An Uplifting Story and Some Upsetting News

 

An example of Lisch nodules

This past week Jane finished up the last of her restaging appointments: ophthalmology and a virtual meeting with our team at NIH.

Jane’s ophthalmologist, Dr Howard, works at a teaching hospital, which means she is usually accompanied by several trainees.  Before Dr Howard joined us in the exam room, we were first greeted by an ophthalmology resident and a visiting fellow from the pediatric emergency department who was observing.  The resident began the exam, and at one point he explained to the fellow about Lisch nodules, tiny growths that can be found on the irises of many people with NF and that can only be seen with a special camera, which he was using.  As he talked, I could see Jane getting uncomfortable because no one had ever made a big deal about her Lisch nodules before. I tried to reassure her that they were harmless, but the tension was broken when the fellow looked through the camera and she gasped, “Oh! They’re beautiful!”

Never, with all of the hundreds of evaluations she’s had in her short life, has anyone ever described any component of Jane’s NF as “beautiful”. Her comment moved me to tears. As Jane’s mother, of course, I am biased and think every part of her is beautiful, but usually our interactions with health care providers focus on the complications of her NF, not the rare beautiful things.

I decided to write a letter to the fellow and to the head of her department to praise her.  I told the story above and commented that I had no doubt the fellow was competent in her knowledge of pediatric emergency medicine, but that what stood out to me was her kind, compassionate heart.  I got this lovely letter from the department head in return:

That afternoon we had a virtual meeting with Jane’s pediatric oncology team from NIH to review all of the recent testing she has had. The good news is that her facial plexiform neurofibroma appears stable when compared to her last MRI in May.  The bad news is that her abdominal tumor, which had previously been stable, has increased in size by 30%. The rapid growth makes the team a little concerned for a possible developing malignancy.  (Neurofibromas have about a 10% chance of becoming cancerous in a person's lifetime.)  The team wants us to do a PET scan to help decide if the tumor needs to be removed immediately or if surgery can wait until the pandemic lessens and the school year is over. The PET scan is now scheduled for just before the holidays and we will meet virtually with the NIH neurosurgeon and urologist (the tumor is next to her kidney) right after the holidays.

How do you tell a child they have to have a test to check if their tumor is cancerous?

Jane is always present for our visits with our team and was listening in on this one.  I asked her later what she understood from the conversation.  She said, “So I might have to get the tumor out sooner rather than later?”  I said yes, but that there was one more test we could do to see how much sooner—whether we had to have it done immediately or whether we could still wait for a school break.  I said the test was a PET scan.  She said she’d heard of a PET scan before but didn’t know what it was.  I told her that the PET machine looked kind of like an MRI, but that it can tell which cells in the body are most active or growing fast and which cells aren’t.  The kidneys, I told her, for example, have lots of cells that are very active, so the kidneys light up on a PET scan.  Jane said, “So if the tumor lights up that means it’s growing quickly and we’ll have to have it out sooner?”  Exactly.  I told her she’ll have to have an IV for it for a type of contrast—she was OK with that.  Then I braced myself and said, “There’s one more thing.  The contrast is radioactive.” 

She broke into a wide grin!  “So you mean I’ll be radioactive??”  I smiled with relief.  “Yes, but only a teeny, tiny, bit for a short period of time.  Not enough to be harmful.”  She said, “Kind of like bananas!  Did you know that enough natural potassium is radioactive that if you ate 10 million bananas at once you’d get radiation poisoning?  Of course, you’d die of other reasons—your stomach would probably explode.”

And that’s how you tell a child they have to have a PET scan <3  Jane and I later found this graphic about radioactive bananas :)

All kidding aside, in my opinion, children should not need to have PET scans :(