Wednesday, April 25, 2012

Moving Up

Both Cammie and Ben got their rank testing notifications today.  They'll be testing for their yellow belt this Saturday.  It was touch-and-go with Ben...I asked Sensei to give him a second chance due his erratic behavior, and she did providing he did well at today's lesson.  He behaved quite well so he will be able to test after all.  Sensei and Ben are learning to work with each other--she understands that he responds better with lots of positive reinforcement (although he hates the word "awesome") and not giving him options like "well, you can go sit in the corner until you're ready to learn" because he'll just happily sit in the corner for the whole lesson.

Emma's in drama club--they're doing the Beauty & the Beast play, which will be performed on May 17 & 18 at 7 pm.  Let me know if you want to come and see it and we'll have dinner afterwards.  I know she'd love to have family and friends come to see it.

Our trip to the hospital yesterday for clinic delivered some good news--Ben has gained 4 lbs.!  That's 10% of his body weight!  He's back on the charts again and has some nice chubby cheeks again.  He's also got some increased energy, as evidenced below...this was after about 45 minutes of swinging in the sunshine the other day.

The rest of the week has been eaten up by appointments, scouts, and I'm hoping for a nice quiet day tomorrow.  Hoping.


Sunday, April 22, 2012

Girl Scouts and Beer

The girl scout meeting went pretty well last Tuesday, and I discovered that the reason that one girl dropped out very recently is because she thought it was "boring".  OK, that's your opinion.  However, it's hard not to take it personally.  We have plans to do a bunch more badges in the next few months and may try to do more from the Retro book.  Then there are two more girls, maybe three, that will have to drop out next year due to sports schedules and other interests, and is very typical for this age group.  I totally understand that--after my schedule this year, I'm considering throwing in the towel myself.  But with three more gone, we'd be down to 7.  That's a pretty good size group for outings and for doing more detailed projects.  I'd like to be able to work with them one-on-one doing sewing and weaving projects, but also be able to go out on hikes and do fun things in the area without requiring more than two cars.

Yesterday was the 11th annual Troll Stroll.  Kelly and I arrived about noon to register in the VIP line, which included a couple of extras like a pair of sunglasses and a bonus drink at one of the pubs.  I'm not sure it's worth the extra $20, but the proceeds go to a good cause.  We met up with Daniel, Lauren, Brandon, Casey, and Shannon.  Later, we ran into Achal, and near the end, Justin and Katie joined us as well.  We started hopping to get the 7 oz. sample beers at each venue.  We ran into Robert, the father of a CF daughter who is now in college.  He said that he had already had a number of beers and could no longer feel his feet.  Awesome...and yes, the maps they handed out had the phone numbers of local cab companies on it for a safe ride home.

A few lessons learned:

  • Eat before the stroll begins--it started at 12:30 but a lot of these places had limited bar food available.  At our first stop, we knew we were going to be there a while waiting for the rest of the group to arrive, but they had only pork products on the menu.  I ended up getting hot dogs from 7-11 .  Ew.  I would have been happy with beef nachos, but they didn't have them on the weekend happy hour menu...just the week day menu.  Huh??
  • Meet earlier to get food and get an earlier start so we can get to all the participating venues--we spent too much time at the first one and got caught in the crowd later.  Getting to all the venues earlier means we could spend more time at the favorite bar at the end.  
  • We should also determine where we're going to have dinner afterwards.  We had dinner at a sushi place that ended up upsetting my stomach, which is still sensitive today.  I'm thinking some of the fish was not up to snuff.  Or sniff...whatever.  Having pepperjack cheese on my sandwich for lunch was probably not a bright idea, either.
  • It was recommended that we wear costumes next year as a few teams did.  There was a nerd team and a bunch of wizards (I think we were far nerdier than the nerds there--a bunch of engineers, soccer hooligans and history geeks).  Two of the girls wanted to wear hot pink, and Achel suggested if he's going to wear hot pink, he might as well go all out and wear a tutu.  I think maybe we could do something a little less tutu-ish and maybe go with a Hawaiian theme; straw hats, island wear, grass skirts.  Coconut bras optional.
I sent a note to the Troll Stroll organizers (at least I hope it was them) suggesting that they make a couple changes.  One is to offer more easy food items at the venues so we're not drinking on an empty stomach.  A few places had some food, but it was mostly french fries.  I thought maybe simple turkey wraps or nachos would be nice.  Also, one of the venues was very small and there was a couple different soccer games being played that day--Real Madrid vs. Barcelona and another big international game.  The George & Dragon was PACKED so the Troll Strollers were having a heck of a time getting anywhere near it.  We stood in line for about 10 minutes without moving and we, along with several others, abandoned the place for shorter lines.  I suggested that they turn the parking lot into a beer garden to allow more people in. I also suggested that they offer a non-beer drink like cider on the menu if the venue had it on tap.  Only one pub offered cider and sadly, we didn't get to that one; at least two other pubs had it on tap but it wasn't part of the stroll.  A few of the venues ran out of one kind of beer or another...that's poor planning on the pubs' one was down to offering ONLY Pabst.  Woo.

This morning, to celebrate Earth Day, Emma and I went out for our shopping adventures driving all over the place.  Nothing in the world like going against the grain of society by using every appliance in the house, driving more than 50 miles in the gas-guzzling beast, and running water for a few hours to wash our cars.  I stopped by JoAnn's where, as one of the first 100 shoppers, I got a free re-usable shopping bag.  Now I really feel like I'm doing my part.  Never mind that I already have a dozen or more re-usable bags that I never remember to put in my car.  I got some scrapbooking supplies for the girl scouts for Tuesday's meeting and some flannel to make Ben some more pajama pants, which he decided that he really likes.  I'm washing the flannel now so that it won't shrink out of sight after making the pants...wash first, then cut.  Now, of course, he wants pajama shirts.  I may go to a second hand store and pick up a bunch of t-shirts for him.  If I can find plain colors, maybe I can use fabric paint to decorate them with night-time images...moons, owls, glowing eyes...

It's official...I'm turning into a soccer hooligan...Seattle Style. :) That means, of course, that we cheer them on at the game, make crass remarks about the opponents, then go drinking with them afterwards.  I picked up this t-shirt super-cheap (compared to the costs at the sport shops, this was about half price) and I will wear it to the next game, which I hope will be the May 5th game.  I used to really like baseball, but I rarely had the opportunity to go to professional games.  Between cost and distance, we didn't have the ability to go more than twice in 20 years.  With us living so much closer to Seattle and having better income, Kelly got his season ticket to the game and we're looking into the possibility of getting a second ticket for me.  If I can't go, we can always sell that ticket so it won't go to waste.  

I've been working on the weaving off and on.  I'm not particularly inspired by this pattern--there's something odd about it--I think it just needs a couple more cards on each side to finish the pattern off or eliminate the black threads.  They hardly show up at all.

I ended the day by loading up about 12 bags of stuff to go to the second hand store.  Baby toys & books, outgrown clothes, and other cast-offs were unloaded in exchange for a single slip of paper.  That certainly takes up a lot less space.  The second bonus is that I had to clean out my car before loading it up, so now my car is clean inside and out.  Not vacuumed...not sterile enough to eat off the floor...but all the jackets and socks and underwear (oh my!  No idea how that got in there) have been hauled out to the laundry room.  I found two school sweaters in we have an even half-dozen available for the kids to wear.  Hopefully I won't have to buy any more.  Ever.

I have been sorting through boxes and bags of scraps and trimming them into mostly 5" and 2 1/2" squares for scrap quilts.  I have plans to make some scrap quilts with them and have been doodling down some designs that take 5" and 2 1/2" squares to make the patterns.  I also found a bunch of blocks in a box as I was sorting and remembered that I had started putting the quilt top together and realized that I had assembled the blocks wrong.  I was so mad at myself that I pitched it into the box and forgot about it for a couple of years.  This is how I wanted it to look, but somehow I mixed up the blocks and sewed them into pinwheel 4-patches then realized my mistake when I had a couple left over...  I frogged the seams and used this photo that I serendipitously had taken to arrange them in the layout I had wanted and began sewing them into rows.  I have three of the five rows done now and hope I can get a few minutes tomorrow to finish sewing the top together.  I have no idea what I'll do with it after that since I don't have any more of this fabric--this was a jelly roll that I had gotten somewhere...probably a Moda Christmas collection.  I should be able to find more of it, but I don't know what color to choose to finish it.  Red?  Green?  Or maybe the blue floral?  Dunno.

Long day tomorrow and a longer day on day.  Ta.


Monday, April 16, 2012


I keep thinking that it's almost the end of the month, and then I realize that it's actually just the middle of the month.  Tax day.  Then I realize that it must just *SEEM* like the end of something.

Today was a crazy day--woke up early and took the girls to school, then drove down to see a neuropsychologist to discuss Ben's ongoing issues with school.  He was happy to get another day off school, but wary about out trip to a "doctor".  Then I told him that this doctor doesn't have any needles.  Much better.

The appointment was set to last for up to three hours, and of course, I had to park on the city streets in Seattle where the meters only go up to 2 hours, so at the 2 hour mark, I went out and fed another small fortune into the meter for another 90 minutes, only to find that we were released within 25 minutes.  Ah well.

Returned home and had lunch with Mr. B, then ice cream (for him), the bank, and finally the school to pick up the girls.  Then I spent 2 1/2 hours at the dojo between 3:30 and 8:00 pm, driving back and forth to pick up and drop off, cooked dinner and then FINALLY got to follow the doctor's orders and sit down, take some Advil and put my foot up with some ice.  So much for following doctor's orders.

Tomorrow is Girl Scouts, so it'll be interesting.  I think I'm going to teach them about Flag ceremonies and honoring the flag.  Oh, and maybe they'll have actually done the "homework" I assigned them on women in history.  I wrote down a bunch of names of inspiring women in history and asked them to look up who they were and why they were famous.  Some of the names included Rosa Parks, Mother Theresa, Marie Curie, etc.  Time will tell if I will actually continue being leader next year.  I'm feeling pretty wiped out lately.

We went to the CF family education day last Saturday (wearing our Sounders shirts!) and I met the mom of the girl who was across the hall from Ben at the hospital.  Her daughter is home now and is gaining a bit of weight again (yey!) and she was happy to have met me, too.  Her daughter "K" was wondering about how he was doing and if he'd gone home.  We were thrilled to give a glowing report of his health and improvement.  He's gained about three pounds since he got home and started his night feeds.  YEY!!

Time for bed.  More tomorrow.

Saturday, April 14, 2012

What Is CF?

Wehe dem Kind, das beim Kuß auf die Stirn salzig schmekt, er ist verhext und muss bald sterbe.
"Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon must die."

I've had several people (OK, just about everyone) ask what Cystic Fibrosis is and how it affects the body.  Interestingly, CF is the most common severe genetic disease in the Caucasian population, and can be found in other forms (although rarely) in other races around the world (except Japan, where it's almost unheard of).

The main reason for illness is pancreatic insufficiency (the pancreas is not supplying enough enzymes to break down food, especially fats and proteins, and therefore not breaking down and absorbing vitamins A, D, E, and K), and the basic defect of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR); this is what makes several organs not function properly.  Although some emphasis is focused on nutrition and enzymes, the focus of the doctors is on the lungs.

With the defect in the CFTR, the lungs slowly fill with thick, sticky mucus.  The mucus is there naturally to catch fuzz and lint that we breathe in.  Under that layer of mucus is water (periciliary liquid), and in the water is a lot of little hairs (cilia) that sway and wave, like seaweed in the ocean, moving the layer of mucus along and out of the body.  In CF patients, the layer of water is decreased, causing the cilia to get gummed up in the mucus and not sway properly to get rid of the mucus.  This causes more cough, which over time can irritate the lung lining causing swelling, and the mucus becomes a breeding ground for infection.  The infection brings on white blood cells, and pretty soon you have a really crowded hallway gummed up with stuff.  If it's not cleared out, it becomes clogged, hardens, scarred and permanently plugged.

Like other illnesses, there are several varieties of the disease--in this case, there are six types (there are actually dozens of mutations, but each of them fall into one of these six categories).  I often think of these in terms of a manufacturing plant, because the cells are, in fact, trying to manufacture chloride ions to deliver from the cell to the outside of the organ, which could be the lungs, the intestines, the liver, the pancreas, or the skin.  So imagine the cell is the manufacturing plant and they're trying to build the chloride.

Type I:  In this plant, you have the Premature Termination Mutations.  Here the workers are trying to build the thing, but they're missing a page (or 10) of the directions.  They have all the parts to put it together and no idea how to do it.  They scrap the project and open the next box, hoping to find a good set of the instructions only to find that it's exactly the same, box after box.  The chloride doesn't make it to the cell surface.  This mutation has a drug being developed, and is now in Phase III study, called Ataluren (or PTC124).

Type II:  This is our battlefield here--the most common of CF mutations, covering more than 85% of patients, is called Delta F508 mutation and has the chloride ions built into long strands and needs to be folded into an accordion-like shape and be sent on to the next phase.  Unfortunately, it doesn't fold properly.  Using our factory model, the materials from step 1 in the manufacturing has been created nicely and this group receives it and has to package up the product for the customer.  Unfortunately, these guys went on a weekend-long bender and it's Monday morning, so they're having a heck of a time folding the thing to fit into the package.  They keep doing it wrong and the Quality Assurance guys pitch them out into the burn barrel.  The drugs being tested now for this is VX-809 and VX-661, called a CFTR corrector.  This is only half of the puzzle that is needed to normalize cell function; the other half is from the already approved VX-770, also called Kalydeco (kal-eye-deh-coh), a Potentiator.

Type III:  This is the Activation/Channel Mutations and requires a Potentiator drug to cause the body to function properly.  This is caused by the chloride channels not opening properly; in our factory model, it's the shipping department being unable to open the overhead doors to get the product out to the trucks.  Some buffoon doesn't realize that the doors are locked or that the spring is broken.  The drug already developed for this is the VX-770 (Kalydeco).  This only serves to help about 4% of the CF population, which amounts to approximately 1200 people in the US, and 2500 people worldwide.  Not very many, but when it's combined with the 85% of patients who have the F508 mutation, it will jump to some 30,000 in the US, and 60,000 worldwide.

Type IV:  Another delivery issue, this one I *think* is where the door will open, but the incorrectly folded chloride ion can't fit through it properly.  I don't know what's going on with QA that they let these mis-folded pieces get by, or if the doors are unusually small, but somehow they still can't get the product on the truck.

Type V:  This is another kind of stop mutation where it looks like the directions are flawed and the factory workers make two pieces and no idea how to join them together.  It's quite rare, so I can't find any information on it, although if you want to wade through heavy medical journals, it's called a "Splice Mutation".  Good luck.

Type VI:  classified as "Turnover mutations", this doesn't have anything written online that I can find.  Must be really rare, but it appears that it's a problem with the bay doors again.  Maybe they forgot to install them at the factory...This is another job for Potentiator Man!

Still an orphan disease by any standard, it will change the lives of thousands of individuals and ease the burden of care for the families of the children and adults who suffer.

What is burden of care?  Let me run down my day.
7 a.m.:  wake up, go into Ben's room with a large syringe full of water to wash out the feeding tube he wears at night.  Tell him to get dressed while I take the pump apart and throw away the feed bag, take the ice pack out and run it down stairs to the freezer.  I run into my bedroom to get dressed.
7:15:  tell Ben to get dressed again and start threatening him.  Emma starts her breathing treatments.
7:20:  Ben finally gets downstairs and I administer the "Red Medicine" (albuterol).  
7:22:  Set up Ben's inhaled 7% saline.  Start nebulizer.  Then I start making his breakfast.
7:30:  Give Ben breakfast and 3 enzymes and tell him to eat quickly.  Emma makes lunches
7:45:  Tell Ben to eat quicker.  Go looking for wallet, keys, phone and my own shoes.
7:50:  Tell Ben to leave breakfast and go get his socks and shoes on.  Make tea for the road.
7:55:  Tell Ben to get his socks and shoes on again, and search for his backpack.
7:58:  Grab backpack, socks, shoes, coat and Ben and throw them all in the car along with the acapella and Flovent inhaler.  He does these on his way to school.
(Sometimes I'm more with it and I monitor him while he gets dressed, so he's downstairs by 7:05 rather than 7:20...even then, there's a lot of dawdling involved.)

At 7 pm, we do all this again:
Albuterol, saline, acapella, another medicine called Pulmozyme, Flovent and another new one, Flonase.

Soon we'll get to add another 20 minute treatment with his vest, which we will be able to combine with the saline and/or acapella treatment.  Still, in all, we spend 90 minutes a day on his treatments.  Every day.  That's a fairly typical regimen--some people spend two to three HOURS a day doing treatments.  That, my friends, is the burden of care.

We're doing a fundraiser next weekend in Fremont called the Troll Stroll.  All proceeds raised will go to the Cystic Fibrosis Foundation to fund research to find a cure, which is how Kalydeco was discovered.  Hopefully we'll be able to find Kaly's sister....I wonder what her name will be?

Sunday, April 8, 2012

Happy Easter!

The weekend has been spent in the delightful company of Heide and her family (three of them are in this photo--the two blondes and the brunette in the center back).  They, along with Kelly & Emma, had plans to go to Sakura-con, a Japanese animation convention in Seattle.  Kelly and Emma went down to sign in at the pre-registered line on Saturday morning, which was blissfully shorter than the un-registered line on Friday, where Heide and family spent five hours waiting in line to register.  This was due to the painfully slow data entry system that was garnering seemingly endless amounts of information for each attendee, printing out badges, and the cut-off of pre-registration two weeks prior to the event.  There has *got* to be a better way...and knowing one of the organizers, maybe I can make a suggestion.  If pre-payment is cut off, they should at least allow people to continue to make reservations online and pay upon arrival.  That would significantly speed up the process.  Name badges should have been "Hello my name is..." and a black magic marker.

Emma and I went shopping at the local costume store (aka Goodwill) and found among its treasures a linen shirt and silk vest.  The shirt was marked at a whopping $5 (I think), and the vest, still new with tags, marked down to $6.  The thing originally cost $58 at a local swanky department store (which I won't mention, but it rhymes with "bored some"), which then spent some time incubating in some rich lady's closet until it was released to the donation bin to find a new life as a costume piece.  Score!  The necktie is just a length of twill tape from my costume ribbons bin, and the sunglasses she already owned.  Likewise, the skirt and tights she wore were already in her collection, although I may have to invest in some new shoes for her--those ones weren't comfortable for a long day of standing and walking.  Here's Emma's close-up photo...

This is the character she was dressing up as...she's called "Shizu-chan".  No idea what her story is, but it appears she's fairly strong.  The smoking this is not appealing to her (for a number of reasons, thankfully).  I must ask her the story about the Do Not Enter sign...was it something she ripped out of the ground, or is it something she found?  And is she a waitress at the local casino?  I dunno.

And here is her complete costume... I suggested that we also (at some point) put some kind of ties at the back to accentuate her waist more and make it more fitted to her.  She agreed that it would be a good idea, but we didn't have time to take care of it before the event.

Steve BlumEmma waited in line for some two hours to see this guy and get his autograph.  What?!  Don't know who he is?  Me, neither.  Steve Blum is his name, and he does voices for cartoons and games.  Kelly said he was a really nice guy, very friendly and made lots of conversation and jokes with the people coming to see him.  Most of them said things like, "I've been following your work all my life," to which he said, "'s like I've raised you I'm your father..."  One of the fans got the autograph he waited for and said, "Thanks, Dad."  He replied, "You're welcome, son!"

Heide was there at the convention waiting in line on Friday, but opted not to return for the weekend, but to hang out with me, Cammie and Ben for the day.  All that waiting is really hard on the body, especially if you have pre-existing medical conditions which can cause days or weeks of recovery.  Even though they had the much shorter pre-reg line, Kelly's foot is going to ache for some time, too.  A3, who is autistic, tolerated the wait for a while, but like most guests, grew weary and ended up lying down on the floor and took a nap.  After being barked at by the security folks, a man standing behind them defended them, and they were moved to a shorter "handicapped" line.  The wait was still five hours long...waaaaay too long for the amount spent on a weekend.  Seriously--it's a three day event, but you spend half of one day standing in line just to check in.  Ridiculous.

We went to the Boy Scout Pinewood Derby Race--district championship.  He won at his Pack level, so this was the winner's circle races for the district.  After six races, and all the times compiled, he placed 8th out of 33 racers.  He was pretty proud of his placing, or at least didn't appear disappointed...or maybe he was just excited about going out for sushi for lunch to celebrate.  His appetite certainly picked up--he polished off 10 plates of sushi from the conveyor belt.

Heide and I went out for dinner at the local pizza/pub/restaurant.  We ate in the bar, eating bar food (steak flatbread pizza and nachos), and watched the second half of the Sounders game.  They tied 0-0...I think I was the only one in there in my Sounders t-shirt, though...a little unusual for game day, although it was an away game.  I guess I'm a bigger fan than they are.  I wasn't sure if they were actually watching the game, though--they seemed rather reserved and there was a baseball game on another one of the TVs.  It wasn't until near the end of the game when Burch (#8) nearly made a goal that the room erupted in sound that I realized that we were all there to watch the same game.  Except maybe Heide, who politely tolerated my interest...or maybe she enjoyed it too.  (I'll hope for the latter.)

On Sunday morning, while my kids were hustling around looking for eggs and baskets, Heide and family packed up and headed back home.  We said our goodbyes, and I went back to bed for an hour or so while Kelly took Emma back to the con.  The weather was beautiful, but Cammie woke up with a fever.  Poor kiddo.  A little Tylenol took the fever down and she spent a relaxing day playing in her room.  I went to the store and got a small turkey and made a mini feast for dinner.

In all, a great weekend!  More posts on arts next time.