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!

It is Sunday PM, June 3, the day after the Ride Ataxia NorCal Research Fundraiser Bike Ride! My body feels strange, like it is asking me "What did you do to me again??". LOL  I'm not sure if it is riding the 23 miles (which wasn't really that bad), the increasing heat of the ride (which really wasn't that bad), the two days labor to help put The Ride together and take it apart, (which really wasn't that bad) or sitting mostly under shade in 98 degree heat at the post-event get together at the Berryessa Brewing Company (it really was that bad). Or perhaps it was the accumulation of all of that. :-) As always I response to my body with "Get over it!". It has worked so far.

After sleeping at Brianne's Wednesday night (4 nights a week these days) I got home at 10:30am to finish helping load the trailer and pull it out out the side yard. Each year our packing prep time gets shorter and our "at the moment" packing gets more immediate, and so we pulled out of the driveway around 3pm arriving at the Lake Solano Campground about 4:30pm. Lake Solano is a wide spot (reservoir) in Putah Creek formed by a diversionary dam just downstream from Lake Solano Campground.

In a change from previous years the Rupel family has sold their trailer and so there was no welcoming committee. The fundraiser this year is dedicated to their FA son Matt who died last Fall from infection complications. He was a wonderfully intelligent and humorous young man. Our campsite was a pull-through so in short time we were set up. We had a quiet evening next to Putah Creek with the appropriate number of creek-side bugs.

Friday morning found us in Winters at 9:30am set to lay out the courses.
- With plenty of volunteers we knocked that out by Noon and had lunch together at the Putah Creek Cafe catching up since last we met! The camaraderie of the group warms my heart.
- At 1:30pm we gained access to the community center hall and set up the tables, chairs and all the trappings needed for a successful ride.
- During that time I had fun with the center's coordinator, a 40-year veteran chef now apparently gliding toward retirement. I got squirted in the face by their hallway fountain and then noticed it never shut off completely. What?! She informed me she had called a number of times to city hall to no avail. So I told her to call with the information that a rabid environmentalist was there and threatening to do a picket with TV cameras on Monday if it was not fixed! lol Within a half hour a city plumber arrived. Ya gotta love the government.
- At 3:30pm Susan and I returned to the cool trailer for a respite. As we came in I stopped at the camp host to request an exception to the 9pm gate-closing promising we would be in by 9:30pm and would honk as we came by their rig.
- 6pm found us in Davis at the Woodstock Pizza four an informal before-the-ride get-together for all and sundry! It was so nice! FA'ers Kyle, Sean, Sam, Dylan, and Sienna were there with families and friends. We got to meet wonderful new people that are motivated by their friendships with FAmilies, neat!
- Slid back in camp at 9:15pm (yes!) with a toot to the camp hosts and after a little TV slumbered off at 11pm-ish with the clock set for 6:15am.
- I groggily awoke at 5am listening hazily to a "beep-whosh" sound! What IS that?? What is breaking or has broken in or around the trailer to explain that noise?? Is water flooding through the trailer?? Could it be Susan's Iphone? Susan whispers, "Peacock", of which they have quite a few in the campground. So we listened as they walked past the trailer giving voice to all that would listen. Susan later suggest it could have been a Canadian goose.
- We are at the community center about 7am. This year there is no official photographer so Susan went off to document the start of the 52 mile at 7:30 while I wash the truck's window for her SAG paint and figure out how to remove the new bike rack.
- The hour is a blur until ride-time! I'm riding with my sister-in-law who has been exercising to beat the band! And I haven't ridden for more than 6 months! I may eat her dust!
- The morning is beautiful and we should be back in the air conditioned center when the temperature hits 84, works for me! S-I-L Karen led and I kept up. The 23 miles flew by. On the third leg we were put into the Southbound wind at about 10mph and we weren't having as much fun. But for the last leg the wind was a crosswind and we had a net downhill ride. Sweet!
- At the center Karen went for her camera to help Susan and I tried to visit each of my FA "nieces and nephews". It wasn't a good day for Sean Mazeres and Ashley is fighting a bladder infection but I met Nina and checked in with others! Family and friends gathered together to help push forward for a cure!
- About 1pm-ish we started the tear-down finishing about 2pm. Off we went to the brewing company for about 2 hours pleased that we could sit with the Helms' family and their friends. Finally the sun shifted onto our backs and we gave it up. Back to the trailer where we ate leftovers and just did nothing. :-)
- This morning we got up at 9am and at 10am broke camp. It was already over 80. Gone by 11am and home at 12:30pm.

I will be thanking the sponsors of Brianne's Team Blue Skies in another document but let me repeat it here, Thank You all that have supported us and all the 15,000+ FA'ers around the world! You are making the research possible that will release these children and adults from the grip of FA! Thus far this year's ride gained $93K for FA research ($98K by Tuesday) including almost $2.1K from Team Blue Skies! This money might support 3 grants to researchers exploring new FA treatment possibilities giving them the opportunity to prove their hypothesis sufficiently to interest a drug company (with the deep pockets necessary to carry on the research).

And the fundraiser is not over! :-) Go to the Team Blue Skies webpage at  http://fara.convio.net/site/TR/Events/General?team_id=3974&pg=team&fr_id=1472 if you are so moved.

Thanks for joining me through my blog this year! See you next year!

Here I am again! Twice in a year! lol Hopefully I will not get distracted and will give you my post-2018 report in June. I was going through my Drafts folder yesterday and found my 2017 post-ride Email, unsent! Lordy-Lordy way too much going on in my retirement! :-)

All is ready for The Ride in 9 days. The FARA folks are wonderfully organized and participants will feel well supported. We are about 56% of the FARA goal of $100K. Susan and I are ready to help Friday and Saturday to support the logistics and grunt-work to make the event a success for the folks who attend. We'll be camping at the Lake Solano Campground West of Winters where we'll see the Rupel family for the first time since their FA son Matt's death last Fall. What an amazing young man and such a loss! Sigh FA doesn't care.

Speaking of ready I am as un-ready as in any year so far! lol But I can make it 23 miles, if the wind doesn't kick up to 22mph as it did some years ago! Have faith!

I'm off to speak an evening of quality time with Brianne in 1/2 an hour!

Ta-Ta

My last blob was in May 2016 so obviously last year I was oblivious to the opportunity of updating my blog. Brianne was not able to attend last year so I did the 23 mile route and had the pleasure of riding most of the way with FA'er Sean Baumstark. Sean was just a couple of weeks into his first recumbent trike so we made it a social ride (which we are both good at!), breaking no land speed record but did some good sharing.

This year for the 2018 FARA Ride Ataxia NorCal bike fundraiser Brianne again cannot make it. At the beginning of the year she was hit by a bad flu, contracted pneumonia and is still pulling out of that funk. She would love your prayers, good thoughts and even a visit if you are in the area.

I with wife Susan do plan on being there! I will again do the 23 mile route while Susan will be on the road watching after the 12 mile route riders as she has each year. This is Year 9 and we are as excited as on Year 1! Over these years the event has moved from Folsom to Davis and now to Winters but the focus is the same, bring multiple treatments to FA patients like Brianne and Sean. This year's ride is dedicated to FA'er Matt Rupel, an amazing young man who passed this last year. He wasn't able to wait for the treatment. See ride details here, http://fara.convio.net/site/TR?fr_id=1472&pg=entry  .

We ARE getting closer to treatments. The MOXIE trials should get a FDA decision in a couple of years, the HDACi is finally out of the lab and moving toward the FDA by the end of the year, there are 4 or 5 gene therapy drug companies surging forward and 2 groups are looking at bone marrow stem cell treatments in the near future (I thought that was still 10+ years out). Go to http://www.curefa.org/pipeline for more details.

So join us for the first time or continue with us my fine and faithful friends from these years past! Sponsor me, Brianne or our Team Blue Skies by going to
http://fara.convio.net/site/TR/Events/General?pg=pfind&fr_id=1472, mail me a check made out to "FARA" (not me) (1921 Alice Dr., Penngrove, CA 94951), or PayPal works too.

We can make a difference for some 15,000 FA'ers in the world, and for Brianne.

Best regards, from Brianne, Susan, Richard and I, members of Team Blue Skies!

Paul

It is Sunday PM of 5/6/16 and we are back home! What can I say? IT WAS HOT! lol

We got to the Solano County Park at Noon on Friday and just dropped the trailer in the campsite so we would not be late to help set up tables and chairs at the Winters Community Center in of course, Winters. The temperature hit 103 degrees that afternoon!

Turns out Winters is only 5 minutes from the campground so since we were early we stopped at a Garage Sale! Susan snagged a two-level garden work bench for $3 and I picked up a street-legal propane tank for $2. In spite of the heat the weekend has started out great!

The community center folks were friendly and the layout was good so in half an hour we established seated eating for 200+. So then we went back to the trailer (A/C was on) and relaxed until time to help set up for the Bicycling Hall of Fame reception at 5:15pm. The evening was nice with many re-connections to the FAmily and guiding riders to the registration area to pick up their packets. Cleanup was fast and we found ourselves back at camp by 8:30pm enjoying conversation within feet of the Putah Creek with the Rupeloton Team. Retired to our rolling bungalow thinking of the next day forecast of 97 with less than a 5mph wind (yess!!).

Ride Ataxia biking events are fairly unique to the orphan/rare disease community and aren't very comparable to the everyday biking event. Yes those folks get to know one another but there is an intimacy and family-ness to RA NorCal that I don't think one can match in those other bike events. Saturday morning found us up at 7am and ready to go easily by 8am. Susan would SAG the 10 mile route and Brianne and I would ride it but neither set of us would be on the road until 10am. So we showed up at 8:30, Susan went off to secure her supplies and SAG instructions while I de-trucked and assembled my (heavy) 27-speed road-altered mountain bike and the wheelchair/trailer combo. Brianne was expected by 9:30am and I was ready for her by 9:15am. At 9:35am I texted "ETA?" and received the reply "tough morning, 15 - 20 minutes". A few minutes later I received the text "no go". Somehow Brianne's morning had gone sideways and she wouldn't make the ride.

An important note here is that for all of us "temporarily able bodied" folks it is difficult to appreciate the fine and delicate balance that exists between a "good" day and a "bad" day for disabled folk. Something in the alignment of the sun, moon and stars trembled for Brianne between Friday evening and Saturday morning to cause her to cancel an experience she had prepared and fundraised for for months.

Well, things happen and we move on. About this time Felicia finds me (precious young woman that she is) and asks am I ready 'cause its time? "Sure" says I so I ride around the building, unhook the wheelchair/trailer and join the 10 mile group. Felicia gives us all the cautions and safety tips (why did she look at me all through her spiel??) and sent us on our way.

The day is "warm", 80 - 85 degrees already but I can still feel a little coolness in the wind, thankfully. In the first half mile I remember and am reminded of the pluses and minuses of my bike. I like the 27 speeds that give me shifting sensitivity as the road rises and the custom handlebar risers that allow me to straighten up to stretch my back (this is an event and not a race after all) but I can feel the drag of the extra bike weight and the fatter tires than my early '80's Schwinn road bike. Ah, the ying and yang of life.

I begin thinking if I should do the 23 mile ride instead since Brianne is not a weight factor any more but by the time I am at mile 3 or 4 I can feel the sucking-out effect of the heat (there is no more "cool" in the wind) on my energy and I could imagine the backside of the 23 mile route in an hour with the increasing heat and decided no, not this year in this heart (last year Brianne broke her leg and I did do the 23 mile route.).

I arrived at the rest stop with Susan taking pictures. Izzy and her mom Zoe were on hand greeting the riders plus Diane Bryant (Kyle's mom) plus others. After a half an hour I launched back toward home base. I believe I average about 17mph which for me is pretty good.




Back at the community center Outback Restaurant had prepared a wonderful lunch of ribs, chicken and veggies! Also present were two FA drug companies (Horizon and Reata) both of which have drugs in clinical trials so I was able to talk to both of them about their research. After helping to clean up we went back to the trailer and hid from the heat. It was hot! We had considered staying an extra day but after seeing the forecast, 77 for RP and 93 for the davis area, it was no contest and we were home at Noon today.

Thank you to all the sponsors of both myself and Brianne! RA NorCal has raised $84K so far and Brianne's Team Blue Skies has exceeded its goal of $3,000 and more is expected! This will help keep the momentum going! Remember you can go to
http://www.curefa.org/pipeline to see the FA research status
and our page http://fara.convio.net/site/TR/Events/General?team_id=2885&pg=team&fr_id=1261 for updates.

Best regards,

Good morning! It is 3 days from RA NorCal!! And of course we are doing everything except getting ready for the ride! lol We are having company over next week so the house is getting a once-over cleaning and projects of course have radiated out from there. So far I've got a sliced finger and a pulled groin muscle, neither of which should bother my Saturday once I'm pedaling.

Wonderful news! Team Blue Skies has exceeded its goal!! Thank you so very much to all the supporters!!! You can see this at
http://fara.convio.net/site/TR/Events/General?team_id=2885&pg=team&fr_id=1261
which is showing 99% but there are several checks enroute to FARA to put us over.

This evening I'll pull out the mountain bike (27 gears!) and the trailer/wheelchair (still attached from last year) to get them aired and lubed for Saturday. All the straps to keep Brianne safe and comfortable should be there so poof, ready (I hope)! If there's time we'll pull out the travel trailer as well to start throwing a few things in it. We'll camp a few miles West of Winters next to a quiet river Friday, Saturday and maybe Sunday.

We are looking forward to Saturday! Another posting to you next week.