Adrienne's History

I started this blog when Adrienne relapsed in early 2005. The goal was to keep friends and family updated on her progress. I never thought it would continue for this long or that so many people would be reading. Since she was diagnosed so long ago, I thought a history might be useful.

1995-1996: Itching began. Adrienne saw her pediatrician, a dermatologist, and an allergist, all to no avail. We thought she had hives but it turns out that the itching caused the welts and sometimes bleeding where she scratched.

November-December, 1996: A lump appeared at the base of Adrienne's neck. Her blood counts showed anemia while a chest xray was normal. By this time, she had constant fevers so it took some time to get a biopsy. The biopsy finally confirmed that she had Hodgkin's Lymphoma, nodular sclerosing type, and we were referred to Lucille Packard Children's Hospital at Stanford, to one of the leading pediatric Hodgkin's specialists in the country. Staging at that time was fairly rudimentary requiring a lymphangiogram, CT scan, and gallium scan (which showed a false negative). Adrienne was diagnosed as Stage IIIB with lymph node involvement through her neck, chest, abdomen, and pelvis, including her spleen. Her largest node in the mediastinum was 5.5 cm and the largest node in her abdomen was 8 cm. In a child of that size, she was considered bulky because more than 1/3 of her chest had tumor.

January, 1997-October, 1997: Adrienne received a study protocol known as VAMP/COP (vinblastine, adriamycin, methotrexate, prednisone/cytoxan, vincristine, procarbazine). The goal was to reduce the toxicity of the standard protocols at that time while providing a comparable cure rate. She received two cycles of chemotherapy followed by mediastinal (chest) radiation, two more cycles of chemotherapy followed by abdominal radiation, and two final cycles of chemotherapy followed by pelvic radiation. She had surgery to move her ovaries out of the field of radiation prior to her pelvic treatments, but they still received scattered radiation. During these months, she had multiple infections and hospitalizations, but no major complications. In those days, there was no neupogen or neulasta or procrit so you just had to wait for counts to come up on their own. They later published the results from the study Adrienne was part of saying it was discontinued it due to a high number of late relapses, of which Adrienne was one.

August, 2001-July, 2002: First relapse with the largest node at 4.5 cm. In the months prior to this, she had pneumonia and fevers but no apparent lymph node involvement until a routine 6-month checkup with her oncologist. Once again, she was diagnosed as Stage III with disease in the same locations as the first time. She received two cycles of ICE (ifosamide, carboplatin, etoposide) and achieved a remission, but this was a very difficult protocol for Adrienne, leaving her weak and requiring multiple blood and platelet transfusions. This was followed by an autologous stem cell transplant with BCNU (carmustine), etoposide, and cytoxan and then consolidation radiation to the upper and lower halves of her body. Her only major complication was radiation pneumonitis, which has left some residual fibrosis in her lungs, as well as ovarian failure and sterility. Adrienne's counts took a long time to recover so she was in isolation for 6 months following the transplant rather than the usual 100 days.

September, 2002-February, 2004: Second relapse with the largest node at 2 cm. Adrienne was treated with one cycle of gemcitabine and vinorelbine and achieved a remission. Then she received a modified Stanford V (mustargen, adriamycin, vinblastine, vincristine, etoposide, and prednisone) without the bleomycin to protect her lungs. After one cycle, the adriamycin was removed because she showed some heart abnormalities. In January, 2003, we went to the Seattle Cancer Care Alliance/Fred Hutchinson for a new reduced-intensity conditioning allogenic transplant with Daniel (Adrienne's brother) as her donor. The transplant conditioning consisted of fludarabine, melphalan, and one dose of total body irradiation. This transplant was very, very difficult and there were many complications, the most significant of which was graft-vs-host disease of the mouth and gut. Adrienne was treated with immunosuppressants and high doses of prednisone for a year following the transplant. At her one year follow-up in Seattle, we found that she had cardiomyopathy as a result of her original treatment in 1997.

January, 2005-October, 2005: Third relapse with nodes in the neck (referred to as cervical nodes). Adrienne started with six cycles of gemcitabine and vinorelbine, which was initially effective, but she had a small amount of progression during the last cycle. Next she had three donor lymphocyte infusions from her brother, but her disease didn't respond. In the meantime, Adrienne was named a National Merit Scholar, received a small merit scholarship to Claremont McKenna College, and started her freshman year in Southern California, where she was seen at City of Hope, an NCI-designated cancer center. They had a new type of radiation, called tomotherapy, and they were able to treat the nodes while avoiding the spine and salivary glands. The radiation was very difficult but there were no long term side effects except that she developed hypothyroidism.

December, 2005: Adrienne developed avascular necrosis (AVN) in both hips as a result of high doses of prednisone required to treat her pneumonitis (first transplant) and graft-vs-host disease (second transplant). She had bilateral core decompression surgery over her break from college and did quite well. She had a clean PET/CT scan just prior to her surgery.

March, 2005-August, 2006: Fourth relapse. Adrienne began having pain in her right hip in March. We assumed that the prior surgery wasn't effective and that she would need a hip replacement. A routine PET scan in April showed activity in the hip. After a follow-up MRI and a very painful biopsy of the bone, this was confirmed as Hodgkin's. This is a very rare situation that none of Adrienne's doctors had ever seen but it was better than some of the other possibilities, such as leukemia or sarcoma. This was treated with radiation once again. In August, Adrienne had a follow-up PET/CT, which showed resolution of the hip lesion but a new 6mm node in the right lung.

September, 2006-October, 2006: We tried to get Adrienne enrolled in a clinical trial at City of Hope with a new drug called SAHA. To qualify for the trial, she had a CT scan, which showed no abnormal lymph nodes, that is, no lymph nodes over 1 cm. Since they couldn't track her disease with CTs, she didn't qualify for the trial so we had to watch and wait.

December, 2006-present: Fifth relapse. Adrienne had a routine CT scan that showed three areas of involvement including the same spot in the right lung, diffuse disease in the spleen, and an epigastric node near the liver. This relapse was confirmed by a PET scan, with the largest node being 1.3 cm. City of Hope suggested that she get tomotherapy radiation again, but given the lung fibrosis from her first transplant and the widespread nature of this relapse, we didn't feel comfortable treating with radiation. We decided to consult another doctor at Memorial Sloan Kettering in New York, who works closely with City of Hope. The plan they agreed on was to try a drug called valproic acid, similar to SAHA but readily available as an anti-seizure drug, and to watch Adrienne very closely. Adrienne took the drug for 5 months while her disease progressed very slowly. In May, 2007, she went to New York to be treated and to work at an internship. During the scans there, we found that she had a 50% progression in a period of about six weeks with the largest node now being 3.8 cm in the right lung and 3.7 cm in the spleen. Adrienne recently started a new clinical trial that we hope will keep her disease under control.