Desmoplastic Infantile Ganglioglioma

[nathans.family]

Hello! We are the Thomas family from Northwest Tennessee. My name is Beth and my husband is Mike. In October 2009, Mike was diagnosed with bladder cancer after a resection at our county hospital. We were immediately sent to Vanderbilt Medical Center in Nashville, TN. where we met a surgeon and an oncologist and Mike returned home to start chemo at the clinic, a surgery date of March 24, 2010 was set up at Vandy where he would have radical surgery and a urostomy. Shortly after Mike's dx. we began noticing some peculiar behavior in our 2 1/2 year old son Nathan. He became very lethargic, vomiting in the morning, and was unconsoleable. He freaked out at snow and would have a melt down if we moved any furniture including the dining room chairs for dinner. Everything concerning Nathan became very difficult. We initially thought it was just stress and being 2. We continued in our somewhat normal lives through Mike's treatments, I worked and Nathan went to daycare and Christmas came. By the middle of January, I knew something was wrong. I took him to the pediatrician and she treated him for bronchitis with an ear infection and told me to come back in a couple of weeks if he had not improved. The bronchitis got better but a tremor on his left side had started and then I noticed his gait had changed almost overnite. I immediately took him back to the pediatrician and she was stunned and set us an appointment at Vanderbilt Children's Hospital for an EEG and an MRI. It was Friday, Feb. 19, 2010 when we got the devastating news from our pediatrician on the telephone in the MRI recovery that Nathan had a 4.5cm. tumor in his thalamus/hypothalamus with an 8cm. cyst in his 3rd. ventricle. We immediately met peds neurosurgeon Dr. Matt Pearson and his team and Nathan was admitted to the ICU. Resection was scheduled for Thursday the 25th. The surgery itself was about 9 hrs. long. Dr. P came out and talked to us after and was unable to get all of it. It is wrapped around the main vessels in his brain. We still had some hope that it would go away. We also had to have a shunt and a shunt redo in March the day before Mike's major surgery. We went home and began a grueling physical, speech and occupational therapy schedule while battling crazy temp. fluctuations and steroids. The resection led to a PICC line and my crash course in nursing and round the clock antibiotics. Nathan had to learn to walk again. Mike was very sick through all of this from his own cancer. After 12 weeks (May) we returned to VCH for an MRI only to find the tumor had already grown back to it's original size but w/o the cyst. We immediately had a port in & started a grueling 10 week course of vincristine and carboplatin. Vandy is about 5hrs. roundtrip and it was very stressful being so far from help. Our local healthcare was not prepared for a pediatric patient and he was very sick. He was hospitalized on week 9 and he got an NG tube. We finished the 10th trmt on Friday and had another MRI on Monday Aug. 2, 2010. The chemo wasn't working and the tumor was growing. His case was again at the Tumor Board and it was recommeded we start radiation and Temadar ASAP. At that point I began searching for another option. The tumor is inoperable this time and the radiation would be devastating to a 3 year old. I got in touch with the Children's Brain Tumor Foundation after reading Elise's story and another story about the McCaslin family I found in a Yahoo search. We made plans for the 13 hour trip to the TISCH Brain Tumor Center at Duke Medical Center in Durham, North Carolina to see Dr. Sri Gururangan (peds neuro oncologist). We had our appointment this past Monday Aug. 23rd. Dr. G has agreed to treat Nathan without surgery or radiation. We are so thrilled to have some hope. Nathan will start a 4 drug protocol next week at Duke. We are temporarily relocating to Durham for his treatment, we may be here 6 mos.-12 mos. or longer. We are anxious to get started and will keep you updated as to the protocols and progress as well as the side effects. Mike finished his chemo the same week Nathan did. In about 8 weeks Mike will have a PET scan and hopefully all is well, if not Mike will be moving his care to Duke too. It's truly amazing we are all still alive and somewhat sane. Thank you all- I'm still new in the tumor world but would love to hear from anyone who has a similar situation or a DIG.`

On August 30th we arrived at Dr. G's office @ Duke. We immediately started an IV antibiotic Pentamodine, to prevent a certain type of pneumonia. Nathan had developed a swelling in his belly that had started in early July but we could not get anyone to address the situation. It was enough of a concern that it was causing him to vomit and waddle when he walked, he grunted all the time. Dr. G was immediately concerned and started doing tests including a CT scan, ultrasound, MRI and shunt series. We also had a baseline hearing test. Nathan started his first round of chemo inpatient on Tuesday the 31st. Day 1 & 2 was Vincristine and Cysplatin, day 3 was Cytoxan. On Friday, Sept. 3rd. they stuck a needle into in peritoneal cavity where the shunt empties and drained 1.6 liters if CSF fluid out. There was immediate concern that he had some kind of infection so we were preparing for a shunt revision, external drain and heavy antibiotics for several days. An infection could travel up the shunt to his brain. They immediately tested the fluid and no infection but also no explanantion as to why his body isn't reabsorbing like it should. Any surgery is risky after starting chemo-accessing the port is a risk. We were discharged on Saturday and went to the clinic for a Neulasta shot. Insurance won't pay for it unless it is outpatient. Anyway, we went to the clinic on Tuesday the 7th & Thursday the 9th for labs. On Thursday, his counts were terrible! He was fine the previous Tuesday and still had no fever and was playing and eating so we were very surprised. It was exactly 10 days since we started chemo and Dr. G told me this would happen. The Neulasta hadn't had time to kick in yet. We looked at the MRI and the tumor had grown about 2mm. since our last MRI @ Vandy, he had been off all chemo for 30 days. The ventricles were already starting to swell because the shunt couldn't drain due to the fluid buildup. We were admitted back into the hospital with 103 fever on Thursday the 9th and discharged today the 13th. He only had fever on Thursday and his counts recovered quickly. Today we learned that the cytology on the drained fluid showed tumor cells in his peritoneal cavity. Dr. G expected this and is very surprised there aren't nodules all over. We weren't even aware this was a possibility. We also learned that radiation will not kill the tumor. Nathan will always have it, because it is inoperable-hopefully it will stabilize and shrink. We were told radiation would kill it and that primary brain tumors don't spread. That is not true if you have a shunt. Dr. G is very confident that the chemo will get all of the cells. Nathan will go back in the hospital next Tuesday the 21st for round 2 of the Vincristine, Cysplatin and Cytoxan. 21 days after that he will start an oral drug called VP-16, that will last 28 days and then it is time for an MRI. If this protocol does not work, we will start a different protocol. The main thing is that we are informed, we know what to expect and when. I strongly encourage anyone to get a second opinion, even if you think your doctor is right. Brain tumors are strange and they do strange things and they don't act like malignant tumors should act. If your medical facility is not familiar with brain tumors go somewhere else. We will keep you posted. Nathan is doing better and feeling better than he has since Christmas. He is active, happy, busy and so loving. We have an armory of meds, and we have bad spells-but they don't last for days on end anymore. Sometimes it's just a rough morning or a sick afternoon. There's hope-just look for the experience. Dr. G has answered all of my what if questions-there are no we'll have to see or we're not sure.