Sophia's Story
written by mom Diane

Sophia Marie was born 8lbs 1oz the day after I wanted her to be born on. The school cut off is August 31st. She was healthy and beautiful and very feisty. She had turned purple on us one night and was rushed to the hospital at 5 months old and the doctors found nothing wrong and kept her for a few days for observation and it never happened again. She went to Early Intervention when she was 7-9 months old because she would not roll over. She hated being on her belly and refused to roll. She finally did at 9 months and she then learned to crawl and walk at 14 months. At 9 months old Sophia had surgery to remove a Dermoid Cyst from her neck and we were told it was benign. She was also watched for a suspected ganglion cyst on her palm, which I found when she was an infant. All of these things, I was told had nothing to do with her final diagnosis.

She never complained of pain and was having a wonderful summer in 2002. Beginning of August she had a slight bruise over her right eye and a little swelling. It did not bother her and no one recalled a fall so I ignored it and it faded in a few days. The following weekend I went away with some friends to New York City for fun and came home Sunday and she was fine. Monday morning she had the same bruising on the other eye. I tried to believe that maybe she had bumped herself again, but fear got the best of me and I called her pediatrician, Dr. Paul Schrieber, and the office told me to bring her in. I remember calling my best friend on my way to the appointment and telling her that I knew she had cancer, possibly Leukemia. Her doctor looked her over and felt around her belly and sent us for blood work, I dropped the kids off to a sitter and went to work. I called Dr. Schrieber in the afternoon and he told me we had to see Dr. Wolf at the Floating Hospital for Children in Boston that next morning, August 14th, 2002 at 9:15 AM.

We had many tests; blood, CT scans, Ultra Sound, Bone Marrow scan, Bone Scan, MIBG scan (specific for Neuroblastoma) and urine to confirm what the doctors suspected. She was also given blood because she was very anemic. All this occurred within two days. She had a biopsy of her tumor instead of a resection because it had grown around her aorta and other organs in her abdomen and a double lumen Broviac line placed in her chest (IV line). Surgery was on a Friday and by Saturday her eyes were so purple and swollen you would think she had been beaten. Sophia was diagnosed with Stage IV Neuroblastoma; a primary tumor on the adrenal gland 15x8x6cm, metastasis to the bone marrow and the orbital of her eyes. She was immediately placed on morphine to manage the pain.

Treatment started on Sunday, August 25, 2002. Her protocol was for 5 rounds of intense chemotherapy every three weeks, surgery, double Autologous Stem Cell Transplant, radiation and accutane. Sophia had five days of chemotherapy and she was hit hard. She was very ill and her electrolytes were off. She was very angry, to the point she was having terrible fits of anger. I learned later that it was due to the morphine. She was given IV nutrition because of the decreased appetite and monitored for complications. She also contracted an intestinal bacteria called C-DIFF, she was placed on antibiotics and confined to her room. The day we were to be discharged, after 3 weeks, the IV line or Broviac in her chest ruptured and had to be repaired. As a precaution, a culture was done on the line for infection and we were sent home. Less than 20 hours later we were called back to the hospital as the culture showed an infection. We went back to the hospital and started 14 days of IV antibiotics and because she still had C-Diff we were quarantined for just about the entire 14 days. During this stay, she had Round 2 of chemotherapy. She did very well, she wasn't too sick and the morphine had ended so she was no longer agitated, well no more agitated than any normal 2 year old would be confined to a hospital room.

After Round 2 was completed and she recovered, a bone marrow scan was done and it came back free of cancer. Sophia was set to have her third round of chemotherapy and after that the stem cell harvest. We were told to expect the stem cell harvest to be at lest 2 days. She was hooked up TP a pheresis machine and the stem cells were spun off, much in the same way the platelet donations are done. She gave more than enough stem cells her first day, we had a CT scan the next day to measure her progress and we were home in time for Trick or Treating that evening.

Round 4 came and went and she faired very well, I compare it to eating a candy bar, that is how well she did. The CT scan she had just prior showed significant shrinkage of the tumor in her abdomen and the tumors in her orbitals were gone. Ready for Round 5. This was a little scary for me as Round 1 was the same as Round 5 and she was very sick Round 1, but she did very well and went home the following day.

While Sophia recovered from chemotherapy and awaiting the surgery to remove the tumor, which had shrunk to 5x5x5 ball in her belly, we made a trip to New York City to Memorial Sloane-Kettering Cancer institute to look into the possibility of being added to a study using Monoclonal Antibody 3F8, this will hopefully teach her immune system to fight off any residual cancer cells that may linger. On Friday December 20th Dr. Gilchrist was able to remove 100% of Sophia's tumor in less than 2 hours. Thank you God.

Sophia recovered very fast from surgery and was scanned again to be sure that the tumor was gone and had not started to grow back. The scan showed a fluid pocket under the surgical sight and had to be watched for infection. It did not go away so she was opened again, the fluid was removed and cultured. After 72 hours no infection was found and we could get started on her Stem Cell Transplant.

Sophia was admitted on January 8th, 2003 for her Stem Cell Transplant. She received 6 days of "industrial strength" chemotherapy had one day to rest and all the poison flushed out of her system. On January 15th my girl had her Stem Cell Transplant, aside for some reactions to the premedicine given to prevent severe allergic reaction to the preservative used to store the Stem Cells, she did very well. She did what she was supposed to do, her white counts went down to 0 and her blood levels dropped and she required several transfusions of platelets and red blood cells. But her counts came back fast and she had no mouth sores, she had some sores in her intestinal tract and had to be placed on pain medication similar to morphine and she started to get agitated again. She went on IV nutrition because she was not eating or drinking much. She recovered very fast and we were able to go home after only 22 days in isolation.

She had a second Stem Cell Transplant in March, 2003 during which she spent the better part of the month in isolation at Floating Hospital for Children. She went through 15 cycles of radiation and started an oral chemotherapy, high dose Accutane. In June 2003, Sophia started Antibody therapy at Memorial Sloan-Kettering Cancer Center in New York City.

In March, 2004, Sophia relapsed and in September, she had an Allogeneic Bone Marrow Transplant. Her dad was the donor.

In December, 2004, Sophia started her third week of radiation to her tibia and was feeling some relief. She had been fitted for a leg brace to support her ankle and that brought her more relief. Everything seemed to be going very well. On Friday December 16th, Sophia started to complain of belly pain. Her doctors thought it may be acid reflux as she was not eating very much and she was put on Zantac. The Zantac did not help and nausea medicines did not help so her doctor discontinued the oral Cyclosporine, which can cause belly pain, nausea, and loss of appetite. She actually started to asking for food but still complained of belly pain. On Tuesday Sophia helped her sister and grandparents decorate their Christmas Tree and they had a blast. On Wednesday they decorated a Christmas Tree at a friend's house, everything seemed fine.

On Thursday morning Sophia woke up complaining her belly hurt. I carried her to the bathroom and put her down and she cried that her belly hurt and cried more when I picked her up. While she was in radiation I went up to clinic and asked for a CT scan, which was done later that day. Sophia was sent home with the explanation that her liver was enlarged but not to worry. On Friday morning Sophia woke with belly pain again but it was different. She was bloated and very very uncomfortable. I gave her some Oxycodone and it didn't help. We went to radiation as scheduled and they started her on IV morphine for pain. We were admitted Friday afternoon. I remember asking Thursday afternoon why when she had been eating much had she gained a few pounds, it was thought to be fluid. I knew on Friday we were not going to be going back home. The doctors ran some blood test to see if she had a virus that effected the liver; hepatitis or mumps. Over the weekend she got progressively worse, she needed IV morphine every 2 hours and it was not enough and her oxygen levels in her blood were dropping so that she required blow-by oxygen. Sunday evening she was sent to the ICU and placed on continuous morphine drip with a button to administer additional morphine as needed.

I said my goodbyes to her all weekend. While she slept I prayed for her not to wake up. I could not bear to watch her decline and suffer. By early Tuesday morning she was placed on a vent as she was not able to breath sufficiently on her own. I did not want to vent her because I did not want to prolong the inevitable especially so close to Christmas. Tuesday afternoon a needle biopsy was done to see if she had a fungus in her liver or cancer. I knew what the result would be, her abdomen was so swollen and her organs were starting to shut down. We had the confirmation that it was in fact an aggressive metastasis to the liver and that she was going to leave us soon.

We decided to have Olivia, Sophia's big sister, brought in to say goodbye. She was a little nervous at first but I had someone from Childlife sit down with her to explain how her sister would look before she was brought in. After a few minutes she warmed up and wanted to hug and kiss her sister. We then sat Olivia down to tell her that her sister was going to be with God soon. She got very quiet but she seemed OK but did not quite understand. I brought her back to the Childlife office and at her request I left her there. After about 45 minutes Olivia came running down the hall with her friend bringing paper and paints, yelling "We're going to paint Sophia's feet". And we did, bright blue, and made imprints for a special Memory Box the Childlife Specialist gave her. I also cut some of Sophia's hair for a keep sake for Olivia. Olivia laughed and giggled and bounced on the bed and while Olivia painted Sophia's toes she wiggled them. We all laughed and said "Look Olivia, you tickled Sophia". It was awesome. We cleaned Sophia up and Olivia said goodbye. She was reluctant to leave with mom or dad so we took a walk. Olivia told me she did not want Sophia to go be with God. I told her it would be best and Sophia would not have anymore pain or yucky medicine or cancer etc... Olivia seemed to understand and asked if we were going to put Sophia in a box, how smart. I told her we might and asked if she would like to pick the box, this made her happy. I told her we could go out and buy a beautiful dress but Olivia said she wanted Sophia to wear her pretty Christmas dress (Thank you Juanita). I told her she could pick the flowers and a bow for Sophia's hair. She did a great job. She was always helping her sister, even off to her final journey she helped Sophia get ready to be with God. Olivia is such a big girl, so full of love.

After Olivia went home with Grammy and Papa we and some family and friends gathered to say good bye to our little baby. On Thursday December 23rd, 2004, the vent was turned off and she was placed in her daddy's arms and there she took her last breath. My baby was gone. I thanked her for not waiting until Christmas to take her last breath. A good girl until the end. Because of the Holiday we had to wait until Tuesday for the wake and a beautiful funeral service on Wednesday. My baby is gone. I am so proud to have been chosen to be Sophia's mommy. I will cherish every minute I had with her. I know she is in a better place, free of that disgusting cancer. I will miss her tremendously.

Thank you all for keeping us in your prayers. She and Olivia loved all the packages and cards they received. You are all amazing people, you bring happiness to the sick, one card at a time. Thank you.