Choking

Lily started Middle School. She's been so excited to be at her own school. She doesn't even mind that some of her teachers remember her siblings. A tough teacher that Phillip and Kiera both struggled with, will probably end up being Lily's favorite teacher ever. They all have such different personalities and learning styles.

The first week back to school was pretty crazy. Of course I had chemo on Monday- so really sent Lily  off to her first day of 7th grade with a lot of faith. She rode her skateboard with a friend. I hadn't prepared my usual pack of information that I deliver to the health room. I felt horrible about it- a new school- they'll never have heard of adrenal insufficiency. The public health nurse took care of Phillip and worked with me over the summer to get some of the items taken care of. In the past - it was usually January before anything was set so part of me decided- what's the rush? Plus I can't even take in the meds until the proper paperwork was filed and rarely was this ever done in the first week of school. For either child.

When I called the health room, the bubbly aide seemed surprised she hadn't met me yet or had Lily's meds- she had already treated several kids that morning. Of course Lily's counselor and the skilled nurse would be in contact with me. I let her know Lily had an audiology appointment on Wednesday and I'd pick her up early and bring the meds then. She said she would make sure the counselor and skilled nurse knew. That was - a bit easy. Ok.

I got the packet of information together- it includes Lily's AI information, all the doctors and emergency numbers- when she needs meds, background on adrenal insufficiency and instructions on how to give the injection. Each year I usually just have to update her picture- this year I had to update- med amounts, and all of her docs and emergency numbers.

I went into the health room, I felt very comfortable she knew who both Lily and I were( check 1) - we chatted, she then took me to meet Lily's counselor who had done his research. Lily mentioned he introduced himself on Monday and showed her his office and they talked about her health and how she handles things. I'm sure he knows more than he expected about a lot of things! This put my mind at ease(check 2)  - Lily is fairly independent with her health- but due to the nature of emergency shots- that is something she just has to have back up on.

So far I'm impressed with her counselor and his interest in Lily's care. Of all the things we have on our plate- it's nice to have another person on the team. He also totally understands we want her to be treated "normally" in all other respects. I warned him that she will visit him or the health aide on chemo weeks- he understood. She really does take personal responsibility for me- it is so sweet and makes me so sad at the same time. All the kids are to get themselves ready for school, pack lunches, etc. on their own. If I'm not awake(some nights are better than others) she will come in and hug me. She's also started leaving notes on the kitchen counter like Phil used to do. I love it so much I can't even describe the feeling.

The week was busy- there were many papers to sign, things to get in order. One day I had the foresight to throw kalua pig in the crock pot. We were home lateish and I was shredding the pig. As I normally do, I tested a piece. The piece lodged itself in my throat. Not the route to the stomach throat. Of course trying to take a deep breath made it worse. I tried pushing myself over the counter chairs- they are too high- I look around while trying to self heimlich. Lily sees me stuggling and runs over- all I hear is what do I DO? What do I DO? I point upstairs- Phil is in the shower- other than that we are alone. I run over to the sink and hurl myself at the edge- evicting the anti breathing wad of slightly dry pork.

I'm shaking pretty bad- not thinking the knees will hold. I sit down, Lily wraps her arms around me. God I hate this. She's crying- I'm so sorry I didn't know what to do. I laugh- she's apologizing- sweetie- you did great.  We talk about choking and how to do the heimlich. Girlfriend can apply first aid to broken bones, sprains, knows the ups and downs of blood sugars and how to inject glucagon or hydrocortisone- I guess we've never covered choking. Kiera used to choke all the time- did the baby heimlich on that one more times than I'd like to remember- and on all of them at some point. But it hasn't been a problem lately. I leave dinner sitting and go sit out back.

Phil comes out- Lily said you were choking. Yeah. Ok? Yeah. You want dinner? Everything is screaming nooooooo- my throat burns, my head throbs- but with the stress- I should probably take hydrocortisone to help my body deal and I have to take it with food. So I eat carefully and laugh- I tell the kids all the time- we've worked so hard to keep you alive- I would hate for you to go down doing something stupid. I tell Phil and Lily- I would hate to have gone through all this to stay alive and then die by choking on kalua pig. We laugh- when the others get home, we go over the Heimlich with all of them. Lily sits closer to me now, she holds my hand more. Like we need one more reminder of how quickly everything can change. Yet somehow we do. We hug more. We love more. We argue and work things out, more. When the monkey has one day notice to play basketball- you say yes, more.

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.