BY RACHEL SALTER
Hello to every single wonderful mummy reading this, wait before we get into anything let me just put down this tea cup that I find is permanently affixed to my hand these days so that I can talk to you all a little better.
Two hands typing now woohoo! I know that you mummies know exactly where I am coming from on the caffeine front but that is not why I am having a chat with you today. I have been asked by the wonderful creators of That Mama Club to start up a conversation about mums with disabilities, invisible illnesses & rare diseases.
Let me begin by telling you all a little about myself. I am Rachel I am 31 and I currently live in Surrey with my husband and our 16 month old little boy Vinnie. I have a rare, invisible disability called Ehlers-Danlos Syndrome (EDS) – Well to be perfectly honest EDS is only one condition I suffer from however I wanted to focus on EDS in my little mini series where I will be chatting about other factors of EDS and the existing overlapping conditions caused by it or that go hand in hand with it.
So, what in the world are The Ehlers-Danlos Syndrome and how the hell am I pronouncing that?
It is pronounced (ELLE-ERS-DAN-LOSS SYNDROME) and EDS is a group of 13 hereditary disorders that affect the connective tissue of the body. Connective tissues connect, support and bind organs in the human body. The disorders are generally characterized by joint hypermobility (joints that extend further than normal) Skin hyperextensibility (skin that can be stretched further than normal) and tissue fragility. The connective tissue a person with EDS is born with is not structured the way it should be. One type of The Ehlers-Danlos syndrome unfortunately can shorten a person’s life expectancy due to organ and vessel rupture. Every individual diagnosed with EDS travels along a unique life unlike another person with the same diagnosis. EDS will never manifest the same in any 2 people and to this day is still incurable. The most effective way to deal with having EDS is the management of each individual symptom caused by the syndrome. EDS is a bit of an annoyance that it can cause multi systemic (effects many organs/systems) issues within the body.
I look back at when I was younger ( a lot younger) and I remember feeling ill all of the time, being in pain constantly, not being able to do things others could do and so easily. I remember knowing I was very much different in the behaviour of my body to everyone else’s however one thing always stood out and strong and that was knowing in my mind and my heart that all I wanted from this life was to be a mum. The yearning and aching I felt to have a baby was so powerful and for many many years while I searched the planet for the elusive ‘One’ that so called ‘Mr Right’ and in true Disney form I kissed my fair share of frogs but alas never found ‘Mr Right’ so I decided that I would try my absolute best to get my body in shape because whether I had a partner or not I wanted to be a mother and if that meant travelling along that road alone then so be it. (This really makes me sound like Bridget Jones ha)
I spent time talking with my specialists because when you have EDS you have more specialist consultants managing your affected body parts and systems than you actually do friends ha! It was in one of my appointments that I told them what I wanted to do and it was at that point they told me that due to my syndrome & because I have endometriosis and polycystic ovarian syndrome that I would struggle to conceive and find it incredibly difficult, however it didn’t stop there. They then went on to tell me that even if I somehow managed to conceive there would be a very high chance that my body simply wouldn’t be able to tolerate carrying and growing a baby due to EDS. It was a lot to take in and I felt that I had all of my dreams crushed in the space of 40 minutes.
In true story form I met Matt (my husband) at what I thought was the worst timing because I was ready to embark on the journey of attempting solo parenting and motherhood and all the scary things I had been warned about by all my specialists like being a mum who uses a wheelchair, or mobility aids, a mother who is heavily medicated due to the syndrome, the attempt of becoming a mother on its own and so much more. I remember thinking “god he’s so handsome, funny and I really want to date him but he’s really getting in the way of my plans” ha ha little did I know that he would accept me for all of my broken parts, flaws, diseases, syndrome, disabilities and most importantly risks.
I realised that my husband was my elusive Mr Right and so I put my plans of motherhood on hold and started building life with him until we were ready to take the plunge together. In the time between meeting Matt and preparing to try for a baby I underwent a lot of hospital admissions for my heart which is being affected by a secondary syndrome that is linked in with EDS called Postural Orthostatic Tachycardia Syndrome (PoTs). PoTs occurs because EDS can cause a permanent dysfunction of the autonomic nervous system in the body which is called Dysautonomia.
So, where did we get to – Oh that’s right the pants part of noticing a deterioration in my syndrome resulting in new and scary syndromes and conditions rearing their ugly head at what I was considering the most crucial and important time in a woman’s life. My heart was being affected, my immune system and lungs started to become affected by another linked syndrome of the EDS. I was getting the most medical help I have ever had in my life, an increase of medications to help with management alongside physios, dieticians, OT’s, and so much more but in my mind I had set out to attempt to achieve something and I was not going to go down without a fight for a chance at being a mum. I got my body into as best shape as I could considering exercise limitation, injuries from simply opening jars and bottles, dislocations of joints from simply coughing and sneezing but I was ready so we took the plunge.
Months and months passed by into what felt like absolute years of time while we waited to see if we could conceive. On this one day after many many negative pregnancy tests and nights full of tears and broken hearts and my husband picking up the pieces sometimes literally because I had sustained an injury from the EDS – I felt a little different one day while I was at work. I noticed food wasn’t tasting the same and I felt a different kind of sick (I have gastroparesis which is a delayed emptying of the stomach due to it being paralysed by the autonomic nervous system so I feel sick constantly) and it got me thinking….Could I be?
So I rushed out and grabbed a pregnancy test in my lunch break & sat there waiting for that annoying spinning timer (the one everyone hates) to finally stop after what felt like a 5 minute wait. The usual feelings of seeing the word NEGATIVE were quickly flooded away by the most overwhelming tears of emotion and happiness I have ever felt in my lifetime as the word PREGNANT appeared on that tiny annoying screen!
I remember feeling the most incredible sense of achievement that the first hurdle I managed to get through regardless of my health taking a bit of a plummet. We were sitting in our very first midwife appointment and she literally could not believe as much as us, that we were pregnant. The team where elated at the news but concerned immediately too because now came the hardest part – Keeping the pregnancy, staying healthy, staying safe & giving this baby every chance to grow and survive as possible while preparing an already very fragile body for carrying a growing baby.
I was completely enveloped in happiness, pure fear, excitement & nervousness all wrapped up in a knot inside my chest. We were told straight away that we would have to have the baby via planned caesarean section due to the risk of significant tearing, bleeding and healing issues due to EDS while undergoing a normal delivery. I also was suffering from my heart and blood pressure significantly now due to the EDS and the midwives feared how my heart would cope with the pressures of bearing down during a normal delivery as my heart rate was incredibly high when doing things like standing, sitting up, sneezing, coughing and other very minimal things. The risk of preterm rupture was increased with EDS alongside the baby itself being born with EDS. It was at this point that it was decided I would be managed under an obstetrician and my son would be scanned very regularly through out the pregnancy and he would undergo a cardiac scan while I was pregnant to check for any defects or irregularity with his heart as I suffer from 2 minor leaking valves due to EDS.
The first trimester for me was okay, I was expecting and prepared by the obstetrics team to be quite ill as my body learned to accept the change and the hormonal changes happening but for me I just inhaled biscuits to stop me throwing up in the car on the way to and from work. I couldn’t have the heating on as the smell made me want to be sick ha and I loved tins of baked beans and mini sausages so I ate those like they were going out of fashion. I still had all of my usual EDS symptoms of dizziness, heart palpitations, painful bones, nerve pain, exhaustion, GI issues, easy bruising and bleeding but considering I was managing all of that and carrying and growing a baby – I was WINNING!!!
This joy continued right through until the final trimester when as suspected my health started to deteriorate slightly. The PGP (Pelvic girdle pain) was very bad, the episodes of dizziness were significantly increased, I was managing my nutrition via artificial food as my gastroparesis was so bad I couldn’t manage a lot to eat. I was incredibly tired & suffered a lot of pain and sprains to my joints while trying to move around and just live life. I had a few bad subluxations to my joints (partial not full dislocations) which took forever to heal and I couldn’t use them until they did. I won’t lie, the last stretch of pregnancy is difficult for any women but I struggled quite a bit with the pressure it had put on my EDS and the symptoms the EDS where causing me to have but every time I felt pain, every subluxation, every moment of fatigue, dizziness, through every cardiac scan, ultrasound and appointment I knew I was achieving something I only ever dreamt of doing and that the end result is worth every ounce of what I am experiencing now.
We had gone this far and achieved this much that our delivery date for our son was drawing ever closer and we couldn’t believe what we had achieved as we prepared for that all important date but my son had decided to give us the fright of our lives when one evening he simply stopped moving and I couldn’t feel him. I drank water (although very limiting because I have gastroparesis) and I changed positions, went for walks and took all advice from the midwives but it carried on and when he was usually active at 11pm at night and I felt absolutely nothing, I started to worry. We were invited into the hospital to get our son checked over and find out what’s happening. The entire drive into the hospital felt like hours, I just needed to know he was okay, alive and safe. We had come through so much and come so far I couldn’t lose him now and I was so frightened that our dreams of becoming parents were falling at the last hurdle because of my health. We arrived in the department and we were quickly greeted by a waiting team who took down my history and as all medical professionals involved in my care, took a very big inhale and exhale before attempting to understand my life, my pregnancy surrounding EDS. We were scanned straight away and instantly there he was, heart beating and all just having a lazy day apparently. Well!! That’s one bloody lazy day to put the fear of god in my eyes I can tell you ha. Vinnie was absolutely fine, his movements had reduced but not to a level of concern in fact this is where they realised Vinnie was somewhat smaller than originally anticipated. Our tiny little trouble maker.
Its time! 5am and we were getting the last things into the car ready to head out to the hospital for the most important day of our lives – DELIVERY DAY! My heart was racing, palms sweaty, everything including the kitchen sink that I thought at the time I needed, packed up high in the car with the empty newborn baby car seat ready and waiting for him.
The pregnancy was one hurdle now I have to survive the delivery of our son.