One of the things that I regret the most is that I didn't do more journaling when Jenna was going through all of her treatment for neuroblastoma and opsoclonus-myoclonus syndrome (OMS). I am pretty good at remembering the big details, but it would be nice to be able to go back and read the little details.
Jenna is eleven now and having more medical issues. She has been complaining of GI trouble off and on for years. We have taken her to see different doctors over the years, but none really ever helped her or really spent much time trying to figure out what the issue was. In February of this year, her belly was quite distended and painful, so I took her to see our family doctor and was referred to a pediatric GI. She saw him and he scheduled an EGD and a colonoscopy for a week and a half later. (P.S. We don't see this guy anymore.) Her pain and distention got worse before that date. After vomiting ten times in a day, and realizing that she had not had a bowel movement in a week, we ended up at the emergency room at Ogden Regional. They took an abdominal x-ray and the size of her colon was shocking! No wonder she felt so painful! They sent us down to Primary Children's in Salt Lake. Over the course of the next few days, she had two surgical disimpactions done and cleaned her out.
Her new GI doctor, Dr. Guthery, put her on a miralax regimen to keep her bowels regular and hopefully shrink her colon. He also tested her for paraneoplastic antibodies and voltage gated calcium channel (VGCC) antibodies, both of which were positive. This is where her situation gets interesting, and also confounding. Paraneoplastic means related to cancer. We know that she has had cancer, but what we don't know is if she has had these antibodies since then. No one is routinely tested for those. The VGCC antibodies are most often associated with Lambert-Eaton Syndrome, but she doesn't seem like a classic case of that. It is often associated with small cell lung cancer. What we also don't know is where in the disease process these antibodies show up. Do they show up before the tumor can be detected?
Jenna had good days and bad days over the next two months. Dr. Guthery ordered an upper GI with a small bowel follow-through on April 29th to see if her small bowel also had motility issues. That required that she drink barium contrast and it plugged her up and made her hurt. On May 1st, she still hadn't passed the barium and was in so much pain that she was admitted back to PCMC. They gave her some IV hydration and toradol for pain. Because she already has gut motility issues, she is not able to have any narcotics for pain or zofran for nausea because they cause constipation. The toradol worked great and she slept for the first time in days.
During that admission, we had a parade of doctors seeing her. Dr. Guthery came in and said that the x-ray that she had there showed lots of barium that she still hadn't passed. He was thinking that her condition might be autoimmune, but there is no particular syndrome or disease that he could point to. He was referring us to the oncology department to explore the possibility of a neuroblastoma recurrence or other caner, and to a rheumatologist to explore the possibility of it being autoimmune.
Dr. Bohnsack is her new rheumatologist. We talked a lot about treatment options and what would be the best way to start. He suggested pulse steroid treatments to suppress her immune system. This means that she will do three days of high-dose IV steroids and then four days off, repeated for a few weeks. The idea is that if she has a positive response, meaning that her gut motility improves, it will indicate that it is in fact an autoimmune problem. She is set to start those tomorrow.
Dr. Fair is the oncologist fellow that we talked to. He said that given the type and stage of neuroblastoma that she had initially, the odds of having a relapse are very slim. He did think, however, that given her other issues it was reasonable to do some scans that will rule it out. Most neuroblastomas will light up on a MIBG scan. She is having one of those tomorrow. We went to PCMC today and she had the contrast injected. She will also be having a neck/chest/abdomen/pelvis CT scan to rule out any other types of cancer, hopefully done in the next couple of weeks. She was supposed to have that one done last week, but she still hadn't passed the barium. Daily enemas over three days got it out enough that she can have her scans this week and start the steroids.
That puts us mostly up to date. Jenna is in good spirits for the most part. She is used to hospitals and doctors, so that is helpful. She has missed a lot of school. We are thankful that we are at the end of the school year. Please say a prayer for us that the MIBG will come back negative for neuroblastoma and that the steroids will work.
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