Monday, April 14, 2014

He's Still Alive

Ben had an assignment to write his autobiography. I found it fascinating to glimpse his perspective. Plus, he can really turn a phrase. Due to illness, Ben missed a lot of in class editing time, so his final draft was still a bit rough. I sat down with him and typed up what he had, correcting some of his minor factual errors, and giving him the chance to expound on the details free of the weight of mechanics.

He gave me permission to share the final product on the blog. I've removed most proper nouns.

“He’s Still Alive” -By Ben

My first breaths were on [date] in [hospital]. When I was only one day old, my heart apparently really wanted to see the eternal city because it stopped working for awhile, but it got better. I arrived three weeks early. My mom was a teacher and my dad worked as a medical tech in the operating room. Then I came and my dad needed a new, better paying job to afford my needs. So he became a nurse and he had special help in the form of a very sick infant named Benjamin.  

When I was a baby, my favorite stuffed toy was a bear named, Tino. He is a white bear about a foot tall with a red bow tie decorated with red hearts. Tino has always been at my side for hospitals and surgeries. 

By the age of one, I learned over 100 words but they were all in sign language because I couldn’t talk because of my trach. When I was three, I got my trach out, along with a bunch of other machines most people don’t need. I still have the scar on my neck. We lived in Florissant but all I remember was the yellow room. I loved trains a lot. I watched Thomas the Tank Engine on television. I had almost the whole collection of wooden and magnetic Thomas the Tank Engine trains, and I went to the train store every other week to play. I also enjoyed the arrival of my brother, Alex. Alex is about three years, three pounds, and three inches behind me. We moved to a nearby town and our dog, Leo became a part of the family.

In my pre-school years sci-fi rockets replaced Thomas as my favorite thing. In preschool I made a plate and decorated it with rockets that had boosters, buttons, lights, levers, grappling hooks, and gizmos I have no name for. I filled the plate with these unidentified colored objects. I still have the plate. Also, Dora replaced Elmo as my favorite character. 

In early grade school, I found out about my muscle disease. Sometimes all my broken parts makes me feel like Frankenstein’s monster. My friends Terrel, John, Elliot, Drew, and I started cub scouts.  Purple became my favorite color and I went to public school.  Later on I liked Harry Potter. I remember playing Harry Potter at recess with straws for wands. The Death Eaters tried to take Hogwarts, which was the tallest point of the playground. The Hogwartians tried to kill all the Death Eaters. I was on the losing side. We lost for two reasons. One, we couldn’t figure out the ever changing passwords and two, we were hopelessly outnumbered. 

I was in the third grade when Papa, my grandfather, died from a heart attack on a boat. I also started to like Legos. Gigi was born. Gigi is a cute little girl who likes the color pink, unicorns, dancing, butterflies, and dancing with pink unicorn-butterflies. 

This year in sixth grade, my grandmother died. I got a fish tank and seven new fish. Their names are Blackfin, Goldfin, Barbara, Gilbert, Cray-Cray, Russle (who died) and Russle Jr, (a replacement fish). I am now going to Catholic school which is cool because I get to love God out loud. This is a really great school year. Also I have started liking Skylanders. We have 21 Skylanders. My favorites are Tree-Rex, Stealth Elf, Eruptor, Ignitor, Trigger Happy, Flashwing, and Wash Buckler. 

In the future, I am looking forward to Confirmation. Also I’m waiting for high school and graduation. My mind is set to go to public high school so I can be with my old friends from elementary school. I also want to play video games on weekends. I’m also going to try to play baseball because it’s the only sport I’m good at. 

I want to go to college and hopefully medical school. I want to be a great nurse like my dad. Afterwards, I’ll hopefully get a nice house, get married, and have kids. One thing I’ve learned in my life is there is always somebody more lucky and somebody more unlucky than you. I may not be very lucky, but I’ve had a nice life. I’m sure that when I am dying only one thought will go through my head. It is this, “Finally, my heart gets to go to heaven.”

Sunday, April 13, 2014

Hibernation's End

Somewhere between the winter weather advisories and the tornado watches we've had a few glorious spring days that enticed us into exploratory adventures.

Basement camping season begins

Jason and Ben just completed a week long camp out with the 6th grade class. It was a grueling schedule of physical tasks for Ben but resulted in lots of team building and support. He wasn't able to keep up for a lot of the activities and came home completely exhausted and a little sick, but he made it through the week.

Gathering for Mass by the Lake

Muddy Face

Jason's Utopia

Thatsa my boy

Our cake hunt has led to many opportunities for learning and fun as well. We've done all the free parts of the zoo, turtle park, World Bird Sanctuary, Lone Elk park, sculpture park, transportation museum, and the (not because of cake and not free) Fox.


Turtle Park

World Bird Sanctuary

Living the cliche

Lone? Elk

At the Fox to see, "Once."

Not my natural habitat
Getting this cake made me tear up a little. It's located at the seminary my late father attended years ago.


Another highlight of the last week, was spending time with my sweet friend, Ann, visiting from Arizona. I think my half-marathon days are behind me, but we keep finding ourselves together during the races we've done in the past. I think it's a sign that we just have to keep on plodding onward one step at a time.

As usual, one morning Gigi asked where all her family members were. Once I'd explained, she replied, "It's just you and me, kid."

We were climbing the modern marble and glass open staircase at the library and Gigi was muttering under her breath, "Don't look down. Don't look down."

We were going through the drive through of Chick-fil-a and I asked Gigi what she'd like to eat. "Hot chocolate with sour cream on top, please," she requested.

Monday, April 7, 2014

Let Them Seek Cake

Our city's 250th birthday is being commemorated in artistic "cakes" displayed at 250 locations of import in the area. The exhibit is called, Cakeway to the West.  Naturally, I want to see them. All. Okay, so maybe that isn't so natural, but I'm obviously not alone since someone has developed an app for that.

So for the remainder of the year, we're going to see how many we can find and photograph alongside a person we know. 250 field trips seems a bit ambitious and many are far flung. With a little help from our friends, we're photo documenting all the pieces we can. Though we've seen and even photographed several before dedicating ourselves to this noble cause, they don't count. So these are our first official cake finds:

Amtrak Station in Kirkwood

Turtle Park


Best of all, the cakes are an excuse to finally explore the oft overlooked gems we've been meaning to get to in our fair city.  I've been feeling like we need to lighten up and play more, so let the Great Cake Search commence!

Friday, April 4, 2014

Diagnosis Dunnosis

Our much anticipated results from Baylor came back today and we're still in diagnosis limbo. I haven't quite made sense of all the big words and 'variants of unknown specifics', and further consultation will be coming, but the gist is non-definitive. It's possible that Ben's neuromuscular disease or mutation is on a gene not yet detected. But because Ben and I shared some variants on the same genes, and because I have moderate symptoms of weakness, Ben's neuromuscular specialist is adding me to her patient load and wants to biopsy my muscle.
La. La. La.

My take on all this is that I sense Ben's disease is progressing, and I've given up hope that we're going to discover that he has a known and treatable disorder early enough to matter for him. I don't think this means all is lost and he won't have a long and enjoyable life. Though I no longer dream we're going to be handed a diagnosis and a bottle of cure, I do feel like a pioneer, gathering potentially beneficial information for the future, perhaps even for my descendants.  I know we've done a fine job of managing Ben's symptoms, accentuating his abilities, and striking a fairly sane balance of seeking answers and help without going overboard.

While one-day-old-Ben was being revived from his first cardiac arrest, I made a promise to God that I would accept His will. At the time, I thought that will was going to be a lot more devastating than it has turned out so far. In that moment, I could never have imagined the Ben that hugged me after walking out of school today. If I could have seen a snapshot of him whiling away the Cardinal's rain delay by discussing alternative novel endings with his brother, as he did tonight, I still wouldn't believe it could be true. I couldn't have imagined his wit, his humor, his intellect, his sheer perseverance. I couldn't know how mothering Ben would change and strengthen me.

When I received this call with the non-definitive genome results, I was having coffee with a bereaved friend who recently lost her son to a non-definitive neuromuscular disease. It was a poignant barb. Not knowing is so hard. Not knowing leads to what ifs and that can beget undeserved guilt. I wish that things could have turned out differently for my friend. After years of bettering and battling, being changed and strengthened by tough love, how does one just stop? I notice there is no word for parents who have lost children. Maybe that's because it is indescribable.

I was struck by the passion in the voice of the doctor that spent a good amount of time on the phone with me today. She was just as dejected, if not more, by the lack of answers. In the last eleven years we've been fortunate to have met a lot of truly caring and wonderful medical staff. I wonder how these doctors deal with the setbacks and losses as they daily battle disease? The good ones are always genuinely invested in their patients and I imagine that leads to emotional fatigue. I'm so grateful to the humane doctors that strive to improve quality of life. It matters. I imagine the callous types are attempting to close their eyes, minds, and hearts to the suffering as a preservation mode. I sort of get it. But I think the adage of 'tis better to have loved and lost, than never to have loved at all' is true. Choose love.

Saturday, March 29, 2014

Feeling Moody

The rest of the pulmonologist's message wasn't so bad, but still perplexing. Ben's sleep study was significant in that he is being awakened several times a night by motor twitches. Kids without unknown neuromuscular disease sometimes do this when iron or electrolyte levels are off, so blood was taken, but Ben's labs were fine. So now what? Is the twitching a tic? Lung doc didn't think so. Are the tics not tics, but whatever the twitching is that he has never exhibited in prior sleep studies? And why the sudden increase in bulbar weakness? Is the sleep disturbance at the root of the migraines and foggy brain? This is what keeps me up at night, folks. That, and the nagging assurance that I should be devoting more of my midnight brain power to other loved ones.

We've always been on the lookout for health changes as Ben approaches adolescence. We selected our house with accessibility as the supreme priority. But all these new issues were not the ones for which I've been keeping watch. By replacing the term dystrophy with myasthenia, our mind frame was turned on end and there isn't a lot of information out there, especially since we don't even know Ben's subtype. If you're curious for a general run through, here's a fact sheet from MDA. 

I continue to fluctuate between grateful and gripeful, wondering if these symptoms are molehills or mountains. As I've spent the last day and a half fighting Ben's latest respiratory crisis, it is snowing on my steep mountain and I'm off the stupid trail.

So we're sitting here watching the new remake of Cosmos, over the buzz of the nebulizer, and pointing out the anti-faith bias of the makers of the show. Sillies, faith and reason coexist, and the Church (aside from a few bad apples) is and has always been a major contributor to learning all we can about the God-given universe. God won't force faith on you, that's called free will, but why not give Him a chance? He's waiting for you and if you've never felt His love, you are in for a treat. We like the compelling and graphically delightful show so far (minus the negative and inaccurate portrayal of the Church), and I think it's a great teaching tool.


Kid Quotes:

Mom: Would you like yogurt for a snack?
Gigi: I have a better idea. I’ll have ice cream with chocolate syrup on top.
Mom: You can have yogurt or a bell pepper.
Gigi: Oh. Yogurt’s my favorite! Thank you, Mommy!

Gigi chose a brown and pink dress. She has brown and pink shoes which I thought would be an obvious contender for footwear. Instead, she selected black patent leather. I convinced her to try on the brown Mary Janes to which she stated, “They are nice, but they are not fabulous.” She went with fabulous. 

Mom: Goodnight my sweet angel.
Gigi: I’m not an angel. I’m just a girl. 

Ah, another theologian is born. 

This one won't be a direct quote because I wasn't there and Jason just worked from 6 am to 2 am, so I'll skip waking him up to ask. Ben laid out all the action figures on the footstool and dubbed it his ottoman empire. 

Alex: Will you sing that song where all the bad presents break?
Mom: Uh, Hush Little Baby Don't say a word. Momma's gonna buy you a mocking bird? I know, right? What kid would want a billy goat?
Alex and Gigi in unison: Me! Me!

Saturday, March 22, 2014

Thanks for Visiting

I've been blogging for seven years straight. All ten readers should be commended for their fortitude. They have hung on through a noticeable lack of plot, random narrative and tense, and the surety of being disappointed by the conclusion. Still, I can't seem to resist telling my stories.

We rounded out our Spring break with a northern jaunt to Iowa to visit Grampa. We helped him out around the house with our many hands lightening the work. We balanced that with a day at the Iowa Children's Museum (the likely source of today's fever). It was good to spend time with Grampa, being useful, and just missing Grammy in harmony.

Flight Simulator

Building an Aqueduct in the the Blue Room

Playing Pizzaria made us hungry for the real thing

Baci sul Naso: Kisses on the nose

When we got home, we'd had a message from the pulmonologist with sleep study results.  He said from a breathing perspective, it wasn't too bad, however...the answering machine cut him off before he could deliver the but. I'll be found humming along to the hold music on Monday.

ENT said Ben has another infection in the operative ear and that the hearing aid is probably contributing to recurrent infection, disappointing audiologists, teachers, and nagging mothers everywhere. Also, swimming is risky business even with the plugs and an ear band-it. Classic quality of life conundrums. Ben's Eustachian tubes do not work normally and that puts the conditions in place for aggressive recurrent cholesteatomas. There is no fix for the organelle so Ben will continue to need regular CT scans to check for masses. We expect the next scan in July.

It is good to have productive and somewhat plan free spring breaks, and it's a relief to schedule medical appointments that don't interfere with school, but one of these years, we're vacationing where the mounds of white on the ground are dunes.

Movie Night

Tuesday, March 18, 2014

Super Powers

Ben saw his neuromuscular doc last week. We were pleased to see his overall strength has improved, which we attribute to the acetylcholinesterase inhibitor he's been taking three times a day since September. However, his underlying disease may be progressing, if that makes sense. His facial weakness is worse and he's developed contractures in his ankles. Though we're still waiting for results from Baylor, it seems very likely that Ben does have some sort of myasthenic syndrome. We've increased the miracle drug to four times a day to see if it helps round out the remaining weaknesses and we've already noted a difference. I'm so grateful it exists and we've been led to it, but really, being dependent upon a strength serum reads like a comic book plot. He'll go back to wearing splints at night for the ankles. Not a big deal, just. one. more. thing.

The other sorta ugh thing I've not really been vocal about is Ben's brain. Concerns first arose at doctor and therapist appointments when Ben was maybe six, but were dismissed because of his tremendous gains and awesome verbal skills, plus neurological deficits have always been expected given Ben's traumatic start in life.  So when he would exhibit unusual or repetitive or forgetful behaviors, we'd move on to frying the bigger fish. New developments, or lack rather, forced us to look more closely. 

As usual, Ben doesn't exactly fit into a typical classification like High Functioning Autism Spectrum Disorder (the autism formerly known as Aspergers), but he shares many similar traits. It is likely that cardiac arrests and multiple doses of anesthesia have contributed to neurological changes along the way, making short term memory, processing, and sensory awareness fuzzy. 

I haven't decided how I feel about it, if it matters. The news really doesn't change much. In researching ways to help improve organization and develop healthy memory habits, I realized we've already been doing so many of the right things to understand what makes Ben tick (ha, see what I did there? Already with the jokes) and develop ways to help him function in the world in which the rest of us reside. Something that has resonated with me is that effort to get Ben's brain working like most of his peers is futile, but rather, the goal should be to understand how he processes and teach him to translate. Not a big deal, just. one. more. thing. 

Ben handled his sleep study like a pro. We await results. He had a surprisingly good audiology test that showed the right ear works better sans cholesteatoma, and the left is still functioning well enough to go unaided. The migraine medication has taken effect and knocked down the severity of the headaches to a much more manageable level. ENT is today for a follow up and as ever we'll chant, "No Whammies" to ourselves in the waiting room.

Sleep Study

And so the state of the first born address contains lots of good news, but it doesn't feel all that positive to him at this juncture. He's tired. He's sick of appointments that keep piling on the onemorethings. He doesn't understand how people can say he's smart when he forgets to use shampoo or can't find the school office. He's tired of having to teach his dang body to do EVERYTHING most bodies do without coaxing.  I so wish I could give him a break. Frankly, I could use one too because I am fresh out of Supermom Serum.