Ehlers Danlos Syndrome

We saw Evelyn’s paediatrician yesterday.

Digression: How many of my blog posts in the last twelve months have begun like that, I wonder? It seems like it’s all I ever begin with. We saw “insert medical professional here” yesterday and BLAH BLAH your baby is WEIRD. Is it just me? Am I the only one boring myself to tears?

Sorry. Back on track.

We saw Evelyn’s paediatrician yesterday, who immediately let us know that Evelyn’s last lot of bloods showed her to be severely anaemic. Her haemocrit levels were a 3, when they should be at a minimum of 30, and her ferritin levels were a 2, when they should be 100.

Iron supplements have been started and thank all that is holy (seriously, rub your Buddha, praise your God, pet your kitten, whatever floats your boat) she is managing to swallow her meds. Sure, it takes me more than five minutes to give 3ml of iron, a drop at a time, but it’s going in and it isn’t being spat or choked on. WINNING.

Of course, her serious anaemia leads into some serious concerns about the fact that the baby isn’t eating anything except breastmilk and the occasional accidental pea.) Thus far, I’m managing to meet her calorie needs, as exhibited by her lovely chubby cheeks and no weight loss, but I’m not managing to meet her nutritional needs any more – not without some form of supplementation happening. And yes, before you ask, I’ve added an iron supplement to my diet as well, just so that we can cover all bases. Because, EXHAUSTION.

Evie has been referred through to the Hospital Dietician, she is being booked in for a Barium Swallow to check for structural issues, and we’ll start the baby steps to get her coordinating her swallow effectively and hopefully transitioning back to solid food again.

“You need to realise though, this process is going to take months, at least. It won’t happen overnight.” says our Paed, as I rock and laugh maniacally in the corner. How do you supplement a baby who won’t take a bottle or cup? HAHAHAHAAA.

They can work that one out for me.

In any case, Evelyn is under the care of a fantastic team, both at St Giles and The Royal Hobart Hospital. I cannot speak highly enough of their care and commitment to Evelyn’s health.

She’s also been referred through to our geneticist, so that he can look at the probability of Ehlers Danlos (dislocating joints AHOY), or whether there is more testing that needs doing, to look for other conditions as well.

In the meantime, we have a sleep deprived EEG booked for next week. I have to wake Evelyn up at 4am to make sure that she is nice and exhausted and angry and OPINIONATED for the EEG sensors, before hopefully falling asleep and exhibiting her constant sleep-twitching. I’m not looking forward to that one. Actually, I’m not looking forward to anything much at all. The thought of trying to get Evie to do anything she doesn’t want to do fills me with a special kind of dread.

Upside: It’s her birthday on Sunday. I have successfully kept this complicated baby alive for almost an entire year now. CELEBRATIONS. CHOCOLATE. CAKE.

I think I’m winning.

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I know, I know. I shouldn’t read Mamamia. I’m pretty sure it is the online equivalent to Today Tonight and A Current Affair, both of which will make your brain cells commit seppaku in protest. But of course, Mamamia is the best troll* website of the lot of them, and I’ve been sucked in like everyone else.

[*Proper definition of a Troll: Someone online who seeks to incite argument, to shock, to cause uproar. See here.]

The debate this week is all about Birthzillas. You know, those crunchy hippy women who bury their placenta under a tree and care about birthing without drugs. Those women that Mia labels “smug and superior”, forgetting that in the same article she was acting just as smug and holier than thou for wanting a legion of doctors at her command.

And here’s where I clarify that I am not judging anyone’s birth choices. I would love all women to have the birth that feels best for them, with the outcome being a happy one. C-section, epidural, home birth, water birth, midwives – I don’t CARE how you birth.

I do however, reserve the right to care about how I birth.

When my first child was born, I went into that delivery suite with no expectations. In fact, that was my entire birth plan. I hadn’t prepared myself for anything, because anything could happen. Surely that’s a good thing right? An open mind about the whole process?

Unfortunately, I was 17 and despite having a loving and supportive partner by my side the whole time, the midwife and doctor who helped me birth seemed bent on making it a “lesson” on why teenagers should not get pregnant. There was no calming influence, no discussion and when it came to getting my daughter out, I was given an episiotomy and a ventouse extraction, while the doctor screamed at me and the the midwife tut-tutted. My entire labour was a bit over 7 hours long and full of more trauma that I ever thought possible.

They left the student OB-GYN to stitch me up afterwards, while I lay in the delivery bed, legs in stirrups, listening to her curse as she re-did my stitches three times. It was her first perineal repair.

It took me twelve months to heal from the episiotomy. That’s twelve months to heal enough to have intercourse without pain, or to urinate without discomfort.

When I fell pregnant with my second child, I was determined to have a better birth. Still scarred from my first experience, I set out to educate myself as much as possible about everything that could go wrong, so that in my own mind, I was more prepared than the first time. I read midwifery blogs, L&D nurse blogs and as much about the natural processes of birth as possible.

I decided I didn’t want to birth with drugs if I could help it and that my birth needed to be as low intervention as possible, not just for my and the baby’s health, but for my own sanity. I saw a supportive midwifery team and despite a pregnancy that seemed likely to have a poor outcome, I birthed my son in hospital, in a calm environment, with two midwives attending and Nathan holding my hands.

No stitches, no stirrups, no screaming, no loss of control and bodily autonomy. The midwife told me afterwards that she was honoured to have witnessed my birth and help deliver my son.

Now I’m pregnant with our third child and determined to have the birth I want again. It may not go to plan – I have fast labours and we have a backup plan if I physically cannot make it to the hospital. This time however, I have a medical reason for needing a low intervention birth.

Turns out I have a rare connective tissue disorder, that affects my collagen. Among other things, I don’t heal easily or well, my joints dislocate and my blood pressure has an alarming habit of dropping dramatically, making me dizzy enough to vomit and pass out.

These things are not conducive to high-intervention births. If, for instance, I was to have a caesarean, it’s likely I would end up in a wheelchair for a good while after birth. An epidural would have the potential to drop my blood pressure low enough to cause serious damage and an episiotomy, as I’ve already discovered, takes a long long time to heal.

As I creep closer to the point where I will birth this third child of mine, I am well aware of my condition and how it will affect my birth. My midwives and doctors have also been appraised of this – but it’s not their job to manage my Ehlers Danlos. Their job is helping me deliver a healthy baby, while not landing me in a wheelchair. My job is to provide them with the information for them to be able to do this, while keeping the baby and I safe.

This is why I have a birth plan and a more-than-passing need for a natural birth. Not because I am a birthzilla, but because I am educated about my choices and the consequences of them.

So before Mia makes a sweeping judgement about all women who want to give birth naturally, based on a trite dinner party anecdote, maybe she ought to educate herself about WHY women make the decisions they do.

It’s not all black and white Mia.

 

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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 007

24 weeks.

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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.

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18 weeks

by Veronica on April 1, 2012

in Ehlers Danlos Syndrome, Pregnant. Finally.

Alternative title: Not an April Fools Day post.

18 weeks pregnant

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.

18 weeks pregnant

 

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.

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