What Is POTS??

What Is POTS??

Wednesday, September 21, 2016

Morbidity--Can You Die From POTS or Associated Conditions?

This post is going to be a hard one for me to write and probably a hard one for some people to read. The possibility or your own mortality is a subject most people try not to think about.

The reason I am writing this is because I had some people get angry at me in a facebook group for suggesting that you could die from POTS.

While it is true that POTS itself doesn't kill people, there is a series of possible events that can ultimately result in death. And I know it to be a fact because my husband died from it. This is what happens:

Hypotension>hypoperfusion>syncope>seizure>cardiac event.
 And it can progress quickly.
My husband went outside, got too hot came in and sat down and a few minutes later, about five, he was in cardiac arrest. And he was never revived by either the EMTs or the ER doctors. Officially, his death certificate says cardiac arrest. But he passed out, had a seizure and then his heart stopped.

I am going to try and explain as well as I can how that is possible.

MASTOCYTOSIS
Here is an article about a man who had a heart attack because he had mastocytosis, which caused him to have syncope, and then the heart attack. Shortness of breath, syncope, and cardiac arrest caused by systemic mastocytosis

Mast cells secrete histamines that cause rashes and hives in an allergic reaction. They also play a role in wound healing and in the immune system.

When someone's mast cells cause them problems, they can have abdominal pain, cramping, diarrhea, flushing, itching, wheezing, coughing, lightheadedness and potential problems with “brain fog” or other difficulties with memory. And there are some patients who have POTS and MCAS/Mast Cell Activation Syndrome. You can read more about POTS and MCAS here: A Tale of Two Syndromes – POTS and MCAS Mastocytosis is when too many of those mast cells remain in the tissues of the body.

 So there is one way POTS can cause a cardiac event, even if it is indirectly because of MCAS.

“Syncope is defined as a sudden loss of consciousness with loss of stature. It is due to the sudden decrease of oxygenated blood perfusion to the brain. If the cause of cerebral hypoperfusion resolves within 1-2 minutes, the consciousness returns. It is not uncommon to observe convulsions during the episode of syncope, which is attributed to the sudden lack of oxygen and glucose to neurons resulting in disorganized neuronal activity. If the cause of cerebral hypoperfusion persists and the individual does not get prompt medical help with cardiopulmonary resuscitation (CPR), permanent damage may occur.” https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c08.html

“It is not uncommon that due to the loss of cerebral perfusion, convulsions can occur for a few seconds or even up to a minute.”

So this says that regardless of what causes the syncope if you don't have prompt medical attention it can progress to the point of needing CPR and permanent damage. Seizures can actually last longer than it says above. “Although usually brief, the convulsions associated with syncope can be quite severe and prolonged.” http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1157.1997.tb01086.x/pdf

Convulsions or seizures in relation to syncope are complicated. Seizure activity in the brain can cause hypotension and syncope. But hypotension and syncope can cause seizures. “While neurally mediated syncope may mimic seizure-like activity, it should also be acknowledged that seizure foci in certain cerebral sites (particularly the temporal lobe) may be the source of apparent neurally mediated syncopal events. Localized seizure activity may initiate the reflex arc previously described, leading to hypotension and bradycardia.” The pathophysiology of common causes of syncope

Bradycardia can happen as a result of a seizure. http://www.seizure-journal.com/article/S1059-1311(14)00070-3/fulltext



"Convulsions suggest prolonged or severe brain hypoperfusion. Shakiness, which can accompany hyperadrenergic activity, can simulate seizure. The duration of loss of consciousness as well as the position of the patient during loss of consciousness is important information. Urinary incontinence and tongue biting during a spell favor a seizure event.

Helping the patient sit or lie down quickly and raising the legs above the heart level permit faster recovery in patients with a typical reflex postural hypotension event. Physicians should check the pulse for amplitude and rhythm. When a patient recovers the acute event, ambulation should be resumed with care because recurrence of hypotension may be inevitable at this stage due to circulatory instability. Oral hydration with salty fluids usually is helpful in the early recovery phase if the patient has no known previous history of heart disease. Serious arrhythmogenic events, coronary insufficiency syndromes, pulmonary embolism, strokes or transient ischemic attacks, and blood loss must be recognized for proper immediate medical care. Injuries sustained during a sudden fall require immediate attention." Cleveland Clinic--Syncope

Asystole is a cardiac arrest rhythm in which there is no discernible electrical activity on the ECG monitor. Asystole is sometimes referred to as a “flat line.”

SUDEP sudden unexpected death in epilepsy.

Apnea is when you stop breathing.

“A principal finding of this study was that apnea and asystole occurred in all SUDEP cases in the early postictal phase after generalized tonic–clonic seizures.” The same article says that usually the asystole or flat-line stops when hypoperfusion and anoxia return to normal. This just means that during a seizure blood flow and oxygen are being interupted but for some people it doesn't return to normal and they die.

“ In conclusion, this patient most probably presented TLOC(total loss of consciousness) episodes due to both reflex syncope and temporal lobe seizures (without a clearly proven link between seizures and syncope).” So you can have both syncope and seizures. The article also says that if seizures are causing the asystole/heart stopping, that a pacemaker is a good treatment option. It was written in 2014. And my husband never lived long enough for us to learn about this. http://www.seizure-journal.com/article/S1059-1311(14)00070-3/fulltext I found a cardiology journal that says the same thing. “Ictal bradycardia/asystole syndrome is mostly related to temporal lobe epilepsy, predominantly in male patients aged older than 50 [44–49]. The diagnosis is based on simultaneous EEG and electrocardiography (ECG) recording with electrographic seizure activity preceding severe bradycardia/asystole or AV-block. A combination of antiepileptic treatment and pacemaker therapy might be required in such patients.” Is it possible to accurately differentiate neurocardiogenic syncope from epilepsy?

“Although convulsive syncope or anoxic seizures are not uncommon, actual epileptic seizures induced by vasovagalor similar forms of syncope have only rarely been reported.” RARE BUT IT DOES HAPPEN. “About 30 s elapsed between the syncope and the onset of the seizure discharge.”
“Of 13 previously reported patients with epileptic seizures induced by syncope, only one had a history of epilepsy with an abnormal interictal EEG.” “it has been speculated by some that a predisposition to generalized epilepsy exists in these patients and that the hypoxic or ischemic syncopal episode might further lower the seizure threshold, resulting in the observed epileptic seizures in these susceptible patients.” http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1157.1997.tb01086.x/pdf

hypotension >Bradycardia>hypoperfusion>sycope>seizure>apnea and asystole/flat-line
seizure>hypotension and bradycardia>hypoperfusion>syncope
That makes two.

Given that one can cause the other, it could be a vicious cycle.


This is another scenario.


This medical paper says that orthostatic hypotension and bradycardia can cause syncope and Atrial fibrillation. “ This is the first report of a patient with persistent atrial fibrillation associated with syncope caused by orthostatic hypotension and bradycardia.” Persistent atrial fibrillation associated with syncope due to orthostatic hypotension: a case report.

This is another article that says that a man had a tilt table test where they induced Afib and that caused him to have syncope. And the next day they did a tilt table test and he had syncope that caused Afib.New England Journal of Medicine--Neurally Mediated Syncope and Atrial Fibrillation


And Atrial Fibrillation can cause sudden cardiac death. “Incident AF is associated with an increased risk of SCD.” Atrial fibrillation and the risk of sudden cardiac death: the atherosclerosis risk in communities study and cardiovascular health study.


hypotension>bradycardia>hypoperfusion>syncope>Afib>Sudden Cardiac Death

And one of the symptoms of POTS is syncope. So, therefore, it follows that the T in POTS or tachycardia causes syncope and therefore can cause Afib and Sudden Cardiac Death.

“The postural tachycardia syndrome (POTS) also presents clinically with symptoms of cerebral hypoperfusion.” Orthostatic Hypertension: When Pressor Reflexes Overcompensate


“This supports the hypothesis that repetitive stable VT(ventricular tachycardia) can play a role in the pathophysiology of cerebrovascular insufficiency.”


“cardiac syncope a variable amount of hemodynamic instability results in cerebral hypoperfusion”

“Systemic hypotension is a common postoperative complication that can cause hypoperfusion and inadequate delivery of oxygen and substrates to organ systems The systemic blood pressure at which the risk of complication increases depends in part on the preoperative blood pressure.”POSTOPERATIVE HYPOTENSION  What this article is getting at is that POTS and orthostatic hypotension patients can go into shock after surgery easier than other people. 

hypotension>hypoperfusion>

Hypoperfusion>syncope
“ These findings suggest that cerebral hypoperfusion, such as with cerebral vasospasms, before the onset of bradycardia might be involved in the mechanism of neurally mediated syncope in patients with an aura.” Possible involvement of cerebral hypoperfusion as trigger of neurally-mediated vasovagal syncope.


syncope>hypoperfusion

hypotension>Afib

“The study suggests that a bout of orthostatic hypotension — a steep blood pressure drop that occurs when a person stands up after a period of lying down — appears to be associated with an overall 40 percent increase in the risk of developing atrial fibrillation over the following two decades.”

Although the above links are referring to orthostatic tachycardia, POTS also is associated with hypoperfusion. Decreased upright cerebral blood flow and cerebral autoregulation in normocapnic postural tachycardia syndrome

"POTS manifests with symptoms of cerebral hypoperfusion and excessive sympathoexcitation.


"The mechanism of POTS is still undergoing a lot of investigation and may be multifactorial. Therefore, any possible overlap with atrial fibrillation is still somewhat unclear and may warrant further study."

Tachycardia>syncope>Afib>Sudden Cardiac Death

It also isn't a far stretch to say:

Tachycardia>syncope>seizure>apnea and asystole/flat-line

Here Is Another Scenario


An enlarged heart isn’t a condition in itself, but a symptom of an underlying problem that is causing the heart to work harder than normal.

“However, if you experience unpleasant symptoms or a permanently increased heart rate is risking heart enlargement, your doctor may recommend treatment with medication or catheter ablation.”Supraventricular Tachycardia

“Tachycardia-induced cardiomyopathy develops slowly and appears reversible by left ventricular ejection fraction improvement, but recurrent tachycardia causes rapid decline in left ventricular function and development of heart failure. Sudden death is possible.” Cardiomyopathy is when the heart muscle becomes enlarged, thick, or rigid. AHA JOURNAL--Heart Failure and Sudden Death in Patients With Tachycardia-Induced Cardiomyopathy and Recurrent Tachycardia

“Some forms of enlarged heart can lead to disruptions in your heart's beating rhythm. Heart rhythms too slow to move blood or too fast to allow the heart to beat properly can result in fainting or, in some cases, cardiac arrest or sudden death.” Mayo Clinic--Complications of Enlarged Heart

So to sum up, tachycardia can cause an enlarged heart which can cause cardiac arrest or sudden death.


A patient can have both seizures and syncope.

Seizures>bradycardia/asystole or AV-block

hypotension >Bradycardia>hypoperfusion>sycope>seizure>apnea and asystole/flat-line SUDEP

seizure>hypotension and bradycardia>hypoperfusion>syncope

orthostatic hypotension and bradycardia can cause syncope and Atrial fibrillation.

hypotension>bradycardia>hypoperfusion>syncope>Afib>Sudden Cardiac Death

Afib>syncope

Tachycardia>syncope>Afib>Sudden Cardiac Death

Tachycardia>hypoperfusion>

Hypoperfusion>bradycaria>syncope

Tachycardia>syncope>seizure>apnea and asystole/flat-line


If those aren't enough for you, here are some more. 


"There are also a number of symptoms and signs that may indicate that a person is at increased risk for SCD. These include:
  • An abnormal heart rate or rhythm (arrhythmia) of unknown cause
  • An unusually rapid heart rate (tachycardia) that comes and goes, even when the person is at rest
  • Episodes of fainting (called syncope) of unknown cause"

RISKS DURING PREGNANCY

There has been debate over whether or not POTS patients should opt for a caesarean section or a vaginal birth.  Due to the physical stress of a vaginal birth, some patients have gone into cardiac arrest (or near cardiac arrest) while delivering.  The reason to opt for a C-section would be to avoid this incidence.  However, a C-section is major surgery and must be discussed thoroughly with a high-risk obstetrician.   Also, some patients may have problems receiving anesthesia which is administered during a C-section and sometimes during vaginal birth (also known as an epidural).  Both of these aspects must be thoroughly reviewed prior to delivery.How does POTS affect pregnancy and labor?



PROBLEMS DURING HEAD UP TILT TABLE TEST
HUT testing is generally safe, but there have been occasional reports of coronary vasospasm, chest pain, hypertensive crisis and tachyarrhythmia. The most frequent adverse effects are hemodynamic changes, such as hypotension, tachycardia or bradycardia associated with orthostatic intolerance, presyncope or syncope. It is noteworthy that patients with neurocardiogenic syncope may rarely experience asystole (defined as ventricular pause of more than 5 seconds) or complete atrioventricular block during HUT testing. Lacroix and colleagues reported 10 asystolic reactions (6%) (average duration 12 seconds) among 179 patients investigated for neurocardiogenic syncope; 8 patients needed cardiopulmonary resuscitation for 20 to 30 seconds. Dhala and associates reported 19 asystolic reactions (9%) among 209 patients with suspected neurocardiogenic syncope and 3 asystolic responses (4%) among 75 healthy control subjects during HUT testing without pharmacologic stimulation. These subjects did not show a worse outcome than their nonasystolic counterparts during follow-up. The fainting patient: value of the head-upright tilt-table test in adult patients with orthostatic intolerance


Autonomic nerve disorders (dysautonomia) refer to disorders of autonomic nervous system (ANS) function. Dysautonomia is a general term used to describe a breakdown or abnormal function of the ANS. 

POTS and Orthostatic hypotension are dysautonomias.

About half of patients with POTS have a restricted autonomic neuropathy with a length-dependent distribution of neuropathy.

"While diabetes is generally the most common cause of autonomic neuropathy, other health conditions — even an infection — may be to blame." http://www.mayoclinic.org/diseases-conditions/autonomic-neuropathy/basics/definition/con-20029053


(This is associated with diabetes but it is possible that other people could have it: see above.)

"Cardiac autonomic neuropathy (CAN) represents a serious complication as it carries an approximately
five-fold risk of mortality in patients with diabetes just as in those with chronic liver diseases. The high
mortality rate may be related to silent myocardial infarction, cardiac arrhythmias, cardiovascular and
cardiorespiratory instability and to other causes not yet explained. Resting tachycardia due to parasympathetic damage may represent one of the earliest signs. Typical findings referring to autonomic dysfunction may include exercise intolerance, orthostatic hypotension
and cardiac dysfunction to rest or exercise. Severe autonomic neuropathy may be responsible for
spontaneous respiratory arrest and unexplained sudden death." Autonomic neuropathy: a marker of cardiovascular risk


"Resting tachycardia due to parasympathetic damage may represent one of the earliest signs of CAN. Experiences from large epidemiological studies indicate that tachycardia of any origin is a major risk factor for cardiovascular and non-cardiovascular death.17 The heart rate-mortality association is observed at any age."

"CAN is associated with a high risk of unexpected and sudden death, possibly related to silent myocardial ischaemia/infarction, cardiac arrhythmias and hypoxia.15 Cardiorespiratory arrests during
or right after anaesthesia have been described."

. Possible factors associated with high mortality and sudden death due to autonomic neuropathy 
● Silent myocardial ischaemia/infarction 
● Cardiorespiratory arrest/increased perioperative and peri-intubation risk 
● Resting tachycardia 
● Ventricular arrhythmias/prolongation of the QT interval 
● Hypertension 
● Orthostatic hypotension 
● Flattening of the nocturnal reduction of blood pressure and heart rate (‘non-dipper’ phenomenon) 
● Exaggerated blood pressure responses with supine position and exercise 
● Abnormal diastolic/systolic left ventricular function 
● Poor exercise tolerance 
● Impaired cardiovascular responsiveness 
● Heat intolerance due to defective sympathetic thermoregulation 
● Susceptibility to foot ulcers and amputations due to arteriovenous shunting and sudomotor dysfunction 
● Hypoglycaemia unawareness (?) 
● Increased risk of severe hypoglycaemia 
● Obstructive sleep apnoea syndrome

Autonomic neuropathy is common in Nigerian patients with non-diabetic Chronic Renal Failure.http://www.ncbi.nlm.nih.gov/pubmed/15171518

Renal Failure isn't something that is associated with POTS but autonomic neuropathy is. 
"The role of obesity is supported by the high prevalence of cardiac autonomic dysfunction in non-diabetic obese people,"Diabetic Cardiovascular Autonomic Neuropathy

"Diabetes can produce the symptoms of POTS (Llamas, Garcia, Gaos, Jimenez, Villavicencio, Cueto & Arriaga, 1985). There are different types of diabetes, including diabetes insipidus, that are associated with POTS symptoms"http://www.dinet.org/index.php/information-resources/pots-place/pots-causes

"POTS is also often classified as primary or secondary.  An example of an 2003 classification of postural tachycardia syndrome by Dr. Grubb (18):

Primary forms: Partial dysautonomic, Immune mediated pathogenesis, Adolescence, Hyperadrenergic state

Secondary forms: Diabetes mellitus, Amyloidosis, Heavy metal poisoning, Sjogren syndrome, Hypermobility syndrome, Paraneoplastic syndrome"

"Both Peripheral (PN), and specifically Small Fiber Neuropathy (known as SFN, a type of PN), have been associated with Autonomic Neuropathy, POTS, and other diseases that cause POTS, such as Diabetes and Sjogrens" what-is-causing-your-pots-and-why-it-is


You can read more on autonomic neuropathy here: Autonomic Neuropathy



Sudden Cardiac Death


Autonomic Neuropathy Causes 2.5 Times a Risk of Sudden Death













Sunday, September 18, 2016

What Is Dysautonomia?

Autonomic Neuropathy Treatment & Management


Autonomic Neuropathy Treatment & Management--Medical Paper

If You go to the above link and create a sign in password this is a great medical paper/article. 




Blood Tests and Labs for Dysautonomia





Lest you think I suddenly became really smart, I got this information from the following: Medscape: Autonomic Neuropathy Workup, Medical Paper

You need to have a complete blood count, basic metabolic panel, liver function testing, and immunoelectrophoresis. More specific testing should be based on the patient’s history of other medical conditions.

Special situations

Depending on what the results of the first blood tests and autonomic tests are, there are more tests that may be ran.


Oral glucose tolerance test to check for diabetes mellitus, if an initial serum glucose level is normal or nondiagnostic.

They can test for SS-A and SS-B if they think you may have Sjögren's syndrome.
Anti-ganglionic acetylcholine receptor (AChR) autoantibodies if the onset was acute to subacute in nature.

Specific genetic tests for the familial dysautonomia can be done.

Specific tests for infections, inflammatory, autoimmune, and paraneoplastic causes can be ordered based upon the history and physical examination.

Measurement of basal plasma norepinephrine levels can be useful in specific forms of autonomic neuropathy. In pandysautonomia, basal norepinephrine levels are low and do not rise on head-up tilt table testing. Following an overnight supine position, low norepinephrine levels can be found in patients with POTS.

A history of neuropathy, mental status changes, and abdominal pain should prompt the physician to evaluate the patient for acute intermittent porphyria. In cases of suspected porphyria, high levels of porphobilinogen and delta-aminolevulinic acid can be found in urine during acute episodes.
Evaluation of cerebrospinal fluid (CSF) via lumbar puncture can be useful in specific cases.

In pandysautonomia, CSF protein is elevated, as is CSF enolase, which may indicate damage to the dorsal root ganglia. (Acute pandysautonomia is a rare disease defined as acute widespread and severe sympathetic and parasympathetic failure and sparing of somatic nerve fibers. The causes of this syndrome are often an autoimmune disease leading to autonomic ganglionopathy.)

In HIV or AIDS, the CSF may demonstrate an elevated protein as well as pleocytosis.

Paraneoplastic varieties of autonomic neuropathies also tend to show an inflammatory picture in the CSF. However, abnormal CSF protein is not specific for autoimmune, inflammatory, or infectious causes of autonomic neuropathy.

Imaging tests

In addition to CT and MRIs,

SPECT and PET scanning may identify cardiac sympathetic dysfunction in both type I and type II diabetes mellitus.

The pattern of sympathetic disturbances tends to be heterogeneous, with denervation affecting mainly the posterior myocardial region, whereas focal hyperinnervation can be observed of the proximal segment.

Autonomic testing

Autonomic testing  should be done to find out which part of the autonomic nervous system is effected and how severely.

Tilt table testing to test adrenergic vasomotor function and cardiac sympathetic function.

Cardiac response to deep breathing and R-R interval to evaluate cardiovagal functions.

Cardiac response to Valsalva maneuvers to test parasympathetic innervation to the heart.

Quantitative Sudomotor Axon Reflex Testing (QSART) to evaluate the postganglionic segment of the thermoregulatory pathway. Four regions are tested: forearm, proximal leg, distal leg, and dorsum of the foot. Electrical stimulation (iontophoresis) is applied to the skin, and the volume of sweat produced can be measured.

Nerve conductions studies and electromyography
Findings on nerve conduction studies (NCS) and electromyography (EMG) can be normal in pure autonomic neuropathies because the involved fibers are small myelinated and unmyelinated fibers, which cannot be assessed with NCS or EMG.

Findings on nerve conduction studies (NCS) and electromyography (EMG) can be normal in pure autonomic neuropathies because the involved fibers are small myelinated and unmyelinated fibers, which cannot be assessed with NCS or EMG.

In autonomic neuropathies with concomitant sensory neuropathy, absence of sensory potentials may occur.

In autonomic neuropathies with concomitant sensorimotor neuropathy, marked loss of motor and sensory potentials is noted.

In cases of suspected neuromuscular transmission defect, such as with botulism or LEMS, a typical electrophysiologic pattern of low-amplitude compound muscle action potentials increasing with high-frequency repetitive stimulation is characteristic of a presynaptic neuromuscular defect.

Specialized studies

In Sjögren syndrome, results of the Schirmer test with a rose-Bengal eye stain, as well as lip biopsy to identify chronic sialoadenitis, can be diagnostic.

Postprandial blood pressures: An abnormal result would be to measure a drop in systolic blood pressure of >20 mm Hg approximately 15-20 minutes after a meal.

Other uncommon bedside stimuli that can be used to assess for a rise in blood pressure during continuous blood pressure monitoring include isometric exercise (sustained hand grip for 3 min), a cold pressor test (immersion of a hand in ice water for 90 s), and mental arithmetic (with serial-7 or serial-17 subtraction), all of which stimulate sympathetic outflow and elevate blood pressure in healthy patients.

Multiple daily blood pressures to examine for diurnal fluctuation: A difference of < 15 mm Hg with either systolic or diastolic blood pressure between daytime (awake) values and nighttime (sleeping) values could indicate presence of autonomic neuropathy.

Specific autonomic tests that are being performed at some institutions include the following:

The thermoregulatory sweat test (TST) complements the quantitative sudomotor axon reflex test (QSART) and is used to assess thermoregulatory pathways. The patient is covered with alizarin red powder, which, when moist, changes from orange to purple. The patient's temperature is then raised above core temperature, and photography is performed to map for areas of color change, revealing areas of anhidrosis/hypohidrosis where color did not change. The TST and QSART can both be useful in idiopathic anhidrosis. A lack of color changes with the TST is essentially diagnostic for postganglionic sudomotor dysfunction.

Sympathetic skin responses (SSR) can be assessed with routine EMG equipment. This test can be used to identify indirect evidence of sweat production via measurement of changes in skin conductance on the palm/sole in response to an electrical stimulus. The stimulation of an afferent somatic branch with SSRs gives an assessment of potential adrenergic sweat production. Brief electrical stimuli are administered at intermittent intervals and a response is measured from the hands or the feet, representing a change in skin resistance due to sweating.

Quantitative sensory testing (QST) can be helpful in autonomic disorders with sensory neuropathy. QST permits comparison of sensory thresholds by using vibration and temperature perception to assess both large and small-fiber modalities. These patients typically have impaired thresholds for heat and pain[54] , but vibration and cool sensitivity may be normal.

Pupillometry measure changes in papillary response and is being investigated at some institutions as a potential marker for autonomic neuropathy.
Quantitative direct and indirect test of sudomotor function (QDIRT) involves making a silicone impression of a patient's skin while sweating is induced by acetylcholine iontophoresis. The presence of sweat droplets can be quantified in the silicone cast, providing a marker of sudomotor function.
Vascular studies are occasionally useful in assessing autonomic neuropathy.


Vascular studies are occasionally useful in assessing autonomic neuropathy.

Adrenergic function can be assessed by measuring skin blood flow, transcutaneous oxygenation, and skin temperature.

Doppler probes can be used for blood flow measurements.

Infrared thermometry and telethermography can be used to measure skin temperature.

Assessment of skin temperature can be useful in patients with small-fiber neuropathy.

Urological studies

Urodynamic studies may be used to examine the lower urinary tract function.

Measurements include urine flow rate, residual volume, cystometry during filling and voiding, urethral pressure profile measurements, and pelvic floor neurophysiology.

An important measure in assessment of a neurogenic bladder is the postmicturition residual volume; this can be measured invasively by urethral catheterization after voiding, but it can also be measured noninvasively with ultrasonography.

Gastrointestinal studies

Videofluoroscopy is useful in assessment of swallowing in the presence of oropharyngeal dysphagia.
A barium swallow study, meal, and follow-through study are helpful in suspected upper gastrointestinal disorders, though endoscopic assessment provides the opportunity for biopsy in particular situations, as well as better visualization.

Esophageal manometry may be of value in disorders of motility and esophagogastric function.
Gastric motility may be assessed by using radioisotope methods and scintigraphic scanning.
In cases of small-bowel disorders suspected to be neurologic in nature, manometry may be of value in discriminating myopathic from neuropathic disorders. Large-bowel dysfunction can be assessed via measurement of transit time.

Esophageal manometry and gastric emptying scintigraphy can also be useful in patients with possible autonomic neuropathy and dysphagia.

Diabetic patients with symptoms of esophageal dysmotility have insufficient lower esophageal sphincter relaxation and a higher percentage of simultaneous waves detected, while diabetic patients with cardiovascular autonomic neuropathy have greater pathological simultaneous contractions.

Esophageal dysmotility and delayed gastric emptying may occur in up to 50% of diabetic patients. In particular, reports of abdominal fullness predicted delayed gastric emptying.

Biopsy findings

Sural nerve biopsy is occasionally diagnostic for types of autonomic neuropathy. In inherited autonomic neuropathies, a selective loss of particular fiber types can indicate the diagnosis. In autoimmune or infectious mediated forms of autonomic neuropathy, small perivascular infiltrates may be visible. In amyloidosis, characteristic Congo red staining indicates the presence of eosinophilic, extracellular, amorphous material surrounding perineurial and endoneurial vessels and within sympathetic ganglia and vagal nerves.

Epidermal skin biopsy can be used in the diagnosis of small-fiber neuropathies. This technique is less invasive than nerve biopsy. In autonomic neuropathies, autonomic fibers are deeper than the epidermal level; therefore, deeper biopsy is required to assess the fibers innervating sweat glands and piloerector muscles. In general, autonomic neuropathies of greater severity are associated with reduced epidermal fiber densities.

As distal endings are primarily involved in distal axonopathy forms of neuropathy, skin biopsy may be more sensitive than sural nerve biopsy to detect early abnormalities. Skin biopsy is also useful in congenital causes of autonomic neuropathy, as in congenital insensitivity to pain with anhidrosis (CIPA), where a lack of nerve fibers in the epidermis and only a few hypotrophic and uninnervated sweat glands are found in the dermis.

Immunologic Findings

Patients with autoimmune autonomic neuropathy can have antiganglionic acetylcholine receptor (AChR) autoantibodies.[66] Patients with high antibody values (>1.00 nmol/L) tend to have a constellation of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light responses, upper gastrointestinal symptoms, and neurogenic bladder. Higher antibody titers correlate with greater autonomic dysfunction as well as increased frequency of cholinergic dysautonomia.

Patients with POTS may also demonstrate presence of ganglionic receptors.

In specific disorders, testing for the presence of autoantibodies can help determine a diagnosis. Antinuclear antibodies and antibodies to Sjögren's syndrome antigens A and B (SSA and SSB) are seen in several connective tissue disorders. Antibodies against voltage-gated calcium channels (VGCC) are associated with LEMS.

The combination of tilt table testing, cardiac responses to deep breathing and the Valsalva maneuver, and QSART comprise the composite autonomic scoring scale (CASS), which may be used to assess the severity of autonomic dysfunction. The CASS is reliable and useful for monitoring clinical progression with an autonomic neuropathy. The CASS is a 10-point scale; 4 points are allotted for adrenergic and 3 points each for sudomotor and cardiovagal failure. Scores are normalized for age and sex. Patients with a score of less than 4 on the CASS have mild autonomic failure; a score of 4-6 suggests moderate autonomic failure; and a score of 7-10 implies severe failure.

The TST can be useful in monitoring progression of idiopathic anhidrosis and Sjögren's syndrome where prominent anhidrosis/hypohidrosis occurs.

A Little Humor




Saturday, September 17, 2016

Living With Pots




The first thing you should do in the morning is to drink a big glass of water or Gatorade. You shouldn't stand straight up either. Sit up for a few minutes and then stand up slowly.

Increase Fluid and Salt Intake

Most patients need to increase their fluid and salt intake to increase their blood volume, due to hypervolemia, venous pooling of blood in the lower body, and hypotension.  You should drink about 2 liters of water and consume 3-6 grams of salt a day.  Six grams of salt is 1 tsp. The exception is the hyperadrenergic subtype of POTS.  Here is a list of some ideas for getting extra salt into your diet:

Anchovies
Bacon
Bread products
Cheese
Corned beef
Chips
Miso
Mustard, prepared yellow
Olives
Pasta sauce
Pickles
Pretzels
Salami
Salted nuts
Sausages
Smoked meat and Fish
Soy sauce
Soup-canned
Stock cubes
Tomato ketchup

Meals 

When you eat a big meal, a large part of your blood is diverted to the digestive tract to help with digestion. This can cause your POTS symptoms to get worse. Eating smaller meals throughout the day will help.

Eating a low carb, high protein diet is helpful. Avoid refined sugar and flour. Low-fat proteins such as salmon, chicken breasts, and low-fat yogurt are good choices.

Some people have gluten intolerance called Celiac Disease. There are blood tests for this. Dairy may also be an issue. Some people are allergic to dairy and some have lactose intolerance.

Some patients find caffeine to be helpful, but others get worse symptoms after drinking caffeine.

Alcohol dehydrates your body, so it can cause you to get worse hypotension.

You need to pay attention to how you feel after you eat and adjust your diet accordingly.

You can sit down on a stool while you are making your meals in order to make it easier. You can also cook a double batch of recipes on a good day and freeze some for later.



Keeping Your Head Elevated

It helps to recondition your body to orthostatic stress to slightly elevate your head when you sleep. It also helps with GERD and gastric motility problems. In order to elevate your head, you can raise the head of your bed with bricks or large books. It isn't recommended to use a wedge pillow because this basically causes a bend in the middle of your body and blood can pool around your lower abdomen. You need to have the whole body on a slant so that your feet are lower than your hips.

Compression 

Compression hose and for some abdominal binders, can be helpful. They work by lowering the amount of venous pooling and hypotension. The hose  will work better if they are te 30 mmHg type and are waist high. Some insurance will pay at least partially if you have a prescription from your doctor. They come in all sorts of colors and patterns. If you get your first pair fitted at a medical supply store, it may cost more. But after that, you can buy them wherever you want. http://ihpotblogspot.blogspot.com/2016/09/compression-stockingshose.html

Excercise

This can be a problem for POTSies. But it is important to get some. Since standing isn't going to work, you can do things like reclined aerobic exercise, recumbent bikes, and swimming. Exercises for Dysautonomia Patients

There is a procedure you can do called counter-maneuvers. You cross your legs and tense the muscles. It is supposed to reduce your symptoms you get from standing. But it is always best to sit or lay down if you get really dizzy or feel faint so that you don't get hurt from falling down.

Try to avoid standing for long periods of time because it makes your POTS worse. Similarly to the counter-maneuvers, if you flex and squeeze your legs, feet, and buttocks muscles, or shift from one foot to the other it will help with your symptoms. It helps your body to pump blood back up to the heart.

I find that a shower chair comes in pretty handy. When you take a shower standing up, as I said above, it will make your symptoms worse. Hot or cold showers will make you worse too. Try to keep the water temperature lukewarm.  Extremes in temperature, especially heat make symptoms worse. Since it will probably tire you out to take a shower, it helps to take one right before bedtime. When you wash your hair, it helps to bend over at the waist and put your head upside down, the way you do when you wash your hair in the sink. This is helpful because raising your hands above your head will make your POTS symptoms worse.

To keep yourself cool, there are things like sun shirts, hats, hand-held misters, and personal fans. Clothes that dry quickly and wick moisture away help you keep an even body temperature.


Wheelchairs and scooters are necessary for some people. This may be psychologically hard for some people. But if your symptoms are bad enough, you really need these. They will make it easier for you to do more things because you won't have to stand and walk as much and you will have less chance of falling. You will have to contact your DMV and find out what is required to qualify for one of the disability placards.

If you can't get around any other way, or safely, wheelchairs and scooters are better than not being able to get out at all. And you might only need them on a bad day.

Medication List

You should carry a list of all of your medicines, what the dosage is and how often you take them. Microsoft Word makes it pretty easy to make a table for this. You also need to include food and drug allergies and emergency contacts. Most cell phones have an ICE in the contacts now. You should also have a list of your medical conditions, especially POTS or other dysautonomias. You would be surprised how many EMTs and ER doctors don't know what it is.
You can find printable ones here:
http://www.fda.gov/downloads/AboutFDA/ReportsManualsForms/Forms/UCM095018.pdf
https://www.ascp.com/sites/default/files/ASCP-PersonalMedList.pdf

Here are some more resources.


POTS What To Avoid

Feeling Rough Tonight

I have too many emotions about being sick. Today I took my BP and did the whole lay down, sit up stand up routine. I'm keeping records for my doctors. My BP went up some, but my heart rate went up 30 bpm from start-point. I got dizzy, I had chest pain, to the point my daughter wanted me to take nitroglycerine for it, and I have had weakness and a numb sensation in my head ever since. I have to hold onto the walls to go to the bathroom.

I've been thinking about how my husband died from this condition and I hate that my family will have to deal with me having it.

This stuff sucks. That's not very eloquent. But there it is.

Our dog has been keeping me company. She tries to be a good nurse.





Hyperadrenergic POTS

I have to share this great post here. I also put it on my facebook PAGE. I think this might be the type of POTS I have. Now to get a local doctor to do the right kind of test.
How would a doctor determine if I have Hyperadrenergic POTS?”: All About Catecholamine Testing in POTS

My facebook page gets some posts that aren't here because it's easier to just share them to my page than to blog them. I Hate Postural Orthostatic Tachycardia

Things that can throw the test off

Some common medications, foods, and beverages may interfere with catecholamine blood test results. Coffee, tea, and chocolate will make your catecholamine levels rise. Over-the-counter (OTC) medications like allergy medicine, might also interfere with the results. Your doctor should give you a list of things to avoid before your test. Make sure to tell your doctor all of the prescription and OTC medicines you’re taking.

Even small amounts of stress affect catecholamine levels in the blood, some people’s levels may rise just because they’re nervous about having a blood test.

http://www.healthline.com/health/catecholamines-blood#Outcomes7

How To Prepare For A Catecholamine blood test

https://lethargicsmiles.wordpress.com  Had a very helpful link that shows that the catecholamine blood test is standard testing for hyperadrenergic POTS, or POTS in general. Journal of Cardiovascular Electrophysiology--Postural Tachycardia Syndrome (POTS)




Friday, September 16, 2016

Causes of POTS/Postural Orthostatic Tachycardia Syndrome







Part of the process of being diagnosed with POTS is wondering what caused it and if you did something to cause it and questioning in general.

The list of things that can go wrong in the body and cause POTS is quite extensive. In addition, there are disorders with symptoms like POTS. The secondary disorders are usually treatable and will in turn, treat your POTS symptoms. I would ideally like to make a separate post for each thing. But it will be more efficient to just make links to information within this post.

Adrenal disorders like Addison's disease is one of those conditions that has POTS-like symptoms, meaning that it mimics POTS. If you have an auto-immune disease, you can have an autoimmune type of Addison's disease. And to complicate that further, autoimmune thyroid disease can put you at risk for the autoimmune form of Addison's disease. Other things associated with it are hypoparathyroidism, hypopituitarism, pernicious anemia, testicular failure in men, diabetes type I, and vitiligo.

One of the problems with adrenal glands can be caused by a form of tumor called a Pheochromocytoma. This causes the adrenal glands to be overactive. This can lead to high blood pressure and cause symptoms such as

  • Headaches
  • Sweating
  • Pounding of the heart
  • Being shaky
  • Being extremely pale
The hormones produced by the adrenal glands belong to a category called Catecholamines, which are released into the bloodstream in response to physical or emotional stress. The ones specific to the adrenal glands are epinephrine and norepinephrine, also called respectively adrenal and noradrenaline. The other catecholamine is dopamine. But it isn't produced by the adrenal glands. Dopamine is produced in the brain. It is a neurohormone that is released by the hypothalamus. Its action is as a hormone that is an inhibitor or prolactin release from the anterior lobe of the pituitary

Pheocychromatoma and paragangliomas cause an overproduction of these hormones. Blood and urine tests to measure the amounts can help detect a pheochromocytoma.

The urine test is a 24-hour urine collection. The blood test for catecholamines is sometimes done when a person has unexplained hypertension or sudden paroxysmal hypertension.


People with Anemia can have symptoms like POTS and if they also have a folic acid deficiency, it will make their anemia and POTS symptoms worse.

Anemia patients sometimes exhibit the symptoms of POTS. Some patients may have a folic acid deficiency, which is contributing to their anemia and POTS symptoms.


Angiotensin II

Some people with POTS have an increased level of Angiotensin II. The renin-angiotensin-aldosterone system (RAAS) helps regulate blood volume by regulating sodium and water retention. Renin and aldosterone can be reduced in POTS patients. But this study found that Angiotensin II was increased. http://www.ncbi.nlm.nih.gov/pubmed/16262605. Angiotensin II is a hormone and it causes blood vessels to constrict.

There may be a gene called the angiotensin II type one receptor gene involved in POTS.

Autoimmune Diseases

Besides anemia and Addison's disease and thyroid disorders, there are several other autoimmune diseases that can cause POTS. See: Lupus, Sjogren's, Guillain-Barre. Sarcoidosis, Crohn's Disease

There is an antibody to neuronal nicotinic acetylcholine receptors of autonomic ganglia that has been discovered. Some POTS patients have higher levels of this antibody. Some patients that also had anhidrosis, constipation, urinary dysfunction, sicca syndrome and pupillary dysfunction had even higher levels. And those that had the highest levels of the antibody, had more severe autonomic dysfunction. And levels of the antibody lower when patients symptoms get better. This indicates cause and effect. Meaning that the antibody can cause POTS and POTS can cause an increase in the level of the antibodies. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2671239/

Cardiac Problems

Usually, doctors try to rule out cardiac disease before any diagnosis of POTS.

Problems with the electrical conduction of the heart such as atrioventricular conduction and ventricular repolarization can cause POTS in some patients. Sinus node abnormalities and abnormal P-waves can also cause it. Some patients undergo a procedure called ablation to treat these type of problems. But studies show that the long-term benefits were not good. And it really isn't recommended as a treatment.
In addition, if a patient has been misdiagnosed with sinus tachycardia, it will make their POTS worse to go through ablation. Heart rate-dependent electrocardiogram abnormalities in patients with postural tachycardia syndrome.

Spinal Problems

Cervical stenosis is when there is an area of the spinal canal that is too narrow, and it compresses the spinal cord and nerve roots. Some patients have had craniovertebral decompression which resulted in a decrease or cessation of their POTS symptoms. But other patients did not benefit from it.

Chiari malformation is when the cerebellar tonsils protrude down into the spinal cord. And it can cause the flow of cerebral spinal fluid to be restricted. Some patients have a procedure to correct the Chiari malformation. But not all of them get better afterward. Some doctors think that Chiari malformation causes all POTS, and some do not. Doctors from the NIH and The Chiari Institue say there is a connection between as well as a connection to EDS/Ehlers-Danlos syndrome. There are lots of links here on Chiari malformation and POTS: http://www.dinet.org/index.php/information-resources/pots-place/pots-useful-links

Syringomyelia is a medical condition caused by a cyst growing in the spinal cord. This sometimes causes POTS. Some people also have Chiari malformation. Many POTS patients also report a worsening of symptoms upon straining. One possible explanation of POTS in syringomyelia patients is partial sympathetic denervation of the legs.

Chemicals

Exposure to some chemicals might cause POTS in some patients. Here is a table of some of the possible chemicals: Chemicals and POTS


Ehlers-Danlos Syndrome (EDS), a connective tissue disorder,is found in some POTS patients. Physicians propose that these syndromes occur together due to abnormal connective tissue in dependent blood vessels in those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures (Rowe, Barron, Calkins, Maumenee, Tong & Geraghty, 1999). Simply put, this connective tissue abnormality allows excessive amounts of blood to pool in these patients' lower limbs when they stand up.

There are a variety of types of Ehlers-Danlos syndrome. Classical and type III EDS were originally reported in orthostatic intolerance patients (Rowe et al., 1999). Many POTS patients with EDS have type III (Grubb, 2002).

There can be many symptoms and problems that are associated with the classical and hypermobile types of EDS. Mitral valve prolapse, gastric emptying or motility issues, dilation or rupture of the aorta, hiatal hernia, premature rupture of the membranes during pregnancy, poor wound healing, bruising, joint dislocation, etc.http://ihpotblogspot.blogspot.com/2016/09/joint-hypermobility-syndrome-and-eds.html

Joint Hypermobility Syndrome is a similar disorder. You can read more on them here:


Electrical injury

Electrical injury and being struck by lightning has reportedly occurred prior to the development of POTS in some cases. Lightning strike and autonomic failure -coincidence or causally related?


Liver Disease

A type of liver disease called compensated cirrhosis is when the liver is damaged but is able to compensate for it. This condition causes hypovolemia and vasodilation, POTS and pooling of blood in the lower extremities. Autonomic dysfunction in chronic liver disease

Mast-cell activation disorders

Some people with POTS or other orthostatic intolerance have flushing, palpitations, shortness of breath, chest pain, headache, lightheadedness, hypotension, or hypertension, and syncope
may play a role in the development of POTS in some individuals. Some patients with orthostatic intolerance suffer from episodes of flushing, palpitations, shortness of breath, chest discomfort, headache, lightheadedness, hypotension or hypertension and occasionally syncope, and it is sometimes brought on by an increase in activity. They may also have fatigue, sleepiness, increased urination, and sometimes diarrhea after they have an attack. There are tests for an increase in urinary methylhistamine, which is a marker of mast cell activation, that can be done to find MCAS.
You can read more about MCAS here: A Tale of Two Syndromes – POTS and MCAS


Neuropathy

Some people with neuropathy have POTS. It may be caused by autonomic neuropathy in the cardiovascular system. There may also be sympathetic denervation in the legs. The Neuropathic Postural Tachycardia Syndrome


Nitric Oxide deficit

Nitric Oxide (NO) controls blood vessel size with through changes in blood flow and blood vessels during inflammation and blood vessel leakiness. If you have a deficit of nitric oxide, you may develop POTS. Nitric oxide and regulation of heart rate in patients with postural tachycardia syndrome and healthy subjects

Researchers have found that NO levels can be increased by blocking the most important receptor for angiotensin-II. This may lead to treatments in the future in select groups of POTS patients.

Norepinephrine transporter deficiency

Norepinephrine transporter deficiency causes POTS is some people. These patients have an irregularity in how norepinephrine is used in the body. Under normal conditions, the body recycles norepinephrine. For some people, the protein that causes norepinephrine to be recycled doesn't work properly. And excess norepinephrine is spilled over. They then have depleted levels of norepinephrine if their neurons continue to be stimulated. They go from having excessive amounts of norepinephrine to having no norepinephrine, at which point they crash.Orthostatic Intolerance and Tachycardia Associated with Norepinephrine-Transporter Deficiency

Some patients have hypermethylation of the norepinephrine transporter (NET) gene promoter. When this happens, the gene for the protein that transports norepinephrine (NET) is turned off because its promoter is turned off. 


This is a condition is which the left renal vein is congested because it is being compressed by the aorta and the superior mesenteric artery. It may cause a disruption in the renin-angiotensin system. And it also may disrupt the sympathetic adrenal system and overproduction of catecholamines.


Thyroid disease

Thyroid disease can sometimes cause symptoms that are similar to those of POTS.

Tumors

When the body tries to get rid of a tumor by producing antibodies to attack it, sometimes they also attack part of the nervous system. This is called Paraneoplastic Syndrome. This is a rare condition. If the autonomic nervous system is attacked, then the result can be POTS or dysautonomia. Some people get better after the tumor is removed. Some people have to have intravenous immunoglobulin or other immune modulating treatments to try and reduce the harmful antibody levels.



Physical Trauma, Surgery, and Pregnancy

Trauma such as surgery, pregnancy, bariatric surgery, and Traumatic Brain Injury, car accidents, etc. have been shown to precipitate POTS


Viruses

It is estimated that 50% of patients with POTS have a recent history of some sort of virus when they become ill. It has been associated with Epstein-Barr virus. It is believed that viruses may affect the autonomic nervous system directly or that they may cause an autoimmune response that results in POTS.Postural Orthostatic Tachycardia Syndrome (POTS): A Diagnostic Dilemma

Vitamin Deficiencies

People with digestive problems, that are common in people with dysautonomia, frequently have B12 deficiency.



Don't forget to check out the videos at the bottom of the blog. You have to scroll down to the bottom. There is a good one on norepinephrine transporters and one on the sympathetic nervous system.