Kind Of A Boring Weekend

Which is really a good thing. We've been suctioning a bit of blood from his trach, but I'm pretty sure it's because it's been so dry here, and we've had the heater going. So we've been dropping a few little drops of saline down his trach to help with hydration, and it seems to be doing the trick.

Daddy and Dakin were playing the other day, giving Mommy some Mommy time, and this is what Daddy came up with...

Democracy in Action

A few weeks back I contacted Senator John Cornyn (our senator--TX) about the SMA Treatment Acceleration Act. We got an email back from him today. I'm sure it's probably a form email, but I felt it sounded positive, and since Senator Cornyn isn't on the list of Senators already endorsing the act I am hoping our writing in will, in some small way, help get him on board. Here it is:

Thank you for contacting me about the SMA Treatment Acceleration Act (S. 2042). I appreciate having the benefit of your comments on this issue. As you may know, S. 2042 was introduced in the Senate on September 12, 2007. S. 2042 would authorize the Secretary of Health and Human Services to conduct activities to rapidly advance treatments for spinal muscular atrophy, neuromuscular disease, and other pediatric diseases. Though S. 2042 was not enacted prior to the adjournment of the 110th Congress, you may be certain that I will keep your views in mind should the Senate consider relevant legislation during the 111th Congress.

I strongly support the goals of medical research, prevention, and successful treatment. America’s commitment to medical research and development demonstrates our resolve to understand and combat these terrible diseases, discover effective treatments, and improve the health of all Americans.

I appreciate having the opportunity to represent the interests of Texans in the United States Senate. Thank you for taking the time to contact me.

Sincerely,
JOHN CORNYN
United States Senator

Pretty cool, eh? I'm excited. Yay Democracy! If you are interested in writing to your own representatives, you can find the info regarding the SMA Treatment Acceleration Act and how to contact your reps here: look at the sidebar. It doesn't take long, and it makes a difference!

Also, Dakin fell asleep in a cute position today, and I thought you all might enjoy his picture. Sorry it's so dark!

AWESOME!

YAY! I got a marvelous Facebook email this morning informing me that The SMA Petition (petitiontocuresma.com) has increased the signature goal from 50,000 to 100,000!! WOW! I am asking everyone who hasn't already signed to please take 30 seconds and do so, and pass the word along! With everyone's help the Act will no doubt be passed soon!! Yay yay yay!

Our inspiration

On the Kidz site today is a request for inspiring posts, so I thought I would take a second and elaborate on my inspiration, which is Dakin, of course. His strength is amazing...he is amazing. He is staring at me at the moment, wiggling around in his Bumbo chair and laughing at me. It is so astonishing to think about what he has done in one year. Despite him being close to doing so, he was too stubborn to quit breathing. He has gone from not eating to consuming nearly a cup of food at every meal. He went from being unable to vocalize (due to his trach) to speaking over his trach incessantly...you can't get him to be quiet! He has a love of books, people, music and butterflies. He is my joy and inspiration every day. If this sweet child can decide to keep going despite being on a six foot leash (stupid ventilator) all of the time then maybe I can find some strength pocketed away in me. And maybe you could too, if you happen to need some. We are so blessed to have an angel living in our home, especially one as stubborn as this one! Thanks for letting me share this, and I apologize for how piecemeal it is...I didn't get much sleep last night...

And the Verdict Is...

We can keep the feeding tube out (at least for another week)! Dakin gained anywhere from eight-tenths (does that reduce? Yeah, I guess so...four fifths) of a pound to a pound (our digital scale is not perfectly accurate). Wow! The dietician was impressed and said to keep doing what were doing for another week and we'll re-evaluate next week. Yay Dakin! He's been eating like a pig! Must be nice to have that stupid tube out of his sinuses and throat. He's eating so much better than he ever did with it in, and he's even burping!

One Year Ago Today....

Dakin "crashed." Today was the day that we nearly lost him and didn't even know it. Thank heaven that I was able to get in to see the doctor, and that my dear friend Heather took us over there on her way to Wal-Mart. Thank heaven for the Nurse Practitioner who recognized that there was a problem, and took us over to see our usual pediatrician. Thank heaven for the nurse who packed us in her car and drove us to the ER, and for Dr. H (pediatrician) who canceled all his appointments the rest of the day to stay with us until the helicopter came to take us to Dallas. Thank heaven that Daddy was safe coming in to Dallas while Mommy went in the helicopter with Dakin. And thank heaven for all the sweet, amazing nurses and RTs who lovingly cared not only for Dakin, but for Mommy the whole time we were in the PICU, and the wonderful doctors who racked their brains to discover what was wrong.

All in all, thank heaven. I have been telling myself that the first year of something like this must be the worst, so here we are, having survived one whole year of it. Incredible.

Successful Week!

The weather took an upturn this week and we got to go on our wagon walk! Here's Dakin with Daddy.

We have made two successful changes this week: Dakin is completely off his Erythromycin (which is an antibiotic but in small enough doses helps with gastric motility--it was to help with his "reflux"). Dr. B (gastroenterology) said we could go ahead and take him off it. Next med to be gotten rid of is the Prevacid.

Secondly, we have lowered Dakin's Pressure Support setting on his ventilator. The last time we tried to do this he ended up pretty tired the next day, so we had to up it again. This time around he's been acting normally, and as far as we can tell there's been no lowered oxygen saturation, so we're feeling pretty optimistic about keeping it down. It was at 16, now we're at 15, and once we get down to 14 I think we can start these trach collar trials.

Hmm

Well, the NG tube trial has been going pretty well. Some meals he eats like it's going out of style, and then others it's a major fight. That was yesterday--he was NOT digging the idea of eating. He kept getting what we call "balloon belly" and didn't want to finish his food. (He can't burp when he needs to--I'm pretty sure it's part of his condition--and so of course thinks he's full when it's all just air.) But I do think he's getting enough calories, at least I hope so. We'll know on Monday at his weigh-in. The other major obstacle to his eating at the moment is that he's teething AGAIN. A big old molar on the bottom. Ouch!

We got a wagon walk in yesterday since PT had to cancel. Hoping for another today.

Day 4 of NG Tube Out

It's gone pretty well so far! The first night Dakin ate a whole mess of chicken parm I had ground up and all of his milk. He did great on Saturday, eating 8 whole ounces (4 PediaSure, 4 potato soup), which he has never done. I was a little worried on Sunday, as he didn't eat any of his table food for dinner, but he did wake up in the middle of the night and ate 4 ounces for the nurse (which shocked me, since he hardly ever eats for anyone but Mommy), and he ate 8.5 ounces for lunch today. Potato soup again...he seems to like potatoes...lolol.

We're feeling optimistic at the moment, since we were convinced that we would have to drop the tube again on Friday. He's eating better now than he ever did with that idiot tube down his throat. He does, however, have some sort of infection that resembles thrush, and so we're hoping that it won't affect him too much. Keep your fingers crossed!!!! He was always beautiful, but he is so much more so without that stupid tube!

Changes!

We had ventilator clinic yesterday, which entails a long drive to Dallas, a 3-4 hour clinic visit, and the long drive back. An extremely long day, but worth it yesterday, because...wait for it...we get to take Dakin's feeding tube out!!! After telling the nutritionist that save for the last little strike Dakin was on, he's been doing well with table food, she suggested that we give him a trial with it out. So today was our first day with it out, and he's done great--our main concern was that he can't burp and we have to burp him through the tube, but today he's had no gas issues. He's eaten quite a bit, but we need to up his calories a bit more to make up for the nighttime feed through the tube he'll be missing. We'll have to weigh him and call it in every week, but hopefully it'll be out for good! Wish us luck!

ALSO, we get to go to breathing treatments every eight hours instead of six! We're also coming off his tummy and diuretic meds, so all in all we're moving up. They even said that we could try a trach collar trial (Dakin completely off the ventilator for a little bit) next time we go to vent clinic! So all in all a great visit. Yay! Mommy needed a good one!

Hunger Strike Over?

Dakin has been pushing a molar for about 2 weeks now. He doesn't drool a whole lot, so it has been taking it's sweet time coming through. No doubt painful, with the result that he was refusing to take his bottle, and for a day or so refused his baby food as well. We had tried everything--he won't take a sippy cup, he did ok with a regular cup but it took forever and he only got a little. BUT, last night he took almost all his bottle and "real" food, and did the same for lunch and dinner today. Thank goodness!!! So hopefully no more having to give him all his liquids through his feeding tube--we are being cautiously optimistic.

We also found a new nurse. She is a good friend of our night nurse, who speaks glowingly of her, so we're feeling pretty good about that. She came out this morning to meet us (Mommy's new stipulation to the company--we must meet and approve them before orienting them) and seemed amenable to all of our rules, so we're giving her a try tomorrow. She's not very experienced on the ventilator, but Mommy's willing to train someone if he/she appears to be neither stupid nor crazy (both of which we've had in our nurses...sigh...). So hopefully she'll work out for us.

Dakin the Prodigy

Dakin loves playing his piano! The "eyes" thing I'm trying to get him to do is a little trick of his--when we say eyes he bats his eyelashes (well, blinks really hard, but it's really cute and always makes me laugh). He however refuses to be a performing monkey and so won't do things when I have the camera out.

Here's another cute shot. I'm not sure what's on his face, but we had his feeding tube out for the day so I thought I'd take advantage of it.

Ooh-de-la-li, Ooh-de-la-li, Golly, What a Day

Yikes. In fact, what a week. Yesterday we lost (he went to another job) our Occupational Therapist--bummer, since we are having bad luck with OTs for some reason. And then today.

Oh today.

Dakin had a GI appointment rescheduled on Monday for today. I almost canceled it yesterday since I had a bad feeling about it, and now I know why. Our day nurse got here at 7, 75 minutes before we had to leave for Dallas. We told her we had to go (she hadn't known--we didn't even know until Monday). So she puttered around for a few mintues, drew Dakin's morning meds, and then informed me she wouldn't be going. The conversation went a little like this:

"Why?"
"Personal reasons. I'll let the office talk to you about it."
"Um, if Dakin had been coughing up blood all night and we had had to go to vent clinic, would you not be going?"
"That's different."
"How?"
"It just is."
"If I cancel this appointment he can't see the GI doc until April."
"I'm really sorry."
"Were you not planning on working all day?"
"No, I was planning on working at home (our house) all day."

It went on like this for a bit, and she ended up saying, "I think it would be best if I left." I'm thinking in my head, "Yeah, better for you. I'm going to start yelling loudly in a minute."

So she left. Daddy had to take the day off work so we could go--Dakin cannot be transported without 2 people. Needless to say, I'm pretty irate and the agency has fired her. What on earth?!?!? That's the whole point of having these nurses here during the day. Anyway. She is evidently going to have to face a peer review and get some sort of nurse slap on the wrist.

Thing #2: When we got to the hospital, I discovered I was MISSING A SHOE. Yeah. One of my shoes was gone. I guess it slipped off when I got in the back seat with Dakin (I ride with him barefoot since there's so much stuff back there with us it's easier). Fortunately, Daddy had a pair of Jordans in the trunk. Too big, but they worked.

Thing #3: We get in the hospital, and they told me the appointment was an hour earlier than they told me it was. I nearly lost it. I think, though, the guy sensed I was about to go nuclear and got me in. The appointment went well--Dr. B is still ok with us not doing the G-tube yet (since Dakin's eating issues are not related to musculature--he's just stubborn.) and said we can come off his tummy meds, thank goodness.

So that was our day. On the bright side, we did have a good time overall. Dakin did great in Physical Therapy today, and it was beautiful outside. I'm feeling optimistic about a wagon ride tomorrow.

Thanks for letting me vent.

This Can't Be Said Better Than This.

I hope no one who's already posted this minds, but I really wanted to share it. It's from PostSecret.com, from someone who sent it in to be posted on that site. Please take a moment to read it and put yourself in the shoes of a parent who is facing this. Treatment and cure for SMA are so close...please please take 30 seconds and sign the petition (www.petitiontocuresma.com). And then please take 10 minutes to tell your friends about it. No one should have to face this. For us, the SMA Treatment Acceleration Act (which the petition is for) won't directly help Dakin's disorder, but it might have side benefits for it, so this is pretty much the only viable star we have to hitch our wagon to. Please. Help us save these sweet little ones and their families. Help us maybe save Dakin too. Please.

Well Thank Goodness...

I was really starting to worry about Dakin's oxygen saturation being so low...he was sitting 96-97 on Friday night. Well, come Saturday, and he's still lower than usual for him, but Saturday night he was back up at 99-100 most of the night. It turns out it was something silly...our weekend night nurse (who herself was trached at one point) propped his head up and that launched his sats right back up. When we first got home we went through a period where he was on O2 quite a bit since his sats were low, and it turned out that all we needed to do was position his ventilator circuit better so it triggered the vent--I guess this weekend was just another period like that. Whew. I didn't think I could be any more aggressive on his chest percussions.

We are praying for all the SMA and SMARD kids in the hospital right now. Get well soon, little fighters!!

No nurse tonight/2008 Reflections

Our night nurse called in sick, so we are going it alone tonight. No biggie, it happens and we are trained in caring for Dakin, thank goodness. I am a little nervous, though, as he is on oxygen tonight. Dakin is NEVER on O2, and his oxygen saturation is typically 98-100 all night, every night. Tonight, though, we're having a hard time staying at 97. Not sure why...we did agressive chest percussions with his breathing treatment, and even did an extra treatment 2 hours later. He's just tired, I guess. It happens.

Anyhoo, one of Dakin's SMA buddies had a year in review posted on her website, and I thought it was a good idea (hope you don't mind, Kim!). 2008 was, in all honesty, the worst year of our lives, and not just with Dakin's problems. I won't go into the other stuff, though, and stick to the D. Let's see...at the beginning of January Dakin began eating less and less, and started gagging profusely. 1/7 he was taken to the doctor and put on Reglan for "reflux." Reglan didn't help, back to the doc where he was put on Prevacid. Two days before Dakin's crash, he cried all day and when he wasn't crying he was sleeping. Thinking it was his formula upsetting his tummy, I switched him that night and he seemed better the next day. Then came January 24th. We woke up that morning, and Dakin was very lethargic. He wouldn't smile and was retracting in his breathing (which I didn't recognize at the time). I thought the lethargy was due to him not eating enough, so I got in to see the doctor that day. My dear friend Heather drove us there, and when we got there they put Dakin on a pulse oximeter (I had no idea what that was at the time.) The doctor gently wrapped Dakin up and told me we needed to go to the Emergency Room. His nurse drove us there. Dakin was intubated in the ER and we were lifeflighted by helicopter to Medical City Dallas. We were in isolation for the first 72 hours or so, thinking it was RSV or something of that nature. Dakin had the "million dollar workup" with every test they could think of short of an EMG (that came later). All they could find was that his diaphragm was not moving as much as it should. They tried and tried to extubate him, but he just couldn't keep his sats up, so they trached him around the 10th or so of February.

Finally the 16th of February we got to leave and go to Baylor Our Children's House. We spent 7 weeks there, learning to care for Dakin on our own. Time definitely well spent. March came and towards the end Dakin's pulmonologist recommended that we see Dr. Iannaccone, a world-renowned neurologist. We were transferred to Children's Medical Center Dallas and she saw him there. We also had more CT scans and an EMG/Nerve Conduction study. She recommended that we test him for SMARD-1.

Then on April 9th, we were FINALLY released from the hospital! We spent April, May and June getting accustomed to having 24 hour nursing in our home, as well as the rigors of caring for a ventilator dependent child. We also worked hard to help Dakin to eat normally.

In July we got to go to Daddy's family reunion, which was a wonderful experience. In July we also got the test results from England saying there was in fact a mutation on one of his genes, supporting the diagnosis for SMARD. We spent August and September thinking he would not be with us for very long.

October, and NaNa (Mommy's mommy) came. 10/1 we saw Dr. Iannaccone, and she confirmed the diagnosis. Thankfully, she said that he should be with us for a long time unless he gets a respiratory infection. We spent October preparing for his birthday.

At the beginning of November, Dakin woke up with a hole on the side of his neck, which turned out to be an abcess with MRSA staph. Nice. So we were hospitalized, since everyone is afraid of a vent kid...lol...We were out soon, though. All better a few days later. We had Thanksgiving and Dakin got to sit at the table with everyone else.

December passed peacefully with lots of holiday celebration. Dakin's second Christmas was perfect.

Whew. Long read, sorry! We are hoping for a better year in 2009. Hope the same for everyone else!!!