My beautiful niece Gracie was recently diagnosed with Neuroblastoma (cancer). It is still early in the diagnosis and we are awaiting results to know more about the fight that Gracie will face. I've created this blog as an outlet for friends and family to stay informed about her journey and a way for friends and family to make anonymous contributions towards her care and the mounting expenses her family will face. Gracie is a fighter and the bravest 3 year old I know. Hundreds of people love you Gracie and are praying for you. "Be strong and of good courage for the Lord thy God is with thee" Joshua 1:9

Wednesday, January 12, 2011


Gracie's EGD showed that her stricture was ok but since he was in there anyway he dilated it a bit more. she still has esophogitis but it is much better than last time. we waited around to get her accutane and GMCSF shots before heading home. the shots were delivered to our room around 2:00 but the accutane wasnt delivered til 5. they were having trouble with the insurance but finally got it figured out. I was a bit frustrated but glad to have the meds before heading home. we made it home around 10:30 that night, it was a long day and we were glad to be home! :) Accutane is used for nodule acne. obviously Gracie doesnt have acne but it is also used to stop the growth of NB cells. weird huh? so anyway, we had to sign a bunch of papers saying she would use two types of birth control, and not give blood, and not share the medicine with anyne! LOL totally all irrelevant but that is the paperwork for the regular use of accutane. you'd think they might could do a seperate form for NB. oh well. The accutane comes in a capsule and she cant swallow it so we poke it with a needle and squueze it out into a bite of pudding and she doesnt have to taste it. She just started it yesterday and will take it twice a day for two weeks every course. a lot of people ahve asked me about the accutane so I copied the information below off the website

Treating Minimal Residual Disease
One of the things that makes NB so difficult to cure is the fact that even when it cannot be found through scans, lab tests, or bone marrow biopsies, the disease can still be present in very small amounts in the body. Doctors believe that this undetectable disease can sometimes smolder and grow, eventually coming back as relapsed NB, which is much harder to cure. Accutane and antibodies are two strategies that have been developed to help eradicate undetectable disease, but the two work in very different ways. Much research on vaccines against NB has been accomplished, but use after frontline therapy is a new development.
Accutane, or 13-cis retinoic acid, is a synthetic vitamin A derivative that has been shown to stop the growth of NB cells. Accutane can cause some NB cells to mature (differentiate) into non-cancerous cells. A five-year (1991-1996) randomized study (CCG-3891) concluded that high doses of Accutane improved the event-free survival for children in remission. Since those findings were published in 1999, the use of Accutane has become widely accepted for high-risk NB.

Accutane is given by mouth in capsule form in two-week on/off cycles—the medicine is taken twice a day for two weeks, then children take no medication for two weeks. Typically, children receive Accutane over six months. In the German NB2004 protocol it is given for six months with a three-month break, and then three more months.

Accutane has many side effects, but most are mild, including dry skin, moodiness and sun sensitivity. Because some of Accutane’s side effects can be more serious, children are monitored during their Accutane treatment with regular check-ups and blood draws (particularly for calcium levels and triglycerides).

It is extremely important that pregnant women, and those who may become pregnant, follow strict safety precautions when handling Accutane, because ingestion of the drug (which can be absorbed through the skin) poses a risk of serious birth defects and deformities.
Monoclonal Antibodies
Our bodies manufacture antibodies that create an immune response to bacteria, viruses, and other foreign substances to help keep us healthy. Ordinarily, a child’s immune system will not attack NB because the cancer is a part of the child’s body. Monoclonal antibody therapy (monoclonal refers to development from one clone) uses mouse antibodies produced in the laboratory from plasma (myeloma) cells. The antibodies used in NB treatment attach to a ganglioside (a fat-sugar complex molecule) on the NB cell called GD2. Because the antibody alerts the child’s own immune system to attack the NB cell the antibody is attached to, the cancer cell is destroyed. Long-term immune response may be initiated by antibody treatments in some cases.

COG currently offers a phase III study (COG-ANBL0032) of the monoclonal antibody ch14.18 (administered with “cytokines” to augment the immune response to the antibody) to patients following completion of frontline treatment protocols that include stem cell transplant. After an early review of 226 children enrolled determined significantly higher survival with antibodies, the study was amended in April 2009 to stop randomization and allow all patients subsequently enrolled to receive the antibody. The study will continue until final accrual goal of 423 is met, and it is anticipated that ch14.18 will be part of standard treatment for all COG protocols. The ch prefix indicates the antibody is “chimeric” or part human (75%) and part mouse (25%) in its formulation. In the current European SIOP trial the use of the same antibody ch14.18 is randomized (but without the use of cytokines). Note that the similar protocol number of COG-ANBL0322 using a different but similarly named antibody, hu14.18-IL2, is a closed phase II study which was open only to children with relapsed or refractory NB. The hu prefix means the antibody is completely humanized.

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