Joe's Fight Against Alveolar Soft Part Sarcoma
Team Member: Alfred Linton
Bridget Agnello wrote -
Hello and thank you for visiting our site. We are Bridget Agnello & Al Linton, Joe's parents, and we are directing his cancer treatment in tandem with his oncologists.
Joseph Linton-Agnello was 12 years old when he was diagnosed on June 27, 2015 with Stage 4 Alveolar Soft Part Sarcoma (ASPS). ASPS is an atypical sarcoma, because it involves a specific breaking and swapping (in utero) of an arm between chromosome 7 & chromosome 11, which is called an “unbalanced translocation.” Highly vascular tumors form as a result, on soft tissue within the body. Symptoms are often undetected until the disease has metastasized and secondary symptoms are present. ASPS is one of the rarest sarcomas in existence striking young adults and children. Between 1,500 and 1,700 U.S. children are diagnosed with a bone or soft tissue sarcoma each year, making up about 15% of all pediatric cancers. Out of those 1,700 sarcoma patients, less than 85 children a year will be diagnosed with ASPS.
ASPS spreads (metastases) to the lungs and to the brain primarily. Unfortunately, ASPS is highly resistant to chemotherapy; surgery and radiation remain the major ways to control the cancer's growth. But this has not proven to be a very successful treatment protocol. Because of the rarity of this sarcoma type, treatment options are extremely limited. Forms of treatment such as chemotherapy, surgery and radiation have not proven fully effective for the long-term management of the disease. Very early clinical trials utilizing inhibitor therapies (TKI medication) have shown some response in less than 30% of patients it was administered to. So further research is required. Because the clinical trials are still in early stages, long-term prognosis of patients is classified as unknown to poor at best. Sarcoma research receives less than 1% of cancer research funds and ASPS itself receives nothing.
In June, Dr. Nathan Novotny of William Beaumont Hospital in Royal Oak, Michigan performed two emergency surgeries on Joe. He removed a baseball-sized tumor as well as approximately 30-35% of the lateral and posterior Quadriceps muscle in Joe's right thigh. The ASPS has metastasized to his right groin lymph nodes as well as to his lungs. The oncology radiologist said there are too many tumors throughout Joe's lungs to even count. At this point, radiation and surgery is no longer an option; the amount of radiation required to shrink these dozens of tumors would destroy his lungs. Surgery is also not an option here in the U.S. because I've been advised that Joe wouldn't survive the blood loss and infection, from the only approved lung cancer surgical procedure. So I've been researching & found several global locations (England, Germany, Japan) that perform "Laser-Assisted Metastasectomy" on lung tumors. This is a successful focused laser beam surgical procedure that's been done abroad for over two decades. Dr. Novotny is helping us vet each location before we decide what treatment(s) to pursue for Joe.
Joe, Al and I flew to St. Jude Children's Research Hospital in Memphis, TN on August 3, 2015. Dr. Alberto Pappo, the Solid Tumor Oncologist in residence, sent us home after 5 days of tests, saying there was nothing he could do for Joe. St. Jude has only consulted/treated on 11 ASPS patients in the past 40 years and there's just not much research data to base a treatment protocol on. However, Joe began taking a full dose of Sunitinib on August 8, 2015. Sunitinib is a vascular inhibitor oral medication that ASPS patients have been put on in the past to shut down the blood flow within the tumor. However the Sunitinib's side effects are not very pleasant. At various times Joe wil experience one or more of the following side effects: nausea, vomiting, migraines, visual distortion, acute tiredness, open sores on his hands and feet, skin rash, hair loss, swelling/bloating, hair pigment change from black to white and acute arthritis in his hips, ankles and knees. That's not quite what I thought puberty would involve when Joe hit his teen years....
Joe will soon be a patient at University of Michigan C.S. Mott Children's Hospital and he also has a Miami, Florida oncologist. Dr. Breelynn Wilky of University of Miami-Sylvester Comprehensive Cancer Center is one of very few ASPS cancer researchers in the U.S. She believes that a new targeted combination immuno-therapy medication is the strongest method available to keep ASPS from not only advancing but even shrink existing tumors. Unfortunately, Joe is only 13 years old and over 90% of U.S. medical trials are geared toward adults. Dr. Wilky is talking with Merck Pharmaceuticals to see if they'll allow Joe into the trial she has beginning in February, since Joe is currently the height and weight of an adult.
For the sake of transparency I want you to know that I'm using CrowdRise to raise funds for several purposes.
1. To help us pay for Joe's rising medical bills and medication cost
2. To help pay for our personal bills. When I take off from work for one of Joe's medical appointments or hospitalizations, I don't get paid; I've used up all my paid time off. (Flying to Miami or to elsewhere in the U.S. costs airfare, ground transportation, housing, meals, etc.)
3. To pay for surgical/medical treatment outside of the U.S., if that turns out to be a viable treatment option.
4. I (Bridget) am starting a non-profit charity to raise money specifically geared toward ASPS research so that Dr. Wilky, Dr. Kevin Jones (Univ. of Utah-Huntsman Cancer Institute) and Dr. Yosef Landesman can concentrate on finding a cure for ASPS and not have to stop working to pursue a new funding source.
There are only a handful of ASPS cancer researchers in the U.S. and they need all the help they can get to save those afflicted with this horrid disease! I'll let you guys know when I get the charity finalized and I'll list it here on CrowdRise in place of this page. Thank you so VERY much for reaching out and helping us to save our son-- we appreciate each and every one of you!