6. Management and Treatment Strategies

← PREVIOUS: [[5.4. Secondary POTS and underlying conditions]] | NEXT: [[6.1. Lifestyle modifications and non-pharmacological approaches]] → -------- ##### Summary >[!Summary] >- There is no specific formula to decide which treatment to use and treatment must be individualized to each patient[[#^12]] >- It is also plausible that response to testing (such as norepinephrine levels or cholinergic sweat testing or leg blood flow assessments) will identify sub-groups of POTS patients who would respond differently to specific treatments[[#^13]] >- Many POTS patients are more sensitive to pharmacological treatments. Therefore, when initiating a therapy the lowest dose should be used first and titrated up to higher doses as needed for symptomatic improvement as long as the drug is tolerated[[#^12]] >- there is no cure for POTS but there are many non-pharmacological and pharmacological approaches to relieving POTS symptoms[[#^9]] >- The FDA has not approved any medications for the treatment of POTS; however, many drugs are used “off label” to relieve POTS symptoms[[#^11]] >- first step in the appropriate management of POTS is the correct diagnosis[[#^2]] >- after the diagnosis has been established, patients should be thoroughly educated about nonpharmacological measures alleviating the symptoms, long-term prognosis and available therapeutic options adequate to patient’s status[[#^1]] (both verbally, using dedicated printed material, brochures, etc. and appropriate Internet links, if available)[[#^2]] >- core impact relies on patient’s education, including avoidance of orthostatic intolerance triggers and better understanding of POTS pathomechanisms[[#^1]][[#^6]] >- exercise training may be very effective and has been shown to alleviate the symptoms of POTS-related deconditioning[[#^1]] >- if symptoms are pronounced, then pharmacologic treatment should be considered[[#^1]] >- management always involves expansion of plasma volume with high salt and high fluid intake[[#^7]] >- management of POTS includes avoidance of precipitating factors, volume expansion, physical countermaneuvers, exercise training, pharmacotherapy and behavioral-cognitive therapy[[#^3]][[#^4]] >- Treating POTS is not straightforward and often involves an individualized approach trying several therapies since no one therapy is universally effective[[#^8]] >- There is also no consensus on whether certain endophenotypes of POTS patients respond differently to treatment or whether all POTS patients should be treated similarly[[#^8]] >- When non-pharmacological approaches are insufficient at relieving POTS symptoms, pharmacological treatments for POTS are warranted to improve quality of life and prevent disability[[#^10]] >- some patients will present with more severe symptoms that may require the initiation of pharmacological therapy even at the first visit[[#^10]] >- making sense of symptoms is very individualistic and a diagnosis does not always lead to instantly reliable management strategies[[#^14]] >- there is an emphasis on the need to “get to know” their own symptoms, some of which are difficult to control[[#^14]] >- slowly, as participants seek out information and become more familiar with their own limitations, they start to develop a “mixed bag of self-management tools”, however, such “tools” and information are not always forthcoming from HCPs[[#^14]] >- even when a diagnosis of POTS is made, there appears to be much misinformation about the curability of POTS and there is a sense that most HCPs, especially family doctors, have little training or information on the condition, with POTS being considered, as something “you will grow out” of[[#^14]] >- the advice provided to “push though” their symptoms, was unhelpful at best, and damaging at worst. Rather than simply pushing through symptoms, patients' engagement in trial and error to get to know and understand their body, symptoms and all, is a more sustainable strategy than those proffered by HCPs[[#^14]] >- it is clinically useful to conceptualize each patient having their own individual POTS thumbprint, requiring detailed clinical assessment and an individual plan to optimize treatment and outcomes[[#^15]] >- available therapies for this condition are limited and directed toward symptom control by either increasing intravascular volume, increasing peripheral vascular tone or controlling HR[[#^16]] >- the response to therapeutic interventions is highly variable, with improvement in HR control not always improving the quality of life, rendering management of this condition highly challenging[[#^16]] -------- >*“After the diagnosis has been established, patients should be thoroughly educated about nonpharmacological measures alleviating the symptoms, long-term prognosis and available therapeutic options adequate to patient’s status [5, 20, 34, 35, 63, 64] (Table 5). Here, the core impact relies on patient’s education, including avoidance of orthostatic intolerance triggers and better understanding of POTS pathomechanisms. Exercise training may be very effective and has been shown to alleviate the symptoms of POTS-related deconditioning [66, 67]. If symptoms are pronounced, as evaluated by different symptom scores such as Orthostatic Hypotension Questionnaire (OHQ)[68], and functional class ‘pyramid’[69] focusing on complaints associated with orthostatic intolerance, or Karnofsky Performance Status [70] focusing on overall function limitation, then pharmacologic treatment should be considered” *([[Fedorowski-2019]], [p. 9](zotero://open-pdf/library/items/BZ35QDLR?page=9&annotation=KFW4H8LV))^1 >*“The first step in the appropriate management of POTS is the correct diagnosis. Patients should be comprehensively informed about the syndrome both verbally, using dedicated printed material, brochures, etc. and appropriate Internet links, if available.” *([[Fedorowski-2019]], [p. 9](zotero://open-pdf/library/items/BZ35QDLR?page=9&annotation=YIDJDT2I))^2 >_“Management of POTS includes avoidance of precipitating factors, volume expansion, physical countermaneuvers, exercise training, pharmacotherapy (fludrocortisone, midodrine, -blockers, and/or pyridostigmine), and behavioral-cognitive therapy.”_ ([[Benarroch-2012]], [p. 1](zotero://open-pdf/library/items/WEZLT9QC?page=1&annotation=4FMMVRN9))^3 >_“The management of POTS includes patient education, volume restitution, physical countermaneuvers, graded exercise training, and pharmacotherapy (fludrocortisone, midodrine, -blockers, and/or pyridostigmine).”_ ([[Benarroch-2012]], [p. 2](zotero://open-pdf/library/items/WEZLT9QC?page=2&annotation=2B3LW3LR))^4 >*“All patients with POTS require a high salt diet, copious fluids, and postural training. Many require β-receptor antagonists in small doses and low-dose vasoconstrictors.” *([[Low-2009]], [p. 1](zotero://open-pdf/library/items/I4WAD8AG?page=1&annotation=X42QKT5V))^5 >*“All patients are required to be educated on the pathophysiology of POTS, aggravating and ameliorating factors, so as to better handle the condition” *([[Low-2009]], [p. 6](zotero://open-pdf/library/items/I4WAD8AG?page=6&annotation=8XQMZP78))^6 >*“Management always involves expansion of plasma volume with high salt and high fluid intake.” *([[Low-2009]], [p. 7](zotero://open-pdf/library/items/I4WAD8AG?page=7&annotation=QF62W57B))^7 >*“Treating POTS is not straightforward and often involves an individualized approach trying several therapies since no one therapy is universally effective. There is also no consensus on whether certain endophenotypes of POTS patients, discussed in another article in this issue (Arnold et al., 2018), respond differently to treatment or whether all POTS patients should be treated similarly.”* ([[Miller-2018]], [p. 1](zotero://open-pdf/library/items/A3CIPE3G?page=1&annotation=ANSNB7P3))^8 >*“There is no cure for POTS but there are many non-pharmacological and pharmacological approaches to relieving POTS symptoms. POTS itself does not lead to an increased mortality, so treatment is targeted at relieving symptoms and improving quality of life.”* ([[Miller-2018]], [p. 1](zotero://open-pdf/library/items/A3CIPE3G?page=1&annotation=IHN4HNGM))^9 >*“When non-pharmacological approaches are insufficient at relieving POTS symptoms, pharmacological treatments for POTS are warranted to improve quality of life and prevent disability (Sheldon et al., 2015). While it may be reasonable to initially give a trial of non-pharmacological treatment alone, some patients will present with more severe symptoms that may require the initiation of pharmacological therapy (in addition to non-pharmacological therapy) even at the first visit.”* ([[Miller-2018]], [p. 1](zotero://open-pdf/library/items/A3CIPE3G?page=1&annotation=6PRKFL49))^10 >*“Treatment is aimed at relieving symptoms and not a specific hemodynamic target. The FDA has not approved any medications for the treatment of POTS; however, many drugs are used “off label” to relieve POTS symptoms.”* ([[Miller-2018]], [p. 1](zotero://open-pdf/library/items/A3CIPE3G?page=1&annotation=KRPNC722))^11 >*“There is no specific formula to decide which treatment to use and treatment must be individualized to each patient. Many POTS patients are more sensitive to pharmacological treatments. Therefore, when initiating a therapy the lowest dose should be used first and titrated up to higher doses as needed for symptomatic improvement as long as the drug is tolerated. Often POTS patients require a lower doses of medications than what is recommended for other diseases and disorders.”* ([[Miller-2018]], [p. 2](zotero://open-pdf/library/items/A3CIPE3G?page=2&annotation=TKDEFWZF))^12 >*“It is also plausible that response to testing (such as norepinephrine levels or cholinergic sweat testing or leg blood flow assessments) will identify sub-groups of POTS patients who would respond differently to specific treatments. This is the holy grail of creating “POTS endophenotypes” within the larger clusters of patients diagnosed with POTS.”* ([[Miller-2018]], [p. 8](zotero://open-pdf/library/items/A3CIPE3G?page=8&annotation=N4DFGE5J))^13 >*“Making sense of symptoms was very individualistic and a diagnosis did not always lead to instantly reliable management strategies. Similar to illnesses such as CFS [23] and MS [24], there was an emphasis on the need to “get to know” their own symptoms, some of which were difficult to control. Determining what worked for them was highlighted by all, but tinged with a strong sense of uncertainty. Slowly, as participants sought out information and became more familiar with their own limitations, they started to develop a “mixed bag of self-management tools”, however, such “tools” and information were not always forthcoming from HCPs. Even when a diagnosis of POTS was made, there appeared to be much misinformation about the curability of POTS and there was a sense that most HCPs, especially family doctors, had little training or information on the condition, with POTS being considered, as something “you will grow out” of. Participants relayed that the advice provided to “push though” their symptoms, was unhelpful at best, and damaging at worst. Rather than simply pushing ([p. 6](zotero://open-pdf/library/items/P2VFKVHY?page=6&annotation=E59YB89J)) through symptoms, participants engaged in trial and error to get to know and understand their body, symptoms and all felt that a this was a more sustainable strategy than those proffered by HCPs.”* ([[Knoop-2023]], [p. 7](zotero://open-pdf/library/items/P2VFKVHY?page=7&annotation=FJYAWN28))^14 >*“Some symptoms are seen to fluctuate with, and some remain independent of, postural changes. Thus, cognitive treatment approaches include those aimed to improve global POTS symptoms, as well as more tailored regimes for neuropsychological phenomena such as depression and anxiety symptoms and impaired cognition. It is clinically useful to conceptualize each patient having their own individual POTS thumbprint, requiring detailed clinical assessment and an individual plan to optimize treatment and outcomes.”* ([[Raj-2018]], [p. 11](zotero://open-pdf/library/items/IYRK4T6C?page=11&annotation=PSJK7ZKT))^15 >*“Available therapies for this condition are limited and directed toward symptom control by either increasing intravascular volume, increasing peripheral vascular tone or controlling HR. The response to therapeutic interventions is highly variable, with improvement in HR control not always improving the quality of life, rendering management of this condition highly challenging.”* ([[Wells-2017]], [p. 2](zotero://open-pdf/library/items/HLELIN7I?page=2&annotation=9DF9M66E))^16