Sunday, June 5, 2016

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,









Wednesday, June 1, 2016

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.

Monday, April 25, 2016

I was just doing some Ride Ataxia NorCal housekeeping on their webpage and decided to click on the "Featured Video" at the bottom of the page.
http://fara.convio.net/site/TR?fr_id=1261&pg=entry
I am impressed! Not only is it an excellent professional product that is an easy watch of under 5 minutes but it actually captured the close up and personal nature of being at the ride! If you have a few minutes give it a watch!

Oh, in the last crowd scene on the steps Susan and I are on the far right. :-)

Sunday, April 24, 2016

It seems "serial thinking" is a characteristic I am picking up (couldn't be my years! lol) and I just saw I had not acknowledged all those wonderful and caring people that donated to FARA for Brianne and I last year and earlier years. Tsk-tsk! You are our angels! Without you FARA would not have been able to accomplish the wonderful progress they have! I thank you and thank you!

FARA is THE model to follow in the orphan/rare disorder/disease world. 9 clinical trials in-process is an amazing feat with more coming through Basic and Translational research behind those. And it goes back to the generosity and caring of all of you.

And these dollars have rippled and will continue to ripple indirectly into the other mitochondrial disorders like Parkinson's, Huntington's, ALS, and Alzheimer's. How leveraged has your generosity become! Kudo's to you all!


Here we are in later April 2016, about 6 weeks from this year's Ride Ataxia NorCal and yes, I have actually been preparing for the ride with Brianne! :-) Her broken leg has long since healed so we are looking forward to pedaling for a FA cure!

This year I have taken a different approach to my preparation: weight loss! On Valentine's Day I weighed myself, 200.3lbs! My dad bless his heart was a pretty big fella (cross country truck driver for many years) and I have always sworn I would not go over 200lbs. Well, there I was. So I cut out ice cream, second helpings, bread and alcohol. This morning I am down to 184.4lbs! 15.9lbs less to lug around the course! And I have been riding about once a week plus cutting, loading and stacking some cords of firewood. I should be ready for this year's wind and Brianne's rig's weight! I feel like Rocky in Russia! lol

FA research moves in fits and spurts it seems.
- Some of the most hopeful drug strategies have been stuck in the lab for the last year, notably HDACi (to "wake up" the bad genes), gene therapy (to put good new genes in) and  TAT-Frataxin (to carry supplies of the lacking protein Frataxin into the mitochondria). These are all incredibly complicated lines of research and are spending the needed time in translational studies to give the best assurance of safety and efficacy before being tried out on people.
- But at the same time some newcomers drug companies have ripped in with new possibilities, and possibilities that can go right into trials! RTA-408 and R001 are two examples.

Take a look at the FARA Research Pipeline graph and the individual reports below that! http://www.curefa.org/pipeline

Brianne is doing OK. She has avoided the flu and generally has been healthy. She continues to control her weight, a good thing for her and her caregivers when away from the house's ceiling track system. Right now she is focused on re-planting for this summer season outside the door of her apartment.

I would love to see some of you at Davis for this special day! Come on Friday and I'll get you into our pre-party at the USA bike museum in Davis! If you would like to help there are always jobs to do! Let me know!

My fundraising Ride Ataxia NorCal page is at
http://fara.convio.net/site/TR/Events/General?px=1001704&pg=personal&fr_id=1261
Prayers for research success, good thoughts about us making the course "one-more-time" and a donation if you are so minded are all gratefully accepted! Any problems Email me at pkonanz@comcast.net.

I'll try for another blog just before the gala affair! Susan and I will be camping West of Winters Thursday through Sunday (or Monday!) so my blog will be early in the week.