Today I went to a cafe.

Today I went to a cafe.

"And.......?" I hear you say.

It may not seem a big deal to most. For many it is a common, or even daily event. But not for me. I can go months without heading to a cafe. Or anywhere really. Well anywhere other than needed medical appointments, which, when you think about it, are hardly a source of excitement.

I have tried many different ideas to help me get out more. Compression stockings. Extra medications. Even a wheelchair, so I at least take the exhaustion and symptom exacerbation that comes with being upright and walking, out of the equation. But my overall level of decreptitude frequently means that heading to a cafe and all the logistical issues that come with that outing (showering, getting dressed, travelling, finding somewhere with air-conditioning, a place I can put my feet up,....) , are just beyond me. I have a finite level of energy reserves, and of late they have been on a steady decline.

"So how did this miraculous outing happen?" I hear you ask.

The answer:

One litre of salty water shoved in my vein. That's it. That's all. No more. No less. No tricks. No secrets. No magical talismans.

I have no idea why this is so effective. There are multiple theories. But in truth I don't care. It works and that's enough for me.

I drink my 2-3litres of fluids each day. I increased my salt intake. I use both Florinef and DDAVP, both medications designed to make my body hold onto fluids rather than peeing them out.

And yet....

I drink. And then I pee. Often more than I originally took in (and yes I have spent a day collecting and measuring my pee, doesn't everyone?).

But with slow IV administration it stays in. I'm not running to the loo every 3 seconds like I am with oral fluids. And because it stays in, it works.

My headache that has been continuous since Christmas day is gone. And whilst I am tired (I arrived at the hospital at 9:30am and didn't leave until 3pm) that general level of malaise that I have every single day is gone or at least greatly decreased. Tired without that ever present level of indefinable unwellness is a whole different ball game. I have colour in my cheeks and feel mentally brighter. I am still tired but feel better, and more importantly more functional, than I have in months.

And when my eldest said, "do you want to go for a coffee, Mum?"

I actually said, "Yes".

I don't care whether IV Saline remains a controversial therapy in some medical circles.

I don't need their approval.

I do know I have a cardiologist, a GP and a local Day Procedure Unit who were willing to give it a go.

And today I sat in a cafe with my son and had a coffee.

And that to me is a successful treatment.

Cheers
Michelle :)



Don't forget to check out my Clicking my Heels for Dysautonomia fundraising for The Greg Page Fund for Orthostatic Intolerance, and the Baker IDI. Nearly at $1,500 already!

Florinef, I think I love you.

(Note: As always I am not offering medical advice of any sort, and not recommending any treatments.  A brain fogged woman sitting on her couch in her flannelette pjs, taping away on a keyboard, whilst binging on macarons and salty chips, is not a substitute for professional medical advice. All medical decisions, including treatment options, should be discussed with your primary treating physician.)

I've been meaning to write this post for ages. Like many plans, appointments, ability to spell my name, it all went into the sieve that is my brain, and fell straight out again. Memory, I had one of those once. I think. Maybe. What was I doing? 

I keep repeating the same tips on various forums. You'd think given that each time I write the same info I think "I should write a blog post about this", it would spark me into action. But no. Damn those bright shiny objects! 

So here it is, finally. My tips for starting/considering Florinef. These are based on my personal experience over the last 6 years, and as stated above always check with your doctor. I have been taking Florinef since 2007. It's my longest standing medication, and the one that was the game-changer in my treatment. 

Florinef (aka fludrocortisone acetate). Ah, you sexy little white tablet, you. You are my favourite pharmaceutical of them all. You let me almost have a life, rather than being stuck in bed or frequenting the carpeting or linoleum of various clothing stores. For that I'll always love you. 

Now, I'm not advocating that all Dysautonomia patients run out and try Florinef. Nor am I being paid in cash, or macarons, by it's manufacturer, Bristol-Meyers Squibb Company.  Any medication should only be taking in consultation with your doctor. The reality with Dysautonomia is that not all medications will work for all patients. It depends on your underlying cause. It depends on your tolerance levels and side-effects. It depends on your other medications. It depends on your other disorders. Some days, I swear it depends on whether Saturn is in retrograde, or if the wind is blowing from the North-East. Often the only way to know if a medication may or may not work is to take the plunge and see what happens. Even if you see an improvement, trying to find the right dose can also be problematic.

But back to the medication in question. 

The most common concern seems to be that Florinef is a steroid, and we are all rightly concerned about the long-term side-effects of continuous steroid use. Florinef is a steroid, but it is not like it's traditional cousins. No shrinking testicles for the guys, and no five o'clock shadow for those of us of the fairer sex. It will not lead to a generation of Dysautonomia patients sporting the physique of a 1980's Eastern block female Olympic athlete. It is what is known as a mineral corticosteroid. Corticosteroids are a combination of corticosteroid hormones, cortisol and aldesterone that are produced by the adrenal glands. Amongst all their varied roles in the body, they help to regulate the water/salt balance, something of importance for many Dysautonomia patients.

At it's most basic Florinef works by helping the body hold onto salt, which in turn holds more water in the body. These two processes combines to increase blood pressure and in turn reduce the reflexive reaction of our bodies to deal with the low blood pressure, tachycardia. Clear as mud, right? If you are hypovolemic, which a large percentage of patients are, or your aldesterone levels are low, it will help to increase your blood volume. 

So what are my main tips for starting and taking this medication?

• Start low: start too high and you are more likely to encounter side-effects. Titrating (going up incrementally) your dose over a few weeks can make it far easier to tolerate. For example, if your doctor prescribes .1mg, start at 1/4 or 1/2, tablet for two weeks. At the end of the two weeks, and if tolerated, add an additional 1/4 or 1/2 tablet. Repeat until your prescribed dose is reached. I have seen many patients prescribed doses such as .2mg who start on the full dose and discontinue within the first few days as the side-effects are to severe. The simple trick of titrating your dose can save a lot of pain and mean that you can take a potentially helpful medication.
• Side-effects length: the worst of the side-effects tend to last about two weeks. If you are titrating your dose be aware that you need to allow about two weeks for your body to accommodate each change. For most long-term users these side-effects become quite minimal over time.
• Side-effect types: These are many and varied. Some of the most common are: gastric distress (nausea, cramps, diarrhoea), increase in headaches, weight gain/increased appetite, fluid retention, thinning/dry skin, easy bruising, visual changes, and decrease in your immune system. I found that I had pain in my joints, particularly my hands, with the increase in fluid. A small percentage of patients will experience psychiatric symptoms when taking Florinef. If you or your family, notice a change in your mood or suicidal ideation contact your doctor and seek medical help immediately. Hypokalemia (low potassium) is a particularly serious side-effect and I'll discuss it in more detail further down.
• Break dose in two: I haven't had this issue, but I know many patients report a slump in the afternoon as the effects of Florinef wear off and blood pressure starts to crash again. It is possible to break the dose in two to maintain a more even blood concentration throughout the day. Warning: Florinef can cause or worsen insomnia. If taken later in the day it may impact on your sleeping.
• Storage: Florinef should be stored in the fridge. Update: have just found out there are some brands (Global Pharmaceuticals, Division of Impax Laboratories, Inc.) who make a form of Florinef that can be stored at room temperature. Double check with your pharmacy if that is an option. Much easier if you could get a room temp version and keep all your medications in one place. Thanks Allisone :)
• Eat before taking: one of the most common side-effects, even after long-term use is gastric distress, eg cramps, nausea or diarrhoea. If you eat something small, even a slice of toast it lines the stomach and minimises the gastric problems. 
• Hypokalemia/Low Potassium: this is an important issue for all patients taking Florinef. Florinef depletes the body's supply of potassium. Unchecked this can lead to hypokalemia, which if untreated can lead to heart attack. I am amazed at the number of patients prescribed Florinef who are not also given a prescription for a potassium supplement eg Slow K or Dura K (note most over the counter potassium supplements do not have the needed concentrations), or told to eat a high potassium diet. Many patients have ended up in the ER thanks to hypokalemia, and although it can be treated with IV potassium, this is an unpleasant experience (it can feel like you veins are burning). Maintaining potassium levels (amongst others) can be difficult for many Dysautonomia patients irrespective of Florinef, so it is important to keep an eye on your levels. A blood test before beginning and one week post-starting can be useful to see how your body reacts. After that, 3 monthly bloods are generally adequate to monitor your levels. (Some also recommend that sodium levels are also checked, but both tests are usually included in a basic blood panel.) Drops in potassium can occur rapidly as I found out. Having run out of my Slow K and thinking 1 day wouldn't matter I started to have quite severe heart palpitations and weakness. An emergency Slow K run to my local chemist and these symptoms disappeared shortly after taking 2 tablets. 
• Water and salt: Florinef's main mechanism is to help the body hold onto salt which in turn holds more water in the body. It is important to maintain a high salt and water/fluid intake for the medication to work. Without it Florinef will not work properly.
• Blood pressure: monitor your blood pressure, especially when starting. Florinef is used to raise blood pressure in those with hypovolemia. It can be a fine line between a normal blood pressure and high blood pressure. It can also take time to find the balance between dosage and healthy blood pressure. Due to changes in your underlying disorder or your body's tolerance to the medication you may need to either reduce or increase your dosage over time. If you are starting to get more headaches or you have a history of conditions such as benign intercranial hypertension, check you blood pressure to ensure you are not developing hypertension. 
• Osteoporosis/Osteopenia: Long-term users should get a DEXA scan to check their bone density as this is a risk factor for some. Especially if you are also less mobile, not outside as much or have a family history. It's no where near the same level of concern as traditional steroids, but like everything just keep it in the back of your mind.  
• Moisturise: Florinef can make your skin very dry, especially if you have been on it for a long time. Invest in a good quality moisturiser, eg one with a high percentage of shea butter and slather it on. I have noticed that as a result of drying out my skin is also more sensitive to a range of products.
• Do NOT stop taking it suddenly: this can cause a rebounding of symptoms. As was recommended for starting Florinef, titrate down especially if you are on a higher dose. For example: I recently developed supine hypertension and my cardiologist recommended a decease in my Florinef to hopefully ameliorate the change. My first attempt at a decrease by 1/2 tablet ended with rebound migraines and more frequent blood pressure drops. I reworked my titration rate and both have settled for the most part, 2 months later.

With all of these tips ALWAYS check with your doctor before implementing. 

As with all medications it is a question not only of if it works, but the risk/side-effect/benefit issue that must be balanced. This will be different for each of us. Side-effects I can tolerate may be unbearable for others and efficacy will differ amongst patients (eg Midodrine made me feel like I was going to either stroke out or have a heart attack, and yet for others it is their most useful medication.) As such, to start taking, or continue with, Florinef will be a very personal decision.

There are no doubt other tips that I will think of after I push, Publish. I will update if I do. But until then I hope these tips are helpful.

Cheers

Michelle :)

There is a lot of information about Florinef on the Internet. 

A simple guide can be found here.
A more comprehensive explanation of the pharmacotherpy behind Florinef can be found here.