End of Summer Clean up

The follow ups this year are a shadow in Bella's arm and a lump that appeared on Phillip's ribcage. Hoping for nothing, but we don't know until we know. I think of the swollen lymphnodes a few months back- were they trying to fight this lump off? who knows.

Phillip was of course referred out to a dermatology consult. Which was fine with me, I've been to Tripler derm a few times and the scars to prove it. Lily was referred out years ago and I was impressed.

With LFS there is a lot of self advocating. I'm trying to give the kids the tools they need to navigate the crazy healthcare world with a rare condition.  I tell them to pay attention to biology, anatomy, in school and in person. Know their bodies. If anything weird pops up and lingers( we have a 2 week rule) we get it checked out.

Phillip is on the cusp of adulthood-he's no stranger to medical stuff as he's been independently managing his diabetes for awhile. I appreciate that he tells me about concerns. It's a hard concept to understand- taking care of things early could mean the difference of life and death for us. I prepare him for my gut reaction- to remove it. He has some time to think on it. I will let it be his call.

The nurse calls us into the room, she recognizes Phillip. I ask if she shops at Safeway, we discuss their Friday deals and great flowers. I share with her a bit of our family history and Li Fraumeni syndrome and why we are here for this little lump.  She looks at it, visibly taken back by our history.

When she leaves I explain to Phillip why I go over LFS and our history. We are lucky to have a team at Tripler that knows us and our history and automatically takes into account our mutated p53. little things that don't concern normal people- could be bad for us. They could be nothing too. I tell him most of our time is advocating for ourselves. Most doctors have never heard of LFS and it's on us to make sure they understand. I show him the living LFS pamphlet I brought as well as the latest journal article on LFS and screening that I printed out. I'll leave both with the doctor.

As we sit- Phillip paces- the view overlooks Aiea and we can see the Arizona Memorial. He asks a few questions, I answer. The doctor finally comes in. Phillip takes off his shirt, the doc draws around the lump. Its about a 1 cm oval, palpable, not fixed. He's not concerned and says considering history, lets remove it. I agree.

He asks me several questions about Li Fralini - it takes about 5 tries before we got the name down_ I tell him we call it LFS for short. He asks me if I'm a nurse. Nope. not officially. He asks me specific questions with medical terms- he's feeling me out and my knowledge- I take the bait and answer equally as medically. He is not familiar with sarcomas, I ask him to consider wider margins than he was planning so we don't have to go back- I hold up my shark bite. I explain about Lily's sarcoma and 15 year old LFS friend was just diagnosed with an extraosseous osteosarcoma- our lumps tend toward weird.  I pray it's nothing- I've had several of those too, we just have to be careful. And oh would he mind doing a once over look at his other skin to make sure everything is ok.

He smiles, almost condescending, but he has kind eyes so I let it go. He says he realizes I've been traumatized but not everything is cancer. I would rather have a non cancer removed then for it to become something that cannot be treated. I explain again that both my brother and father died of brain tumors, phillip's sister had adrenal cancer in addition to sarcoma( which was just a nothing...) He interrupts- medullary tumor? No- It was adrenocortical carcinoma. That got his attention. In addition to her plexiform fibrohistiocytic tumor, mine was also fibrohistiocytic but I think they are calling them pleomorphic something or other's now- I can't keep track. That was the same time as my breast cancer which metastasized to my lungs, bones and brain- so yes there has been LOTS of trauma and lots of cancer flags.

He pulls up Phillip's shirt and makes a wider margin around the existing one. He turns to me- you should really consider starting a patient advocacy group. I pick up the living LFS pamphlet and say - I have. This is our group.

Living LFS? What does that stand for?

Oh holy fuck- again with the Li Fraumeni Syndrome pronunciation guide. I tell him a bit about some of the cancers and rarities our families face, but it is the most phenomenal group of people. He asks to keep the literature- of course- please.

We schedule the surgery- it'll be minor, but we have to work around his college schedule now. And so now we wait.