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

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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!