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Today is an Exciting Day!

Today started, much like every other day, with no indication of the grey-plastic-covered excitement to come.

The usual medication, followed by medication, with a side-serving of medication, was followed by a rather disheartening phone call from one of the cardiac arrhythmia nurse specialists at King’s. I left her a message yesterday, hoping against hope that she might be able to fix my body and/or stop this overreaction to the hot weather. She couldn’t, of course, but did let me know that she was receiving dozens of desperate messages like mine. That shouldn’t have helped, but it did. I’m not alone in hating my dysfunctional physiology, hating the hot weather, and hating all the ‘normal’ people, basking in the sun.

Medication, medication, medication

Medication, medication, medication

It turns out that I’m doing all the right things. Which didn’t come as a surprise. No arrogance there; just that there are so few things that can be done, and so much time to lie flat on my back, thinking about solutions. If I had been able to sit up, I’d have colour coded my thoughts, sharpened my pencils, and put sticky tabs on every pages of The Textbook, just in case.

In many ways, I’m luckier than others with dysautonomia, as I have a Hickman line (see previous post for more details). I can get IV fluids, lots of IV fluids, without having to go to hospital or to a clinic, and explain to the overworked, overtired and disbelieving junior doctor that there are conditions barely mentioned in Kumar & Clarke that have treatments and medications not even listed in the British National Formulary, and that I need them. Now.

The biggest disadvantage of IV fluids, at least for me, is that they’re not very portable (initially typed ‘potable’, which is also true, but less relevant to the story). In order to run, the bag needs to be higher than my heart, and needs to remain upright with the tubing straight. The rate of IV fluids that I receive depends on my position, my blood pressure, the amount of fluid left in the bag, and whether I sleep under the tubing or on top of it.

Until now!

Because now I have a PUMP! And not just any pump. A pump that is lightweight and portable and battery powered and small.

In case your imagination didn’t fill in the pitch and tempo of that last sentence, it started just above my normal speaking pitch and tempo and got faster and faster, and higher and higher, until all the bats in south east London came to a crashing halt.

All I need to do before starting to use my pump, is read the 83 page manual. Yes, that’s just the bit in English. No-one ever accused the Germans of cutting corners.

More pictures of the pump, and its newly-upright owner to follow (for those not able to witness with their own eyes this weekend). I will be unstoppable!

 

 

 

 

 

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Hickman Line – what is it and why do I have one?

Most of you will have seen the transparent dressing on my upper chest, and the white plastic line underneath it. This is my Hickman line.

What is a Hickman Line?
In technical terms, it’s a tunnelled central line.

Central because the tip of the line sits either just in, or just above my heart (that’s pretty central, no?). To get the line to that spot, it was inserted into a vein in my neck and just gently pushed through the vein until it arrived at my heart. All roads lead to Rome; all veins lead to the heart (with one exception, in case my pedantic medic friends are reading this).

If the line had been left like this, it would be just a ‘normal’ central line.

This is a 'normal' central line

This is a ‘normal’ central line

These are used in hospitals all the time (usually in intensive care units). However, because the line comes out of the skin right next to the vein, it provides a direct route for infection to get into the bloodstream. This is a Bad Thing.

To reduce the risk of infection, long-term lines like mine are ‘tunnelled’ under the skin so that the exit point is a long way from the vein. Hickman lines (a brand name) have a cuff around the line that sits in the tunnel, usually an inch or two away from the exit site. This is designed to encourage the skin to heal around the cuff inside the tunnel, providing another barrier to infection, and anchoring the line firmly in place.

Hickman lines can have one, two or three lumens (separate lines with a plastic outer coating). My line has two lumens: one is reserved for IV nutrition and the other one is used for my normal daily medications and fluids.

Why do I have a Hickman line?
If you were paying attention, you might notice that I just answered that, but there’s a more detailed answer. Because my stomach and intestines are too stretchy, they don’t work very efficiently to break up food and move it through the GI tract, absorbing nutrients along the way. We know that I absorb some oral medications extremely erratically, because there are blood tests to check absorption. We also suspect that I don’t absorb other medications, fluids or nutrients from food. Because I have severe autonomic dysfunction (dysautonomia), my body needs about 3 litres of fluids per day, but I can take in less than 1 litre orally, of which some is not absorbed. In this hot weather, I’m running 3 litres of fluids per day through my Hickman line.

In hospital, it is commonplace to use peripheral cannulae (peripheral being the opposite of central), usually in the hands or arms. In the year or two prior to getting my Hickman line, I had literally hundreds of these peripheral cannulae placed, and hundreds more unsuccessful attempts. My veins are very small, and very fragile, like the rest of my skin. If someone is skillful or lucky enough to get a cannula into one of my veins, it’s almost certain to fail within 24 hours (the policy at my local hospital is to change them every five days routinely, or more often if necessary). After trying all the usual places, I ended up with IV lines in places as unusual  as my fingers and feet.

Some of the medications that I need are very irritant to veins, so must be administered into a large vein, i.e. via a central line, to avoid causing local damage. This includes TPN – the IV nutrition that I am likely to need at home at some point. I had an appointment with one of the specialists at the Intestinal Failure Unit at St Mark’s Hospital (one of only two such units in the country), which lasted for over two hours, discussing my medical history and possible treatment options. All my doctors seem to be agreed that I will need TPN at some point, as my GI tract will continue to deteriorate. The Intestinal Failure specialist was in favour of a trial of TPN sooner rather than later, despite the fact that I’ve managed to gain back much of the weight that I lost when things went really pear-shaped back in 2012, but my local nutrition specialist would like to delay TPN as long as possible, as there are so many risks associated with it, and once started, it’s likely that I’ll be on TPN for the rest of my life.

Finally, I need to be able to use this line and care for it at home. There are very long lines (called PICC lines), which are inserted into a vein, usually in the arm, and are then threaded up the vein to the heart, under x-ray guidance. The main problem with PICC lines, from my point of view, is that if the line is in one of my arms, I can’t take care of it myself. PICC lines are also more likely to come out than Hickman lines and don’t have the cuff that protects against infection.

What could go wrong?

There are lots of reasons not to make the decision to have a Hickman line too lightly. There are a number of potentially serious complications:

  • Infections – any ‘foreign’ object within the body is a playground for bacteria, and a playground that leads straight into the bloodstream is a big risk. Having IV nutrition into a Hickman line increases the infection risk, because TPN is sugary, and bacteria just loooove sugar! I’ve had a number of episodes of TPN, always in hospital, and usually for around two weeks, but I’ve only had one infection. Bloodstream infections can be really serious, leading to organ failure or even death. I was lucky that the bacteria that got into my bloodstream was sensitive to the usual antibiotics, and that I got the appropriate treatment quickly enough to prevent serious damage to any of my organs, and that the bacteria was sensitive enough to antibiotics that I was able to keep my line (with certain types of bacteria, the line has to be removed, as the bacteria can form a ‘biofilm’ within or around the line, which allows them to ‘hide’ from antibiotics).
  • Blood clots – foreign objects, again, provide a point where the blood flow slows down, and if the walls of the blood vessel get damaged, e.g. by the line rubbing against them, chemicals can be released that encourage the formation of blood clots. These clots can cling to the walls of the Hickman line, where they can grow and grow, and then float off around the body. The size of the clot will determine where it gets stuck – big clots stick in big blood vessels; little clots stick in little blood vessels. Mostly, large clots cause more problems than small ones, but if the clot lodges in the lungs, heart or brain, they can cause really serious problems. I’ve been lucky not to have any clots so far, but I’m not on TPN, which is a significant risk factor for clots
  • Damage to the line – some lines can migrate out altogether, especially those that don’t have the cuff around them, but even if the line stays put, it can degrade over time. I lost my first Hickman line due to degradation of the plastic of one of the lumens, allowing a large hole to form. I was very glad to have the second lumen, so that I could still receive the medications and fluids that I need at home, in the month or so that I had to wait for the line to be replaced
Oh dear... Look at that hole!

Oh dear… Look at that hole!

 

Who looks after the line?

The short answer to that is that I do. I get supplies from a number of places, including my local hospital, clinical commissioning group, district nurses and on prescription from my GP. Most people would have district nurses come in to change their dressing, and possibly also to administer medications. The district nurses did come out to watch me change my dressing the first few times when I left hospital, but they couldn’t tell me when they would arrive, beyond ‘Tuesday’, which I found frustrating, and which would have been even more frustrating if I’d needed the nurses to come out to give antibiotics or other medications up to four times daily.

I’m lucky that my line was placed at a hospital where they have an IV team committed to patient safety, and that they were supportive of my wishes to take care of the line myself. It’s not just being obsessive (though it is mostly that), but every person who touches my line is a potential carrier of infection. I can’t check everything, such as the handwashing technique of my local nurses, or whether they have pushed their hair out of their face with sterile gloves. If I take care of the line, I know exactly the standard of care that I receive, every time my line is accessed. If something goes wrong, the blame lies squarely in my court.

 

I hope that answers your questions, but feel free to ask questions in the comments, or by email. I’m always happy to share…

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St Christopher’s Hospice

Friday is the day that I go to my local hospice – St Christopher’s, in Sydenham. I’d like to explain a bit about palliative care, about what the hospice is like, and why I was referred there.

The St Christopher’s website, rather starkly, states that they provide ‘expert palliative care for the dying’, which – to me – feels a bit like a punch in the stomach. I don’t think any of us want to think about ourselves as dying, though death is one of the only certainties in life. I also think that this mission statement under-sells the nature of the hospice.

Palliative care, in its modern form, really started with Dame Cecily Saunders, who founded St Christopher’s Hospice in the late 1960s. For a long time, the emphasis was almost exclusively on people who had a diagnosis of cancer. Palliative care specialists became experts in pain management, and in the control of complex symptoms. Palliative care specialists are also experts in people, and in the emotional, spiritual and practical needs of people facing death.

Increasingly, palliative care is being offered to people with other non-cancer diagnoses: those with conditions that are regarded as life-limiting (conditions for which there is no cure and which are likely to significantly shorten life expectancy).

Palliative care doctors based in hospitals have often complained that they aren’t included in patient care until it is very clear that all medical and surgical options have been exhausted, when the patient may only have a few days to live. In my opinion, this may be because the medical and surgical teams don’t like to feel that they’ve ‘failed’ to find a cure, or that they’re ‘giving up’ on the patient. Whatever the reason(s), involving palliative care specialists only at the v-e-r-y end of life is too late. In addition to expert management of symptoms, if involved earlier, palliative care teams (in hospitals, hospices and in the community) can offer advice on benefits and financial help, funeral planning, and equipment and aids to make life easier (such as wheelchairs, ramps, aids for daily living). Many hospices offer counselling to patients and their families, bereavement services, complementary therapies, and spiritual and emotional support.

In my case, I have lots of complex and interrelated conditions, most of which have an effect on my daily life. I have lots of specialists who know a lot about, say, the changes in pressure within the first part of the small intestine in people with Ehlers-Danlos. Niche doesn’t even begin to cover it. As one might expect, the specialist described above isn’t terribly au fait with the pressure changes in the bladder that occur in women with Ehlers-Danlos. That would need to be dealt with by a different specialist at a different hospital. Not even exaggerating the teensiest bit. I see more than 20 specialists at 12 different London hospitals.

I started to feel as though I was being buried under layer upon layer of medical complexity. Doctors often say that they find me interesting, but really they’re just interested in my joints, my skin, my heart or my DNA. At the same time,the things that I used to use to define myself have gradually been stripped away. I’m not a medic, not a physicist, not an investment banker. I don’t work, don’t have a family. I rarely bake, can only play my cello for a few minutes at a time, and can’t even knit for long these days. With brutal honesty, I’ve been no more use than a bump on a stick recently; I haven’t figured out how to handle having to lie flat, and still hope that things will improve soon enough that I won’t need to adjust all my expectations. Again.

I was referred to St Christopher’s by my GP, mostly for pain control (the pain team at the hospital can only see me every six months, which isn’t much help when my pain is out of control), but also for their expertise in providing quality of life-focused care. My pain is certainly in the process of being controlled, but far more importantly, I’ve been shown that it doesn’t matter what I can or can’t do – I matter because I’m me, and I will matter until the very end of my life.

I look forward to Fridays now. I love the totally accessible hospice building, and thoughtfully-landscaped gardens. I love participating in the art group, physiotherapy and relaxation sessions. I love chatting to the other patients, volunteers and staff, and meeting new people.

The thing that sets St Christopher’s apart from most other medical facilities is how far they go beyond essential care. One example of this is their tradition of afternoon tea. Proper tea, from a teapot, in a cup with a saucer. And homemade cake. Enjoyed while one of the therapists and a volunteer play guitar and sing. Oh, and they offer patients a beer or glass of wine with lunch. All the food is cooked on site, and because lots of the patients have lost their appetites, either because of their illness or a side-effect of medication, the chefs go to great lengths to make the food look beautiful, even using edible flowers from the gardens as decoration. One more example, in case you’re not convinced: if patients aren’t well enough to get out of bed, the staff will take the whole bed outside so that patients can enjoy the sunshine in the beautiful gardens. And they encourage visits from pets. I know I said one more thing, but I just had to sneak that last point in there.

As Ira Gershwin said, “Who could ask for anything more?”

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Applying Make-Up Whilst Lying Down: A Practical Guide

Many people with chronic conditions don’t like to hear that they look well. They hear a hidden implication that questions whether they really are as ill as they say they are. I’m in the opposite camp. I use make-up as a pick-me-up; a way of erasing, or at least hiding, some of the effects that my illness has on my face. You really wouldn’t believe the number of lipsticks I own. When standing up is not such an effort, I’ll take a picture of my lipstick collection to make you gasp.

So I was applying make-up today, whilst lying down, as is my current norm, and I thought I’d share a few of the lessons I’ve learnt:

1. Look carefully at your face before you start. Be honest: which bits can you highlight, and which bits really need to be left alone? The corners of my lips are currently very cracked and red, which would make any lipstick look as though it had been applied by a very enthusiastic, but not particularly dexterous, four-year-old. Normally I wear bright lipsticks to draw attention away from my under eye bags (we’re not talking neat clutch bags – these are more like suitcases), but today it’s all about the eyes to draw attention away from the cracked and bleeding lips

2. Do NOT use liquids of any kind – foundation, eyeliner, blush/lip stain. They have a nasty habit of ending up where they are not wanted (in my case, on my dress, hair and IV tubing), which will make you feel cross and sad. The same applies for loose powders (unless you’re prepared to find caches of powder in unexpected places for several hours, if not days).

3. Consider how much time you have, and how much energy (factor in extra time to get changed if you ignore point 2). Be realistic: there’s no point looking perfectly groomed if you spend the rest of the day asleep. Don’t forget that whatever you put on your face will have to be taken off at the end of the day

4. Use cream compacts or pressed powders wherever possible, and use your fingers if you can – brushes will jump out of your fingers and onto the sheets (if loaded with dark eyeshadow) or onto the floor out of reach (if you’ve only done one eye) and this will make you cross and sad

5. Prioritise: You’re probably going to have to compromise on something, so make sure it’s not something that is important to you. Don’t spend ages covering every tiny blemish and setting the concealer with powder. Yes, your skin will look wonderful, but you will still be cross and sad if you ran out of energy and didn’t put on any eye make-up

6. Ask yourself if you’re likely to cry today (you can blame hayfever, if that makes you feel better). If so, use waterproof eye make-up, or skip it altogether. You can curl your lashes and use clear mascara if you like (apparently clear mascara didn’t go extinct back in the ’90s – who knew?)

7. Be prepared for spills (see point 2): Have a box of tissues, cotton wool pads, cotton buds (Q-tips), a towel and make-up remover to hand (preferably not a liquid one, as this is just as likely to get dropped or spilt as liquid make-up, and will make far more mess). Put the lids back on products as you use them, and keep everything together in a bag, box or tray

8. Use a pillow or rolled-up towel to support your arms. You would be amazed at how tiring it is to hold your arms up off the bed, and no-one will be sympathetic about a shoulder dislocated whilst applying lipstick. Absolutely no-one.

9. Go for products with a high return on investment (ROI) – consider how much time/effort you’ll put in, compared to the effect it will have on your face and general appearance. My ROI top-scorers are blusher, lipstick and eyelash curlers. Medium scorers are BB cream, mascara, gel eyeliner, brow pencil, sheer finishing powder (pressed NOT loose)

Next time I’ll squeeze a small amount of foundation or tinted moisturiser into a small pot and then PUT THE LID BACK ON TIGHTLY to avoid feeling cross and sad when it goes everywhere

If you have some warning that you’re going to be stuck in bed, e.g. after surgery, you can have your eyelashes curled and tinted and your brows tinted, so all you need is a smudge of blusher and a slick of lipstick and you’re ready to go. Or not to go. There you go.

 

 

 

 

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Update

I’m FINE*

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

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