PoTSFrequently Asked Questions

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Glossary

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Autonomic nervous system: Part of the nervous system that controls involuntary functions of the body such as the heartbeat and breathing. When something goes wrong it can cause problems with blood pressure control.

Autoimmune disorders: These occur when the immune system produces antibodies that attack the body’s own tissues rather than fi ght infections.

Chronic fatigue syndrome (CFS): A long term condition with a wide range of symptoms, the most common being extreme tiredness. It is can be referred to as ME or myalgic encephalomyelitis.

Cognitive behavioural therapy (CBT): A talking therapy that can help us to accept and adjust to a condition. It is not a cure but will help improve recovery, ability to function and quality of life.

Joint hypermobility syndrome (JHS): A tissue disorder that aff ects the joints and ligaments. It is an inherited condition and sometimes referred to as Ehlers-Danlos type III.

Hypotension: A medical term for low blood pressure (less than 90/60)

Hyperadrenergic PoTS: This is thought to have a genetic link.

Orthostatic hypotension: A form of low blood pressure that occurs when you stand up from sitting or lying down.

Sjorgen’s syndrome: This is an autoimmune condition that is identifi ed by dry eyes and dry mouth and some individuals will also struggle with joint pains and fatigue.

Syncope: The medical term for fainting or unexplained loss of consciousness caused by a sudden lack of blood supply to the brain.

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1. What is PoTS? PoTS (Postural Tachycardia Syndrome) is a complicated disorder that manifests itself in a variety of ways. Symptoms can vary and are often exacerbated by daily activities such as modest physical exertion or what you eat. It is at last gaining increasing recognition by medical professionals.

PoTS is defi ned as a persistent increase in heart rate of over 30 beats per minute (bpm) or to higher than 120 bpm when standing upright over 10 minutes. Typically, there is no fall in blood pressure although fainting can occur.

2. What are some of the symptoms of PoTS? PoTS can manifest symptoms in a variety of ways including light headedness, fatigue, brain-fog, palpitations, chest pain, shortness of breath, anxiety, sweating, and lack of focus.

3. What is the difference between PoTS and dysautonomia? Dysautonomia is an umbrella term used to describe several diff erent medical conditions that are due to a malfunction of the autonomic nervous system (ANS). These can include a fast, resting heart rate, orthostatic hypotension, irregularities with the body thermostat amongst other things.

PoTS is one form of dysautonomia with major symptoms that arise when an individual is standing and relieved by lying down.

Frequently asked questions

This booklet is for individuals who have been diagnosed with postural tachycardia syndrome (PoTS) and are struggling with the many issues raised by this condition.

The questions are those that are most frequently asked by both patients and carers and the answers have been provided by medical professionals with an interest in Postural tachycardia syndrome.

4. What causes PoTS? PoTS can be associated with a viral illness such as glandular fever, a growth spurt or pregnancy. The condition can also occur in autoimmune disorders or following a traumatic event such as surgery, an accident or a serious illness. Unfortunately, in some cases a cause is never identifi ed.

5. How common is PoTS? PoTS mainly aff ects young individuals aged between 15 – 45 years who are predominately female. However, due to misdiagnosis and lack of awareness, the condition may be more prevalent than we think.

6. What are the conditions that PoTS is misdiagnosed as? Chronic fatigue syndrome (CFS), inappropriate sinus tachycardia (IST) although questions remain as to whether this is really a distinct condition, panic attacks and anxiety.

7. Is PoTS a serious condition? PoTS is not life threatening but can lead to signifi cant impairment in quality of life, resulting in the inability to study or work, leading to lost employment or schooling.

8. Is PoTS classed as a disability? PoTS aff ects individuals in diff erent ways. Some people may be able to cope with regular schooling or working, but others may suff er signifi cantly from their symptoms which aff ects their ability to hold down a normal job and pay their bills. If this is so, they may qualify for disability benefi ts.

9. Is PoTS genetic? It is estimated that about 40% of PoTS patients have a relative with the condition or who are struggling with some of the symptoms but have not been diagnosed. A common association with PoTS is the joint hypermobility syndrome (JHS), which can run in families.

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10. What medication and treatment options are currently available for PoTS? Medications like salt tablets, fl udrocortisone, and midodrine can be prescribed to help control the unpleasant symptoms. Some consultants may also consider Ivabradine or beta blockers. Diff erent patients respond diff erently to diff erent medications – sometimes this can be a matter of trial and error.

Thigh-high medical compression stockings or exercise compression leggings are sometimes suggested to help prevent blood pooling in your legs by pushing the blood upwards.

11. Can dehydration cause PoTS? Dehydration needs to be avoided, as it will worsen symptoms of PoTS. Fluid intake should be increased in POTS patients as they often have low blood volume and additional fl uid will help boost this. If resting blood pressures are on the low or low normal side, then increasing salt intake (6 to 10g: 1-2 teaspoons) may also be helpful.

12. What is IV fl uid infusion? IV fl uid is a saline solution which provides essential hydration directly through the veins. However, it should not be used regularly as the intravenous tubes used to get the fl uid into the veins can cause serious and even life-threatening complications. The chronic use of IV fl uid can be dangerous and should be avoided.

13. When is IV fl uid given? IV fl uid treatment is rarely prescribed but can be used to treat signifi cantly low blood volume if a PoTS patient is severely struggling with symptoms and admitted to hospital.

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14. Why do I feel worse in the morning? Kidneys eliminate excess fl uid in the body when you are lying fl at for some time, which is normally at night. Whilst sleeping under a duvet or blanket, we continue to sweat, and these two factors contribute to relative dehydration fi rst thing in the morning. This is the reason why people with PoTS can feel worse in the morning.

A glass of water taken before getting up can help.

15. Do beta blockers help PoTS? Betablockers can be helpful for patients with hyperadrenergic PoTS but they can increase symptoms of hypotension; often patients fi nd that small doses can be helpful but larger doses may make things worse again.

16. What does ‘off label’ mean? If a medicine is prescribed that is “off label”, it means that it has not been licensed for use which requires an application to a medicine organisation such as the MHRA. This usually happens for medicines that are used in thousands of people but in PoTS and related conditions numbers are too small for this to be done. There are a number of medicines, however, that are thought by experts to be eff ective and your doctor will discuss the possible benefi ts and risks with you.

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17. Does exercise help PoTS? One symptom of PoTS is exercise intolerance. Patients tend to feel exhausted or unwell during or after limited activity. There is evidence, however, that if exercise is undertaken in the correct manner, a patient’s symptoms and quality of life can improve. The most benefi cial is regular exercise, engaging in daily, mild-moderate aerobic activities in a more supine position (recumbent cycling, rowing, swimming for instance). Exercise will not only improve fi tness and increase energy but retrains the autonomic nervous system to regulate blood fl ow correctly and also increases heart size and blood volume which are thought to be a problem in PoTS.

It is strongly advised that exercise should start at a low level of activity with intensity and duration increased very gradually. One simple strategy is to purchase a pedal resistance bicycle and to sit on a chair and pedal for 20-30 minutes each day gradually building up resistance. This machine can also be placed on a table, to exercise arms and shoulders.

18. Are Isometric exercises helpful to PoTS patients? Isometric exercises involve contracting your muscles without moving your body which will push the blood back up towards the heart. An example, if you are feeling faint and unable to sit or lie down, is to cross your ankles and tense your calf muscles tightly. Combined with buttock clenching to make eff ects more pronounced, this exercise will help to get the blood pumping around the body and increase blood pressure – thus relieving symptoms. One recommendation is to practise this in bed before getting up to prepare your body for sitting and standing.

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19. Can diet affect PoTS? Blood pressure and pulse rate can be infl uenced by what we consume. These changes can be quite dramatic in a PoTS patient. Some PoTS symptoms can be improved through smaller and more frequent meals as many patients with PoTS experience deterioration in their symptoms after meals. It is preferable to eat ‘low GI food’. This means that the food is digested more slowly and does not therefore produce rapid peaks in blood sugar levels. These foods are often lower in carbohydrate and higher in fi bre content. Diff erent patients fi nd diff erent diets to be helpful - there are no hard and fast rules and you may need to experiment.

20. Can you have high blood pressure and PoTS? It is possible to have higher BP in PoTS; some may refer to this as hyperadrenergic PoTS, although the distinction between diff erent forms can be diffi cult. Some BP drugs may make PoTS worse and some PoTS medication may be better in this condition than others.

21. Can anxiety cause PoTS? PoTS is not caused by anxiety but can be confused with it. PoTS’ debilitating symptoms of fast heart rate and palpitations can also be symptoms of anxiety. Unfortunately, some patients are misdiagnosed with anxiety.

22. Is there a link between PoTS and Chronic Fatigue Syndrome (CFS)? Chronic fatigue syndrome is of uncertain cause and produces fatigue; there remains a lot of discussion as to what CFS might be. PoTS also causes fatigue and some patients diagnosed with CFS in fact turn out to have PoTS. The exact cause of both conditions remains uncertain. In some cases, therapies applicable to both conditions can help patients.

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23. Can PoTS affect pregnancy? PoTS should not be considered a contraindication to pregnancy nor does pregnancy need to be managed diff erently. Many expectant mums will say their symptoms improve during pregnancy or at least stay the same. Hyperemesis gravidarum (excessive vomiting during pregnancy) may be a problem in the early months for some but can equally be a problem for those who do not have PoTS.

A multidisciplinary team approach with consultant obstetrician, anaesthetist and patient’s consultant for PoTS is recommended. A pregnancy fact sheet is available to download on www.stars.org.uk

24. Should medication be stopped before pregnancy? As many patients take midodrine and other medications with this condition, it is important to discuss with your doctor before becoming pregnant. This also applies to whether some medicines can be taken when breast-feeding.

25. What help is available at school and university for PoTS patients? Many colleges and universities will have a disability offi cer who can provide support and guidance appropriate to your needs. In secondary school there should be a Special Education Needs Co-ordinator to provide help.

26. Will I get better? Although there is no cure for PoTS, it is a disorder which can eventually go away. It can be debilitating but if the symptoms are well managed through a combination of lifestyle and dietary changes, together with medication, a person can continue with a reasonably active life. Fortunately, teenagers, a group commonly aff ected by PoTS, may grow out of the condition during their early twenties.

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27. Is there any therapy that can help PoTS patients? PoTS is not a psychological illness but a long term physical illness which can have an eff ect on mental health which can worsen symptoms and cause distress to the patient. Cognitive Behavioural Therapy (CBT) can help to improve mood and provide help to adjust and cope with a chronic condition.

28. Can young children develop PoTS? There is evidence that children as young as ten years are showing symptoms of this condition. Unfortunately, because of the lack of paediatric cardiologists who are knowledgeable on the condition, there may be many who are being misdiagnosed.

29. What causes PoTS in teenagers? It is thought a rapid growth spurt at the start of puberty, often between 12 – 14 years, can be the cause of these debilitating symptoms in young teenagers. As they grow, the symptoms may improve in some although there are no hard and fast rules.

30. Are there any other conditions linked to PoTS? Hypermobility is often associated with this condition. There are new diagnostic criteria for the diff erent forms of hypermobility now but, in general, we recognise that abnormalities in connective tissue structure seem to be relevant in PoTS.

Autoimmune conditions are also associated with PoTS such as Sjorgen’s syndrome. Mast cell activation disorder is also considered which is a poorly understood condition where excessive and inappropriate release of histamine and other allergy chemicals can cause symptoms.

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31. Can alcohol affect PoTS? PoTS patients are normally advised to avoid alcohol because • It can cause vasodilation (widening of blood vessels) and blood pooling in skin and gut • It is a diuretic (ie causes more urine formation) and so can lead to dehydration If an occasion merits a glass of wine or champagne, then it is advisable to follow this with a large glass of water to counteract the eff ects of alcohol. Continue to stay hydrated even after having alcohol, as the after eff ects can last until the next day.

32. What is brain fog? Patients complain about diffi culty in computing information, focusing, communicating and simply thinking. Typically, contributory factors can be lack of sleep and chronic fatigue, dehydration and prolonged standing if the patient is able! High fl uid intake with salt and avoiding becoming too hot can help some patients. A fan in the bedroom or in the offi ce is helpful.

The reason why patients develop brain fog remains incompletely understood but may be something to do with low blood pressure, and pulsating and oscillating blood pressures, typically seen in PoTS.

33. What is the best advice a doctor can offer? Be patient, be motivated, be hopeful! Treatment of PoTS is seldom dependent on medication but an entire lifestyle change. You may have to adjust your diet, including understanding the important impact of fl uid and salt on your symptoms, and alter activity levels in your daily routine but never forget your goal to return to a quality of life that you can enjoy.

This booklet has been written to support PoTS patients who struggle to fi nd information on this debilitating condition. Without donations and fundraising,

we would not be able to provide support through our award-winning resources and helpline.

Please donate to support our vital work at www.stars.org.uk/donate

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If you would like further information or would like to provide feedback please contact STARS.

Please remember that this publication provides general guidelines only. Individuals should always discuss their condition with a healthcare professional.

Acknowledgements:STARS would like to thank all those who helped in the development of this

publication, including Dr Nicholas Gall, Dr P Boon Lim and Dr Charlotte D’Souza.

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