Concerto in the Key of SMA

While we don't focus on it, SMA takes away quite a bit of what one would consider "normalcy" in life - including the ability to be independent.  Taking Lucy anywhere is a production with two people; doing it with one person can be intimidating.  Fortunately, if I am told that I cannot do something, you bet your sweet asterisk that I will find a way (or goad someone into helping me!)

Walks with Lucy are one of the things that I feel relatively comfortable with, at least as far as SMA is concerned.  She can hold her own respiratory-wise and is pretty good at controlling her secretions.  We take along her pulse-ox and suction, and an ambu bag just in case - we've never had to use it, thankfully.  Other variables (stray dogs, creepos) are less under my control, but what's life without a few calculated risks? :)

So Lucy and I ventured out on our own tonight, to a park that holds summer concerts bi-weekly.  It's a goodish walk from our house, but not so far that I dread having to walk back once it's done.  The weather cooperated and it wasn't sweltering; in fact we needed pants and Lucy made good use of her blanket!

Like any toddler, she was pretty impatient.  We got there while they were still warming up, and she didn't seem to think that they needed to play the same few notes over and over.  They launched into their first song (it was a symphonic orchestra, by the way) and Lucy sang along.  She loves clapping at the end of each piece - but the introductions?  Not so much.  The first time was pretty close to a full-on tantrum as the conductor spoke, but the next few pauses she just hummed peevishly.

We both had a great time!  The lady who was so kind to Lucy last year that she brought along a teddy bear for her on the teddy bear parade night was there.  She came over right away and said she had been looking for us and was wondering if we would come!  She bent over to say hi to Lucy, and Lucy beamed at her. :)

There are many things I cannot do with Lucy - but how could I think of those things while there are nights like these to enjoy?

Like that smirk?  She smiled for one second 

when I held up the phone, and I wasn't quick enough, 

and this is the most she would give me after that.  Stinker.

Lucy Loves

Some things that can usually, on any given day, produce the shining smile you see above:

- asking her if she wants to brush her teeth
- hearing the title or seeing the cover of one of her favorite stories
- talking to Daddy on the phone
- talking on the phone in general
- her bipap
- hearing how pretty she is
- hearing how smart she is
- hearing how wonderful she is
- hearing how sweet she is - if daddies have an irreplaceable impact on their daughters' self-esteem, Lucy will be running for Miss America and President when she is 18, if not sooner
- the theme song to almost any NickJr show, whether she likes the actual show or not; Pocoyo is a current fav
- stretching and rubbing of her feet after a day in AFOs
- being told it's bath time
- asking her if she wants to go on a walk
- helping her dance with the Gabba gang (go, go, go Lucy!  do the Lucy!)
- me whipping my now long hair at her
- bedtime hugging of her dolly
- having songs sung to her (Old MacDonald and any Ernie tune are at the top of the list)
- me biting off her fingernails and spitting them out (I'm still afraid to use a clippers!)
- the end of her bedtime prayer, when we say "Please help us cure SMA, and please help us... understand... Lucy!"
- asking her if she wants some ice cream, or juice
- biting her cheek and making "nomming" noises while telling her that's what tigers do (new today!)
- asking her if she wants to "fly!" (tossing a SMA kid in the air sure has built up my upper body strength!)
- me shrieking into her chest during dance time
- telling her "no" (we're working on that being less funny and more discipline-like)
- this song
- telling her she made a good outfit choice after she picks one out
- us sniffing her hands and telling her they're stinky
- falling asleep - she closes her eyes and starts smiling and squealing softly
- Sesame Street!
- asking her if she's happy

Things that tend to tick Lucy off, causing tantrums of sometimes epic proportions:

- us not doing something fast enough for her - not grabbing a book immediately when we ask if she wants to read or not turning a page promptly, mostly; or mentioning something she wants and not immediately producing said thing
- nasal suctioning - we don't do deep-nasal suctioning, this is just generally booger removing; guessing she wouldn't be a nose-picker
- being woken up before she wakes up of her own accord (doesn't it annoy everyone?)
- not letting her immediately talk on the phone when she sees me (or hears me) on it; the phone has become a big thing for her lately
- quarterly blood draws - Noni is the best phlebotomist on the face of the earth, and Lucy hates her with a passion
- Daddy brushing her hair - ouch!

So she's a pretty typical toddler.  Besides the machines, being almost completely paralyzed, and not having the ability to talk.  Inside that sweet, beautiful, immobile body - the body kept alive with bipap, cough assist, pulse ox, g-tube - there is a regular toddler.  One who is usually happy, sometimes tired and cranky, sometimes impatient and belligerent.  One who is sassy and sweet and determined, and one who gets frustrated.

One who fights.

A Thank You and a Clarification

Anyone who read my last post could probably tell I was a bit perturbed.  I get hotly defensive regarding Lucy, and I have a bad track record of not being able to silence the nagging voice that says "Just say something... just say something..."  And the internet gives me the forum to say those somethings.

Thankfully, it also gave me the opportunity to contact Avery's dad, who so graciously listened to my rambling explanations about why this was haunting me, and very reasonably agreed to change the wording on his blog, which is getting some real exposure.  The initial post now does not include any statements about quality of life for children with SMA.

I also wanted to clear up - for those who read this blog but do not have access to my Facebook page - that I was not trying to make any accusations regarding parents who choose hospice care for their children.  As I said on my page, I will not make absolute statements about any path but the one we chose, and then I can only attest to our own personal experiences.  That being said, do I think all parents are only thinking of their children when making the decision of whether to go with hospice or something more aggressive?  No, and that goes both ways.  We see day after day in the news horrific stories of what some parents are capable of.  I know this world is not full of parents who only take their child's well-being into consideration when making life decisions.  We are all human.  Even special needs parents.  Just a few weeks ago, I read three separate stories about kids with cerebral palsy whose parents killed them, in various ways.  Obviously we can't say that all parents with special needs children are doing what's best for them.

I do believe, though, that the majority do what they think is right.  Whether that is to pursue treatments or to let their children go is not up to me to decide - I am responsible for Lucy, no one else.  It's not my place to make those calls, or judge them afterwards.  I am not the one who has to live with the outcomes of those choices, so it's really none of my business.  I just do my best to share hope, because that is what I know. 

I'd like to leave you with another blog written by a truly well-spoken mom to a SMA angel.  She weighed in on this debate as someone who has experienced the loss of her child, and what this debate over palliative/hospice vs. non-invasive/trach has meant to her.  Thanks Audra, for always speaking your heart.

If you find it in your heart to help us fund research that has the hope of being lifesaving and life changing for children born with SMA, please check out the "HOW YOU CAN HELP" tab located at the top of this blog.  There are a few charities that you can donate to, and the money donated will help fund programs that will change Lucy's life, and the lives of many living with and those yet-to-be-born with SMA.

R-E-S-P-E-C-T - This is what it means to me

Time to dust off the blog.

Lately I have been seeing more parents of children with SMA talking about quality of life - specifically, newly diagnosed families blatantly stating that children with SMA do not have a good quality of life.  This is disturbing to me, to say the least.

If you are one of the 35 people who will probably read this post, you know Lucy and know that the previous statement couldn't be farther from the truth.  But for the thousands of people who are reading posts from blogs like this - where it says "In some cases children like me can survive with the dependency of a respirator, but their quality of life is not very good and more often than not, the life expectancy is still less than ten years of age and even lasting past two years of life is not guaranteed" - they aren't exposed to a Lucy, and will not know any different.  They will think that an extended life with SMA is all doctors appointments and crying and misery, and "prolonging the inevitable."  

Incidentally, is anyone guaranteed a long, healthy life?  Because you are born without SMA, are you guaranteed to survive past two years old?  Shouldn't you just stop eating now - you're only prolonging the inevitable?  Death is inevitable for all of us.  Life is terminal.

I do my best to keep my opinions - because that is what they are, opinions - to myself, out of respect and compassion for other families.  Not everyone chose the road that we did, and it is not up to me to decide whether their choice was the right one.  But if it is offensive for me to say that I think people may choose a palliative (read: hospice) route because they are not willing to put in the effort, or because they think they may have to alter their lives too drastically, isn't it equally offensive for them to state as gospel truth that children like mine have a poor quality of life?  Both statements are making assumptions regarding a life they know nothing about - yet one is appalling and one is acceptable?

I ask - who has determined that these children have a poor quality of life?  Their doctors?  Nurses?  Grandparents?  Neighbors?  Parents?  Or the children themselves?  Sadly, since most are diagnosed so young, we can't ask them how they feel when we get the news.  We don't know what they would want for themselves.  We can only do what we see to be in the best interests of their health and happiness.  What I see in Lucy is a happy girl.  Is she happy all of the time?  No.  Is your "healthy" child happy 100% of the time?  

Will there be hardships ahead for her, and for us as her parents?  Most likely.  Have our lives had to drastically alter?  Yep.  Do I think that I made the wrong choice?  Hell no.  I will make any changes necessary to my life to help her live hers.  Does it look like I am selfishly "prolonging the inevitable?"  What do you think?

If people really want to make a difference, they will get the message out there with a link to an organization that is FUNDING LIFESAVING RESEARCH - something else that blog misrepresented.  There is in fact clinical research going on, right now.  Take your pick of the sites below if you *really* want to make a difference.

The Gwendolyn Strong Foundation 

Miracle for Madison and Friends  

I have respect for parents' choices for their child, though I may not agree, and would not presume to say all parents who choose hospice are lazy and don't want the trouble of caring for a special needs child.  I expect parents who choose hospice to have that same respect for families who choose to fight, and to refrain from making sweeping false statements regarding the quality of life for children they know nothing about.

 

Yay for Good News!

When Lucy was admitted at Children's Hospital in Milwaukee in September of 2009, we were completely blindsided and overwhelmed.  We had no idea what to expect for her, but the prospects were grim.  She was subjected to countless blood draws, catheterizations for urine samples, muscle testing, and an MRI.  We left with tons of questions and few - if any - answers.

While they refused to verify in any specific terms what they were testing Lucy for, someone did mention an anomaly in some bloodwork that Lucy had done.  The culture had come back positive for trisomy 8 mosaicism.  They wanted to do yet another blood draw, because it was possible that it was just an abnormal culture.  After watching Lucy get poked and prodded (mostly unsuccessfully) for three days, we declined.  We had bigger fish to fry at that time, and anything non-life-threatening was cast aside in the concern department.

Immediately after Lucy's SMA diagnosis, it was the farthest thing from our minds.  We had so much to learn in a very short time to be able to care properly for Lucy.  But as time progressed and Lucy stayed stable, we started to wonder.  As she got older, it was hard for us to try to teach her something and, seeing her disinterested or flat out ignoring us, not question whether it was a an issue with will or skill.  Since she's virtually immobile and non-verbal, it's hard to tell what she's capable of - and whether or not it's just toddler attitude that is keeping her from doing what we're asking her to do.

We discussed it with Dr. Schroth, and she pulled up Lucy's diagnostic paperwork and sent us to the Waisman Center in Madison.  We took Lucy in three weeks ago to talk to them about pursuing whether or not trisomy 8 mosaicism was something Lucy had in addition to SMA.  To get a more accurate testing, they had to do a skin biopsy on Lucy's leg, which she was not at all happy about.  She was irritated at the numbing shot, and when he was pinching her leg to see if it had yet numbed, she shot him the nastiest look I have ever seen her give anyone.  The biopsy itself was painful for her, but she was okay by the ride home and has not complained once while we've been caring for the site post-op :)  In fact, she laughs when we pull off the band-aid for the daily cleaning - my girl is crazy!

Waiting for the test results was a bit like waiting those two years ago, albeit on a much smaller scale.  I even forgot we were waiting for results for a day or two here and there - back then I did not, though I tried very hard to.  I wasn't sure how we would proceed with Lucy's care and therapies if she was diagnosed with the trisomy, but we would figure it out, just like we figured out SMA.

Today someone called and said that Lucy's cells had not grown a single trisomy, which means we can be reasonably sure that the initial culture was a false positive.  I didn't realize how concerned I was about it until I felt the relief when I heard her words.  While we would have been fine, and Lucy would have been fine, I'm glad that another area of our lives doesn't have to be adjusted.  Now I know that it's pure stubbornness when she's not cooperating or communicating, and I plan to react accordingly ;)

D-Day Deux

This is Lucy, with the outfit she was wearing on the day she was diagnosed with SMA.  The same day she was diagnosed - October 14 - two years later.  It's incredible to see how big she is now - or rather, it's incredible to see how tiny she was when we found out.

What's not incredible to me is that she is still with us, despite the dire outlook the geneticist delivered along with her diagnosis.  I remember standing with Lucy, looking down at her in my arms in disbelief.  I refused to believe that doctor, plain and simple.  She is not God.  And for her to have the audacity to dare to tell me how long my child will live is shameful, in my opinion.

I don't think it's fair to say that Lucy is alive because of our prayers.  Not because I don't believe that God has something to do with it - in fact, I think He has everything to do with it - but because I know plenty of parents who prayed long and hard to be able to keep their sweet babies, but ended up with different outcomes.  And it's not that I don't pray for Lucy to have a long and fulfilling life.  I do.  But it's not up to me.  I'm just glad that for now, my hopes for Lucy and God's plan for her are on the same path.

Two years post-diagnosis, Lucy has lost most of her movement.  She can move her limbs fairly effectively when suspended, but cannot lift her arms off of a surface as she once used to be able to.  She can still go for very long stretches (and sometimes take naps, tsk tsk, I know) without her BiPAP and maintain perfect oxygen saturations.  When she chokes on her secretions, oftentimes she can self correct if we get her flat.  After a tantrum (and we see enough of those, HELLOOO terrible twos!), she doesn't need me to cough her or get out the oxygen for her because she can't recover.  And she still has quite a few facial expressions in her repertoire, including a beaming smile. 

Every day I thank God for making her so strong.  Every. Day.

Given the circumstances, that may sound funny.  She can hardly move, but she's the strongest person I know.  She can't cough on her own, but she's tougher than a lot of adults - and whines a lot less than some, too.

Every day we share with her is a blessing.  I don't say that because we think our days will be limited.  We don't.  Every day that *every* parent shares with their child should be viewed as a blessing.  We are just fortunate to be more aware of that fact.

Glory be to God.  And in your face, SMA.

Anniversary #4!

Thank goodness for Facebook reminding me our anniversary was coming up last week!  Our day-to-day lives are so busy, those things creep up before you know what's happening.  Four whole years of being married to a wonderful man who I'm blessed enough to be able to call my husband.

On our anniversary, we saw each other for about half an hour, total.  No special date, no private romantic dinner.  No problem.  With our lives being the way they are, I don't expect - or miss - those things.  Really.  We made a choice when we became parents, to put our child first in all things, and for us, eschewing traditional anniversary celebrations falls right in line with that.

What did we do on our anniversary, you ask?  Lucy and I spend the day (part of a long weekend, actually) at her grandparents' house while Noah worked diligently on our house - specifically, our bedrooms.  Yes, plural.  Lucy is well on her way to getting a bedroom worthy of Lucy-habitation.  Since we had problems with our roof this spring and summer, there was some water damage done to the ceilings in both bedrooms, and that had to be addressed.  Thankfully, it wasn't major damage; nothing a can of sealant and some spackle didn't fix.  And fresh paint, of course, in fun new colors :)

That is my anniversary present: another room to inhabit during winter isolation.  Up from one, this is going to be like a luxury suite for us!  Better than any diamonds or dinners could ever be.  We get downstairs as much as we can, but if Noah is sick or around someone sick at work, we try to stay away from him.  Unfortunately, since there is no shortage of sick people in the winter, we spend quite a bit of time upstairs, keeping Lucy as far away from the germs as possible.  And now we have two rooms to do that in!

So happy anniversary to my wonderful, hardworking husband.  I won't say tireless, because he is very often tired, yet he works hard anyway.  He works to give Lucy everything he can, and to keep a smile on her face.  He works to relieve my stress and make my life enjoyable.  He is amazing, he is ours, and I look forward to spending the rest of my life with him.  I love you Noah.