Gerry Webber (Brazil)

DSRCT Diagnosed 10/99 - Passed Away 8/2000

From Gerry's Web page:                           Treatments:
    Treatment of DSRCT varies from patient to patient, but the most effective treatments have consisted of early resection of the tumor(s) when possible and high dose multi-agent chemotherapy followed by a stem cell transplant.  Many cases include a mid-chemo surgery to remove any residual disease and make the subsequent chemo treatments more effective.
    There are two main protocols reported in the literature: the P6 Protocol developed by Dr. Kushner at Memorial Sloan-Kettering Cancer Center in New York and the Eicess EVAIA protocol used mainly in Europe.  Both protocols use similar chemo drugs, use extremely high doses of chemo, and have a similar number of courses.  Most successfully treated cases report the use of these, or variations of these protocols. It must be remembered though, that these protocols are not the only options.  Each individual case is different and must be researched independently to determine the best approach for treatment.

The P6 Protocol:
    The P6 Protocol consists of seven courses of chemotherapy with surgery after the third course (if necessary), followed by a stem cell transplant.  The courses are described below or see Abstract #68 on the Research Abstracts page for Dr. Kushner's 1996 article.  Maximum tolerable doses for each course are calculated by the patients weight and age.

        Courses 1,2,3 and 6
                (Cyclophosphamide is administered with
                two 6 hour infusions on the first 2 days.
                Doxorubicin and vincristine are administered
                by a 72 hour continuous infusion)

        Courses 4,5 and 7
                (Both infofamide and etoposide are administered
                by 1  hour infusions over 5 days)

        Suplimentary Drugs
                 (Mesna is administered by intravenous injection
                 with the cyclophosphmide in courses 1,2,3 and 6,
                 and with each infusion of courses 4,5 and 7.  It is
                 used to prevent bleeding in the bladder and kidneys)
            filgrastima (G-CSF)
                  (G-CSF, a blood cell growth stimulant, is
                  administered by
subcutaneous injection
                  for 10 days after the completion of each

       Additional Drugs
            Zofran (anti-nausea)
            Sulfametoxazol + Trimetoprima (anti-pneumonia)
            Ranitidina or Zantac (anti-ulcer)

    After each course, blood counts are monitored closely and transfusions of whole blood and/or platelets are given if blood counts get too low.  Subsequent courses are started after blood counts rise back to normal levels, usually 18 to 20 days after the completion of the infusions.

       Stem Cell Transplant

    The stem cell transplant procedure consists of first harvesting stem cells from the patient, administering high doses of thiotepa and carboplatin, then re-introducing the harvested stem cells into the blood stream along with G-CSF.

The Eicess EVAIA Protocol:
    The  Eicess EVAIA Protocol has been used mainly in Europe and has been commonly used to treat Ewings Sarcoma.  This protocol consists of eight courses each consisting of:

        Courses 1 through 8

        Suplimentary Drugs
                 (Mesna is administered by intravenous injection)
            filgrastima (G-CSF)
                (G-CSF, a blood cell growth simulate, is
                administered by
subcutaneous injection
                for 6 days after the completion of each

        Stem Cell Transplant
            (I don't have specific information on the drugs
            used, but stem cell transplants are not
            unique to either protocol)

   November 22, 1999 - December 3, 1999:    During the first week we met with the oncologists to discuss what treatment protocol we were going to use.  Two oncologists where to be my doctors, Dr. Luciana Coura and Dr. Jose Bines.  There had only been four other DSRCT patients in the history of the hospital.  They had all been treated by other doctors, who no longer practiced at the hospital.  Dr. Luciana and Dr. Jose both had been researching treatments and had decided on using Dr. Kushner's P6 Protocol from Memorial Sloan-Kettering Cancer Center in New York.  My own research had come up with the same conclusion.    I had called Dr. Kushner's office a few weeks earlier to ask about the protocol and to see what the cost of treatment would be at Sloan-Kettering. I wasn't able to talk directly with Dr. Kushner, but his secretary gave me his e-mail address and suggested that I write him a message.   Dr. Kushner never answered my e-mail, I guess he was reluctant to discuss these things directly with a patient.  I gave Dr. Jose Dr. Kushner's e-mail address and he wrote him to see if there were any changes to the protocol since his 1996 publication.  Dr. Kushner replied promptly with his latest updates and suggestions on the protocol.  I was scheduled to start treatment on November 29th.

   Course 1, November 29, 1999 - December 21, 1999:    I was admitted into the hospital on Monday November 29th to start the first course of treatment.  I was to be hydrated on the first day then start infusion of the chemo drugs starting the next day, but due to some problems in the chemo department, I didn't start the infusion until the fourth day.   There was a choice of two types of catheters Peripherally Inserted Central Catheter (PICC) or a Hickman Catheter.  The Hickman catheter would be surgically inserted under the skin in the chest or neck.  Though complications with the Hickman are rare they can include nerve damage causing pain and/or blurred vision.  Insertion of the PICC is a simple procedure with a small silicon tube inserted into a vein in the arm and extending about 16 inches up the arm and down toward the heart.  Both types of catheters require weekly flushing with saline solution and heparin to prevent blockage by clotted blood.  We chose the PICC and it was inserted by the nurse in the chemo room with no problems.
    Infusion of the chemo started on Thursday and continued through Sunday.   We returned home on Monday.  At first I felt no adverse effects of the chemo, especially no nausea.  For the next few days we just stayed home, monitoring my temperature every few hours.  My appetite was not what it was before and the food tasted a little bland, but I had no problem eating. 
    The most common side effect of the chemo is low blood counts of red cells, platelets, and white cells.  Low red cell counts, or anemia,  cause fatigue, weakness, and dizziness when standing suddenly after sitting or lying down or a period of time.  Whole blood transfusions are used to keep the red cell count above acceptable levels.  Platelets help clot the blood and prevent bleeding.  Very low platelet counts are dangerous not only from cuts, but from bleeding of the gums and internal bleeding.  When the platelet counts are very low, hospitalization is required for close monitoring and you are not allowed to walk or brush your teeth.   The impact of your feet on the floor can cause rupture of small blood vessels in the feet.  Low platelet counts are treated by platelet transfusions.  White blood cells are the primary defense mechanism of the body to fend off infection from bacteria and viruses.  With low white cell counts, the body is basically defenseless against infection and even bacteria normally present in the body can cause problems.   The only treatment for low white cell count is the use of antibiotics, but G-CSF is used as a growth stimulant to help prevent low counts.  G-CSF is administered for ten days after infusion by daily subcutaneous injection, similar to insulin injections.   Constant monitoring of body temperature is required, with any temperature above 38 C (100.4 F) requiring hospitalization.  Even though Dr. Kushner told Dr. Jose to expect high fevers for every course, I was determined not to get a fever.  But, by the 6th day at home, my temperature went above 38 degrees and I was admitted to the hospital.  They administered antibiotics and took cultures from the blood and urine to determine which type of infection that was present.  My temperature went back to normal within two days and the cultures came back negative.  No transfusions were necessary, but I was still required to stay in the hospital for five days.
    When we returned home, I was feeling normal again.  My hair started to fall out in clumps that week, so I got out the electric shaver and shaved my beard and head to avoid the mess.  I was scheduled to start the second course on Wednesday.

    Course 2, December 22, 1999 - January 23, 2000:    Infusion of the second course went well, though the nurse missed one of my Zofran injections and I had a vomiting spell at four o'clock in the morning.  We spent Christmas in the hospital and were released on the sixth day.   After we returned home, my mother flew in from the U.S. to stay for three weeks, and I was doubly determined not to get a fever this time.  Luckily this time this turned out to be true.  The first couple of weeks at home we just lounged around, visited with my mother, played cards and watched TV.  One of the blood tests during this time showed that I was anemic, so Dr. Luciana ordered a transfusion of two units of whole blood.  By the third week my blood counts were almost back to normal and we ventured out to explore Rio with my mother.  We visited Sugar Loaf Hill, Copacabana, Impanema and downtown.  Rio used to be a beautiful city in the 1950's.  Now it is run down, dirty, and dangerous.  But we had fun anyway.  At the end of the third week, my mother returned to the U.S. and started the third course.

    Course 3, January 24, 2000 - February 20, 2000:     Infusion of the third course again went well and I was released from the hospital after six days.  A few days later my a hole was found in my PICC during flushing and it had to be removed.  The timing was fortunate since the next two courses won't require a catheter.  What a relief!  For the next two months I can take a shower like a normal person, no dancing around the shower with a plastic wrapped arm raised above my head.  The next week was uneventful, but on the 8th day after the infusion a fever struck.  It was back to the hospital, this time the blood culture revealed an E. coli infection.  This infection caused chills, followed sweating spells.  One of the chills was extremely severe with uncontrollable shaking so strong that it became difficult to breath due to the chest spasms. This happened within the first few hours after being admitted to the hospital.    Luckily the antibiotics worked their magic quickly, there were no more chills, and the fever only returned on and off for the next three days.  My blood counts were so low this time that I was ordered not to walk or brush my teeth and I received a total of five units of whole blood and three units of platelets.  Finally my blood counts raised high enough to be released on the seventh day.
   Another CT scan was done which showed no evidence of tumor growth. The next week we stayed at home where I spent my time creating this web site.   In the mean time, the doctors were debating whether or not to perform exploratory surgery to see if there was any disease that had grown since the first surgery that could be removed.   This will be determined by Monday when they will either schedule the surgery or start the fourth course.

    Course 4, February 21, 2000 - March 21, 2000 :     Dr. Luciana, Dr. Jose and Dr. Kushner conferred via e-mail and agreed that in my case surgery at this time was not required.  Dr. Kushner has been very affable in conferring with the doctors here, and we are all grateful to him for sharing his experience.  Dr. Luciana expects the next two courses to go smoothly since they are less severe than the first three courses and are generally well tolerated by other patients.  We may even be able to make a trip back home for a week or so sometime early next month.  Let's hope she's right, we could really use the break.
    So far I've only lost about 10 pounds (due mostly to the stuff they call food that they attempt to feed me in the hospital).  While at home I still eat like a pig and haven't had any problems with nausea.
    Courses 4 & 5 consist of one hour infusions of infofamide and etoposide over five days.  And best of all, they don't require hospitalization.  I was scheduled to start on the 22nd.
    The total time for the infusions was around five hours, 1/2 hour in the waiting room, 1/2 hour of prep, 1/2 hour of hydration and anti-nausea medication, 1 1/2 hours for the etoposide, infofamide with mesna, and 2 more hours of hydration with mesna added in the last 1/2 hour.
    All five infusions have been completed and there has been no nausea so far, I haven't even needed to take the anti-nausea drugs prescribed to take at home between infusions.  The only side effect has been some general swelling, especially in the hands, arms, feet and ankles.  This is minor and is presenting no problems.
    This cycle has been much less trying than the first three.  I guess it was designed that way to give us a rest.  The chemo drugs for cycles 4 & 5 are a little less severe.  No problems with fever at all, as a matter of fact I feel so well that we are going to take a trip back home to Goiania for 10 days or so.   We leave on Tuesday and will be back to start the fifth cycle on the 20th.   It's the middle of Carnival here in Rio and it'll be good to get away from all those girls dancing naked in the streets.

    Course 5, March 22, 2000 - April 10:    We got back from our trip home and have completed the infusions for the fifth cycle.  Again, no problems.  I just felt a little weak for the first few days after starting the infusions.  There's a blood test scheduled on Monday to check the blood counts.

    Second Tumor found, April 10, 2000:     Well, the CT Scan revealed another tumor.  Fortunately it is not attached to any organ and is located in the peritoneum so there should be no problems removing it.  Surgery is scheduled for April 24th and the remaining chemo cycles have been postponed until after surgery.  The tumor is around 5 cm (2 in) in diameter.   The previous CT scan done on February 11th didn't show the tumor, so it looks like it grew to this size in about 5 weeks.  This is not a good sign since it means that the chemo may not be working.  The surgery will show if the pinpoint lesions left after the first surgery have disappeared ... we're hoping.

    Surgery Postponed, April 24, 2000:     After four days of eating nothing but clear broth and Jell-O, three enemas, and one very clean intestine, the surgery was postponed due to low neutrophil counts.  I got as far as the operating table where the surgeon and anesthesiologist reviewed my latest blood counts and decided that the risk of infection was too high to perform the surgery.  I must now wait for the neurtophil count to rise to acceptable levels.  The surgery is tentatively scheduled in 10 to 14 days.   By the way, red Jell-O is by far the best.  I don't know why they even make the other colors, and of course the hospital never serves red.  Hummm.

    Surgery Postponed Again, May 11, 2000:     The surgery was once again cancelled due to low neutrophil counts.  For some reason, my white cell count will not rise to normal levels and are hovering between 1000-2000.  A possible explanation of the low white cell count could be invasion of the bone marrow, but this is not very likely.  The doctors have ordered a bone marrow biopsy just in case.  The surgery has been rescheduled to June 5th. 

    Bone Marrow Biopsy Results, May 30, 2000:     The bone marrow biopsy was negative, so there's no problem with invasion into the bones.  This is a good thing.  The last blood test on Monday showed that the white cells have popped back up to normal levels.  So, the surgery is to proceed as planned next Monday, June 5th.  I'll be checking into the hospital tomorrow for the pre-surgery torture ... same as last time.  I should be back home by the end of the week.

    Surgery June 5, 2000:    

Latest  Update: 6/22/00
   Finally, the surgery was performed on Monday, June 5th.  Unfortunately, the results were not what we had hoped for.  They were able to remove the second tumor that was found with the last CT Scan, but they also found extensive spreading across the peritonium and onto the bowels.  They also found many small tumors on the peritonium and bowels, some of which were removed, but not all.  There was also a third, larger tumor found attaced to the edge of were the epiplum was cut from the first surgery.  This tumor is quite large and extends across the spleen and stomach.  It was not removed.
    Since the growth of these tumors took place during the chemo treatments, it shows that the treatment so far has been ineffective.  Therefore the remaining two chemo courses have been cancelled and an alternative treatment must be found.  We have exhauseted the resources at the National Cancer Institute in Rio and therefore will be returning to the U.S. to search for alternative treatments.  I will have recovered from the surgery enough to travel by next week, so we will be leaving for Belfair Washington on Tuesday the 27th.  
    In the mean time, I've talked with Dr. Kushner at Sloan-Kettering.  He was kind enough to listen to my case over the phone.  Though he requires much more information to give his opinion on the next course of action, a few alternatives were mentioned.  1) receive full abdomenal radiation treatment, followed by another type of chemo.  2) try to get involved with the soft-tissue sarcoma vaccine trial just starting at Sloan-Kettering.  or 3) try to get involved with the anti-angiogensis trial at Dana Farber in Boston.  These last two suggestions just happen to parallel with information that Richard Bergman sent me in an e-mail.  Thanks Richard.  Richard was kind enough to send some contact information to Dana Farber and I 've been trying to get ahold of them for the past couple of days.     desmoplastic small round cell tumor