PID = Primary Immunodeficiency
CVID = Common Variable Immunodeficiency (currently the best label/name we can give my PID)
SPS = Stiff Person Syndrome (this is an autoimmune disease)
CF = Cystic Fibrosis
Azithro = Azithromycin - prophylactic (proactive) antibiotic for CF and other infections that I take daily

Primary immunodeficiency (PID) are genetic in nature. i.e. you are born with gene defect(s). PID refers to a group of more than 430 disorders that can affect your immune system. These disorders prevent your immune system from functioning properly and it is dysregulated. This can make you more susceptible to infection (or other infectious diseases / ailments e.g. gastro), allergy, inflamation, and acquiring certain diseases such as autoimmune diseases and cancers.

Autoimmune diseases occur when a person's immune system mistakenly attacks their own body tissues. Usually something that happens later in life. Normally, your immune system creates proteins called antibodies that work to protect you against harmful substances such as viruses, cancer cells, and toxins. But with autoimmune disorders, your immune system can't tell the difference between invaders and healthy cells. Genes can contribute to autoimmune diseases.

Put another way:
When your immune system fails to respond adequately to fight something it should, it's called an immunodeficiency, and you may be immunocompromised. It is a PID if you were born with it. So my body can't fight a cold or flu or other infectious diseases like normal people/immune sytems can. My PID means my body lets its guard down and lets things in easily and then can't fight them once they are in.
Whereas an autoimmune response is the opposite condition, an overactive immune system that attacks healthy cells as though they were foreign bodies. e.g. my body thinks perfume and flowers and cute dogs are dangerous.

Triggers are:
- Being startled (unexpected noise, a bird flying past, someone yelling when it wasn't expected etc) anything that wasn't epected and is a shock.
- Emotions & Stress - both good and bad! i.e. if I've had an overwhelmingly happy day or experience that can be just as based as if something awful or stressful happens! This really sucks because you don't want a monotone life!
- Cold - being cold is my biggest trigger. Going from warm to cold quickly is really bad. Keeping the body warm is best.
- Touch can be a trigger. For me it is my feet, there have been many an episode where a massage therapist or physio would cop a kick to the head as my foot and calf go into spasm. Or a startled touch. i.e. someone coming up behind me and touching me when I wasn't expecting it.

What's it like:
- The spasms range from a twitch, pulsation that doesn't stop, through to a full blown cramp but one that is like 100 times worse than a cramp. i.e. my neck one came on life a bullet and like a vice in the woodworking room and clamped my spinal cord into my neck.
- Sometimes the spams can be literally from the way I simply move my body. e.g. I reach for a pillow in bed and next minute they are tugging on my underarm and arm pit. Or I climb into someones car and if the action isn't perfect they trigger off through my hip and back and thigh. Basically - you have no ability to stop them or know. But I try to be mindful on how I'm reaching or moving for things as a basic.
- The rigidity is literally like your body has turned to concrete. I've lost count how many therapists have touched my body as asked WTF is wrong with your X (calves, ribcage, neck, shoulders, hamstrings, stomach, bicep, hand etc). This is because they can't get the skin and underlying body to move. It if frozen down, locked down, like concrete. And, usually it takes up to an hour to get the concrete to move. But, to then just shut down again by the next day.
- The stiffness is the feeling you might have after you've ran a race the day before and pull up with stiff calves or hamstrings.... or if you've been camping and wake up with a sore back. That stiffness is there permanently. It pretty much doesn't 'go after a couple days' like the run or the camping!
- Weakness & fatigue: all of these things lead to extreme weakness. The specialists have said both the spasms and rigidity going off in your body almost 24/7 is like your body is out on a running marathon that doesn't end and all body parts are involved and there is no down time and rest time. It is constantly under physical stress so that leads to weakness and fatigue.

All in all these aspects are in my body all day every day and I've just become match fit. Every day hurts, some days are hell. But no one would really notice unless big flare came on as I just have the hang of the feeling. They came on full blown in April 2016. But in hindsight they'd started in my feet and calves back when Irish wifey was my live in carer (I think like 2012??) and one of her jobs was to very quickly fetch warm/hot water in a bucket with magnesium oil to help relieve what was though were supercharged cramps in my feet and calves that would last hours. Everyone thought they were from steriods and antiobiotics and being bed ridden in hospital or hospital from home etc.

Foods = onion (includes shallots, chives, spring onions, but garlic is good); vinegar (often in sauces eg. mayo or mustard. Balsamic in extremely bad. Rice vinegar is good). Sulphites - anything that says 'contains or may contain sulphites' or has a perservative number in 220's. Whilst mushroom is not an allergy it harvests yeast like a bonfire which then spurs on bad infections, so I avoid mushroom.

Drugs = most opiods (e.g. endone), anything with sulphites (e.g. panamax has it). Note that most drugs won't list sulphites on their label! You have to google for extra ingredients not listed. Anti-inflamatories like Nurofen, Asprin. Doxyclyine makes me vomit. Tramadol makes me vomit my own poop (yep!). Discharge notes from prior admission say sensitive to penicillin.

Aloe Vera = it likes to turn up everywhere from shampoo to lipbalm.

It is preferred we don't just go to emergency or call an ambulance - unless we can't get ahold of one of these drs in the first instance and it is a genuine emergency.

The preferred hospital is the Mater where my CF Dr A/Prof Lucy Burr is and where my neuroimmunologist Dr Reuben Beer or Andrew Swayne are based. The opinion of my specialists is that mainstream drs who don't know my rare situations will provide treatment that could be detrimental / fatal.

Dr/Prof Stuart Reece - GP - 0424 439 639 or 07 3844 4000
Cathy Chuchwood is Lucy's assistant at the Mater so during business hours can be got on 07 3163 1205 and has me flagged in the system as needing specialised care.
Dr Maurice Heiner - Respiratory who has uncovered everything with Dr Reece - 0409 576 270 or 07 3870 4468 or 07 5598 0765