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So Rachel can be a low-information friend, meaning, she doesn’t quite tell me what is going on or what is going to happen. I think this can be a cultural thing as my family can be a need-to-know-basis (my parents often go to the hospital and don’t tell me until everything is resolved) and Jeremy’s family will go into deep analysis of logistics of very small things, like comma placement in emails. So as I slid into the car on Friday morning at 4:30 am with a strange driver, I did not question it. Was it a friend? An uber driver? An additional guest flying with us? I did not know and I did not ask. And Rachel had insisted on sharing a room with her at the hotel, but I was not quite sure if we were renting a car in Miami or if I was going to be able to check into the room while she was at the rehearsal. Was I expected to attend the rehearsal? I was not sure. Also, I had at some time been under the impression that I was supposed to attend the rehearsal dinner (usually reserved for family), but, again, unsure. This was a little problematic because usually, I have no problem wearing the same dress to all the weddings I’ve ever been invited to, it was not possible to wear the same dress to three different events on three consecutive days with the same people (there was a morning-after brunch as well). I was also under the impression that Pat had local family and was going to be staying with them. Anyways, why did I not ask about clarification of these things? Well, again, it felt very culturally familiar – Rachel is about 10 years older than me and calls me her little sister and kind of bosses me around. So I was enjoying watching how things unfold. And one of the unfoldings was that as we were checking into the hotel without Rachel because she was at the rehearsal, I realized that there were two beds for the three of us (Rachel, Pat and me) and that since Rachel was the mother-of-the-bride, naturally it meant that she got the bed to herself and so I spent the weekend sleeping with a woman I had never met before. It was great that Pat and I got along so well that on Friday night, while the rehearsal dinner was happening, we just got a quick bite to eat at the Whole Foods next door to our hotel and we changed into PJs and were in bed by 9 pm (together, of course) – her planning out the next day on her iPad and me trying to fall asleep because I had gotten up at 4 am and spent the day on the beach with the sun leeching energy from my body at an accelerated rate. At 10 pm, Rachel bursts into the room with her other non-bride daughter, the boyfriend of said daughter and Joon, the brother-in-law to find us 2 PJed women in bed together. I’m not sure what they thought of that – I was like – Rachel, you need to send a text! I need to at least get a bra on (though I told Jeremy, no one wears a bra in Miami, so I guess it was OK). We made some introductions and chatted for a while (Pat, bemused, sat in bed the whole time, nonplussed). So then everyone else left besides Rachel, Pat and I and that night, before the wedding, was hilarious and funny and really, was one of the highlights of the wedding for me. We made sure all of Rachel’s outfits fit, that the Spanx worked it magic wonders and we fell asleep at 11? 12?.

The next day, the women needed to be at the venue at 10 am for a 6 pm wedding (!). I have no idea how hair and makeup can take 6 hours, but it does apparently. We went to breakfast with Rachel’s brother and wife who came in from New Jersey.

Joon wanted to spend the day resting, so Pat and I were let loose on Miami by ourselves. We went to Wynwood Walls by taking the free people mover and walking.

Pat loves to try restaurants, but actually can’t eat that much solid food because she has issues with her mouth from a biking accident years ago, so we ate “lunch” like three times – each time ordering drinks and only one dish which sometimes was just an appetizer. At the first place, we ordered a pizza for one, she had one slice and I had the whole rest of the pie. The second place we ordered only fried pickles and sangria (for me) and cold brew (for her). So I did drink a lot (based on my baseline) on this trip and I did not feel tipsy in my usual way after half a drink and I chalked it up to weak drinks or the heat or that I was eating a lot. But I think it was weirdly dampened by the Effexor.

Between the restaurants, we went to the paradox museum which was a funny optical illusion museum.

Look at this gorgeousness from the bridal suite.

We got to the wedding and here we are pre-wedding in the courtyard adjacent to the outdoor space where the ceremony and reception were going to take place. I stopped taking photos at this point because it felt like everyone else was taking photos including a very professional three-person crew with large cameras slung across their bodies. It’s a bit ridiculous to stop taking photos, but I did.

Anyways, it was a lovely ceremony and dinner (Rachel was like – OMG the servings were sooo small! Everyone ate everything! In Asian cultures, at weddings, one must always have too much food, so no one leaves hungry – if everyone finishes everything, it means that you didn’t provide enough food. I told her it’s the opposite in the US, the more expensive the food, the less you get of it.). I had heard whispers of a surprise at at the reception and boy, it was it a surprise. Here is the video. Mind you, it was the bride’s idea for this entertainment. I bet this has never happened at any wedding you’ve gone to. It was, honestly, a lot of fun and fun to party in Miami. I love weddings because it reminds me of my own marriage which I’m so happy to be in. To a lifetime of happiness!

I took a very early morning flight on Friday to Miami to go to Rachel’s daughter’s wedding on Saturday night. I had a wonderful time – full of quirky experiences and nice memories. Rachel is a nurse coworker of mine. It was going to be a lavish celebration and Rachel worked so hard to make it happen for her daughter. We travelled with Rachel’s late husband’s brother Joon who had flown in from Korea for the occasion. He’d been in the states for about 4 days already.

It was a 7 am flight because Rachel needed to be in town on Friday morning at 11 to attend the wedding rehearsal. Rachel’s best friend Pat, who I had briefly met here in Maryland a few months ago, has family in Florida was driving in from her family’s house to meet us at the airport. We then proceeded to drop Rachel off at the rehearsal and as she flew from the car towards her anxiously waiting daughter/bride, she yelled at Pat and I – take care of Joon today for me! And Pat and I looked at each other and then looked at Joon and we laughed and settled in to the afternoon. Pat and I, not really friends to start off with, spent the next two days together roaming around Miami. We actually get along well as travel partners – the pace, the things we wanted to see, the places we wanted to eat, the amount of money we wanted to spend all lined up very nicely and we had a good time with each other. Joon, joined us on that Friday – to which I used Google translate a lot. Like a lot. I have no idea how well it worked, but Joon seemed to understand various things like – we are going to the beach and we need to buy Spanx for Rachel. Pat wanted to head to South Beach and we Ubered there after checking into the hotel and had mojitos and walked along the beach.

Here I am on the beach! The weather was perfect. I had the first of many cubano sandwiches. Everyone in Miami has a designer purse, beautiful breasts and tiny dogs which they move around in strollers. If I lived in Miami, I might have to purchase those things.

We walked down the main shopping strip in South Beach looking for the Spanx (which we did find) and Pat is a coffee fiend (she likes to plan the next cup as she is finishing the last cup) and as I have been trained by Jeremy to look for coffee while on vacation, I was happy to hunt for coffee with her. Also, one of the small pleasures on this vacation was interacting with handsome young men in various hotels/restaurants/bars which I never really get to do when I’m at home because while Jeremy is still very handsome – he is no longer young. Don’t worry, all the young men who smiled at me only did so because I reminded them of their mother.

I enjoyed introducing Joon to fresh coconut juice. He is a funny man (as far as I can tell, the google translate could only do so much).

Spring break is over this morning. After a rough start to it, all of us, had a very nice spring break and I think we all feel rested and rejuvenated. Jeremy was a bit upset because we didn’t actually “plan” a spring break. I had a bunch of travel which was to happen right after spring break and I didn’t want to take any more time off of work, but this travel did not involve Jeremy, so he was determined to do a spring break vacation with just him and Edda and his father, but that became a little ridiculous because Bob was getting busier with his new job, the meeting that Jeremy wanted to go to could be done by zoom, Edda had a doctor’s appointment and Mike & Sofie really needed someone to look after Jojo, so there we were, at home with no plans.

What rescued us was a few things, Edda’s aftercare ran a camp every day during spring break from 12-6 which made Edda very happy, I’m not sure I’ve seen her so consistently happy each day. They had aquarium day, bowling day, pizza day, movie day, planting day and the weather was mostly superb. Look how happy she is at pickup! The other thing that rescued us was that Jeremy did take a lot of time off and kept that time and spent it on himself – doing long bike rides and keeping work to a minimum-ish. And the last thing was that I did travel last weekend to Miami – which really made me very happy and feel adventurous and even though I lost a lot of sleep, I feel quite refreshed and rejuvenated. I feel good (finally!), not too moody or upset or rage-y. All good things.

Last Thursday, we had an appointment at CHOP to get the prescription for Daybue. Since I was taking care of the doggies and was travelling the following day, Jeremy did the appointment on his own with Edda. A day trip with a short lunch with Bob at the downtown Philly Shake Shack. So everything went well at the appointment, including figuring out how to log into Edda’s account – after she turned 18 (Vincie too, but it’s easier to ask him to do it), all her medical accounts shut down access to us, so it’s been a journey to get access to them, especially at large-ish institutions, but Jeremy managed to get the password reset and hopefully the drug refills will be more straightforward now. Edda’s dose is 60 mL twice a day, at $20 a mL, this would be close to $900,000 for a year’s dose. Did I tell you that my dad woke us up calling at 11 pm when he found out how much it cost? Anyways, I think the drug company wants us to take it, so they have a team to duke it out with the insurance company. They said the paperwork will start on April 17. Who knows when the drug will start shipping to us. We are cautiously excited – though this morning, as Edda was smiling at breakfast so broadly after a great spring break for her, I kind of didn’t want her to try it because she’s in such a good place right now. Her poor brain! Anyways, it’s a complicated feeling.

In front of Ben Franklin. The original list-maker!

I used to cut Jeremy’s hair before we had kids. I did it very slowly and carefully, oftentimes taking an hour or so. But then we had kids and he found a barbershop he liked and had his hair cut there many, many times. But then during the pandemic, I went back to cutting his hair and since we all cared less about how we looked and I practiced more, I can give him a good haircut in about 15 minutes. I thought he’d go back to the barbershop after everything opened up and he did a few times, but he was still wary about the masking and possibility of germs, so I went back to cutting his hair for him. Now I think he might never go back to the barber and I’ve written it down on my list of things to do that repeat every 6 weeks. So at the six week mark, I asked – hey – it’s time for a haircut, but he demurred until 8 or 9 weeks until he was clearly shaggy and then we proceeded with the haircut. So my question is – should I leave the repeating to every 6 weeks to remind him (which would make him look clean cut all the time?) or mark it at every 8 weeks? Or should I just wait for him to ask? All possibilities.

Elka on a dog walk with two sticks perpendicular to each other to form a cross to ward off evil spirits.

It was a lovely weekend. Rainy on Saturday, but pretty the rest of the weekend. Jeremy was social this weekend with a meet up in DC on Saturday. We only had Edda-care on Saturday and I needed to get out of the house so even though it was rainy, I took Elka on one of my favorite Elka walks around Clopper Lake – about 2 hours. We did get wet, but the trail was empty which is my favorite.

On Sunday, I met up with Lana and it was really fun. We haven’t seen each other in a long time – before Vince got into college, before I even got my nursing job, so there is a lot to catch up on. We talked about retirement! I can not believe it. Our plans (which I will keep mysterious) might rejoin in the next decade – it’ll be funny if it works out the way it’s supposed to work out.

Jeremy hosted a very quickly arranged Sunday night dinner which was fun and quick.

What made me the happiest this weekend is the solidification of Vince’s summer plans. All of his friends (it felt like) had lined up internships and science-y/engineering-y summer jobs months ago (or a month ago) and Vince had applied to a few (maybe 10?), but hadn’t heard back from any of them and we had talked about this. He was both resigned and happy to be a camp counselor this summer – either at home at Edda’s camp which he was a counselor at after freshman year (they would take him back in a heartbeat, all last summer (the summer after his sophomore year when he spent the summer in Davis), they asked where Vince was or if he was coming back) or at Philmont scout camp (which I was encouraging him to go to – it would be nice to spend a summer outside). It would have been fine, but I knew he felt a little disappointed, but on Friday night, he got an acceptance into an research experience for undergraduates at UC Irvine. He’s very, very excited and so am I. You can read about it here. He said after had written back accepting the Irvine position, arghhhh, applying to things is hard! I was kind of sad that I hadn’t gotten anything, I was going to play off the Philmont thing as – yeah, I’m a mountain man this summer, but this is better.

Jeremy rode to work yesterday and spent a lot of it outside in the daylight. It was a nice ride and he got to socialize with people in the office which was nice. It was nice for me to have the house entirely to myself. I’m not sure why it makes a difference, Jeremy won’t talk to me for hours at a time and, I, am of course, completely comfortably with him, but maybe I can “let go” more? I have no idea what that means.

I spent a few hours with Vince on the phone/video doing some taxes, financial stuff, renewing his driver’s license – this year we upped the technology a bit by sharing screens, lol. He totally is a having a great year – doing very well in classes (like impressively well, I think better than I was ever doing in a major that is harder than mine – I’m so impressed!), having a nice social life and enjoying his time as a junior. This is his spring break week – a little late for most colleges and he had plans to go to a friend’s home in SoCal for the week, but that fell through when the logistics became hard. Then we asked if he wanted to spend some time in San Francisco or go somewhere local in the area (or even come home – our friends’ kids have run the gamut from going home and playing video games to going to Mexico where there is a running tap in the hotel room for vodka), but he said he was looking forward to a quiet week on his own, catching up with sleep and just meandering around Davis doing what he wanted to do on his own. We are all homebodies in the end.

Edda’s spring break is next week. We have no idea how to travel so we are managing only to work and Edda has camp! Instead of traveling, we are taking in another animal – Jojo, our doggie friend next door for the week. She is sleeping at my feet right now. She also likes to sleep on the bed and lick me intermittently throughout the night and wag her tail – thumping on the bed – in the middle of the night. We’ll have to work on that.

Elka is still not reliably housebroken and it gets worse when there is “action” in the house – so Jeremy and I decided to have her wear the pants that she wore when she was in heat. But now we call them her “party pants”. I’m hoping this will save us some arguing.

I moved into a new position at the patent office this week where I’ll spend 30% of my time in Zoom meetings. I did this primarily because after I gave up my nursing job, I felt like I needed to do something different professionally – it wasn’t going to be going to nurse practitioner school (which I had turned down for a Fall 2022 start and had been the original plan back in 2013 when I started regular nursing school). It’s a program where they selected 36 examiners from across the agency to work together to brainstorm ideas to improve the examination process and then test them out before implementing them across the corp. There are 8,000 examiners across the country, so it could impact a lot of people’s workflow. I also did this because I kind of missed the entire pandemic driven evolution of work-from-home in the sense that my work from home job rarely involved other people, I really just worked by myself. And my not-work-from-home job involved absolutely no zoom meetings at all. So I felt like I wanted to understand this new “work culture” of zoom meetings. Which is different! I’ve had 3 hour meetings every day this week in which I’m just listening and participating in only occasionally. The first thing I noticed I needed was something to do with my hands so I wasn’t so completely bored and wander from the meeting, but nothing too exciting so I wouldn’t pay attention. Jeremy immediately recommended solitaire (he’s been at this zooming for a long time). I find sewing to be the right fit – I used to hand sew all of Edda’s bibs (which are a bandana with a small square face towel sewn in to make it absorbent) and I had to give it up to machine sewing many years ago because I didn’t have the time and she would go through them too fast for my hand sewing, but I reverted back to the hand sewing to get me through the meetings. (I like the hand sewn ones better. I wonder if I have any of the original ones left from when Edda was small. Unlikely.)

I’m more time constrained now, given the frequency of the meetings – I’m sure it’ll taper off in a few weeks. I was supposed to meet up with a pal on Tuesday to walk, I made it to the hiking spot, but her GPS took her in the opposite direction and we couldn’t meet up fast enough to get me back to my desk in time. So Elka and I walked on our own (quickly).

The day after the party, Jeremy and my parents embarked on Operation kitty kat. Emy and Seth have finally settled down on the west coast and we needed to get Ivy over to them. It just happened that my parents had a booked direct flight from DC to Portland. So we came up with the idea that the cat should travel with my parents to Portland. Kudos to my dad who is a good sport and was willing to fly with a diabetic cat. Jeremy found out all the requirements and bought the crate and managed the logistics. My dad practiced putting the cat in/out of the crate for going through security (crate through the X-ray, cat through the metal detector).

Jeremy packed the insulin in a thermos and a sponge-bob lunch container with some insulin syringes. Everything went through security fine, but Jeremy did such a good job with the cooling that the insulin froze (and therefore, was ruined :(, poor Jeremy).

We had an Apple Air Tag on Ivy and could see him traveling through the airport.

Emy and Seth met my parents on the West Coast and picked up everyone and drove them home. Ivy did great! My dad did great as well. Now we have like 3 days with only Elka until we have other animal border. Yikes! We like to run a pet sitting business on the side.

Acadia got FDA approval for a new drug a few weeks ago that is going to be marketed as Daybue and it will be the first drug specifically to treat Rett Syndrome. You can read about it on their website: here.

There are two major Rett Syndrome charities: The International Rett Syndrome Foundation and the Rett Syndrome Research Trust. Both were started by parents with children with Rett Syndrome. One might think the community affected by Rett Syndrome is so small, wouldn’t one organization be enough? And wouldn’t it be better if they joined forces? But it is not unusual in these small medical communities where the stakes seem to be so high to have two or more groups because of different philosophies, different strong personalities, etc. There is a long, long history of the two of them and if I had to describe them in a few words, I would say IRSF is more family-support-based and RSRT is more science-cure based. This was more true when Edda was diagnosed but over the years, IRSF has certainly incorporated more scientific discovery, etc into their repertoire. Now if I had to differentiate the two from a scientific standpoint (with no real evidence, just my feeling), IRSF likes to work with existing drugs and/or traditional modes of finding treatments/cures, RSRT probably focuses more on the “home run” cure and leapfrogging into newer genetic technologies. I am forever grateful for the passion, hard work, tenacity with which both organizations help families and our loved ones. We have taken advantage of both organizations’ programs. I have friends who are passionate about one or the other organization. I, as I am generally low conflict, wish that there was only one so I don’t have to choose, but I can see why they needed two. We’ve generally supported RSRT because I prefer their scientific approach and their transparency regarding their finances.

But! This first approved drug trial was funded and managed by IRSF, and Dominique, the Chief Scientific Officer, is a friend and did come to Edda’s party to say hello. And we were able to talk to her about the drug.

I have mixed feelings about this drug. I’m ecstatic that there is a specific drug for Rett Syndrome. I’m hopeful that Edda might show some improvement – I’m most focused on having her, perhaps, to not need her arm braces to control her involuntary arm movements. But there are some drawbacks – first, the biggest side effect is explosive diarrhea which is for sure a big deal for someone who needs someone else to help clean them up. Eric Marsh, our neurologist at CHOP, and his team have developed a pretty good bowel regimen to control the diarrhea. The second issue for us is that it’s a 50 mL dose twice a day and that it tastes pretty terrible. Edda is not a big drinker, so to get her to reliably take the medication will be something we’ll have to see. The list price for this drug is about $20 a mL, so that’s $2000 a day and almost three-quarters of a million dollars for a year’s treatment. Now, Dominique says that the company is ready to work with the insurance company to get it covered, so I’m not sure how that is going to work out, but I anticipate that it will be covered long enough at least for us to try it out and see if it is worth the trouble.

A few days before the party, RSRT released a fact sheet of their interpretation on the data: here which sets forth a more unsympathetic analysis of the clinical trial data. The most important takeaway from this analysis is that 61% of the people saw no improvement. The clinical trial promised that after the double blinded study, your child would have access to an open-label trial, so if you signed up, you would be promised to have a chance to try. In that open labeled period, almost half of the participants withdrew from the study.

We have an appointment in the first week of April with our neurologist where I anticipated we’ll get a prescription for Daybue and we’ll try it out. Dominique says that it should be covered at no cost to us through our insurance and it’ll be delivered to the door via refrigerated shipments. We’ll see!

Friday was Edda’s 19th birthday and, though it always brings me a little sadness, it was also mostly a happy day. She went to both school and aftercare and Megan, one of Edda’s teachers, sent me the most wonderful birthday gift as a video. Edda is often not very engaged in activities and because she is limited in how she can control her body, she can not participate in many things on her own. But here she is, in the balloon inflating room (the special needs kids run a little in-school business where they sell balloons and they learn the skills needed to take the orders and fill the balloons, etc), totally entranced by the balloons and actually reaching out to play with them! This purposeful use of her hands is extremely difficult for Edda and it warms my heart that she was able to do this and have some fun on her birthday. She is also in such a great mood in this video.

We spent a bunch a lot of Saturday preparing the house for the annual pancake breakfast birthday fundraiser on Sunday. We cancelled in 2020, 2021 and 2022, and we decided only a few weeks ago that we would hold it this year. We got almost 80 RSVPs, and so we set to work. Most of it was Jeremy as he prepped all the food (pancakes, bacon, coffee, juice, blueberry compote, fruit salad). To host 80 people in the house, the whole downstairs needs to be reconfigured, so I ended up doing most of that. Eliana helped on Saturday with the decorations and Megan came by on Saturday night to help with last minute arrangements.

The morning of, our sous chef came (Shaney – Ginny’s son), who was amazing and totally is welcome to assist any with cooking any day.

Both sets of grandparents were able to attend.

We were able to have some outdoor seating, we lucked out on the weather.

Lauren made the most beautiful cake!

Edda and I sporting matching outfits, lol. I wish I got more pictures, but I did not. The house was full of people and pancakes, we got to see old friends that we haven’t seen for three years. One of the most comforting/exciting things was that we got a good cohort of Rett families to show up. We used to get together more regularly, but it hasn’t happened in a long long time, so it was nice to catch up and see the girls and to chat about the new drug that just got approved to treat Rett Syndrome a few weeks ago, which, based on an informal survey, we are all going to try. We all have some reservations, I will explain in the next post perhaps. It’s a symptomatic treatment for Rett, generally suppose to – as I say – “make Edda 10% more awesome”(to which Mr. Pat, another one of her teachers, says – well that will be very hard because she’s already super awesome).