I guess Kyle doesn't care for hospital stays.

Locally here in the southwest, we have an amusing commercial for a tire company.  The premise is that a man in the hospital is completely bandaged with broken limbs is listening to his doctors exchange niceties about a tire deal and is so moved that he leaps out of his bed--and out of traction--ripping out tubes and IV and all, conceivably in order to get in his car and take advantage of such a deal.

The first few times Kyle saw this commercial, he was upset and disturbed because he thinks the man looks like a mummy (he does).  But now he loves watching this commercial.  Why?  He has recognized the patient's free will.  That guy can get up and run out of his hospital bed.  Yes, Kyle envies the actor in that commercial because that man can leave his bed and leave the hospital.


Kyle: I wish I was like that man with the casts, I wish I could get out of my bed and leave the hospital.

Me:  Oh, it's okay, his doctors were right there taking care of him and he didn't really HAVE to leave.

Kyle: Mama! When I'm in the hospital, I wish I could jump out of bed and leave.

Me:  It's okay, hun.  If you're in the hospital, I'm with you and you're right where you're supposed to be. I wouldn't want you to leave, not until the doctors say we can go home.

Kyle:  Mom, the next time I'm in the hospital, I want my legs to get strong and I want to run out of there like the man on that commercial.


My heart = broken.

This is SMA.

This is SMA.

Kyle just turned four.  He adores baseball, golf, soccer, tennis, football, boxing, and hockey.  He thinks he can play these sports when he "gets bigger".  He will get bigger as he grows older, but he will never stand.  He won't play these sports, at least not how he imagines he will.  And we are the ones who have to tell him that.  He is in a wheelchair.  Cognitively, he is advanced (that means smart) (I only clarify that because even I wouldn't have known what that meant until years ago when someone told me that he is cognitively advanced).  This week, he told the O.R. staff at the hospital that he can't wait until he gets older and he can stand.  He happened to be in the O.R. to have his Achilles tendon completely sliced through so that his feet won't turn out.  This means that even if a cure for SMA was announced two hours later, he can never stand on his own.

This is SMA.  

This is Lauren.  She is Kyle's fraternal twin sister.  She also has SMA.  And a sense of humor.  She is hilarious.  She sings nursery rhymes and Disney show tunes.  She can speak "Dora Spanish".  She loves to eat melon, any kind of melon.  And chocolate chip waffles.  She wants to be a veterinarian when she grows up.  She also wants to ride a bike and ice skate "when my legs get longer".  We get to be the ones to explain to her that no matter how long her legs get, she won't be able to do those things.  But she is special--besides driving her own power wheelchair, she can do things that other four-year olds can't, like competitively play Connect 4.  She wants to go to Disney World and be a princess, but her favorite Disney character is Daisy Duck (awesome!).  She can't crawl, turn over, lift up her head while on her tummy, or walk.  She is in the 3rd percentile for her age, and her g-tube surgery is scheduled for later this month.  She struggles to hold herself upright in her highchair, so her scoliosis surgical consult is scheduled for next month.

SMA stands for Spinal Muscular Atrophy.  From the moment of conception, my twins' motor neurons weren't right.  They were born crying and altogether pretty pissed off (the lighting made them look sort of bad, I don't blame them), but many newborns with SMA are born silent--these are the SMA Type 1's.  They struggle to breathe, struggle to move.  Mother Nature, and evolution, are screaming at them to scream! when they're born, but they are thrust into our world still struggling to take any sort of breath.  Breathing requires muscles and motor neurons that do what they're supposed to do.  Those babies don't tend to last long.  Sometimes they die before they even get the bloodwork back with their SMA diagnosis.  Other times, their parents are faced with life-and-death care decisions before their child can even have her first mall portrait taken.  Other times, SMA babies seem completely typical until 6 months old, or 12 months, or two years.  It's the "atrophy" in Spinal Muscular Atrophy that makes the parent suspicious that maybe there's a problem--these are the SMA type 2's and 3's (Kyle and Lauren are type 2).  Even then, the prognosis is grim.  Children born with SMA (non-type 1's) sometimes live until their teens, and in some cases even beyond their teens.

What lames the one causes the death of another.  They're all missing the same motor neuron.  There is no prejudice with SMA.  And there is no cure.  But if there was (but if there was!), the thing that cures the one can cure them all.

SMA is one of many muscular dystrophies.  1 in 40 of us are carriers.  There is a screening test to determine whether or not you are a carrier.  You can ask for the test, before or after you become pregnant.  

I haven't worked since just after my twins were born.  I left my career to schedule and keep up with regular appointments with the neurologist, pulmonologist, orthopedic surgeon, gastroenterologist, dietitian, orthotics clinic, wheelchair clinic, home health, and surgeons.  I never managed to find the time to become PMI certified when my office was a cubicle, but given my experience sitting in waiting room after waiting room, I could teach volumes about "scope creep".  I would give all my Six Sigma certifications, and more, to not know what I know about children and terminal disease.

SMA is life changing--not just for its direct victims, but for their parents and families.  We are exactly like you.  In fact, many of us had typical healthy children before we ever needed to know about SMA.  If there was ever an ideal Project Manager, it's the parent of a medically fragile child.

That's all.  I just wanted to tell you that.  August is SMA Awareness Month.  If you didn't know what SMA was ten minutes ago, you know now.  It's all about the kids.  Ask me anything.  I've heard it before, and assuming you are not personally attacking my children, I won't take offense.  I'd be happy.  Not bothering to ask means that we're being ignored, and my children will not be ignored.  Ask away, I'm thrilled to explain.  I rarely get asked questions, other than "how fast does his wheelchair go?".  (5.2mph.)  Ask me.  I'll tell you.

Walk N Roll 2010, Baby!

This Sunday will be our second year raising money for the Families of SMA Arizona Walk N Roll. FSMA serves as a critical support system for families facing the devastating diagnosis of Spinal Muscular Atrophy--they fund critical research for treatment and cure for this type of muscular dystrophy, which affects newborn babies the hardest. Half of babies born with this incurable muscular disorder die before age 2.

When strangers strike up a conversation with me about the kids and their disease comes up, they usually ask me if they can walk--they are in wheelchairs, so while I think the answer would be obvious I still find the patience to explain that not only can they not walk, but they don't have the muscle strength to sit up, roll over, lift their arms to comb their hair, or cough. What they lack in physical strength they make up for in charm and intelligence, but the fact remains that they will live their lives dependent on wheelchairs and caregivers and that just sucks.

Families of SMA was a key resource for us when the twins were first diagnosed with this terminal disease at age 16 months. FSMA sends "newly diagnosed" care packages to new families, put us in touch with other local families on whom we can rely for support, host a message board forum so that families can organize ourselves network and rely on our worldwide counterparts for support, directly fund cure-based and treatment-focused research, and compile summaries of laboratory and clinical research in layman's terms for the families.

FSMA is the very definition of "a good cause" and if you have a few bucks to throw to it, I'll take them. If you do or don't, the fact that you're just aware of the disease that defines us now is a win, as far as I'm concerned. Thanks for all your support over the last two years--I feel like a broken record saying that all the time, but it's true. Managing this stressful life in a vacuum, with nobody to listen to me or hug me or bring me bottles of wine, would blow chunks.

Not a Pity Party. But Ugh.

Me: Is there anything you can't reach anymore? You're tall enough for all the faucets and shelves now--we should get rid of this step stool.

Jenna: But in case someone cures SMA soon then Kyle and Lauren can use it when they can walk. We should keep it a little longer.



Sometimes--sometimes it hits me right. here.

Two Years.

Two years ago today was when the twins were diagnosed with Spinal Muscular Atrophy. Two years since we stared in disbelief at our happy one year olds while we listened to the neurologist deliver anyone's worst nightmare to us over the phone. It was entirely life changing.

SMA is a type of muscular dystrophy which affects voluntary muscles. SMA patients are weak, because they're missing a specific muscle protein. They are highly intelligent but are frequently described as "floppy" because their limbs have so little muscle tone that they flop around unless we're very careful in handling them. They cannot sit, roll, crawl, or walk. Their respiratory health is of utmost concern because they cannot produce an effective cough. Think about it--a good cough requires the cooperation of your diaphragm and other abdominal muscles. SMA patients can't cough like you or I can--in fact, many SMA babies can't swallow, or even breathe on their own. The severity of muscle weakness is broad, but the thing that all SMA patients have in common is the deletion of that SMN protein--so regardless of how severely one is affected, the same cure will cure them all.

Despite the dozens (hundreds) of families that I've come to meet and chat with who are affected by SMA, the disease is still considered rare. Because of this there are limited research dollars available to hunt down a cure or treatment. There are some extremely intriguing advancements in the fields of gene therapy and stem cell treatment, and I am on the edge of my seat every morning when I turn my computer on in hopes that a breakthrough for treating SMA will pop up on my RSS feed. So far, no luck. SMA is terminal. There is no treatment. 50% of SMA patients pass away before they turn two years old. My twins have so far beaten the odds, and I am very much aware of this every single day.

Families of SMA is our central resource for support when it comes to handling daily life with this diagnosis. FSMA not only keeps the families all networked together and provide moral support, but they fund critical research to find a cure or treatment for SMA. This year, on November 21st, the Arizona chapter of Families of SMA will host a Walk 'N Roll to Cure SMA in Scottsdale. It would mean so much to us if you could be there. We should have our wheelchair van by then, so both kiddos can show off their pink and blue wheelchairs, respectively.

If you can't attend our Walk N Roll, then consider throwing a few bucks our way via this link. If you represent a business, consider donating your product or a gift certificate for our raffle that day. Register to walk with Team Double Trouble--or submit your donation--by clicking here.

I am confident that a cure (or very effective treatment) for SMA will be discovered in my lifetime. I wake up everyday hoping that a cure for SMA will be discovered in my children's lifetimes. And the best part is that researchers think that a cure for SMA could unlock treatments or cures for other diseases, too--ALS, Duchenne's Muscular Dystrophy, and Parkinson's, specifically. The possibilities are exciting, but we need money. If you can donate money, I will take it. If you have some spare prayers, I will take those, too. Your moral support over the last two years has been critical to my sanity, and I thank you for it very much. As I continue fumbling my way through motherhood--and parenting twin toddlers with a physical disability--your continued support and shoulders to cry on and acting as sounding boards deserve their own awards.

There was a time when I cried over this every day. I don't hardly cry about it anymore--at least, not on a daily basis. Grief is exhausting, especially when my gorgeous children are still alive and happy and bring such joy to my life. I'll save the tears for later when I really need them. For now, though, life is good.

Much has changed in two years.

This is SMA

Today some of my favorite people are blogging together, simultaneously--it's an "SMA Blog Party". Of course, there isn't much to celebrate. Severe physical disability isn't the stuff that balloons and clowns and ponies can fix (or is it? hmm). I wish I didn't have a reason to know these people, though. I mean, they are perfectly fine individuals--awesome people that I'd be lucky to be friends with under traditional circumstances--but I only know them because our kids suffer from the same terminal illness.

That figures, huh? "Great to meet you, but I'm sorry I have a reason to meet you at all." That's what I tell other parents of kids with Spinal Muscular Atrophy. I admit, that sounds pretty shitty of me to say, but they always understand. They understand because their other, pre-SMA friends don't. And they don't want to know me anymore than I want to know them. I don't want my kids to suffer a debilitating and ultimately terminal disease anymore than they do. But you know, if wasn't our kids then it would be someone else's kids. So I set aside my typical "woe is me" point of view for today.

You want compelling? Read about Andy here.

If you want to learn more about SMA, certainly visit ByrdsForaCure.org. Look for the new video I posted from our recent MDA telethon segment.

If you want a tiny slice of life with kids in wheelchairs, visit this gem of an article.

I was granted the opportunity to guest post on my friend Debi's blog, in honor of today's SMA Blog Party. Check it out.

What I'd really love your support in is regarding the Jimmy Johnson Helmet of Hope contest. You don't have to create a profile or log into anything. Just click here, scroll down to select the Gwendolyn Strong Foundation, enter the security phrases, and hit enter. Piece of cake. You can do this daily until the end of September.

Gwendolyn is the same age as my twins, but she has a more severe form of SMA. The amazing thing is that the same cure will help all of them, and a cure for SMA may unlock cures or treatments for other diseases, too. So do me a solid and vote to give the Gwendolyn Strong Foundation $20,000. All the money goes towards funding cure-focused research. The research community agrees that we're on-the-edge-of-our-seats close to a cure for SMA. Let's make it happen, my friends. Click, vote, and let me know that I can add you to my long list of people who support the Byrdlets and our other SMA friends.

SMA: A Mom's Perspective

It's been exactly eighteen months since the twins' diagnosis of Spinal Muscular Atrophy type 2.

At first, I would cry daily--multiple times, daily--over the injustice of this diagnosis. This went on for months. Maybe a year? I was pretty good at timing my breakdowns properly, so that they weren't terribly inconvenient. Always happened when I rocked them to sleep at naptime and bedtime. There's nothing like nursing your baby in a rocking chair in a dark room while a lullaby plays to make you imagine what life without him, or his twin sister, will be like one day. I know that none of us know what the future really has in store for our children, even our perfectly healthy and normal children. But to be diagnosed and then watch a progressive disease become more and more debilitating in your baby.... I don't know. I don't have experience with other awful childhood diseases, but I might suggest that SMA is among the worst. It's like, "hey the good news is that your kids qualify to Make A Wish. And the bad news is that your kids qualify to Make A Wish." Thanks, but no thanks. I just want to drive my kids to a park and let them play. I don't want to have to research wheelchair accessible parks and then coordinate to make two trips to this park (because I can only transport one wheelchair at a time) so that the kids can be "normal" and ride up the ramps that the other kids are running up. And make no mistake, their wheelchairs are $65,000 machines that I have had to fight tooth and nail for and fight through the bureaucracy and withstand my impatience and beg and plead and wait for. And I'm still waiting on Kyle's chair. I'm just talking about Lauren's. And don't get me started on the shit I've wrecked around here just trying to move her chair around, in and out of door frames.

Anyway, for the first 12+ months, I really couldn't talk about their diagnosis with people. With doctors, it was one thing--an exam room is a very clinical setting where I can detach myself. But in a public setting, or even just sitting in my hairdresser's chair....those were the worst moments. I prayed that nobody would make me explain that my two babies are not normal, and that they have a terminal disease and have already beaten the odds because they've made it past their second birthday and that we take no healthy day for granted. But now? Man, I can't remember the last time I cried. Well, yes I can--Logan's would-have-been birthday (he would have turned 5 years old last month, if he hadn't passed away 11 months ago), and several other children this year who died of complications of SMA, I cried for them. But I can't remember the last time I cried over the future of my own children. It's been months. Wow, what progress I've made. (Can this really be considered progress? I've stopped my daily breakdown over my children's fate? Let's save that talk for some other time, though.)

A lot of it is from coming to terms with the fact that the twins continue to stay so healthy. They don't really "look" like they have a disease. They've had few respiratory problems in their lives, overall. Another reason that my tears seem to be drying is that I've watched other families struggle with far more critical SMA-related circumstances and I realize that I don't have much to complain about here. Our "troubles" are more "inconveniences"--trouble with weight gain, the occasional cold, trying to procure wheelchairs and a minivan to transport us all, etc. Our daily challenges here do not typically include life saving measures, like they do for some SMA families. We don't yet have to have an end-of-life morphine strategy, or DNR documents prepared or anything like that.

You know, I used to work in IT before I got pregnant with the twins--I was a real person, who worked in a real cubicle for a real company with a real 401k plan and a real cafeteria with really crappy yet cheap food. I think I feel like pointing all this out because SMA took me by such surprise--you probably don't know what is SMA is, and neither did I. I used to be just like you. But ignorance doesn't make SMA go away or make babies stop dying. But let me just say that if ever there was a use for MS Project, it's when you become the parent of a child with a chronic disease. Let's assign "achieving acceptable bi-pap settings" as a "work object" and assign resources to it, right?

Before I get further off-track, let me drive home my point: I want to make sure that everyone who knows me understands what SMA is and how it affects my family. I want you to care. I want you to ask me specific questions. I didn't before. But now I do. And the good news is that I probably won't even cry now when I answer your questions. But not asking questions? That makes me think you don't care, which in turn makes me not want to share things spontaneously. I consider myself an attention whore when it comes to my kids and their disease, but I try not to invoke SMA into conversations with non-SMA families unless someone else approaches the topic first. But really, don't be afraid to ask questions now. I might even keep my sense of humor when I answer.

What you might be wondering: Yes, they are taking medication that we think helps them. Yes, they are pretty happy children. Yes, I do imagine what life would be like if all three of my children could walk. No, there is no therapy or cure for SMA. No, SMA doesn't affect their intelligence--they may even be smarter than you (but not me--I am their mother). No, they cannot sit up on their own, or crawl, or roll over, or walk. They can't even cough, or lift their heads up after a good sneeze. But they can sing, and they can dance in their chairs, and they know the anatomical names for most of their skeletons (that was my teaching) and they can brighten your day over and over again all day long just by being in the same room as you.

So there's my point. I don't cry everyday anymore. Can we please cure this disease? And then we can talk about unicorns and rainbows and hockey and golf. (My obsessions with hockey and golf are my escape, in case you haven't already guessed.)

Thanks for listening. And ping me if you want to golf. I'm always up for golf. Unicorns too. And hockey tickets (the free kind).

Byrds For A Cure

The Byrd twins have made it BIG. They have their own logo, blog, website, Facebook fan page, and Twitter account. They also have their own tax id, but it's not presently 501(c)(3), so I won't bother elaborating on that yet.

For those of you new to my blog, Spinal Muscular Atrophy is a degenerative and terminal neuromuscular disease. There is no treatment or cure for SMA. My twins (Kyle & Lauren) have SMA type 2 and are two and a half years old. Their big sister, Jenna, is 6 years old and is SMA-free.

Our good friend, Guy Saenger, designed the website for us. He's brilliant, not to mention funny and clever and Good People and also another parent of twins. His company is Avacata and based in Fort Worth, TX, and I heart him. And his wife, Heather. xoxo to you two!

If you're reading this and have the inclination to do so, please consider clicking over to byrdsforacure.org and signing the guestbook!

My next project. Ahem--I'm talking to YOU, PVUSD.

My pregnancy with the twins was a high-risk pregnancy, and I fought to stay pregnant with them for thirty-six weeks and four days. I did this through enduring 11 weeks of bedrest, managing well my gestational diabetes, giving up general possession of my body (sigh), and taking the medications my doctors told me to take.

My twins' diagnosis of SMA was even tougher, but I fight for them by taking them to the best specialists around, in-state and out-of-state. And I fight for them by adhering to a strict germ-free lifestyle and by subjecting them to daily respiratory treatments and yucky-tasting medicine.

My twins needed expensive respiratory equipment last year--which our insurance company deigned to pay for--but before I could fight hard for that, the manufacturer donated it. All of it. Muchas gracias, Respironics.

My twins deserve power wheelchairs in order to move themselves. Studies show that toddlers are developmentally able to manage powerchairs as early as 18 months of age. We applied for their powerchairs at age 24 months, and I fought for eight months to get Lauren's powerchair. Kyle's paperwork was inexplicably put on hold by our insurance company, so the fight for his chair is just beginning.

My twins qualify for an amazing preschool curriculum offered by our state/school district. I met the staff there and toured the facility on Tuesday. The twins will love it! The adults-to-children ratio is 1:5; the classrooms offer challenging yet developmentally appropriate centers and activities; school bus transportation is included for free--including for wheelchair-bound children; there are physical, speech, and occupational therapists for every classroom. There are even a handful of "typical" children in each classroom to act as models for the rest, and those kids pay handsomely for the education and experience.

However.

Sigh.

This special-needs preschool has two playgrounds, and neither of the playgrounds are wheelchair accessible. Yes, the school built especially for special-needs kids, with a playground designed by their physical therapists, excludes my children from participating in that activity, and by design. The playground, where arguably some of the most important preschool social experiences take place, is off limits to my babies due to their physical limitations. Wha...?

You want a fight? I can give you a fight. Are you familiar with the roar of a mother lion?

I fought for months to bring them into this world. I fight the daily fight to keep them happy and healthy. And--oh yes--I shall fight to get them the accommodations they need to board that playground and play with their friends. Reference this info from the University of Colorado and this useful advice from Kompan, a playground equipment manufacturer.

Paradise Valley Unified School District? Get ready. I am a whole-lotta woman to deal with, and you're about to meet me. You have 6 months.

It. Is. On.

Sponsor A Mile for Gwendolyn

First--please consider dropping ten bucks to sponsor one of Gwendolyn's miles. Her family is trouping all over America this summer to raise money for SMA research. Their goal is to raise $50k, and they've raised well over $35k. Go find where you wrote your PayPal password (or just go get your credit card) and donate at sponsoramile.com. Gwendolyn has SMA type 1, and her parents have proven tireless in their efforts to advocate for her health and quality of life, and are excellent role models for the rest of us.

So, the babies have had some doctor appointments lately. They saw their GI doctor, who is concerned about their growth and nutrition. We're working with a nutritionist to try to cram a lot more well-balanced calories down their throats, but things aren't going so well. Every idea involves the twins drinking Pediasure or some equivalent, and they refuse them all, no matter the flavor. It is disheartening. I wish I could explain to them what a G button is--an invasive, surgically installed feeding tube--and explain that this is what we'll be forced to consider if they do not start eating better. Lauren is something like the 3rd percentile for growth, and Kyle is at 0 percentile. This means that 97% of Lauren's female peers and 100% of Kyle's peers are bigger than they are. It might just be mildly concerning if they were otherwise perfectly healthy, but fighting against the side effects of SMA most efficiently requires a good nutritional foundation, which these babies clearly do not have. It's been a bummer lately. Blah.

Kyle's also seen his orthopaedic surgeon. I love Dr. Segal. He loves kids, and seems to like parents pretty okay, too, which can't be said for lots of doctors. But he had to give us the bad news that Kyle's hip is dislocating. The muscles can't keep it in it's socket. Thanks to SMA. Seven months ago, his hips were about 30% dislocated (subloxed). Last week, they were 70% subloxed. So long as it it's not causing Kyle pain, the plan is to do nothing about it, since he'll never walk anyway. There are surgical solutions to this problem, but Kyle would have to be a bit older before we consider that. We know at least one other child who has had this surgery, and it doesn't sound like a picnic.

As for wheelchairs, we are currently in appeal #1. I don't want to think about it too much because I am seriously going to have a coronary over the stupid emm-effers at the insurance company, but suffice to say that the decision makers there are quite certain that the twins are impossibly young to be able to manage their own power wheelchairs. But they are willing to buy them a stroller. Oh, really? You'd prefer to give us a $1,500 special needs stroller instead of the $25,000 (times two kids) Permobil K300 PS Junior? What a shocking turn of events. Whatever. Readers, just don't expect me to update super often about the status of all this equipment. I'll let you know when we start the next appeals process, etc, but thinking about the minutiae of this complicated bureaucratic nightmare is taking minutes off the end of my life.

Our beloved physical therapist, Gayle, loaned us this Tumble Forms chair to try out with the twins. She delivered it to us yesterday, and we are in love. We're ordering our own versions (for the lowest available price of $239) for the twins, and I, for one, am thrilled. I feel like I spend half my life sprinting over to the family room to pick up a slumped over twin--a chair like this would allow them to play on their own and be belted in enough to keep them safely upright. It's almost as good as winning the lottery.

Okay, that's all for now. Don't forget to sponsor a mile for Gwendolyn. Peace out.

Successful first day of carnitine/valproic acid

I've been having some anxiety about how cooperative the twins might be when it came to taking their new meds, prescribed by Dr. Swoboda. Fortunately, it's not been bad at all.

The carnitine is a clear liquid, and only 1/2 teaspoon at a time surely is manageable. And while it's no white chocolate raspberry cheesecake, it certainly tastes better than most medicines, I'd say. They put up a little protest, but both managed to consume nearly 100% of the dose.

The valproic acid comes in little capsule. Since the babies are too young to swallow it whole, we just open the capsule and sprinkle the contents into a spoonful of food. Today, I put it in their first spoonful of oatmeal. And they ate it (suckers!). Just like that, they've had their first doses of carnitine/VPA. I only hope they're as cooperative tomorrow...

It may help strengthen them, or may do nothing. Time will tell. They'll be re-evaluated in 12 months to determine whether we should continue it.

More info: http://www.fsma.org/UploadedFiles/Research/Publications/CurrentPublications/SMA-compasssumm08.2[1].pdf

Update on the babies

It's been only a month (!) since the babies were diagnosed with SMA. Boy, that day feels like a year ago, not just a month. I thought I'd update on what's going on in our little corner of the world.

Since SMA affects the muscles, there are a variety of concerns and precautions to be taken to preserve their health, now and in the future. We have a half-dozen specialists involved in their care now. Whew! We need to find a house closer to Phoenix Children's Hospital!

They see a pulmonologist now, because when an otherwise healthy SMA patient gets a cold and secretions can't be expelled from the lungs completely, infection sets in and it can be deadly. We have on order a machine called Cough Assist, which uses positive and negative air pressure to force a productive cough, which I'd like to think we'll never have to use, but I imagine we will. The pulmonologist also has ordered sleep studies on them, to monitor their oxygen levels at night while sleeping. Something about if they aren't breathing deeply or adequately at night, it can cause the lungs to not fully expand and affect the way the chest cavity develops as they grow. (Or something like that.) They may need to sleep with something called a bi-pap machine, which I don't know much about except that it looks sort of like a c-pap machine that my father-in-law used to have to use at night.

The babies also see a pediatric gastroenterologist, to monitor them for feeding issues. As the mouth/tongue/throat all rely on well-funtioning muscles to keep food going the right direction, any weakness in these muscles could cause them to aspirate liquids. And if they can't give a good productive cough to expel those liquids, then again there's a risk of infection, which could result in a hospital stay and can certainly be deadly. The GI doctor has ordered swallow studies on the babies, where they consume barium and the act of swalloing is monitored on fluoroscopy. This way, they can see exactly how well the tongue and other muscles are serving them and we can address any issues before aspiration becomes a danger.

The babies have an appointment with a pediatric orthopaedic surgeon next week. Evidently, curvature of the spine (scoliosis) is a certainty for SMA patients. It makes sense if you look at Kyle and Lauren today--when they sit, they are not strong enough to sit up straight, so they sit hunched over all day long. After a couple years of this, it's a permanent problem with the spine, and next comes spinal surgery to try to correct it. So we expect that the orthopaedist will prescribe back braces for them (there better be some pretty ones out there, because I am not dressing my sweet babies in ugly black equipment! Maybe someone makes back brace covers on eBay...).

As for their current state, they continue to progress very slowly. Lauren can push herself backwards in her walker several feet over a period of a few minutes. She can roll over all the way to her tummy and push herself to her back again. Her language skills--both verbal and sign--seem like they're just exploding (though the therapists assure us she is not ahead of that curve, just right on track!). Kyle still struggles to sit on his own for very long, so he spends much of his day in his Bumbo seat, in his toddler recliner, or in a special foam seat that the physical therapist concocted for him. In addition to "grandpa", Kyle's current favorite word is "play". He always wants to play! From the moment I get him from his crib in the morning, to the moment I bring him upstairs to put him to bed, he says, "Play! Play!". The kid loves his blocks. The babies' current favorite activities include block stacking, button pushing, little cars that they can vroom vroom back and forth, and anything Elmo or The Wiggles. Lauren is on track, or a little ahead, when it comes to fine motor skills. Kyle struggles to catch up, but he's getting there. He loves to please us and impress us with the number of blocks he can stack on his tray. And Lauren is just a joy to be around! I have a video clip to upload of her, when she sees our lit Christmas tree for the first time. She's just a doll and such a riot.

We'll be talking to the doctors about getting them their wheelchairs in December. Even if I don't take them everywhere with me in their wheelchairs, even if they're just something to use at home only for a little while until we absolutely need them all the time, it will be good to have them. We don't have many safe places to put the babies since they don't sit very well. Even in their high chairs, if they lean over and down the side (for some reason they like to do this all.the.time!), they can't right themselves and I have to come over and lift them back up into sitting position. It drives me crazy, and they know that and seem to revel in the thrill of my scowl. So I'm looking forward to getting their wheelchairs. Even if it means we need a one-story house now, in order for them to get around on their own, eventually. I hate thinking about that, because I love my house and my neighborhood, and Jenna starts kindegarten next year and her school is just 5 houses down the street. Sigh. Oh well, I know it will work out somehow.

We all, except Chris, got our flu shots this year. And I'm to keep the twins' exposure to the weather and to the public to a minimum. We don't want to risk them catching a simple cold and have it develop into something bad like pneumonia. Chris didn't get a flu shot because a) he wasn't there, b) he travels so much that if he's exposed to the flu, it likely won't be the strain against which he was innoculated, and c) he's in the flu-shot-is-hogwash camp anyway. He knows that if he catches the flu, he's going to have to camp out in Jenna's plastic playhouse outside, or check into a hotel or something because I will not have those kinds of germs in this house! Not with these babies who are so susceptible to a cold turning into so much worse.

So, that's it from here. We're having fun getting ready for the holidays. Choosing gifts for the babies this year is a challenge, since they have such physical limitations. But somehow I'm managing to come up with some good ideas that they'll love (hello, TMX Elmo). And I'm all but finished with our Christmas cards--neener neener neener.

Happy December to all!

Spinal Muscular Atrophy

The babies were diagnosed with Spinal Muscular Atrophy (SMA) type 2 this week. It's pretty devastating. But I think it's better to know what we're dealing with right now, rather than them being "medical mysteries", right? I'm so glad we have Phoenix Children's Hospital to work with. They've been great so far. In the past, with other random issues and doctors, it seemed like we managed to slip through the cracks in terms of test results and call backs and such, but this has absolutely not been the case with PCH. My only complaint is that PCH isn't closer to my house! But actually 20 mins drive isn't that bad. And it's awesome that their facilities are so kid friendly--we had to bring Jenna with us to an appointment and she felt right at home at a coloring table watching Sprout, so that was a relief.

If anyone knows of tactics I can use to convince my husband that we now more than ever need a big family trip to Disneyland, please share! I want to go now, while the babies are still small enough for our normal double stroller and we don't have to travel with wheelchairs or other equipment.

I could post so much more about SMA and the ramifications, but I have no energy left. I've been talking about it and crying about it for four days now and I'm all out of words and tears for now. Many, many thanks to anyone who includes us in your prayers.

And in honor of Halloween tomorrow, here are a couple photos to get you in the spirit of the holiday--enjoy!