Summer Break LFS style

Part of our summer routine is scans. With Li-Fraumeni Syndrome, we try to catch cancer early with the hope that we can treat it. Ultimately we hope to find nothing. We try to stay optimistic- we know lots of people who have LFS who don't have cancer or have had really good success catching tumors early. There is a certain amount of stress associated with this looming cloud- we call it scanxiety. It is real. It is vicious and no matter how much you tell yourself not to worry- you do. I get a little manic- trying to get things in order- just in case. Nothing goes wrong if everything is in order, right? Not exactly, but your mind plays these games because we are human and we have feelings and a need to preserve our future.

Before we can get the scans ordered, we need to have an annual visit with the kids' oncologist for the general clinical exam and talk about any concerns. There are a couple this year, but one of the good things about these appointments is that the docs are reassuring- most concerns fall along the "normal" kinda kid development curve but my concerns give them an opportunity to order more or less tests to rule out problems. The kids know the way to the clinic. Phillip and Lily are followed more closely because of his diabetes and her history of cancer. Unfortunately with LFS, once you have had a cancer, the chances of another one developing are much higher. Lily's already had 2. Sometimes we forget about the sarcoma in her leg because it was a lesser threat at the time.

 They line up for weigh in. A good math exercise- converting Kilograms to pounds.  I try to pay attention since I know I will need this information when we schedule MRIs. But my memory is a bit suspect these days.

 Then we pile into the treatment room for vitals. Our nurse was a saint and helped us out by managing to get all 4 kids seen at the same time. I appreciate this a lot.

 After vitals we wait in the oncology waiting room while the kids are seen in pairs. They meet with our oncologist, who will be leaving soon. I am very sad, she's been with Lily since the beginning of this journey. 2 residents also sit in. Tripler is a teaching hospital, but I also like to expose as many young doctors to our weirdness as we can. Some days I'm not in the mood- but I know how important it might be for someone else down the road just to hear- sure I've seen LFS before.

 Lily practices taking Phillip's blood. I warned them we will have to do bloodwork and a poke is involved. My kids all have my tough veins. Sorry about that munchkins.

 We LOVE special visitors like Lili the dog. This helps settle the nerves and pass the time. We talk about our other furry friends Bailey and Indy. We visit with our favorite nurses and child life specialists and docs. We miss them but also kinda grateful our visits are infrequent.

 Lily gets a bonus urinalysis this round. Love that she's old enough to handle this like a champ. Hate that she has to and that she mentions that this is one of the easier tasks. Some families do ultrasounds and other exams for their checks and get blood done every 3-4 months. After careful consideration with our docs, we agree with all our other visits and since we aren't actively monitoring problems- annual visits are good for now. It is a balance.

 Time for bloodwork. A quick poke by the lab specialists- they are the best- I've really never had a problem in the Tripler lab. I know this is stressful for the kids. It's easier afterwards when they all say it was easier than they thought it would be. Lily still has some pretty bad memories of bad poke experiences and a bit of PTSD but other than a stress tear that leaked out she breathed through the process and lived to tell about it.

 7 tubes for each kid. They look at the blood counts, adrenal hormones, and other possible markers of cancers.

Who gets a bonus 5 tubes drawn- this kid! Grand total- 33 tubes of blood from the kiddos.

 The car ride home is a good chance for nap time. I am glad we can take these steps, even if it is stressful. As they get older, it will be part of their routine and they will have the experience and tools to make the best decisions they can for their health. I would love to protect them from all the bad stuff, but some of it is just unavoidable. The best we can do is hope for some good breaks and deal with what we have to. Really it's true for everyone. Until then we have fun and try to do normal fun summer things!

Next week I have treatment and my Brain MRI- really hoping things are stable. The following week I have my PET scan and the kids each have their full body MRIs on different days. Each one will take about 3-4 hours. It will be a long week but hopefully will give us some peace of mind. We will take all boring and clear thoughts we can get!

Just Part of It- the Giving side of Cancer

As a cancer family, we have lots of organizations that have helped us as a family. Some with very tangible gifts, the Hawaii Children's Cancer Foundation that helped us with some unexpected bills when Lily was first diagnosed or the Make A Wish Foundation, who sent our entire family to Disney World after Lily finished treatment. Other groups like HUGS here in Hawaii, offered respites, surf outings and fun with other families going through tough times.  They also helped us celebrate the lives of some of our dear friends in their annual Celebration of Life ceremonies and we could always look forward to wonderful Christmas Parties. It is humbling to be on the receiving end of charity. But it is also an important thing for families to be a part of it. For years I volunteered in many different ways, we always raised money for St. Jude's in grade school, I helped run a Christmas card campaign at the Children's Hospital in Denver when I wasn't spending time wiping down the play room, delivering books to patients or doing arts and crafts.

Hawaii doesn't have as many resources as some other states, but the ones they do have are very special. Somehow we seem to miss out on the sibling camp and cancer kid camp every year. I think it would help my kids, but then part of me is truly grateful we are not in clinic enough to be aware of or recommended for these resources and too busy with normal activities. As I've learned, adult cancering is very different than kid cancering. As it should be. Kids shouldn't get cancer. I've always walked the fine line between awareness and normalcy. I've known adult survivors of childhood cancer who let cancer be the very foundation of who they were. You can not go through something like cancer and not have it BE part of you, but I want more for my kids, I want them to have moments of normal in the world of LFS. It's a balance. I shy away from letting my kids be the spokesperson or poster child for events. I didn't want them to be known as the kid who has cancer, to be labelled. Yet let's face it- it is a big part of who we are. So this year, Lily is old enough to understand more about cancer, more about the organizations we support and why we do it. I gave her the guidance and let her make decisions. She loved being up on the stage and shaving her head for St. Baldrick's. Part of me wonders is it because of me, am I influencing her in some way. Which is silly- OF COURSE I am influencing her. By my actions, my guidance every day I influence her and her siblings. And this is one of those times when she took the reigns and I have to say I'm extremely proud of what she's learned, the awareness she took to school and how honestly happy it made her to be a part of the St. Baldrick's family.

A couple weeks ago they called and talked with us for a blog post. I was tentative but also decided that it helps me immensely to hear positive stories. Lily is a positive story. The 5 year survival for her tumor was poor. Throw Li-Fraumeni in there and well the odds are kinda stacked. BUT we make our odds. By making the best of our time and efforts. I am extremely proud of our family. It's not an easy balance and they all are doing amazing and cancer is just a part of it.

St. Baldrick's Blog- Lily's Story

Life Rafts in Mutant Land.

I got to spend some quality time with my family this past week. My online mutant family, some  of my own mutant family and some non mutant family. Sometimes when we are far away on an island, it's hard because I miss my family so much. The milestones, hanging out for holidays, the craziness. Sometimes I'm glad to be on an island and missing out on some of the craziness. But there is nothing like the loneliness and helplessness you feel when cancer wallops your family over and over and you are an ocean away. Add in I'm not the biggest fan of air travel and the carefully orchestrated planning required to keep our routine afloat- that means my travel plans are thoughtfully considered and often come down to last minute luck. I sit and fretfully worry about the deluge of problems that might befall me anytime if the cancer Gods so much as get a whisper that I might be planning a trip. Yet, we have carefully navigated this superstition and the outlook is good.

When the Li-Fraumeni Association announced the 2016 LFS conference in Columbus, Ohio I was a bit disappointed- Columbus. It's not as far as the east coast, but still a bit of a trek. Of course the last time I was actually in Columbus was 20 some years ago and we merely drove by in 2012 during our summer tour. Man has Columbus changed! The airport and city were clean and very friendly, I was really impressed. The other really big positive was that it was within driving distance for a lot of my family and so many of my online support group friends. Several sponsors of the conference are local to Columbus- Nationwide Children's Hospital, and The Ohio State University. They have worked closely with the Wyss Family(Soccer for Hope), who has been profoundly affected by LFS and has dedicated over a decade to helping foster LFS research and advocacy.  The other bonus is that the Ohio State University has a really good Biotechnology program and I have a high school senior who will be looking into colleges this year. Perfect timing for a mother-son trip and maximization of my traveling to visiting people ratio. Talk about getting your proverbial mutant bang for your buck. Probably shouldn't say that out loud. or at all.

Families, real and acquired can be a tricky business. In mutant land, I've acquired some extra family by choice who are as genetically volatile as I am. I've heard my uncle say more than once that our genes were fished out of the shallow end of the pool. But some of our other ones come from the deep end of the ocean- the ones for resilience and empathy and ability to triage bullshit into a palatable sandwich and laugh about how crappy it tastes. These traits make for some really interesting after conference discussions. Many of which are fueled by adult beverages.

There is no way to get from Hawaii to Ohio without losing a day. Phil, being the dedicated compassionate, wonderful husband he is- not only phenangled travel arrangements, his schedule and childcare for the girls so Phillip and I could attend- he understood how important it was for me to go. The time I got to spend with the younger Phillip was invaluable. He used to be little. He's not anymore. I know most moms wish to slow down the growth, I don't. I'm grateful for every moment, every step. Even the ones that are icky and exhausting and I have to chant over and over- I'm grateful for this. I'm grateful for this. I had a few panic moments of- shit- he might actually be going off to college in a year and I might still be here. That's where the panic set in- I'm not upset we are to this step- we live on an island- he hasn't toured colleges- he doesn't know what's out there, how can I make this happen much less even consider it financially or medically. It makes me momentarily miss normal until I realize there is the illusion and opportunity of normal and this is it. We can't spend weekends driving here and there to peruse the interstate options. He's seen colleges, but he was younger and your perspective changes. Columbus was a good opportunity. He's dealt with diabetes and what it means for him for years, but learning about LFS is more of a gradual education in our family. Gradual in the sense of disaster preparedness training, I set up the foundation of appointments and we work our way into what exactly the risk means as they get older and can understand more. It's lining up your life rafts and hoping you never need them. Sometimes you get schwacked with information rapid fire as the tidal wave of each cancer diagnosis happens. College will be a strategic placement near someone we are close to- so if the shit hits the fan-he is near someone or at least within throwing distance of a lifesaver.

This year, young Phillip came home from AP Biology with a lot of questions- they were learning about TP53 as a tumor suppressor. He was a bit overwhelmed by the implications. But then he started asking questions- how do we treat it- what about this- what about therapy- what's being done in research? I think I want to help find a cure. I will admit that the fervor and hope I had as a young woman about the idea of a "cure" in my lifetime faded a bit by the constant barrage of cancer and loss in my family. As my dad laid dying he never once discouraged me, even though the hope for a cure for him was not even a possibility. He had hope for me and I have hope for my children. Suddenly I mentally transported to 1994, considering college with a sick parent, being unsure of finances and life and this big step- not wanting to be too far from family but wanting to be far enough for some space. I could see the weight on Phillip's shoulders. Normal college kid wants with the shadow of cancer. Taking Phillip to the Columbus achieved so many goals, quality time with each other, family time, learning about our syndrome, and checking out a college. I contacted friends in the area to coordinate visits and managed to line up a tour of OSU with a mutant friend's son. I love it when a plan comes together.

We landed in Columbus early Tuesday morning. Fortunately a room was ready and after 19 hours of short naps on 2 different flights- I was ready for a couple hours of horizontal napping which would refuel me for mutant shenanigans. I woke to texts from arriving friends and so began 4 days of therapy and hope. Like I said, mutants are a bit volatile. Not in the aggressive way, but rather the combustible nature of our cancering. Treatments and screening take time and resources and often times- that time and those resources have to be filched from normal sick leave and budgets. It is not easy to find a chunk of time or money to make it to a conference in Ohio. We tried to help as much as we could through Living LFS, and there were quite a few scholarships granted from the other sponsors- which helped many get there who would haven't otherwise been able to. The therapy was immediate and tangible- usually in the form of laughter through tears- the best kind of tears. The evidence of wounds gradually healing and sadness released into the wild. It is a rare gift to be able to meet friends you've met online and feel like you've known each other forever. This wouldn't have been possible without donations(remember those fun t-shirts?! yep- THANK YOU!) and the sponsorships. It was also great to see the number of family members and researchers that took the effort to be there. When you have a really super rare disease and you get to be in a room with a couple hundred others who get it, who are all working towards the same goal- it's pretty inspirational. And humbling. And exciting. And let's face it- mutants are awesome and so are their families.

I got to hug old friends who I haven't seen since the very first 2010 NIH LFS conference, new friends we met in Boston and Portland and new new friends who will now be the Columbus friends. I talked with mutants about how to talk to kids about LFS, how Phil handles being a spouse and the ever present balance of loss and hope. I talked with researchers about new developments in adrenal cancer, like Lily's and mouse models for LFS. I talked with Genetic Counselors about resources and needs in the community, the possibility and hope for family camps. Talk about one of the many times I wish I could clone myself- so I could just sit and talk with everyone.  Yet in true mutant fashion- we packed as much as humanly possible into the short time we had. We set up a Living LFS table with hand outs and information and of course- hugs and support. The young Phillip got to sleep in and decompress from 4 AP exams the previous week but also got to chat with people about LFS and hang out with family. It was meaningful to look over and see him respectfully trying to communicate with a pediatric oncologist from Japan or hanging with Aunt Kathy and Uncle Tom. Or to look across the room and see my uncle chatting with a friend we met in Portland. Or to see my mom and aunt hugging a friend from the support group who just lost her daughter.

We talked a lot about the ones we lost, in a way where you didn't feel overwhelmed by the loss of it, but in a way you  feel when you talk to someone who understands, who has been there. In ways that honored their memory. In a way that says- LFS sucks but look there's a life raft off there in the distance- the hope for a better LFS reality! You say their names and it doesn't sting, it feels like you are saying hi to them. You skip the conventional formalities and jump right to the heart of communicating and relating. Sometimes it's difficult to interject Bob and Dad into conversation without the emphasis on loss- I've dealt with it, but sometimes the people you talk to have a tough time. There are awkward silences and discomfort. But in this group, I could talk about them freely and there was none of that, just hugs of encouragement and nods of understanding. I missed Bob and Dad, as I do every day. Having my son, mom, cousin, aunt and uncle there with me fulfilled a need I didn't really realize I had until then. I missed my aunts. I missed my cousins. It was nice to have a strong Connolly contingent there.  I know for many it's hard, these gatherings sometime emphasize the loss.  Feeling lonely in a sea of people. Until you connect with others who are floating in their raft- fishing for those qualities in the deep end of the ocean and you latch your rafts together. And I realized that in taking Phillip to this one conference in Columbus, I opened up some options for college. Not just OSU, which he was really really impressed by- but the number of potential life rafts grew exponentially as he got to know the residents of Mutant Land. And although we are not genetically family in the normal sense, we are linked by mutations and common challenges and just like I'd toss a raft out for any one of them, I feel pretty confident there are safety options available for him. I remember going off to college and feeling bad that Bob never had that opportunity. I look forward to the day that LFS is a more easily managed condition- like diabetes and conferences like this one are a huge step in that direction. There was realism, there was hope, and there was family. And as Phillip and I settled into our seats on the plane in Columbus, I felt sad to be leaving because in our world- you never really know which visit will be the last. No one does really. Then the captain came on to welcome us aboard and introduce himself, Captain John Connolly. Which is my dad's name. And I thought what are the odds and knew we wouldn't be needing the life raft today.