ELI THE PIONEER

Our twelve-year-old son, and third child, Elijah Thomas Williams was a brain cancer pioneer. As in Linda Ellis's poem "The Dash," Eli's short little dash - the few years between his birth and his death -  was filled with more joy, more courage, more encouragement than dashes triple its length.

 

He was diagnosed with metastatic (high-risk) Medulloblastoma of the posterior fossa with leptomeningeal spread, group 4, in December 2011, just after turning seven years old. Eli had been battling nausea, double-vision, fatigue, and general body pain for three months.

 

After multiple trips to the pediatrician during those three months for answers, an emergency MRI on December 29, 2011, revealed that he had over half-of-a-dozen malignant tumors in his brain and on his spine, with his spinal fluid also being infected. The second largest tumor, near his pituitary gland, was inoperable.

 

Eli was immediately sent by ambulance to LaBonheur Children's Hospital in Memphis, Tennessee, where the primary tumor was resected on January 5, 2012. He recovered well from the eight-hour surgery, with little deficits to overcome, but the neurosurgeon was surprised by the extent of his disease compared to Eli's outward presentation. He escaped the rigorous brain surgery with mild side effects despite the warnings of possible deficits in talking, walking, Swallowing, seeing. Eli enrolled at St. Jude Children's Research Hospital in January 2012 under treatment protocol SJMB12, which included extreme radiation and aggressive chemotherapy. He started blanket radiation as soon as possible from the original brain surgery, then while in treatment a couple of weeks later, he had a second brain surgery to repair a tear in the patch from the first surgery. Because of the location and size of the original tumor, Eli's ventricles were not working to keep the cerebral spinal fluid flowing normally. So, again during radiation he had a third surgery to insert a Ventriculoperitoneal (VP) shunt from his brain to his torso area, near his stomach, to relieve hydrocephalus. After radiation, the treatment requires a six-week recovery period. Our family had only ever been to the beach one other time when the kids were little, so we took that time to go again. While there, an inguinal hernia caused by the fluid draining to his lower body via the shunt sent him back to Memphis for his fourth surgery. He recovered well from that repair.

 

Chemotherapy was planned to begin, but the static valve he received during the initial placement of the shunt was pulling too much CS fluid causing hemorrhaging in his brain. He had a fifth surgery to exchange the static valve for a programmable valve causing a delay in the start of chemotherapy. He recovered from the fifth surgery. Later, during his second round of chemotherapy, it was discovered that he had fresh bleeding on his brain because the setting was still too high and was again over-shunting. With the programmable shunt, the setting was changed merely by using a magnet to turn the dial on the appliance in his head instead of surgery. A wonderful invention.

 

He completed six weeks, five days a week, of radiation with two of the above-mentioned surgeries taking place during the radiation period. Radiation included a "blanket" of his brain and spine, then directed, targeted "boosts" to each tumor site. He began his first of four courses of chemotherapy on May 25, 2012, after the delay of five weeks due to recovery from the unexpected fifth surgery. The delay was unprecedented and caused distress to the doctors and us as the tumors had shrunk but were still present after radiation. He began his second round of chemotherapy on time but ran a high fever on the first day. It was thought he had a line infection, so he was given antibiotics to which he had allergic reactions. The allergic reactions, coupled with the effects of the chemo drugs, played havoc with his body. He became high-risk for sepsis and was moved to ICU where he stayed until his blood pressure was stable and his kidneys were functioning properly. The third day of chemo for that course was canceled because he was not well enough to receive the treatment. After the second course of chemo, an MRI showed that only one tumor of the more than half a dozen remained, which was the second largest tumor located at his pituitary gland. The tumor had shrunk considerably, and it was unknown if what remained in imaging was scar tissue, a dormant tumor, or active tumor. A lumbar puncture was also done at that time to determine that his spinal fluid was clear of disease. He began and completed his third round of chemo on time. He again ran a high fever and was treated with antibiotics, but because of what was learned about his allergies during the second round, he was premedicated. Though the course was difficult, he was closely monitored, and he received all three days of chemo. 

 

At this time, it was discovered that he had significant hearing loss in both ears after two courses, which was expected. Eli completed the fourth and final round of chemo on August 18, 2012. Because of the hearing loss, one of the drugs was given at a half dose to prevent further loss. The fourth round was difficult, but lessons learned during previous courses helped control the effects. For chemotherapy, he was treated with high doses of four chemo meds and one rescue med. 

 

The standard regimen for each course/round was as follows: On Day Minus Four he received Cisplatin via IV over a six-hour period. It was this medicine that was cut in half in the fourth round to prevent further hearing loss. Over the entire treatment of Cisplatin that is a 12 percent decrease. During the six hours on this day, he received a “push” – which is when the nurse hand pushes from a syringe into his line. It only takes a few minutes, but it is intense for him. This is also how they administer stem cells - it is rough - Amifostine and Vincristine. This happens at the beginning of the Cisplatin delivery, then again three hours later. One of those decreases his calcium level, so he received calcium according to the loss. On Day Minus Three, he got an hour of Cyclophosphamide via an IV along with a rescue drug called Mesna. Cyclo is rough on the urinary process, and the Mesna is used to protect the bladder and kidneys. It was required that he urinate every two hours for 24 hours after each dose of Cyclo. This was repeated on Day Minus Two. Day Minus One was a day of “rest” and Day Minus Zero he received his stem cell transplant and was supposed to be discharged from inpatient status. Only after the fourth course was he ever well enough to be discharged on time. On Day Plus Six, he received another dose of Vincristine out-patient. During all of this, as he fought heavy nausea and intense diarrhea and uncontrollable fever. He received two or three nausea meds, Tylenol for high temperature, a stomach acid medicine, mouthwash, ear drops, betadine baths, bottom cream, and then any antibiotics - sometimes multiple - that the cultures determine he needs, or that they think he might need, and Benadryl to thwart the effects of the antibiotics. As his electrolytes got out of sorts, he received any supplementing needed to offset that as well. Physically, he had significant weight loss and showed some instability in his gait and coordination. But, overall, his strength and abilities were impressive. We tried our best to walk every day, even during treatment when in pain. Just to walk to the door of his room from his bed was an accomplishment on some days. His nutrition during chemo was solely via a temporary feeding tube (TPN) because he would not eat, although he tried to get two or three bites of something each day to maintain some digestive function (literally, two McDonald's french fries was our deal for an entire day). After each chemo round, he received a stem cell transplant. His own stems cells were harvested before radiation, and they gave them back to him during each round. This helped his body’s immune system recover faster after it bottomed out due to chemotherapy. He received blood products often, to which he had allergic reactions, so he was always premedicated.

 

Physically, he had significant weight loss, coupled with muscle loss. But, overall, his strength and abilities were impressive compared to other patients. Eli's exit MRI scans were taken on September 5, 2012. The scans showed that all tumors and tissue were gone except for the second-largest tumor at his pituitary gland. It, however, showed no change, growing or shrinking, which would indicate it had no live tissue. Furthermore, there was no blood circulation activity, indicating dead tissue. 

 

Eli left St. Jude on September 14, 2012, and was cheered on by friends on the side of the road along the route. As he arrived at his home by limosuine, the streets of our neighborhood were lined with family and friends to cheer his return.

 

At home, he continued on the temporary feeding tube for a month then doctors removed his Hickman line. He struggled with eating but maintained his weight. He ate, walked, talked, and performed all self-care duties on his own. He was well-behaved, agreeable, as energetic as he ever was (which wasn't ever much), interested in activities and subjects. He was a complete and absolute joy. He saw a tutor three times a week after treatment to keep up with school work. He started back to school, returning to his original class in January 2013 after a year of just tutoring at St. Jude, and while at home after treatment.

 

He attended a small private Christian school with friends and family that loved him. They made special arrangements in preparation for his return, and he did well in class. We are blessed to have a school that loved him and wanted the best for him. Eli was not on any medication for anything after his return from the original treatment. Outside of St. Jude, we loosely followed a homeopathic diet for maintenance. The hope was to give his body the best chance of having what it needed to fight cancer cells trying to find a welcoming host environment. We juiced raw, organic vegetables and fruit for him, and we used organic food when available and tolerated. At the time, oils, a detox drop, lact-ez, vitamin D3, immune support, and a raw vegetable powder are among some other things that the homeopathic doctor prescribed to add to his diet. In reality, what he actually got of that each day varied.

 

His first four three-month MRI's were NED (No Evidence of Disease). In December 2013, Eli's MRI showed relapse. He had multiple lesions in his brain and on his spine. Less than five percent survive relapse for even five years because there is no durable treatment for relapse. We began an intense homeopathic regimen under the direction of a local homeopathic doctor and with the help of a local homeopathic dietician. Eli participated well with this, better than expected, even following the regimen through a 10-day trip to Disney. The dietician and friends would ship his food to us overnight so that we could continue strict enforcement. Despite this concentrated effort for a month and a half, a scan in late January 2014 revealed continued growth. So, Eli began an off-trial Children's Oncology Group (COG) treatment plan that included Irinotecan, Temozolomide, and Avistan. St. Jude administered it from its clinic in Huntsville, Alabama, allowing us to stay home.

 

The treatment was low-dose chemo and data showed that it had the potential to add the most months to his nine years at the time of relapse. The data was also clear that the disease would continue to progress, so this treatment was considered palliative. We hoped that in the following months, as we tried to buy time with the COG treatment, there would be more palatable options for treatment. His first MRI after two months on the COG treatment was encouraging as the tumors had shrunk more than the doctor expected. Two months later, April 2014, the MRI showed the brain stable and the spine clear. In June 2014, the MRI showed more shrinkage, and again in August 2014, the lesions were almost undetectable. Eli's check-ups were spread out to 3 months. His scan in November 2014 showed no sign of active disease, while his body began to show signs of chemo toxicity, so the dose was lessened. Routine scans in February 2015 showed new spots in the tumor bed. Eli's cancer began to grow again, so treatment at St. Jude was stopped, and it was suggested that we go home to hospice. During that 14 months of palliative treatment, several phase-one trials (experimental) opened up, which is why “months” is so important. Now, having had time to learn more about the relapse world, we were a little more adept at finding what treatment we felt was best for Eli, and seeking it out ourselves and not depending on St. Jude, which had sent us home for hospice. 

 

Four trials were pursued (3f8 trial at Sloan-Kettering in New York, a vaccine therapy at Kosair in Louisville, Kentucky, and Immunotherapy at MD Anderson in Houston), and Eli was accepted as patient number one into the heralded NOAH protocol at MD Anderson, sponsored by Noah's Light Foundation. It was expected to be the answer, highly anticipated by the world in which we lived. The NOAH protocol was a phase one trial in which his natural killer cells were harvested and "beefed up" in the lab. The cells would then be injected into the fourth ventricle of his central nervous system via an Ommaya Reservoir placed inside his brain - a procedure which was still in experimental stages itself. He was scheduled for his first injection April 27, 2015. However, after growing his cells in the lab for two weeks, the sample was not pure NK cells. There was an additional, unexpected marker still in the blood. The sample was unusable. As of April 24, 2015, everything was put on hold while MD Anderson doctors requested from the internal ethics committee that they, basically, try again. The committee agreed, and his blood cells were re-harvested. The sample had the same problem, so Eli was dropped from the trial. Research doctors at MD Anderson said they have never seen a cell combination like Eli's, and could not find it documented anywhere in the world. All this while, the cancer was growing. MD Anderson echoed our St. Jude doctor’s opinion to go home to hospice, though with less conviction. 

 

Despite the devastation, we continued to pursue the sliver of a slim chance for something to help him due to his exceptional stamina. Paperwork was compiled for a phase II trial at Sloan-Kettering, and two new trials had opened up during this time. Mayo Clinic opened an immunotherapy trial, and a Houston hospital under the same doctor that placed Eli's ommaya reservoir in the fourth ventricle had opened a trial using the ommaya as a catheter for methotrexate. The placement of the ommaya, using it in the fourth ventricle to infuse chemo was the trail. Traditionally, an ommaya is put in the lateral ventricle in the top of the head. Since Eli's ommaya was already in place from preparation for the NOAH trial, we decided to pursue the trial at Memorial-Hermann in Houston with the doctor that placed the catheter. It was a three-month trial to determine the safest, most effective dose that can be infused in the catheter in the fourth ventricle. It was not expected to cure the cancer, but would hopefully control it a little while longer. Just as we bought time before with a palliative treatment while trials opened, we hoped to buy more time to wait out more trials. We spent the summer in Houston. June 15, 2015, was his first injection. It was a once a week injection that took about 30 seconds. The trial completed August 28, 2015. The exit MRI three days later showed stable disease - albeit a wobbly stable - from the three months of treatment. By that last month, Eli began to show signs of neurotoxicity. He had a bout with double-vision, slurred speech, altered gait, pain in his legs, trouble urinating, and his appetite was significantly affected. Two weeks off treatment, all of these issues resolved on their own. Despite the promising effect of the treatment, no other doctor, besides the trial surgeon (and his offer was only if we were desperate. However, when we became desperate, suddenly it couldn't be worked out) was willing to continue the treatment because the placement of the ommaya was unconventional, and there had not yet been research results published on the use of it.  So, giving up that anyone would continue the ommaya treatment, we continued a couple of band-aid doses of intrathecal methotrexate with MD Anderson until Eli could get on a new trial. At that time, in September 2015, MD Anderson considered Eli to have six weeks to live. Coming home, we continued to pursue phase one trials. He was rejected from a Kentucky trial; rejected from a Sloan-Kettering trial; rejected from a Florida trial; was not a good fit for a trial at Stanford University; rejected from a UAB trial; rejected from a Duke University trial; the Mayo Clinic trial continued to be on hold; he was not a good fit for a treatment with a specialist in Michigan; and a doctor in Boston did not respond after initial contact. Meanwhile, a trial with the Pediatric Brain Tumor Consortium opened up with an immunotherapy drug called pomalidomide. Since St. Jude is a member of the PBTC, Eli was able to join the trial through that hospital. After being off treatment since October 1, 2015, Eli began the trial with pomalidomide on November 17, 2015. His first scan in January after two months showed progression. He was covered in 18 tumors that could be counted, plus the “sugar-coating” or leptomeningeal disease. Our St. Jude doctor was baffled as to why he was asymptomatic with such heavy disease, but indeed, we were sitting on a time bomb. His opinion, once again based on the scan, not based on Eli, was that we had done our duty by him, done more than most, but it was time to call hospice and go home.

 

I was determined that no doctor would tell Eli when he was done. Only Eli's body would tell us when Eli was done. I again shopped Eli’s scans and his summary to many doctors and hospitals with trials, or just with doctors known to be experts in medulloblastoma. But he didn’t qualify for many of the trials, and some doctors I could tell just didn’t want him on their trial because of the “extent of his disease burden” (he was too far gone. He would basically be a check in the loss column before they even got started). Plus, his little body and his bone marrow were so tired. His body was telling us, it couldn't take much more. So, we agreed, and he was admitted into hospice care in February 2016. Thankfully, hospice care for children allows you to participate in treatment while remaining under hospice care.

 

Through our rejection from the University of Alabama-Birmingham for one of its trials, we learned of a doctor in Augusta, Georgia, who had opened an immunotherapy trial that included an arm with radiation, plus temozolomide. It was freshly experimental, but one for which Eli would qualify even with his treatment history and his weak immune system. Plus, the fact that Dr. Ted Johnson was even willing to see him was beyond any steps we had made with other doctors at this point.

 

As patient number two on the second arm of the Augusta trial, he enrolled on March 8, 2016, with 18 tumor sites, and heavy leptomeningeal disease. The arm included a second round of blanket radiation for him. Scans in April 2016 showed that the disease had stabilized, and some spots were either the same or one shrunk. Scans in June, after two cycles of temozolomide with the experimental drug Indoximod, showed that everything had either shrunk or was gone, and the "sugar-coating" was no longer detected. He had gone from 18 tumors with heavy leptomeningeal disease in March, to half the burden. Indoximod, an immunotherapy drug that turns off one of the pathways in the immune system that is susceptible to tumor growth, caused no side effects or symptoms. He did have more hair loss due to the mild chemo, but was otherwise strong and enjoyed a normal life during those months. He did everything we did, went everywhere we went, participated, and was plainly wonderful. He took the pills from home and had no symptoms from the treatment other than occasional mild diarrhea from the chemo. However, by August 2016, routine scans showed six lesions, one of which appeared to be growing. This was devastating news, as it could be a sign of a third relapse. A scan in September 2016 showed only four tumors, but the one was still growing, and also a second of the four was growing. Thankfully, due to Dr. Johnson's compassion as a physician, he had written the trial to allow for deviances. So, Eli had a double craniotomy in October 2016, his fifth brain surgery, during which the two progressing lesions were removed. During recovery, he had temporary paralysis of his left side, and could not swallow. He was moved to ICU, and it was found that he was having almost constant focal seizures at one of the surgical sites. He was put on seizure medicine that immediately stopped the seizing, and he began to regain use of his left side. At one month prior to treatment, all surgery-related deficiencies had resolved. A scan in November 2016 revealed that lesions were growing again, and he was back to four spots of concern (two of which were the sites where tissue was removed in surgery). The pathology report from the latest resection showed that his current disease was the same as what he had in December 2011, the original cancer. It had never gone away. We stayed on the trial another round hoping that the growth detected on the scan was just signs of swelling from the tumor tissue being attacked (this had been seen in other patients), but a scan in May of 2017 revealed that the disease was rampant in his brain and spine. His blood work was not good enough to join any other trials, nor was he able to handle any high-dose chemo, or get another round of radiation. We left the trial in Augusta that had gained us 16 months with Eli and made a desperate attempt to save him by enrolling as a patient with Children's of Atlanta specifically to be a patient of Dr. Dolly Aguiela. She had more tools at her disposal and had an excellent reputation for thinking outside the box. Dr. A received Eli's history and scans before she met him. When she walked into the room, Eli was playing with Legos. She turned back outside to the front of the room to look at the room number and then stood watching him for a moment. She said, "I'm sorry, I thought maybe I was in the wrong room. This is Eli?" She was genuinely amazed at what she saw in him versus what she read and saw in the scans. It seemed to sort of rouse an energy in her to help him and gave us a little bit of hope. Much progress in research and diagnoses had been made since his original diagnosis in 2011. So, Dr. A took advantage of some additional testing of the tumor that was available, but getting into the system in Atlanta, getting the tumor tissue tested, receiving results, and getting appropriate drug changes was too slow for the aggressive cancer and the disease overtook Eli by the end of July. He was no longer able to travel.

 

He became bed-ridden on July 30, 2017, after making his last trip to try to attend to worship at church. He took this last picture, just seven days from his death, dressed for church that morning. You can't tell it, but he could not walk without help, and could not speak, steadying himself by the grip you see he has on the chair arm. His head held high seemingly out of strength, but because he could not see me very well taking the picture. After a few snaps, I helped him step through the door behind him, sit on the hospice bed seen through the window, and he never got up again. He had a peaceful week of friends and family coming to see him, and then left his worn and beaten body for a new one with his Creator at 10:40 p.m. on August 5, 2017.

 

Eli's body was cremated. The most beautiful and appropriate service was held in his memory a week later. Our neighborhood decorated their mailboxes with royal blue ribbons as we left to attend. A string of 50 mustangs and vehicles lead Eli's cremains to the service where state troopers honored him by presenting the box to the front of the auditorium. With the foyer decorated with all the things Eli loved, guests in his signature royal blue were lead in congregational singing of some of Eli's favorites. Boys from his class lead prayer and Bible reading, and one of our ministers who is the father to one of Eli's friends performed the ceremony. The ceremony closed with an "End of Watch" recording from the Georgia State Patrol to honor Trooper Eli Williams. Thanks to our friends who loved Eli, it could not have been a more perfect event. The magnitude of the good that God fashioned out of the evil that victimized our gentle Eli will never be known this side of Jordan, and the hole that Eli left behind in our lives and hearts will never be filled.

 

ELI THE BOY

Over the five and a half year journey, I was often asked what Eli was like, and what kinds of things he liked so that people could send him toys or something. Well, he was a pretty easy and simple guy. He liked vehicles number one, top on his list for sure, cars, trucks, anything on the road. His favorite was Ford, especially a Mustang and an F-150. We sold some personal property, and an online drive raised money for him to buy his own Mustang. He bought a 2014 GT Mustang Premium that has been significantly performance modified. He and his siblings agreed on the name "Bruce" for the car after Bruce Wayne, the Dark Knight, Batman's alter ego. Eli enjoyed going to car shows, so the car was for him to take to shows, and just enjoy. He also liked Bugatti, Lamborghini, Monster Trucks with Grave Digger being his favorite. Top toys for sure were Legos, and Transformers, with mostly bad guys as his favorites. By far his top three toy likes were 1:18 diecast cars, legos, and transformers.

 

He liked to read about wild animals, especially snakes and sharks. His favorite color was blue and sometimes green, and he liked to watch movies, but he also liked SpongeBob, Arthur, and Phineas and Ferb. He collected baseball or football cards, although he didn't necessarily follow any teams or people. He liked racing, NASCAR mostly, although he (and we) didn't know anything about it, but cars were involved, so that's all that mattered. He was somewhat of an Auburn fan but also liked to follow Tennessee with his daddy. He liked old cartoons like Tom and Jerry, Bugs Bunny, old Disney, and he loved the Three Stooges. He liked Angry Birds on his Kindle and racing games, especially Need for Speed, Mario Kart, and Forza, and Minecraft, and Skylanders. He had a Wii U, and all the kids enjoyed playing 4-player games on it.

 

He received his Make-a-Wish Foundation wish of a camper just before Christmas 2012 from Bankston Motorhomes in Huntsville, Alabama. After two years of only using it twice (we wanted to, but when he relapsed, camping was difficult), we decided to sell it to help pay for the Mustang.  After his first relapse, the community and his friends raised money for our family to go to Disney World before he started this treatment since we had never been. In February, we visited the Flat Rock Assembly plant in Flat Rock, Michigan, where the Ford Mustang is manufactured. It was a fantastic experience and a huge entry into the family memory book. We continue a relationship with the community there as they host a car show in Eli's memory every year to raise money for our foundation. In 2015, the Marty Lyon's Foundation granted him a second wish, which was to visit Jay Leno's garage in Burbank, California. It was wonderful. Eli had hoped to travel out west to the Grand Canyon, or he always wanted to go to Hawaii (Vic and I went to Maui on our honeymoon, so he has seen pictures). Eli was a shy little guy, not very friendly or outgoing if you met him, and didn't really like new people. He was socially awkward because he just was not often in social situations and I think he couldn't hear well in crowds. But he was agreeable more than most kids and tried to always do what was asked of him as far as his treatment and most other things, too. His hopes were to drive an F-150 when he got his license, have four kids when he got married, and he wanted to be either a FedEx driver or a police officer when he grew up. He was beautiful inside and out and will forever be a little boy.