good reason. It legit looks like a small red bag of Kool aid hanging there but the side effects were horrible. Mostly for her was mucositis. Mucositis is the painful inflammation and ulceration of the mucous membranes lining the digestive tract. Basically horrible sores in the mouth and digestive track. Katie-Anne had them mostly in the lower digestive track. It kept her from eating or drinking and made her so sick and miserable. The second worse one irenotecan, also known as “I run to the can”, also known as horrible diarrhea.
Katie-Anne also had her stem cells harvested. I think the official name is peripheral stem cell harvest. Sounds very exciting doesn’t it? At the time it was pretty traumatizing. All of this was very new and I have to say that looking back, it was one of the easier parts of all this so far. We gave her a shot called G-CSF for a week before. This made her bone marrow start producing so many stem cells that they get pushed out into the blood where it gets run through an apheresis machine that looks a lot like a dialysis machine. It took a few days and we got enough to do 13 stem cell rescues if needed. To date we have used two. Here is a picture of what the line looked like. It’s huge.
After stem cell harvest we did two more rounds of chemo and had her first of two surgeries. The first surgery was in her lower abdomen to resect (take out) the big one on her kidney, the lymph nodes, and any other small tumors they saw. The big worry was that the tumor “on” her kidney looked to be growing out of it so they thought they were going to have to remove part, if not all, of her kidney with the tumor. It turned out though, that the tumor was only pressing against her kidney and peeled off like, well… I don’t know what like. I was going to say grape but that doesn’t make any sense. It just peeled right off and they didn’t have to take any of her kidney. They got the nodes and a few little tumors. All in all, a huge success and she bounced back very quickly and now sports a sad face frown on her tummy. Here is a post operation pic from that surgery.
We finished up chemotherapy with the last two rounds. Then came surgery number two. This is the biggest one and the hardest for me. The tumor was growing out through her ribs. Dr. Murphy thought that he’d have to take about three ribs out. How do you send your baby to have ribs taken out. The original plan was to put a sort of tyvek material over where her ribs were to protect her internal organs. You know…like her lung. After surgery he came out and explained that her took ribs 1-5 out and part of her sternum. I was in shock. This seemed so impossible to me. He connected her clavicle (it’s usually connected through tendons and such to rib 1) to the top of her remaining sternum with wire, just a piece of tiny wire. He also did the same to rib six to the bottom of her sternum. Instead of tyvek they used pig collagen to fill the hole. The thought process behind this was that they tyvek might break as she grew and would require another surgery later on. The pig collagen would form scar tissue that would grow with her and also form a thicker protection layer to her now more exposed and open organs…again, like her lung and stuff. I cannot tell her how frightened I was and how guilt ridden. I truly felt that I had handicapped her for life with this, but anyone that has met her knows that you can’t even tell unless you know to look for it and even then, there is barely an indent. The other concern was that he was disrupting and shaving off some of the underlaying muscle that was against the tumor and that her breast buds might be affected. This means that she would not have grown a breast on that side. Happily though, he didn’t have to touch it and she should develop normally. On the rather funny side, if she developed like I did, then she might be too big and need another surgery for some kind of support of reduction. I have never prayed more for small boobies in my life. They has anticipated a week in PICU and another two weeks on the surgery recovery floor. She was out of PICU in three days and home day 8. She is a beast I tell you. Also now she is part bacon. Seriously, part bacon. Here is a couple of pictures post surgery.
After surgery is usually Stem Cell Transplant. This is where they give the patient such strong doses of chemotherapy that it completely obliterates the bone marrow. Katie-Anne never had this (of which I am glad because it is horrific) because her curie score (the number of “spots” of neuroblastoma) was too high. She was still at about 9-10 and they want you to be at less than 5. So we went off protocol and moved on to Antibody ch14.18. Immunotherapy is a type of treatment that helps the immune system to fight cancer. The ch14.18 antibody specifically targets a substance called GD2 that is found on the surface of neuroblastoma cells. When the immune system detects the presence of the antibody on the cancer cells, it responds by trying to kill the cancer cells. The thought was to have Pooters do two rounds to see if we could clear enough spots down to go to stem cell transplant. The thing about ch14.18 is that it doesn’t only affect the cancer cells but also the pain receptors. What the heck does this mean? It means that it cause pain while it is being infused which requires a morphine drip to control the pain. There are other side effects like capillary leakage, itching and low blood pressure. For Katie-Anne these first two rounds were handled relatively well in comparison to most kids. We did have a lot of itching and some pain. She got puffy but had no capillary leakage that anyone could tell me. The hope was that it would get her spots down but it didn’t, not really. We saw great response the first first round and then nothing the second. Because of this, it was decided to go onto mIBG therapy.
mIBG is a test you may have heard me talk about. An mIBG Scan is a nuclear medicine scan which involves an injection of a liquid called meta-iodobenzylguanidine – mIBG for short. The mIBG then attaches itself to the cancer, specifically neuroblastoma cells so that the special scanner can see where the neuroblastoma is. Some amazing doctors somewhere in the world saw potential in this and came up with mIBG therapy. They got the idea to attach radioactive iodine (I-131) to the mIBG to deliver targeted radiation therapy to try and kill the neuroblastoma. Think of it as really really really tiny Trojan horses. Radioactive ones. The thing with mIBG therapy is that she became, well, radioactive. She was put into a lead lined room with movable lead shields. Because she was radioactive , we couldn’t be in the room with her. In fact we got about 5 minutes every hour or so. We could see her through a window and had a com system to talk to her through. We had to wear a special suit and a Geiger counter which was monitored very closely. In addition, after release, she couldn’t be around her 1 year old brother for about two weeks and we couldn’t really cuddle with her. I failed at this miserable btw and now have had twice my life time worth of radiation. We also had papers to carry with us in the car because apparently police vehicles (some) have radioctive detection devices and if you get pulled over, you have to be able to prove it is her and not a bomb. Anything in the room with her in disposed of or put into a basement lead tank for six months in case you want it back. Crazy right? She handled this very well for a 2 1/2 year old with the help of a few drugs. Here is her in her mIBG suite. Notice the 3M everywhere? That is to protect the walls because if any of her body fluids got on the wall, they would have to cut it out and replace the whole wall.
We did two rounds of mIBG and then went back to antibody ch14.18. We didn’t see the response that we had hoped to from mIBG and the hope was that going back to ch14.18 right after mIBG would help boost the effects that mIBG could produce. We never did see that huge reduction in spots but it seemed to have worked gradually over the next 13 rounds.
In total she did 15 rounds of ch14.18 and we scanned often. Today they think her remaining disease may be mature but there is still more work to be done. We are heading to New York to Sloan Kettering for their antibody treatment, hu3f8. It is completely humanized which means that unlike ch14.18 (the ch means chimeric) which is partly made of of mouse stuff as well as human stuff, hu3f8 (the hu means humanized) is all human stuff. It’s suppose to be stronger. See the thing with neuroblastoma is that you want to throw everything you can at it. We never did make it to stem cell transplant because the mIBG had the same effect in obliterating her bone marrow. She had to have two stem cell rescues to help her marrow rebuild.
Holy crap what a lot of information. I know I’ve left some stuff out and possible gotten some of it wrong, but gees what a lot to digest. It’s kind dry I know, but there it is, her treatment, her medical side. It’s been such a long road already, almost two years, with lots more to come. But hey, that’s just how it is when cancer happens.
PS. One more picture of her right before she was diagnosed. I can’t think of a better place to put it.