A very long awaited update – having major surgery soon.

I’ve been meaning to write this for quite some time, I’m really not very good at keeping up with this whole posting frequently thing anymore it seems – but was I ever?! To say a lot has happened since I was in hospital under my GI doctor in June is an understatement, and a very long story. From discharge on the nutritional drinks I continued to go downhill, loosing weight rapidly symptoms just worsening and not able to tolerate more than 800 calories of the drinks a day, and the amount I could tolerate of them was continually decreasing. Bed bound and needing a wheelchair for a combination of reasons in order to leave the house, which was generally only for doctors appointments. Constant abdominal pain, nausea and many other different unexplained pains and symptoms. By August I had lost 20kg since the beginning of April and was very underweight. However, in August I also found out what was wrong…

I have a couple of very rare vascular compression syndromes:
– Superior Mesenteric Artery Syndrome (SMAS), to simplify this is where the 3rd part of the duodenum (first part of the intestine) is compressed in-between the Superior Mesenteric Artery and the Abdominal Aorta. Which makes eating incredibly problematic as there is not much space for anything to get through your duodenum from your stomach.
– Nutcracker Syndrome – yes this is a real thing and yes this is actually its name. Nutcracker Syndrome is where your left renal vein is compressed in-between your SMA and Aorta. For me this has also caused Pelvic Congestion Syndrome due to the blood flow issues with having a compressed left renal vein. Both of these conditions cause me a significant amount of pain and problems too.

By September I just couldn’t keep going as I hadn’t been able to sustain myself nutritionally on the elemental drinks, I was continually losing weight and was down to only being able to do about 200-300 calories a day of them. I was admitted to hosptial under my GI again for him to try and stabilise me. I ended up having to have a nasojejunal tube feed put in, which actually ended up being a completely horrific experience of placing the tube. I ended up having to have it done in radiology rather than endoscopy which meant I was unable to have any sedation. It unfortunately was not a simple or quick procedure due to my compressed duodenum, it was incredibly painful and long, involving a lot of crying, my whole body shaking and passing out at the end. I was on the NJ tube for a week however I was not able to tolerate it, whilst feeding it caused me a significant increase of pain that was not bearable, and it was causing me a lot of tachycardia when on the feed too. I ended up having to be taken off and the only option was to put me on Total Parenteral Nutrition (TPN). TPN is IV nutrition which goes in through a central line (I have a PICC line), it completely bypasses the GI system, providing you with nutrition straight into your blood stream, the end of the catheter sits just outside the heart in the Superior Vena Cava. The fact that it means no nutrition is entering my GI system gives me relief from the increased amount of pain and symptoms I have when I have anything going in there.

We did some tests to confirm my diagnosis with another scan in order to send it to an experienced vascular surgeon. I met with the surgeon and really liked him, he was easy to talk to, understanding, I liked what he proposed and he had a sense of humour! He wanted me to have an angiogram and a venogram to assess things a bit further before discussing next steps. Not a pleasant test to have done, especially when you end up being able to feel the entire venogram happening inside of your abdomen – very painful and uncomfortable! Not to mention having to lie completely flat and still for 5 hours afterwards which is not Ehlers Danlos Syndrome friendly in the slightest, my unhappy and unstable joints were screaming at me in pain for the entire 5 hours begging me to move them.
The scan showed it all clearly and we went ahead with planning surgery to fix the SMAS and Nutcracker.

I’ve now been in hospital for the past 10 weeks on TPN waiting for surgery. Have had several complications including sepsis, and ongoing very abnormal haematology blood counts and liver enzymes through the roof. The last two being unrelated to the sepsis, and deemed incredibly odd to have happened for several different reasons. I had the lipids in the TPN reduced and switched to the old type as my liver enzymes at one stage went up to 37x the normal value. My haematology blood counts I ended up with thrombocytopenia and low WBC, RBC and Haemoglobin. Though platelets being the worst and were dropping each day, there is still no clear explanation for this, but it most likely is due to the TPN for some strange reason.

We have had to try and re-stabilise my PoTS prior to surgery to make sure I am safe enough for it with the anaesthetic and also post op. My PoTS has been getting progressively worse pretty much since I came off the medications that kept it stable, which was about a year and a half ago. Initially after coming off it wasn’t too hard to cope with, but things just started getting worse and worse to become in a really awful state. I’m back on two medications, which I was on previously, however they are not effective enough and I’ve got some very strange things going on with it all too, my PoTS professor did want me to get checked over by cardiology prior to surgery as well.
I have also had some incredibly weird things going on with my headache too, but don’t know what is going on with that and hopefully we can figure that out more once I have recovered from surgery for the compression syndromes.

Surgery is imminent, I am having two procedures in one operation. Vascular surgery to transpose my left renal vein and gastrojejunostomy for the SMAS to bypass the compressed section of the duodenum. Having surgery will hopefully allow me to eat again and relieve me of all the pain and symptoms that both these conditions cause. I have two very experienced surgeons and an incredibly supportive GI doctor and I feel completely comfortable with the plan. Just keeping my fingers crossed that it all goes smoothly and that I will be able to sustain myself nutritionally in order to go home, as I need to get off of TPN in order to go home from here. So fingers crossed I will be able to and will be home for Christmas!

It’s been a very long hospital stay, however I actually can’t believe it’s been 10 weeks. I’ve been incredibly lucky to not only be under some amazing doctors but to also have been looked after by the most incredible and lovely nurses I have ever met and I don’t think I will ever meet any as great as them all again! And despite pain and feeling really unwell, distraction in the form of incredible nurses to chat to, laugh and joke with has truly been the best medicine. I have managed to maintain being completely mentally stable, upbeat and positive – and I am very proud of myself for that.

Will write more about everything and my recent experiences once I’m able to after surgery.

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The downside to always searching for a effective treatment/reason.

If one day you got sick and you weren’t getting better, what would you do? If one day you got hit with severe constant headache that didn’t go, what would you do? You’d go to a doctor right because that’s not normal and there has to be something wrong with you and the doctor should be able to fix you, that’s their job, right?

But what if they can’t? What if they can’t find anything wrong with you, yet you still have all these horrible painful symptoms that won’t go away. So you go from doctor to doctor for help, trying various treatments, maybe even eventually getting some diagnosis, yet still you’re sick or in pain or both and the doctors can’t make you better, you are now chronically ill or now suffer from chronic pain. Yet you never give up because there has to be something actually wrong with you, an underlying cause, a reason why you are in pain. From doctor to doctor, treatment to treatment but nothing works and you are still sick and in pain and maybe even now depressed.

I have the diagnosis’ of New Daily Persistent Headache NDPH (my main issue), Postural Orthostatic Tachycardia Syndrome POTS and Ehlers Danlos Syndrome Type 3 EDS3. NDPH means I’ve had a constant severe headache 24/7 since the 14th January 2010, POTS means basically that my heart and blood pressure can’t cope with the force of gravity when I change positions, so my blood pools when I stand up my heart rate races and my blood pressure plummets to compensate and my brain then doesn’t get enough blood/oxygen and I faint and have weird seizure type things, EDS3 means my joints are hypermobile because my connective tissue is faulty leaving me prone to joint pain, partial dislocations, dislocations. My POTS is controlled by medication now so I no longer faint and have seizures multiple times a day, EDS3 I experience quite a bit of joint pain and partial dislocations (subluxations) but luckily I have never fully dislocated a joint and I’ve just started physio to see if that will help my pain and subluxations. My NDPH has been the thing that has affected me the most, as you can imagine having a severe often migraine level headache 24/7 can take it’s toll on a person, and if you read my blog regularly you will already know how badly it has affected me.

I was diagnosed with these three conditions in 2011. Since the beginning of my headache in 2010 I had been searching for answers like any normal person in pain would. But the doctors in Dubai were useless and I was getting no where. So I came back from Dubai for the summer of 2011 to go to London and see a bunch of doctors. I started with a cardiologist even though my main issue was my headache, my GP in Dubai noticed that my resting heart rate was constantly way too fast and my blood pressure way too low. The cardiologist in Dubai was pretty useless, told me I needed to stop exercising as my heart was working too hard and if it continued like that I would go into heart failure. Pretty extreme and not really true. To cut a long story short I went to England saw a cardiologist who didn’t say a lot and then sent me to neurologist for my headache. He was pretty useless also other than the fact that one of his team noticed I was hypermobile, she use to work for a professor of autonomic dysfunction and thought I may have POTS, so they sent me to the professor. After some tests I was diagnosed with POTS but he said my headache was completely unrelated and sent me to a headache specialist, where I was eventually diagnosed with NDPH. He also sent me to a rheumatologist because I was hypermobile and the rheum diagnosed me with EDS3.

Throughout this period of searching for answers I was very depressed and actively self harming. I was then put on meds for my POTS which worked and I became stable POTS wise. And my new neurologist put me on medication trials. Then for a period of 3 years I flew back from Dubai every three months to see my neuro, each medication trial lasted a minimum of 3 months, so every three months I would get reviewed to see if the medication was working, but every time they didn’t work so I got put on a new one and so on and so forth. I got no benefit from any medication, all I got were side effects, extreme fatigue and sleepiness, lost of weight gain, and on one I lost over half the hair on my head which wasn’t pleasant and then there’s the fact that I literally was like a walking zombie because of the meds for three years, they didn’t do me any good. I also tried more aggressive treatments, nerve blocks, botox, and infusions, none of which helped the pain in my head either. And I was just left feeling disappointed, miserable, depressed and exhausted after every failed treatment. Last November I was told there was nothing more my neuro could do for me, which yeah hit me hard and made me more depressed for quite a while as many of you know.

But it was probably for the best. All this searching for answers searching for an effective treatment was exhausting and defeating. When I finally gave up my search, it took a while and a lot of therapy for me to get to a point where I can now say that it was for the best. My neurologist saying that to me was obviously not the best thing ever because the best thing ever would be no pain but it was the best thing he could do for me. My search for an effective treatment wasn’t me living my life, it was me being drugged up on headache preventative meds that were causing me more harm than good, that meant I couldn’t try to live my life despite pain.

There is no answer as to why I always have a headache and as I’ve found for me there is no effective treatment either. So I’ve accepted the pain and I’m learning to live despite it, I spent 4 years searching for answers searching for a treatment that would work so much so that I wasn’t actually living.

Giving up the quest for relief may seem scary, but what if the relief just doesn’t exist, what if there are no meds that will make it better. Ask yourself are you living? Because I wasn’t. It’s normal to search for answers and try to see if it can be fixed, but I suppose you just need to know when enough is enough, when the search is doing you more harm than good, when you are not living a life anymore.

If you suffer from chronic pain you probably think I’m crazy for saying this, but it’s been the best thing for me to stop searching for relief and try to accept my pain and try to live my life despite it.

Physio.

Yesterday I went to London for my first physio appointment. It went well, the woman I saw was really nice, she asked me about all my medical history and what brought me there today so I spent a good while explaining that to her. Then we discussed my affected joints and the problems and pain I have been having with them. She then asked me to change into shorts so she could assess my joints and how I move. 

She said that I have really strong rectus abdominals, because well that’s kind of obvious as I actually have a six pack, but she said the muscle underneath my bottom abdominal that joins onto the pubic bone and extends all round the hips and lower back is very weak. She said that this needs to be stronger in order to support my hips and back and before she can start work on trying to prevent the pain in my hips and prevent them from constantly subluxing (partial dislocation). She looked at my knees another area I have problems with and noted that when I squat or bend down my knees don’t go down straight they bend inwards, which I never knew I did before she pointed it out. 

So she has given me a load of exercises to strengthen this muscle and I have to do them everyday and see how I get on and then make another appointment to go back up for another session in 2-3 weeks depending on how I progress with these exercises. 

My hips and knees were killing me with pain yesterday after I got back from London, all evening and night and I had real trouble getting to sleep even with the meds I take to help me sleep. Ended up having to take some paracetamol which seemed to ease the pain a bit so that I could get to sleep. Hopefully these physio sessions will help because joint pain on top of my headache is a lot of pain to deal with. 

EDS appointment in London.

So today I traveled up from Dorset/Hampshire (I’m never quite sure where to say I’m from as I live pretty much on the boarder between Hampshire and Dorset.) to London (which is just over an hour on the train if you get the fast one) to the Hospital Of St John’s and St Elizabeth’s for my appointment with a rheumatologist professor. The last time I saw him was back in 2011, my Professor of autonomic dysfunction who I see for my POTS referred me to him as he thought I had hypermobility along with POTS, the rheum diagnosed me back in 2011 with Ehlers Danlos Syndrome Type III Hypermobility (EDS). At the time I didn’t have many symptoms other than some lower back pain, so we just left it with that; a diagnosis as at the time nothing needed to be done. 

Three years later and I developed joint pain within 6 months it has spread to most of joints being affected by pain, not constantly but enough to warrant another appointment to see what can be done. So today I saw the professor, he said my pain is related to the fact I have EDS. We spoke about the last three years and what that has entailed for me, and then about the joint pain I have been experiencing, he then examined my joints and came to the conclusion that I need to have physiotherapy to see if that will help my pain. We spoke about pain meds and I said I am not allowed pain meds like opiates because of my headache and that if I want surgery then I’m not allowed opiates as I would then not be a candidate for surgery. So we are going to start with physio at the hospital in London, where the physiotherapists are educated about EDS and know how to treat it with physio, whereas if I went to someone local they probably wouldn’t be familiar with EDS and how to treat it. Which is true, I went to my local physio in October about my wrist and he had never even heard of EDS, so of course had no idea how to treat it successfully. I have an appointment next Tuesday up in London with the physio department. So that means the long and expensive journey up to London for an hour and a half of physio. Hopefully it will help ease my joint pain and help my joints not pop out of socket at inconvenient times.

I asked about the relation between NDPH and EDS, he said that my neuro is the specialist on that front and that he said they are not related so I should take what he says. He said there is a relation between EDS and migraine and that EDS patients are more prone to migraine but not NDPH, but the impression I got from what he was saying was that he hadn’t come across many people with NDPH before. 

So will see if physio helps and go from there. But right now my head is at an 8/10 on the pain scale after my busy day in London, so I need to go to sleep and hopefully it won’t be so bad in the morning. 

Bit of a break, so bit of an update.

So, I’ve had a bit of a break from writing, I suppose it hasn’t really done me any good but it hasn’t done me any harm either but I was just struggling with what to say and how I am feeling; which I still haven’t quite figured out but hey ho. 

The pain hasn’t been too good the past few days, it could potentially be the weather because it’s got quite hot and that’s the only thing that has really changed, I know I don’t do well in the heat because of my POTS, and I’ve never really pinned down whether the weather makes any difference to my pain levels. So I haven’t been feeling too bright because the pain hasn’t been too good. 

Tomorrow I’m off up to London to see the rheumatologist professor that diagnosed me with EDS III back in 2011. So will see what he says about my joints and the pain and problems they have been giving me and whether not it is going to get worse. And also I want to know about the relation of my EDS and my NDPH, my neuro says there is no relation but my research tells me different, so I want to know from someone who is an expert in EDS about it. 

I will finish my overcoming depression series of posts, but it will probably be Wednesday when that is published now. 

Struggling on day two.

So I’ve been staying in Lymington with family friends for two days now, and it’s okay but I’m struggling a bit. Yesterday I took my dog fora walk by the sea and then went into town to have lunch with the guy I’m staying with which was nice but by the afternoon I was knackered and my joints were really hurting, so I led in bed till the people I’m staying with got home from work. I’m finding it quite hard to be sociable with them in the evenings, when at the minute I just want to lay in bed because not only my head hurts but my joints too.

Today I went to Bournemouth with the guy I’m staying with to an Indian food shop to get all the ingredients for the first night of our charity pop up Indian restaurant we are doing on Friday night. J the guy I’m staying with is raising money for a breast cancer charity for this huge 100 miles bike ride he is doing in August, and he asked if I would help with the cooking. We are doing 3 nights of it, it’s a 4 course meal and you pay what you think the meal is worth, we are aiming for £1000 so hopefully we will get there. I even have a Saree to wear and J is wearing a turban and an Indian outfit and so is his partner Claire. So it should be pretty good.

After doing the shopping I was meant to take my dog for a walk, but I haven’t because I am so tired and my joints are really hurting and I can’t really face going on a walk today, which I feel bad about because my dog has practically been led in her bed and my bed all day. But I just really needed to give my joints a rest today and couldn’t face a walk, but I will give her a nice walk by the sea tomorrow. I’m finding the fact that I have to walk my dog every single day quite hard also, usually my mum does it most days and then I go with her a couple of times a week, but cause she’s away for two weeks the responsibility falls on me.

I’m getting pretty fed up of this joint pain to be honest, by the end of the day most of my joints are hurting especially my knees at the minute, they practically all seize up in the evening and if I sit down it’s hard to get back up and it’s hard to walk. Typing on the computer isn’t doing me much good either to be honest, it hurts my hand joints after a while so that sucks. Trying not to worry about it all till my appointment with the rheumatologist who diagnosed me on the 10th June.

I think I’m really going to struggle these coming two weeks, I like the people I’m staying with and get on with them really well it’s just hard to be sociable all evening and also hard walking my dog every day when my joints hurt.

Struggling with acceptance.

I’m feeling pretty low right now. Everything from the past two weeks has just got on top of me, the pain in my head the joint pain and issues I’m experiencing, giving in to the temptation of trying to find a reason why I am always in pain; why I always have a headache, putting my acceptance on hold to go after said quest. And it’s just got on top of me and I’m sat here feeling pretty damn miserable about everything right now. Even the good news I got last week of getting into college isn’t enough to make me feel okay. And I’m sat here in pain with my mind racing about what next year at college is going to be like and worrying about it. Worrying about the fact that my joints hurt and will that get worse?! I’m worried about my future, the future I recently seemed to get back on track.

I saw my psychologist today, we had a good chat about all my issues that have come to light over the past two weeks. I told her about my recent wavering and trying to find a reason why I have a headache, that my acceptance has wavered because of it. I told her I knew that what I was doing wasn’t healthy for me to be getting back into but I couldn’t resist the temptation of it. She said that there were bound to be bumps in my acceptance and this was one of them. But she knew I was aware that this wasn’t a good thing for me to be doing and that was the first step to getting back on track. We talked about my joint pain and she said she knows the rheumatologist I’m seeing from when she use to work in London.

I need to get my acceptance back on track but I’m just not sure how I get back there with added pain. I’m trying to stop worrying about my EDS until my doctors appointment in a month time. But it’s hard when my immediate reaction to things that happen is worst case scenario and over think things. I stopped my recent quest to find a reason why I have a headache, I guess that is the first step to getting back on track again.