Alison (Adrienne's Mother) started this blog when Adrienne relapsed in early 2005. The goal was to keep friends and family updated on her progress. Since she was diagnosed so long ago, Alison thought a history might also 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 X-ray 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. These drugs caused renal insufficiency so she had daily IV hydration for that entire first year. At her one year follow-up in Seattle, we found that she had cardiomyopathy as a result of her original treatment in 1997 but she was free of disease.

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 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, just a few months after her surgery and clear PET/CT. We assumed that the prior surgery wasn't effective and that she would need a hip replacement. A routine PET scan in April showed disease activity in the hip, but given the unusual location, doctors didn't think it was Hodgkin's. After a follow-up MRI and a very painful biopsy of the bone, this was confirmed as Hodgkin's. None of Adrienne's doctors had ever seen a presentation like her's but it was better than some of the other possibilities, such as leukemia or sarcoma. She was treated with conventional radiation since the area had never received treatment. In August, Adrienne had a follow-up PET/CT, which showed resolution of the hip lesion but a new 6 mm 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-May 2007: 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 Dr. Owen O'Connor at Memorial Sloan Kettering (now at Columbia) who works closely with City of Hope. The plan the doctors agreed upon 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.

May, 2007-December, 2007: She went to New York to be treated and to work at an internship. With her initial scans, 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 started a clinical trial in June, 2007 but scans in August once again showed progression. Adrienne then went on another clinical trial with a drug called ABT-263 but she got pneumonia and several infections so treatment was discontinued.

December, 2007-February, 2008: Adrienne returned to California and completed two cycles of conventional chemo with gemcitabine and oxilaplatin. Her PET scan after two cycles showed some reduced disease and two areas of new involvement. The side effects of this chemo were severe so she discontinued treatment.

May, 2008-November, 2008: Adrienne enrolled in clinical trial using MGCD (an HDAC inhibitor) and Vidaza (an FDA approved chemo). Initially, she had a great response with a 50% reduction of her disease with minimal side effects. In the fall, she had major side effects, not the least of which was grade 4 hemorrhagic cystitis, so she had to drop out of the trial in early November. By mid-December, her disease was out of control with many new areas of involvement and large bulky disease, leaving her in extreme pain. It seems that the MGCD had a rebound effect and her cancer grew faster than ever.

December, 2008 to February 2009: Adrienne went back to New York to see Dr. O'Connor and he put her on a new drug called Bendamustine. It's actually an old drug developed in East Germany over 40 years ago but it was only FDA approved in 2008 for non-Hodgkin's lymphoma and CLL. Adrienne got the drug off label and was feeling better within days. Her scans after 2 cycles showed almost complete resolution of her disease. She continued on the drug for 4-6 cycles, hopeful for a positive response in an attempt to get her disease under control.

April 2009: Adrienne was found to have multiple new patchy densities in her right lung, in the right infrahilar, and in mid and lower and peripheral lung field. The largest lesion was 3.3 cm in size, not huge, but still surprising. She also has pneumonia in the lower right lung.

June 2009: All of Adrienne's doctors have agreed that SGN-35 was the best option available to her. Unfortunately, she didn't qualify for the trial because of her allo transplant and the company refused exceptions. Her doctor at City of Hope made a plea for single patient access or compassionate use, even offering to cover some of the costs, and they again refused. Late June: A CT shows there are so many new nodules in both lungs that I didn't quite look like Hodgkin's. The consensus was that it is probably a fungal infection along with disease progression, but perhaps not quite as much disease progression as first thought. The only way to be sure is to do an open lung biopsy and everyone agrees that it would be too risky. Adrienne is put on an anti fungal medication and watched closely.

July 2009: Adrienne saw the oncologist at NVCI to get counts, assess her pain, and determine how to move ahead. Her CBC showed low hemoglobin, which is typical when she has a lot of disease, making her pale and tired. Her lymphocyte count was almost nonexistent so she needed to be extra careful as she would be susceptible to opportunistic infections. Her CT showed a small problem around the bile duct that connects the liver and gall bladder. An elevated alk phos confirms that it's something that needed to watched. Adrienne received vinblastine (chemo) during the middle of the month. She was in some pain for the remainder of the month and was hospitalized while they got it under control.

August 2009: Adrienne was in a great deal of pain and a decision was made to visit her primary oncologist Dr. O'Connor in New York to discuss new treatments (biologics). On arriving at Dr. O'Connor's office Adrienne is found to have a large pleural effusion that takes up 1/3 of the space of her left lung as well as new disease throughout her spine. Adrienne had been short of breath, which was probably caused by the effusion. After treatment for the pain Adrienne (and Alison) return to Nevada.

August 23rd, 2009: 911 is called because Adrienne is having problems breathing. She is put on oxygen and admitted to a local Las Vegas hospital. About 1.3 liters (1.5 times the normal amount) is drained from the larger effusion. The next day the other lung is drained.

September, 2009: Throughout the month of September Adrienne fought for her life. Her health was up and down, but it was clear that her respiratory system was collapsing and there was little anybody could do about it. The doctors admitted her to the ICU where she was placed on oxygen (first a BIPAP, then on a CPAP, and finally she was fully intubated). By September 30th Candida (yeast) was pretty much everywhere: in here lungs, urine, and blood. Everything possible was done to save her life, but in the end the ventilator that was helping her to breath couldn't deliver oxygen fast enough. She could not breathe on her own and the hard decision to remove life support was made.

October 1st, 2009: The saddest day in our lives. Adrienne passed away at 10:55am on Thursday, October 1st 2010.