SMA CANDLES

We are so blessed to have wonderful family and friends that support us in our fight against SMA.  On August 10th, there was nation-wide candle lighting for all families affected by SMA.  We asked anyone that did this in Benson's honor to send us pictures.

from Grandma and Grampa Purdy, the Hales, and the Croslands

from the Lyms

from the Doughertys

from Jared and Michelle Aller in California

from Natalie Stockdale Pakiz

Thanks to everyone for everything.  Here are our pictures. 

 

Heaven Help Me

Dramatic, I know.  But seriously I feel like that is my plea every night lately.  Just help.  Help me with all of it.  But, really, I am so blessed and things are going well.  This past week has been more drama than I care to re-live anytime soon.  But, we'll get to that soon.  Let's back up a bit.  A few Saturdays ago, we went to look at a van.  We had been looking online for awhile (like a year?) but this was the first in-person visit.  It ended in a purchase with a deal that Jon and I both feel was pretty great.  The necessary pic to celebrate such a savings damaging purchase.  Immediately after purchasing this van, we drove to St Louis.  Well, on Monday anyway.  We went for Benson's appointment with Dr Connolly, a neurologist.  The trip was supposed to be more play than it turned out being, but it was still fun to have Jon home for a week (it was his vacation week).

 Brigham is such a funny kid.  I feel like I should update his antics of late.  He loves tools.  Jon's tools particularly, but really anything he should not play with he is enthralled with.  He likes to sing "Ashes ashes" and turn in circles.  He can say a few words, and has amazing baby jabber.  He has a few phrases too.  He knows some body parts and lots of animals.  His understanding has probably increased the most.  He suddenly understands what we ask him to do.  It's fabulous!  He has a current shoe obsession.  If he isn't wearing shoes, he will try every shoe on that he sees.  If he can't get the shoe on, he will scream his frustrated scream.  It has a very distinct sound.  Brigham typically eats three breakfasts every morning.  I feed him breakfast shortly after he wakes up.  He's usually the first up (aside from Jon who is gone by 6).  Then, when Vanessa and Benson eat, he'll climb up on the table and beg for more food.  Sometimes it isn't so much of a beg and more of a take.  If I eat, he demands to have some as well.  He's basically a tank in the morning. 

 Our little lady is growing so quickly I can hardly stand it.  She started preschool this week.  She is in Miss Phelps class at the Early Childhood Special Education Program.  She is in an integrated classroom with kids who have special needs.  I signed her up for this program feeling it was not about the academics but about the life lessons of learning to get along and be empathetic with children who are different and sometimes difficult to be around.  I'm finding I am having a hard time being ok with her NOT learning all her letters and numbers.  Basically, I think she should be in college or something.  It's my problem, not hers.  It just feels weird and I have slight mom guilt for sending her to a program that only actually gives instruction one of the three hours they are there.  The rest is social time, with people besides her family.  Sigh.  Vanessa has six in her class, though it can hold up to 12 and will fill as the year goes on.  Children can't be in the program until they are 3.  Right now, she is the only girl.  She seems ok with it, but it is the first thing she told me about her class.  She seems to really enjoy her teacher though.  She doesn't seem super thrilled about it when I ask her after school, but she is also really insistent that she likes it and wants to go.  So, I guess it's worth it.  It's clear on the other side of town, about 20-30 minutes away, depending on how late I am and traffic.  That's two hours of driving a day.  I'm worn out after week one, hence why I am sitting on the couch blogging instead of cleaning.

I feel like the last two weeks have been big ones for Benson.  We met his new dr.  We are transferring care to Dr Connolly in St Louis.  She will most likely be a site for ISIS if they go into phase 3 trials.  In which case, he would be a perfect candidate.  She was really good with Benson and kept him at ease the whole time.  She assessed him according to his skills, rather than doing a CMAP, which I liked.  I don't really care what those numbers say and it's EXTREMELY stressful.  According to her assessment, based on his function, he is a solid type 2.  She feels good about the care choices we have made thus far and wants us to monitor his weight really closely for the next two months.  Then we may need to do a gtube, if he just can't pack on the pounds.  He's still hovering around 23 pounds.  On Thursday, we went to our local news station to do a commercial with their anchors for the MDA telethon.  It is basically a fundraising promotion for the MDA.  Benson didn't want anything to do with being there and cried pretty much the whole time.  Everyone was really nice and patient and we returned the following day and he just made some muscles by himself.  They put a cute little clip of him in the commercial.  KSPR is also coming to the house tomorrow to interview Jon and I and do a profile story about Benson.  This is where the cleaning comes into play.  A nice side point of this though is that we are finally hanging up our pictures.  It only took having the news come film our house to get us in gear on that one.  We are also in the process of getting Benson a power wheel chair.  We hope to get it before the end of the year to get maximum insurance coverage (we are close to our out of pocket max).  Benson has been getting a lot better about doing his stander and wearing this vest.  This vest is to help with his scoliosis, he has a 12 degree curve.  He is also wearing his AFOs a lot more.  Those will help with foot pronation.  And, he is working on going in the big potty.  He actually went a whole day with only one accident (that happened to be a poopy accident) and then the next day threw a fit when I tried to put him on the potty.  I have backed off since then, but I would really like him to be done with diapers.

Jon applied for a new position at the hospital and got it.  He's excited about the change.  It doesn't change salary or anything, but he will now be working at the Outpatient Surgery Center.  The patients will be less sick and the overall atmosphere is more relaxed.
Last week Jon had a vacation, and we got a lot done around the house.  We also spent a day at Silver Dollar City.  We enjoyed this visit a little more than our first and found more rides Vanessa could do.  There still isn't much for the boys, which is kind of a bummer :/
Last week I also started to get a weird irritation almost in my left arm pit.  I asked Jon a couple times if there was some kind of a rash there, but nothing appeared.  Finally, late Monday night, I got a blistery rash.  On Tuesday night I realized it was probably shingles and scheduled a dr appointment.  Sure enough, it is.  Let me tell you, shingles is fabulous.  I saw the nurse practitioner since the dr didn't have any appointments that day.  And.... she was a joke.  I always seem to get the nurses that don't know what the heck they are talking about but they are pretty sure they know e.v.e.r.y.t.h.i.n.g.  At least she gave me the right script, but about everything else she said about shingles was wrong.  And, because I am a jerk, I may or may not have sent her an email with a link to the CDC website to correct all her misinformation.
 

Things I Have Learned About Gardening

We are going to call this year a gardening crash course. I did a decent amount of reading before any planting but it really does take doing it to figure things out. Here are some things I learned this year. 

1) put tomato cages on when they're still tiny. They don't grow for what feels like forever and then grow overnight.

2) Space is beautiful. Let there be space. We over planted and now the ginormous plants are overcrowded. It just looked so empty when the plants were bitty.

3) herbs really do try to take over the garden. Especially parsley and basil. Planters really are better. They don't lie.

4) variety is the spice of life. Plant one kind of each tomato plant, etc and label them.

5) not all varieties are good. Darn it.

6) cucumbers can't be by squash or cantaloupe 

7) cloves and oil will get rid of bugs but may burn the plant too

8) Japanese beetles love to eat corn.

9) corn has to be picked when its ripe. It doesn't stay fresh on the stalk for long.

SMA Awareness Month

August is SMA awareness month!  It's a great opportunity for people to learn about SMA.  The more people know, the more funding there is, and the closer we get to a cure!
The National Institute of Health has said that SMA is the neurological disease closest to a cure.  And, the cure for SMA could help further the progress of treatments and cures in other diseases, such as ALS and DMD.
SMA has three types.  Type 1 is the most sever, with children often not living more than a few years.  Type 2 and type 3 are progressively stronger.  What was once considered adult-onset SMA is now being classified as type 4.
Type 1 SMA a.k.a. Werdnig- Hoffmann disease is the number one genetic killer of children under the age of 2.
SMA is autosomal recessive meaning both parents must carry the gene for a child to have SMA.  It is fairly common to be a carrier for this disease.  1 in 40 is a carrier.  It is estimated to affect 1 in 6,000-10,000 births.
SMA is progressive.  Once there is an onset of symptoms, there is typically a steep decline in strength with an eventual plateau. 
SMA affects all muscles in the body, usually manifesting in the legs and core first.  The hardest part of SMA to me is the part that is unseen.  It's the loss of strength in the intercostal muscles.  The muscles that I never knew existed.  They are the muscles in between your ribs that help you breathe and cough.  This weakness is what causes so many deaths in children so young.

There is a very in-depth drug pipeline underway for SMA with a lot of hopefuls.  There are two that seem to be most promising. 
One is a treatment via spinal cord injection being done through ISIS pharmaceuticals.  It is gene therapy, meaning they use drugs to actually alter the DNA.  They are onto phase 2 of human clinical trials and so far things are going well.  The injections seem to show increased muscle strength for a period of about six months with almost no side effects noted yet. 
The other is looking more and more like a potential cure.  Dr Kaspar with Nationwide Children's Hospital in Ohio is conducting research that is currently being tested on primates.  It is hopeful that they will move to human clinical trials within the next few years.  Mice that received the injections within the first few days of life, showed almost no signs of SMA 100 days later.  It may be that this proves to cure SMA in children who are diagnosed invitro, or human trials may show that the treatments can be effective for all ages with SMA.  I try to not get my hopes up yet, it's still a long ways off.

Boating

We had a last minute invitation to go to the cabin of one of the doctors Jon works with.  He also happens to be in our stake presidency and his son-in-law is an anesthesiologist, also in our ward.  We didn't get to the cabin until around 10pm on Friday night and the kids slept most of the way, a little over an hour.  That night it poured!  The lightning and thunder was crazy too.  It started clearing up around 10am so we hit the lake.  Jon and I were supposed to be back in Springfield at 2 for  meet and greet with the doctors but we just couldn't leave.  It was seriously amazing.  The kids had died and gone to heaven.  And I just relished every minute.  I miss boating so much.  I love boating.  And I now love that my kids love it.  Vanessa tried out the knee board and did fabulous.  He first try was up and down but then she went for a really long time.  She told grandma and grandpa that she went so long we couldn't even see Clay anymore.  I was seriously so impressed she tried it AND she wanted to do it again.  Her most favorite though, was tubing.  They have a HUGE tube that is very stable so we took all three kids on it and Vanessa belly laughed the whole time.  I think the boys enjoyed it too.  They were on our laps and a little more quiet so it was hard to tell.  We rode the SeaDoos and I think this was probably Brigham's favorite.  Benson loved it as well and insisted on being in the front so he could help drive.  I was amazed how well he did balancing and holding his head up.  We were going about 30 mph and he seemed to have no problems!  I can't even tell you how excited this makes me.  I wasn't sure boating would be a good family activity with our situation but now we know that it will work and that Benson will have lots of fun!  Jon did some waterskiing and I did some wakeboarding.  I am definitely out of shape and definitely don't quite have it anymore.   I didn't get any pictures of Jon, because I'm a horrible wife.  I realized he took pictures of me while juggling the kids but for some reason I felt like I couldn't.  Haha.  Next time, babe.  I felt like this was something I needed so badly.  Getting away from all the stress and worries of everything going on with life and just enjoy family and friends.  I am truly blessed.

Gymnastics Camp

Last week Vanessa had gymnastics camp ALL. DAY. ALL. WEEK.  Or, at least Mon-Thur 9am-3pm.  It was a LONG week.  It was long for her, long for me, and just crazy.  She was SO tired by the end of the week and such an exhausted drama queen.  She did have fun.  They did gymnastics in the morning until about noon and then did an activity.  They did swimming two days, horseback riding one day and then open gym the last day.  While she was gone all day, I had to entertain the boys.  It felt so much harder without my little mini mom there to be in charge of their playing.  Vanessa is pretty good about playing with both boys and they all play really well together.  I know, I never thought I would say that either. 

To fill our time, we ran lots of errands since Vanessa hates running errands.  We also did some planting in the garden for fall.  I added more dirt since we didn't quite fill the beds in the spring and the boys felt it was necessary they play in the dirt.  Typical boys.  This made Brigham really thirsty though and he went inside to find himself a drink.  We keep the cups low and he can now let me know he wants a drink by walking to the cupboard, finding a cup and taking it to the fridge for water.  If I don't see, he will stand there and yell, "MA! MA!"  until I do see.  He also found himself some oranges.  Benson also did some finger painting during a couple of Brigham's naps.  And quite honestly, I'm not sure how we filled the rest of our week.  Probably rolling cards down the driveway :)

 

 

SMA Run

Back on July 27th Jon and I helped out with a 5k run/1 milk walk for SMA.  It was a lot of fun.  I wasn't much help with the preparations but I showed the day of the race and tried to be helpful.  One day, I won't have three very needy toddlers and a house to remodel and then I'll be able to do other things.  :)  A family that just happens to live just down the street from us recently lost their 10 month old son to SMA.  He was type 1, diagnosed around 3 months old.  It's such a cruel disease.  I count my blessings every day that Benson is a type 2 and "strong" in the SMA world.  I can't imagine losing him already.  At the race, they had face painting and Vanessa got butterflies.  Jon got pictures of her reaction while looking in the mirror and they are quite possibly my all time favorite.  Benson didn't want his face painted at the moment, then changed his mind later but the line was insanely long so we had to leave to go to our next activity.  He was upset for the whole next week that we didn't let him stay to get his face painted.  We owe him big time.

After this we went to our ward pioneer day celebration.  They had a picnic at the park and then had stations the primary kids could go around to and play pioneer activities.  Jon and I joined in so Benson could join.  We did some serious square dancing.  I was the luckiest girl to get two partners (Benson and Jon) and Benson had a giant grin on his face the whole time.  I think he liked it.  Although their most favorite was "panning" for gold.