Rucker

Symptoms & Diagnosis

Rucker’s story begins in March of 2018 at the age of 3 when he began complaining of leg pains at night.  An X-ray followed by an MRI showed a small lesion on his left fibula.  A biopsy conducted the next week appeared to be negative for malignancy, although the pathologist noted some findings of inflammation and giant cells (which were benign).  Due to the lesion's potential to cause a fracture in his bone, his orthopedic surgeon recommended that the mass be surgically removed. 

While waiting for the outpatient surgery to be scheduled, Rucker proceeded to live his life normally celebrating his 4th birthday on June 9th and going on a beach trip with his family. 

The severity of Rucker’s ailment was discovered when he fell in the driveway and injured his right leg.  Imaging during a trip to the ER showed a broken femur above the knee - and another lesion, similar to the one already seen in his left leg.  

This is when the doctors realized there was a bigger problem. Full body x-rays were ordered to determine whether other lesions could be seen in his bones. Although the x-rays did not reveal other bone lesions, the interpreting radiologist notified our attending physician that there appeared to be a large mass in Rucker's belly. 

On June 26th, 2018, a CT scan showed a large tumor on Rucker’s kidney, which the doctors believed to be the primary source of the disease.  They also found some nodules in his lungs and at least one additional lesion near his shoulder bone.  He was immediately admitted and placed in the oncology wing (8th floor) at Children’s of Alabama.  An open biopsy on his kidney was scheduled for the following day.

On June 27th, Rucker went into surgery.  In addition to performing the biopsy, the surgeons installed a chest port for IV meds (and later, for chemotherapy).  And while he was still under, an orthopedic surgeon put a cast on his leg for the broken femur.  Unfortunately, the cast also wrapped around his abdomen, which meant that Rucker could not walk (or go to the bathroom by himself...) for at least 8 weeks. 

A couple of tough days followed.  Over that weekend, Rucker’s pain from the surgery began to subside.  Because the doctors were still waiting on pathology results and weren’t ready to start chemo without a formal diagnosis, they decided to send us home on July 3rd, just in time to be able to watch the fireworks.

Pathology ruled out some of the more common tumors (e.g. Wilms) and developed a working theory that it was clear cell sarcoma of the kidney (CCSK).  However, to be sure, the head of pathology at Children’s of Alabama consulted with Dr. Elizabeth Perlman, the top pediatric kidney tumor pathologist in the country.  After reviewing Rucker’s pathology, she agreed that it is most consistent with CCSK, but she ordered additional stains and testing in order to confirm that theory.  A bone marrow biopsy was performed to rule out an incredibly rare type of blood cancer that presents in the kidneys.

On July 25th, CCSK was ruled out and his disease was being classified as an undifferentiated sarcoma, which meant that the cancerous cells didn’t resemble cells found in the body tissues in which they developed - in Rucker's case, his kidney.

The biopsy of his primary tumor (in his left kidney) was then sent to pathologists at the Ann & Robert H. Lurie Children's Hospital of Chicago, St. Jude Children’s Research Hospital, and Cincinnati Children’s Hospital.  Genetic testing was also done at both Foundation One and Circulogene to better determine specific gene mutations.  

On August 18th, Rucker’s diagnosis was confirmed to be Sclerosing Epithelioid Fibrosarcoma, or SEF.

Treatment

It took almost 2 months to properly diagnose the disease as SEF.  While awaiting results of additional testing, Rucker’s team decided to begin chemotherapy. 

 On July 12th, Rucker went in for his first round of chemotherapy, part of a 9 month plan that also included radiation and surgery to remove his left kidney and the tumor.  

On September 20th, Rucker had scans to check on the primary tumor and metastases, and they showed no progression since we last scanned. We were encouraged by this result considering the aggressive and rare nature of this disease. 

On October 1, we started five weeks of daily radiation to target the primary tumor and a few other sites in hopes to reduce the size of them and better allow for surgical resection. 

A Setback

 In mid-October, Rucker broke his right leg again, less than 1 month after being out of his cast. 

On November 12, at MD Anderson Cancer Center in Houston, Texas, Rucker underwent curettage surgery on his right femur to remove the tumor that had been causing him so much trouble since June, when he first broke his leg.  The procedure was successful in removing the tumor and surrounding areas of bone.  Bone graft was placed against the growth plate, along with cement and reinforcement rods.  The main goal of this procedure was to give Rucker his mobility back.  The secondary purpose was to get rid of the tumor causing all of the side effects.  If it were not causing issues, or not presenting side effects, we would have treated this site later in the plan.  

On November 26, Rucker’s left kidney and the primary tumor were removed by Dr. Bierle at Children’s of Alabama.  The tumor was LARGE for his tiny little body (approx. 5x12 cm), and Dr. Bierle said it felt like a rock and had been pushing his stomach and intestines up into his rib cage. But, we received good news from pathology that the margins were clear and Dr. Bierle was able to remove all traces of it from a gross and microscopic standpoint.

In January of 2019, after seven months of chemo, Rucker finished his last doses of Ifosfamide and Doxorubicin. These were two very nasty and toxic chemo agents that constantly wiped Rucker out. Except during his surgery phases, Rucker would receive this chemo over 4 days every three weeks. And almost without fail, he would be back in the hospital 10 days later (usually for 3+ days) with a fever and pretty yucky side effects. His blood counts and immune system would be totally wiped out and he was tired and miserable. 

In February of 2019, we were nearing the end of our initial treatment plan that was discussed with our medical team back in July.  At this point, Rucker had undergone the following:

1) Two right femur fractures (June and October 2018);

2) Seven months of chemotherapy;

3) Five weeks of radiation on his right femur and left kidney tumor;

4) Surgery on November 9, 2018 in Houston at MD Anderson to curettage the tumor in his right femur and repair his femur fracture;

5) Surgery on November 26, 2018 at Children's of Alabama for his total left kidney and tumor resection.

And while Rucker was still having difficulty walking, we moved him to a new physical therapy regimen which helped him to begin walking unassisted. 

Rucker’s last round of scans on February 22 appeared stable, so in late February, we were able to move forward with a surgery to remove the nodules that had been present in Rucker’s lungs since his diagnosis.  Our surgeon Dr. Bierle was very confident she got all of the nodules out, including a few very small ones that weren’t detected in previous scans.

A Little Setback

In late March, Rucker’s right knee became inflamed near his surgery site from his previous femur operation. Apparently, the return of his mobility and possibly a little growth spurt made his bones shift in his leg and one of the pins that had been placed in his leg popped out of his   knee. We quickly made arrangements with our oncology team and surgeon at MD Anderson and flew to Houston on Wednesday March 27. 

Rucker had surgery the next day.  The pin in his right femur were cut back, re-cemented into the bone, and his wounds were re-sutured. In addition, the lesion in his left fibula (the first lesion that was detected back in March 2018, when we just thought Rucker had a bone cyst) was removed. Since his diagnosis in June, that lesion seemed to remain stable and was in Rucker’s “good leg,” so we initially decided to monitor it instead of removing it. A recent MRI report indicated that the fibula lesion may have grown slightly.  Therefore, we decided the best way to manage it was to have it removed.  

In March of 2019, Rucker started a new drug called Pazopanib that has recently been shown in a clinical trial to be effective against soft tissue sarcomas in pediatric patients. This drug acts like more of a cell inhibitor rather than a chemo drug so it should be much easier on his body.

 >>When was Pazopanib discontinued and why?

April thru July of 2019 were great times for Rucker and our family.  We made the most of this time period where Rucker’s treatments were non-invasive and non-debilitating.  We will forever know this time period as “the Sweet Spot.”

A recurrence and time to recalibrate

On July 30th, 2019, we received the news that Rucker’s disease had returned to his lungs. 

We had taken him to the hospital because he was having leg pain and a fever. We scanned his legs and determined that the spots in his legs were enhanced, but not bigger. On Tuesday, upon scanning his upper extremities, we found that the disease had returned to his lungs. This recurrence, while shocking, was not altogether surprising. In fact, we had been mentally trying to prepare for this all along, due to the stubborn nature of sarcomas. 

At this point in time, we recognized that conventional treatment options, such as chemotherapy, radiation, and surgery are decreasingly likely to cure Rucker and we vowed that we would not stop looking for alternative measures to treat his disease. 

Enter Sheri Spunt

John spent countless hours the following week researching and putting together a document outlining Rucker's case that would be circulated to every reputable institution and expert he could find. While he was down one of his many internet research rabbit holes, he came across a 2-minute youtube video which showcased a panel of oncologists discussing their opinions on immunotherapy for sarcomas. One of the panel members was an oncologist from Stanford, and her comments kept resonating with John. He watched the video several times and then decided it might be worth a shot to reach out to her. Our oncologist was able to find her contact information, and John emailed her Rucker's story and asked her if she was someone who might be able to help us. 

Two days later on a Saturday evening, John received a phone call from a number he didn't recognize. On the other end was Dr. Sheri Spunt, the pediatric sarcoma expert from Stanford. Dr. Sheri Spunt is an expert in soft-tissue sarcomas and rare tumors in children, and research and experimental therapies in this area are her "passion." She immediately reeled off several thoughts and ideas to John about possible testing and treatment options for Rucker. She currently oversees many trials, some of which he may qualify for, and she is very knowledgeable about other promising immunotherapy trials across the country. 

Renewed Hope

When John got off the phone with her, it was the first time since we had received the latest news about Rucker that he had hope in his eyes. John and Dr. Wadwha spent the next week consulting with Dr. Spunt to come up with a plan.

Stanford Surgery

On August 16 at Stanford’s Children’s Hospital, Rucker underwent surgery to remove the new tumors in his lungs. The surgery was completed thoracoscopically, so it was minimally invasive.  The primary reason for the surgery was to get fresh tumor samples for RNA sequencing to more fully determine if there are any markers in the tumors that may allow for targeted medication. Test results were not scheduled to come back for a few weeks, so there was an added benefit of having the tumors removed while we await the results and put a treatment plan in place. 

A New treatment plan

On August 27th, Rucker began a new treatment plan, starting with a chemotherapy called Trabectedin, which has shown some efficacy for advanced soft tissue sarcoma. The drug is intended to keep the cancer cells from multiplying. The plan is to do 24 hour infusions of the drug once every three weeks. Rucker went through two rounds of it and handled it amazingly well with almost no side effects. However, scans after the second round showed some slight progression of disease in his bones. We were obviously disappointed, but quickly went to work to figure out what to try next. 

Slight progression > Change in treatment

On November 12, 2019, Rucker started his new chemo regimen of Gemcitabine and Docetaxel. This chemo has considerably more side effects than the Trabectedin did, but it still wasn’t as bad as the very first chemo that he did (which led to numerous fevers, infections and hospital stays). All of the chemo infusions were administered outpatient at our weekly clinic visits. 

Intro to CC-TDI

Although the results of the RNA sequencing gave us a lot of additional clues as to what may be driving the cancer and what genetic pathways are affected, we still had few treatment options available (especially any that are actually FDA-approved for pediatric use), so we knew we had more work to do. Our research on SEF (what little there is out there) made it clear that general, traditional front and second line treatments will not cure SEF, and that novel, experimental therapies must be explored. 

Understanding our mission, Dr. Spunt ultimately connected us with Dr. Charles Keller of the Children’s Cancer Therapy Development Institute (“cc-TDI”), which is a non-profit clinical research institution in Portland, Oregon that focuses on closing (or at least narrowing) the "pre-clinical gap" in childhood cancer research, and particularly focuses on finding novel and/or experimental therapies to treat rare pediatric cancers. 

After several phone discussions with Dr. Keller and his team about our fight against SEF, John flew out to Portland to meet the team in person. John was beyond impressed not only with cc-TDI’s facility, staff, and professionalism, but he was most impressed by their energy and passion for their work. Their team doesn’t bat an eye when a family tells them they’ve been told their child’s disease is incurable. Rather, it seems to drive them even more, to think outside the box and reach beyond the outdated standard pediatric treatment protocols that have gone unchanged for nearly 40 years (and at this point I have to really restrain myself from ranting about the unnecessary governmental red tape, lack of research funding, and lack of access to any drugs that aren’t approved for children - issues that pediatric cancer parents are unfortunately all too aware of - but that soapbox is for another day!). 

The cc-TDI team works incredibly hard every day to connect cutting-edge research to actual potential drug trials as quickly as possible. Their brilliant minds are exactly what we need in order to push to find a cure for SEF.  

After John met with cc-TDI and their affiliates in November, he went into action and figured out what needed to be done to get a study on SEF started. And we don’t mean some study that may be helpful years down the road to the next generations of SEF patients. We mean a study of action, with the purpose of finding targetable therapies as soon as possible and to make SEF universally survivable.  

SO...on January 10, 2020, a SEF-specific study was funded and implemented at cc-TDI in Portland, Oregon! The initial aims of the project are to create a SEF patient registry, to collect, bank and test cell lines and live tissue, and to generate xenograft (mouse) models in order to simultaneously conduct active drug studies. The ultimate goal will be to take these results to actual human trials as soon as possible. We are now working to connect with other SEF patients (adults and children) and their oncology teams to collect tissue, compare genetic testing results and treatment histories, and to get started on finding a cure. 

>>Need to fill in information here for Spring of 2020

Decline

On June 15, 2020, shortly after Rucker’s 6th birthday and after 17 days of Rucker battling severe headaches and nausea, we found out that the disease had spread to Rucker’s brain. This was not news that we had been expecting at this point.  In fact, scans of his brain were clear just two weeks before.  

Our primary goal became to get Rucker to a place where he was comfortable and could tolerate both travel and treatment.

His headaches became debilitating and he was unable to sit upright and eat.  He was receiving fluids and pain medication to help him cope with the headaches and nausea.  Our oncology team’s goal was to start radiation on his brain and begin steroids under the premise that this should give him relief to allow us to get to the next step.

June 19th was a roller coaster of events and emotions - Rucker’s condition declined very rapidly on Tuesday night/Wednesday morning. We almost lost him, and we were faced with some very terrifying and incredibly difficult decisions, sometimes with only minutes to make them. 

In the course of 8 hours, we moved from the Oncology floor (8QB), to Special Care, to the ICU, and back to 8QB.  Radiation was totally off the table.  But Rucker is a tenacious little FIGHTER. And he battled HARD.  Slowly but surely, he stabilized all on his own and - amazingly - was able to make it to radiation, the first of five rounds.

A week later, Rucker was stable but still very sleepy. He did not like to stay awake for more than a couple of minutes. This was attributed to one of the following: increased pressure, disease around his brain, and/or side effects of radiation.  His pain lessened and his stats were much improved - his heart rate had been very low for the past several days.  He is off of oxygen support and all of his meds have been switched from IV to oral. He had been having trouble eating, so we decided to put the NG tube in to increase his calorie intake. 

When the palliative doctor came in to see him before the weekend, she couldn’t believe the 180 he had done since Wednesday when she was called in. She told us he is truly a miracle and then looked at him and said, “Rucker, you must have a lot of people praying for you.” We just smiled and said “you have no idea.” 

Oh, 2020...

The summer of 2020 was rough.  After the disease progressed into his central nervous system, he experienced some significant neurological issues - ex. headaches, lower limb movement problems, vision impairment, lethargy, etc.  He was uncomfortable for most of the summer.

But we persevered. In July, we were able to gain approval from the FDA (in a single-patient investigational new drug trial) for Rucker to try a novel treatment that targeted his specific gene over-expression. He had two rounds of this at MD Anderson, and we waited patiently.

On Thursday evening, October 29th, Rucker’s time here on earth came to an end and now his new heavenly life began. He passed away comfortably at home with his Daddy, Momma and his little brother Charlie at his side. He was “safe and good” as we would often say to comfort him, “right where he needed to be.”

<<Lots more text in the CaringBridge that we can include.