Hi point of today was getting to meet Jenesis Rothblatt. Jenesis is the daughter of Martine and Bina Rothblatt. (Martin board member of Beta). Jenesis gave a wonderful talk today at beta about her life and her role at United Therapeutics 

It’s an amazing story… Jenesis was born with a very rare congenital disease called pulmonary arterial hypertension which was usually fatal. The only therapy for the disease in the past was a full lung transplant. Martine rothblatt, who is the founder of XM radio is an amazing inventor, as well as a loving father. Through his persistence and help with many others she  was able to invent a drug treatment regime which allowed Jenesis to live a relatively normal life.… Based on that experience, Martin created a company called United therapeutics that treats this disease as well as many others. United therapeutics as invented a process which would allow organ donation of lungs that would otherwise not be suitable for transplantation. The lungs heart, the last organ to be donated, and often are too sick of themselves to be placed inside a sick person. The company takes the donated lungs and nurses them back to health, and then provides them to recipients… Which creates thousands of more transplantable lungs in a year. 

Our sam was a multiple organ donor… He was not able to donate his lungs. 

OK… I’m getting pretty choked up here. I’m not exactly sure how to explain how hearing this made me feel… I think everyone would understand that anything that makes organ donation more successful and easier would be a gift that comes out of tragedy.

The treatment fo Jenesys’s condition used to involve taking seven medication’s a day… Now through innovation it’s down to a single pill, and she’s able to live a very normal life and is an executive at United therapeutics. She’s a fantastic ambassador for that company… Living proof of what science can do

What united therapeutics is doing now is really amazing. They have been doing genetic research on pigs to create organs are engineers to be implantable in humans without major rejection, disease. The implications of this are tremendous…. United Therapeutics  have already done  2 modified pig heart xenotransplants as part of a special  protocol which can be used in cases where no other viable option for the recipient exists.   In both cases, the patient who received the heart had no other options, and under took the experiment as a way of getting a short bit of more life and contributing to scientific knowledge. So far both patients died after several weeks of extended life.… That’s likely going to be the case for any such innovation… It will take  many tries. But the goal is so important that innovations like United therapeutics is doing with Xeno  transplant must continue..

Another option is going to be a totally implantable artificial heart.. That’s where my brother Billy and his colleagues at Biavacore comes in.. (watch this space)

In addition to organ rehabilitation and genetically engineered animal organs, UT is also working on hybrid organs that will be  grown from human tissue on a 3-D printed matrix… T Martine’s investment in BETA is targeted at being able to move these organs quickly and in an environmentally sound way.. he vision for the company is truly inspiring.

 

Jeneses’s talk was also inspiring.. she told the human side and the technological side in a very moving and personal way. I was watching her talk remotely while working at home., but I was so excited that I drove into work and was able to meet her and tell her about our Sam’s story. I gave her a Samstone..too

My deep thanks to Jenesys, Martine, Bina and all the folks at United Therapeutics for all they are doing. 

nite all, nite sam

nite all, nite sam

-me

Pretty routine day for the first work day of the year.. Spent most of it catching up and prepping for the new year. 

The highlight of the day was Diane’s Yoga class she does these on Tuesday nights whenever we’re around…  We do an hour or so of relaxing yoga then have a potluck.. It’s a wonderful way to get friends together .. especially in the winter.. 

Love these potlucks !

nite all, nite sam

-me

Happy new year everyone!… Who knows what this year will bring?… All I can say is I’m going to try to make it a fun and good one.

We slept in late today. Are they at fluffy yesterday was really fun and pretty hard work being outside for that long. I got to say though being around all those people really energized me.

Once we were properly awake, we decided to go for a New Year’s hike. We haven’t been up Robbins mountain… Which is in our backyard… For a good long while so that’s what we chose. There was very little snow and lots of ice and even some mud. And the thing is straight up. Just the thing to s kick in the new year.

 

At the top of Robbins mountain, there used to be an airplane beacon tower.… Our house f was called the “beacon light grange.” or many years the car our house is about 2000 feet below.… we used to hike to it all the time and climate… We’ve even slept up there. Now, though it is a massive cell tower… Kind of imposing and maybe even dangerous to approach. Even so it has its own beauty.

 

On the way up we ran in to our good friend Clint.. We saw his and his dogs footprints before we saw him. Once we got to the top, we had fun following his footprints to our favorite lookout. It’s a spot that used to be on the long trail.

 

The hike down with much easier than the hike up. We were marveling at the cool ice crystals that were emanating from the ground. Somehow related to the perfect temperature and the perfect (?) Amount of rain we’ve been getting.

Later in the evening we tried out one of dianes newest toys. I got her a a Pasta “Chitarra” (guitar).. Weed first seen one when we visited Navellie in Abruzzo. It’s a box with a bunch of stretched small wires that is used to cut pasta.… Deb came over and we tried it out. It was really fun fast and very delicious. ‘Diane made pasta with browned onions and fish sauce… Sounds weird, but was so tasty. 

 

Great first dinner of 2024… we’re off to a good start 🙂 

 

nite all, nite sam

-me

Today is New Years Eve.. and we have our work cut out for us. Today was Fluffy’s first event for the public since the dry run back in July. Yesterday we got her lights, fire control and other systems working. Today Duane drove her fully clouded from Earthkeep in Charlotte to the burlington waterfront.   We all me up down town about 10 to beging the task of getting her ready for a big show

First order of business was to get the railings up on the two dance floorsa (we later figured out that we could even leave those on and still get in/out of the big door at Earthkeep !)

 

We put out some  welcome  tables

 

 

And walked aroudn to see soemohter stuff. Chris’s CHamp was beutiful and ready to burn tonight

 

Next came rigging the fire pole and setting up audio

Rebar did all the a hard lifting and acrobatics to get the cables attached.

 

At 3 that gates opened annd  it was time to get in costum.. we just keep adding layers/ it wasnt that cold (about 20F) but we were goign to be outside all day

It was still too light outside to fully appreciate the lights.. but we tr\urned them on anyway

 

friends started showing up.. and continued to come by all night

 

Finally it was dark enough for fire and lights

 

Our fellow Fluffyite Kristin showed up to share the night.. She’s just back from heart surgery !. It was soooo good to see her !

 

Then something unexpected happened.   The team had thoguht that one of the 100 pound propane tanks was full whul the other was empty. We discovered around 5L45 that they were both now empty.. Uh-oh !

No worries, we improvised a plan ‘B’. Luckinly Diane had suggested that we bring along a full 20 poind  tank we had at home (for my own fire poofer. James , Diane and I quickly rerouted the gas hoses to feed from the little tan. The big problem then was how to keep the tan warm. As the tanks are used. they get very cold.. think of the last time you used a spray paint can.. it gets cold as it sprays because the expanding gas that comes out of the nozzle absorbs heat from the can.. 

We found a tub that would fit the small tank and siphoned some of the water we’d warmed from around the tank we had thought was full.. Then, for the rest of the night, diane took some of that water and boiled it in an electric teapot and then poured it back on the tank. This allowed us to keep the tank from freezing up. We were able to make that switch over without even turning off the pilot flame.. no interruptions from the audience experience.. in parallel, Duane called is lovely wife Laura and had her pick up an additional 3 20 pound tanks.. (we ended up using 3 20 pound tans dfo r the rest of the even !) 

 

As we were doing the swap my friend Eliot and his family came by.. baby Ezra, girlfriend Leia and her son Gustof.. Super fun seeing them all 

THen the first of our 2 DJ’s started.. first was DJ Umamni, then DJ Rice Pilaf. They were both so good !

Then came the fireworks !

Our friends Meagan and her wife Gina came by !

We got to watch chris’s champ burn from the bus.. (I had to stay and mind the fire system). It was a really beautiful burn.. The audience loved the Cirque du Fuego fire dancing and Champs burn !

I was amazed at how long these 20 pound tanks have lasted given that we had the pilot tuned up for the wind

Friends continued to drop by through the evening. I loved gettign to show them around. Here’s my good friend shannon

Tonight was such a great celebration for all the people who contributed to making fluffy real.. their names are all printed on the back.. So many creative folks !

FInally It was tiem to shut down. The event closed at 10.. we shut down the poofer, took down the poofer pole and stowed the audio and ligth contorl. The idea was to drive fluffy back to Charlotte with everything wlese rigged.. that means the clouds and the dance floor railings. We figured we had about 4 inches of clearence using the big door at Earthkeep. THis is gonna make it so much easie to take fluffy out for events

As Duane was preparing to drive out, he asked me if it was possible to rig the lights to still be on and reacting as he drove. I gave it a little thought and realized that as long as the geni was running,. I could stow the llight control computer in a safe space that would make that no problem.. so with a little more wrangling, fluffy drove off into the .. sunset ? .. no moonset ? Diane and I drove away abotut he same tiem and got to see fluffy roll on by.. a great end to the evening

We got back home at about 11:30.. Got a fire goign and drew a hot bath.We barely stayed awake until midnight .. but we made it !

We’d been outside and working from 10 am till 11PM in winter cold…

the bed sure felt warm and cozy..

Happy new year everyone.. lets make it a good one. !

nite all, nite sam

-me