I feel that I should start this post with an apology:
For longer than intended, I have been brushing the details of my medical conditions under the proverbial rug. I wanted to update each of you in person, to give you the chance to ask questions, and to be able to hug you afterwards. I’m so sorry that hasn’t happened.
It hasn’t happened because I haven’t been well enough to arrange dates with all of you, and the times when we have been together in the same room have been happy occasions – weddings, christenings, housewarming parties. There is so much good news being shared, and I hate to pour out the ice-cold rain of my own news over your wonderful, joyful parades.
Another reason that I haven’t updated you is that things keep changing. With almost every hospital appointment there are additional diagnoses, tests, and changes to my medication. Not so long ago I had seven appointments in one week. Too much information, not enough time to process internally, let alone to work out how to share it with my loved ones.
I’ve been trying to write this update for you since last week. I wanted to write about my medical condition in an accurate, but upbeat and accessible, way. However, I’ve been bedbound since last week, which is having an effect on my ability to feel positive about anything, let alone about the medical condition that is keeping me in this prison, comfortable though it is.
Let me rewind a bit:
I have Ehlers-Danlos Syndrome (EDS), which is a genetic disorder. Collagen is a protein that is found in various connective tissues within the body, and is responsible for giving strength and rigidity to those tissues. EDS collagen is stretchy and weak (‘incompetent’ to quote one of my orthopaedic surgeons). Having stretchy collagen in my ligaments allows my joints to dislocate incredibly easily. My left elbow has dislocated eight times in the last month, six of those times while I’ve been asleep. Rather unfairly, sleep is when my joints are most vulnerable, as the muscles relax, leaving the ligaments to hold my joints safely in place. Which they often don’t (or can’t).
Having joints that dislocate all the time is painful. I take a LOT of medications to try to control the pain. Unfairly (again) the pain nerves are wrapped in collagen, which seems to result in inappropriately high pain signals and low response to painkillers, local anaesthetic and sedatives. Apart from the pain, having stretchy collagen and unstable joints leads to fatigue (from the extra work that my muscles have to do to compensate for incompetent ligaments, and from waking up due to dislocations/pain) and weakness.
I don’t intend to write in detail about all the effects that EDS has on my body. Not today, anyway. To be brief (a talent I share with my wonderful husband), connective tissue is found in just about every organ/system of the body, so EDS can cause all sorts of problems, ranging from easy bruising and abnormal scarring, to bladder failure and immune dysfunction.
So why have I been stuck in bed?
The full answer to that is complex, but basically my body struggles to maintain status quo. Heart rate, blood pressure, body temperature, etc. All those things that we take for granted. This heat wave has given me the opportunity to discover just how inept my body can be. Lying flat with the curtains drawn and IV fluids running though a drip, I’m absolutely fine. Within two minutes of standing or sitting upright, I’m bright red and dripping with sweat (just my head and face), I feel dizzy and as though my heart is racing. Lie down again, and everything is peachy. I very nearly fainted this afternoon because I wanted to spend 3 minutes picking blackberries from the end of the garden. By the time I got back to bed, my heart was hammering away at 150 bpm (normal: 60-100 bpm), my temperature was 38.4*C (normal: 36.5*C – 37.5*C), my speech was slurred, and my vision was starting to ‘grey out’ at the edges – usually the first stage of fainting for me. My carer helped me to bed, elevated my legs and restarted the IV drip, and within a few minutes I felt back to normal, though somewhat chastened for not knowing my limits.
You may notice that I said ‘carer’ – yes, I have two carers, who work 30 hours a week between them. They help me to prepare daily medications, IV fluid drip, and tube feed, and to organise medications and supplies. They do all the boring things around the house, which allows me to save my energy for fun things. They are pretty good at spotting when I’m not feeling well and know what to do about it.
Having daily care is one of the biggest recent changes. I can’t think how I managed without their help, and am so glad to have them around, especially when I’m stuck in bed, which seems to be happening far more frequently than I’d like.
There are two other big changes that I need to tell you about:
1. I’ve been given an NHS electric wheelchair to help me get around. I’ve been using it more and more, even inside the house. It saves my poor joints from injury, and allows me to adjust my position, with my legs up and the backrest reclined, so I can be almost horizontal in the chair. Not very practical for moving around, but very useful in hospital waiting rooms, which are often hot and crowded.
2. The focus of my care has shifted somewhat – there is no cure and very few treatment options for EDS and all its secondary conditions. My life is likely to be shortened by EDS (though no-one can say how much), so I want the main goal of my treatment to be better quality of life. I was referred to my local Palliative Care team about a month ago, as they specialise in complex symptom management, and offer the kind of holistic approach that has been lacking in my care. I have been going up to the outpatient centre of St Christopher’s Hospice every week. I’ve been having massages, participating in art and music therapy, talking to the family support therapist, and doing physiotherapy, and relaxation/meditation. The centre is beautiful – light and airy, and with beautiful gardens. Most beautiful, however, are the people in the centre: the patients, visitors, staff and volunteers. They really do light up the place, and fill it with the kind of vibrancy and life that wouldn’t normally be associated with a hospice.
This post is already too long, so I won’t write much more. There will be many more posts – about my health, my thoughts and feelings about my illness, life and death, and about my daily life. I’m sorry that this has been so long in coming, but hope that it explains why I’ve been absent from so many social events recently.
I hope to be able to see you or speak to you soon – I may need to adjust my expectations somewhat, but I’m often able to receive visitors (as long as you know that I might be lying down), and am a huge fan of Skype.
Thank you for reading this far, and for sticking with me through these rocky times. I’m determined not to cry (or sound as though I’ve just been awarded an Oscar), but thank you, thank you, thank you.
Until next time, Jo
*FINE = f-ck-d up, insecure, neurotic and emotional