Families of SMA - North and South Carolina Chapter Meeting

I got an email from Families of SMA last week with exciting news for families (and their support networks) living in North and South Carolina!

It read:

There is a meeting planned to get a North Carolina and South Carolina Familes of SMA Chapter up and running!

On June 27, 2009 from 12:30pm to 2:30pm

At the Downtown Sheraton in Raleigh, NC

For more information, please contact Jessica Adams at qst4it@earthlink.net

Obviously, Neil and I are planning on being there with our (large) support group. I'm so glad that it's after I get back from the conference since I'll have lots of information to share!

If you or someone you know is interested in attending the meeting, please contact Jessica Adams at the email address listed above.

Hope to see you there!!!!

Have You Contacted Your Members of Congress for Ollie Yet?

No? Well, check out the Families of SMA's Legislative web page. Follow the "Contacting Congress" link. Look at the "Tips for Contacting Congress" and the "Top Ten List."

Alright. Now go get your phone.

Got it? Good. Dial (202) 224-3121 for the Capitol switchboard. Ask to speak to your Senator or Representative's office. (You'll speak to an intern, but that's okay.) Tell the intern your name and that you are a constituent. Tell them you would like for the Senator or Representative to co-sponsor the SMA Treatment Acceleration Act. Tell them about Ollie. Tell them that the SMA Treatment Acceleration Act is important to Ollie and important to you.

That's it. It'll take you about 2 minutes. And when you find out that your Senator or Representative has co-sponsored the bill, be sure to call back and thank them.

Now, pat yourself on the back for a job well done!

Ollie thanks you from the bottom of his adorable, little heart!

Fundraising and Ollie's Needs - So what's next?

So Bekka and I have been floored by the outpouring of support for our special little guy but anticipation and planning never stops for us.

We've been thinking about what we can do to raise additional funds for Ollie and have heard ideas for walks and golf tournaments and car washes and the like. Neither of us has ever organized anything like that but we are willing to learn and do for the little tyke. If anyone out there has thoughts, please let us know!

When Bekka gets back from the SMA conference, we are going to take a long look at what we need and when we need it. In many ways, we never anticipated Oliver would be with us this long. Now he's almost a toddler and we have to continue making plans and anticipating his needs.

We have had questions about what sort of expenses and needs Oliver is incurring or we are expecting. The following is a short list and is in no way complete.

• Better mobility for Ollie. We will be trying to figure out a wheelchair or stroller that allows him to lie almost flat while also allowing us to more easily transport equipment. It's quite a sight seeing us trek to and from a doctor visit!
  
• Appropriate toys and games for Ollie. It's hard enough entertaining a regular 10 month old, but entertaining an SMA child is even tougher. We will be looking to get creative to provide Ollie with the stimulation and entertainment he needs beyond television and videos.
  
• Medical expenses including copays, coinsurance, and out of pocket expenses for Ollie's multitude of medical equipment. It appears we have been approved for CAP-C (we are awaiting the official letter). This will help relieve the burden, but not completely.
• Insurance. We don't know the final resolution of Bekka's job situation yet, but it is likely we'll be paying out of pocket costs for her insurance and Oliver's insurance for the foreseeable future.
• In the nice to have but not yet critical category: A larger vehicle. Bekka and I both love small cars (We have an Integra and a Mazda 3) but as Ollie grows and his equipment needs grow, we'll be looking at acquiring something larger to make it easier to transport us all. We have wonderful friends that loan us vehicles on a moments notice -but you hate to abuse that!

This is why we are so thankful to everyone. Imagining all the additional stress we'd be under right now without the meals and visits and fund raising is staggering.

$12,500 (and growing)

Wow.

Thank You!

Between anonymous donations, the incredible yard sales and bake sales, jewelry sales and cookware sales, the fantastic fundraiser in Deep Gap and all the wonderful folks at Powder Horn Mountain, Ollie is one well-to-do 10 month old. In fact, he's sending his mommy to Cincinnati to learn more about SMA!

We can't possibly thank everyone directly, but we want you to know that we feel the love and hear the prayers everyday.

We wish you all a day full of Ollie smiles!

Ollie's Big Weekend

Ollie had a big weekend. Aunts and Uncles and Grandparents and Greatgrandparents oh my! He was a smiling little guy all weekend long.

Thanks for coming everyone and Happy Birthday Papaw Clay (even though you'll probably never see this!).

We'll try to get some pictures up shortly.

On "Being Social with SMA" and the SMA Treatment Acceleration Act

I've started following several children with SMA on the web recently. One of those children is Gwendolyn Strong. Her parents, Bill and Victoria Strong, have an online Petition to Cure SMA. The petition is an effort to support the SMA Treatment Acceleration Act (H.R. 2149 and S. 1158).

There are two things that everyone can do to support families affected by SMA: sign the Petition to Cure SMA and contact your Senators and Congressmen or Congresswomen about the SMA Treatment Acceleration Act. The Muscular Dystrophy Association (MDA) has a great advocacy web page that allows you to input your zip code and send an email to your representatives. It has a generic letter that you can adapt or you can type your own. Be sure to contact both the House of Representatives and the Senate.

Back to the Strong family. One of the recent posts on their blog was about Gwendolyn being social. This is one of the hardest things about SMA. The affected children are typically very bright and social. But you have to get very creative in order for them to "play" with other children without compromising their health. The Strongs' post covered that topic quite nicely. Here it is:

Gwendolyn is truly a social child. She has been since she was a tiny baby. During our first hospitalization when she was only 9-weeks-old, she showed no fear toward strangers, and instead beckoned every nurse with a sweet grin and a look that said, "Hi, come play with me." And she hasn't slowed since.

This is something we have struggled with since Gwendolyn's SMA diagnosis and something that all SMA parents have to constantly weigh -- risk of germs vs. social interaction and that ever present "quality of life" we try to measure. In the beginning, there was no way I wanted to be near anyone, but my baby had turned blue in my arms half a dozen times in two months and I just couldn't take any more. Because of my fear Gwendolyn had very limited contact with others or with the outside world, but soon we all grew claustrophobic. We started with walks on deserted streets, to parks where not a single person was currently gathered, at times when others were still at home. It felt good -- not right away, but each time we stepped out the door it became more comfortable. Slowly that gave way to pushing ourselves a bit further for Gwendolyn's sake and that felt good, too. As we started to come out of our shell shock winter hit and the threat of a common cold or the mild flu that almost everyone seems to get during that season shifted the scale again and so we hunkered down. It is not uncommon for SMA families to literally never leave the house in winter. And I can completely understand why. But, Gwendolyn is social, our social butterfly, and began demanding more stimulation. We continued our walks and as the sick season began to end, we started going to places like zoos and museums and even restaurants (usually at off times). But, Gwendolyn is ever-changing and so we are adjusting our barometer once again by doing more with other children. This is obviously a risk, little children mean little germies, but she so wants to be with her peers. She loves watching them play; when they laugh, she laughs; when they cry, she looks at me with concern. And she adores it when they stop their darling toddler busy-ness and come over to say hello, share a sticker, include her in the fun. She has a small group of pals that we now see on a regular basis. It makes a world of difference that I adore all of her buddies' parents and they understand our germaphobia and support whatever makes us comfortable.

Yes, sometimes being out, being social, pushing our boundaries makes me nervous -- make that I'm always a little bit nervous -- but we bring anti-bacterial wipes, try to minimize any obvious risk by avoiding crowds or places people go when sick (the grocery store is off limits), and leave if we are uncomfortable (I hear a sneeze from a mile away...not kidding). And, ultimately, if it makes Gwendolyn happy, we find a way to do it.

Isn't that what any parent wants is for their child, to be happy? Sadly, this horrible, terrible, awful disease makes that so much harder than it should be.

Thinking of Mackinley

We're thinking about Mackinley and his family today. His surgery is scheduled to start at 10:15am. Dr. Rice who performed Oliver's surgery is also doing Mackinley's surgery. We hope that things go as smoothly for Mackinley as they did for Ollie.

Ollie & Mackinley Meet

We had a "routine" visit at Duke today with Dr. Smith. He sees Oliver every month or so to monitor his progress. At this visit we also got to meet Joanne Mackey and Dr. Kishnani. They will be part of the team that will be running the clinical trial (CARNIVAL I) starting around June 23rd! We are excited and hopeful about this study. It's not a cure, but we hope it at least leads to some treatment options for kids with SMA.

After our appointment, we all went to visit Mackinley and his family. You can follow Mackinley's journey on his CaringBridge site. It was really cute to see Ollie and Mack lying beside each other. They really seemed to enjoy looking at one another. And little Mackinley had a LOT to say! We're not sure, but we think they may have been confirming their plans to take over the world. Or at least Duke Children's! Personally, I think they plan on doing it by flirting with all the ladies. =)

Neil and I had a nice visit with April, Kyli and Paige (who turns 6 years old on June 9th...she wants everyone to know). And we got to meet Brian as well. Please keep April, Brian, Mackinley and their family in your thoughts and prayers. Mackinley's g-tube surgery is scheduled for this Thursday, June 11th.

Cincinnati Bound

Families of SMA is having its 25th Anniversary Families and Professionals Conference this year. It will be held in Cincinnati from Thursday, June 18th through Sunday, June 21st (which is Father's Day). I'm excited that Ollie's Auntie Amanda and I are going to be there. I hope to come home with lots more info and lots of great ideas. =)

Of Awesome People and Spaghetti Dinners

So today in Deep Gap, NC, the wonderful folks of the community and the Stewart Simmons VFD are having a fundraiser for Oliver. A spaghetti dinner and a raffle are on tap.

It's things like this...compassionate actions from complete strangers or those whose life Ollie has only peripherally touched that keep us going each day. There are a lot of wonderful people out there. It's easy to be bitter and cynical in today's world...there are lots of perfectly valid reasons (see previous post), but every time we get a check from someone we barely know (or don't!) or someone holds a fantastic fundraiser in honor of our little guy or brings dinner or just drops by to chat with Bekka during the day, it lightens the load a little bit.

Neighbors, friends, family, coworkers, strangers - so many people have stepped up to help. For that we thank you and will continue to thank you. It may take a few months to get that thank you card out (it may not even make it at all, I'm sorry to say), but please know from the bottom of our hearts we thank you.

It's going to continue to be a struggle and it's hard not being able to handle everything ourselves sometimes, but we are so thankful for the help. As much as of a challenge as it is to meet Oliver's daily needs and as tough as the loss of Bekka's job is, it would be so much harder without you.

Of Robinul and Job Loss...

So two disappointing things to report today (Please pardon the somewhat bitter tone)

1. The issues with secretion thickening due to the Robinul have resulted in several de-sat incidents that have forced us to wean Ollie from the medication. In a couple of days, we are probably going to try again at 1/2 dose to see if that will help secretion amounts, but without the excessive thickening.

2. As of last Sunday, Bekka no longer has a job. Well, at least not one with benefits. Her FMLA leave was finally exhausted and being bound hand and literal foot by corporate policy, she was reduced to part time with the requisite pay cut. They have been wonderfully flexible with Bekka and her schedule, but time was not on our side. In many ways we never expected Oliver to make it this long, but he's been such a champ and an unexpectedly strong little boy!

There is a terrible irony in this story: We are pretty sure the only reason this happened when it did is because we called to ask about Bekka's status. We were trying to be proactive and make plans for ourselves and Ollie over the coming months and I asked Bekka to call HR to see where we stood. They hadn't the foggiest idea. It took them over a week to figure out her leave balance. She confirmed it one night by sitting down with all her pay stubs and adding up time worked.

Unfortunately, they let Bekka know Friday morning (6/5) that retroactive to 5/31 she was part time. One really wonders that had we simply played the military's game of "don't ask, don't tell",
if we might have gone indefinitely before someone figured it out. Who knows!

Would that have been "moral"? I don't know, but we are the ones with a terminally ill child, so our judgment might be clouded on the question.

It should be noted that all of Bekka's local coworkers and her entire management chain have been nothing short of fantastic. Wonderfully warm and supportive people all and all equally disappointed in how things resolved! This is strictly a corporate issue and it's not unique to Stride Rite. We aren't angry with Stride Rite. We think they have been more supportive than many companies would have been in the same position. We ARE angry about the difficulties involved in this entire process when we really just want to take care of our little boy.

So where does this leave us? Honestly, things wouldn't be so bad if I didn't work for the state. Working for a government that some sources now rank 50th in the nation in support for worker's families, it's actually better for us to COBRA Bekka and Ollie at the cost of $671 a month out of pocket than it is for us to put them on my insurance.

Yep. You read that right.

It would be a little cheaper (but not a lot!) to put them on my insurance each month, but the loss of benefits is too large. The things not covered by the state or at higher costs are legion. If you had any misconceptions about the "great benefits" offered by the state, you can put those to bed now.

It will be interesting to see what happens to the state employee base when economic conditions begin improving...they made multiple "adjustments" to our health plan this year that resulted in the doubling of deductibles and many copays while also increasing the costs for family coverage. It's quite obvious state employees aren't resources - they are the proverbial cogs in the machine, and it's doubly obvious they really only want healthy single people working there now.

So who wants to step up and tell me just how awesome the American medical and insurance system is right now? Bueller? Bueller?

For happier news, see the post I'm about to make...

CAP/C Approval....Almost

I got confirmation today from the nurse case manager that our CAP/C approval letter from the Department of Medical Assistance (DMA) had arrived in her office. Yippee! She still has to get a few things arranged with the Wake County Medicaid representative. And the Wake County Medicaid representative still has to get a letter from Disability Determination Services that says Oliver is disabled.

It's one hoop after another hoop after another hoop to jump through to get everything approved.

I spoke with the representative at Disability Determination Services and pointed out to him the Spinal Muscular Atrophy Types 0 and 1 are on Social Security's list of Compassionate Allowances. He either didn't know about the Compassionate Allowances or didn't care. Regardless, the Disability Determination Services doctor has to review Oliver's medical records to determine if he's disabled.

I'm still excited. One hoop down, who knows how many more to go!

Mommy's Technical Notes re: ER visits

When we first started researching SMA, just before Oliver's "official" diagnosis, I remember reading different sites - university web sites, FSMA, various blogs - and wondering to myself what all these different terms and numbers all meant. What is a de-sat? Why is it important to have a pulse ox? For that matter, what is a pulse ox?

As we've walked along our SMA path, we've vowed to not only give Oliver the best care that we can but to also re-pay the SMA community: to "mentor" other families much like we were; to answer as many questions for our friends and family as we could; to raise awareness of the disease; and to raise awareness about carrier testing.

This post - the one you are currently reading - is meant to help answer some of those questions. We've told you about our ER trips and Ollie's fondness for riding in the ambulance. (He's a little boy, after all, and they like vehicles that go fast, drive on medians, and make loud "woo wooo wooo" sounds, right?) But how do we know when to suction or do CoughAssist? And when do we decide to call 911? And why does Ollie need to go to the ER so often? (Please feel free to ask us any other questions you may have. We're happy to answer them.)

So, we've been to the ER three times. On April 8th, the pediatrician's office called the ambulance for me. Our second visit was on May 4th after a routine visit to Duke to see the neurologist. And our most recent visit on May 29th. All three visits are likely due to mucus plugs - a fairly common malady for folks with SMA. Since SMA weakens the muscles used to breathe and to cough, small amounts of mucus - that unaffected people would just cough and clear - build up in SMA patients and clog a portion of the lung. In severe cases this can result in a partial collapse of the lung.

When we have these little adventures to the ER, they are usually precipitated by oxygen desaturations (often called de-sats). In Oliver's case, his baseline (or normal) oxygen saturations are between 97 and 100 percent at rest and between 95 and 98 percent when asleep. If he is awake and his sats drop below 95, we can usually bring them back up with some suctioning and CoughAssist. Dropping to 95 isn't usually a panic situation.

Everyone's sats vary at different times. When you sleep, your sats will decrease because all the muscles in your body relax - including the muscles that help you breathe. At night, when Oliver is asleep, he will often de-sat into the mid to upper 80s and his pulse ox will alarm. (The settings are adjustable on his pulse ox. We have it set to alarm if his oxygen drops below 90 or if his heart rate rises above 200 beats per minute.) This may happen only once a night or it may happen repeatedly. Usually, he will drop long enough for the alarm to sound once (or maybe twice) and then he'll bounce right back into the 90s. Sometimes it will sound more than once or twice and it takes turning his head or rubbing his leg to get him to take deeper breaths and bring his sats back up. Occasionally, we may even have to wake him to do CoughAssist or to suction.

If his sats drop and his heart rate rises at the same time, it's a pretty good indication that he's distressed. Each time we've gone to the ER, his sats have dropped into the 70s (or lower) and all of the CoughAssist and suctioning we've done haven't brought him back into the 90s. Right before the first ER visit, from the pediatrician's office, we were getting sats of mid to upper 60s when they called 911. By the time the ambulance arrived, the pediatrician had done quite a bit of suctioning and Oliver's sats were in the upper 70s.

The second trip by ambulance to the ER was right after we had gotten back from Duke to see Dr. Smith, Oliver's neurologist. Ollie had been sounding raspy while we were at the appointment. I'd done several rounds of suctioning. I checked his sats and suctioned a little - to be on the safe side - right before we got in the car to head home. Oliver slept the entire ride home.

When I got him out of the car, he was obviously distressed. His little face was covered in beads of sweat. He looked at me with concern in his eyes. I took him in the house and hooked up his pulse ox. I was expecting a reading of low 90s or maybe upper 80s. Nope. He was registering in the 70s. I did CoughAssist and suctioned repeatedly and only got him "stablized" into the mid-80s. My dear friend, Cathy, got to make the 911 call that time.

The third ER visit Neil has told you all about in his post. Will it be the last trip to the ER? Probably not. As his SMA progresses, all of Oliver's muscles will continue to get weaker....including the ones used to breath and to cough. In spite of all of our "drama", we consider ourselves very fortunate. The only hospitalization Ollie has had was for his g-tube and Nissen surgery. And, so far, he's not had a collapsed lung. Many families are not as lucky.

For Ollie's New Friend - Mackinley

Late last month, our neurologist, Dr. Smith, put us in touch with a local family whose son, Mackinley, had just been diagnosed with Type I SMA. He turned 4 months old on Sunday. And, on Friday, when Ollie was at WakeMed ER, Mack was at Duke's ER.

Mackinley has been admitted and is being monitored by the same doctors that Oliver sees - Dr. Kravitz (pulmonologist) and Dr. Smith. Mack will even have his g-tube surgery done by Dr. Rice, the same surgeon that did Ollie's surgery. His surgery date was moved up to next week from late June .

Neil and I feel for Mack and his parents and all of his family. These are tough decisions to make. So, please, pray for Mackinley as you have for Oliver.

ER #3

Friday (5/29) proved to be an eventful day, but not in a good way. I arrived home a little early so that Bekka could head out to an appointment. I'd only been home a few minutes when Ollie's sats begin dropping. They very quickly descended into the 70s. Vigorous suction and cough assist managed to get him into the low 80s, but no higher. When he dropped into the 60s before bouncing back into the 80s and after we had been at this for several minutes, we made the call to 911.

By the time the paramedics were here, he was back to around 90, but still sounded horrible. They gave him a hit of supplemental oxygen and we headed out the door. Mom loaded up with Ollie into the ambulance and I followed a little bit later with some of the necessary bags.

By the time, we were all at the hospital and in an ER bay, he seemed to be doing really well. In fact, they were drawing up the discharge papers when his sats crashed again. We got his numbers back up pretty quickly, but his heart rate remained elevated. This is always a sure sign that he is still experiencing respiratory discomfort, even if his sats are in a more normal range.

We requested they send in a respiratory therapist to do deep suctioning. Several rounds of this brought up a large amount of very, very thick (but clear!) mucus. He began to improve slowly and Bekka and I were having discussions with the Doc about admission vs. going on home. The decision was not an easy one and they let us continue hanging out in the ER while we made the decision. Pretty soon, he began to perk up to normal Ollie mode and grinned at Daddy! We knew then we could head home.

In the meantime, members of the WakeMed branch of the Ollie fan club came by to see him. I don't think any parent wants to be a "regular" at their local ER, but it's a comforting feeling to know the nurses like your little fella so much they go out of their way to visit. We've been so impressed with our experiences at WakeMed and with Wake County EMS. We can't begin to offer enough praise for their professionalism AND their compassion.

As of now, we have backed off his Robinul dose (though not completely, as that is not a tenable situation) and are being even more vigilant.