What does hope really look like?

08.01.18

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.

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.

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.

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.

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.

03.29.18

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.

Retarded

06.06.17

?4 years ago I wrote a post called Retard. Read that one first, I guess?

I’m in all these special needs groups on Facebook. There’s rarely an absence of at least one person losing their minds because they heard/saw someone say “retard”. I recently saw a woman posting a huge load of screenshots of a public showdown she had with some guy on Facebook (a place not exactly known for facilitating the intellectual awakening of ignorant folk) and -man- did she tear him a new hole.

And it leads me to ask…

People, what is going on?!?

Exactly WHEN did we start being cool with policing everyone and waging holy war on anyone and everyone who doesn’t agree with us, behave according to our beliefs, adhere to our worldview, et cetera? When did it start being not ok to be offended once in a while?

Listen, retard isn’t a nice word to lots of folks. I imagine the ones using it like it just fine. But if one is not a fan, I’m guessing that one’s distain has something to do with the Golden Rule? That we shouldn’t use that word because we need to show care and empathy for others? So in what universe do we say “you’re breaking the golden rule and making me feel sad, you asshole! Stop using mean words, you contemptible dickwad! Be nice – like me!”

Model the behaviour you’d like to see. It’s a basic rule and even works on toddlers (and husbands!)

We simply cannot police others and force them to behave as we want them to. I like to say, “The only person I can control (on a good day!) is myself.” Desiring -and attempting- to control another person is an illness. And yes, I do see irony in the fact that this post is about me policing people. But to take on every person who uses a word you don’t like is to live your life in a constant state of anger and, frankly, I like to channel that emotional vigour into taking care of my special needs kid and writing pissy blog posts.

The only person you can control is yourself.

There are 7 billion people in the world. Some people don’t have the emotional intelligence to know that “retard” or “that’s so gay” are slurs that (can) hurt. And you know, lots of people use slurs on purpose because they know that it offends. They are trying to offend. Are you gonna give them what they want?

Captain Obvious and Dr. Christmas Jumper

06.05.17

Note: this post has taken me 6 months to publish. It’s not pretty. A disclaimer about the names I will mention. Dr. Martul I noted his name myself. Dr. Peter Ellis was a name given to me by looking into her records. If that is inaccurate, it will have been a mistake of the hospital. Which, as you’ll see below, wouldn’t be so surprising. Two names I was unable to procure and I will give them nicknames. But the hospital will know who they are and that’s the most important thing.

I firmly believe that one of the greatest contributions I can make in Rett world is to empower other parents to be professional-level advocates for their kids. To tell you stories of learning things the hard way. To tell you how I got Grace her DLA even though she was rejected 3 times. How I got the education system to pull their finger out and give her the eye gaze she was entitled to. And today, I will tell you how I failed to force the hospital to find her broken legs.

I want you to know that I cancelled our lawsuit just so I could tell you this. Because I was told I couldn’t speak openly until that was over and this is far too long to be sitting on the information I have. Far too long to be silent and not be able to tell you what I should have done. Too long to wait to say to you:

Do not fear being too forceful if you feel your child isn’t given the care they deserve.

I believe that change will only come if people are educated. If I simply sue, it will help me vent my anger at the hospital, yes, but what won’t likely happen is that the Drs I name below will not hear me tell them how they failed my daughter. They won’t hear me say: non verbal children need to be taken seriously. They won’t get the message that they shouldn’t let their guard down because it’s Christmas. They won’t learn that people who don’t walk are at a risk of osteoporosis (which I thought was common knowledge but I’m happy to educate them). And, mostly, they won’t get a chance to give us the apology we deserve.

I will name names. And I will call certain doctors to account for their actions. Hold on folks it might be a bumpy ride.

The crux of all of this is that Grace broke her legs a couple days before Christmas. And I believe that Christmas spirit, hospital staff jollies and a complete disregard for the severity of Grace’s situation caused her to be in excruciating pain for 5 days. So much pain that her body was shutting down.

It’s been said that breaking one’s femur (the longest, thickest bone in the body) is “more painful than childbirth”.

So imagine that happens to you on both legs. And you can’t talk. And it remains for a whole week and on top of it all, your parents think you just have soft tissue damage (I have that in writing from the hospital) and so they keep trying to exercise your legs, get you dressed, move you around like it’s no biggie.

She did what any body under such strain would do…she shut down. Grace checked out of reality for 6 whole days. She slept about 23 hours a day. She shook. Moaned. Sweated. Ground her teeth. Curled up like she was bracing herself constantly. We thought she had the virus which had been going around so we gave her simple pain relief and monitored her temperature. Bloodwork showed that she didn’t have markers for infection and so we can only conclude that this was her body responding to excruciating pain.

So now that I’ve painted the picture, here’s what happened:

On the 22nd, her legs broke during a powerful seizure. We didn’t realise this until the 23rd.
On the 23rd, she was ambulanced to Queen Alexandra hospital in Cosham.
She was the only kid in the E.D. (emergency department)
She was seen by a Dr. _______ (we’ll call him Dr. Captain Obvious). I told him I was concerned about her femurs, hips and knees. In the letter at the top of this post you will see that “mom was concerned about her knees but we didn’t examine them.” Smoking gun. I’ve since been told by her bone endocrinologist that, yes, that spot at the knee is the most common place to break your femur. So it wasn’t rocket science, really. It should have been found.
While we were seen by Dr. Captain Obvious, a more senior Dr. (in a sweater, not a uniform…so we’ll call him Dr. Christmas Jumper) was within sight but he never came to examine Grace. Instead, he jollied with nurses, talked openly about co workers and patients (by name I might add) and they all were enjoying the Christmas spirit and empty E.D. while Grace was experiencing the worst pain a person can experience.
Dr. Captain Obvious ordered some x-rays, but not of the knees. Hell if I can understand why, considering that her discharge letter admits that I asked them to.
Dr. Captain Obvious said it just seemed “too unlikely that she would have broken both legs” and that she was probably just not bending her legs because she pulled a muscle. So she was diagnosed with ‘soft tissue damage’ and sent home.
Here is where I failed. My gut told me she had broken her femurs because I’d seen it so many times before in other rett girls. And I knew that this was the age when it often happens for the first time. But I let my guard down, I gave in to the luxury of just trusting the hospital and convinced myself I was a crazy mom going overboard.

So we took her home, suffering, and started trying to stretch and exercise her pulled muscle. I massaged her legs, further displacing the bones, while she screamed and shook and looked me dead in the eye begging me not to touch her anymore.

On the 28th:

I took her back to the hospital where we met a Dr. Peter Ellis. (the only Dr. not on my shit list). He was on it like a rash. He did was needed to be done and in quick order. He was kind, caring, cautious and uber attentive and he delivered the news I was dreading: she broke both femurs just above the knee. He said, “don’t give her another feed. She may need surgery soon.”
On that same day, we encountered an orthopaedic registrar, Dr. Luis Martul, who told us that teams at Southampton General Hospital and Queen Alexandra Hospital were in talks about which would be the best hospital for Grace. He claimed that “Southampton are better equipped to care for Grace” so she may be transferred there.
Grace was put on morphine and given temporary casts while still in the E.R.
Dr. Martul eventually delivered the news that because “we didn’t catch it sooner”, Grace couldn’t have the surgery required without needing to drill into her bones. Too much healing had already taken place. It was at this point that I schooled this Dr. on exactly why it “hadn’t been caught” and that this was 100% down to hospital neglect. He admitted fault and quickly apologised (which according to our attorney is NHS policy).
In the meantime, they called social services and an emergency social worker flew in and took the whole department to task. She happened to remember that we called to let social services know what happened on the 23rd. She claimed that social services would be opening an investigation for neglect of care against the hospital and not us. I don’t know what happened with that as of now.
While in the presence of this social worker, Dr. Martul quite ungraciously attempted to pin this disaster on me when he asked, “remind me…what did Grace hit when she fell and broke her legs?” I had told him 3 times over by this point that she broke her legs during a seizure. When he left, the social worker told me exactly why he was so crass and that he was perhaps attempting to trip me up and make me say I did it.
In addition, Dr. Martul claimed that “because she doesn’t walk”, surgery was pointless. Straight legs are a luxury afforded to kids who can walk, I guess. My bad.

Grace was admitted to the Shipwreck Ward (great name, right?) and there she stayed for 10 days getting a variety of different casts and a chest infection. At this point, I have to say that the nurses were incredible. Apart from the time they tried giving Grace a nurse with a cold (I kicked her out), the care she got was incredible.

Wheelchair services…we have still not heard from them. They never adapted her wheelchair. We had to hack it in order to take her home.

There was a point during the morning rounds where I asked the orthopedic surgeon guy (who in the UK are called “mister” and not “doctor”) Mr. Richards, “why didn’t Grace get surgery for this?” He said it was because QA Hospital wasn’t equipped with the high dependency unit she would need post-opp (which Southampton General did have). So I asked, “Ok…so why didn’t she go to Southampton?” Answer: “Because she didn’t have surgery.” Again: “Ok and why didn’t she have surgery?” Answer: “Because she didn’t go to Southampton.” Now, I’m no surgeon. But…what?! I think we call this circular reasoning, yes?

The fact is, Grace didn’t get the care she required…she didn’t get surgery and she was in horrific, excruciating pain because, as Dr. Martul and Dr. Peter Ellis both admitted, her broken legs weren’t found soon enough. Because on the 23rd December, while there were no other children in the E.D.,Dr. Captain Obvious didn’t listen to me, Dr. Christmas Jumper was having Christmas jollies with the nurses and I let my guard down and trusted them.

What are the takeaways from this?

Non verbal children are discriminated against
Non ambulant children are discriminated against
People who don’t walk are at a risk of osteoporosis. And #sorrynotsorry but if you’re a Dr and you don’t know that, then you, my friend, are an imbecile.
Doctors can be dumbasses and need to be told what to do by “expert parents” who know more about Rett syndrome than they ever will.
Do NOT give up the fight. Do not worry about being a bitch, rude, a difficult parent, a pushy person. Your assertiveness may be misinterpreted as aggression and you just need to be ok with that. Their feelings do not matter. Your daughter does. A sore ego will heal. But I doubt that Grace will ever forget her 5 days in hell.

Grace is now all healed up but our family has been forever changed by this experience. We have learned new ways to handle her, care for her bones and now we embark on a lifetime of being on high alert, uber vigilant, overprotective, to avoid this ever happening again. And Heaven help the next doctors who don’t listen to me.

Vegan formula recipe for tube feeds

05.04.17

CORRECTIONS :: in the video I said there is no calcium and actually each bottle has 75% daily recommended calcium.

UPDATES :: This thickened up too much in the fridge so the next morning, the pump wouldn’t handle it. We left it to get to room temp and tried again hours later and still too thick. So now we’ve cracked the case: leave out room temp and leave room for water. Thin down just before feeding and works fine in pump this way.

Ok guys. I’ve had a brain storm. Today I posted the first video about switching Grace to real food. I’ve been sad, frustrated, confused and depressed about this whole thing. Like it’s another mountain I don’t have the energy for. Feeling like we’ll never find a way to make it work with our busy lives. And tonight it hit me: why are we trying to switch Grace to 100% blended food i.e. liquidising a PB&J sandwich or feeding her a liquid version of everything we’re eating? How about just starting with making a replacement formula which we can get through her feeding pump and then start adding in more bulky foods here and there? Duh!

In the beginning of all this, the goal was to get away from pumping her with a litre per day of the ready-made formula which includes things like maltodextrin which is the SECOND INGREDIENT after water and high on the glycemic index which causes your blood sugar to spike unhealthily fast. But I guess I was thinking that the only alternative to the formula was to find a way to get cooked food into her until this brainstorm that actually, I could just replace the formula as a first step.

Recipe

1 carton organic, unsweetened Alpro organic almond milk unsweetened (I recommend almond or hemp but I had a bottle of soy in the fridge for this video)
100g organic natural peanut butter (4 tbsp)
1 banana
100g raw cashews

Cost (versus £9.78 per bottle of the pre made formula which is £586 per month if bought privately. The NHS pays £4.88)

Total cost = £4.44
Per 500ml bottle = £1.48
Per 250ml feed = 0.74 pence
Cost per month = £88

Enhancement ideas

Liquid vitamins
Fish oil
Vit C powder during cold season
Green/superfood powders

Nutritional Values per 500ml bottle.

The first number is my recipe and the second number is the pre-mixed formula. My recipe is higher fat because of the nuts but also higher protein. It’s higher sugar, but it’s lower glycemic index sugars and perhaps also the sugar in the peanut butter which is a more processed brand than I’d like to be using.

Calories 500/500
Fat 37g/19g
Saturated 6g/2g
Protein 24g/20g
Fibre 5g/0g
Sugar 9g/5g
Carbs 22g/61g
Calcium (my recipe delivers 75% of daily recommended calcium per bottle so grace gets 150%)

Pros/Cons

Needs to be made on a daily or bi-daily basis
You need a high powered blender (like a Vitamix) if you’re going to do the cashews and this is about £500 OR you can soak them overnight and use a regular blender.
Needs to be kept in the fridge but I imagine sending her bottle to school isn’t a huge deal cuz it’s just a few hours
Here in the UK, the feed is delivered free and this will cost £88 per month. BUT it’s only free to us. It costs the taxpayers about £3,500 per year to feed Grace and I don’t see why that’s necessary if we can do this.

Ditching formula & going back to real food

05.04.17

Note: this post has video in it. If you’re viewing in your inbox, click here.

Grace got her feeding tube 5 years ago yesterday. It was the best decision ever. Not only can we get medications into her without fighting, we can keep her hydrated and fed when she isn’t feeling well. And of course, she hasn’t had aspiration pneumonia since we did it.

With the tube comes a monthly supply of liquid formula delivered to our door (thank you NHS!) and for the last 5 years we’ve been using nothing but that. I mean….it’s so eeeeeeasy. So why not? But recently I started feeling like I was just being lazy. I work so hard feeding the rest of my family but Grace just gets the easy stuff. So anyway, my first attempt can be seen below. It’s a journey we’re on. Weekdays are hardest while she’s at school. I don’t know if we’ll ever be 100% off the formula, but if we can cut it back by half I’ll be happy.

Epilepsy Mumbojumbo: Refractory or Intractable Epilepsy

03.21.17

I’m so absolutely wiped today so this post won’t be full of the witty smartassy brilliance I know you love (eye roll) I’m just popping by to share some new words I learned today (tadaa!)

Grace had her neurology appointment today and the Dr said something to the effect of: “…Grace’s intractable epilepsy…”

“Her…what?”

So here’s the lowdown: I guess 70% of epileptics can be controlled and made to be “seizure free”. When I asked what was the threshold for being seizure free, she said it’s 2 years without a seizure. It never crossed my mind that this was even a goal – that there are people with epilepsy who can achieve going for years without a fit. We can hardly go 2 weeks (or sometimes days) let alone 2 years. So Grace is in the 30% of epileptics with “intractable” or “refractory” epilepsy meaning it can’t be controlled.

She was referred to the clinic for information on starting her on a ketogenic diet. Cannabis could also be an option, but not in the UK.

So there you have it. And now I must crash…ZZZZZZZ