27 May 2015

EDS Awareness: My experience with Ehlers Danlos syndrome and Pregnancy

It’s EDS Awareness Month in May and I’ve decided to write about something that may be on the minds of many young women with Ehlers Danlos- how will having EDS affect me when I want to have a baby?

Ehlers Danlos syndrome is a multi-faceted condition that, much like autism, has a number of different types and within those types, each patient is affected in a different way. The most serious is Type 4, or Vascular EDS. If you are female and diagnosed with this type, you will no doubt already understand that pregnancy is very risky and potentially life-threatening as it can increase the possibility of a catastrophic arterial or organ rupture.

Vascular EDS and pregnancy: statistics

A study published in 2014 found that pregnancy-related deaths in women with Vascular EDS occurred in 30 of 565 deliveries (5.3%). Interviews with 39 women indicated that 46% had uncomplicated pregnancies, while the most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and preterm delivery (19%).

Life-threatening complications occurred in 14.5% of deliveries and included arterial dissection/rupture (9.2%), uterine rupture (2.6%), and surgical complications (2.6%). This is just one study however, anyone with this diagnosis should always seek their own personalised medical advice before attempting to become pregnant.
I've also added some free to access resources at the end of this post regarding pregnancy with Ehlers Danlos syndrome.

Just before Son2 was born

My Experience

While I am not a medical professional, I can talk about my own experience, as someone with EDS Type III, who has been through two pregnancies. Please feel free to leave your own experiences in the comments as well as they will be helpful to other couples.

At the time of my pregnancies, I did not have a diagnosis of EDS, nor was I seriously affected. In fact, my two sons were born 15 years before I fell life-changingly ill with chronic pain.

I had always experienced symptoms at one time or another but as they were transient, by the time I'd decided to visit the doctor they had either disappeared or the doctor wasn't particularly interested. These include unexplained pains that would disappear as quickly as they came, joint pains, neck and back pain, dizzy spells, weird stabbing pains in different parts of my body, cold extremities, weak ankles, low energy, poor sleep and so on.  They just didn't happen, as they do now, all at once and so no one ever put them together into one whole, and even if they had, they probably wouldn't have heard of Ehlers-Danlos syndrome anyway.

If I had realised, I would perhaps have been able to get specialist advice but at the time, EDS was even less known than it is today. Plus, I was working as a television journalist with a new contract with a top agent and I was more concerned with how I was going to manage work and being a mother after the baby arrived.

Hypermobility means that the joints can be loose or unstable and the collagen, which makes up most of the body, is more stretchy than normal. Pregnancy hormones themselves will also increase the elasticity of every expectant mother's body so for someone with EDS, this can mean joints, muscles and ligaments are much more likely to be over-stretched and injured.

Son1 comes home

Early to mid-pregnancy with EDS

Within weeks of being pregnant for the first time, I was suffering with back pain severe enough to send me to A&E. It wasn't regular back pain, but shooting pains and aching in the lower half of my back. As I had previously suffered with back pain, no one was too bothered and the baby was growing well. Very well. I suffered morning/all day nausea and sickness for a while, enough to miss a few days off work, but not too bad.

But the fatigue! It seemed, at times, that the baby was sucking every single ounce of energy out of me. I was working near Tottenham Court Road in London and would stand at the bottom of the steep flight of steps at one of the exits and just look up, wondering how the hell I was going to get up to the top.

In the end, I had to stop work at 6 months pregnant. A 12-10pm shift reading pan-European TV news bulletins may seem like a cushy number, but not when you're hefting around an unceasingly active growing human inside you.
As soon as I knew I was pregnant, I began to use lots of moisturising lotion on my abdomen to stave off the dreaded prospect of stretch-marks. Little did I know at the time, but having stretchy skin comes in rather handy when you're pregnant. I had always thought that making sure I remained oilier than a tanker spill was the reason I didn't end up with a single stretch mark after two babies. However, when EDS finally engulfed me in my forties and I consulted the eminent Prof. Rodney Grahame, he asked if I had developed stretch marks in pregnancy. When I said no, he laughed excitedly, “Ha ha! He said, why do you think that was? Because of EDS!” A small comfort, I suppose, but a comfort nonetheless. However I have heard of some women whose body became crisscrossed with stretchmarks, so as with many “spectrum” type conditions – when you've met one person with EDS- you've met one person with EDS. In other words, you can't generalise. Those women may in fact have a type other than Type 3. 

 Of more concern to me at the time, was that we had a "double" blood test and we were called in because the test indicated an elevated risk of Down's syndrome. Did we want an amniocentesis? My partner and I discussed it and decided that on comparing the risk of miscarriage with the chance of DS, we would not go ahead with the test. If our child had Down's, so be it, he would still be ours.

Son2 with his splint for unstable hips that he wore for 10 weeks

Late pregnancy with EDS

Not long after I stopped working at six months pregnant, moving about at all became very difficult. The baby was growing rapidly and his weight was pushing down on my pelvis. I became unable to walk far without a lot of pain. Getting in or out of the car required my husband helping me in or dashing around to the passenger side to haul me out, laughing, "I'm a comin' Lambsy.." as he scooted round the car.

The problem was Pubis Symphysis Dysfunction, which is when the pelvis over-softens from progesterone and causes pain and instability from the weight of the baby. It usually resolves after the birth but it was one more easily missed sign.

Sheepdog, with his Lambsy

Meanwhile two months before the birth was our wedding day! You might think we'd left it a bit late, but this baby wasn't expected - we had only been together for a short time - but I really wanted to be married before he arrived. The day was long but wonderful and of course, we couldn't go on a big honeymoon. We promised ourselves one at a later date. 18 years on, we still haven't been because raising two sons with autism is pretty all-consuming, especially when you get tired easily (even though I didn't know why at the time)

Here he comes. I said here he comes! Come on!

By my due date on 22nd November, I was desperate for this baby to come out. He, however, was quite happy in my comfy, stretchy body, turning from side to side and sticking a foot out now and again so hard that you could see the outline of his toes. It felt like having an alien inside me.
The date came and went. And then some more days, and some more. I began to feel embarrassed at this failure to appear. I made my husband do three rings on the phone, hang up and then call again so I knew it was him before I would answer it.

I was admitted for an induction on 1st of December. The baby had missed my Dad's birthday, today was my late Grandad's birthday and the 2nd would be my sister, Fiona's birthday. Would it be today? I hoped not actually, as I had shared my birthday, 6th June, with my uncle and it had felt like I had never had a special day all to myself when I was growing up.

The induction medication is given as a pessary, but for me, nothing happened. My husband visited and went home, still nothing but some regular early pains that weren't going anywhere. They gave me an injection of pethidine - it did nothing to make me more comfortable but gave me a huge, painful bruise from the injection - again neither of which is unusual in someone with EDS - if I'd known I had it. I spent the night kept half-awake by the wailing of women whose babies knew that womb time was up and were preparing to emerge into the big bad world. Unlike mine.

Late morning the next day, I was already exhausted from a poor night's sleep. Just as they were preparing to administer a second pessary, my waters broke.

Labour came on so hard I thought I was going to die – no, I was sure of it. My whole body was engulfed in agony and I was moved to a side room as soon as my husband arrived. An epidural was arranged while I enjoyed some gas and air. It didn't help the pain, possibly again, related to the EDS resistance to anaesthetic - but I just didn't care as much as they wheeled me down to the delivery suites giggling and hurting at the same time.

Unfortunately, even though it was a walking epidural, it seemed to slow the labour down and more than 24 hours after full labour had started, the baby was still nowhere near ready to emerge.

In order for a baby to be delivered, the cervix has to dilate (open) and efface (thin) and whether this was as a result of EDS or not, I don’t know, but in me, this process had only occurred fully on one side, not leaving enough room for the baby to exit.

I was exhausted and so was my husband, who was cramming for his accountancy finals in the hospital room in between walking around the corridors with me to try to move things along. First eleven days overdue and now this? Are you kidding?

Finally, it got too much. When I eventually got a midwife who would understand what I was saying - most of the staff were from overseas - a c-section was arranged. My reasoning was that as I was already exhausted, I would not have the energy to push and even now I wasn't sufficiently dilated. I did NOT want anyone using forceps on my baby's head to drag him out, thanks very much. 

At 10:40pm, 32 hours after labour started in earnest, my 8lb 3oz boy was pulled out, kicking and screaming so loudly, even the doctors looked shocked.

I briefly held him, distracted only by the voice of a doctor stitching me up saying, “We’ve got a bleeder here…” What?

I was barely able to believe that this large baby had grown inside of tiny me. I then quickly had to give him to my husband so I could throw up from the effects of the anaesthetic.

Son2 at five months old, his first Christmas

Caesarean a mistake?

I have since wondered if it was a mistake to opt for a c-section, given what was to ensue, but after a day and a half of fruitless labour, I felt there was little choice to avoid the dreaded forceps. I would rather be cut open than have that happen to him. 

So why a mistake, when there was really no choice?

My second baby, 19 months later, was also a c-section. We did try labour but the same failure to efface and dilate was happening. The doctor later noted that I had the formation of some adhesions in the abdominal cavity from the first section which had made the second c-section more tricky.

My second son was born with an unstable hip and needed to be in a splint for 10 weeks. Still no one thought of any connections. He also now has an EDS diagnosis.

Within a year, I was plagued with almost continual abdominal pain and an investigation revealed that the adhesions had spread and were sticking all my internal organs together. Adhesions, or scar tissue, are very common in EDS (which I still didn't know I had).

Son2 had suffered from Reflex Anoxic Seizures from the age of 13 months so I didn't have a lot of time to think of myself and why this pain was happening. On top of this, my older son who had made his embarrassingly late arrival, was making up for it by walking at nine and a half months old, and now dashed everywhere head on, resulting in numerous trips to the hospital to be stitched up again.

No more babies...

To try to resolve the pain, the only option seemed to be more surgery. This time, a hysterectomy, aged just 35, to remove the spidery adhesions that had welded my inner stomach wall, my bowel, bladder, intestines, uterus and ovaries together. No wonder I was in pain!

It did improve things for a while, but I do wonder if I had had an earlier diagnosis, my maternity care could have been better prepared, potential difficulties might have been foreseen so that more pro-active options for a better labour could have been tried.

At the time of my first delivery, being so big and uncomfortable I just wanted the baby to come out. If I'd had more confidence, I would have avoided going in for an induction and just waited at home for labour to start naturally so I could have been more rested.

It was the fatigue, which plagues my life again today, coupled with poor maternal care in the central London hospital ward, that led to the chain of events ending in my first c-section.
Knowledge is a powerful thing; for people like myself it can mean being able to make informed decisions for our own care and being able to advocate effectively for ourselves (and for our families). I hope that by writing this it may help other women with EDS or who have similar symptoms to know what certain decisions may lead to before they are faced with making them.

Some free to access medical articles about EDS and pregnancy:

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9 May 2015

#SilentSunday: The art of smiling while screaming

That moment when you perfect the art of smiling so everyone thinks you're okay...
But inside, your head is still filled with the sound of screaming

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5 May 2015

Thoughts for politicians - and for us all- on doing the right thing

Cowardice asks the question 

- is it safe?
Expediency asks the question 
- is it politic?
Vanity asks the question 
- is it popular?
But conscience asks the question 
- is it right?
And there comes a time when one must take a position 
that is neither safe, nor politic, nor popular; 
but one must take it because it is right.
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