Month: March 2020

Us Grievers are a funny lot.

We look normal.

We act normal…..most of the time????

And many of us function fairly well in everyday society.

But tiny variables throw us off a bit.

And slightly bigger issues, say a worldwide pandemic, trigger emotions that search for a place to live. Emotions buzz the face, fester, invite other friends…..and left unattended create a pool of emotional ugliness that demand to be attended.

I had to sit in my ugly on Tuesday. It was more of a plop. It was bit of hard landing which told me perhaps I had been avoiding it for a while.

I was on our mito support group call. We were talking about COVID19 and the anxiety these days produce for our medically complex community.

I should have no anxiety. Hubs and I are social distancing. We are healthy. We have our jobs.

We are fine.

We are fine.

We are fine.

But the underlying current of our fine-ness feels surface level. Underneath my skin, is a layer of trauma from times when we were not fine. A time when we lived every second with a medically complex child; this layer tends to bubble up when the earth is off its axis.

What is difficult is that my trauma has no tangible place. Our children are no longer with us. Hubs and I are healthy. I worry for my other Loves but the amount of worry for my other Loves seems sometimes seem insurmountable.

On Tuesday, as I sat in my ugly plop, it hit me. It hit me on a support call with mito patients.

“I don’t want you to die,” I blurted to the group.

Queue silence.

And more silence.

And so I went on, “I feel like such an ass. I am healthy. I am fine. Hubs is fine. Our jobs our fine. I have nothing, nothing, nothing to complain about.

“But in losing my Littles, I lost so much. And nothing else seems sacred. If I can lose them, I can lose anyone.”

I paused.

And then went on.

“I’m like Tom Hanks in the Green Mile.”

“Who?” asked a friend.

“The Green Mile. Tom Hanks had to live forever watching his loves die because he let an innocent man be executed. Well, except the mouse. The mouse was still alive. Maybe I’m the mouse.”

“You’re a mouse?”

“What? A mouse? No. I’m not. I’m okay. I just don’t want any of you to die. Really. Please don’t die.”

My support group promised to try to not die.

And so I sat a bit long in my ugly. I recognized the smell….survivors guilt with a touch of paranoia. I held it up to my nose before remembering I should not touch my face.

“Heather?” Said someone on the call.

“Yes?”

“I’m sorry. I am really, really sorry.”

“Yeah. But no, really.” I said. “Oh no. Don’t be sorry. I’m okay. I’m just happy to put a name, a face, a smell,  to the uncomfortable I have been feeling. Oh, whew. I smell.”

And with that, I heaved myself from the ick and excused myself to go to another meeting.

Be kind to you, sometimes you don’t know the battles you are fighting.

And don’t die.

Being in the rare disease space, I have been privy to several clinical trials. Clinical trials are extensive, they can be difficult in the rare disease space and many times, companies who have developed a drug for a rare condition do not get that coveted FDA approval.

This lack of approval is devestating. The financial impact to a pharma company that has dedicated time, resources and money can force a company to close it’s doors. And to families who invested time and money (many trials do not offer stipends), the biological investment of their bodies (to put THIS lightly) and the emotional investment of hope, make a failed trial hard to digest.

Clinical trials are complex. A pharmaceutical company has to gather a statistically valid group of patients with same disease, they partner closely with a medical center that can manage a clinical trial and report findings back to the FDA.

A couple of years ago, our doctors at Children’s Colorado were granted a clinical trial of a drug in Phase Three called Elamepretide. This drug showed efficacy in helping those with mitochondrial myopathies. With mitochondrial disease, the membrane of the mitochondria become ragged, making the transmission of energy even more difficult. Elamepretide smoothed out the membrane.

Brilliant! Let’s do it!

And because it addressed the foundation of the mitochondria, not the complex genetics behind it, this drug could potentially be used for multiple genetic mutations impacting the mitochondria.

WHERE DO I SIGN????

I was so stinkin’ excited for this clinical trial! Which is funny because I would only be a bystander and not a participant…..no matter…..bring me your wonky mitochondria!

And so we started to recruit for patients. This too was a challenge because is it hard to diagnose the gene that contributes to mitochondrial disease. Participants had to have a confirmed genetic mitochondrial mutation be a part of the trial.

My dear Bro was accepted into the trial. When you are a part of a clinical trial, you become one of two control groups; those who get the med and those who get the placebo. This trial was double-blind in that no one knew, not even our doctors, what version patients were getting.

I would ask him about the trial in a trying not to pry, really wanting to pry type of manner. Those who know me know how relentless I can be.

Please send notes of respect and admiration to my Bro.

For six months Bro injected himself with the (maybe) or maybe not med. He reported how his day went and attended monthly appointments.

Monthly appointments included various tests; fasting, walk tests, strength, it really is a lot to ask of patients.

I was allowed to go to the last appointment of his six month trial. On this last day, he would get the real med, no matter if he was on the placebo before. He could receive the med until we got FDA approval.

I say ‘allowed’ because sometimes I can be a tad overzealous. And I was really excited about this. We had raised so much money for our mito clinic and now they were a part of this trial and my brother could benefit………I was not just a bull in a china shop. I was like a bull who had spent four days at a rave in a China Shop.

LETS DO THIS!!!!!

Here is what I realized very quickly….people who manage clinical trial sites can smell people like me out…..immediately. And they are awesome at shutting us down.

Boom. Within five minutes she had me hog-tied and gagged in the corner. While she politely asked questions to my brother.

How was he feeling? Energy levels? Good week? Bad week? They took blood, tested his leg strength and then prepared to send him downstairs for a six minute walk test.

The walk test is a standard FDA assessment. It’s also a toughie when you have a neuro-muscular disease and walking is a challenge (this is a whole different post! Stay tuned for part three!).

“Can I go down with him?” I meekly asked from the corner.

The clinician sized me up, “Yes, my assistant will take you both downstairs for the test.”

And off we went to the bowels of Children’s Hospital. It was a long, silent hallway. The lovely clinical assistant took a tape measure and marked a certain distance. And then it was time. It was time for the walk test.

My God it was quiet. So quiet. Bro walked along. I could not just stand here all quiet and meek in the corner. I started to cheer him on.

“WHOOP! WHOOP! You got this!”

And then I decided he needed some music.

Queue the Rocky theme song which I just happened to have downloaded on my phone.

And how can you play the Rocky theme song and not SING to it????

Gettin’ strong now!

Coming on Now!

Gonna Fly Now!

FLYING HIGH NOW!!!!

Every once in a while someone would poke their head from around the corner. No matter. It was me and Bro. And a clinical trial.

And an assistant that looked really uncomfortable. Like she might throw up uncomfortable.

No matter. We owned this trial! We owned this walk test!!!!

Yeah, we really didn’t.

Here is what I realized later about collecting data during a trial. Variables need to be the same all along the trial so that data has consistency. Apparently the Rocky Theme song is not a consistent variable.

I knew none of this.

We got back up to the room to a Clinical Trial Manager who was fit to be tied.

“You cannot sing the Rocky Theme song during the walk test!’ She scolded.

“I didn’t know!” I contested. “And no one said anything! Your assistant didn’t say a word. I would have stopped singing!” In truth probably not, I mean really; I didn’t start the fire. It’s been always burning since the world’s been turning.

“I am just as unhappy with her as I am with you.”

Turns out, all was forgiven (somewhat). I took my brave Rocky-walking Bro out for sushi and a beer. And the Pharma company allowed the final report to be submitted under the caveat that data was skewed due to a disruptive variable.

A disruptive variable! How perfect.

The other day I listened to our President announce FDA approval for Chloroqunie. I watched as Dr. Fauci put his hand to his forehead.

Alas, another disruptive variable.

And onto Part Three!

The last 18 months, I have had the amazing opportunity to see how the FDA operates in regards to drug development.

It really is amazing to witness the process. The mission of the FDA is as follows: The Food and Drug Administration is responsible for protecting the public health by ensuring the safety, efficacy, and security of human and veterinary drugs, biological products, and medical devices; and by ensuring the safety of our nation’s food supply, cosmetics, and products that emit radiation.

Regulating all of these elements is a tall order; managing a country that seems to want to super-size, smoke or self medicate itself out difficult situations. We have grown up thinking that there has to be a pill I can take- there has to be a cure- because there is always a cure.

To be fair; my first run-in with the FDA was 14 years ago was when Samantha was in the hospital with a form of seizures called Hypsarrythmia. Here poor little brain was seizing 90% of the day these seizures had to be stopped. We were given two options: a steroid; which would be really hard on her compromised system or a medication called Vigabatrin.

“Well let’s go with the vegamite drug.” I told our doctor.

“Vigabatrin.” She said.

“Yeah, that.”

“Well, funny thing about that drug,” she said. “It’s no longer FDA approved.”

I sat back in chair, prepared for the very worst, “Oh God,” I said. “What does it do?” I thought of all the movies about a drug gone bad that in hindsight I never should have seen.

“There are some cases where use of the drug limits peripheral vision.”

“What? Peripheral vision? So maybe she can’t catch a ball?”

“Or drive when she gets older,” Our doc said.

“Well, she is my daughter so her hand-eye coordination is suspect to begin with. As for driving, I will drive her to the moon and back. We just need to give her brain a rest.”

Our doctor agreed and for a year we smuggled contraband seizure medication from Canada. We paid out of pocket and were happy to do so. This form of epilepsy disappeared from her EEG in 8 weeks.

And so began my relationship with the FDA. At that time I was perplexed by how they deemed this drug safe or not. In my mind, the outcome of the disease was much more harmful than the side effects of the drug. In their mind, they had too many data points that said no.

As a side note, Vigabatrin is now again FDA approved and used often and effectively for this form of epilepsy. And I got to live life as a smuggler running the goods from Canada.

Win-Win, right?

But the disruption does not stop here. The FDA and their meticulous data capture and me with a knack for chaos will collide again.

Onto part two.

Dear Readers:

I would be lying if I did not say that the last couple weeks has triggered some pretty deep PTSD for me.

And so maybe some posts might be hard to read. But if you know me, you know that I put more to paper sometimes than I put to voice. I will tell you I’m fine but pour my heart out in a blog.

This is my safe space.

And so I will tell you.

I am so sad.

I am so fucking sad.

I am sad about the blame. I am sad about the denial. I am sad that the leaders in our great nation, a country that I love, have to gaslight and bolster and pontificate.

We would prefer honesty.

Honestly.

Because we know.

We know that our ICU beds can only accommodate 2 per 1,000 people. We know how many ventilators are available. We know how many people can get a COVID-19 test.

Don’t pee on my head and tell me it’s raining.

Not when it comes to my health. Not when it comes to my safety or the safety of my loves.

It is easy to dismiss medical mishaps as some else’s issue. But when we do, we forget who we leave behind.

When our son was born still at 41 weeks, we were discharged the next day with a pamphlet on grief. It was a lovely trifold in glossy paper but somehow…….

ironically……..

That tri-fold brochure on grief did not ease my pain.

One doctor called a week later. Not my primary doctor. But a doctor I remember and will admire for the rest of my life.

He asked how I was doing.

I said this was really hard. Harder than I ever thought was possible.

And he apologized.

He said in a litigious world, no one ever wants to say they are sorry. But that he really was. And he thought about Hubs and I often.

Ah. To take responsibility. It might not have been his. But as a doctor, in his practice, he took it.

Imagine. As a leader

I think about that honest conversation when I have to make really hard conversations. It compels me to pick up the phone.

I wish we could stop denying what was happening and what has happened in the last month. I wish we had a leader who would let us talk about our fears without attack. Because even if you side with our President or not, you still might be nervous, or disappointed. It would be nice it we could meet six feet across from each other and talk about this.

Because lives are at stake.

And lives cannot be returned once they are gone.

And if we cannot bank that as currency, I’m not sure where we go from here.

I’ve been thinking of you Mamas and how crazy life is right now.

I wrote this 12 years ago about my own Mama-time. The biggest judge is you. Hang in there

“You are such a great mother.”

This statement takes me aback. I guess I should be flattered. Friends and family say it and I smile, thank them and try to change the subject. When I hear it from strangers however, it makes me a little uncomfortable. Who are you? How long have you been watching us? Did I scratch my butt? Pick my nose? Make a disparaging remark to my husband? I am not used to the attention that comes with a special needs child and when I hear the great mother comment I am tempted to come back with something smart-assed. Really? ‘Cuz I just shotgunned a beer in the bathroom.

My favorite is “You are such a good mother for taking care of her.”

I have a choice?

She is my daughter. I love her to the moon and back.

But I didn’t sign up for tube feedings, seizures, therapy and the many issues that come with being Samantha’s mom. And I would give my right arm, left leg, heart and soul to make her better and to make our life different.

Am I still a great mother?

Oh……I have days when the T.V. is on, when Samantha is wiggling around on the floor, perhaps a little too long before being repositioned. I have nights when she is awake after having a seizure and I am wandering around the house raking my sleep-starved brain for a strategy to soothe her.

I curse God, rage at the heavens and console myself with yet another glass of wine.

I do not feel like a great mother on those nights.

“I will pray for you.”

I really do appreciate this one. Any healing thoughts sent off into the universe is a good thing. However, after a tough night when I have told the world what I think of their crappy divine plan for me, when I have flipped off the heavens with both fingers, I am really tempted to say. “Well thank you but you might want to wait a day or two; God and I are in the midst of a heavy duty argument. You might not get through.”

I did not write this as my pity party. Well, okay, maybe I did. Bring some Ritz crackers, and that really funky orange cheese in the squeeze can. We can talk about your pain, my pain, examine the ingredients of that funky orange cheese and perhaps the ingredients of our lives. As parents, as humans, as people in this world, we all have pain. Just because my pain, my daughter’s disability, is visible to the world doesn’t make anyone else’s pain any less real.

It certainly doesn’t make me a better mother.

“That which does not kill you, will make you stronger.”

I have evaluated the super-beings with super-human strength and they all have issues. The Incredible Hulk, major anger issues. King Kong, a great big monkey with an attachment disorder. Even Superman lived a life hiding his true identity; misunderstood and yearning to belong.

Yearning to belong.

Well heck, pass the Ritz crackers.

Whatever our pain may be; a disabled child, an ailing parent, cancer, divorce, foreclosure, it’s not the life we signed up for. When I imagined my married, parental life years ago it did not include anything messy or ugly. It did not include tough decisions. It did not include a daughter with a fatal genetic disease.

It did not require me to be such a grown up.

Am I still a great mother?

“Life is not fair.”

My Granny, Emma Mae, used to say this to me when I didn’t get my way. I used to think that life was not fair because my brother cheated at monopoly or he got the bigger slice of pizza. My reaction would be to stomp my feet, throw a couple pillows around, pout in the corner.

It still is but no one thinks it’s cute anymore.

Emma Mae never told me that the UN-fairness in life is doled out in disease, sickness and death. No one told me that ecstasy and despair are secret bedfellows and that they walk hand in hand. As an adult, moments of shear joy are coupled with moments of pain so intense it’s like someone ripped your heart out of your chest.

And I used to think fairness was all about the last slice of pizza.

I do confess….there are days where I am the Supermom. Days when Samantha feels good, the feeding pump never clogs and we don’t see a seizure. I savor those days; inhale them like lavender and the sweet smell of my daughters head. I puff out my chest. Give my best profile shot and stare knowingly into the horizon; my cape flowing in the wind.

But I do keep an eye on my back. The heavens are smiling down and yet have a complete record of the last time I flipped them the double bird.

And I do confess…..my pink Wellies and bottle of Merlot are by the door. You never know when life’s muddy slog will get the best of you.