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.

My biggest fear.

My biggest fear is that I will get sicker, maybe it’s completely irrational to have this fear but I do and it’s hard to get rid of.

I have a total of three conditions that have been diagnosed, chronic pain in the form of New Daily Persistent Headache, which is my main problem and is what I talk about most on here, and then Postural Orthostatic Tachycardia Syndrome a chronic illness which is a dysfunction of the autonomic nervous system, and Ehlers Danlos Syndrome Type III which is a genetic connective tissue disorder which is meant to be reasonably rare.

Postural Orthostatic Tachycardia Syndrome or POTS is a dysfunction of the autonomic nervous system, the hallmark symptom is an increase of heart rate up to 30bpm upon going from sitting to standing position. When I change positions from sitting to standing or lying to standing my resting heart rate which is high already at 120bpm increases to over 150bpm and my blood pressure plummets even lower than it usually is, which is low already. This causes me to effectively pass out, however with me I got it so bad I would have what looked like a seizure although it’s not epileptic. I would effectively shake and jerk to the floor and continue shaking and jerking once I had hit the floor. This use to happen multiple times a day for years before I got diagnosed. I got diagnosed in summer 2011 when I was searching for answers for my headache which I eventually got diagnose in summer 2011 also. It was never my biggest problem and I kind of learnt to live with it as it started when I was about 12 and got progressively worse, I was 17 when I got diagnosed and put on medication which has helped control it to the point when I no longer pass out and have seizures. I don’t have a fear that this will get worse as it’s now controlled by medication and I’m pretty much stable on that front.

At the same time I was diagnosed with POTS I was diagnosed with Ehler Danlos Syndrome Type III Hypermobility EDS, my POTS professor thought I had it in conjunction with POTS as they often go together so sent me to a rheumatologist who officially diagnosed me with it. It never really caused me much of a problem, but fitted in with me having painful knees when I was younger and every time I went to the doctors about it he turned me away saying there was nothing wrong with me, and the fact that my hip pops out of joint every now and again.

Recently I’ve been having problems with my joints, it started with my left wrist a few months back, it hurt and I couldn’t put and weight or pressure on it or bend it back without pain. I went to physio but it didn’t really help and the physio guy didn’t know much about EDS when I told him I had it so couldn’t really comment on what was causing my pain. But physio didn’t help so we splinted my wrist for a while and that seemed to help a bit. But over the last few weeks it’s got worse again so I have had to go back to splinting it. And now I seem to be having problems with my right knee as well, it feels like it’s the problem is with the tendon in back of my knee, it hurts and pulls and twinges when I do things. So I’ve been wearing a knee support when I’ve been doing Tae Kwon-Do but it doesn’t seem to be helping. And then last night my right ankle started hurting and becoming painful when I was walking. And then I have a problem with my hip popping out of place and being painful when I do certain things in Tae Kwon-Do. It has to be my EDS that is causing all of this and there is nothing I can really do to help it. I’ve ordered some kinesio tape so I can tape myself up for Tae Kwon-Do as it’s meant to really help athletes and also EDS. But I’ve also come to the conclusion that I am completely the wrong sport for this medical condition, but I will NOT be giving it up, it’s a big part of me and no medical condition is ever going to take that away.

Back to the fear thing. My biggest fear is getting sicker, and I’m scared it’s happening with my EDS. I’m scared it’s getting worse and I won’t be able to do anything to stop it. But I’m scared it will get worse and worse until I can’t do simple things like walking without huge amounts of pain.

Note to self.

Note to self: Sian take your POTS meds properly otherwise you will have black outs and seizures again.

So this morning I got up went downstairs came back upstairs, sat on the sofa in the study/landing for about half an hour catching up on Facebook notifications I had got in the night, got up to go back into my room, didn’t even get to the doorway of my room before I started to feel faint and before I knew it I was blacking out and seizing, shaking and jerking to the floor uncontrollably, hurt my arm in the process and it wasn’t fun. 

I know why it is, I hadn’t taken my midrodine yet, the last time I had taken it was last night and I was yet to take it this morning, and I’ve been very lax about taking my fludrocortisone recently. And lets not even mention that I haven’t been injecting much recently. Which is bad I know, so because of that I blacked out and had a seizure, oh the joys of having POTS. So from now I will start taking my meds properly again, as I don’t really want to go back to having multiple seizures a day every time I stand up cause it’s not fun at all. 

 

I don’t know what’s happening.

So I have POTS, Postural Orthostatic Tachycardia Syndrome. I don’t talk about it much because it doesn’t bother me so much anymore as long as I take my meds for it. I’ve had symptoms of it since I was 12 but was only diagnosed in 2011. I use to black out/faint and have seizure type things every time I stood up and it was awful and kind of scary as well. In 2011 when I was diagnosed I got put on lots of medication for it, Fludrocortisone, Midrodine and injecting a medication called Octreotide 3x a day which is very annoying to have to do but the combination keeps me stable. I do however still have a very high resting heart rate of about 120 bpm, so exercising can be difficult and I have to take a lot of breaks cause I get tired really quickly. But I still do taekwondo despite all of this.

Over the past few days I’ve been starting to black out again. In the morning first thing when I get up. I get up go downstairs, and I’m on the verge of fainting the entire time I’m stood up. I feel faint, my vision starts to blur my hearing goes funny and I have to sit back down to be able to control it and come out of it. I don’t know what’s going on. My meds have always kept me stable up until now. This happens for about an hour or so and then I start to feel okay again. It’s weird. I’m hoping it will settle back down soon, I hope!

30 things.

30 Things about my invisible illness you may not know:

1. The illness’ I live with is: New Daily Persistent Headache (NDPH) Postural Orthostatic Tachycardia Syndrome (POTS) Elhers Danlos Syndrome Type III (EDS III)

2. I was officially diagnosed with it in the year: June 2011

3. But I had symptoms since: NDPH Since 14th January 2010 and POTS since 2005

4. The biggest adjustment I’ve had to make is: Learning that I can’t do everything and that doesn’t make me a failure.

5. Most people assume: That I’m lazy, faking it or exaggerating.

6. The hardest part about mornings are: Waking up and realizing that I have to spend another day in constant pain.

7. My favorite medical TV show is: House and Greys Anatomy

8. A gadget I couldn’t live without is: Laptop.

9. The hardest part about nights is: The pain is usually at it’s highest at night.

10. Each day I take __ pills & vitamins (no comments please): At the minute 4 different types and injecting 3x a day. It has got up to over 10 different types of medication though.

11. Regarding alternative treatments I: Have tried, Osteopathy, Physiotherapy, Acupuncture, Massage, Reiki, you name it I’ve probably tried it.

12. If I had to choose between an invisible illness or visible I would choose: Visible.

13. Regarding working and career: I had to drop out of school last October on my 3rd attempt of year 12. And I am unable to work.

14. People would be surprised to know: That I’m actually sick, and that’s the reason why you think I’m lazy and that I most definately am not faking it.

15. The hardest thing to accept about my new reality has been: That I can’t do every single thing I want to do, and having things taken away from me that I’ve wanted to do but can’t like school and university.

16. Something I never thought I could do with my illness that I did was: Pass my driving test in Dubai.

17. The commercials about my illness: They don’t exist, most people don’t even know that my illness’ exist let alone have commercials about them.

18. Something I really miss doing since I was diagnosed is: Being able to go to school and participate properly.

19. It was really hard to have to give up: School

20. A new hobby I have taken up since my diagnosis is: Lying in bed and watching TV series does that count?

21. If I could have one day of feeling normal again I would: I don’t even know.

22. My illness has taught me: That you should never take a pain free, healthy normal life for granted.

23. Want to know a secret? One thing people say that gets under my skin is: When people say i get headaches NO I have ONE headache, headaches implys that it comes and goes that is not correct it has never gone.

24. But I love it when people: Ask genuinely how I’m feeling.

25. My favorite motto, scripture, quote that gets me through tough times is: I don’t actually have one, I’m quite a negative person (don’t tell me that I will feel better if I’m positive tried that it doesn’t work).

26. When someone is diagnosed I’d like to tell them: Keep fighting and stay strong, you are not alone, the chronic pain and illness community is actually quite big on facebook and the blogging community.

27. Something that has surprised me about living with an illness is: Just how sick I actually am and how much pain I am in all the time, I never thought this much pain existed, I was wrong.

28. The nicest thing someone did for me when I wasn’t feeling well was: My sister giving me a cuddle and kissing my head to make it feel better.

29. I’m involved with Invisible Illness Week because: It’s so very important to spread awareness of invisible chronic illness’ as it’s the only way we will get rid of the stigma of ‘it’s all in your head’.

30. The fact that you read this list makes me feel: Great, Thank you for taking the time 🙂

General update.

Haven’t been feeling so great. With my Triptans not working and the Mirtazapine I’m on as a headache preventative not working either, I’ve been dealing with a lot of disappointment. 

I’ve also been feeling quite lonely, miserable and stuck. All my friends are off to uni like this weekend. They are all moving on with their lives and I’m just kind of left the only way to describe it is stuck. And I’m angry. Not at them, but at everything. At the fact that I’m not normal, I’m sick, and I can’t go to uni like everyone else. 

Then today there was a stress with my medication. I was meant to now be getting my POTS medication prescribed by my GP, instead of my POTS professor in London. Got a call from the pharmacy today saying that less than a months worth of only one of my 3 medications was going to cost just under £500. Which is ridiculous as it never costs that much when I have previously got my medication from up in London. So my mum made some calls, and found out we can get my medication from the hospital in London where my POTS professor works at and we could get a 4 months supply for under £50. Compared to just under £500 we were quoted from the pharmacy at my GP’s. So I emailed my professor and asked if he can do me a prescription and if I could pick it up next week. I run out of one of my meds on Tuesday so if I can’t pick up the prescription and get it into the pharmacy before Saturday so they can order it in cause it’s quite large quantities. Then after Tuesday I will go into withdrawal. Which means I will be blacking out, fainting and having what looks like a seizure all over the place. So that’s not good. Waiting to hear back from my professor now to see if he can do it in time. 

Saw my psychologist today. She gave me some sheets to fill in. Ones a pain diary, another is an activity diary and the other is writing down when I’m specifically feeling bad and what happened. So I’ve got to do all that till I see her next week. 

Also my flights are booked back to Dubai for 10 days on the 29th of September, so just over 2 weeks to go till I get to go back for a bit. Which I’m really looking forward to.