GNA0-1 sucks, Mira is awesome

Hello it’s James.  It has been a while since I have posted anything, and not for lack of effort.  Last week I tried to post something but accidentally deleted and saved changes immediately on accident so the entire post was forever lost into the suck tube that is the internet.  But, I’m up in this shit again and I’m going to really work to make sure I don’t delete the entire post once again or else I will forever give up.

To start with, I will sort of recap what I was trying to talk about last week.  I was proud of my post and then destroyed it, but felt catharsis anyways so whatever.  ANYWAYS – last Monday (not yesterday but the Monday before), I took Mira to school as I do most mornings (I take her, Nikki picks her up).  Dropping her off at school is a most excellent experience almost every time.  Typically, when I bring her in, the other kids are all yelling “MIIIIIIRA!”  She lights up and is so happy to get strapped into her Rifton Activity chair (which is sweet) and join her classmates and teachers.  She LOVES school.  But, last Monday sucked for a few reasons as I will explain:

When I strapped Mira in to her carseat in the back seat of my car last Monday, I had a hard time getting her in for some reason.  The carseat in my car is kind of a piece of shit, but it works for what we need it to do, so NBD.  But, I got her in after the usual struggle.  However, about 8 minutes in to our drive, after I was already on the highway and stuck in a traffic jam, Mira started crying.  I turned around to see what was going on, and her shoulder had come out from the strap and she had slipped out of the harness and was slumped over.  Mira is physically very weak, and she lacks the ability both from weakness and from lack of motor neuron functioning to right herself when she falls over.  So, she was just stuck there, completely slumped over and miserable, screaming for help – and I could do nothing to help her.  I reached back, grabbed her hand (I hold her hand a lot while I drive her), and tried to comfort her but it wasn’t enough.  All I could do was tell her, and have her not understand, that we would be at school soon and I could fix the issue then.

It took another 10 minutes before we got there, after getting stuck in another traffic jam and seeing a car crash, but we got there.  I got out of the car and ran around to get her out and she was absolutely miserable.  Tears, red eyes, slumped over, some snot coming out of her nose – the works.  It was not just an uncomfortable look in her eyes, but a sad look in her eyes.  It was too much for me, and I walked her in to her classroom, comforting her and trying not to cry myself.  I put her in her chair in her classroom, promised her I would fix the carseat, and walked out with barely a word to her teachers for fear of losing it.

Its times like those that Mira’s vulnerability jumps right out and kicks me square in the chest.  She cannot right herself when she falls.  She cannot sit up if she is laying down.  She would not be able to roll over if she somehow was face down in the bathtub.  She is vulnerable in the most profound sense of the word.

On top of these moments in her life, we see moments in the lives of others that bring fear to us as they highlight the vulnerabilities of medically complex children.  A friend’s daughter has been in the hospital for aspiration related pneumonia for over a week, and despite being a fighter and a survivor, she has had a hell of a time getting better.  Mira aspirates.  She is too weak to cough hard enough to dislodge water or food that may go down the wrong pipe and cause an infection.  She cannot eat free of vulnerability.  She cannot drink water free of vulnerability.

Or constipation.  Mira is too weak to push hard enough to remain regular.  She is on a double dose of Miralax every day to keep her system running properly.  Her digestive system is vulnerable.

Want more vulnerabilities?  They’re all there.  Think of something trivial in your life – drinking some water, eating a sandwich, sitting up, holding a pencil, talking, driving, going for a walk, taking a shit, etc.  Those things are not realities for Mira, not right now and maybe not ever.  Her body is her second biggest obstacle because of one stupid gene that somehow was written incorrectly and will dictate the way she interacts with the world for her entire life.  And what’s her first biggest obstacle?  A society that doesn’t take care of people.

We have tried not to get political on this blog, and I don’t want to get political because its the one little place where I have refrained from that so far.  But, politics affect us in a very real way.  If you have heard about the new budget, we are the people who are under attack.  My family is under attack.

Mira is on medicaid right now to help pay for her high medical expenses and costly medical equipment.  We will lose that under the new budget if it passes.

Mira has a whole slew of pre-existing conditions that effectively disqualify her for affordable medical insurance on the private market if pre-existing coverage provisions are eliminated, and her insurance through Nikki’s employer – the State of Kansas – could opt-out of pre-existing coverage provisions under the AHCA and probably would because its Kansas and its full of dickheads.

Mira’s dad – me – works for a non-profit that is partially funded by Federal money.  We are on the chopping block under the new budget.  Additionally, student loan forgiveness for public servants is on the chopping block as well.

So we lose health insurance, then I lose my job, then I lose my student loan protections?  Great job, America (sarcasm).

You guys are great (not sarcasm).  We have a strong circle of family and friends and they support us.  They hold us up, they care for us, they want the best for us.  I’m not making a call for action here, I’m just venting frustrations and more so, I’m venting fear.

I am afraid for my daughter.  Her disease is destructive, it is sad, it is intensely and profoundly effective at disabling individuals, and it is incurable.

I am afraid for my family.  I am afraid of how my marriage will be affected by Mira’s future, how my amazing wife will cope with difficult times, how I will cope with difficult times, and how our extended families will cope with difficult times.

I am afraid for my family’s financial future.  I am afraid of loss of insurance, loss of employment, loss of social safety programs, student loans, and more.

I am afraid of not having more kids, but I am afraid of having more kids.  We want more, but the risk of having another kid with a GNA0-1 mutation does exist even though the mutation is supposedly de-novo and not expressed in either of our phenotypes.  We want Mira to have siblings and peers who love her and care about her and include her and make her life bright and allow her to make their lives bright.  But it is scary.  Everything is scary these days.

Most recently, on top of my more existential fears, I have more temporal fears.  Mira is getting to the point where we are talking seriously with her doctors about getting a g/j tube for feeding.  Due to her feeding concerns, and due to a spirit of proactiveness that exists within Nikki and I when parenting Mira, we want to get her on a feeding tube before her movements get to the point that her nutrition is suffering and surgery becomes dangerous.  Also, this past week Mira’s ear drum ruptured after her millionth ear infection so it looks like she is going to get some ear tubes.  My baby’s perfect little belly is going to have a tube sticking out of it.  I know that it is the right thing to do, but it is scary nonetheless and all of a sudden, as she grows out of being a baby and into being a toddler who is having procedures done, she is seeming more and more like someone with a profound disability than just a big baby.  And that is scary to me.  Reality is the most terrifying thing humans have ever fallen into.

To be honest, I have been very sad lately.  I think that special needs parents learn a style of functional depression that keeps them going.  When life is moving, you have to move with it, despite how bad you feel.  Mira needs us to keep being her parents – regardless of how we feel or what we are dealing with.  Mira needs us to keep providing her food, safety, entertainment, school, therapy, etc.  Her needs don’t change even though ours might.  And she doesn’t deserve any of the hand that was dealt to her.  The thing that keeps me going is that my daughter is incredible.  She is stunning, she is perfect, and she is my daughter.  She is a light that shines on dark days.  Her smile is golden, her laugh is beautiful, and her eyes are powerful.  I can feel like dirt on a shoe and see her smile and instantly be taken somewhere else when I walk in the front door.

She is stunning, she is perfect, and she is my daughter.

Anyways, I have stuff to do but needed to let some things go.  Thanks for reading, and come spend time with us.

Nikki, I love you.

Be kind –

James

1 thought on “GNA0-1 sucks, Mira is awesome”

  1. James,

    I am deeply touched by the honest and heartfelt words you’ve written. I frequently see the beautiful pictures Nikki posts of Mira, but I know that with her challenges being happy can sometimes feel like another check on a day’s to do list. I am so sorry that today’s political situation makes an already stressful situation even more frightening. Recently, I have heard a chorus of similar fears from other ITS-JC families.

    You and Nikki have had to become incredibly strong this year. You are strong for Mira, and you are stronger together. I see that you have surrounded yourself with a fantastic group of parents (Laurisa Ballew, I especially mean you!). They will understand and support you no matter what. They will understand your hardest moments like noone else can. Lean on them. They will embrace you and help build you back up.

    Some days, you may have to just get through the day. I agree with you: reality is often hard and scary. Mira’s genetic disorder is a fluke. It is no one’s fault. It isn’t fair. In fact, it sucks. It sucks for all of you.

    I hope that other days will strengthen you, especially the days you share Mira’ s triumphs and love. You will have your own (meaning both of you) personal successes and professional advancement. You have memories of your amazing trips this year to Italy and your west coast family trip. I’ve seen the love of Mira’s grandparents and even her great grandma first hand. Please remember how much your family loves you and wants to be a support for you, even if they can not always fully understand what you are going through.

    Facebook – where we look for our friends- is the place we also find the perfect lives people project on social media. To me, Facebook is an advertisement, a presentation. I hope that Facebook is a source of strength for you. If it isn’t, opt out for a while or longer.

    You and Nikki have a lot to be proud of. I hope you are proud of the family life you have created together. I am proud for you. Mira is happy and loved. Look at how you’ve learned to navigate the medical system for her. You’ve learned to take care of your medically fragile child. You are embracing and questioning the ways to best care for her. That is why you are an amazing dad. Your trip gave each other permission to take a break when you need it. Protecting your own physical and emotional health is perhaps the most important thing you can do for yourselves and Mira. Your health and endurance will be the base of your strength reserves on the hardest days.

    James, the day I picked up the equipment from you, I saw your love and concern protectively envelop Nikki and Mira. I’m sorry Mira slumped over in the car. It’s not fair when things like that happen. That was a shitty day. Mira loves you and knows that you are her protector. I hope you can forgive yourself, because accidents just happen sometimes. I am sorry that your family has to go through this.

    When Mira smiles, her whole face lights up and her eyes tell you exactly how she’s feeling. She is a beautiful child. I will continue to look for Mira’s sweet smile on fb. I know that you will get through this hard time.

    With Sincere Good Wishes,
    Miss Trina

    Like

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