Ryan Eric Schafer
DSRCT Diagnosed 2/91 at age 12 - Passed Away 12/17/95
|From Ryan's web page:
In the summer of 1990, when Ryan was 12 and vacationing in Florida, Ryan complained of a bad stomach ache. It came and went during the last two days of our vacation and Ryan wanted to wait until we got home to see his doctor. I remember as he boarded the plane he was holding his stomach and wincing in pain. Ryan's doctor at home ran some tests and said it was stomach ileitis. Our family history contains a long list of appendectomies at age 12/13 so perhaps, I thought, this could also be a signal for a possible inflamed appendix. During the following months Ryan felt fine even though he was tired from basketball practice.
The Horrible Night
On an ordinary Thursday in Feb.1991, I came home from work and found Ryan
doubled over in pain, ashen in color, and truly hurting. He asked me to take
him to the ER because he couldn't stand the pain. This from a child who
never complained about pain. The ER doctor ran some blood work did an xray
and called in a surgeon who promptly told us he thought it was Ryan's
appendix and it had to come out before it ruptured. So at 10 PM Ryan went
Twenty four hours after Ryan's abdominal surgery, we transferred him to Children's Hospital of Philadelphia (CHOP). Our local hospital had mentioned Wilm's tumor but CHOP Drs. said Wilm's was very rare in children over five years of age. They thought perhaps Burkitts Lymphoma. The team of doctors decided another surgery was needed to try to remove the tumor and do a biopsy on it. Exactly one week later on March 8, 1991 Ryan's 13th birthday, Dr. Arthur Ross, removed a 7cm x 8cm x 9cm tumor from Ryan's pelvic floor. He had to peal it off the other organs in a five hour operation. Other much smaller tumors were also found sprinkled throughout his abdomen. A Dektal mesh sling was inserted into Ryan's abdomen to elevate the abdominal organs to allow the radiologist to "cone done" into the pelvic floor to deliver more direct radiation. The sling would descend by itself in six weeks. Pathology slides were sent to many University hospitals. We were told it was synovial sarcoma at first. The Dr. Rosai from Sloan Kettering diagnosed Ryan's cancer as Desmoplastic Small Round Cell Tumor of the Abdomen.
Ryan presented with abdominal pain and was found to have a pelvic mass (palpable from the rectum). He underwent resection at CHOP. In addition to the pelvic primary, he had omental and peritoneal metastases studding the large bowel. The pathology revealed a diagnosis of DSRCT that was corroborated by Juan Rosai ( the pathologist who originally described this pathologic entity).
This DSRCT is so rare that very little information was found. Ryan's doctors had no protocols to follow. Ryan's treatments were developed for him as the doctors went along. First he received Vincristine/5-FU along with seven weeks of irradiation. A portacath was inserted but malfunctioned shortly thereafter and was subsequently replaced with a Broviac. After irradiation Ryan received a chemo cocktail of Carboplatin, Cytoxin, and VP-16 along with IV and oral Ondansetron (Zofran) for four rounds. Several times his chemo had to be delayed because of low counts or infection. At one point his platelet count was 3,000. Several CT scans were done after radiation and October 91 his Broviac was pulled and we were told he had "No Evidence of Disease". Ryan's oncologist transferred to Albany Medical Center in July 1992 and Ryan wanted to follow her there. They had built a great rapport together and both were very "in tune" with each other based on mutual trust and respect.
Ryan received total abdominal irradiation accompanied by weekly Vincristine/5-FU, followed by four cycles of Carbo/VP/Cytoxan. He tolerated this therapy quite well and was disease free for two years (10/91-12/93).
For two years we thought Ryan had beaten down this horrible cancer. We
traveled a lot -Ryan especially loved Hawaii and standing at the Arizona
memorial at Pearl Harbor - because lurking in the back of our minds was that
constant fear of relapse. Our fears became reality when on Dec. 31, 1993,
after severe abdominal distress and all efforts to dislodge an abdominal
obstruction failed, Ryan underwent his third abdominal surgery hoping to
find adhesions. That was not the case. A large tumor had choked his
intestines by the ileum, where the large and small intestines join. Another,
smaller tumor was found partially blocking the small intestine "upstream".
An ileostomy was performed to eliminate the blockage. Once again, numerous
small tumors were found throughout his abdomen.
In Dec 93 Ryan presented with a small bowel obstruction. At laparatomy he was found to have a large cecal mass, as well as, diffuse peritoneal metastases studding the serosal surfaces of the abdominal cavity, covering the small and large bowel. In addition, the surgeon noted palpable small nodules in the left lobe of the liver(not visible by CT). He underwent ileostomy for decompression and several biopsies were taken. The pathology was identical to his original tumor. Chest CT, bone scan, and bone marrows were negative for metastases. Ryan was begun on the Sloan Memorial P6 protocol consisting of Cytoxan/Adriamycin/ Vincristine alternating with Ifos/VP. He received two courses of this regimen before running into trouble. Following a round of IFOS(15g/m2over 5 D) he became obstructed. We initially suspected tumor, however after several days of bowel rest and serial examinations it resolved spontaneously. The obstruction was thought to have been caused by a terrible ileus with bowel edema. Therapy was changed to a protocol used successfully at MD Anderson which was a combination of Bleo/MTX/Cisplat/VCR alternating with Cytox/Actino/VCR. Ryan received 3 cycles of B/M/C/R and 1 cycle of ACE.
Dr. Pizzo at the National Cancer Institute had suggested shortly after Ryan's diagnosis to consider harvesting his bone marrow in case the cancer traveled to his marrow or ,if more powerful chemo was used, it would be needed to restore his depleted marrow. On April 4, 5, 6, & 7, of 1994 Ryan's stem cells were harvested. This procedure had to be timed just right with the administration of Neuprogen. The harvest was successful. Ryan's stem cells were in the bank.
During the first half of 1994, while Ryan was receiving his chemo, we had decided to try to find a surgeon who would cut away as much of the small tumors as possible and also repair his ileostomy. which was seriously limiting Ryan's activities. Dr. Ross gave us several doctors names. Finally in August of that same year, Dr. Eugene Wiener at the Children's Hospital of Pittsburgh performed Ryan's fourth abdominal surgery and removed his ileostomy and restored him to normal. It was a very happy time for Ryan. Dr. Wiener however, did say that the tumors were "sprinkled like salt" throughout Ryan's abdomen and it was impossible to remove the small tumors. We journeyed home to begin Ryan's junior year in high school.
Ryan underwent re-exploration by Gene Wiener who found that the cecal mass was 1/3 the size of the tumor described in 12/93, there were no palpable liver nodules, however there were persistent diffuse carcinomatosis studding the serosal surface that had active tumor. Ryan was able to be closed with reanastomosis of the ileum and recovered uneventfully. Since the use of Cisplatin, Ryan has had persistent magnesium wasting and is on daily supplements.
It was a given that Ryan needed some sort of treatment for his active cancer. Because none of the other drugs had obviously completely stopped the tumor growth, it was decided to try Taxol. Ryan began Taxol in Nov 1994. This was Ryan's junior year in high school and he missed very few days. His Taxol treatments were "a walk in the park" compared to the heavy duty he had received. previously. Ryan felt though that his body was not responding to Taxol. He was concerned that he was still have intestinal spasms and weakness. At this time he developed bleeding in the bladder (hemorrhagic cystitus). He prodded me to contact a bone marrow transplant center to begin preparation for a transplant. Ryan felt that this more radical treatment offered him hope.
Fred Hutchinson Center in Seattle Washington turned Ryan down sighting a thickening of the bowel wall. Pittsburgh agreed but we felt they did not feel it would accomplish anything. Ryan finally settled on the University of Wisconsin Children's Hospital. Ryan left for Madison, Wisconsin on Sept. 4, 1995 via Angel Flight a terrific organization of pilots who use their private planes and corporate jets to fly children with cancer to treatment centers all over the world.
During preliminary testing it was discovered that Ryan had two small
tumors growing behind his bladder. They did a transverse biopsy to identify
them. They were again, DSRCT. Ryan underwent total body irradiation and
then, two weeks later, a triple lumen Hickman (central line) was inserted
surgically to replace his double lumen Hickman. He then received chemo -
Melphalan, carboplatin, and etoposide. On Oct.19 Ryan received his stem
On Saturday, October 22, at 6:15, Ryan coded. They intubated him and rushed him down to for a CAT scan telling us they thought he had a brain bleed. Large quantities of platelets had to be administered during the scan. Ryan did not have a brain bleed.
Ryan was now in full blown septic shock and they identified the source as his new triple lumen catheter which they pulled immediatelatly. Two days later on Tuesday, we were told that Ryan's infection, klebsiella pneumoniae, was in his superior vena cava (where his central line had been) and traveling towards his heart. If open heart surgery was not performed that evening Ryan would die the next day. Since Ryan had no white count, no platelet count and very little red count, his chances of surviving the operation were 50/50. If he had to go on bypass, via heparin, his chances of survival were 10%. We had no choice.
Ryan's heart surgery lasted six hours. He lost four liters of blood. The superior vena cava showed near-complete occlusion and had to be cleared and bonded with a bovine pericardial patch. Ryan would have died immediately after the infectious clot blocked nearly all blood flow, but Ryan, fighter that he was, had TWO superior vena cavas so when the flow of blood was blocked in the one vein it rerouted itself through the second vein. Ryan did not go on bypass. We had Ryan back and fighting.
Ryan continued to get better a little each day, so it seemed. His white cells came back in ten days. He kept getting fevers and had constant bouts with falling blood pressure. Just when we thought the sepsis was under control it would appear in another location. His dependency on the ventilator became less and less. On the Monday before Thanksgiving Ryan was down to only four breaths a minute which meant he was practically breathing on his own. The doctors wanted to pull the respirator on the day after Thanksgiving.
On Thanksgiving Day our family who kept vigil over Ryan, ate a fast turkey dinner at the Ronald McDonald house. When we returned after two hours we were met at the door by Ryan's nurse and told things had taken a turn for the worse. When I saw Ryan I couldn't believe my eyes. He had gained about thirty pounds and his abdomen was distended. The two liter bag of hydration they had hung to flush his bladder was till flowing and his catheter bag was empty. They had blown his bladder. Ryan was rushed to surgery at 11;30 at night to repair his bladder. From then on Ryan's body started to slowly give out. He had to go on kidney dialysis and all the time fighting this infection hiding out somewhere in his body. Swan dance lines were inserted and at one time Ryan had fourteen pumps. He was being transfused with platelets every four hours. Ryan fought bravely and valiantly. We know he heard us as we talked to him constantly. We made sure he was in no pain. Finally, after almost two weeks we told Ryan he had to make the decision - we would honor his decision as we had always honored him. After his sister told him that she would watch over his family, Ryan quietly and lovingly died with his sister by his side on December 7, 1995 - a day he picked we're sure because of his great love and admiration for airplanes and Hawaii. Ryan will always be the wind beneath our wings.
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