Challenges in Treating Orthostatic Intolerance

Part 1 of this series covered Challenges in Diagnosing Orthostatic Intolerance. I apologize for the long delay in writing this second part on Challenges in Treating OI -  a bad crash last fall left me with only minimal writing energy for several months...but here it is now!

If you missed the first part or are new to the topic of Orthostatic Intolerance (OI), go back and read Part 1 and/or my overview post on Orthostatic Intolerance and ME/CFS. The bottom line is that if you have ME/CFS (and possibly if you have fibro, tick infections, or MCAS), then you do have some form of OI, where your body can not hold blood pressure and/or heart rate steady while upright. The good news is that treating OI can improve all symptoms--often dramatically--and treatments are quite cheap and readily available all over the world. Treating OI is what got my two young sons back to school full-time and even playing soccer again, and it is a big part of what allows me to be active again - I now walk and exercise almost daily and can walk for up to about 90 minutes (on a good day) without crashing afterwards!

So, step 1 is to diagnose OI - for details on that, see my ProHealth article, Diagnosing Orthostatic Intolerance, and that previous post on Challenges in Diagnosing Orthostatic Intolerance. Once you have the diagnosis, then you can start trying treatments! My ProHealth article on Treating Orthostatic Intolerance outlines some of the most common treatments that usually help people with OI. However, as with diagnosis, there are some challenges to treatment, and too many people give up too easily the first time they hit a challenge.

Why Treat OI?
Why bother in the first place? Because, though most of us are unaware of it, OI is behind many of the symptoms we think of as ME/CFS - it contributes greatly to fatigue, pain, and brain fog. Most importantly, OI is a big part of exercise intolerance. Treat the OI, and you will greatly improve your ability to tolerate exertion without crashing! That is the piece that was life-changing for my sons and I and so many other people around the world. 

The tips below come from LOTS of experience - both our own and that of hundreds of other patients that we have helped get the treatments they need. Keep these things in mind as you embark on finding your own effective treatments for OI. You'll be glad you did!

Salt and Fluids Are Only the First Step
Many doctors recommend extra salt and fluids after diagnosing a patient with some type(s) of OI, and that is certainly very helpful, but it is only the first step in treating OI. Part of the reason behind why OI happens in ME/CFS and related conditions is low blood volume caused by endocrine dysfunction, and extra salt and fluids can help to increase your blood volume...but only by a small amount. Ingesting salt and fluids only has a minimal impact on blood volume, so while it's necessary, there are still hormonal abnormalities that prevent our bodies from holding onto those fluids, which is why we need extra help. For starters, extra salt and fluids will help, though you need more than just a bit of salty foods. Try these approaches:

• Add extra salt to food (use sea salt or Himalayan Pink Salt) and eat salty snacks, like nuts.
• Drink lots and lots of water, herbal tea, and other non-caffeinated, non-alcoholic drinks.
• Take buffered salt tablets after meals - we like Thermotabs brand. SaltStick is another popular option. Buffered means they have the right proportion of sodium and potassium for your body. They can make some people nauseous, so taking them on a full stomach helps.
• Drink 2-4 liters of electrolyte drinks daily. We started with things like Gatorade but then had to reduce sugar in our diets (you can get Gatorade Zero, which has no sugar, but it still contains dyes). There are all kinds of drink mixes and tablets in all kinds of flavors to add to your water, so it can take some experimentation to find which ones you prefer. We like GU Brew tablets because they have no sugar, no sorbitol (which can upset the stomach), and no artificial colors or flavors. Nuun tablets are similar and contain a very small amount of sugar - they seem to have removed the sorbitol recently also. There are lots of other options - powders sweetened with various things and unflavored salt water drops, like Elete, that you can add to any drink. You can even just add some sea salt to water on your own with a squeeze of lemon or lime. Personally, I'm not one for sweet-tasting drinks, so I like V-8 juice (just don't get the low-sodium version) when I need an extra salt boost - loads of sodium, plenty of potassium, no sugar, and extra veggies!

Again, that's just the first step - you may see some improvement just from adding salt and water, but much more improvement is possible. Some people try weekly saline IVs (my son did for 2 years), so you are pumping up blood volume directly instead of going through the stomach. Those can work well, but their effects are very temporary - your body quickly comes back to its own "normal" state within a day or two - and it's an invasive procedure.

Know Which Type(s) of OI You Are Treating
Refer back to the diagnosis resources I linked to in the opening paragraphs. It's important to know which of many types of OI you have because that will guide treatment. The most common forms of OI in ME/CFS patients are POTS, where the heart rate (HR) goes up while standing, and NMH, where the BP drops while upright; however, some people have rarer forms of OI, where the HR goes down or the BP goes up or HR and/or BP jump all over the place. OI is just an umbrella term meaning that your body can't hold HR and/or BP steady while upright, so find out which types you have. As I explain in the post on Challenges in Diagnosing Orthostatic Intolerance, some doctors tend to focus solely on POTS (it seems to be the best-known OI condition), so be sure to ask for the raw data and details on exactly what your HR and BP did during the test. Your treatment approach may be different if you have "just" POTS or both POTS and NMH or some other form of OI entirely.

Tips for Using Florinef (fludrocortisone)
One treatment option that can work for all types of OI is Florinef. Its sole effect is to help the body hold onto more salt and fluids, so it can help to increase blood volume, which will help with all types of OI. A few facts about Florinef:

• It often works for kids and only rarely works for adults. Experts aren't sure why - it may have to do with the larger blood volume in adults. That said, if it works for a kid, it tends to keep working even after he becomes an adult - that's been the case for my son. So, if you are treating a kid, definitely give Florinef a try. It's what got my sons both back to school full-time. And, if you are an adult, it is still worth a try because it does work for some adults; just don't get discouraged if it doesn't work for you after increasing the dose  - move onto the next thing.
• Florinef Does Not Act As a Steroid and Does Not Suppress the Immune System. This is a very common misconception among doctors, and even the Physician's Desk Reference (PDR) is misleading on this topic. Here are the facts and an explanation, from the top pediatric expert in the world on ME/CFS and OI, Dr. Peter Rowe of Johns Hopkins:

"Some basic physiology might be helpful to people. Florinef (fludrocortisone) is a mineralocorticoid that helps primarily with salt re-absorption in the kidney, hence the mineralo-corticoid label. It has some gluco-corticoid properties, meaning that it helps in stress responses and glucose metabolism like cortisol does. 

Florinef is quite potent mg for mg in comparison to cortisol. But, the glucocorticoid effect of fludrocortisone at doses commonly used for treating orthostatic intolerance (0.1 mg daily) would be equivalent to just 1.5 mg of cortisol per day. This is a very small glucocorticoid effect, especially when compared to an adolescent's maintenance daily cortisol needs of 12-24 mg per day. 

One confusion is that the Physician's Drug Reference book (PDR) lumps fludrocortisone under the glucocorticoid class, mainly because fludrocortisone does have some glucocorticoid properties. Doing so confuses people, because there is no mention about how little glucocorticoid effect it might have when given in the recommended doses. The PDR then incorrectly suggests that Florinef can suppress the immune system. We know this can happen with cortisol and prednisone, and many people stay away from it because of the other widely known effects of glucocorticoids in high doses (so-called Cushingoid weight gain, acne, mood changes, immune suppression, yeast infections). But in about two decades of using Florinef, I have never seen any evidence of immune suppression attributable to the drug, nor has anyone developed unusual infections while being treated with it. It does not suppress cortisol levels. It can increase BP, which is part of why we use it, but this is a mineralo-corticoid effect, not a gluco-corticoid effect.  

Similarly, Florinef has no anabolic (muscle building) steroid properties as might be seen among those who want to gain an unfair advantage in sports. 

It isn't always the right medication for everyone, but it definitely can treat a subset of those with orthostatic intolerance."

Armed with those facts, here are some tips for trying Florinef:

• You need lots of salt and fluids. Since Florinef works by helping the body hold onto more salt and fluids, it can only do its job if plenty of them are available in the body. So, first increase salt and fluids, and then start Florinef (and keep up the salt and fluids).
• Start low and gradually increase dose. Start with just a half tablet (0.05 mg) per day. After a week, if there are no ill effects, increase dose by another half a tablet.
• You might not see any effects until you get to your effective dose. Everyone is different. 0.15 mg (one and a half tablets) a day was enough for our younger son, and on that dose, he was symptom-free about 90% of the time (his ME/CFS was milder than ours). For our older son, though, we patiently worked the dose up to 0.15 mg a day and saw absolutely no effect. We were ready to give up, but our doctor (the wonderful, now-retired Dr. David Bell) told us to stick with it and go up again, to 0.2 mg a day. Wow! It was like someone had flipped a switch! Our son suddenly felt better, had energy, could think more clearly, and was up off the couch, wanting to go to school and play with his friends. It was like a miracle ... and we almost gave up befiore getting to it. Later, as he grew, that dose no longer seemed effective, and we again increased up to 0.3 mg (3 tablets) a day, which did the trick. I have heard of doses as high as 0.4 mg a day, though most people (particularly kids) do well at 0.2 mg a day.
• Florinef can deplete potassium. Since Florinef helps the body hold onto more sodium, it can deplete potassium in the body. For this reason, Dr. Rowe recommends that anyone on Florinef also supplement with potassium chloride (KCl), at a dose of 10 meq KCl for every 0.1 mg of Florinef. So, with 0.3 mg of Florinef daily, my older son takes 30 meq of KCl. Note that this potassium can only be obtained with a prescription; you can not get high enough doses with over-the-counter supplements.

Tips for Trying Beta Blockers
Beta blockers work by blocking certain hormones, like adrenaline (which ones depend on which beta blocker you are taking) that can cause HR and BP to jump around. They will immediately reduce a high HR and are commonly used to treat high blood pressure, but I have found that a low dose beta blocker also helped to stabilize my BP and stop it from dropping when I am upright, so don't discount them if your BP is low or you have NMH. I wrote an entire blog post with information on and tips for finding the right beta blocker, so if you are trying them, please refer to that post for more detailed information.

Here are some quick highlights:

• Start with the lowest dose available. This is SO important because doctors often prescribe too high a dose, especially for ME/CFS patients. Too much beta blocker actually causes fatigue, and we certainly don't need that, right? So, start with the smallest dose available of the one you are trying--some patients even cut them in half to start. You can always increase if there is little effect. When I took my first pill of propranolol at the lowest dose available, my HR dropped 30 bpm within an hour! I was stunned...and excited!
• Avoid short-acting beta blockers. The short-acting ones have to be taken multiple times a day and wear off within 3-6 hours. Each time they wear off, your HR jumps up again. This can actually make your OI worse by introducing these spikes of high HR every time the meds wear off. I started on a short-acting one and found that I felt great while it was working but worse every time it wore off. Plus, I woke up every morning with none in my system and had to lie in bed for an hour waiting for it to kick in before I could get up. Instead, ask your doctor for 24-hour, long-acting type (some, like propranolol, come in both types).
• Take a long-acting beta blocker at bedtime. This accomplishes two things: it helps to prevent random bouts of tachycardia (high heart rate) during the night that can disrupt your sleep, and you wake up in the morning with it already in your system and working effectively. Switching from short-acting to long-acting was a huge improvement for me--I could wake up and jump out of bed and into the shower like a normal person! (yes, treating OI effectively will allow you to shower without crashing, too).
• You might not immediately "feel better." I noticed this myself. I was thrilled to see my HR drop to almost-normal levels and my BP stabilize, but I didn't immediately notice an improvement in my symptoms. However, if it is working (i.e. lowering your HR and/or stabilizing your BP), stick with it. I found that the symptom improvements came over time. By reducing OI, beta blockers (and other treatments) will automatically reduce exercise intolerance. With my HR at lower levels and my BP not plunging when I stood up, I could begin--very slowly--to do more and be more active. I began to do simple exercises, like leg lifts and core strengthening, while lying on the ground, carefully and while monitoring my heart rate. I began to walk, a bit more each week, slowly building up my strength and stamina. This is NOT Graded Exercise Therapy (GET) because you have treated the exercise intolerance first before even attempting to exercise. Improved muscle tone further reduces OI, and the improvements build. The positive results increased for me over time, like a domino effect (a positive one, for a change), as I was able to do more and be more active without crashing afterward.
• If at first you don't succeed, try, try again! There are almost 40 different beta blockers to choose from and some have entirely different mechanisms of action. Just trying one tells you nothing. If you have side effects at the lowest dose or it isn't effective for you even when you increase the dose, then try a different one. Those most commonly used for ME/CFS patients include long-acting propranolol, nadolol, and atenolol, but there are over 3 dozen others to choose from! Even for those in the same class, sometimes a patient will do better on one than another. There are also newer medications like ivabradine that are not technically beta blockers but can be used in similar ways to treat OI.

Patience and Persistence Pay Off!
This is the final thought I want to leave you with. I have lost count of how many times patients have told me "I tried treating OI, but it didn't help," and it turns out they just tried 1 beta blocker (probably at too high a dose) or only a low dose of Florinef. All treatments for OI can require a lot of trial and error to find just the right combination for each person because we are all different. Also, what I have outlined here are just two of many different treatments available for OI - they are the ones most commonly used but not the only options, by far. For more on treatment options, check out my ProHealth article, Treating Orthostatic Intolerance, and Dr. Rowe's summary brochure on OI. Both of these are also excellent for sharing with doctors because they are footnoted with plenty of scientific research to back them up.

Remember, keep trying! It is well worth the effort for the improvement in all symptoms that results.

Have you tried treating OI yet? Do you have any tips to share?

.    .    .    .    .    .    .