Ehlers Danlos Syndrome

The reality within the ideal

by Veronica on May 15, 2012

in Ehlers Danlos Syndrome,My body is broken.,Pregnant. Finally.

I went to lie down this afternoon with my son and my book, only to wake up two hours later, kindle akimbo and pelvis screaming at me. I’d call it narcolepsy, but I suspect “pregnancy” is a better diagnosis. I woke up to find that Isaac hadn’t wet himself, that Nathan had cleaned the house and that everything was pretty much perfect. I am inordinately grateful for this.

Ten minutes later as I was sipping a cup of tea to wash down the buckets of panadol I required, Isaac ran up to me, threw his arms around my neck and squeezed tightly, before racing off again.

In contrast, I saw a physio yesterday who reminded me about the importance of good posture, gave me millions of pelvic floor exercises to do, looked a bit stunned when I discussed what joints of mine actually dislocate and sold me a pelvic brace.

It was an interesting appointment, rehashing things I already knew about how to correctly sit at my computer, how to not destroy my vagina forever and how to ease strain on my shoulders.

All of which are perfect in theory, but a little messy in reality. I pointed this out and she responded “It’s about finding the reality within the ideal”.

Which I guess is a perfect description of life, isn’t it?

As much as the ideal version of sitting at a computer looks great, if I sit properly, my blood pressure bottoms out and I either puke, or pass out. Neither are conducive to working, in case you’re interested. And so I write blog posts either lying in bed with a laptop, or with my feet resting up near the mantelpiece. Definitely bad for my posture, but it’s about finding the least bad thing and attempting that.

I keep reminding myself that pregnancy is not forever, that eventually I’ll be allowed to take the good drugs again and that I will have a smooshy new baby as compensation. Sometime in September, I’ll stop wanting to puke all of the time, my joints will stabilise a little bit and my skin will clear up.

Everything is falling apart and yet, it’s all going to be okay. Even if I spend the next three months unable to actually do anything other than write things and lie down, it will get better. Things will ease and I will not remain this unwell for ever.

This is shades of Amy’s pregnancy over again and I survived that, in worse health to start with. I can get through this. Pregnancy is meant to be glowing health and skipping through rainforests, while birds serenade me.

However, I’ve got to get used to the reality, within my ideal.

24 weeks.

{ 4 comments }

This pregnancy is killing me. Figuratively.

by Veronica on April 6, 2012

in Ehlers Danlos Syndrome,My body is broken.,Pregnant. Finally.

First, I will start with a disclaimer:

Yes, I know how lucky I am to be pregnant. All of my pregnancies have been flukes and I am very grateful that I conceived naturally, despite being told that my chances were pretty terrible. But being pregnant was not the end result – having a real live baby at the end is. Thus, I reserve the right to hate the means and love the end.

—

Fourth pregnancy, third baby. I underestimated how hard this was going to be on my body.

I have a disability, which I don’t think about very often, because this is just me. I pass it off as “dodgy joints” or “crappy genetics” but when you get right down to it, I have a disability and my joints dislocate spontaneously, leaving me writhing in pain. I also throw up, can’t regulate my own body temperature properly and have a slightly leaky heart valve, although it’s “nothing much to worry about yet”. I probably also have POTS, but having a complicated genetic disorder means that no one really wants to talk to me about the secondary issues that a fucked up genetic code causes.

This is amongst other things that I try really hard not to think about.

The good news is, my brand of Ehlers Danlos doesn’t come with spontaneous arterial rupture or aneurysm, and they’re pretty sure that if I’ve managed to carry two pregnancies to term without my uterus rupturing, then it’s unlikely that there will be any major complications with this pregnancy.

I’m also incredibly lucky that unlike many other women with Ehlers Danlos, I have two and a half babies to show for my four pregnancies and we are incredibly hopeful that my success rate will be a whopping 75% by the time August rocks around. If I was a duck who’d hatched three babies out of four eggs, you’d keep me. A lot of women with Ehlers Danlos will go through miscarriage after miscarriage, failing to bring a child to viability at all. I seem to have missed that part and for that, I am grateful to my uterus.

All that said, my joints are falling apart. At almost 19 weeks pregnant, the relaxin is firmly coursing through my system and my ribs have forgotten what their main job is meant to be. I keep dislocating my left shoulder while I sleep and my pelvis is more like a wobble board that a supportive girdle of holdi-togetherness.

Last night, after running my children a bath, I turned around and felt my pelvis slip. One hip went one way and the other went in an entirely new direction, while I wondered if I was going to be able to walk again. A little bit of quick thinking and some serious remembering of what a physio said to me and I gingerly managed to get onto all fours and rock my pelvis back into place. The baby didn’t aid me in this, considering s/he wanted to lie transverse, with each end pushing on one half of my pelvis. I guess it was trying to make things roomier in there.

I joked to one of the mums at school that if I can stay walking throughout this pregnancy, I will be incredibly proud of my joints and I am scared that it isn’t going to happen. The pain is pretty bad and somehow, panadol is pretty useless on the ‘your whole body is falling apart’ pain.

Pregnancy is miserable, for me. The baby at the end is not miserable, but pregnancy is the hell I have to go through to get a baby. Even labour is not this tough, or this bone crushingly painful.

My blood pressure and various autonomic nervous system functions are not working as well as they ought and I seem to spin between feeling moderately unawful, to wondering if the floor is going to come up and smack me in the head. (For the record, I’ve not passed out yet, but I’m well versed in laying down wherever I am, in order to avoid the blackout)

It’s exhausting, feeling this crappy. Amy is at school full time and while the break is amazing, she keeps asking why I’m not doing parent help. I tell her it’s because I’m unwell, but really, it’s not all that pleasant to be the one who can’t do anything, because you’re too sick.

I was reading on a “your guide to pregnancy week by week” site about all the symptoms of pregnancy that should have eased by now. The second trimester is meant to be the golden trimester and all I want to do is shoot the writers. The nausea should have eased! Your exhaustion should be a thing of the past! Headaches are caused by hormones and should stop by the second trimester! I want to shoot them, and then bring them back so that I can shoot them again. Pregnancy is miserable.

Finally, in a moment crowning glory, the midwifery appointment that I was meant to have a few days ago – they wrote down the date incorrectly, so that I missed the appointment, because of an admin error. When they remade the appointment, instead of being at the clinic closest to my house, it’s now at a different clinic, a further 25 minutes drive away (40 mins away all up), at a totally inconvenient time, if I wanted to spend any time at home between school drop off and school pick up. I’d ring them and change it, only I’m scared that it will make things even more inconvenient for me. Better the devil you know, and all that jazz.

It’s a good thing I can feel this baby wiggling and kicking around in there and that I wasn’t relying on the midwife to provide me with proof of life, isn’t it?

I know that most of this discomfort will fade into the background once the baby is born and that by 6 weeks post partum, I should be feeling somewhat better. All of this will be a vague memory of discomfort and that is what I’m hoping for.

In the meantime, I am just very glad that this is the last time I am going to be pregnant.

{ 12 comments }

18 weeks

by Veronica on April 1, 2012

in Ehlers Danlos Syndrome,Pregnant. Finally.

Alternative title: Not an April Fools Day post.

I feel huge. And I know in reality that I am not that big, but my lungs are squished, my bladder is squished and my ribs are increasingly uncomfortable. We won’t even discuss the wonky things that my pelvis is doing.

The bad bits:

Nausea continues. I’m still medicated for the nausea, which due to the type of medication has made my breasts leak early. I call this blatently unfair. The meds also give me a constant low-level headache, which is miserable. At least, I’m blaming the meds, it could be the Ehlers Danlos and hormones.

Thrush. You know, thrush is pretty rotten at the best of times, but this pregnancy has thrown my system off seriously and I can’t seem to clear it up, no matter how many probiotics I eat. Hat tip to Blackmores Women’s Bio-Balance stuff that I first tried after a Bloggers Brunch, which seems to keep things manageable, if not cleared up. It’s miserable.

Itching breasts. WHAT IS WITH THAT? The last two pregnancies that worked, I didn’t get itchy skin until the stretchmarks started to appear. This time, my breasts are constantly itchy. It’s driving me batty.

Reflux. Something I am also still medicated for, considering the generalised laxity of my gastric system. My meds keep it mostly under control, until I try to go to bed with anything less than three pillows in strategic positions.

My inability to sit up in a chair comfortably. Apparently, my internal organs have decided that the best place they can relocate to is my ribcage. Unfortunately, my ribcage houses my lungs and there is not enough room to sit up straight and also breathe. I was hoping to get to Melbourne in May for the Emerging Writers Festival, but I may have to give it a miss, considering I don’t think I could sit up for long enough to attend any events.

My blood pressure, which is sitting slightly above dead and requires copious amounts of water and salty food in order to stop the dizziness.

The Good bits.

The increasing pokiness of kicks. I find myself worrying less about the baby dying and more about where it’s going to be placing its feet next.

No new stretchmarks.

An actual baby hanging around in there. That really trumps all of the bad bits, doesn’t it?

And hey, I got to go to the movies with Nathan last week, which was a huge deal. We saw The Hunger Games in gold class, care of vouchers from a mate and seriously, that’s the ONLY way to watch movies, especially when you’re pregnant.

I am contenting myself with the fact that I am almost half way there and that in a fortnight, we have our big ultrasound that will confirm that there is only one baby in there (one wiggly baby, who can kick in three places at once) and what sex that baby is.

If you’d like to start placing bets on what flavour of baby we’re having, feel free to do so.

{ 21 comments }

And thus we begin the week of hell

by Veronica on March 19, 2012

in Autism,Ehlers Danlos Syndrome,Isaac

Today we had the appointment with our new Paediatrician. You know, the one who comes highly recommended? The one who we probably ought to have been referred to eighteen months ago, but we’ll ignore that.

Firstly, I will say, he exceeded my expectations, in a big way. My expectations were low and my nerves were high, but he was fantastic. He read the letters from our old Paed and then asked us to tell him about Isaac.

And so we did.

We covered the EDS, the autism and finally, the bowel issues.

(Oh god, the bowel issues)

All said and done, through a chain of super lucky events – he managed to palpate Isaac’s stomach. This is something no other doctor has been able to do and our last paed didn’t bother to palpate for the last few appointments. Of course, Isaac didn’t let the old paed touch him, so there’s that.

Lo and behold, Isaac has a major bowel impaction, which is probably causing the leakage. His bowel is almost blocked, so therefore, the only poo getting around the blockage is liquid and leaking.

I’m not sure whether to laugh, or cry at this point.

The obstruction is much too large to pass all on its own, and so at this stage, our only option is to use as much medication as possible, to flood his bowel with water, to hopefully start dissolving the impaction.

Our two options at that point were whether we were going to do this at home over 7-10 days, or hospitalise Isaac with a nasal gastric tube and flush his system in 2-3 days. We’ve elected to try at home first (obviously), knowing that if it doesn’t work, we’re headed for the hospital.

And thus begins our week of poo hell.

Our instructions are to dose Isaac every day, until his bowel runs pretty clear and we can no longer feel the obstruction. I can feel the impaction myself and I would say it’s the size of my fist.

I guess we’re just lucky that nothing ruptured? Is that my bright side here?

I’m really pleased with how the new Paed treated us, how he spoke to Isaac and his plans for the future (which at this stage are “I have no idea, but I’m going to research EDS and bowel issues, see what has worked for other patients and I will have a plan in a fortnight when we talk again”), as well as his general knowledge of EDS and Autism. It’s really very nice to have a doctor that you can trust to be a doctor and FIX things, rather than having to deal with everything alone.

I’m just hoping and praying that the meds work and that we can break this impaction down and avoid a hospital stay.

And in the meantime, we medicate, hydrate and wait.

{ 24 comments }

Isaac is more fragile than I’d like to believe

by Veronica on March 8, 2012

in Ehlers Danlos Syndrome,Isaac

Last week, Isaac, aged three, dislocated his fibula running through the house.

I know this, because I watched him fall to the ground clutching his knee and screaming the same sound I make when mine dislocates. It’s a primal scream, designed to bring mothers running, their hackles raised.

It was a quick dislocation and it relocated itself with a slight click as I picked him up. Something I am inordinately grateful for, as I’m not sure I could have reduced a fibula dislocation on my own. Ribs, wrists, ankles? Sure. Fibulas make me vomit because I know very intimately how it feels.

I cuddled him and tucked him into my chair with a warm drink, while I sorted out a support bandage and my urge to throw up and cry.

As a side note, you would think in a house full of dislocating people, it would be easier to find tubular support bandaging. You would be wrong.

He sat quietly for 45 minutes, before getting up and running around again – a little more careful, but not scared.

A few days before the fibula dislocation, he woke up with a black eye. I was baffled, until I remembered him complaining about his sister bumping him in the face. Three days it took for the bruise to fade, while I wondered how his easy bruising would be likely to manifest in the future.

Prior to that, he tore the skin on his back, in a perfect pattern of his lower vertebrae. I wondered aloud at this, until Nathan remembered that he’d changed Isaac on a linoleum floor earlier that day – obviously accounting for the torn skin and bruising.

The mere pressure of having his legs lifted, tore his skin.

We’re more careful now to pad him as we change him.

We’re careful of him, but he is not careful of himself. He is Three! and Three is for running and jumping, for leaping off furniture and pushing the boundaries. He is not scared and I am grateful that fear won’t rule his life yet, even as I watch him with my heart in my throat.

Today he piled a feather doona into a ball on the loungeroom floor and dived into it – tearing the skin on his forehead and probably leaving pieces of himself on the carpet.

It scares me that he is so fragile and that his skin tears so easily.

He is three years old and he thinks he is bulletproof.

While I? I stock up on bandaids and support bandages, lignocaine cream and a hearty dose of guts for myself.

You’d think it would be hard to go through these things yourself. No, in fact, it’s harder to watch your child do it.