Hi! I’m Tony, Niko’s dad. I asked Natasha if I could guest-author a post about the treatment Niko recently finished at UCSF, both because I think it is scientifically interesting and because I’ve had a few ask for more details.
I’ll start by saying that I learned a lot during the week, both about cancer and about radiation. I’ve also now had a lot of experience keeping a spirited 3-year-old in complete isolation for a week, which is incredibly difficult and hopefully none of you every have to experience it. First a little bit of background. I remember first learning about the substance called MIBG when Niko first got diagnosed. In a nutshell, it is the radioisotope of regular iodine (I-123), and localizes to neuroblastoma cells. It is therefore the primary tool used to study and monitor a Neuroblastoma patient’s disease.
Example of an MIBG Scan
So far Niko has undergone 4 MIBG scans – the first at time of diagnosis (baseline, disease assessment) and the others all for the purpose of checking how his treatment is progressing. As you might imagine we’re always on pins and needles while we await the results of one of these, since they tell us quite definitively how the treatment is going. A day before he’s scheduled for a scan, he’ll go down to ‘NucMed’ (nuclear medicine) where they have a carefully dosed injection of I-123 ready in a lead-lined case. They inject the substance and give it 24 hours to make its way to all of the neuroblastoma cells in Niko’s body. The following day they put him into a specially designed camera that picks up on the isotope, producing an image similar to what you see on the left. In this example the patient has MIBG uptake in the left adrenal gland, signaling that is where the cancer resides (typical for Neuroblastoma). You’ll also notice that the thyroid (near the neck) is “hot” – this is because your thyroid naturally absorbs iodine. The bladder gets any of the waste I-123 that doesn’t get absorbed. The end result is a fairly good visual study of where the cancer has spread.
Niko’s MIBG IV dose encased in lead
When Niko was diagnosed and a bucket full of cold information was dumped on our heads, they called the extent of his disease and scan “disgusting”. Since his disease was very progressed (stage IV, see staging) they also mentioned a new clinical trial studying the effectiveness of treatment for high-risk Neuroblastoma children using this same MIBG concept. It is based on the premise that by using a similar compound, but with a stronger isotope, I-131, as your transport mechanism, you can radiate the cancer cells wherever they are in his body with a targeted approach. It is commonly referred to as MIBG Therapy. Procedurally it works like the scan, except that the MIBG injection contains a far higher dose of radiation (252mCi vs ~1mCi scan dose). This is because the goal isn’t to just pick up the radiation with a highly sensitive camera, it is to eradicate the cancer with heavy radiation. You can see a photo of Niko’s dose being prepared to the right by a radiopharmacist.
The amount of radiation used in this therapy is through the roof. Seconds after Niko was infused with his dose, his little body was emitting 0.37mSv/h of radiation at 1 meter away. To put it into perspective, this means that he was emitting the same level of radiation that a person’s recommended limit of exposure is for an entire year in just 3 hours. Get closer than 1 meter and the levels skyrocket. For this reason, once he had received his injection Niko was deemed “hot”, and a bunch of stringent precautions were put in place. It is actually spectacular to imagine the thousands of radioactive particles blasting out from his body like beams of invisible light, passing through everything in its path, and of course hopefully the cancer cells.
The room itself is specially designed for this treatment and has lead-lined walls and floors. He is kept behind a 3″ thick lead shield for the entire treatment and everything in the room gets covered in plastic to prevent radiation contamination. Since we’re dealing with Gamma radiation, you can’t inhale it, but it can easily pass or “contaminate” through liquids. We got extensive training on avoiding contamination and keeping our exposure limited. Before starting the treatment they had placed a catheter and setup a pump to push his urine out of his bladder and the room continuously. This is because a high concentration of the radioactive I-131 is expelled in the urine and since his bladder is healthy, you want to avoid radiating it. Entering the room is an entire process. You have to put on a protective layer including “booties”, an apron and a double layer of gloves (see my getup in the gallery below). This is to prevent getting any radioactive contaminants on your skin or clothes. You’re also required to put on a dosimeter to continuously measure your exposure, which gets documented in a log and closely monitored by the radiation team. Anything you bring into the room has to stay there, again for risk of contamination. As his caretaker, you’re responsible for keeping him comfortable, feeding him, keeping him occupied and giving him medicines. The nursing staff is only around for IV maintenance, remote observation and any emergencies that arise. Grandma, who also stayed at the hospital, was also there to help. I would say the biggest challenge was keeping him comfortable and calm, especially when the side effects began to kick in. As part of the clinical trial (NANT-2011-01), Niko got randomized to arm B; one of three arms which also includes chemotherapy while he receives the MIBG therapy. The theory is that the chemo amplifies the effects of the treatment, but of course adds to the already huge list of side effects.
Though it was an incredibly hard week, the staff did what they could to make things comfortable for the kids and the parents. They put him on a sedative designed to keep him calm, and to eliminate anxiety. They had a mirror in the room so he could see and communicate with us from the hall, hopefully reducing the amount of time you spend actually in the room while making it feel like you’re nearby. He also had a huge TV which as you might imagine was crucial in keeping him distracted. Chromecast was an absolute essential here, because it allowed for me to remotely start whatever media he wanted, including home videos and photos.
Cisco Telepresence 2-way video
There was also a two-way video system, with the other camera setup in the Family room as you can see in the photo on the left. We kept his video rolling the entire time so we could observe him, and hear him waking up at night, which was really nice. You of course have the option to put your camera feed on his TV, but this tended to upset more than pacify him. He had two nurses on his team at all times, one for general care-taking and the other from the PICU (pediatric intensive care unit) who’s sole responsibility was to monitor him while he was on the sedation. They do this from a “control center” by continuously monitoring 4 special video cameras installed in his room and a remote feed with all of his vitals. This nurse also helped a lot with keeping him comfortable, responding to any of his needs and backing up his care nurse. All of the nurses he had were wonderful, and experienced in the special treatment.
I think the hardest part was not being able to hold and hug him. There was times were he literally begged me to “give him a hug”, and I couldn’t do it. It broke my heart. But he is a smart child, and he understands that it isn’t because we don’t love him, but that he is sick and has medicine making him better that is dangerous for others. Mom did an amazing job of packing goodies for him, wrapping a bunch of toys for him to open and keep him busy. At the end of the treatment, when his radiation levels are below legal limits, anything he had in the room was scanned, and items testing “hot” were left for them to hold in quarantine. For this reason they recommended not bringing anything you care about losing for 3 months.
It’s done, he was incredibly strong, and with any luck the treatment blasted the life out of his cancer cells. I expect that this will make it to mainline treatment for any child with high-risk Neuroblastoma very soon, so hopefully Niko’s contributions to the trial and my account of the experience will help other children and parents in the future.