GI Follow Up


Last night was my follow up appointment with Dr Levi, it’s been playing on my mind a lot lately as I’d had lots of test in such a short space of time.

Andrew picked me up at 6pm and we got to the clinic just in time for the scheduled 6;20pm appointment. The nurse called me through and took my weight, there was a little confusion as my weight was different by a kilo, however I was in cold weather clothes so this may have changed the measurement.

I went in to see Dr Levi and he immediately asked how things had been recently, I explained about my general health and the shoulder. This obviously required additional pain relief which impacts on my gastric system so I explained that. He asked how I was doing with my eating, I said I was trying my best but I didn’t feel it was improving. I was still only managing one solid meal a day and was then only eating the meat part of that! Some days I wasn’t even getting to have my Complan just because I felt so poorly.

We then went through the blood results everything had come back ok, my vitamin levels were borderline but they were satisfactory. Everything else was good, no indications of what was wrong. On the CT scan there was a thickening in the lower bowel region, which is why the MRI had been ordered. Again this showed up on the MRI but it has been unable to be diagnosed at this stage as anything.

Unfortunately Dr Levi needs one final stool sample for a particular enzyme in my pancreas. If this is missing then it can be fixed very simply, if it isn’t this then I don’t know what! He also needs to do a Flexible Sigmoidoscopy to actually see what’s going on inside, I am dreading this. It’s intended on having this done before we go away so I at least I have a diagnosis or decision on my health.

It was then Dr Levi realized that we had originally met at The Spire Hospital, therefore the weight change was probably just down to the different scales in different clinics. It was only a kilo and like I say I was in jeans, boots and a cardigan as I was so cold, so that was probably the weight gain 🙂

So in the meantime I’m really no further forward today, hopefully in the next ten days this will all be sorted.

Occupational Therapy

Out of the blue yesterday I received a call asking if I was feeling up to an occupational therapy assessment.  I explained about my shoulder injury and the assessor suggested we leave it until today, I’m not sure what difference 24 hours would make with this shoulder.  I was pretty impressed anyway as I had been led to believe that the waiting list was months and I have been waiting only a few weeks for this appointment, in my mind I was only expecting a meeting in which we would discuss how to make my life simpler, so he could write a report for my housing association.

The appointment was planned for 10am and as Andrew has a hectic week ahead I knew this wasn’t really an issue, after another rubbish nights sleep, I woke up a little later than normal, so was trying to get myself organised when the phone rang, the poor OT was lost, so I needed to guide him to our house.  A few directions later and Andrew was opening the door to Joseph, he was a lovely amiable man, we sat down as Andrew went to work and started talking firstly about my diagnosis’  I was so impressed that he knew all about Ehlers-Danlos and PoTs, I explained about my two seizures earlier in the year as well, it was a time to be completely honest about what has happened so far.  He had a form we needed to work through, first off bathing/showering.  I explained that we had purchased a shower stool as the yanking me in and out of the bath was hurting my upper arms and I was completely unable to stand in the shower.  He was concerned that the grab rails hadn’t been fitted and assured me the work would be done within the week.  He then suggested a bath lift, I think the face I must have pulled said it all, he then showed me a video on his iPad, he explained this was to help me.  He said I needed to think about my shoulder injury and that moving forward I would no longer be able to get into and out of a bath. He wanted me to have this item, and put one on order 😦  Who was I to argue, this is to help, to provide me independence and to actually stop me from getting hurt any more.  He asked about my hips, and I explained that initially that is what I could sublax most, he wanted to know about me getting into and out of the shower, I said I needed help, so he said I would need a step, second item on the order.

Next question related to the amount of toilets we have in our house, now for some stupid reason they didn’t bother to instal a downstairs loo. Don’t ask me why, maybe by the time they got to our house the builder ran out of money.  So he asked how I cope with getting upstairs during the day, I crawl on all fours going up and then bottom shuffle on the way down.  When he saw this he was less than impressed, he suggested a commode, I drew the line at this, oh no, I was not having one of these, I would drag myself up the stairs if need be, but I would not have a commode.  Sorry that is one indignity I can do without.  However due to my weakness in my arms and legs I have agreed to a frame for the upstairs loo.  Compromise is the name of the game.

For the bedroom they are going to provide me with a grab rail to help with with getting into and out of bed, this will be really useful, I do struggle especially when I feel faint.  For my medication they have a really fab dispenser with an alarm that sounds, it holds a weeks tablets, this means that Andrew no longer needs to get back to remind me, he can focus on his business again rather than worrying about me.  For the kitchen they are going to get me a perching stool so I can sit with Andrew while he preps dinner, I don’t cook anymore as it isn’t safe, but it would be nice to be out there while he is.

There are so many adaptations and changes they are happy to consider to make my life easier, he explained that at 46 I should be enjoying life and having a wheelchair is a fantastic start, but getting more tools to enjoy life would help.

The biggest thing for my safety is he wants me to have a personal alarm pendant, this will go round my neck and should I fall, faint or have a seizure it will send a signal to a call centre and an ambulance will be sent for.  He thought I would be most resistant about this, but this one I was actually most interested in, Andrew had been told about it at his carers meeting in June but we haven’t looked into it since.

Finally he is going to refer me to physiotherapy for my shoulder, which I think is brilliant, I know I need it I can’t get the correct type of exercises and whatever I do it just hurts.  So it is just a case of waiting until they have a space for me to get my sore bits fixed 🙂

So today started really well to be honest, Joseph was lovely, he has left me his contact details in case we have forgotten anything.  We discussed so much that I am not sure there is anything we could have forgotten, but he has given me such a boost.  Now I better go and get ready as I have my GI appointment to prepare for!

Off to the Doctors

So I gave in and rang the doctors this morning, amazingly after a bank holiday there was an appointment spare so off I popped to see the doctor.

I’d woken up and my head was absolutely thumping, I put it down to the Midodrine increase, so decided immediately that it would be sensible to drop straight back down to only two a day. I need to slowly titrate up to the three a day, not suddenly go up, my poor head obviously can’t cope. So I didn’t take the first dose this morning and would discuss this with Dr Mason.

Andrew took me along at lunchtime, the weather was awful and the surgery was packed, thankfully there were two doctors on, so the wait wasn’t too long. Dr Mason thankfully organised to see me in a downstairs consulting room as I can no longer make it upstairs.

Anyway, first I discussed with him my shoulder which I seem to have sublaxed on Saturday night when I was getting into bed. The pain is deteriorating and the range of movement is getting worse too. He examined me and got me to show my range of movement on my good side, well my Hypermobility is quite marked and if I do say so myself a bit freakish, so when I was unable to even partially reach up my back with my bad left arm it was obvious I had hurt myself. He has now prescribed me some anti-inflammatory tablets to bring down the swelling inside the joint and advised on some exercises to keep it mobile.

We talked about pain relief as I don’t want to over medicate, and albeit he understood my concerns, he said in the short term I obviously needed to take some additional medication. So I have agreed for the next 48 hours to increase my painkillers.

I updated him with the news regarding Dr Levi, as I haven’t seen Dr Mason for a few weeks, and he was pleased that I had managed to “fast track” the NHS. I explained that Thursday was the day of reckoning and we would be getting the results of the CT scan, MRI and blood tests so he wanted to be kept up to speed.

Finally he explained that Dr Lim’s letter wanted me prescribed for compression garments, however I need measuring and there were no nurses available. I said that wasn’t an issue as I was starting the Midodrine initially so it would wait a week or two, he was happy with that.

The plan is for me to now monitor my own HR and BP as I take and increase my new medications and see how I am getting on, as I explained to him there is no point keep taking lots of tablets if they aren’t helping me. Fingers crossed all these tablets start working their magic.

Long Weekend

I had forgotten it was a Bank Holiday today as I was hoping to go and see the GP this morning.  I’ve been really struggling the last few days and I’m not sure what to do with myself.  I don’t feel it’s worthy of an A&E visit however I get the feeling I will get to Tuesday and by the time I sit in the surgery the doctor will be furious with me.

The countdown to Thursday really is on for the Gastrointestinal consultant, but I am finding that no matter what I put in my mouth to eat, be it solid food or a Complan shake, I am in agony as soon as it is working its way through my digestive system.  When I wake in the morning I have noticed that the acid reflux is back so I have a very sore throat again. I was originally combatting it by over propping myself in the bed with pillows but this was causing me neck problems.  I went back to using my tempur neck pillow and my sore neck has gone, but the acid reflux is back.  Solve one problem but get one back.  Then there is the lower abdominal problems which without giving away too much information are nightmarish.  So I have found if I don’t eat I don’t hurt simples, and guess what it works, so I do need to tell the doctor this, but I know he is going to be angry.

Then there are my bones, over the weekend as I went to bed on Saturday night I partially sublaxed my left shoulder, the crack as it went back in was so loud, and the pain was unbearable.  It has left me with pins and needles if I keep my arm still for too long, for example when I wake up I cant move or feel my arm or hand, I think I may have trapped a nerve somewhere, so this isn’t good news.  As I am overcompensating now with my right shoulder this is getting sore too, so I now ache in both upper arms.  I am so used to having dodgy hips thats ok but for my shoulders to be playing up so much that’s not so good and as I have mentioned before I am not willing to up my medication.  I am on a lot of strong drugs, to keep taking more and more, this will make my headache worse and I want it to get better not worse, I want to the medical profession to see that to take me seriously.  I’m trying to use this positive attitude that Dr Lim mentioned it’s really hard to find all the time but if I go in and explain what my intentions are then maybe they will be more inclined to do as I ask when I request referrals to London specialists etc.

I also realised from Dr Lim’s letter that I am not taking enough Midodrine I shouldn’t be taking one 2.5mg tablet a day (as per Dr Mayadunne’s letter), I should be taking 3 x 2.5mg tablets a day titrating up to 3 x 7.5mgs a day.  No wonder they aren’t really doing anything at the moment even the crawly skin side effects stopped yesterday, so its incredible how the body quickly learns to tolerate the drugs.  I will need to get my repeat prescriptions rectified to ensure the Midodrine is added because it would seem that the headache isn’t exacerbated by this medicine.

So my plan is to call the GP on Tuesday morning after the long weekend and arrange an emergency appointment as I really need to go and see them about these few bits and pieces.  In the meantime I’m just going to take it easy and try not to break anything 😦


**** Ice Bucket Challenge Update ****

My darling husband was nominated to do this obviously for ALS/MND, however I was super proud that he also asked that everyone raise awareness for EDS as it is something we are going through.  He requested that people take time out and go and look it up. Here is the link to his video I’m super proud of him for suggesting ALS or EDS 🙂 so thank you hunny xx

It is possible to donate to Ehlers-Danlos Support UK and all the fabulous work they do to support sufferers in the UK.  You can follow this link


Ouchies and Updates

The Ehlers-Danlos syndrome for me has always incorporated a certain amount of joint pain. This has always been in my hips, I had always put this down to carrying a very large baby at a young age but now I know the real reason it the EDS and the lack of collagen in my bones.

This week it hasn’t just been my hips though, it’s been really bad in my lower back, predominantly in my sacro-iliac joint.

No matter whether I sit, stand or lay down it’s just really painful. I am using the fentanyl patches and four co-codamol tablets a day for pain relief, I really don’t want to take anything more than this, because if I do it will worsen my head pain, causing rebound headaches. It’s a lose lose situation. So I have to learn to suffer with the 9/10 joint pain and enjoy the 5/10 tolerable head pain, or start messing around with my medication and I’m sure I will change those numbers around.

I received the follow up letter from Dr Lim and the only option I now need to consider, which might help this joint pain is some private physiotherapy treatment at St Johns and St Elizabeth’s Hospital, this is a specialist private clinic that concentrates on Ehlers-Danlos/Hyper-mobility. Now the hope is they may be able to strengthen up my pelvis and give me more mobility, so next week I plan to get in touch with their senior therapist and discuss what they can offer me.

Reading Dr Lim’s letter always makes me reflect on how an appointment has gone, and what kind of impression I have made to the Dr. When he starts with it was a pleasure to meet with me, I know it was a good meeting. Everything he had written down was positive and said I was doing the right thing, some days it doesn’t necessarily feel that way but medically if that’s what the good doctor says then so be it.

Reading the results from my TTT confirming the diagnosis. PoTs is diagnosed when the heart rate increases by 30 BPM’s. Mine jumped from 80 BPM to 120 BPM just in the passive phase this is when they just elevated the bed. When they put the GTN under my tongue it shot up to 170 BPM. I can vividly remember the test, it was horrific.

So apart from listing all my medication … Wow that’s one hell of a list. The letter detailed what I had got up to recently, our break away in France, losing my job and trying to pop into the shop once a week, just to get out of the house really. It sounds like I’ve been doing a lot but when you work out this is over the space of two months this is nothing really.

The only additional request at the bottom of his letter to my GP was for compression stockings, to assist in the raising of my blood pressure. So that’s me signed off from Dr Lim, he’s asked that I email with any news which I think is quite nice, I like Drs who do that (although last time I did send an email I did get an abrupt response 😦 )

I’ve been on the Midodrine for three days now, it hasn’t made the headache any worse that’s the good news, I think it’s helping my blood pressure I can’t hear the pulsing in my ears that I normally suffer with, so that’s got to be a positive. The downside the crawling scalp side effect is horrible, it’s like having a million creepy crawlies all over your body …. Makes me really itchy and it’s unbearable, but I need to tolerate it, I’ve read and heard it will get better, my body will accept these changes. I’ve also been having some strange palpitations but again it’s only day three, let’s not panic, I need to give my body time. I don’t do anything strenuous I take it easy every day, so I will just titrated up carefully. I believe this is the drug for me along with Ivabradine.


Finally I’ve received a follow up appointment from Dr Levi to discuss all my CT Scan, MRI, and blood tests. Hopefully there will be some kind of diagnosis and nothing too bad. It’s on Thursday evening so I don’t have too long to wait but I’m worried about it none the less, I’ve had a nosey at the MRI images they are really cool but everything looks normal, two kidneys, spine, bowel, but I’m not a radiographer lol. What I need is a specialist who can tell me what I am looking at… Anyone?

So that’s been the end of my week hopefully next week I’ll get some answers and we can move onwards and upwards.

Midodrine – Day One

After much debating and hand wringing I decided today was the day I would give the new medication a go.


So what is Midodrine?

Midodrine is a drug that can be used to treat people with disorders of the autonomic nervous system which include low blood pressure, neuro-cardiogenic syncope (fainting) and postural tachycardia syndrome. It is used only after other measures have been ineffective in controlling symptoms (eg high fluid intake, additional salt in some patients, counter manoeuvres, small frequent meals, gentle exercise, compression tights etc as appropriate).  It is a vasopressor/antihypotensive agent.

Midodrine hydrochloride is the generic (chemical) name but some manufacturers use their own brand names for the same drug which include Gutron, ProAmatine and Orvaten. It comes in 2.5, 5 and 10mg tablets.
How does midodrine work?

Midodrine is an α1 adrenergic agonist drug, meaning that it stimulates receptors that noradrenaline normally works on. After swallowing, it is quickly converted into another chemical that causes blood vessels to narrow, thereby increasing blood pressure. Indirectly, it can also reduce heart rate.

It reaches peak concentration in the blood about an hour after swallowing a tablet, but the effect is brief, with levels falling to half about 2-3 hours later.

The brain has a protective mechanism that stops some drugs from entering and very little midodrine crosses this blood-brain barrier. It is removed from the body by the kidneys.
How do I take midodrine?

As its effect is short lived, midodrine needs to be taken frequently throughout the day. It works best if the first dose is taken an hour or so before getting out of bed, then at 3 to 4 hourly intervals throughout the day, the last dose being taken at least 4 hours before going to bed. If necessary, the amount may be gradually increased to a maximum total dose of 30mg per day.


For me personally I am taking 2.5mg once a day at the moment, next week it will be upped to 2.5mgs twice a day as long as I have no nasty side effects!!


What are the risks of taking midodrine?

The main risk of taking midodrine is ‘supine hypertension’. This is excessively high blood pressure on lying down. One advantage of midodrine is that it only works for a short time. Not taking it within 4 hours of going to bed reduces the risk of supine hypertension.

Symptoms of supine hypertension may include palpitations (awareness of the heart beat), pounding in the ears, unexpected headache or blurred vision, although it can occur with no symptoms. If you develop these symptoms, you should stop midodrine and inform the prescribing doctor. In addition, midodrine should not be continued if it causes high or unstable daytime blood pressure.
Who should not take midodrine?

Midodrine should not be prescribed in patients with the following conditions: severe heart disease, hypertension, peripheral vascular disease (narrowing of the arteries in the legs), enlarged prostate gland causing difficulty passing urine, urinary retention (when the bladder can’t empty properly), phaeochromocytoma (adrenaline producing tumour), overactive thyroid, narrow-angle glaucoma, allergy to any component of the product. It should be used with caution in kidney disease, diabetes and cor pulmonale (large right ventricle due to severe lung disease).
What are the side effects of midodrine?

Common – tingling and itching of skin-especially scalp. This may improve with time. Goose bumps. Feeling cold
Less common – supine hypertension, urinary retention (inability to pass urine), slow or fast heart rate, palpitations, irregular heart rhythm.
Rare –nausea, indigestion, headache, agitation
Interactions with other medicines

Midodrine should be used with caution in combination with the following drugs:

Digoxin, beta blockers (eg bisoprolol, atenolol, propranolol), steroids (prednisolone, fludrocortisone), alpha adrenergic receptors stimulators (phenylephrine, methoxamine), tricyclic antidepressants, antihistamines, thyroid hormones, MAO inhibitors, dihydroergotamine) rauwolfia alkaloid medicines (reserpine), atropine, some decongestants (including over the counter preparations) and appetite suppressants. Midodrine should not be given to people taking alpha blockers (phentolamine, prazosin).

What does ‘unlicensed’ mean?

Midodrine does not have marketing authorisation in the UK for the treatment of postural hypotension or for any other indication, therefore it is an unlicensed medicine in the UK. In the USA, midodrine is licensed by the Food and Drug Administration (FDA) for the treatment of symptomatic postural hypotension. It is also licensed for use in several EU countries. An application for licensing has been submitted in the UK.
How do I obtain midodrine?

As midodrine is unlicensed in the UK, it can only be prescribed by a doctor with experience in using this drug. This is usually a hospital consultant and the midodrine is dispensed by the hospital pharmacy. Sometimes hospital consultants will issue an FP10, which is a green prescription that can be dispensed by your local community pharmacy.

GPs are often advised by their pharmacists to decline to prescribe midodrine unless they are experienced in its use. However, if the consultant provides a ‘Shared Care Agreement’ (a document which provides advice to the GP about how to prescribe, side effects, monitoring and how to contact the hospital team if there are problems), then the GP may be willing to issue a prescription.

Now I am desperately hoping this miracle drug along with the Ivabradine helps my PoTs symptoms considerably, because in the last ten months I haven’t seen enough of an improvement with just the Ivabradine.  So after just one dose and with a tingly scalp I know something is working, lets just hope this is the start of finding the pieces of the jigsaw that we have been looking for.