Pain vs Suffering

Omg you guys.

Oh.

Em.

Gee.

I have had one helluva few weeks after recording what you’re about to hear.

Did you even know that there’s a difference between pain and suffering? And that one is healthy while the other is not. And that one can even alter your future AND you perception of the past?

No, neither did I.

Some background on this recording: I have a business where I support brand owners with photography and marketing strategy. That business is called You is Alpha. Part of the marketing for that business is a podcast called How to be Alpha. There are new episodes every Monday. And on today’s episode, I invited in a psychotherapist who is the program coordinator for the workshop I recommended to Rett parents in this post recently.

Well, I thought we were going to be talking business and instead, we ended up talking about Grace’s diagnosis and I discovered the many ways it’s affecting the choices I make in my life and the hope I have for any kind of joyful future.

Some ? trigger warnings ?

We talk about suicide, self harm (cutting) and of course, I talk a lot about Rett syndrome and the feeling that I’ve lost my daughter.

So because this episode ended up being so applicable to you, my dear Rett friends, I’m also sharing it here on this blog.

How to Listen

You can find How to be Alpha wherever you get your podcasts (iTunes, Spotify, iHeartRadio, Google Play, etc) or you can leave this window open and stream the episode below…

Apraxia and learning disability in Rett syndrome

Over on our About Rett page, I discuss how Rett syndrome is NOT an intellectual disability (insert middle finger here). Hell, it’s not even a learning disability (not in the traditional sense – more on that later).

RETT SYNDROME IS A PROCESSING DISORDER

The number one most impairing thing about Rett isn’t intellectual impairment (what they used to call “mental retardation”).

IT’S APRAXIA.

Can you believe that? Apraxia! Do you know what that is? It’s one of the -xias (cousin of dyslexia, sister of dyspraxia). Imagine you’re driving (hey, stop texting!) and you turn the wheel left but your car goes right. You switch on the wipers and the volume turns up.

AND WORSE YET?

You hit the gas and NOTHING. HAPPENS. This is Rett syndrome. It’s an inability to order your own body’s movements. Stuff happens (cuz you’re not exactly paralyzed) but it’s not the stuff you’re trying to make happen. Like hand wringing or tremors or weird breathing patterns.

And this is an example of why Grace can’t eat anymore. Slowly, it became harder and harder to order all the movements required to eat. To eat, she has to engage her tongue, throat, jaw and breathing and as her processing ability diminished, so did her ability to do all those things at once.

Definition of apraxia

A (redacted) definition from Wiki:

Apraxia is a motor disorder in which the individual has difficulty with the motor planning to perform tasks

I’m not an expert, but I know a few.

So I asked Rett communication expert Susan Norwell about the role of apraxia in Rett syndrome and here’s what she had to say:

“People with Rett syndrome process auditory and visual info very well unless the are also diagnosed with CVI. They conceptually understand information at an abstract level: get humor, get the “big idea”, are able to read, write and learn new information quickly, are able to do the abstract reasoning to do analogies. None of these things are possible for those with cognitive issues. Apraxia impedes their OUTPUT of information thus making the uninformed think they are cognitively impaired.”

Yaaaas Queen! This is SO GOOD! Susan explains it perfectly there when she says apraxia prevents our kids from getting things OUT and it doesn’t stop information from getting IN. So it would lead “the uninformed” to believe that they’re intellectually disabled, cognitively impaired, mentally retarded (however you prefer to describe that phenomenon).

What about learning disability?

I asked assistive tech expert Hector Minto about whether learning disability is a feature in Rett syndrome and here’s what he had to say:

“It’s difficult to say that Rett is not a learning disability, but eye tracking suggests that the kids have normal attention and understanding. If apraxia stops you in your tracks at a developmental stage of life then it leads to an acquired learning lag. We see the same in some people with muscular dystrophy. It’s purely a muscle condition, but it stops them reading, interacting etc, so that impacts learning.”

I love this explanation for how we can think about learning disability in Rett syndrome. It’s not that the girls CAN’T learn. It’s that this processing disorder can prevent them from accessing the world in a way which promotes a level of learning on par with their peers.

There are many things about living in a Rett body that might cause you to have a hard time engaging in a learning environment. A very simple example is breathing. You may know that breathing abnormalities are a staple in Rett syndrome. Grace’s breathing pattern of choice is breath holding. She holds her breath so long that her oxygen levels drop to the 70s before her body allows her to breathe again. Other kids hyperventilate, swallow air or have shallow breathing.

While it’ll be impossible for you to experience most of the effects of Rett for yourself, this is one you can try right now wherever you’re reading this!

It’s as simple as holding your breath until you almost pass out or hyperventilating until you’re dizzy. Then imagine that you do this once every minute while you’re at work or at school and ask yourself: could I learn this way?

In terms of my daughter Grace, I think about it this way:

You might think someone who doesn’t know the dangers of touching a hot stove or running into oncoming traffic is stupid, unlearned or intellectually disadvantaged. Grace doesn’t know those things. But not because she can’t learn about hot stoves or moving vehicles. It’s just because she’s never had to learn those things due to her disability. There’s a lot Grace will never know about the world. Does she know what color her hair is? I have no idea. Does she know that she has 10 toes? Again…NO IDEA. Does that mean she’s learning disabled? No. If she doesn’t know those things it’s because looking in the mirror isn’t a part of her daily life and, frankly…I’m not so sure we’ve ever told her how many toes she has. Or what toes are. It hasn’t really come up.

But does she know she’s about to have a seizure? Yes. Does she calm down when she sees me filling her feed bag because she knows that’s about to be pumped into her hungry tummy? Yup. Does she know her morning routine and what’s coming next? Yes. Can she anticipate I’m about to take off her shirt and lift her head to help me? Yes. She learned those things because they’re a part of her life. She retains that info and displays that she has learned it.

Now that you know about apraxia, you can become more aware of how it affects your loved one with Rett syndrome and help the professionals in her life to better understand the difference between being cognitively disabled and just having a really hard time with motor planning. In short, Rett syndrome (I imagine) is like living inside a runaway train so be patient and give her a good long time to perform tasks (like saying hello). You’ll be surprised what might happen if you wait for 30 seconds for her to respond!

Grief like an onion

Keep falling until you can’t fall no more
Until you feel like you’ve been falling for way too long
Then you will know ’bout me -fink

I usually know what I’m about to write when I get to this blank screen. I have a number of posts that sit in my drafts. This has been one of them sitting here for about 2 years and it’s just been a title with nothing in the body. Because what can I possibly say that hasn’t already been said. I feel like I already wrote pretty exhaustively about grief in this post from 2015.

I feel like all I do is bleed.

The day Grace was diagnosed I carried her into the hospital in her carseat. She was 13 months old. Chubby, cheerful and holding a cookie. And as I rounded the corner into the waiting room, I was met with a room full of disabled children. Wheelchairs, drooling, mangled little bodies, strange noises. And I vividly remember telling myself (so loudly inside my own head) “Grace will NEVER be like that. My baby isn’t like THOSE kids.”

And sometimes I fear that newly diagnosed families will land here and see my blood flowing and say “MY life won’t hurt like that will it?” This is not the waiting room you want to be sitting in when you have a chubby little baby carrying mutant genes and a cookie.

I definitely do write about hope, though. If you’re newly diagnosed, you might want to look away. Here’s some more hopeful reading:

There’s a thing called perpetual grief. People say “life is hard and these things happen”. And I tell them, “yes and hard things are supposed to happen, be done. And then another one happens again.” Rett syndrome is grief after grief wrapped in a skin of grief. The initial diagnosis is only the first time the ground gets pulled out from under you. The cycle of grief is perpetual and doesn’t end with acceptance. That’s not part of this cycle. The illusion of acceptance maybe? But then epilepsy starts. And you accept it. And scoliosis happens. And you accept that too. And every 6 months you might have an X-ray where the spine gets worse and you accept those. And she grinds all her teeth down to little nubs. And breaks her legs. And breaks a hip. And spends her birthday in the hospital. And Christmas in the hospital (10 days). And another Christmas in the ICU (16 days and counting). And you accept. And accept. And accept. But the overall package of all this suffering is unacceptable. Rett syndrome is a motherfucker. I try to accept it (with the serenity to accept the things I cannot change) but when I gain some ground, it gives me another black eye.

Life becomes an abusive relationship you cannot leave.

You become a casualty taking care of another casualty. A POW taking care of another POW who is starving just a little less than you. My broken heart is caring for her broken body.

At any given moment -on any given day- I’m experiencing one or more of the stages of grief: denial, anger, bargaining, depression or acceptance. And more than one at a time, because there’s the overall grief of chronic Rett and whatever stage I’m in that day/week/month/year. And then the stage of grief for the current acute crises of the day. Grief on top of grief. A grief sandwich. A layer cake of grief. Grief like an onion.

In this last mid-term election, I heard for the first time of something called rage-voting. Voting in anger. Voting against something instead of for something. And this same vernacular can be applied to any number of normally-healthy things. Rage eating. Rage donating (literally, there’s a website for this ragedonate.com).

The bargaining part has been the hardest phase to become present to in my own life and witness playing out in the life of others. It feels intimately linked with anger. It’s where I think Rett parents can get stunted and there are whole charities currently propped up by being enablers to denial-fueled bargaining and rage-raised funds. I truly believe if Rett charities offered mental health support to parents, their fundraising would decrease substantially. Because until I went through therapy and grief counseling I thought that battle was all I could do. And then I learned to forgive myself for believing this and move through the pain. And I move through pain every day like walking with heavy boots through an ocean of molasses.

You see, for me, bargaining took the form of charity. First supporting a charity of choice (at the exclusion of all others) and then starting one with a group of other parents. I traveled the world trying to be important in Rettdom. Races. Raising money. Mastermind of awareness campaigns. Starting this blog. All under the guise of “hope”. Of “fighting” of “battling” for a cure.

Many calls to action I see are posed in that embattled language of war that feels so familiar to me having grown up in 1990’s American Charismatic Christianity. “Armies” who are “Fighting Rett”. As if we have any power to do such a thing. Rett isn’t cancer. No poison can kill it. Any hope for a cure is for the future. Not for our children now. Can you really imagine a body having been ravaged by thousands of seizures, muscle wasting, ICU myopathy, dependence on drugs, extreme osteoporosis, a throat which hasn’t eaten in 10 years or ever spoken a word…do you really think there will be a magic bullet cure that takes this body and makes it normal? Would I even want that? The lifetime of pain, rehabilitation and disability this would bring? Trading one unworkable situation for another.

Better the devil I know than the devil I don’t.

This is bargaining, my darling friend. Rather than accept that she’s different, single-mindedly focusing on anything you can do to change the outlook. Desperate to reach into the very fabric of time and pull something into the present which doesn’t exist (a cure).

All of that will be a painful read. I don’t blame you if you’re disgusted. I’m disgusted too.

Some people reading this will be too early in their walk. Others will wish they’d heard that sooner. I cannot control who is reading this and when. That’s up to the universe. Maybe this site won’t load for the people who aren’t ready to see this. Stranger things have happened. But I so wish that someone gave me the permission to be ok knowing all that earlier. To gently take the weapons out of my fists and let me fall apart and move through bargaining and the anger and the denial and the depression. I had to wrest that permission from the hands of god-knows-where and give it to myself as a gift. And THAT is the serenity to accept the things I cannot change.

The best therapy program for Rett parents

If you’re suffering with your child’s diagnoses (and really, who isn’t?), the greatest gift you can give to yourself is coming to Orlando and attending a P3 weekend. It’s like months (years?) worth of therapy in one weekend. I have never moved through so much pain so quickly and with so much support as I did on that one weekend.

Many people travel from other states to attend.

Having gone through a lot of different counselors and therapists, I can tell you that P3 is singlehandedly the most effective program that exists for dealing with the pain of life. If you decide to do it, tell me and I can be there for you as an “angel” if you want. They know about Rett syndrome because of me being in the community and they also support my oldest son in the adult program, however there are also P3 weekends geared for children.

P3 happens every 6 weeks

It’s not cheap (about $600) but far cheaper than the years of counseling you’d spend to achieve the same results. There are scholarships you can apply for if money is a challenge, and I would highly recommend fundraising for this if you need it. Because our daughters need us to be healthy. Self care is a gift you give everyone in your life.

If I ever had the energy to be in the charity world again, it would be to raise funds for the Rett community to access this kind of emotional and mental health support.

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www.orlandop3.com

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I’ve been afraid of all the wrong things

Grace is in the ICU in critical condition with aspiration pneumonia. And I did NOT see this coming.

All this time, I’ve been afraid of the wrong things. I’ve been afraid of the seizures. Bracing myself for her next bone break. X-raying her spine every 6 months. Testing her for heart conditions she does not have. And I thought I had this respiratory thing under control because we got her feeding tube as soon as it as apparent that she was aspirating her food. I checked aspiration pneumonia off the list of things to be afraid of.

And then last Monday happened.

Grace got the RSV virus. A common cold. Would give you a runny nose. But for babies or immunodeficient people, it’s more serious. So she had a wet cough. And we expected that at some point (as usually happens) she would choke and gag on her phlegm, have a great big but super productive puke session (which we would catch in a towel) and be clear of all her congestion.

And she did choke and gag on her phlegm BUT it happened in bed while she was laying on her back (before we knew how sick she really was) and she aspirated that vomit into her lungs and instantly was in severe respiratory distress. She was passing out, couldn’t breathe, eyes rolling around. We bypassed the ambulance because they take too long. Although it’s not the closest hospital, we rushed her to Nemours. She quickly went from oxygen to BiPaP to intubation and she’s been this way for 7 days. Her diagnoses are RSV, aspiration pneumonia and ARDS (acute respiratory distress syndrome). Add to that the ICU myopathy which happens when you have long admissions in the ICU undergoing multiple procedures with many different medications, sedations, periods of paralyzation.

The outlook is hard to process. There were definitely days last week where we had to face low survival rates (her level of ARDS one particular day put her at 50%) and we’re not even talking about extubation yet (removing the breathing tube). Sometimes this stuff leads to tracheostomy. When she’s done here, she will likely go to a rehab unit. She will definitely not be home for Christmas. And we will be adding quite a few new pieces of machinery to our lives. All of this happening just weeks before our new health insurance kicks in. I’m stressed TO. THE. MAX.

But back to that fear. And being afraid of all the wrong things. When Rett girls die, I go on a hunt to figure out why. To check the boxes that read “things you should be afraid of”. People rarely say “this is why my daughter died” and so I dig through photos, blog posts, social media…anything they had put out about their daughter. Did she have a bone break leading to a blood infection? Did she have one massive, gripping, seizure? A spine surgery gone wrong? Long QT syndrome? The pneumonia ones didn’t scare me. She never had a chest infection again after getting her feeding tube. I was in control of that one. Except that I wasn’t. And you know what? Lower respiratory infections are the leading cause of death in Rett syndrome. And I didn’t know that until now.

So now I have all the questions. Should we let her go to school anymore? Should we shield her from germs, from crowded places, from ourselves? However, I have this nagging suspicion that whatever I do, I will never be in control of this.

What does hope really look like?

Pretty soon here, we’ll be hitting our 9 year D-Day anniversary. In the beginning, “hope” looked vastly different than it does now. A newly diagnosed mom recently wrote to me and said:

“I haven’t read a single thing that has inspired me or given me confidence, as much as anything can with this ridiculous syndrome, but reading your blog has been heartwarming and given me hope that if you can do it, I know I can and I will be alright on this path despite the complications heading our way.”

*Standing ovation* If that ain’t the truth!

This made me reconsider what we consider “hope” to be. And how do we know when we’ve found it? This was my response to her…

Yes…hope…in the beginning I found “hope” in all the science stuff. In the belief that she would be cured. And it actually just stunted my grieving process by many years. Because the real hope is the fact that we have the ability to parent these kids. To lift them no matter how big they get. To fight with insurance companies like a badass and teach doctors things they never learned in medical school. This disease will make you what the medical community calls an “expert parent”. You’ll grow muscles – figurative and literal. Your capacity for empathy for the pain others experience will open you up like a flower. The ability to know that kindness matters because other people you encounter may be fighting battles you know nothing about.
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SO. MANY. THINGS that would have bothered you in the past just don’t matter anymore. Lots of parts of life that we found hard before get easier. Because when you deal with Rett syndrome every day, getting rained out of a concert, a dead car battery, missing a party, losing your keys, having a bad hair day…those things are hardly the hardest thing you have to deal with that day. The script gets flipped on parenting. You had this kid thinking that you would have to teach your daughter about the world. And now you have to teach the world about your daughter.
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In the Rett syndrome community, you’ll encounter both cynical people AND positive people. Judgmental people AND those who hold space for others’ pain. Assholes AND saints. Insecure bullies AND confident cheerleaders. But as you meet people and connect, you’ll soon find “your tribe”. And some of them will just be in the disability community at large. Because people raising kids with cerebral palsy have many of the same struggles we do so they can be a valuable part of your circle.
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And while we’re talking about “your circle”…it gets smaller. We lost friends when Grace was diagnosed. People who just couldn’t get on the road with us because its too hard. And it changed us. The Halfords were suddenly not the go-to party people. Shit got real. And many folks just aren’t in it for that long haul. But you’ll start to meet people who didn’t know you before Rett. And to them, the new you will be normal.
p
And while we’re talking about “normal”. You will find a new normal. Diapers forever will become normal. Seizures (if she has them) will be normal. Wheelchairs (if she needs one) will be normal. And everyone will marvel at what a hero you are, but to you? It will just. be. normal.

A new diagnosis. And update on the fish oil thing.

Hellooooo friends! Wow it’s been ages. So much has happened. We’re in a beautiful home now. It was a really long haul to get to this point but we’re settled and happy. Grace is settled in school. The bus is working out just fine now. She has all her insurance and doctors and meds in order. She just got fitted for a custom molded wheelchair. Her new sleep system is on the way. We still need a ceiling track hoist which you have to pay for yourself here. And we still don’t have a wheelchair vehicle which is sad but at least she has a lovely house to be stuck in when she’s not at school! We’ve applied to a charity for help and they’re meeting at the end of April to discuss our case. Positive vibes/prayers/thoughts please! Or throw some salt over your shoulder (is that a thing?)

I got another email from a new Rett mom this week. As usual, it was in reference to my fish oil post from 5 years ago. Don’t bother looking for it – I trashed it now.

The question, as usual, is “are you still giving Grace fish oil?” and my answer this time was the honest one. I gave Grace so much fish oil that she got a nosebleed. I threw it away immediately following the first bleed. But when I look back on that time in our lives, I realize that I was so desperate to believe that something could help her. That she could gain use of her hands. Or eek out a couple words. Or learn to use her legs. I would do anything to make this happen. Any snake oil would do.

I read every research paper. Tried every natural thing parents were currently raving about. Cried myself to sleep that we were stuck in England and I couldn’t put her in the drug trials. Cried myself to sleep that I couldn’t afford daily private therapy to try to force her to do things that her body just isn’t built to do.

I think the first time I ever threw in the towel was when she got her feeding tube 5 years ago. I remember the dr very carefully trying to broach the topic with me and I just went “Yep. I know what you’re gonna say. She can’t eat anymore. Let’s do it.” But even after that, I remained in a haze about the truth regarding Grace’s illness. That it’s irreversible and permanent.

Until this year.

Grace got a diagnosis I haven’t talked about yet. Ultra severe scoliosis. Currently, the curve in her spine is 70 degrees. A kiddo with a 20 degree curve is referred to the surgeon. So imagine our surprise that her Drs in the UK failed to notice the severity of her spine and it took moving to Florida to learn this. For YEARS I insisted that she clearly had a severe curve. You can see it even from the front of her. But no. They said no.

This news hit me as hard as her original diagnosis. And she’s had many diagnoses since we learned she had Rett. But this one…this one is deadly. Rett isn’t even deadly. It’s only deadly when they have things like this. Surgery is an option, yes. Rods in her spine. But her bones are so brittle that we’re not sure if they will just crumble around the hardware. We’re in the process of figuring this out.

Anyway…it was this diagnosis that just made me crumble. I swear I let out a breath I didn’t know I’d been holding for the last 8 years. She really, truly, absolutely has Rett syndrome. And literally every single thing that goes with it:

  • Intractable Epilepsy
  • Long(ish) QT
  • Scoliosis
  • Osteoporosis with multiple breaks in the last few years
  • Premature puberty

And now? No more snake oil. We give her all the drugs. After 8 years, I’ve finally come to terms with this diagnosis and the fact that I’m not a superhuman. I can’t fight genetics. I just love her so so much and sometimes all I can do is hold her hand and put my head on her lap and cry with her. And sometimes, we just look at each other and she smiles so big that it looks like her face might explode. And I guess this is gonna have to be enough.

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