Paul's Post-rideATAXIA NorCal Rid Report

Saturday was a good day with good memories!

Financials:
- As of now the 2020 NorCal total is $91.4K! Hoo-Haa!
- The donors to Team Blue Skies achieved $4,150 toward finding treatments for Friedreich's Ataxia! So sweet and how wonderful! Thank you, Thank you!

Saturday:
   Friday night was my night with Brianne so at 8am the next caregiver came early so I could get the last minute details done for our 25 mile ride around RP and Cotati. We were ready to go shortly after 9am!

   At 11pm Friday night the weather report was still a 70% chance of rain. Two of my in-laws (see our team page at https://secure2.convio.net/fara/site/TR/Events/General?team_id=4411&pg=team&fr_id=1633 ) joined me in the ride in spite of the threatened rain with Susan as SAG and lunch-chaser. A close-friends couple 'fessed up to being fair weather riders but promised to drink hot chocolate for us that morning. ;-) (I'm fibbing on the chocolate, but it sounded good, and I could have used the chocolate! lol) The rain largely never appeared during the ride except for 2 drops apiece around the expressway and Redwood Ave. intersection. Immediately after the post-ride party broke up at 2pm we did get a wet-the-ground shower though.

It took us right at 2 hours for the ride, averaged 12.3 mph and had a maximum speed of 21.5 mph (the long downhill to Petaluma Blvd.). Here's the route. https://ridewithgps.com/routes/32798726

Yes by the end I was weary, and I was using all the gears to get up that "big" hill at mile 16! lol The last uphill went from Petaluma Blvd north up Old Redwood to Petaluma Hill Rd to home. That's an almost 5 mile uphill pedal and had me worried because what little stamina I had would pretty much be gone by then, BUT enter the northbound wind! I helped push me and us along to the finish! Done at 11:08. Whew! Richard breezed through the course and Karen pedaled right along. Good teammates!

Susan arrived home moments after us with JavAmore sandwiches for lunch on the front yard gravel drive with social spacing. During lunch two more BIL's joined us. A nice birthday lunch.

About 12:15pm my phone rang with the message from organizer Felicia that they were singing happy BD to me in the Noon celebratory Zoom meeting! Oh gosh, I was so distracted by the attention at lunch I had forgotten the Zoom meeting! So I excused myself and logged into the meeting of about 30 people to see Kyle playing some kind of number guessing game. A few minutes later our Zoom meeting was CRASHED by about a hundred uninvited people!  LOL! While I was sorry for the disruption to the meeting, what a singular happening! I had just read about this happening! We were told to leave the meeting and re-enter to see if that would "fix"it, so I did. Now it was all of THEM and none of US. For a moment it was funny until the XXX video was launched! That ended the experience for me! HaHa. So I went back outside to report to that group what they had missed. They were OK with missing it.

A few minutes later daughter Miranda called to say she was a couple of minutes from arriving and to hang around in front. Mmm?? Indeed shortly there she was with a caravan of cars tooting and shouting HB as they slowly crept by. The two grandsons were very excited to be participating! Last Monday they were in their driveway as we were part of the HB caravan for Kieran's BD.

Shortly after everyone went home, we rescued the chairs, tables and popup from the starting rain and retreated to our assigned positions in the house, I in front of my computer and Susan by the TV as she worked on her art projects. We had pork ribs for dinner, watched a fact-based movie called "Breakthrough" and at 10pm I called it quits. Shoulda put on my favorite Leslie Gore party song as I was brushing my teeth! lol https://www.youtube.com/watch?v=mIsnIt1p978 I don't know why I've always liked it (since 1963). I slept 11.5 hours Saturday night. I was wearied.

So let me thank all of you that provided good thoughts, prayers and your support for Brianne and Team Blue Skies this year in the search for treatments for Friedreich's Ataxia! See the latest research status here, https://curefa.org/pipeline . Progress continues to be made in spite of the coronavirus, and the momentum FARA has built up will help carry drugs to FDA approval! We've gone from "never" to "someday" and now to "soon". :-)

The Ride continues. It's not too late if you want to support Brianne, the 6K of FA'ers in the USA and the 15K world-wide. https://secure2.convio.net/fara/site/TR/Events/General?team_id=4411&pg=team&fr_id=1633

God bless everyone, stay safe and stay well!

Team Blue Skies

Tomorrow's the Day! Last Pre-Ride Update.

Well it is 2am on Friday morning! The ride for us is 1 day and 7 1/2 hours from now. I thought I'd give a last update.

We are presently both an informal team of 5 and a formal team of  5! lol Saturday morning at 9:30am 5 riders should be rolling away from our home to accomplish a 25 mile ride around Rohnert Park/Cotati, and separately there are 5 registered team members actively fundraising (https://secure2.convio.net/fara/site/TR/Events/General?team_id=4411&pg=team&fr_id=1633).

There are two routes we have mapped. Tomorrow we'll need to choose which one.
https://ridewithgps.com/routes/32798726 runs Snyder Lane to Petaluma Hill Rd and back to Todd Rd and around (25.2 miles), while
https://ridewithgps.com/routes/32801159 runs from SSU to the Smart Train bike path, North to Golf Course then further North to Todd Rd. and around (24.8 miles).

Oh, did I mention Saturday's chance of rain is about 75% right now? Yep. Yesterday it was 91% so it's lookin' better!

The organizer Kyle Bryant said he wanted us to do a "challenge" ride. On a personal note I'll be riding on my 75th BD using a completely unconditioned body and a 40 year old Schwinn road bike for 25 miles in the rain. I ask, should I pray for rain, Kyle?! lol It will be fun and I've been wet before. The pic to the right was part of the 2 1/2 days of rain of the 4 day Portland to Seattle Ride Ataxia. THAT was wet!

Team Blue Skies is at $3100 and the overall NorCal total sits at $73,105 right now! Whoo-Hoo! And there's still time to join in! Go to our link above!

One Week from The Day

One week from The Day and I'm rollin' now! My friend Dave Wait and I just did a whole

16 miles looking at a possible May 30th Rohnert Park route and to make sure my classic 1978 Schwinn (think T-bird) hasn't frozen up from last year's ride. 

Neither it nor I froze up so 2 or more of us will pedal away from my house May 30th at 9:30am on my 75th BD to synchronize with all the rest of the 23 milers! Since our RP Outback will not yet be available for outdoor dining we will support the Penngrove JavAmore with a sandwich socially-distanced lunch on us in my front driveway afterward while we watch Kyle's Noon presentations! Whoo-Hoo, a good morning that will be!

Remember YOU are invited to ride in this year's really local RA NorCal. Just give me a call, Email or text that you will be a my house by 9am so we can take off by 9:30am. You might preview JavAmore's online menu, https://www.javamorecafe.com/info/cafe_menu  . 

Our fundraising has had a good start springing up to $1,150 almost overnight! Goal is $2,000. Help keep it climbing:

   - hand me cash (or throw it from 6'. I'll sanitize it later), 

   - mail me a check (1921 Alice Dr., Penngrove, 94951) and made out to "FARA", or 

   - go to our Team Blue Skies web page where you can decide who to sponsor (Brianne, Paul, Richard or The Team). 

https://secure2.convio.net/fara/site/TR/Events/General?team_id=4411&pg=team&fr_id=1633  

Now that I've stretched and challenged my riding muscle groups I think I'll let them rest until next Saturday! Good strategy, eh?! ;-)

I hope to see you then!

It's the Pandemic RA NorCal Bike Ride Fundraiser for FA Research!

What a first 4 months of 2020 for our families, city, state, country and world!

• - Pandemic, COVID-19, Shelter-In-Place, Masks and Gloves plus Sanitizer galore! 
• "The Flu on Steroids" as it has been called, to date has killed 57,000 Americans out of 1 million (5.7%) of those who got this virus, and we're not nearly done! Susan's and my condolences to any of your family or friends you have lost.
• And who are especially at risk?! The old and infirm ("underlying medical conditions"), like many of us retired folk and certainly every Friedreich's Ataxian! That USA 5.7% overall death rate swells by a times 3 factor at least among this demographic.
• The ever-present danger to first responders and hospital staff! Today's heroes!
• And all those temporarily or permanently put out of work?! Businesses that won't recover. A sad day for the economy. Recession or depression, you pick the term. 
• And what will the New Normal look like? Even after this virus will we look at "the flu" differently as we newly realize many thousands die of it each year? 

So now I've painted this somber backdrop to FARA's 2020 NorCal Ride Ataxia fundraiser. But FA continues to kill young men and women far short of their expected and deserved lifespan independent of and in addition to COVID-19, and so we continue this battle in parallel with the virus battle. 

Brianne won't last much longer, and it will be the miracles of miracles if she is in time to get a drug that can pull her back from the brink. But she continues to want to win this FA fight for all the FA'ers coming after her. And so we will, all of us, for her and those coming after her!

I'm disappointed to share that, like many other public events, the May 30th NorCal event has been changed from a real-time get-together to a Virtual Challenge Month of May!

  - Each rider now will decide on a COVID-19-environment-appropriate challenge to complete. 

  - Mine will be to complete my originally planned 23 mile ride on or before my 75th birthday! (which would have been on the event day of May 30, 2020!) 

So what challenge strategy should I follow??

   - I could ride .7 miles every day for 30 days and finish with a rousing 2.7 mile ride on the 30th! No it wouldn't be very hard on my body, but "the challenge" would be to do that ride rain or shine (what rain??) every day for 30 days. The additional REAL challenge would be to get YOU to ride with me every day! Oh, I hear you. Yes that would bring us back again talking about Miracles. lol

   - Or I could ride once a week for 5 miles and make a strong finish in the last week of 8 miles! Mmm, 5 or 8 miles on a bike is not very much and managing to ride 4 times in the month of May isn't much of "a personal challenge" either. I think this option will be scrapped.

   - The third option is to do the whole 23 miles on or very close to my birthday! Either the 29th or 30th. I'm in such out-of-shape shape it would certainly be a challenge to finish in one ride! Let's do that! Yes, I did say "let's". The Rohnert Park/Cotati/Penngrove route I will ride offers several shorter mileage "return-to-home" options that we can discuss closer to the day of the ride. 

So that's it! A local 23 mile ride for me (or shorter for you) on the 29th or 30th of May with 6' spacing and masks as needed included. Let me know if you would like to ride with me. It would be great!

And of course this is a fundraiser for the cure and treatment of Friedreich's Ataxia so if you are in a lucky place in this economically strapped Springtime I would love for you to sponsor me, and/or if your will ride with me and want to gather sponsors for yourself that would be grand! We'll get you officially signed up with the Ride (no entry fee for this virtual event).

To sponsor me you can 

   - hand me cash (or throw it from 6'. I'll sanitize it later), 

   - mail me a check (1921 Alice Dr., Penngrove, 94951) and made out to "FARA", or 

   - go to our Team Blue Skies web page where you can decide who to sponsor (Brianne, Paul, Richard or The Team). 

https://secure2.convio.net/fara/site/TR/Events/General?team_id=4411&pg=team&fr_id=1633 

There are 4 weeks in May! Our team is at $0.00 now but we hope to make the goal of $2,000 by my birthday. Sponsor me instead of a present! I'm good with that! :-)

May you all stay safe during this time!

Paul

Tuesday morning and the weekend is already becoming a blur! :-) And my body is almost my own again. So I can and should roll out this blog.

Life if you let it is such an adventure! Last Tuesday another chapter in my life officially closed when I sold our 1988 34' Mallard motor home. 

- Ah the short memories of risking life and limb in that thing! lol The first time we took it out camping on the coast, after 20 minutes it would only go 20 - 30 mph. Plugged fuel filter. Then we took it out again after a rain to the coast and as the 18,000lb behemoth hurtled down one of the coastal hills at close to 55mph we saw the road at the bottom was flooded out. Too late to stop so I piled through the water like a blunt snow plow! Coming out the other side suddenly the exhaust noise was MUCH louder than before. As so it went until we parked it to become a storage unit for 12 years.

- This couple that bought it assured me it would be a simple thing for AAA to tow it to a repair facility. "Uh, what about the 15 year old tires, expired plates and it's not an emergency tow", I asked? "No problem with a big tip and plates from a Honda" was the answer. Ouch! My answer was once it is on the street in your name, not my problem.

- Several AAA scheduling attempts later it is Thursday afternoon and we need to be leaving for Winters in our camper to help with course marking and hall setup Friday morning. They said the tow truck is coming(!) so I pulled the MH out onto the street ready to be picked up and dragged away, and we left (at 6pm instead of 1pm). 

- During the next two days I got several depressed texts reporting on their non-progress and when we returned Saturday PM it stayed until about 6pm that evening when finally it left my life forever.

- A couple of neighbors were not happy but another 8 I polled didn't think it was a problem and suggested I "consider the source". I like those neighbors! :-)

So we arrived at the Lake Solano Campground and Susan asked, "which campsite?" and when I replied "upper number 40" she replied, "TURKEYS!", which brought to mind when I made the reservations we thought Susan would not be coming because her dad was sick and needed her. Oops. Turkeys are active and noisy well into the night. Susan is a very light sleeper. It's only 2 nights?

The next morning luckily we were able to sleep in and still rendezvous at 10:30 with the FARA team where we got the 1.5 mile walk and 13 mile course. Paul's second tactical error (after the turkeys) was to suggest walking the 1.5 mile walk placing signs and ground arrows along the way instead of using the truck. We don't walk much, Susan has a bit of a bad knee and I had sprung my knee a tad a few days ago falling part way out of the MH driver's door. 1.5 miles is not a long way but we were feeling it by the end. Then we drove out to the 13 mile course halfway point rest stop putting signs and arrows as appropriate along the way.

Then the team met for lunch and at 2pm tore into the hall setup finishing by 3:30pm. By the 7th or 8th year in the same venue you know where everything goes and it's quick.

That evening we had a potluck BBQ and were back at camp by 9pm (camp gates close at 9pm). 

Saturday at 7:30am found us mingling with all the special people that make up the FAmily. FA'ers, parents, sibs, relatives, friends, volunteers and professional like Dr. Gino Cortopassi from UC Davis who is working on Nrf2 channels in FA. 

J.D. to the left and I to the right of the big fellow

By 8:30am I'm as ready as I was going to be and feeling the least prepared in any of the rides. I told my ride partner for the morning J.D. Abouchar, dad in a recently diagnosed family, that as we approached the 13 mile halfway rest stop I would decide whether to wimp out and return, or decide I could repeat that stretch 3 more times. I did decide to continue and barely made it to the 23 mile halfway rest stop. Whew it felt good to dismount! 

of It's like old home week at the rest stop, the same wonderful volunteers have served here for most the years in this Winters' venue. My special sweetheart niece Ashley wanted to know WHY I haven't been talking to her for ages (I asked if her phone could still do outgoing calls. :-) ). I'll be upping my game to keep in touch with her. My B-I-L Richard appeared at my side with his biking and entertainment partner Bhavani explaining they had come up from SF too late to do the 52 mile course so they were lazing through the 23 mile course. LAZING THROUGH?! I changed the subject. lol The bustle and noise of the crowd was warm and friendly. 

J.D. and I continued on after 20 minutes or so and shortly caught up to triker Sean Baumstark, one of the four 2010 FARA RAAM riders (We had 3 of the 4 riding this year; Kyle, Sean and John, missing only Mike). J.D. and Sean hit it off immediately both being avid bikers. After a bit my body started telling me enough already, REST(!) so I told my two companions to keep on while I inspected the grove next to us. After a 10 minute break I continued on for a time and then had to give it up again for 10 minutes. Right after that I rejoined the main road back to the finish which was mostly a gentle downgrade so I was able to finish at a reasonable clip. At the finish I had to stand straddling the bike for 2 or 3 minutes waiting for my body to get used to this new unstressed position. I've never had to stop once let alone twice on this course and my body was telling me loud and clear something better change by next year. I think I'll listen. :-)

The Outback lunch and pie dessert was great as was Kyle's talk. He reported $81K in research donations - that's great!

Reata, a drug company with a drug in their phase III clinical trial reported their trial will be over around September and by the end of the year they will be able to state if they think their results with pass muster with the FDA. It will take most of 2020 to move the petition of approval through the FDA. I am hopeful one of the 5+ gene therapy companies will move one of their products into Phase I by the end of the year. Take a peek at our research status at http://www.curefa.org/pipeline .

The temperature was closing in on 88 by then so Susan and I went back to the chilly air conditioned trailer for a couple of hours before breaking camp and heading back home and my shift with Brianne at 10pm.

Today we are at $86K which makes the point that if you haven't financially supported me yet and would like to the fundraiser is still going. :-) Go to 

https://secure2.convio.net/fara/site/TR/Events/General?px=1001704&pg=personal&fr_id=1541 

or mail me a check made out to "FARA" to 1921 Alice Dr., Penngrove, CA 94951. 

That's it for another year! Thanks for reading along. Stay safe, stay healthy and remember this day will never reoccur in eternity. How did you spend it? :-)

Paul, Dad to Brianne, FA, 41

A year later, now 2019, a dollar shorter but this time posting before the Ride, not after! Kudos to me! lol

Each year brings its own adventure, challenges and ambiance so looking at this year so far it is the recent loss of my valued father-in-law Joe, an equally recent mis-adventure with my knee and the interesting weather forecast for this Saturday, day of the ride. 

Joe Turner's death at 95 will not affect my ride Saturday except perhaps during a few of those mind emptying times grunting with effort into the wind I might reflect on some of the laid-back quality times I had with him. Genial and friendly but with the ability to rise with some passion on a topic or two Joe and I enjoyed sharing humor and friendship. While it is OK he's gone to be with his childhood and lifelong sweetheart Margaret I'll still not like the void he's left in Susan's and my life. But then such is life.

About the same time of Joe's death I was out fooling with our somewhat derelict 34' motor home in preparation of selling it as a fixer-upper (as a vacation cabin or limited local RV it still has some potential, or so I suggest to potential buyers. ;-) ). Perched up on the edge of the driver's seat I was attempting a dismount when my left foot slipped off the saddle-sized metal stirrup foot step and my body plunged down to the arms-length of my grip on steering wheel and door strap, leaving my right leg in a 180 degree bend at the knee supporting way too much of my body weight. Good news was I didn't free fall 6' onto a disordered pile of wood but bad news was my knee did not enjoy the quick-force burden of my body on it. I did disengage myself only feeling the average amount of foolish and to my surprise aside from a little twingyness in the joint I thought I had once again, skated. But of course later as I levered myself up from the couch, surprise, a really sharp pain across the top edge of the patella suggested the connection tissue between patella and thigh muscle might have been just a tad overextended with my less than agile gymnastics move. Mmm. In the days since my knee has reminded me all is not forgiven yet. We'll see what 23 miles has to "say". 

The weather forecast this morning for Saturday suggests if I am wise and hurry I can beat not only the 91 degrees high for the day but also the 30% chance of rain, both occurring around Noon. The forecasted wind of 10mph should be my friend as through the hours of my rectangular routed map is seems to be trying to stay at my back. I'll take it! :-) But we'll see. Murphy's Laws may apply.

So tomorrow we roll the trailer once again to Lake Solano campgrounds, Friday we will help mark the courses and Saturday I'll do the ride while Susan SAG's and takes pictures, and then after lunch we will socialize for a short time at the post-ride micro-brewery  nearby before hooking up to return home in time for me to spend the night with Brianne. 

From the note I emailed to my supporters on 5/16,

---------------

To give you a bit of an update since last year:

-        Brianne’s quality of life has not changed and is almost as low as life can get I’m afraid, but she is stable and still in her apartment. She has few visitors but would love more! 760 Laguna Dr., Rohnert Park, phone 585-6112. Call to schedule a visit only please. The telephone is not her friend these days.

-        We are trying a mild untested treatment that came my way through FARA and FAPG connections. No trial approvals or big studies, just something that worked a bit for a Spanish dad of a FA’er. Not expecting much and not seeing much so far.

-        This should be a great watershed year for FA research! Follow with me on http://www.curefa.org/pipeline . Look for details below the graph.

o    RTA-408 will finish the Phase III trial late this year and have results and FDA approval decision early next year.

o   TAK-831 will complete their Phase II trial this year and we’ll know how strong the drug may be for FA. 65 enrollees.

o   Going into Phase II testing because of the positive results from Phase I!

§  MIN-102

§  Dimethyl fumarate

§  Resveratrol

§  Nicotinamide

o    5(!) gene therapy companies competing to get into phase I testing!

o   And FINALLY a phase I stem cell trial coming this year!

Every year I am amazed at the level of research activity for this little known orphan disorder! How can we have 11 treatment possibilities in clinical trial this year?? One because we’ve captured the imagination of the researchers through FARA’s work and two much of this work will impact ALS, Huntington’s, Parkinson’s, MS and even perhaps Alzheimer’s.

If you would like to continue to support me in pushing this research to its needed conclusion I invite you to

-        Go to https://secure2.convio.net/fara/site/TR/Events/General?px=1001704&pg=personal&fr_id=1541 for a credit card donation

Or

-        Send me a check made out to “FARA” and memo-noted “2019 RA NorCal – Paul Konanz. 1921 Alice Dr., Penngrove CA 94951

--------------------

You can find Brianne at  https://secure2.convio.net/fara/site/TR/Events/General?px=1002797&pg=personal&fr_id=1541 if you would like to support her Virtual Rider fundraising effort. 

I thank you all for "following" this blog each year and for being personally close to us! Many Hugs!