POTS Treatment in MECFS
Basics
Postural Orthostatic Tachycardia Syndrome aka POTS is a form of dysautonomia that causes tachycardia (high heart rate) in response to postural changes, in particular, standing and sitting upright.
While POTS is a specific diagnosis, many forms of dysautonomia exist in MECFS. In addition to the heart rate and blood pressure changes in POTS patients dysautonomia affects all parts of the nervous system we control unconsciously like temperature and digestion.
Estimates for POTS prevalence in ME vary wildly. But Bateman Homes NASA Lean study estimates it to be around 40%. At least 4× higher than the general population.
Note: There are many forms of orthostatic intolerance in ME patients. If you do not have POTS but do not tolerate standing you should be investigated for other forms of OI.
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Symptoms
The primary symptoms of POTS are a rapid heartbeat, dizziness and lightheadedness of fainting upon standing (or sitting in severe patients).
However, because POTS is a form of dysautonomia it can also be responsible for multisystemic symptoms such as
Sleep disturbance
Lethargy
Systemic pain
Brain Fog
Bladder symptoms
Gastrointestinal disturbance
Headache and neck and shoulder pain
Heightened sensitivity to light, noise, smell and taste
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Diagnosis
POTS is diagnosed based on changes in hr and blood pressure. You may be able to test yourself for POTS at home prior to seeking a diagnosis using the NASA lean test.
To qualify for a diagnosis of POTS when you stand you must have
A sustained heart rate rise of ≥ 30 bpm
An absence of the alternative diagnosis of orthostatic hypotension (defined as a ≥20/10 mmHg blood pressure drop)
BP in POTS patients most often spikes briefly and then drops over time leading to low BP and potential fainting (hypopots) but in some patients may continue to rise to dangerous levels (hyperpots)
Additionally, there should be over 3 months of orthostatic intolerance symptoms (dizziness, fainting) that have no other known cause. You do not have to faint to be diagnosed with POTS!
A tilt table test is the most common way to diagnose POTS. Standard blood tests, an ECG, an echocardiogram and 24 hr Holter monitor may be used to help rule out other conditions and confirm diagnosis.
Note: this section was edited on Jan 2 2023 to provide more detailed BP information and clarification on fainting.
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Treatment
Treating POTS is normally done in 2 ways.
Lifestyle management:
-Increasing salt to 10-12g and water to 2-3 liters (must monitor BP)
-Use of compression stockings
-Avoiding heat
-Graded exercise*
-Dietician intervention if gastro symptoms are severe
Medication:
-Propanolol/ivabradine (Lower hr)
-Fludrocortisone (Increase blood volume)
-Midodrine (Vasoconstriction)
-Simulants (Fatigue / concentration)*
Additionally, IV fluids are a powerful intervention for POTS particularly when symptoms are severe.
*These treatments can be quite harmful in ME. See next slide for warnings.
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Complications & Considerations of Treatment in ME
Ivabradine may be preferable in pwME because it increases stroke volume and ME patients often have low brain perfusion.
Propanolol may be useful in patients with comorbid migraine as it is also a migraine preventative. However, beta-blockers are contraindicated in MCAS patients as they are histamine liberators and can block the effectiveness of adrenaline in anaphylaxis.
Stimulant medication used to fight fatigue and brain fog in POTS may be dangerous in ME patients as it can cause them to overexert. Stimulants can cause ME progression from mild to severe.
GET is an effective treatment of POTS but is also known to cause decline in ME patients. Careful judgement must be used when prescribing any form of PT and judged on an individual basis with the patient being well informed on PEM risk.
It is important to know that deconditioning is not the cause of OI in MECFS patients* In fact, ME patients often report much worse POTS following exertion.
*https://translational-medicine.biomedcentral.com/articles/10.1186/s12967-021-02819-0
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Adaptive Options
In patients with ME, the tachycardia caused by POTS is often interpreted as exertion and can cause PEM. Therefore, taking steps to avoid upright posture can be a way for ME patients with POTS to reduce exertion.
For mild to moderate patients this may look like:
-Using a shower stool
-Placing seating around the house and using when cooking, cleaning, etc.
-Using a cane with seat attachment
-Using a wheelchair or walker with seat
For severe and very severe patients this may look like:
-Using a tilt in space wheelchair and/or shower chair
-Using a bed that can assist sitting at various angles and raise legs
-Using a bath lift
-Being transported horizontally in vehicles by creative seating arrangement or patient transport
Regardless of severity remember that if you feel you are about to pass out you should always seek to get to a horizontal position as quickly as possible regardless of location. (Yes sometimes this means sitting and lying down in weird public places, but its better than fainting)
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Benefits and Risks of IV Fluids
IV fluids are one of the most effective treatments for POTS, particularly when it comes to immediate relief. Many pwME also report substantial relief from IV fluids not obtained from oral fluids possibly due to the chronically low blood volume ME causes.
While IV fluids are a very safe intervention for any given administration, the frequency with which POTS patients often need IV fluids can lead to a large cumulative risk of IV infection and sepsis. This can be deadly.
Therefore, while IV fluids are an excellent tool for dealing with acute crises of POTS they are less ideal as a management option.
In addition to long-term procurement of IV fluids through a port-a-cath or central line, increasing fluid intake through feeding tubes may also be a viable option that can offer a lower-risk alternative to increase hydration.
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Very Severe ME and POTS
Patients with very severe ME often have severe POTS as well. The extreme level of illness in these patients requires special accommodation.
Tilt table testing and in office visits are often not appropriate for severe patients as they may cause significant PEM and transportation difficulties. A home holter monitor can be an easy remote way of gathering data for diagnosis, especially in combination with a home visit. Telehealth should be used for follow-up.
Because of their severity of illness, severe patients with ME should be strongly considered for more aggressive treatment of POTS including central line insertion and IV fluids. Feeding tubes can also be used to give additional saline.
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Harm Reduction
Harm reduction is essential when treating POTS in severe ME. Investigations should be done using only activities tolerable to the patient. Due to the severity of POTS simply sitting up often provides sufficient hr and blood pressure data to confirm a diagnosis.
It should also be remembered that patients with severe ME must pace very aggressively to avoid PEM. Therefore they may keep their heart rate in a very narrow window for most of the day. However, they will experience extremely disproportionate hr for the activities they are doing.
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Postural Orthostatic Tachycardia Syndrome aka POTS is one of the most common comorbidities of Myalgic Encephalomyelitis and while it is a chronic illness unlike MECFS it is very treatable!
POTS is a form of dysautonomia in particular orthostatic intolerance. In other words, it causes symptoms when you are upright for too long.
While syncope or fainting is often associated with POTS you do not have to faint to have POTS. What you do need is a sustained increase in hr of over 30bpm in response to standing (or sitting up if bedbound) as well as a lack of a substantial drop in bp. (If your blood pressure drops a lot when you stand you have a different form for OI called orthostatic hypotension)
It is important to know that POTS in ME is not caused by deconditioning. It is caused by autonomic dysfunction in the nervous system. While deconditioning may occur as a result of severe ME patients becoming bedbound and this may worsen their POTS and OI it is not the root cause.
In many POTS patients, graded exercise therapy is essential to recovery. But in severe ME patients this is extremely dangerous. Therefore, it is crucial that POTS in ME / CFS patients be treated differently to patients with primary POTS or POTS related to Fibromyalgia or EDS.
In very Severe ME patients simply testing for POTS may require accommodation. The ability to stand for a NASA lean test may not be possible and tilt table testing could lead to severe PEM and a permanent relapse. Therefore, at home holter monitoring and taking hr and blood pressure readings between lying down and sitting up should be considered. In very severe patients these readings are likely to be sufficient to demonstrate POTS.
Finally, IV fluids should be more strongly considered in patients who are fully bedbound with severe ME. It has been shown that insufficient brain perfusion is prevalent in severe ME even in absence of POTS as is low blood volume. Thus fluids can be a life-changing intervention for these patients despite the high risk of infection.
