Friday, September 5, 2014

So it's been quite a while....

It's been 16 months since our last post. I apologize. We rely way too much on Facebook. I will try to summarize.
Cam continued on 5FU over the summer of 2013. Robin, Caitlin and Campbell attended a national sales meeting for Brooks Brothers in August 2013 to speak as a St. Jude family. Cam was to be featured in the St. Jude Thanks and Giving Campaign, with a focal appearance in Brooks Brothers catalogs, on their website and in their stores. Cam's grandfather works for Brooks Brothers, so her story really hit close to home. That was an amazing experience.

A few weeks later, Robin and Campbell went to Memphis for Cam's Cycle 3 scans. They showed mixed results. Some tumors were continuing to shrink, some were stabilized, but unfortunately one had grown significantly. Cam was no longer eligible to continue on the clinical trial at St. Jude. We went home where Cam's amazing neuro-surgeon, Dr. Jeffrey Greenfield, removed the growing tumor. It was a successful surgery, removing all of the tumor. Around this time, Dr. Greenfield's research lab was receiving federal approval to perform full genetic sequencing on cancerous tumors. We were offered this service as part of a research grant. We jumped at the opportunity. This should provide just the information we needed to help guide our next treatment choices. While we waited for those results, Cam would continue on 5FU off-trial, under the sole care of MSKCC, since the remaining tumors were still responding.

In November 2013, Cam had another scan which showed her compression fractures in her spine had worsened. Surgery was discussed, but deemed not an option. The surgery would have involved rods and screws. The screws would need to be anchored to good bones to stabilize her spine. Cam did not have any good bones in her spine. She had the bones of an elderly woman suffering from osteopenia. She was started on high doses of calcium and vitamin D3 and an IV drug (received monthly) to help with calcium absorption and increase bone density. She went from a night time scoliosis brace to a day-time TLSO brace that kept her torso immobilized. Around this time, because of the increased risks the fractures posed, we made the difficult decision to pull Campbell from ballet classes. She was devastated.

In December 2013, Campbell had her next full brain and spine scan. Unfortunately another one of the tumors had started to grow. It was pressing on the brain stem and causing symptoms. This surgery was much trickier, even trickier than her first surgery, simply because of the location. It basically was two tumors that had each grown and had merged into one tumor with a large blood vessel for the brain running through it. The surgery required two different entrance points and two different surgeons. Dr. Greenfield and Dr. Souwedaine tag-teamed it. The surgery again was considered a success, although due to the location of the tumor(s), there were two cranial nerves permanently damaged. This resulted in an eyelid that did not close fully (causing dry eye and corneal abrasions) and an eye that did not move at an even rate as the other eye. Regardless, we were working with a neuro-opthalmologist at Weill-Cornell who assured us that with patching the tracking problem could fix itself and that the eyelid problem could be surgically fixed at some point.

Cam came off 5FU. We were still waiting for the results of the gene sequencing of Campbell's tumors (the January 2014 tumors were added as well). Campbell tried a different drug, based on some lab studies done at St. Jude, gemcitabine during February and March 2014. It did not work.

In February 2014, Cam began complaining of occasional double vision. We tried patching to help this (you patch the weaker eye to improve the muscle), but it frustrated Campbell because she simply couldn't see as well out of the bad eye because of the corneal scratches. Everything was hazy and blurry. It just wasn't a fight worth having. We started discussing prism glasses to fix the double vision.

In March 2014, Cam was becoming increasingly irritable, having short-term memory problems and headaches. The symptoms were severe enough to do a brain scan early. Cam had a large tumor growing back in the surgical site from September 2013. The surgery was another total resection, however we knew that until we found the right drug or drugs, these tumors would continue to grow back. Even a total resection leaves microscopic cells behind. Also in March, the sequencing results came back showing one new drug as a good option. Catch was it was in adult clinical trials. Drugs have to be trialed in Adults before Pediatric Clinical Trials will even be written, let alone enacted. Campbell's neuro-surgeon applied for compassionate use of BKM120 (buparlisib). We knew the approval process would likely be several weeks. We needed to try something else in the meantime. We enrolled Campbell in a single agent study at MSK of a drug called perifosine. Again, it did not work... Cam needed a VP shunt to manage overall CSF absorption issues caused by the multiple surgeries and radiation treatments. The shunt was placed in April 2014.

May 2014, compassionate use approval comes through (thank you, God). Cam starts on buparlisib. It was well-tolerated, with minimal side effects for Campbell.

However, at the end of June 2014, buparlisb was shown to not be working. A tumor never before removed had grown significantly, causing slurred speech, mood alterations, and short-term memory problems again. The brain stem tumor was also growing. Even worse, it was growing into the brain stem, rendering it impossible to remove the entire thing. At our request, Campbell's neuro-surgeon removed one of the tumors, the one causing the most mood disturbances in her frontal lobe. He was able to get most of the tumor. A small piece was attached to a major blood vessel in the brain and had to be left untouched. We had a few more treatment options to choose from, including one that was being created based on Dr. Greenfield's gene sequencing.

We had a good family trip to Long Beach Island the week of the Fourth of July. Cam, Greg and Robin had a brief day trip up to Tufts Floating Hospital for Children to discuss the personalized multi-agent protocol Dr. Klement had created for Campbell. We went home from the beach with two plans to choose from. We opted for the plan we could enact faster - a two agent protocol created based on research at Sick Kids Hospital in Toronto. We hoped that this combo of azacitadine and Vorinostat would stall the growth or even cause the brain stem tumor to start to die, making it easier to remove or at least debulk surgically. We were keeping the Tufts plan in our back pocket since it would require alot of insurance hassle and timely appeals for costly oral medications not previously proven for ependymoma. This is one of the many problems with Childhood Cancers - there are few, and in the case of Ependymoma, no, proven drug treatments. Insurance companies will only approve a costly treatment if there is proven medical benefit. This is the catch 22 childhood cancer families often find themselves in. You have a cancer that there is no proven treatment for, so we need to get creative and guess based on what might be causing your cancerous growths, but insurance companies do not approve treatments based on these growth reasons. They approve drugs that have been proven effective in clinical trial.

By the end of July, Campbell's status had continued to deteriorate. She had stopped speaking, communicating solely by squeezing your hand or blinking her eye. One eye was permanently closed. She had stopped eating. She could not support the core of her body sitting or standing. She could not walk. She was sent home from MSK on hospice on July 26. Robin started sleeping with Cam at this point. We didn't want her to be alone or scared.

The next week was a whirlwind. The Power of Team Campbell came into play once again. Cam and the family got to meet Harry Styles and the other members of One Direction, with upgraded VIP treatment at their concert at Met Life Stadium on August 4. It was amazing! It was so incredible that Campbell even started eating again the next day, not much, but it was baby steps!

The plan was to start the next cycle of the two drug protocol on August 11, with her next set of scans on August 27. We started the next cycle with Cam starting to do a little better. As usual, she continued to amaze people. As the week progressed, Campbell's status deteriorated again. She had stopped eating and was barely squeezing hands in response to people. She would have moments of alertness, but slept nearly all day.

The morning of August 17, Campbell had her first seizure. It was terrifying. Before the seizure she couldn't move anymore; she couldn't talk. I was laying in the trundle bed next to her and heard funny sounds, almost likely gurgling coming from her. I was concerned the chemo was making her nauseas and she had vomited and was now choking on it. She was awake. Her eye was open, with a terrified look in it. I tried to open her mouth and move her onto her side. Her jaw was locked closed. I turned her on her side and started screaming for Greg. I didn't know what to do. I vaguely remembered the hospice nurse mentioning that Ativan (which we had for the mood disturbances) was good for seizures so we slowly got that into her mouth. We called hospice and decided to keep our chemo appointment at MSK. We went in for the last dose of azacitadine. We came home on a higher pain patch with the goal of just keeping Cam as comfortable as possible, and most importantly not scared. Campbell did not regain consciousness after the seizure on August 17. She passed in her sleep at 11:55 pm on August 21, exactly 5 years and 1 day after being diagnosed with Ependymoma of the Brain.

This is the worst update I've done yet. There was so much information to pack into one update, including the worst possible part of the story. Campbell Grace Hoyt has left an impression on this Earth that will far outlive her short beautiful life. She was laid to rest on August 26, 2014 at the age of 8. No child should die. There is a way to stop this madness. We need more funding, more research... but that is a post for a different day. Right now, I just needed to get the facts out there. Emotions and pleas and anecdotes will follow at a later date.