My name is Bridget Agnello & I’m the single mother of 13 yr old Joseph. Joe was diagnosed with Stage IV metastatic ASPS on June 27, 2015 when he was 12 years old. We live in Rochester, Michigan (USA), 30 miles north of Detroit. He’s a patient of pediatric oncologist Maxim Yankelevich, who’s affiliated with Children’s Hospital of Detroit.
Joe had a 7.5cm tumor excised from his right, posterior thigh on June 19th. With less than a 1mm margin of clearance, his pediatric surgeon took him back into surgery on June 29th & in all, removed approximately 35%-40% of Joe’s right quadriceps. His pediatric surgeon is Dr. Nathan Novotny, an extremely gifted surgeon as well as a genuinely supportive, caring person! He is the reason that Joe did not have to have his leg amputated nor did he have to have any radiation therapy to this primary site. Joe still has some residual weakness in the leg at the end of the day, but otherwise you can’t tell from seeing him walk what happened to his leg.
On June 18, the night before Joe’s first emergency surgery, he underwent the following tests:
1. Lung - X-ray
2. Femur – MRI
On June 30, after his second surgery, Joe underwent the following tests:
1. Full body PET scan
2. Brain CT scan
3. Lung CT scan
At the time of his Dx, he was a patient at Beaumont Hospital in Royal Oak, Michigan. The Chief of Pediatric Oncology told us Joe has multiple bilateral lung lesions (approx. 15), with the largest ones being a 6mm in the right upper lobe & a 4 mm in the left upper lobe. Beaumont Hosp. has never treated a case of ASPS, so we were referred to St. Jude Children’s Research Hospital in Memphis, Tennessee. Joe was evaluated by Dr. Alberto Pappo, who is touted as the world’s authority on ASPS. (St. Jude has treated only 11 cases of ASPS in the last 30 years I found out later.) Pappo said St. Jude couldn’t do anything for Joe that we couldn’t get done back in Detroit with Dr. Yankelevich. So he recommended Joe begin 37.5mg of Sunitinib.
Pappo & Yankelevich both told me that there was nothing we could do about the tumors in Joe’s lungs. Apparently the amount of radiation his lungs would need to kill or shrink his tumors would also destroy the tissue. Joe not only has surface tumors, but he has them deep in each lobe. Upon receiving a PET CT from Children’s Hospital on October 16th, Dr. Yankelevich corrected Beaumont Hospital’s diagnosis of approx. 15 pulmonary tumors, and told me that there were too many to even count.
Even though I’ve been researching ASPS since the moment of my son’s diagnosis, I realize now that I let myself get lulled into a fall sense of security with the Sunitinib use. Four months after his Dx, he doesn’t appear to have any disease progression. But who knows if that’s because of the ASPS is in a stable state right now or if the medication is working. I have to say that I hate what the medication does to Joe. He’s had bouts of uncontrollable nausea, vomiting, diarrhea & migraine headaches (spent Sept. 2nd -4th in the hospital on Zofran & Morphine). The palms of his hands and soles of his feet have peeled, cracked and bled; his hair has begun to fall out while the remaining hair follicles on his entire body are turning albino white; he is retaining fluid and is perpetually puffy; and he’s developed acute arthralgia (arthritis) of his knees, ankles and hips. Five weeks ago I had him at the ER in a wheelchair because he couldn’t walk. He was in tremendous pain and he said it felt like he had 50lb weights attached to each leg.
After the arthritis, the edema & the hand/foot bleeding side effects all presented at once (the ER visit I wrote of), Dr. Yankelevich D/C’d the Sunitinib for 8 days. The side effects for the most part disappeared, except Joe hasn’t lost all the all-over edema. Dr. Yankelevich started Joe back on 2 pills instead of the full dose of 3 pills, and so far so good. Yesterday was the first day Joe began taking 3 pills again. I’ll be keeping a close eye on his symptoms. If he goes toxic again, he’ll either go to 2 pills and remain there or be switched to Pazopanib.
In all of my sleepless nights since this started, I found Dr. Rolle and all of you and Dr. Novotny has reached out to him. He’s preparing all of Joe’s scans to send to Dr. Rolle next week. Most of our doctors are telling me that Joe’s tumors are so small, that I shouldn’t put Joe through more surgery. But I feel as if I need to lessen his tumor load. Do you think this is too hard of a surgery for a child? Joe has no other medical conditions except for controlled Asthma and I worry about his lungs’ ability to heal.
Has anyone on this forum researched Non-Invasive Ultrasound Surgery? It was pioneered in Israel for brain tumors and has now been adapted for other parts of the body. Joe’s pediatrician is researching for me where else it’s being performed and its success rate.
Are there any other children on this forum with ASPS? I haven’t been able to connect with anyone else in Michigan who has this god-forsaken disease. I’ll write more later… too sleepy to continue for tonight. Thank you again for being here and listening and offering advice!
Love....Bridget
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