Sorry we have not updated Jon's page in such a long time. We have been so hectic with doctor appts. and decisions that have weighed so heavy on us. After Jon's June MRI which showed progression of his tumor, Jon, JonJon and I traveled to Duke University to see Dr. Guruangan of their brain tumor clinic in Durham, NC. It was a very depressing trip for us as Dr. G told us in 30 years he has never seen anyone survive a DIPG tumor. Most kids die within 6-12 months and it is rare for a kid to live more than 18 months. The DIPG tumors are very resistant to Chemo and since they can not be biopsied it is hard to know which drugs could work. He did give us a road map of a treatment plan that has used with adults with different tumors but is still experimental in pediatrics. The plan involves "cocktailing" different chemo drugs the main ones being Avastin and Irinotecan alternating them in a 12 week cycle. The list of potential side effects are extremely frightening in addition to the usual chemo effects (nausea, diarrhea, nose bleeds, fatigue and poor blood counts). The Avastin can cause hypertension, protein in the urine, blood clots and brain bleeds . Jon and I have struggled with what to do, we don't want Jon's time on this earth to be spent feeling horrible.
We discussed with Jon's oncologist and Dr. Kathy Warren from the NIH who has seen Jon's scans and history and they both concurred that the Avastin and Irinotecan are the next logical step, if anything about this is logical. The hope would be to stabilize the tumor or slow it.
After much delay and frustration, (doctors out of the country) Jon Jon has had a mediport implanted at Children's of Buffalo . Aug 2nd he had another MRI showing even more progression, we figured that could be the case as Jon has had poor speech, balance and strength issues, drooling, and trouble swallowing. The poor little guy has choked several times so we have to be very careful that he takes small bites and chew thoroughly. On Aug. 6th we were back at Children's to see if the mediport was healed enough t0 begin infusions that day, it was and his port was accessed for the first time for blood draws. The nurse had a very hard time getting the needle in the right spot and that was very painful for Jon but he was his usual trooper self. We waited for 8 hours to see if we could have an infusion of Avastin only to be told by Dr. Fitzpatrick that his latest scan was reviewed again with the neuro surgeon and a tiny bleed was found within the tumor which we were told is not uncommon but the Avastin could put him at risk for a bigger one. We all decided to be cautious and not proceed with the infusion until Dr. Guruangan from Duke reviewed the latest scan but he is out of the country. Another Duke doctor said we should wait and monitor the bleed.
This Mon. we hope to hear if we can come in for the Irinotecan with maybe another drug combo. We feel so helpless and desperately want to get started on something!! Jon is getting weaker and needs help getting up stairs and in and out of the car, he can only walk a short distance. We keep hoping and praying that there has to be something to stabilize his tumor. We have asked Jon Jon how he feels about all he is going through and if he has questions but he does not have much to say. I think it has gotten to be a way of life and he just rolls with it . Jon's doctor arranged to have Essential Kids come in to our home and do physical therapy and draw blood and flush the port when it is accessed.
On a lighter note Jon has gotten to do some fun things this summer in small doses. Last Saturday he went to a Star Wars convention at the Summit Mall with Aunt Julie and Uncle Roger and daddy. He got autographed pics from the man who played Darth Vader and the man who played the ewoks and added some trinkets to his collection. Thank you to his aunt and uncle for making it happen. Uncle Roger got him started with his Star Wars obsession and they have enjoyed it together! Our family went to the St. Chris carnival and Jon managed to ride the Ferris wheel and go down the slide with Jon. Of course daddy won Claire and Jon some stuffed treasures! Claire and Jon went to paint pottery and to lunch with Uncle Steve while waiting for Aunt Elizabeth to get out of work they had a sleep over and Claire was treated to a manicure and pedicure done by Liz. I think we have a little diva in the making!
This past Thurs Jon Jon was able to ride the golf cart with Jon for a few holes, we will post some pics. Little Jon stayed a few extra days with me and my 3 sisters and his grandma Patsy in Syracuse at a horse show I was working. Our horse show family was happy to see him and he enjoyed being surrounded by some of his favorite people. Claire and Jon have been seasoned horse show attendees since they were infants.
Claire has been enjoying her summer in grandma Susie's pool and our giant Banzai water slide. Her favorite playmates, Jamie and Jacob come over and slide for hours and when they have had enough they go across the street to their house and do the trampoline and pool. She also has been going to Gleason's gymnastic camp 2 days a week in the am and loves it!
Well I think that catches everyone up to speed for now, we will keep you posted on what Jon's next treatment is and how he tolerates it.
We really cherish the strong support and prayers from our family and friends we are blessed. Please keep praying that Jon will see some stabilization and that we will make the right decisions for our dear sweet boy. He is so strong and brave with all he has been through he still smiles and giggles so easily!
Love, The Cinelli family