Month: April 2023

4.24.2023

POTS 3: Exercise

For the past two blog posts, we have been talking about Postural Orthostatic Tachycardia Syndrome (POTS). This is an imbalance of the involuntary nervous system which may occur in a number of different health situations, such as post COVID, but is common especially for patients with hypermobility spectrum disorder (HSD) and hypermobile Ehlers-Danlos Syndrome (hEDS).

POTS is the more severe end of a spectrum of symptoms all due to imbalances of the involuntary nervous system (the autonomic nervous system) called dysautonomia. The main symptoms of POTS are a racing heart that happens when the person stands up and feeling lightheaded, dizzy or just ill.

There are conservative and medication treatments for all of these types of POTS, and these are summarized in the last blog post (POTS 2). But one conservative treatment that research is showing to be beneficial for all of the types of POTS is exercise. {6}

Studies of patients with POTS have shown that cardiac atrophy, or weak heart, is a key component of the pathology. {4} Exercise improves but does not necessarily normalize heart function. {4} Exercise study participants lowered their resting heart rate and improved pain, sleep, overall energy, work capacity and several other indicators of quality of life. {3} More than one study has shown that exercise can help patients move fully into remission from POTS with 47-70% of participants going into remission in two studies.{3} One study found that exercise was better than the drug propranolol in helping the heart improve function. {1}

What Kind of Exercise?

Now we know from experience of working with patients that exercise is not always an easy sell for someone who already feels bad day to day. But we also know the incredible strength and courage of our patients. So, take a deep breath, and let’s ease in to talking about this.

Overall, we are talking about both aerobic conditioning exercises such as swimming, rowing, cycling, elliptical trainer, treadmill walking, or running. Additionally, in many cases resistance training, such as therapy bands, weight lifting or Pilates, is a very important part of the program. As we have talked about previously, POTS is a syndrome which represents the more severe end of dysautonomia, but even in this more sever end there is a spectrum of severity and so the exercise program itself must be tailored to fit.

Aerobic Exercise {2,3, 4, 5}

     What Does it Mean: Exercise Tailored to Fit?

There are a number of variables that are considered in designing a plan: intensity, frequency, and duration are the most common.

     Intensity

Before deciding the intensity of the exercise, how hard it is, the severity of the POTS case must be considered and the type of exercises chosen. For patients who are having severe symptoms, all exercises are started lying down. The more severe the case, the closer to lying flat the program is started. There are types of exercise bikes that can be done by someone lying supine. Swimming can also be done with the body in the horizontal position. After a number of months, these exercisers can progress to more upright exercises such as a recumbent bike or a rowing machine. And after additional time, upright exercises can be added such as an upright bike, elliptical trainer, walking, and running.

 For each individual exercise the cardiac intensity, how hard the heart is working, can be judged one of three ways: heart rate, rating of perceived exertion and, sometimes simply, a “talk test”.

     Judging Cardiac Intensity

For each person there is a maximum number of heart beats per minute which their heart is capable off, called heart rate max (HR Max). The number that is normally used as a reference in textbooks is 220 beats per minute. But this number gets lower with age. So, heart HR Max is estimated with the a formula: (220-persons age) +/- 5 beats per minute.

For example, a 30-year-old would have a theoretical HR Max of (220-30) = 190 +/- 5 beats. For exercise, a percentage of this is taken to determine how hard the person is working, usually between 60-85% of HR Max.

Another good indicator of intensity is to ask the exerciser to rate how hard they are working. This is called “rating of perceived exertion” (RPE) and while there are some different scales for this, a common one is the “Borg Scale” based on a number rating from 6-20.

RPE	How hard are you working?
6	No exertion; sitting, resting
7	Extremely light
8
9	Very light
10
11	Light
12
13	Somewhat Hard
14
15	Hard
16
17	Very Hard
18
19	Extremely hard
20	Maximal exertion

In Germany, for many years, there have been hiking clubs where they keep exercise at a moderate level by singing, or at least talking as they hike. If a person is having trouble singing while they exercise, they are working too hard. This is one kind of a “talk test”.

     Workout Intensity Levels

In the exercise programs for POTS there are three levels of intensity for workouts whether the exerciser is supine, sitting or upright: Base Pace, Maximal Steady State and Recovery.

Base Pace. This is the basic level of exercise and is normally preceded and followed with a 5 minute warm-up and a 5 minute cool down. Base pace intensity exercise just starts to make it challenging to talk, perceived exertion is rated between 13-15 (between somewhat hard and hard), and results in a heart rate around around 75% of HR Max.

Maximal Steady State. This is intense exercise usually undertaken later in the workout program when fitness levels are improving. During this level of intensity, the patient will not easily be able to talk or sing, rates their exertion between 16-18 (very hard) and results in heart rate around 85% of HR Max.

Recovery. Recovery workouts are done on days after a Maximal Steady State workout. At this level of intensity, the talk test is easily passed, rating of perceived exertion is between 6-12 (fairly easy) and results in heart rate around 60-65% HR Max.

     Frequency and Duration

How often (frequency) the person exercises and how long (duration) — meaning both the total program length and the daily workout length — will all vary depending on where the person is on the spectrum of POTS symptoms. Again, patients at the severe end of the spectrum will start all exercises with their body horizontal, such as lying down or swimming.

The Children’s Hospital of Philadelphia plan {5} describes a 5-month program. Exercisers will progress to more upright forms of workout at the end of the first or second month depending on their body. The researchers at Texas Health {2} describe a 3-month program. Exercisers with a mild or intermediate level of POTS may start all exercises sitting or sometimes even standing.

Initially, exercisers will participate in 3-4, 25-30 minute workouts per week at a Base Pace. Progression to the next level of workout may be started at the end of the first to third months of the program or even longer depending on how the person’s body responds. In the next harder level, exercisers will progress to one and then two Maximal Steady State workouts per week. This level of workout is always followed by a Recovery workout. By the end of program, exercisers will be performing 5-6 workouts per week of 45-60 minutes each.

As with all exercise, to attain the desired response from the body, persistence and consistency are needed. So, if more than two workouts are missed in a week, that week is repeated. If two weeks or more are missed, the entire program is restarted.

Resistance Training {2, 3, 4, 5}

Especially in cases where hypermobility is involved, resistance training is another part of the exercise program. Resistance training can and often does mean weight lifting, but may also include resistance bands and the resistance of the exercisers own body weight. Pilates exercise is a good example of this.

Exercisers are well advised to be mentally ready to be sore. This means sore muscles but should not include flaring up sensitive areas. To avoid this starting at the right level, which may be very light for some, and progressing gradually are important.

Especially for those with more severe POTS will begin lying down or sitting. Those with mild or intermediate symptoms may begin standing but should sit immediately after exercise. Workouts begin once per week for 15-20 minutes and gradually increase to twice per week building to 30-40 minute sessions. In selecting exercises, special emphasis is placed on lower extremity and core strengthening. It is important to take at least two days off between resistance workouts – this is the time when the body adapts and grows stronger.

As you can tell from reading all of this, the direction of a skilled therapist will be very important for this kind of treatment. Even though it can be challenging to find a therapist who is knowledgeable about hypermobility, dysautonomia and POTS, seeking one out can be very worthwhile.

We are still thinking about what to write about next in this blog series. Future topics may include mindfulness, digestive issues in HSD and hEDS and others.

Until then, cheers!

Zebbie

Zeborah Dazzle, PT, WWF

Spokes-Zebra and Patient Educator for Good Health Physical Therapy and Wellness.

Thanks to Dr. Mark Melecki, PT for his assistance in preparing this blog. (It is very challenging to type with hooves rather than fingers. Thanks Mark.)

References:

1. Fu, Q and Levine, BD, Exercise and Non-Pharmacological Treatment of POTS, Auton Neurosci. 2018, December; 215:20-27.
2. Fu, Q, et. al., Exercise Training Versus Propranolol in the Treatment of the Postural Orthostatic Tachycardia Syndrome, Hypertension Volume 58(2), August 2011; pages 167-175
3. Adger, K and Lynch, H., Exercise in the Management of Orthostatic Tachycardia Syndrome, Journal of Kinesiology and Wellness, 9(2), July 2020, pages 28-37
4. Shibata, S., et. al., Short-term Exercise Training Improves Cardiovascular Response to Exercise in the Postural Orthostatic Tachycardia Syndrome, J. Physiol 590.15 (2012) pages 3495-3505
5. Children’s Hospital of Philadelphia, Instructions for POTS Exercise Program, unpublished paper.
6. Zadourian, A., et. al., Postural Orthopedic Tachycardia Syndrome: Prevalence, Pathophysiology, and Management. Drugs (2018) 78:983-994
7. https://www.texashealth.org/ieem/Patient-Care/Syncope-and-Autonomic-Dysfunction-Clinic

4.17.23

In the previous post in this series, we started talking about POTS (postural orthostatic tachycardia syndrome). POTS is the more severe end of dysautonomia which means an imbalance of the involuntary nervous system.

As we talked about in the last post, dysautonomia and POTS are common in people with hypermobility spectrum disorder (HSD) and with hypermobile Ehlers-Danlos Syndrome (hEDS). There are physical mechanisms: 1. Neuropathic which is caused by damage to the small nerves in the legs that control the size of the small arteries; 2. Hypovolemic which means low blood volume caused by the kidneys getting rid of too many electrolytes and too much fluid; 3. Hyperadrenergic which means that the fight or flight side of the involuntary nervous system is too active for any one of a number of reasons.

Like many medical problems, dysautonomia exists on a spectrum. When we think about a spectrum, we can imagine a graph that charts severity of symptoms across all sufferers. A good illustration with this is the graph shown here. For all the patients with dysautonomia, some (on the left side of the graph) will have very few symptoms, many will have a moderate number of symptoms (center) and some will be on the severe end (right side of the curve).

All of these kinds of POTS have both conservative, non-drug, and medication treatments. In this post we would like to review some of the treatment approaches.

Conservative Treatment

For our purposes, conservative treatment of POTS is any kind of treatment that does not involve medications. And there are a number of tips and techniques to keep the problem at bay.

     Things to Avoid

There is no one uniform way of treating POTS. There are a number of medications that can make POTS worse. It is not the purpose of this article to direct medications. A list of some medications which can worsen symptoms is in Table 1. These should be discussed with the prescribing physician.

Table 1: Medication Types Which Can Worsen POTS {2} Alpha receptor blockers Angiotensin converting enzyme inhibitors Beta blockers Calcium channel blockers Diuretics Ganglionic blocking agents Hydralazine MAO inhibitors Nitrates Opiates Phenothiazines Sildenafil citrate Tricyclic antidepressants Oral contraceptives containing drosperinone Norepinephrine transporter inhibitors Serotonin reuptake inhibitors

People with POTS are also advised to avoid alcohol and extreme heat.

     Hydration and Electrolytes

Patients with POTS and other levels of dysautonomia frequently have an imbalance in the hormonal system the kidneys use to determine how much water and how much of the body’s electrolytes to release in the urine. So, these people are chronically dehydrated and low on minerals. The answer is to drink and supplement electrolytes.

Recommended levels of fluid intake per day are between 2-3 liters {1,2,3}. This translates into 68-101 ounces, or 8.5-13 cups, or 0.5-0.8 gallons.

Recommendations in the literature for salt intake vary from 8-10 grams {1} to 10-12 grams {2, 3}. We have found that these numbers are individual and can vary. One way to manage salt intake when seeking to improve symptoms of POTS is to start with about 3gm of added salt per day and increase by 1 gram per day up to a maximum of 12 gm per day depending on symptoms. In other words, if the person feels best at 9 gm/ day, that should be their intake unless symptoms change. Doctors can also test for sodium levels by blood test which can give a measure of how well supplementation is working.

All of the references we have found so far describe electrolyte supplementation in terms of salt intake. If taking an electrolyte supplement such as Nuun, Pedialyte or Gatorade, the number of milligrams of sodium can be used as a reference or follow the gradual increase approach described above. (Remember, 1000mg = 1 gm)

     Elevation of Head

There is some evidence that sleeping with the head of the bed raised by 4-6 inches may help with POTS symptoms. {3}

     Muscle Squeezes

Since for many with POTS the pooling of blood in the lower part of the body can lead to increased pounding, feeling ill and/ or dizziness when the person changes positions, movements or muscle contractions that help move the blood up out of the legs are a part of avoiding symptoms with position changes. These can include exercises done lying down before sitting up, exercises done sitting before standing, and exercises to be done while standing. A physical therapist can prescribe specific exercises.

     Compression Garments

Persons with the Neuropathic and/ or Hypovolemic types of POTS generally benefit from wearing compression garments. Here again, depending on the severity of symptoms, different levels and lengths of garments may be useful. For those with more severe cases of POTS, a compression garment worn over the entire lower extremity and including the abdomen is recommended. {3} Here again, for stronger POTS symptoms, garments that give compression in the 30-40 mmHg (millimeters of mercury) range are suggested {3}. We have had many patients that get very good benefit from calf high or thigh high compression. And too, we have had many patients who get good relief from wearing compression garments giving 20-30 mmHg. Experimentation with different garments is key.

   Exercise

Regardless of the type of POTS, exercise is a recommended non-medication form of treatment {1,2,3}. Current research strongly suggests that patients with POTS have some degree of heart muscle atrophy, weakening of the heart. This can be improved with a carefully structured exercise program. We will explore this in much more depth in the third and final blog post, but meanwhile here is an interesting tidbit. The original exercise approaches for people with POTS were modeled after exercise programs developed for astronauts weakened by prolonged weightlessness in space, such as the crew of the space station. These approaches have also been modified for use with people who have been confined to bed for a sustained period. More later.

Medical Treatments with Medications

While non-drug approaches to treatment form a very important foundation for treatment, many patients whose symptoms are not well controlled using conservative approaches alone may require supplements and/ or medications.      

     Neuropathic POTS

In this kind of POTS, dilated blood vessels in the lower extremities lead to pooling of blood there with less blood available for the brain. Medications used to counteract this can include the following. See Table 2.

Table 2. Medications Targeting Vasoconstriction { 1, 2} Midodrine Droxidopa Octreotide Dextroamphetamine Methylphenidate

     Hypovolemic POTS

If increased fluid intake and electrolyte intake are not adequate, there are several medications which can help to increase fluid volume in the body. See Table 3

Table 3 Medications to Improve Fluid Retention {1,2} Fludrocortisone Desmopressin Erythropoietin

   Hyperadrenergic POTS

Regulating the sympathetic nervous system can be much more complicated than the other two types of POTS above. The medications are often not as well tolerated and may have more unwanted side effects. For people with high resting heart rates, Propranalol is the single most common medication prescribed. People taking any of these should work closely with their physician to achieve the desired results. See Table 4.

Table 4 Medications for Sympathetic Support {1,2} Propranolol Ivbradine Clonidine A-Methyldopa Pyridostigmine

      MCAS and POTS

Some patients with POTS may have symptoms largely caused by mast cell activation disorder {5}. Simply said, mast cells are naturally occurring cells in the body which cause inflammation as part of the body’s defense system. These can get overly sensitive and release too many inflammatory chemicals. Some of these chemicals, like histamine, may cause opening of blood vessels in the legs in some patients with hyperadrenergic POTS. We hope to do a series of blog posts on mast cell activation later this year.

In the third and final post of this series, we will look at exercise as a form of treatment for POTS. As you might imagine, we PTs have some thoughts about this and there is a good deal of information.

Until then, cheers!

Zebbie

Zeborah Dazzle, PT, WWF

Spokes-Zebra and Patient Educator for Good Health Physical Therapy and Wellness.

Thanks to Dr. Mark Melecki, PT for his assistance in preparing this blog. (It is very challenging to type with hooves rather than fingers. Thanks Mark.)

References:

1. Raj, S., Postural Tachycardia Syndrome (POTS), Circulation 2013; 127: 2336-2342.
2. Zadourian, A., et al, Postural Orthostatic Tachycardia Syndrome: Prevalence, Pathophysiology, and Management, Drugs (2018) 78: 983-994
3. Fu, Q. and Levine, B., Exercise and Non-pharmacological Treatment of POTS, Auton Neurosci. 2018 December; 215:20-27
4. Olshanksy, B., et al, Postural Orthostatic Tachycardia Syndrome (POTS): A critical assessment. Prog Cardiovasc Dis. 2020; 63(3): 263-270
5. Shibao, C., et al, Hyperadrenergic Postural Tachycardia Syndrome in Mast Cell Activation Disorders, Hypertension Volume 45, Issue 3, 1 March 2005; 385-390