DSRCT Diagnosed 7/96 at age 16 - Passed Away 4/30/97
From Timothy's web page: Initially, doctors diagnosed Tim with a stage IV carcinoma of unknown primary site on July 2, 1996. He was a 16 year old football player/body builder weighing about 235 pounds. Tim's mother, a nurse, wanted his biopsies sent to the National Institute of Health (NIH). At first, the doctors balked, telling Judy, "to quit obsessing and accept the diagnosis." However, Judy persisted and soon the NIH forwarded a diagnosis of desmoplastic small round cell tumor.
At the time of his diagnosis, Tim had multiple metastatic sites including: liver, spleen, pancreas, adrenals, a pelvic mass, cardinal nodes, mesenteric nodes, thoracic nodes, bone mets and, most unusually, bone marrow involvement. He could not lie on his side or stomach and required 60mg of long acting morphine every eight hours and an IV push in between the oral doses. Tim also had a long history (seven years) of Common Variable Immuno-deficiency for which he received IVIgG.
He began Dr. Kushner's protocol (See Kushner et al, 1996) at the Children's Hospital of Pittsburgh around the beginning of October, 1996. Tim experienced major neutropenia after the first treatment of adriamycin, vincristine, and cytoxin and nearly went into hypovolemic shock. He required substantial blood support (six units of packed red blood cells and 26 units of platelets).
Despite these difficulties, Tim showed a definite response after two cycles of chemo. He was now able to lie on his side and stomach and no longer required morphine. By the time Tim completed five of seven planned chemo cycles, his masses were shrinking. Shortly thereafter, his bone scan became nearly normal. (By March his bone scan improved to completely normal) He required some sort of blood support during the majority, if not for all, of these chemo cycles. He also experienced several more episodes of neutropenia.
He has this agenda for December.....Christmas dance at school, gotta date, must be home. Christmas, must be home. December 31, birthday must be home. Around this plan we must fit 2 chemo sessions, a predictable "crash" with hospitalization and MORE stem cell harvest because we didn't get enough in these 7 sessions. Somehow I think it may not work out but the kid is an eternal optimist and if anyone can convince the Docs it is he.
A bone marrow transplant is a critical part of Kushner's protocol. Since Tim's siblings didn't match as donors, he began pheresis to harvest his stem cells. He underwent nine sessions to harvest the required amount of stem cells, finishing on Christmas Eve, 1996..
Tim completed the sixth and seventh chemo cycles consisting of ifosfamide and etoposide early in 1997. Complications like neutropenia, cellulitis, and pneumonia continued to follow him, but with somewhat less severity than previous treatments. At one point, however, he needed 10 units of platelets.
In the third week of March, 1997, doctors made a decision to perform debulking surgery prior to his stem cell transplant. Tim's bone scan was now normal, but small masses remained in his abdomen, liver, and pelvis.
Tim's surgery, performed in the first week of April, went quite well. Surgeons removed his omentum, biopsied his liver, and removed tumor seeding from the peritoneal wall and diaphragm. As expected, they were unable to completely resect the tumor. The liver, especially, was found to still contain a large amount of tumor.
Tim and his family understood the risks associated with a stem cell transplant. His doctors warned of the likely possibility of nasty mucositis and also of infection. Other possible complications were kidney failure (requiring dialysis), respitory distress (requiring respirator support), and malnutrition (requiring TPN).
Conditioning for the stem cell transplant began on April 14th. This time the chemicals of choice were thiotepa and carboplatin, given over a period of six days. The transplant took place on the 23rd.
Tim's playfulness surfaced even under the duress of this strenuous treatment. Judy described a late night incident to me in a phone conversation. One night Tim became hungry and mother and son left the transplant unit to go down to the cafeteria. It seems that Judy is not especially comfortable in an elevator. Preying on his mother's anxiety, Tim began to jump up and down in the elevator. He not only succeeded in scaring his mom, he also managed to get the elevator stuck between floors!
Sadly, Tim soon developed a severe infection with multi organ failure. He
had cardiac and kidney complications previously undocumented in patients
undergoing Kushner's protocol. Tim passed away at 7:30AM on April 30, 1997.
He was seventeen years old.
www.dsrct.com desmoplastic small round cell tumor