Meet my precious Pablo

Medical equipment is a pain in the butt! But given it's still Dysautonomia awareness month I thought I'd introduce you to one of my more hidden pieces of equipment that helps me get by.

His name is Pablo the power port and I keep him very close to my heart. Infact, he is actually in my heart (well the top of one chamber anyway). He is a central line and I love him! 

To give a very brief rundown, a portacath is a central line that is implanted under the skin in the upper chest which runs from a special septum into a catheter tunneled under the skin, going over the collar bone and then enters the large vein in the lower neck (the internal jugular vein) and then usually finishes in the superior vena cava. It appears as a small bump under the skin, and a small scar from the insertion. Most people probably wouldn't notice it's even there under clothes and it is quite discrete when not in use which I really like. 

I had to get a port or some form of central line because after years in hospital and countless blood tests, IV medications and treatments my veins have gone on a permanent holiday. They are completely useless, except for occasionally one little bugger in my index finger which can draw blood...but it's a pain and very unreliable too. It became too difficult to cannulate me and so we changed to PICC lines for a while, but I got sepsis from it so we decided on Pablo.

Yes it hurt, but the skill to place it =100%

I receive regular intravenous treatments so when we ran out of veins central access just made sense as the only logical step.  It is not a light decision to have a central line placed as it comes with huge and potentially life threatening consequences, of which I have experienced first hand. There are a few ongoing reasons that I needed Pablo in my life and they are all linked to my Dysautonomia. 

The most important reason I have a port is because I receive regular blood transfusions of plasma and antibodies in the form of IVIG, which stands for intravenous immunoglobulin. Each treatment I receive more than 5000 different peoples' antibodies which flood my own body and reduce it's ability to attack itself. This treatment is slowly but surely helping me regain some of my life and I couldn't be more grateful to the amazing blood donors, Red Cross and my amazing doctors who make this possible. We call it liquid gold in my house as it is so precious to me to have something that is finally allowing me to see some gradual improvements.
Another reason I have Pablo is because I receive IV fluids & electrolytes that increase my blood volume so that my body has more blood to circulate around which makes me feel a bit better. It is actually one of the most effective and common treatments used for POTS and Dysautonomia in general in America, and pretty much worldwide, but for whatever reasons, Australian healthcare makes it much more difficult to access (which I feel is ridiculous)! I am grateful to have some access to this, as the result of extra fluids pumped in intravenously for someone with my condition is that it increases the blood volume because the fluid enters directly into the bloodstream, which means I stay more hydrated and rather than the gut and tissues absorbing the oral intake or it being expelled, it helps to increase blood pressure and reduces tachycardia which feels reeealllllly nice! 

Finally, there are other things Pablo is used for are when I'm not well like receiving some medications and antibiotics etc etc.  Funnily enough, sometimes I, and others with central lines can actually taste the medicine that is injected into my bloodstream, which is both gross and weird. Crazy hey!! 

So as you can see, Pablo plays a very important role in my life and the great thing is he can stay in for many years to come as long as I don't get an infection or he causes me any problems. He is my secret weapon and one of my most important pieces of hidden equipment.  

Now, I decided to write this blog about my hidden friend as part of dysautonomia awareness month as I wanted to inform you that although my wheelchair and other bits and pieces may be obvious and not able to be easily hidden, they are definitely not the only things that keep me going. And I am certainly not alone in this situation.

Most people refer to Dysautonomia as an 'invisible illness' because many people can function and live their daily lives without obvious aids or equipment and appear seemingly 'normal' whilst often disguising their struggles. This invisibility should Not invalidate anyone's struggles. Something that this illness has definitely taught me is  that just because you can't see something, it certainly doesn't mean it's not there. 
They say that 1 in 3 people suffer from some form of chronic illness, which is quite amazing when you think about your circle of friends or family alone. That is a lot of people fighting and suffering in silence and discreteness. Too many people if you ask me. 
People everywhere around you are fighting battles you cannot see. Many people require different medical equipment and help in some form or another, that will be invisible to the naked eye. Dysautonomia is just one of many invisible illnesses.
 So I guess as Dysautonomia month is closing, this is my little reminder to you:

in any instance, just remember things are not always as they appear. Some people are fighting battles that we know nothing about.

Be kind. Be gentle. Be human. 

xoxo

The dirty "D" word that changed my life

This month is a very important month in my calander because October is Dysautonomia Awareness Month. This month is aimed to raise awareness and promote understanding and greater knowledge about a condition that has significantly changed my life since it began in 2010.

Because it is awareness month I thought I'd give a little run down on the symptoms myself and others with dysautonomia suffer on a daily basis. Whilst there is a significant range in disability and impact, there are many people fighting the good fight to kick some dysautonomia arse! 

I suffer from two forms of Dysautonomia, primarily Autoimmune Autonomic Ganglionopathy (AAG) and secondary to that, Postural Orthostatic Tachycardia Syndrome (POTS). Dysautonomia is an umbrella term for conditions which involve malfunctioning of the autonomic nervous system. There is involvement of both the sympathetic and parasympathetic systems, with 'automatic' functions of the body impaired. In my case, I have a very severe form of both, with no other known case of equal severity that we (my team) are aware of internationally...not a title I want to own, nor keep. 

So a bit about AAG.

AAG is a very rare condition, with limited research suggesting an incidence of 1 in 1 million. Basically AAG causes widespread dysfunction in all aspects of the autonomic nervous system, so things like blood pressure, heart rate, temperature regulation, breathing and many other 'automatic' functions don't work. Typical symptoms of AAG include, but are not limited to:

- severe orthostatic hypotension (very low blood pressure upon standing)
- fainting
- gastrointestinal dysmotility 

- urinary retention (neurogenic bladder)
- fixed and dilated pupils 

- dry mouth 

- dry eyes

For me, I live with all of the above symptoms to varying degrees depending on the day. Some days are managable  while others are really challenging which makes this illness very unpredictable and difficult to live with. I pass out/faint and loose consciousness after a maximum of 7 minutes in unsupported sitting (which is a HUGE improvement), my eyes need treatment multiple times per day for dryness, my mouth is an eternal desert, I have a bladder that has failed and ongoing GI dysmotility and symptoms. Other symptoms I experience are neuropathic pain, muscle weakness, deconditioning, dizziness, nausea and much more that I won't go into which just fade into the daily grind. All parts of what makes up 'Erika' and all hard work. Many of these issues can be hidden or disguised from the general public and fit under the category of an invisible illness, but unfortunately many of them I cannot hide making my disability quite obvious.  

                                                 

POTS

In my case, POTS is a symptom of my AAG, but brings its own significant challenges to the table. Researchers say that a person with POTS experiences a similar feeling to that of someone suffering COPD and congestive heart failure. For me, it feels like my body never rests. I can feel my heart beating in my chest 90% of the time, night or day and my heart rate rests tachycardic, at over 100bpm permanently despite medication, where a normal person rests between 60-80bpm. Upon sitting up, putting my arms above my head or rolling over my heart rate can jump to around 150- 200bpm on any given day.

While the diagnostic criteria focus on the abnormal heart rate increase upon standing (or even sitting in my case aswell), POTS usually presents with symptoms much more complex than a simple increase in heart rate. POTS patients often have hypovolemia (low blood volume) and high levels of plasma norepinephrine while standing, reflecting increased sympathetic nervous system activation. Approxiamtely 50% of POTS patients have a small fiber neuropathy that impacts their sudomotor nerves. Many POTS patients also experience fatigue, headaches, lightheadedness, heart palpitations, exercise intolerance, nausea, diminished concentration, tremulousness (shaking), syncope (fainting), coldness or pain in the extremeties, chest pain and shortness of breath. Patients can develop a reddish purple color in the legs upon standing, believed to be caused by blood pooling or poor circulation. The color change subsides upon returning to a reclined position. Everyday and even sometimes within hours my situation and POTS symptoms can change. I can go from my usual level of function to nauseated and dizzy in minutes and this fluctuation is not uncommon in POTS. It is an uncomfortable and highly debilitating illness.

                               

Whilst there is NO cure for AAG or POTS, for me there is hope for better management and better quality of life. It's all about finding a personal balance, and for me, keeping my frame of mind in the right zone to stay hopeful but realistic. As my neurologist says, we like to remain "positively optimistic". 

I will live with Dysautonomia for the rest of my life. It may progress, stabilise or somewhat improve - there is no set pathway and not enough evidence to truly know. I just thankful I am on the improvement pathway, rather than progressive for now. 

The reality is though, until more research, funding and interest is taken into Dysautonomia the struggle will continue for all of us affected. In Australia there are very few doctors aware of this condition which makes diagnosis and treatment extremely difficult and frustrating, often resulting in mis-diagnosis and lack of understanding. 

So spread the word people, it all starts with awareness!!! 

The more people who know about these horrid conditions, the closer we might get to a cure someday. 

xoxo


**Info taken from Dysautonomia International