fibrolamellar

  • DIARY ENTRY:

    • The Rarest of the Rare

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    So me being me (Danielle that is), I’ve been digging hard when it comes to whatever I can find online. Studies, research, foundations, charities, medical breakthroughs, therapies, memorials, blogs, facebook groups… And I am insanely amazed with how much information does not exist surrounding Fibrolamellar. There are less than 10 official groups on facebook that relate to some sort of foundation or mass gathering of people affected by this type of cancer. When it comes to studies, a consistent thing that keeps coming up is that 1 in 5,000,000 people get this disease and only a few hundred in the US are diagnosed a year. This means that when it comes to research or clinical trials, there’s a small number of people that can help contribute and because of lack of expenses for travel lack of financial backing for these studies, several studies include numbers under a hundred. It’s unreal.

    When it comes to support, there’s an incredibly small number of foundations. While there are a couple that seem to have a large following, most are independent memorials and charities for lost loved ones or people currently going through it. The most official so far (the only one that pops up for several pages on google) has been the Fibrolamellar Cancer Foundation. They have a whopping 1,759 people who have liked their facebook page (no offense to anyone from there reading it). I’m just in awe of how small this world seems. Yes, this is rare. Yes, it makes sense that there hasn’t been a whole lot of facetime for it because of how small it is. Yes, it’s hard to round everyone up when there are so little to round up.

    I’m not going to lie, earlier this week I felt insanely defeated and so incredibly unintelligent. I can usually find anything I want online with a little digging, but man, to have dug all the way to the bottom and to realize that there’s nothing else has been a total blow to my spirits. BUT, I do have to say, I did find some gold (even if it wasn’t a lot of it).

    Dr. Sandy Simon and Elana Simon

    I came upon an interesting set of researchers. A woman named Elana Simon was diagnosed with Fibrolamellar when she was a pre-teen, beating it years later. As she got older, she decided she wanted to pursue research on her cancer. Their goal is to pursue and understanding of this cancer using gene sequencing. Over time, they were able to find consistent genetic mutation in most of their patient studies; meaning that they may have found the common link. After this first round, they realized a few things: 1. There isn’t one database that documents all Fibrolamellar cases and 2. They need more participants and tissue samples in order to help push the data towards a cure. So they decided to start something called the Fibrolamellar Registry. Out of most of the research that I’ve done, I feel like they’re onto something. If you wanna read through an insane Reddit interview they did (it’s a little crazy but really informational if you get through it all) you can find that here. And if you wanna hear more about the breakdown of their study, Elana can explain it all to you here:

    Immunotherapy and Cell Therapy

    Right now, there is no “cure” for this type of cancer when it comes to treatment. When Dr. Finn was saying that there is no direct route when it comes to treatment, he’s telling the truth. There isn’t one type of chemo that does the trick, and because of that, people have been looking elsewhere for answers. A couple of them (which I find super interesting) are Immunotherapy and Cell Therapy. Here’s the general breakdown. This type of cancer is part of that person in the literal sense that it is not this outside entity that entered their body, it’s made from their DNA. Using this logic, the idea is to utilize each individual person’s genetic constructs to fight back. And easy way to understand this was an article in the New York Times: Setting the Body’s ‘Serial Killers’ Loose on Cancer”. Basically, the idea is to take that person’s blood, modify it so that it has a weapon to specifically fight the cancer cells, reintroduce it back into that person’s system which then kills off the cancer. From my understanding, it’s somewhat of the same concept for immunotherapy (weaponizing the immune system to fight back). What’s crazy about this is that so far, cell therapy is only working on a small number of blood cancers and has yet to make any way with others like breast cancer or other organ cancers. As for immunotherapy, there seems to be some trials that are hopeful for results. What makes this complicated is that it is again based on genetics and has to be applied to each person individually. There are obviously side affects and it’s not 100%, but they could be hopeful treatments. Right now Megan’s results are being sent for genetic testing, which could mean that if they find something that corresponds with this type of therapy, these could be an option. Note: If some genius scientist/researcher is out there reading this and is saying to themselves how much I’ve got it wrong or how much of a dumbass I am, please email me because I want to understand and this is what I’ve got so far. I want to get it right!

    So after finding this small, small amount of info I have, I still feel like I haven’t done anything. I’m gonna keep digging and keep looking; I hope there’s more to find. I’ve got some ideas percolating, so we’ll see how they play out, but I know there’s more I can do right now and much more that I can offer even though I’m just a designer. Anyways, to update everyone, our next doc appt is on August 26th. Hopefully we’ll have a gameplan by then. Until then, hit up Megan at the email below. She’d love to hear from you!

    MeganEmail2

  • DIARY ENTRY:

    • The Details

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    MeganIceCream

    The What

    So as Megan has updated everyone, she has a rare form of liver cancer called fibrolamellar. It’s found in young women, is slow growing (in Dr. Finn’s words: has a long natural history – Megan has likely had this for years already) but it’s still serious because well, it’s cancer. As for the triplets, it seems that the Tims are definitely masses but if they are directly attached to the liver or free floating is still up in the air. As for Tiffany, it seems like she’s just a really irritated lymph node that’s just hanging out by the pancreas. That’s tricky stuff- it’s very hard to tell from the imaging and makes them hard to surgically remove.

    The Why

    Again to quote Dr. Finn, “It’s just bad luck”. It’s not genetic, this isn’t the form of liver cancer that you can get from birth control and it’s not because of her diet or lifestyle. It just plain bad luck. Fun.

    The How

    So when it comes to treatment it’s a bit of a conundrum (again, Dr. Finn’s words). There are big lymph nodes and masses and it’s a rare form of liver cancer. This means that they don’t have a whole lot of research or studies that show a certain treatment course is the proven route. In general when it comes to cancer, treatment is often basically a trial and error sort of process. They play with the amounts, how often, how long, and what type of chemo and check periodically to see if things are working. For Megan, this is a little hard because again, they don’t have a whole lot of info on it. Dr. Finn even said that there’s not a specific type of chemo or radiation that is known for directly combatting this type of cancer. So here’s the plan:

    Genetic Testing – They’re going to send Megan’s biopsy out for testing. Considering that they don’t have a go to treatment for this, they’re hoping that by identifying it’s genetic markings they could find a corresponding treatment (a certain type of chemo is most likely) that works for that specific marker and go on from there.

    Tumor Board – Dr. Finn and the whole UCLA cancer crew are meeting tomorrow to discuss Megan’s case. As noted in last posts, it has a couple of surgeons, radiologists and several other types of doctors that all weigh in. While they wait on the genetic testing to move forward with medicine, there is the possibility that a surgeon on the board might want to do something called “debulking”. Debulking basically means taking things out. It’s not clear if they would suggest whole pieces of liver, just a mass or two, or what, but that might be a possibility to help with the spread as well as make Megan more comfortable (because the Tims are getting a bit big and it’s not comfortable for Megan to do certain things anymore). Just to note, but kinda relevant, a liver transplant is not something that they are considering, at least not now. There are problem areas around the liver as well, just replacing it doesn’t fix the problem and because Megan is healthy and has a functioning liver it’s not something to consider now.

    Just keep doing what we’re doing – Obviously, we feel like we are twiddling our thumbs because we aren’t smart enough to do our own genetic testing and it’s not like we get to sit in on the tumor board (or would even understand anything going on). We asked if we needed to change anything to her diet or daily regimen and Dr. Finn said “don’t try to go on any extreme kind of diet… people have good intentions, but it’s all kind of hokey.” (Haha! This guy’s bed side manner is ridiculous, right?!) She’s had it for this long and it’s not going to rapidly change anytime soon… So I guess we just keep eating healthy, working out regularly and going on with our day to day.

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    Several of you have just found out via FB. For those of you who are new to this blog, we’ve made an email for words of encourage, love, anger (towards the cancer obviously), etc. so that we can keep track of responses and that there’s some privacy in things. I’m sure Megan would love to hear from you guys and we’ve got a long road ahead of us, so feel free to save a stamp and send a message instead!

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