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    Information On POTS Syndrome

    Postural Orthostatic Tachycardia Syndrome

    What is POTS

    POTS is a common form of dysautonomia which largely affects women of child-bearing age.  The prevalence of POTS worldwide and in Australia is not currently understood, however some experts suggest that as many as 1 in 100 young women may experience POTS in their lifetime.

    Triggers

    POTS may be triggered by physical stressors such as viral infection, surgery, concussion, and pregnancy. In more recent times there has been increased reporting of POTS post SARS-CoV-2 infection, with some evidence that people with ‘Post Acute Covid Syndrome’ also have dysautonomia. POTS may also appear gradually and may be associated with familial genetic factors such as collagen disorders.

    Symptoms

    POTS often presents with the hallmark cardiac symptoms of orthostatic dizziness, light-headedness, and postural induced tachycardia.  However, as the autonomic nervous system is broadly affected, POTS is also accompanied by a constellation of symptoms that span multiple organ systems. A detailed health history may reveal:

    • Sleep disturbance
    • Lethargy
    • Systemic pain
    • Cognitive disruption (‘brain fog’)
    • Bladder symptoms (over-active bladder, urinary retention)
    • Gastrointestinal disturbance (bloating, early satiety, anorexia, constipation and/or diarrhoea)
    • Headache and neck and shoulder pain (‘coathanger’ pain)
    • Heightened sensitivity to light, noise, smell and taste.

    Comorbidities

    There appears to be multiple comorbidities that have a higher prevalence in POTS than the general population.  These include (but are not limited to):

    • Chiari Malformation
    • Coeliac Disease
    • Chronic Fatigue Syndrome/Myalgic Encephalomyelitis
    • Irritable Bowel Syndrome
    • Hypermobility Spectrum Disorder
    • Hypermobile Ehlers Danlos Syndrome
    • Migraine
    • Fibromyalgia
    • Mast Cell Activation Syndrome
    • Median Arcuate Ligament Syndrome
    • Sjogren’s Syndrome

     

    Prognosis

    POTS is not a condition that is normally ‘outgrown’. Depending on the underlying cause and severity, some individuals may continue to experience symptoms throughout their lifetime. It is important to know that 25% of patients diagnosed with POTS are debilitated to the extent that they cannot attend school or work.  Diagnostic delays are common, and many patients are mis-diagnosed or have their symptoms attributed to psychogenic causes.  Not only does this compound the impact of their illness but it likely delays access to appropriate care and treatment.  As POTS has a peak onset in the adolescent/young adult years, it has the capacity to disrupt education, social, economic, and vocational engagement, and development.  Early recognition and intervention are paramount to maximising positive outcomes for patients. Compassionate, multidisciplinary care is essential to improving quality of life.

    Diagnosis

    If you suspect your patient may have POTS, the following assessments may be helpful in confirming a diagnosis.

    • Thorough health history to identify common POTS symptoms and co-morbidities
    • Assessment of symptoms to establish onset triggers and chronicity
    • Standing Test/Poor Man’s Tilt Table. Repeat blood pressure and heart rate readings:
      • After 5 minutes of laying supine
      • After standing (without leaning or moving) at intervals of 2 minutes for a total of 10 minutes

    POTS should be suspected if there is:

    • A sustained heart rate rise on standing ≥ 30 bpm (or ≥40 bpm in adolescents).
    • An absence of orthostatic hypotension (≥20/10 mmHg blood pressure drop on standing).
    • Chronicity of symptoms ≥3 months from trigger
    • Associated orthostatic symptoms such as light-headedness, visual disturbance, pre-syncope
    • An absence of a known secondary cause of symptoms (dehydration, medication, anaemia, sepsis)

     

    Investigations

    To establish the absence of secondary causes of symptoms the following investigations are recommended before confirming a POTS diagnosis.

    • 12 lead ECG
    • Routine pathology including a full blood count and thyroid function
    • Holter monitor (to rule out arrhythmias)
    • Echocardiogram (to rule out structural abnormalities)

    POTS Treatment

    Treatment

    Hypovolaemia of unknown aetiology is common in POTS. (However, routine pathology rarely identifies this as both red cell and plasma volume remain relative to each other.)  For this reason, POTS treatments predominately target plasma expansion and venous return. A multidisciplinary approach to lifestyle adjustments is also encouraged as the first step to treatment.  Some treatment options include:

    Lifestyle management

    • Where blood pressure allows, increase consumption of NaCl 10-12g and water 2-3 litres daily.
    • Encourage wearing medical grade compression-wear (from ankle to waist) during waking hours to reduce blood pooling and increase venous return.
    • Encourage avoidance of hot environments
    • Graded exercise can help to increase blood volume and improve venous return. De-conditioning may precipitate functional decline however, in some patients, post-exertional malaise may complicate participation in low grade exercise. Early involvement of an Exercise Physiologists familiar with the treatment of POTS is highly recommended to manage this aspect of recovery.
    • Consider dietician/nutritional involvement where there is reduced oral intake due to gastrointestinal disturbance

     

    Pharmacotherapy Management

    Pharmacotherapy in POTS may require expert input and response is dependent on the severity of symptoms as well as the underlying pathogenesis of the condition.  Pharmacotherapy is usually targeted at increasing plasma volume, slowing heart rate and increasing venous return.

    Some examples of treatment options are:

    • Fludrocortisone for plasma expansion
    • Beta Blockers/heart rate control (propranolol) and ivabradine
    • Alpha receptor agonists (Phenylephrine, Midodrine)
    • IV fluids for acute decompensation or for refractory symptomology
    • Stimulants for fatigue/concentration
    Adeline
    As a family we have experienced firsthand the benefits that POTS research & information provides. Adeline (aged 19) has been suffering since the age of 15, and took us 3 years of searching to find a diagnosis, and explanation of her conditions. It was a very frustrating search, involving many weeks in hospital, over 200 tests & multiple specialist appointments each week. We had to keep searching for answers as you cannot fight the battle when you don’t know who/what you are fighting against. Adeline went from a typical 15 year old student, to someone who couldn’t function, socialise or attend school, basically overnight. Her sister Sophie (aged 16) had a very similar onset of the condition. At the age of 15 her first symptoms were pre-blackouts, multiple times per day, and a racing heart rate. Because we knew the signs of POTS, and the appropriate medical specialists, her diagnosis was much quicker. Sophie recently experienced a POTS flare whilst playing football, requiring an ambulance. The paramedic knew all about POTS, which made getting the appropriate treatment quick and easy. Adeline spent weeks in hospital while doctors tried to work out what was going on, after her many attacks. In contrast Sophie received the treatment & medication she required, and was discharged within hours. This is the power of knowledge and information, based on quality research.

    Adeline

    A POTS Braveheart

    Kimberly

    My name is Kimberly, and I am 43 years old.
    I remember the day my world changed (06/09/19).

    I came down with severe right sided abdominal pain, and just presumed it was my endometriosis playing up for the first time in years. I thought I would wait out the pain but by the end of the week I was sent to hospital with suspected appendicitis.

    Multiple hospital admissions followed an don one occasion my heart rate became rapid while I was showering and I felt like jelly, so I hit the emergency button. Despite a heart rate of 186bpm I was then told it was a panic attack. I had never had a history of anxiety or panic attacks and I felt completely dismissed. I had several trips to the ER, blood tests, kidney function tests, stress tests, ECG’s, Holter monitors. I saw approximately 11 different specialists, including 3 heart specialists, and was told there was nothing wrong with me and to see a psychologist. It was so hard knowing that there was something really wrong and no one believing you.

    14 months after all other medical investigations failed, I was lucky enough to find a POTS specialist. I was diagnosed and treated with medication that completely changed my quality of life. Although it is still not that of an ‘average’ person, I am able to cook, shop and perform small activities whereas before it was impossible.

    Through all this I still believe I am one of the lucky ones, although I am still unable to work and rely completely on my husband. I feel very blessed to have his support. He never once doubted me. He held me through the tears of frustration, and always said ‘never mind the cost’ we will get through this. I call this journey ‘my own personal hell’ because even now it’s hard for people to see me look like I do on this photo, and next minute I cancel plans and am bed ridden. I constantly get asked why I don’t work. It’s not because I don’t want to. It’s because I can’t, and I wish there was more recognition of how seriously POTS affects people’s lives.

    My biggest hope is in the future is to get back to work, have POTs recognised as a condition that entitles people to government support, but most of all, to find a cause, and prevent it from happening to other people. It effects so many other aspects of your life including friendships, marriages, parenting, your children, mental health, self-worth, social life, work life, to name a few. I live life to my fullest on my good days, and endure the bad. I have become very skilled at being still, and finding out who I really am inside. I reflect a lot. I look forward to more improvements and milestones now I have started my 12-month rehabilitation programme with an exercise physiologist.

    Kimberly

    Alexa & Monique, Adelaide SA

    Alexa & Monique AGE OF POTSIE: 18

    Growing up, Alexa was a happy, healthy child. She loved school and enjoyed many extra-curricular activities. At 15, she had a severe bout of Epstein Barr virus that landed her in bed for weeks. Recovery was slow; and while most of the initial symptoms eventually resolved, fatigue was ongoing and relentless. There were many days when she was simply too tired to get up. In addition, she suffered with headaches, debilitating brain fog, and feelings of anxiety with nausea when upright. My once active, rosy-cheek child had turned into a pale, listless shell of her former self – and despite multiple consultations with a number of doctors, no-one had any answers.

    By 16, it became clear that Alexa remained too unwell to attend school full time. Thankfully, her teachers were supportive and the decision was made for her to complete her senior schooling over three years instead of two. But, even though she now had a reduced subject load, attendance remained a daily struggle; and she missed more classes than she was able to attend.

    The future looked bleak; Alexa felt she could no longer make plans to pursue her dreams – she could barely function, let alone study, work or travel. She dropped all extra activities and gave up on her music studies. Her once busy social life became non-existent, and her peers celebrated their graduation without her.

    As a parent, it was heartbreaking to witness. I felt helpless and extremely frustrated at the lack of understanding and care available; looking for answers and chasing treatment options for a condition with no name was both expensive and very time-consuming. Alexa needed daily support and as a single parent, I could no longer work full time.

    It was after another prolonged episode of brain fog, heart palpitations and severe nausea that our paediatrician suggested Alexa, then 17, may have POTS. We were referred to a specialist cardiology clinic and that day, I did cry – finally, we had an answer! Finally, there were treatment options available. We were also very excited to learn that vital research was underway.

    While life is nowhere near normal yet, Alexa’s health and quality of life have improved under care of her POTS clinicians. She has had some success with medication, and she has taken the suggested lifestyle adaptations in her stride – salt, water and compression tights are her constant companions. Postgraduate studies are back on the cards; and, under guidance from our fantastic POTS team, we are both looking forward to the future.

    Alexa & Monique, Adelaide SA

    A POTS Braveheart

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