Friday #GoHugo and an Update.

Here is Marianne's most recent update (Dec 9th) on everyone's favorite trooper:

This is Hugo on his first birthday, June 5th of this year. Today marks six months since a tumor was found on his kidney, and I thought I would give an overview of his diagnosis and treatment to date, to answer some questions I've gotten. It's a long post!

On June 9th, four days after his first birthday, doctors found a mass on Hugo's left kidney. In the days that followed he was diagnosed with stage 4 atypical teratoid Rhabdoid tumor (ATRT) of the left kidney, with severe metastases to the lymph nodes and lungs. Surgery was not an option because the tumor wrapped completely around the aorta adjacent to the kidney. ATRT is typically a brain and central nervous system cancer, cases of this tumor in the kidney are very rare, around 25 cases a year worldwide.

The prognosis for stage 4 ATRT is very poor (but as Hugo's doctor said, it's not zero), so we jumped right into finding the best treatment possible. We were very fortunate to be contacted by Dr. James Geller at Cincinnati Children's Medical Center, one of two oncologists in the US studying rhabdoid kidney tumors, and he agreed to treat Hugo in tandem with Dr. Ray Pais, Hugo's oncologist at East Tennessee Children's Hospital. I can't stress enough how wonderful it has been to be able to undergo the bulk of Hugo's treatment here in Knoxville at ETCH.

A week following Hugo's biopsy and diagnosis, he underwent surgery to have a dual lumen Broviac power port placed in his chest, and then the chemo regimen prescribed by Dr. Geller began. Hugo gets two different combinations of chemo every three weeks (though there have been delays as we have gotten further along): VDC (vincristine, doxorubicin, cyclofosfamide) and ICE (ifosfamide, carboplatin, etopicide). This is a very tough regimen for anyone, much less a one year old baby. The first round of VDC left him with mouth sores and he lost over 10% of his body weight.

At the end of Hugo's first round of chemo we traveled to Cincinnati to meet with Dr. Geller. While there Dr. Geller decided to go ahead and order scans. Waiting for the scans we were gently reminded that this was a very aggressive, very challenging cancer, and Hugo's was very advanced. We were told that some doctors would not even treat stage 4 ATRT, moving straight to palliative care. Thankfully we do not have those doctors.

And then a miracle happened. The results came back and Hugo's tumors had shrunk by an estimated 80%. His lungs, once so filled with tumors they looked like they were stuffed with cotton candy, were virtually resolved. Suddenly our boy had a chance.

Hugo's second round of chemo yielded continued shrinkage of the tumors on the kidney and adjoining lymph nodes. After four rounds of chemo (in the middle of which we dealt with a central line infection and surgery to place a new broviac) we were scheduled to go back to Cincinnati for stem cell harvesting and surgery to remove the kidney, tumor, and all compromised lymph nodes.

Back in Cincinnati for two weeks, Hugo underwent surgery to place an apheresis line, stem cell harvesting (yielding 4 times the number needed, these are on ice should we ever need high dose chemo), a nuclear GFR to test kidney function, and surgery to remove the kidney and cancerous tissue. Surgery was successful, and now we had to wait two weeks for pathology reports to come back. In the mean time, once Hugo had recovered from surgery, we headed back home to Tennessee to begin round 5 of chemo.

Waiting for the pathology report was painful. We were hoping for completely dead tumor tissue but at the very least good margins of dead tissue if there was live tumor. On September 17th we got the news--there was no viable tumor! Everything removed was dead! Because ATRT is so aggressive, there is a high risk of microscopic tumors undetected by scans, so it was quickly decided that Hugo should finish his entire 10 courses of chemo.

Another scan before round six showed continued "NED" (no evidence of disease). Which brings us to now, about to start round 8 of chemo (VDC, which means mouth sores) We will continue to update this page as we learn more and thank each and every one of you for your support!

Here are a couple of fundraising efforts currently underway:

https://bumpersforhugo.wordpress.com/

https://www.crowdrise.com/gohugo/fundraiser/kellygriff