I’d like to share with you a story about a very special boy, it’s a long story, but one worthy of being told…
It’s been just over a year since Andrew Jones, now 13, was diagnosed with a large tumor on the left side of his chest. The news of this tumor hit him and his family especially hard because this wasn’t the first time they had to deal with tragic news.
You see, when he was just six years old it was discovered that his left lung stopped functioning. Doctors were perplexed at what happened to it but only knew that it had turned into a petrified mass and had to be removed. For years after the surgery to remove Andrew’s left lung, Andrew and his mom saw specialist after specialist and completed test after test to try and figure out what caused his lung to stop functioning and harden.
On December 21st, 2016, the rug got ripped out from under their feet once again. A massive tumor was found where his left lung once was. The doctors reviewing the CT imaging told them that Andrew had a malignant teratoma germ cell tumor. CANCER. As you can imagine, Andrew’s mom and family were devastated. What were the chances that this kid survived having his entire lung removed, only to get hit 6 years later with cancer?
The doctors and staff at McLane Children’s Hospital in Temple, Texas were quick to act with urgency and ordered a biopsy to be completed on the tumor. It was now Andrew and his family fell down a rabbit hole….
The first news to come back was that the tumor wasn’t cancer. Celebration quickly turned into frustration as weeks went by with no news or updates. Andrew’s biopsy traveled all across the nation after the pathologists in Temple was left scratching their heads. This tiny little slide of tissue got first class travel accommodations to Houston, Colorado, and Boston, to name a few. After nearly two months of waiting and anticipation, they finally received confirmation. Andrew has IgG4-RD.
IgG4-RD is a rare autoimmune disease. Even rarer is that Andrew was diagnosed at such a young age. In fact, there are less than 15 known cases, WORLDWIDE of this disease in kiddos.
Here’s a quick breakdown of what is known about this disease:
IgG4-RD is a disease of a chronic inflammatory condition characterized by tissue infiltration with lymphocyte and IgG4 secreting cells. This disease has a tendency in forming tissue destructive lesions which can lead to organ destruction.
Even with just the cliff notes version of this disease, certain things started to make sense. Was this what happened to his lung in 2011? Could this be the cause of Andrew’s diminished weight and height?
With so many questions about this rare disease, a recommendation was made to reach out to Dr. John H. Stone of Massachusetts General Hospital. You see, he was directly responsible for recognizing and researching this chameleon-like disease. He found that often times, this disease would be misdiagnosed as cancer, among other diagnoses.
With high hopes that Dr. Stone would be able to assist Andrew’s team of doctors in Texas, bags were packed and Andrew and his mom took off for Boston! Dr. Stone was kind but a bit taken back with Andrew’s age. He stated that Andrew is the youngest confirmed patient with IgG4-RD and it was likely he’s had this disease since the age of 4 or 5 and possibly, even since birth.
An official recommendation was made for Andrew to undergo a targeted chemotherapy regimen that has shown significant promise in the treatment of this disease.
A quick flight back home, a fight with the insurance company on the chemotherapy approvals and by May of 2017, Andrew was scheduled to start his 1st of 5 courses of Rituxan, the chemotherapy that could put this disease into remission.
Within 5 minutes of Andrew’s first dose, he had a life-threatening allergic reaction to the chemotherapy. The infusion was stopped and Andrew was rushed to the emergency room.
Andrew’s doctors were disappointed but hadn’t yet given up hope that Rituxin would be the answer to his health issues. You see, not only did Andrew have only one lung, a massive tumor that was now pushing up against and wrapping around his heart, causing strain, he was also in the -1 percentile for his age and weight, he suffered daily with flu-like symptoms. Some days were better than others, but the bad days were really bad days.
Andrew’s doctors prescribed to him a steroid treatment. There were two great reasons to move to the highly risky long-term steroid treatment. One was that Prednisone was known to help reduce the inflammation this disease had caused and two, Prednisone was known to help diminish allergic reactions to Rituxin.
At the end of May 2017, Andrew was taking 80 mg of Prednisone a day, with the intention to taper him down to 5 mg a day before attempting the chemotherapy again, this time in the pediatric ICU, instead of as an outpatient in the clinic.
Once again, the family found themselves fighting denials and calling insurance almost daily.
Finally, by August and three months after Andrew had started his steroid treatment, all the approvals and schedules were in place to attempt the chemotherapy for the 2nd time.
It went amazingly well! Andrew had some tightening of the chest but was able to successfully receive the first full dose of his chemotherapy. Hope was soaring for Andrew’s family. Finally, 8 months after this tumor was discovered, they were finally able to take some action to help improve Andrew’s daily life.
After Andrew’s 4th treatment, he developed severe nausea, vomiting, headache, was unable to eat or drink anything.
Andrew’s team of doctors were at a loss as to what could be causing his symptoms. Possible kidney infection? Could it be his gall-bladder or appendix? Maybe he got a strange virus since his immune system was compromised by the chemotherapy.
21 days in the hospital, 6 x-rays, 2 CT scans, daily blood cultures, endoscopy, colonoscopy, and lumbar puncture and the only thing that made any kind of sense to the doctors was that Andrew had Rituximab Serum Sickness. Yes, the very medicine that was supposed to cure him, also almost killed him.
Andrew was sent home with a feeding tube and on growth hormones to try and recover years of lost growth (that was later denied by insurance) and with the grave news that he would never again be able to receive Rituxan. This was especially disheartening because often times, one would need to receive several treatments of Rituximab in order to truly put this disease into remission.
This period of time that Andrew was recovering from the side effects of his chemo was also a period of time that Andrew’s family was hurting all around. You see, Andrew’s older sister, who just started high school in the fall, was the victim of such severe bullying at school that charges were filed by the local police against the bully. Andrew’s mom was also called into her office to be told that her position within her company had been eliminated and she could either lose 30% of her pay with a demotion or take a small severance package and lose her job completely.
21 days of confusion, frustration, heartache, and fear but Andrew and his family still hadn’t lost hope. Maybe the chemo could still work. Maybe justice could be found for Andrew’s sister through the court systems, and maybe there was a reason that Andrew’s mom had to take a step back at work. Time would tell.
Fifty-five days is how long it typically takes the chemo to work and to destroy the cells that were attacking Andrew. During that time, Andrew had a chance to celebrate his 13th birthday and to get caught up on his missed school work from home.
The day finally came and Andrew, his mom, and his Grandmother all packed up and started the hour drive to see his doctors in Temple. First up, a CT scan, followed by a visit to his cardiologist. She has growing concerns over the strain this tumor was causing his heart but wanted to wait and see what the results of Andrew’s CT showed. Last stop was in to see Andrew’s hematology and oncology specialist. The nurses took some blood samples to check his IgG4 levels and few other tests as well.
Then came the news they didn’t want to hear. The chemotherapy didn’t work. Andrew’s tumor continues to grow, but worse, it’s calcifying. Just like his lung did years ago. This time though, his heart arteries are involved and any continued calcifying could become life-threatening.
This brings us to today, just barely over a year and Andrew and his family are right back where they were a year ago. The doctors and staff in Temple are trying to arrange for consultations in Houston, but have essentially shared they’ve done all they know how to do for Andrew. There are no other courses of medical treatments known. Two options are left on the table. A possible bone marrow transplant, if Andrew’s sister is a match and/or surgery to remove the tumor. The only surgeons that may be able to remove this tumor would be at Texas Children’s Hospital in Houston, but even then, it may be too high risk for them to even consider.
Andrew and his family continue to fight through the red tape of insurance, the confusion of doctors and advocate for awareness of this rare but deadly disease. Andrew himself has become a bit of the community’s mascot. Having missed three-quarters of the school year last year, he still made honor roll and placed in the Master’s level in the state testing. This year he has been in the homebound program and continues to get all A’s.
We don’t know how Andrew’s story is going to end, but one thing is clear, this amazing kiddo has a lot more to tell the world.