Blind Panic

By the time this blog post is published hubby and I will be up and getting ready for our trip to The National Ehlers Danlos Unit in Bath, a good two-hour journey to a hospital that has no parking. I am particularly looking forward to hubby losing his cool whilst trying to find a place to park.

Last week I was unable to write a new blog post so took the easy way out and re-blogged a piece from last year. I hate doing that but if I hadn’t there would have been nothing at all. Last week was filled with massive bouts of insomnia / painsomnia (if you're not familiar with that term it means being unable to sleep due to high levels of pain) add in getting my hair cut on the Monday and a trip to the dentists on the Wednesday, I was good for nothing.

I can’t lie I have a terrible phobia of the dentist, which is bad when EDS has really done a number on my teeth. Apart from my front teeth every tooth in my head has a filling, my front teeth are losing their enamel and are also crumbling. I should be a regularly attending patient but three years ago I stopped going due to my health being bad and never arranging an appointment to go back. The dentist that I had built up a good relationship with, that knew about EDS and would listen when I told her to stop has left to work in the private sector. If we could afford to see her, I would but we don’t have that kind of money, so I am now having to “break in” a new dentist.

The last appointment I attended had been on my birthday, it didn’t go well as I was so tense I could barely open my mouth and he seemed not to believe me when I told him he was hurting me. Not a great start when all he was doing at the time was examining the condition of my teeth. I learned from this appointment I needed two fillings as I had developed large cavities in my rear molars. One on the right side and one on the left, the cavities were so large and deep they could be seen on the x-rays he took. You would be correct in thinking that I was not looking forward to getting them sorted out.

Before I left the appointment on 1st November I went through with him that I needed a local anesthetic without adrenaline, one with adrenaline will cause me to face plant when leaving the dentist’s chair, as one previous dentist found out many years ago before I had my diagnosis of EDS and PoTs. Due to getting my diagnosis at 37, it means I have endured more than my fair share of painful dental procedures, with dentists shouting at me that I “couldn’t possibly feel anything” because I had been given local anesthetic. It’s not only dental procedures that have been plagued by pain but also small procedures such as having part of my toenail removed and a lumbar puncture. Until the diagnosis I got shouted at a lot by the medical profession, simply because they didn’t believe that the local anesthetic they had injected wasn’t working. Now although they acknowledge that anesthetics don’t work properly they underestimate how long it will last for.

I knew in my heart I was going to be in trouble yesterday when the dentist administered the injections for both fillings at the same time. I wanted to pretend it was going to be ok and that it was a situation where it was mind over matter. The first filling went ok, although I really could have done without the running commentary of what he was doing. I spent the whole of the first filling trying not to vomit which is my normal feeling when I hear the dentist’s drill. I really didn’t like it when he told me that the next bit of equipment was going to vibrate a lot making my tooth feel like it was going over cobbles. Another wave of nausea hit me when he told me that he was removing the “soft stuff, to get down to a good hard base”. I wanted to tell him, I really didn’t want to know but as I had his hands in my mouth and the dental nurse sticking the suction device to my tongue constantly, he wouldn’t have worked out what I was saying anyway.

Filling number one went off near perfectly, this was a good dentist experience, other than the running commentary. I was a perfect patient apart from the hyperventilating at the start and gripping the armrests until my knuckles went white. I didn’t find my happy place, which I try to do when stressed to the max during medical procedures. I am pretty good at meditation and can drop off during MRI scans because I just go into my head, the dentist is a different story coloured by so many bad experiences.

I was pleased, the dentist was pleased but it was short-lived due to filling number two. I should have said something or made some sort of guttural noise (as you do when the dentist has your hand in your gob) when he blasted the tooth with cold air and then stuck the metal prong into it. I tried to convince myself that I was imagining the sharp stab of pain. Concentrating on my breathing, I braced myself for the drill, trying very hard not to panic.

Within seconds of the drill hitting my tooth, I entered what can only be described as a state of blind panic. Until yesterday I had presumed that the state of blind panic was down to a weak mind, an inability to control one's fears when faced with them. I have a fear of heights yet know that if my life depended on it I would overcome my fear, I really don’t like daddy-long-legs or spiders yet will deal with them if I am alone in the house. I am scared of them but I would never describe it as a phobia. People tend to throw the word phobia around quite a lot but I can say hand on heart that my “fear” of dentists has now developed into a phobia. Yesterday I experienced a state of blind panic, where I couldn’t think clearly nor see the danger I could have potentially put myself in. I know now that it is nothing to do with self-control or a weakness of mind and I unreservedly apologise to those people I have doubted in the past.

As soon as the drill hit my tooth, I had immense pain (I know my pain threshold for dental procedures is considerably lower than my normal pain threshold) without thinking I grabbed the dentist’s drill and threw it out of my mouth. I immediately sat up and had the dentist not put his arm across my chest I believe I probably would have attempted to leave the room via the window opposite me. It all happened so quickly that there was no thinking involved, I just wanted the pain to stop and to leave the chair. The dentist was really freaked out but didn’t lose his cool with me. He did say “never do that again, it’s so dangerous, you could have really hurt yourself” in a very quiet voice. I apologised and then burst into tears, not my usual tears which are quiet and restrained but full on chest heaving sobs where I could barely speak. The dental nurse gently rubbed my arm and dried my eyes. I cried so much that the shoulders of the top I was wearing were wet. When I later looked at myself in the car on the way home, I resembled something like Alice Cooper with mascara all down my face.

I think at this point the dentist didn’t think I would allow him to continue but rational Rach walked back into the room. As long as I had adequate pain relief I would be able to continue. I had told him on 1st November that local anesthetic wears off quickly with me and he would only have a small window of opportunity to get the job done. I guess sometimes they don’t believe their patients and need to see it to believe it. I wish they would take what we say as the truth because if he had, I wouldn’t have had to go through that.

I often joke that it’s been a good appointment if the dentist still has all his fingers, I guess now the joke will be it's been a good appointment if I haven’t grabbed the dentist's drill or tried to escape the chair mid appointment.

The dentist was a little surprised when I could feel the third injection go in, that part of my gum should have been numb but it wasn’t and proved the point that the original injection for the second filling had worn off. He waited a few minutes before beginning work again. I told him before he started that I would sit on my right hand so that should the urge to grab the drill come over me I physically couldn’t. We agreed that should I feel anything I was to raise my left hand and he would stop. This was the system I had in place with my last dentist and it had worked well, although I have never had to sit on my hand before. But then I have never ripped a dentist’s drill from my mouth either.

After all that drama the second filling went off smoothly. I left the dentist’s without face planting but by the time I got home I needed to just crawl into bed. Today I feel the same but my back is so sore from having so many days in bed lately that I am hanging on for as long as possible before returning.

I don’t have to attend the dentist until March 2017 and that is to see the hygienist, my teeth still need cleaned. I am apprehensive about it as it seems all the nerves of my mouth are on the outer edges of my teeth. At least the anxiety of the dentist’s appointment and the recovery time needed after this little jaunt out have stopped me thinking about my trip to Bath. every cloud eh?

Could it be Postural Orthostatic Tachycardia Syndrome - PoTS?

I posted this in August last year. Due to having a crazy week (Insomnia/painsomnia)  and a dentist's appointment today I find myself too exhausted to write a new blog post.

I had one of those moments this week when searching through the archives of my blog I realised that I had never done a straight forward, honest to goodness post about PoTS. I may have mentioned it in passing but there is no one post based solely on it. I know that many people use my blog to try to explain their medical conditions to friends and family, so I am sorry its not happened until now.

My own diagnosis has moved away from "just" PoTs (although that is still part of it) and is now called Severe Autonomic Nervous System Dysfunction, which as far as I can tell is just a long winded way to say Dysautonomia. Which isn't a medical term that seems to have caught on in the UK. All it means is instead of just the basic PoTs stuff going on I have developed O.I (orthostatic intolerance - my blood pressure drops in to the toilet on standing), breathing difficulties which have been with me since the MG/ not MG diagnosis, bladder problems ( I get acute urinary retention on a regular basis), unable to maintain my body temperature just to name a few. This is a new and emerging area of medicine and many medics are yet to catch up on PoTS let alone Dysautonomia. Straight away please let me direct you to this website pots.uk.org so if you are currently battling to get a diagnosis you can print this guide for gp's and take this with you at your next appointment.

The first symptom I developed was sweating heavily when sleeping, be it a nap or a proper night's sleep. That was back in 2006 and which I now know is reactive hypoglycemia (blog post) from monitoring my blood glucose levels. Throughout my life I had been told I had low blood pressure (not a prerequisite for PoTS as people with all levels of blood pressure can have it), I had a lot of dizzy spells, odd cravings for salt, palpitations. I put my palpitations down to anxiety, as I had always been quite an anxious person. However once my anxiety issues had been dealt with and I learned how to combat them I continued to be left with a feeling of my heart wanting to beat out of my throat on random occasions. I would also on a regular basis get very lightheaded when changing from a sitting to standing position.

So many patients with PoTS are misdiagnosed as having anxiety, health anxiety or depression or other mental health labels before they eventually get diagnosed correctly that it is criminal. Unfortunately once you have had the mental health label attached by the medics it is a hard one to shake off. Even now when going into hospital as an emergency, I still get somatiform disorder brought up when it has been completely and utterly disproven. The problem is that to an uninformed doctor PoTS can sound very like anxiety, racing heart rate, tremors the feeling of adrenaline coursing through your body and an exaggerated flight or fight response. With the majority of PoTS patients also being women it can be even harder to be taken seriously. It seems the modern medical profession still believe in the condition hysteria.

By the time I discovered the little known condition of PoTS, like many I had been written off by the medical profession as overly anxious, internet searching for syndromes with probable somatiform disorder. It took me over a year of battling to finally get the test that would prove beyond all reasonable doubt I had PoTS. A medical condition at the time my old gp had told me didn't exist. For more info on my experience of the Tilt Table Test please click here as I don't want to bore you by repeating myself.

My main symptoms at the time of diagnosis were as follows

1. palpitations
2. feeling faint or fainting (aka pre-syncope and syncope. Please remember only about 30% of PoTS patients faint but many feel faint)
3. feeling like an elephant was sat on my chest and not being able to breathe properly
4. racing heart rate every time I changed position, even rolling over in bed.
5. greying out, especially after eating.  (My peripheral vision greys out)
6. Insomnia (BIG TIME)
7. Dizziness
8. Migraines and shockingly bad almost migraines
9. Cognitive deficit (aka brain fog /aphasia/ short term/long term memory problems)
10. Chest pain (too many times to mention I thought I was going to have a heart attack)
11. Reduced sweating (some people start getting excessive sweating, I only get excessive sweating at night)
12. Fatigue

This list probably doesn't cover all my symptoms and unfortunately many PoTS symptoms also merge into EDS symptoms as in the gut problems and bladder problems that many of us with both conditions have. It can get very difficult to differentiate the two. For a full list of symptoms please click here.

So what can you do if you suspect you have PoTS?

I can only base this on how I went about getting my diagnosis but it is the same advice I give everyone who asks my advice. 

Firstly get hold of a decent blood pressure monitor especially one that can also take your pulse at the same time. Here is a list of blood pressure monitors that have been validated by the British Heart Foundation as accurate enough to monitor your blood pressure at home. If you can afford it get one with a memory so it records your readings so that you can't be accused of making the numbers up. It is important to understand that blood pressure does not play an important part in getting a diagnosis. Some people with PoTS have normal blood pressure, some have low and others have high. Why you need the BP monitor is so that you can start tracking your pulse and your blood pressure. If you can't afford a blood pressure monitor for whatever reason, a pulse oximeter ( a little device than can read your pulse by clipping onto your finger) is the next best thing and you can pick them up online from as little as £10. 

Please remember people with PoTS tend to have very cold fingers (and toes) so before using a pulse oximeter ensure your fingers are warm enough to provide an accurate reading. Before my second tilt table test (yes I was stupid enough to do it twice) I had to sit with my fingers in a bowl of warm water as the pulse ox couldn't get a reading.

Secondly understand what PoTS is!

PoTs is a rise in your pulse / heart rate of at least 30 beats per min (bpm) or hitting 120bpm or over, within the first ten minutes of standing. In children (up to the age of 19) the rise needs to be over 40bpm. If your heart rate doesn't increase by 30bpm or over 120bpm within the first ten minutes of standing it is unlikely to be PoTS. 

When a normal person goes from sitting to standing their pulse / heart rate will increase on average by 15-20bpm. Within a minute or so their heart rate will go back to normal. A person with PoTS could start with a baseline heart rate of 80bpm and on standing increase to at least 110bpm. Then as they continue to stand their pulse could increase further. I know on my own tilt table test my own heart rate increased to 150bpm, unfortunately I don't know what my baseline measurement was. Usually my pulse is in the 80's and if that was the case I certainly managed the increase of 30bpm and smashed the 120bpm threshold.

Because many people (but not all) with PoTS also seem to have low blood pressure that is why I recommend getting a monitor. It helps to see what your blood pressure and pulse are doing after specific triggers such as climbing the stairs, raising your hand above your head, eating - particularly carb loaded meals. Many people with PoTS find their symptoms are much worse after eating heavy carb meals, so it is recommended to eat high protein low carbohydrate meals which don't cause postprandial symptoms.

Thirdly conduct your own poor man's tilt table test.

You maybe wondering what on earth is a poor man's tilt table test, that is ok I was left scratching my head after first hearing this term on a forum called DINET. Because none of us own our very own tilt table designed for testing people for PoTS amongst other things, we can replicate how we will respond to the actual test by completing the poor man's tilt table test and some gp's who are PoTS savvy will do this in their consulting room or at the very least conduct a standing test.

To conduct a poor man's tilt table test, you need somewhere comfortable and relaxing to lie down, without interruptions and preferably away from harsh or natural light. A wall to stand against which is adjacent to the relaxing lie down area however if you don't have this don't worry you can still conduct the test. You also need your bp monitor / pulse ox and a handy sidekick to record your test results. Also your sidekick can rescue you should you faint whilst completing this test so please be careful and do not attempt to complete it without having someone with you. Please also ensure that the area is safe should you decide to face plant, so free from things you could hurt yourself on. Cushions or pillows on the floor can help prevent injury!

To start the test you need to lie down and relax, no talking, no interruptions for 20 minutes. If you can darken the room in anyway before starting the test do. At the end of the 20 minutes with as little movement as possible take your blood pressure and pulse readings. These are your base line readings and what you will use to compare against the reading you get during the test.

Once you have your baseline readings stand as quickly as you can placing your back against the wall. The wall just helps prevent you from moving, people with PoTS tend to be natural fidgets because we either faint or feel like we are going to faint. Moving keeps the blood flowing, we don't want you to do that whilst you are standing up as it may mess up the results. If you don't have a wall to prop yourself up against try and keep your legs as still as possible.

Then at 1 minute, 2 minutes, 5 minutes, 7 minutes and 10 minutes take readings of your blood pressure and pulse or get your trusty assistant to do it. After the ten minute reading has been taken, sit down get your legs elevated and have a drink of water. You may notice that your feet / legs have gone a nice shade of red or purple. Don't be alarmed this is called blood pooling and is very common with PoTs.

Now take a look at your readings if it shows an increase of 30+ bpm / 120 bpm this is positive for PoTS. Do not despair if it doesn't show PoTS one negative poor man's tilt table test does not mean it's not PoTS, it may just mean you weren't particularly symptomatic that day. Try to complete the test on a day when your symptoms are more evident.

Other things that might show during a poor man's tilt table test are things like orthostatic intolerance ( your blood pressure goes below 90/60 on standing), you may have also fainted, if so I am sorry. The blood pressure readings could also reveal if you suffer from hyper-PoTS, which is where you have high blood pressure and PoTS. This needs to be treated differently to "normal" PoTS as most doctors will avoid giving you medication that will increase your blood pressure.

If you can't for whatever reason do the poor man's tilt table test you could perhaps try what is known as the standing test. Instead of lying down you sit down for 5 to 10 minutes to get your baseline pulse and blood pressure readings and then stand for as long as you can. Taking your readings at the same intervals as the poor mans test. This can also show PoTS. Remember PoTS stands for postural (position) orthostatic, tachycardia (fast heart rate / pulse), Syndrome (collection of symptoms and no two patients are alike).

What to do next?

Print off the gp's guide from http://www.potsuk.org/gp_guide and book an appointment with your gp or a doctor at the practice who has been the most supportive. Sometimes these aren't necessarily the same. Whilst waiting for your appointment take a look (if you are in the UK) at the Doctors list on the same website. This will give you an idea of who you can ask to be referred to, not all cardiologists are knowledgeable in the area of PoTS so it makes sense to see an expert, rather than someone who will give you the run around. For international readers DINET also has a Physician's list.

If you have a disappointing gp visit and they still will not listen to you even when provided with your own test results and the gp guide, do not give up. Try every doctor at the practice if you have to and if that doesn't work contact the practice manager. If you get no joy after all of that try reaching out to one of the many UK facebook PoTS groups. They have members all over the country and they maybe able to help you find a new gp practice with more sympathetic gp's who are aware of the condition. 

If you have a disappointing consultants visit because despite your request of being sent to a specific doctor who is knowledgeable about the condition and you didn't get to see one, you have a right to ask for a second opinion and ask once again to see one of the doctors named on the potsuk.org website.

You will get there in the end!

Most doctors will not confirm a diagnosis of PoTS without conducting a Tilt Table Test, ECG and possibly 24 hour blood pressure / heart monitoring. I was diagnosed on my tilt table results alone possibly because from 2007-2011 I had so many other investigations conducted they decided nothing more was needed.

The good news is that many people recover from PoTS especially if they have primary PoTS. Primary PoTS is usually caused by pregnancy, a virus, trauma or prolonged bed rest. Astronauts suffer from PoTS when returning to the Earth's atmosphere and when their bodies re-adjust to the Earth's gravitational pull their symptoms disappear.

Teenagers who develop PoTS also have a high chance of growing out of it. Researchers believe that teenagers develop PoTS due to the hormonal changes and growth spurts they are subjected to. Patients like these tend to grow out of their symptoms by their mid- twenties.

Patients with secondary PoTS, so PoTS that has happened due to a primary condition such as cancer, autoimmune diseases (like M.S, Lupus, Sjogrens), Ehlers Danlos Syndrome can also look forward to on the whole having the condition managed through medication and lifestyle changes (exercise, increased fluids, salt, adequate rest).

However I would be remiss if I didn't state that there are patients like me that have refractory PoTS (it doesn't respond to medication) and go onto develop more issues relating to our autonomic nervous system. We are the minority though not the majority. I don't want to scare anyone who has just started on this journey I just want to be truthful.

My symptoms now are pretty much the same as when I first started on this journey with PoTS / Dysautonomia. I still have Ptosis on and off which no one has ever been able to explain. I still have issues with my breathing, fatigue, syncope and pre-syncope. A good day means that my heart isn't continually feeling like it is fighting out of my chest on every change in posture. A bad day means not being able to sit up in bed without feeling faint, my heart rate not dropping below 100bpm even at rest. 

With my combined conditions each day is very much different with a new set of symptoms to overcome. It is exhausting, it makes me angry, it makes me sad and quite a lot of the time it makes me laugh because my body is just so screwed up!

One last thing.....if you are diagnosed with any type of tachycardia, including PoTS and you hold a driving licence you must inform the DVLA. You can do that via this Link. You will also need to contact your car insurance providers.

Another great source of information is Stars another charity which helps people suffering with a variety of conditions including PoTS.

#5 a comedy of errors

On 31st October I made yet another trek to the hospital for caffeine infusion number 5. They do work and give me a little relief from the awful pain that is being caused by the cerebrospinal fluid leak. The results of each infusion is different, sometimes I get complete relief from pain for several days other times it is just a few hours. This time it was just hours, with the pain knocked down the pain scale for the following few days. Alas it is starting to increase again.

Although the infusion had been planned meaning I didn’t have to make numerous phone calls to get it booked in, the word disaster would cover it quite aptly. We arrived early, hubby, Sharon (my hospital buddy) and I at 9:30am, my appointment was 10am, so when the nurse ushered me straight through to get the cannula set up I was pleased as punch believing the infusion would be under way quickly and we would be able to get home soon. How silly of me.

We were greeted by the nurse that had an issue with my wheelchair (he claimed it took up too much space) last time, which made me uncomfortable as we had brought my new chair, which doesn’t fold down but is much more comfortable if I have to sit in it for a protracted amount of time. On getting me settled on my usual chair / bed thing he immediately told me that my wheelchair would have to go back into the waiting room, leaving me stuck if I needed a wee. I presumed that the offending wheelchair would be allowed back in with me once Sharon and Jay were allowed to join me on the unit. Yes this nurse also demanded my husband and friend stay in the waiting area until my cannula was in. Why I have no idea but as I was going to be in his care for several hours I thought it best not to antagonise him.

Before any cannula attempts were made, the nurse filled out a medical record form. Every time I am in this unit something different is done, this time I was asked for next of kin and what I was being treated for. The nurse had put low-grade headache, to which I nearly exploded but instead  contained my simmering rage and informed him that I had the same CSF Leak that I have had every four weeks since the end of June. I know they have lots of people to remember who go in and out of the unit but they all remember I am the caffeine lady and that I have shit veins to cannulate. So much so that some of the nursing staff actively avoid having me as a patient because my veins are such a nightmare.

Twenty minutes later with one attempt at a cannula made, Sharon came into the unit. The nurse made a face, as to let her know she wasn’t welcome and she stood up to him by telling him something along the lines of “I have come to spend time with Rachel, I am not staying in the waiting room. And can you get me a chair please.” I loved it, as I could see how pissed of he was. She told me Jay had told her to come on in and that there would be nothing they could do. Jay was going to stay with my wheelchair out in reception, as he doesn’t do needles.

Thankfully cannula attempt number three worked (after twenty minutes of having a surgical glove filled with hot water placed on my arm) but I was now waiting for the caffeine to come from the pharmacy. The reason offered was that the pharmacy wouldn’t have processed the prescription that had been completed (4 weeks ago) if it had been sent in on Friday (28th Oct), so they had given it to them today. That was a little concerning as caffeine is such a rarely used drug that I was now at the mercy of the pharmacy having it in stock.

Cannula left arm

I had seen my neurologist the week before and had asked him if it would be possible to have my occipital nerve block injections. I was unaware at the time that he was the duty neurologist, so he popped by and saw me. He also introduced me to the “headache” nurse who would be able to perform the injections today. I was so grateful I shook his hand as the occipital neuralgia pain had been getting much worse since my last lot of injections ten weeks prior. Again I shouldn’t have got excited as the experience was brutal.

Thirty minutes later (now an hour after I have arrived) the caffeine infusion still hadn’t been started as the caffeine hadn’t been dispensed from the pharmacy, the “headache” nurse came back and asked if I wanted to have them done, to which I agreed. She then produced a consent form, I told her I had never had to sign a consent form before to which she replied “nothing surprises me”. The consent form then had to be corrected as where it stated what my medical condition was it said “chronic migraine”. As she hadn’t seen my notes she had presumed what was wrong with me. That wasn’t the only mistake to be made on the form, after I had signed my name I had to print it underneath. I managed to print my maiden name, that hasn’t happened in years. I can only guess that having Sharon there reminded me of the days before I was married.

This nurse had a totally different approach to the occipital nerve block injections than the doctor who had been doing them previously. The doctor would inject the local anesthetic in 4 different locations, this nurse just did two injections at the base of my skull and rammed them in. It hurt like hell and it was all over in seconds. Sharon was in a state of stunned silence, when the nurse left she said “that was f**king brutal Rach” and she wasn’t wrong. Needless to say I wasn’t impressed, I have been left with pain from the injection site and I still have occipital neuralgia. I have been using my Tens machine placed on my neck and the base of my skull to combat the pain. This is the first time these injections have failed to work, I am guessing it is something to do with the way they were performed.

Jay had now been sat in the waiting room for 90 minutes and was not best pleased that the caffeine infusion hadn’t yet been started. Nor was he happy that a wheelchair that would cost over £2,500 to buy new was having to be left unattended in the waiting room whenever he wanted to come and see me. I could sense he was getting tense so told him to go and pop into his parents, as he doesn’t get to see them very often. I also told him to take the wheelchair with him as I didn’t want my chair to be left in the waiting room with no one keeping an eye on it. He took me up on the offer deciding it was the best thing to do because at this point we had no clue how long it was going to take to get the medication from the pharmacy.

At midday, 2.5 hours after arriving the caffeine infusion still hadn’t been started. Sharon went and got us some lunch as we were both starving. She brought back a delicious pile of goodies so we got stuck in. I was so happy to avoid the hospital sandwiches which are totally vile but I usually end up eating because I couldn’t face breakfast. I have to say that was probably the highlight of the day, the cookies she bought for dessert were divine. Good old Marks & Spencer's, even the crisps were out of this world.

As I was stuffing my face another nurse came over to start the infusion. She flushed the cannula that had been in situ for around 90 minutes but the vein had collapsed and it felt like someone was pouring battery acid down it. I would now need another cannula inserted. Knowing that he had been defeated by my shitty veins and no one offering to have a try, the original nurse went off to get someone else to have a go. None of the nurses in the unit wanted to try, as all of them have at some point and know how difficult it is. I was starting to get concerned that they would abandon the infusion if they couldn’t get a line in, leaving me with ever-increasing head pain and no end in sight.

The ward manager was brought in to attempt to get a cannula in, he was cocky which bothered me as those types don’t listen when you tell them not to dig around if they fail to get a vein. He did however ask me where was good for a vein on my right arm, my left arm was now covered in horrendous bruises that had huge lumps under the skin.

The first attempt failed after looking promising initially by providing a flash of blood, which then stopped. My veins like to tease people by doing this on a regular basis. When it stopped working, I wasn’t surprised and nor were the rest of the staff in the unit. All of them at some point have been conned by my veins. Only two people so far have got a vein on their first attempt and both of those were junior doctors but they were only brought in after several other attempts had failed. He then went for the back of my hand which up until now had been a good place to try as on more than one occasion it has been successful. That one failed, so the tourniquet was applied tightly to my upper arm and I was asked to make a fist.

It seemed that I was doing this for ages when all of a sudden the nurse jumped back, as he did that I lifted my hand up from the table only to realise the back of my hand was soaking wet. When the attempt on the back of my hand had been aborted, there had been no blood at all so the nurse hadn’t bothered to put anything on it. Now blood was oozing out all over the table and dripping onto the floor. The nurse had jumped because he thought he had cut himself when he saw the blood and hadn’t realised it was coming from me. It took a while to stop the bleeding with firm pressure applied. Finally on his third attempt he got a cannula in and the caffeine infusion was started without delay.

About an hour after the infusion had been started Jay came back onto the unit with my wheelchair. The nurse who seems to have an issue with mobility aids was on his lunch, so I told Jay to sit in it whilst we waited for the infusion to finish. None of the other medical staff on the unit have a problem with my wheelchair, there is more than enough room for it, it’s just this nurse seems to make it his mission to make me feel uncomfortable about its presence. Due to the fact I don’t want to have to make a formal complaint about him I have held my tongue however should he start on my next scheduled visit I will speak up. It’s bad enough having to come to terms with the fact I need to use a wheelchair, without some idiot making me feel uncomfortable about its presence in a hospital unit.

Five hours after we arrived at the hospital we left, with the next infusion date booked. This time I have booked it for a Thursday, as the nurse who I have seen since June has told me he will be able to put the prescription in on the Wednesday, so that we should be in and out in three hours if my veins play ball.

Left arm 24 hours later

Right arm the following day

Right arm 48 hours later

Left arm 8 days later

My birthday on Tuesday (1st Nov) was a sedate affair as I was completely worn out after the hospital debacle. Jays birthday the following day was mainly spent in bed. We both had really lovely presents given to us and lots of messages on social media. On Sunday 6th November it was Mollie’s 12th birthday. So here is a photo of the old girl, who was thoroughly spoilt.

Frankie, Mollie & Willow

The most un-birthday-ish ever

Next Tuesday (1st November) or last Tuesday by the time you read this, is my birthday and this year I am feeling the most un-birthday-ish, I have in years. I just can’t get excited about it, nor my husband’s the following day. I have bought and wrapped all his presents, I am looking forward to giving him his presents but if we could just gloss over mine I would be more than happy.

Saying that though I have been incredibly irritated when hubby has tried to open my cards (that have arrived by post) at the same time as his. I have also banned the cards that have arrived being put up on display before the day. Hubby has a dreadful habit of opening everything on the lead up to our birthdays and this year this has got on my very last nerve. I have so little this year to look forward to, that I have decided none of my cards will be opened until the day. I have no problem with him opening the post the rest of the year but my birthday cards are just that mine.

There are a lot of things happening on the lead up to the big day, at the weekend hubby is going out with his friends, so I have a really good friend coming to spend Sunday into Monday with me. Up until now I have been pretty excited by it, we have been planning it for months but now I am filled with anxiety that she won’t enjoy herself or that I will be ill (well sicker than normal) and she will end up changing her role from great friend to carer. Something I hate because it will mean yet again chronic illness has taken over a day that should be fun.

On the 31st October I am heading to Plymouth for my caffeine infusion and meeting up with my oldest friend Sharon, who I have known since we were 8 years old. I can’t thank her enough for spending her free time with me in hospital (a place we both loathe) so that we can catch up. I am looking forward to seeing her as I haven’t seen her for about ten weeks. We have gone years and years before without seeing each other but now because I have seen her on a more regular basis, the hospital visits just aren’t the same when she isn’t there. I also have to thank her for being my photographer when I had my occipital nerve block injections back in September. She admitted to me afterwards that she had almost passed out when she saw how far the needle went in!

Maybe I have my husband to thank for me not feeling particularly birthday -ish after all the muppet did book me in for my first dentist appointment in 3 years on my birthday…..Even the receptionist questioned his wisdom at the time of him doing this. I have been quietly seething ever since as I have a rabid fear of dentists and I know my teeth are quite a mess thanks to EDS.

Most people (other than Dentists) have no clue about the damage EDS can do to the teeth. I am rapidly losing the enamel from my two front teeth, so the base of them is very thin and over the last three years bits of them have chipped off. Also the braces I wore as a teenager have left pits in my teeth, I was lucky enough to wear the old train track style braces top and bottom. Where the brace was cemented to my teeth it has left holes that rapidly became gaping pits in 2013. My dentist at the time filled the holes and they looked great for a while but now, due to copious amounts of coffee (due to my CSF leak, caffeine increases cerebrospinal fluid production) and fags (I seem to smoke more when I am in a lot of pain, when I can get out of bed to smoke at the back door) these “fillings” have become stained and I hate looking at my teeth.

I know I am going to be in for a painful session and will probably have to return quite quickly for more work. I think I need a couple of fillings too which will be an adventure as due to dysautonomia I need adrenaline free injections or I will pass out on standing. I also need a Dentist who works quickly as due to EDS affecting the local anesthetic. I tend to burn through the stuff very quickly and due to my extremely low pain threshold when it comes to my mouth (everywhere else it’s high). So as you can probably imagine I am less than thrilled with the prospect of attending one of my least favourite places on my birthday!

In my husbands defence I did say to him accept the first appointment they have………...maybe I should have given it a bit more thought.

The 1st of November would have been used by me as a crash day. Not that I book them in but I know from experience the day after the trip to Plymouth for a caffeine infusion I am fit for nothing and can barely move. The trip to Tavistock for my neurology appointment landed me in bed from the minute we returned until the Friday (28th October). I was just so tired that I couldn’t function, I kept dropping things, my eyesight was blurred and I had double vision. The pain in my legs and spine from sitting for so long meant all I could do was lie down as my normal painkillers weren’t touching it. I am expecting all of this on Tuesday along with a trip to the dentist. Wednesday 2nd November hubby's birthday is going to be a total washout. It’s just as well hubby is on holiday that week, as my capabilities are going to be severely restricted.

I don’t know if my lack of birthday excitement is also being caused by the fact for the first time in years we won’t be holding a party. Since our 40th birthday we have held two parties a year. This year however we have had to cancel them all, as I am just no longer well enough to have 15 plus people in the house and all the stress that goes along with putting on a do. I hate what this CSF Leak has done to me. It has robbed me of everything this year and what hurts more are those who fail to understand how bloody restrictive it is.

My life is ruled by my head pain, yes on a good day if I am lucky I can have a few hours sat up. So when friends come over I deliberately lie down for hours before they arrive so that I can be sat up for their visits. However even those have become few and far between. I know that people have their own lives to lead and I don’t begrudge them of that, it’s just this year has become particularly isolating. I have to thank Imogen and Sharon for always being there for me. Imogen for coming every two weeks to see me and looking after our mutts on a moment’s notice and Sharon for accompanying me on my frequent trips to hospital. Without you both I really would have been lost this year. If anything this year has taught me a lot about friendship. There have been others too who have kept in regular contact via text and for that I am grateful.

This birthday is the 9th birthday I have faced being chronically sick. In a normal world I would have gone out for dinner and had a few drinks with my closest friends. Yet again the day will probably be spent lying on the sofa / bed wishing I was anywhere but here. Birthdays were always so exciting when growing up, now they mark another year of failing. Failing to celebrate like the rest of the human race and doing it the chronic illness way. Grabbing what pleasure you can while you can. In an ideal world I would love to go pony trekking for the day or go on a day trip or see a film or play. Those days are long gone and a birthday is now a bitter reminder of stuff I can no longer do.

I should be grateful many people aren’t lucky enough to see their 43rd birthday and I am grateful for the things I have. I just wish that it would be vastly different.

Too much of this year has been spent lying in a darkened room due to head pain, which in turn has left me with far much time to think. I am even anxious about Christmas and whether I will be well enough to supervise the cooking of our Christmas dinner. I have never worried like I am worrying at the moment as I have no control over when a bad day will happen and there are no takeaways open on Christmas day should I find myself incapacitated.

I wish I could get myself into the swing of things and look forward to this birthday but I just can’t. I haven’t felt this un-birthday-ish ever. Hopefully next week I will be able to tell you that I had a fab day….even if some of it was spent in the dentist's chair!