Just another quick update

I am afraid it is just going to be another quick update as I am in another cycle of insomnia / pain which means I am exhausted nonstop. My current sleeping habits are, crashing shortly after 6pm and then sleeping until 1am. Then I spend the rest of the night wide awake. Every few nights I manage 12 hours but they are few and far between. It is a little draining to say the least.

I saw my PoTS consultant a couple of weeks ago but I am so annoyed about the way he spoke to me I have found it difficult to process. He told me I spent too much time on the Internet looking up syndromes to have. This was all because I dared to raise the possibility of Poly-cystic Ovary Syndrome. It runs in my family, the condition is hereditary and due to its links with Fowler's syndrome it would fit with my bladder problems. I wasn't plucking something out of thin air. I don't want any of the conditions I have and would willingly give them back to have a normal life. I was then told I was being defensive when I pointed out that if I hadn't of spent time on the Internet looking up syndromes I would have never have found out about PoTS or EDS and that I wouldn't be sat in front of him. He tried to make light of what he had said but it was too late the damage was done.

It seems to have been the month for people being told that they are collecting labels. I have seen many other PoTS & EDS patients complain of the same accusation being levelled at them over the last month or so. I think I have traced back the origin of this accusation to a talk held in May at the EDS UK conference, which is summarised in the Beating your limitations   blog post by Donna. The slides that accompanied the talk can be found here.  The first part of the talk appears to have been about collecting labels.......obviously at some point the words collecting labels has been switched for syndromes. 

If there had been one or two of us accused of this by one doctor I wouldn't think anything of it. However it has been said, exactly as it was said to me by a number of different doctors up and down the country to a large amount of patients. That is scary, we aren't allowed to mention any problems we are having for fear of being told we are spending too much time on the internet? That statement sort of falls apart when one of those patients is later diagnosed with cancer and the doctor missed it because he didn't believe the patient when they told them about new or worrying symptoms.

I will be honest I am still deeply hurt and upset by my consultants attitude. He has never acted in that way towards me before and I have never raised any other conditions with him previously. I just don't understand why it was said and to what ends?

It's made me very upset just writing about it and the appointment was several weeks ago now.

I had a gp appointment booked for the following day and that went better. My  gp thinks my gynae problems are fibroid's and I am having a pelvic ultrasound in September. I was very upset when I spoke to my gp about the consultants accusation the day before and ended up breaking down on him which I hate doing. I feel sorry for the poor chap, he must have a never ending stream of crying patients to deal with all day. I don't think I could cope with it.

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I need to end this blog post on a high so I am happy to announce the arrival of my new nephew Elijah, who was born 25th August, weighing in at 7lb 4oz. Mum and baby are doing well. I am looking forward to some cuddles soon!

Update ....

Things have been hectic here health wise over the last week and so the blog post has been sacrificed so that I can rest and recuperate.

Some of you know I suffer with the skin condition Hidradenitis Suppurativa, which is where for some reason unknown to science the body produces abscesses. I am lucky in that in all the years I have had HS it has only ever been stage one and hasn't moved up in stages. However that isn't to say that it isn't painful or leaves you feeling like crap when it happens.

Last week I developed one right under my left butt cheek, which meant sitting down was very painful. I did all the usual things I do to try to treat it without antibiotics but it didn't work. I ended up on antibiotics and then suffered horrific side effects from the antibiotics. Side effects that can only be described as explosive shits for 17 hours and playing Russian roulette every time I went to break wind.

The side effects wiped me out for three days, I had my first good day yesterday but then I ended up in a massive pain flare where every movement caused pain. I think it was the changing weather fronts that caused the pain flare and it was the worst I had ever experienced. 

Today's health fun comes in the form of vertigo. I am typing this with only one eye open and the chromebook balanced on my chest.

Someday's I feel like hiding under the duvet until 2015 is over......

Could it be Postural Orthostatic Tachycardia Syndrome - PoTS?

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

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

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

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

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

My main symptoms at the time of diagnosis were as follows

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

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

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

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

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

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

Secondly understand what PoTS is!

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

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

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

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

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

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

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

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

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

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

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

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

What to do next?

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

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

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

You will get there in the end!

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

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

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

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

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

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

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

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

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

The dogs in my life part two

It has taken me far longer to produce the second part of The Dogs in my Life, than I ever imagined it would. Life just gets in the way and as always my good intentions fall by the wayside. I also knew that writing it would stir up a lot of emotion and I have tried to avoid it for as long as possible.

See the first part of the post here : The dogs in my life part one

Last time I had introduced you to Mollie our oldest Weimaraner who will be 11 in November. She still behaves like a puppy and the vets can never get over how good she looks for a nearly 11 year old dog.

Mollie asleep on my bed.

As with all dogs they don't live forever, Travis our first dog as a couple left this world in October 2006. Travis had first become sick at 6 months old which we initially thought was just a stomach upset. Over time he began to have many more stomach upsets and we would find ourselves at the vets at all hours of the day and night with him. When he wasn't sick you would never know that there was anything wrong with him. By the time he was nearly three years old he had, had many investigations performed on him at the major veterinary hospital in the southwest and no one could tell us why he was getting sick. In the end his kidneys started to fail, I believe that this was due to a type of medication he was repeatedly given. My other dog Frankie can not take this medication without passing blood when he urinates. With my own research I have found that susceptible dogs die of kidney failure. I believe Travis actually had lungworm. At the time very little was known about lungworm, his symptoms fit the description even down to the spontaneous nose bleeds and hemorrhaging from his penis.

When we made the very painful decision to let Travis go, just days short of his 3rd birthday, it was the kindest thing that we could do. However when he passed he took a little piece of my heart with him. I will never love another dog in the same way that I loved my boy. My husbands scream of pain as he held Travis's lifeless body in his arms still haunt me, next year it will be ten years since his passing. Writing this brings all the pain of that day back to the surface. As my dad told me today, "all the love you poured into him is equal to the pain you feel with his passing". It is so very true. I have been sobbing my heart out writing this because the pain is still so very intense.

Trav's ashes are still at home with us, they are on a shelf in the lounge along with his collar and the stones he chased on the very last walk my husband took him on. One day, long in the future I hope those ashes will be mixed with my own and spread on Woodbury Common, so we can be together in the place we both loved exploring.

Travis on Woodbury Common

When Travis died the timing was bittersweet, it was ten days after Mollie had given birth to her first litter of puppies. Our emotions were all over the place ten days earlier our hearts had been filled with joy and now we were at the depths of despair. Mollie gave birth to a litter consisting of 6 boys and 3 girls. Eight of the pups were large, weighing well over 500g and then the runt of the litter was born, screeching inside the membrane sac. It was love at first sight with this pup and weighing in at just 420g we knew that we would be keeping her. I was terrified that our little runt, named Willow wasn't going to make it. In an attempt to increase her odds of survival I started to bottle feed Willow every two hours day and night. Puppies can die from a condition called fading puppy syndrome, where they can appear healthy one minute and yet within a couple of hours be dead. I have known breeders who have had this happen to them and I have to say it was always my greatest fear when we were dog breeding.

Mollie with her first litter

We had decided before Mollie's pups were born that we would be keeping a girl from the litter. We had already been given Travis's devastating prognosis and I was adamant that we would never have another boy dog. I felt that a boy dog would be constantly compared to Travis and would never be able to get out from under his shadow. It is funny how things work out and the universe laughs at all your plans.

We managed to find forever homes for all our pups, well we thought we had. A week after the last pup had been sold we received a distraught phone call from one of the new owners. They would have to return a pup called Frank to us as their daughter had developed a serious allergy to the dog. As a responsible breeder I took the puppy back the following day. I have to say it was an awful experience. The couple arrived visibly distraught, my heart broke for them. The wife had to do all the talking because every time the husband went to speak he couldn't get the words out through his sobs. They brought all the puppy stuff they had bought for Frank with them, a bed, a bowl, a collar, lead you name it they had it. I knew from their reaction that these people had fallen in love with this little guy. I can't imagine having to return a pup back to the breeder and I salute them for doing it and not trying to sell him on (as I have heard about people doing).

Frank (now named Frankie) was never supposed to stay with us. I was resolute that he was being sold and had an advert in the newspaper the following day. He fitted back in with the girls like he had never been gone. They seemed genuinely attached to him and within four days I was in love. I never thought I would ever be able to give my heart away again to a boy dog (it is not the same love I had for Travis). Hubby was as determined as I was that we were not having three dogs. So I hatched a plan to ensure I could keep him, I wrote a letter from Mollie and Willow and left it for him to find when he came home from work. It went a little like this.

Dear Daddy,

Please can we keep Frankie? We love him very much and we know it would break mummy's heart if she had to part with him. We are sure you love him as well but are scared mummy will say no. Please let us keep him.

Lots of love

Mollie and Willow

xxx

When Hubby returned to work after his lunch break he came and found me (we used to work together and could work our hours around the mutts). He simply kissed me on the cheek and said of course we can. This was met by a massive cheer from my staff who had been nearby watching as I had confessed my plan to them. Quite romantic really, so that is how Frankie came to be part of the family. I wrote to his previous owners to let them know that we had kept Frankie and to say thank you to them for returning him to us. I never heard from them ever again, I like to believe that having to return him was just too painful for them. I hope that they were pleased with our decision.

Frankie and Willow approx 10 weeks old

My illness began in January 2007, just a few months after the first litter of pups were born. We bred again later in 2007 but the stresses and strains of working full-time and raising a litter were exhausting. We didn't know it but that would be the last litter of puppies we would ever raise. 

The second litter consisted of 6 girls and 3 boys, in a complete reversal of the first one. This time I helped Mollie birth her pups alone (with the first Litter I had help from J & K - thank goodness), it was both exhilarating and terrifying all in one. The first pup was born without a hitch but the second pup was very slow in coming and I nearly had to rush Mollie to the vets for help. The puppies were enormous all around 550g and were born on day 57 (a dogs pregnancy is approx 63 days). All the books I had read on whelping had said puppies born on or before day 57 die, well in this litters case it just wasn't true. They were absolutely enormous and there was only a 20g difference between the biggest and smallest pup. As with the first litter they were born on a Saturday (an odd coincidence).

However when the pups were ready to sell the world financial crisis was just beginning to bite. People didn't want big dogs that ate lots of food and had big vets bills. We had an absolute nightmare time getting rid of the puppies, were messed around by potential buyers on numerous occasions. If our second litter experience had been our first litter experience we would never have done it a second time. Eventually after much stress and selling a couple much cheaper than we imagined we would, all the puppies were found forever homes. Well what we thought were forever homes. 

A few months after all the pups were sold I had a distressing phone call from a woman who had bought one of my pups through a newspaper advert. The pup had been sold on from its original owners without my knowledge and what was worth in its short time with them it had been abused. The lady informed me that the puppy now 6 months old was extremely nervous and had bite mark shaped scars all over its head and ears. It broke my heart that one of our pups had fallen into the hands of such awful people. We had met them, talked to them for hours to be sure that they were the right people to take one of our precious pups on. I hated the fact we had been unable to protect this pup once it had left our care and we had been fooled by these people into parting with it. At least the puppy now had a good home, how do I know that? Because the lady that took her on rang me as when she bought her she came with her original puppy pack which included my telephone number. Someone who didn't care about the puppy would never have rung me to check out if she had any health problems, what food she should be fed etc.

Breeding is fun but is also incredibly hard work. People always used to ask me "do you get upset when they leave you?" The answer is yes and no, yes I miss their individual characters and cuddles. I don't miss 9 piles of dog poo or puppy presents as we used to call them, 9 pups trying to chew your shoe laces off or the general mischief they cause. However if I thought 9 Weimaraners could cause damage I had no idea what utter home wreckers my three would be. That is material for another blog post!

Nurse Frankie

Willow snuggling on my bed

Mollie drooling waiting for a Bonio