Exhausted.com

Exhausted is an overused word however it is the only way I can describe the state I find myself in. Yesterday (Tuesday) was my trip down to Plymouth to see my new consultant, (after I got rid of the one who accused me of spending too much time on the Internet looking up syndromes to have) and catch up with family and an old school friend. 

The appointment went really well however due to two road traffic accidents in Plymouth which caused complete gridlock in the city, the rest of my plans went awry. The traffic was so bad it actually took us an hour to get out of the multi storey car park at the hospital. With my husbands blood pressure slowly climbing and me rapidly fading, I had visions of the red cross coming to our aid as I couldn’t see how on earth we were ever going to get home. It took us over two hours to get back to my parents house, a journey normally of 20 minutes to pick up my medication and various other bits and bobs that I had left there before setting off for my appointment. We then had another hours travelling time to complete the journey from Plymouth to Exmouth. All in all yesterday I spent over 4 hours in the car. This catastrophe was not in our plans and being in the car so long has caused my legs to refuse to work properly and climbing stairs is now an endurance sport which has to be completed by shuffling up the stairs on my bum. What a glamorous life I lead!

So my blog post this week will not be a long one, when I say that it just depends how much I can write before crashing completely. I have got up very early to compose this post because the worry of not having it completed was keeping me awake. I do like to have something to worry about.

As usual I suffered very badly with pre hospital visit jitters which meant a pretty much sleepless night Monday into Tuesday. I woke up at 02:30am and didn’t get back to bed until gone 9pm, I have woken up at 4am this morning. This is just begging for a Meniere’s attack to happen, which I can already feel building up. I had been told by numerous people that the doctor I was going to meet was lovely but in the back of my mind there was a niggling doubt that I could be the one patient that he wasn’t nice to. You see my neurologist I saw many years ago was treated like a god amongst the Myasthenia Gravis patients, not a bad word was said about him. The problem was that he was a consummate performer who was very nice to your face but had a nasty habit of writing utter shite about you in his notes. The neurologist treated me very badly and I highly suspect that it was him that diagnosed me with somatiform disorder. So I guess you could say we (both hubby and I) have trust issues when it comes to doctors.

When the doctor called my name in the waiting room, I had my back to him due to the lack of space provided for wheelchair users (that is a rant for another day that hospitals seem to be so unfriendly towards wheelchairs). When I turned around to face him, there was a look of shock on his face. He then said something like “you need no introduction, I can see the family resemblance”. This Dr treats my sister also and due to that has also met my mum. People say that we all really look-alike but I just don’t see it. Yes we look like each other but it’s not (to me) like we are clones! So that was nice as he hadn’t seen my sister for a few weeks but he recognised me. My last consultant also saw my sister but never actually acknowledged the link between us despite me referencing her on more than one occasion simply because we have a lot of the same symptoms.

The new doctor was really friendly and open to ideas. In fact he brought up stuff that I had on my list of the most important things to discuss with him. My list read as follows

1. Mast cell activation disorder

2. Fluid intake

3. Stomach / bowel problems / adhesion’s

4. Alice in Wonderland syndrome or Todd’s syndrome as it is also known. This would be brought up if I was feeling brave enough as I have never mentioned it to any doctor before in case they thought I was nuts.

As it happened one of the first things we talked about was the Alice in wonderland syndrome. This was due to the fact he brought up the Myasthenia Gravis / Ptosis / Nystagmus and breathing issues. He doesn’t think I have MG, he thinks my Ptosis (droopy eyelid –You can see a picture of it here) is being caused by hypoperfusion (so not enough blood flow to my brain, essentially low blood pressure in my head). I brought up the fact that when I have Ptosis or MG symptoms my blood pressure is normal but he quite rightly pointed out that it doesn’t mean my blood pressure is normal locally (as in my head the pressure is low). I can’t argue it makes sense, mestinon or pyridostigmine bromide as it is also known raises blood pressure, if the blood pressure is low in my head the mestinon would have the effect of raising it. I did mention about the ice pack test also being positive and that is an indication of a problem at the neuromuscular junction. He said he felt it was possible that both my sister and I had issues with the ganglionic receptors in our brains. As I told him I roughly understand that but much more and it goes over my head.

As we were talking about MG symptoms I brought up the nightmare of 2013, when I had all the constant nystagmus and the weird visual symptoms that lead me to believe I have Alice in Wonderland syndrome. He agreed with me that it sounded like I did indeed have this, what shocked me more was the fact that he had heard of it and didn’t treat me as if I was a complete loon, when I was describing watching birds flying across the sky only for them to rewind and do it again.

He also believed I could be suffering from another weird syndrome that causes sudden desaturation (not enough oxygen in your blood). He did tell me what it was but its name was far too long for me to remember. He told me at the start of the appointment that he had only had a quick look at my notes however it soon became clear that the poor man had taken an in-depth look at them and had already come up with a treatment plan.

Due to my exhaustion I can’t remember the exact flow of the conversation and what we talked about next and in what order. So I will be brief and explain what I can remember.

My excessive fluid intake would come under psychogenic polydipsia, as I do not have diabetes insipidus. He thinks that title is harsh because I have had excessive thirst ever since I was a baby and I could in no way be able to form a habit of drinking too much from that age. So there is something wrong with the hypothalamus part of my brain that controls thirst but we will probably never know what.

He believes I have Mast Cell Activation disorder just from reading my notes and looking at the strange reactions I have had with many if them. The closest I have come to full-blown anaphylaxis was when I had CT contrast dye injected. It’s an experience I don’t want to repeat. I was also able to show him the blotches that had come out on my neck that very morning, despite not eating / drinking or doing anything different. Due to the Mast Cell Activation Disorder or MCAD for short he wants to change my Betahistine that I take for Meniere’s disease to Cinnarizine (stugeron). As MACD is caused by histamine the Betahistine will not be helping me and by strange coincidence since taking it I have noticed that my skin has become much more reactive. He isn’t convinced that I have Meniere’s disease, I am, so he is requesting a copy of my audiology notes as they hadn’t been included in the file that was sent to him.

He thinks a lot of my stomach / bowel / adhesion issues are being triggered by MCAD. If we could get the MCAD under control it maybe that we can get those issues under control. The adhesion pain has been quite bad since the 29th November. I am having sudden onset of really sharp pains which take my breath away and leave me doubled over. As fast as they come on they go again.

The weirdest revelation of all was that he doesn’t think I have PoTs……….. He believes that my problems stem from my blood pressure being all over the place, my heart (or brain you could say) is responding by beating faster in an attempt to keep my blood pressure elevated. He wants to do another tilt table test, I have politely declined for the moment just due to the fact how ill I was after the last one.

He was incredulous that I had never had an echo cardiogram ( a bit like an ultrasound for your heart) to check that it was actually working ok and that I don’t have  a mitral valve prolapse. This should be a standard test that is carried out of everyone who has been diagnosed with EDS. For some reason my last consultant didn’t think so and I can’t seem to get my gp to move forward with this probably because I have so many problems it just gets overlooked. So my new doctor is going to ask that I get one done. If I don’t get it he will arrange for it to happen in Plymouth.

So due to his theory on it being my blood pressure causing the tachycardia he has started me back on Midodrine at 2.5mg once a day for a few days to then increase it to twice a day, then three times a day. Then I am to slowly get myself up to 5mg three times a day. We all hope that by slowly titrating it I won’t react as badly to it as I did the last time when I ended up with awful bladder problems and adhesion pain. 

When we talked about the bladder problems, I brought up the possibility of Fowler’s Syndrome. When I brought this up with my last consultant this was when he accused me of looking things up on the Internet, so I could have more syndromes. This doctor knowing my sisters history, that she has a sacral nerve stimulator so that she can use her bladder, agreed with me. He told me because not many doctors had heard of Fowler’s Syndrome this was perhaps why I had encountered such hostility. The Fowler’s syndrome would cause the acute urinary retention and the problems I have had when in hospital or at home when they have fitted a catheter and my bladder has still refused to empty due to the fact the valve at the neck of the bladder had clamped around the catheter. Again he couldn’t understand why I had been taught to self catheterise and hadn’t had the bladder issues fully investigated. I explained to him that when I raise this issue with my doctors I get told I have already had lots of tests done. It is infuriating because the doctors seem to blame me for the wrong tests being done, so now nothing gets investigated and it is only the symptoms that get treated.

I have just looked back at the amount of stuff I have written after claiming I wouldn’t write much. I don’t know how I am putting this together as I am so tired I can barely string a sentence together let alone see the screen.

Anyway back to the Midodrine, once I can tolerate this medication at the full dose of 5mg three times a day, the doctor wants me to then slowly add in Ivabradine. I have tried this drug before and it was wonderful at stopping the tachycardia but my blood pressure dropped very low. This is what my new doctor has picked up on, where as the old consultant was happy to just say “you obviously need a fast heart rate to keep your blood pressure elevated”. This doctor wants to get to the bottom of this and raise my blood pressure and then drop my heart rate. It was actually something (on my extended list) that I wanted to suggest. I have to say I was  in shock when it appeared both the doctor and I were on the same page. That has never happened.

I can’t really remember much more from the appointment and I will be honest I am starting to fade a bit here. He wants to see my print outs from my tilt table tests to see what my blood pressure and heart rate did. My old consultant handily forgot to include them in my notes. My new consultant ordered a load of blood tests, one for my testosterone levels (due to my facial hair), a repeat of the prolactin to see what that is doing and countless others, I was robbed of 4 vials worth of my blood. 

The assistant that took my blood wasn’t great, she had a constant sniff (that always makes me feel sick and is a pet hate of mine), she managed to jab the first attempt straight into a nerve and then had the gall to shout at me when I moved. She tried to tell me she hadn’t hurt me, I asked her “why would I shout out and jump if you hadn’t hurt me?” I was furious. I have never moved during a blood test, I have had 100’s of them, I have had 3 doctors jabbing me in the feet and both arms at once to get blood. So I don’t jump about when people are trying to get blood from me. My point was proved when she then got a vein on the back of my hand and I continued chatting with her whilst she got my blood. It didn’t help that I was tired and dehydrated when she was getting my blood but to try to tell me she hadn’t done anything to make me jump was ludicrous. My husband was getting quite tense at this point as he has seen me having blood taken on many occasions and has never seen me react like that.

Despite the horrible blood taking episode I would describe myself as slightly elated after that appointment. This doctor wants regular email updates on how I am doing……I know what doctor does that? My last doctor gave you ten minutes of his time and you didn’t see him again for 6 months if you were lucky and they remembered to book you an appointment. I had this doctor for 80 minutes.

My husband informed me last night as we were going to bed, I didn’t let the poor man finish a sentence without interrupting him. He said I was gabbling away like a loon, like I always do when I am nervous. Hubby is happy, he said he feels for the first time in a very long time that we are starting to get somewhere. As I told the doctor yesterday, I know that there is no magic wand, I know that there is no cure, I would just like to feel better and be taken seriously.

 

I’ve been gone a while

I am sorry that again I’ve been gone a while, well actually over a year. Sometimes I couldn’t face typing out what’s been happening in my life as the act of typing it out would some how make it more real.

 Other times the blog has left me feeling exposed. On facebook on the whole I can choose who I share the information of my life with and delete and block those who are unsupportive, fake or just rude. With my blog my soul is bare to the world.

I’ve decided I need to continue with my blog and now have it set up on my tablet so I can update it whenever and where ever I like.

My story and others like mine need to be heard even if it is just by a select few, maybe no one? At least if I co tinue writing I can’t be accused of not putting the information out there.

In the last year or so my health has declined rapidly. On February 7th 2014 I had another tilt table test but this time with glucose loading and its sent my health into a nose dive. This tilt table test revealed more than Pots ( postural orthostatic tachycardia syndrome) it also revealed Orthostatic Intolerance ( my blood pressure drops on standing), an arrhythmia ( my heart never stays in a rhythm but likes to disco dance its way through the day), post prandial hypotension ( my blood pressure drops after I eat and I black out) and at the end of the of the test my consultant told me I have severe autonomic dysfunction – that was never said during the 2011 tilt.

I have been booked in for a drug trial in April this year. The drug is called ocetreocide and its the last chance saloon for me. The ocetreocide will be injected three times a day before I eat in the hope that it stops / reduces spleenic blood pooling ( too much blood being diverted to my belly after eating). It can have some nasty side effects but if tolerated by me it could make a difference. On the second day of testing  if I’ve tolerated it then I will be taught how to inject the drug myself.

I’ve tried all the usual drugs Fludrcortisone ( triggered migraines, Ivabradine dropped my blood pressure too low and midodrine stopped me being able to urinate properly and also gave me really awful stomach pains. I’m still on my salt tablets and they’ve been increased to the maximum dose after my nose dive this month. Eight of those beauties a day!

I’m also back on the fludrcortisone so far so good. Its made a small improvement as I can now get out of bed for a few hours every day. For over a week I was bed bound and hubby had to take time off to look after me. So its been a definite improvement. The side effects so far have been fat ankles, fat hands and puffy eye lids but I can put up with them if I’m not stuck in bed all day!

Last year I had a few investigations performed as my eye sight was awful continual ptosis ( right eye shut constantly) nystagmus ( rapid uncontrolled eye movements) it was a great deal of fun. I had to be seen by a neuro opthalmologist one of the rudest doctors I’ve ever met. Who treated me like all I was after was a myasthenia gravis diagnosis. To be honest I’ve gone past caring if they diagnose it or not. I’m sick of the whole MG thing and the neurology department!

I also had investigations done for my bowel adhesions as my stomach pain increased and made eating impossible as it was so painful. I ended up on liquids only and lost 2 1/2 stone in weight ( 35lbs). It was needed as I wasn’t svelte and I’ve pretty much managed to keep the weight off. Getting down to a UK size 16 for the first time in years! See every cloud and all that!

So that’s a quick summary of where I am at the moment although I forgot to add I’m also having breathing problems so I’m using my oxygen concentrator most days.

Again sorry that I abandoned this blog.

Update after Consultant Visit

I visited my POTS consultant this week. Unfortunately I got myself in a complete state before I went which meant I was dry heaving, snappy as hell and a little uncommunicative! At least my husband / family and friends all know that this is how I act when I am nervous / stressed out and know that as soon as the visit is over I will be back to my normal charming self!

We were actually seen 20 minutes early (due to nerves we actually arrived at the hospital at 10.08am the appointment wasn’t until 11am!) so that was good as my consultant can run a little late – purely because he likes to spend time with his patients and address the concerns that they may have. As usual my husband and I were the youngest people in the waiting room by about 30-40 years. I am seen in the geriatric clinic! Although my consultant is geriatrics and general medicine. Its always quite amusing as the other patients cant work out why these young whipper snappers are attending their clinic!

I confessed that I was no longer taking midodrine – the side effects were just not worth it. I did have massive improvements on the drug – no more ptosis, mental functioning better able to be out of bed for longer. The down side was severe stomach pains and being unable to urinate. The pain from both of these side effects would land me back in bed…. so I admitted defeat. My consultant said it was a shame but that’s the way it goes sometimes. So I have now been started on Ivabradine a medication that is used for congestive heart failure and used off label to treat inappropriate sinus tachycardia and postural orthostatic tachycardia.( some drs are saying that POTS and IST are actually the same thing) Unfortunately one of the side effects can be lowered blood pressure, which as my regular readers will know a lowered bp is something I do not need. So I have started on 2.5mg twice a day.

Ive already noticed that my heart isn’t pounding out of my chest when standing. Ive checked my pulse and its still running fast over 100 bpm but not going above 120bpm. My pulse needs to reach over about 110bpm before I notice it. It sounds strange but I guess my body is so used to it running fast I no longer notice it. It does feel really strange to stand and not have my heart pounding! The downside is I am light headed, there has been no massive drop in blood pressure and with the heat we are experiencing at the moment its difficult to tell if its the heat or the medication causing this, time will tell. My consultant is keeping a close eye on me as he feels I may need my pulse to run that high to counteract the low blood pressure, so we need to proceed with caution.

My consultant checked my heart as I have been experiencing a lot of chest pain on exertion. He didn’t detect anything and those of you with POTS will know that chest pain is quite common. So I am not immediately concerned about it.

The good news is that my consultant is trying to start an EDS clinic for the southwest as most EDS patients find they will see a rhuematologist to get diagnosed and then be discharged from their care within 12 months. Basically EDS patients are falling through the cracks with no service looking after them. My consultant is trying to team up with a geneticist who is keen to identify the gene behind EDS 3 or HEDS as it is known.

That would be fantastic if they could get this clinic started as my consultant told me there is no NHS physio therapist in the southwest trained in EDS patient care. Which means unfortunately most of us seeing a physio will come away with more problems than when we initially started with them. My physio claimed to understand EDS but had limited knowledge of POTS which is where the problems started and ended up with me packing physio in. This cant be right and we need better understanding across all fields of medicine.

Ive been resting up since my visit, I also managed to have another migraine on Monday. They are just wiping me out. So its been a pretty exhausting week.

Thanks for reading.