How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office

Abstract

Orthostatic intolerance (and its subtype postural orthostatic tachycardia syndrome [POTS]) is an increasingly common complaint among adolescents associated with significant decrease in quality of life and frequent visits to primary care offices. As orthostatic intolerance becomes increasingly discussed online and through social media, we have observed that family angst and anxiety only heighten, often leading to requests for specialist referral to manage the symptoms. This often leads to delay in diagnosis, which further exacerbates the situation. In truth, however, a pragmatic approach applied by the primary care provider, including a ready explanation for the experienced symptoms, not only provides the bedrock for patient improvement, but also eases the anxiety of having a seemingly nebulous diagnosis. As physicians caring for these patients, we have seen success with treatment and seek to share this blueprint. Key points that will be covered include how to make an accurate diagnosis (using the “dizziness rule”), how to counsel about treatment, and clinic pitfalls to avoid. The description of care that follows is not based on systematic review and instead is exactly what is used in our office and what we hope will be successful in yours as well.

Introduction

As physicians taking care of patients with orthostatic intolerance, or its specific subtype postural orthostatic tachycardia syndrome (POTS), we have seen a significant rise in referrals of adolescent patients with these concerns over the last few years. In looking at our practice’s new pediatric cardiology referrals in the first 6 months of 2024, orthostatic intolerance outpaced chest pain, heart murmur, and palpitations as a referral indication. While the specific reasons for this pattern are not certain, what is apparent is that much of the care of these patients can take place in the primary care office, especially when providers are well-informed and well-equipped to do so. Indeed, a patient’s primary physician knows them best, and therefore will be trusted to shepherd patients through these symptoms even more so than a specialist who is met for the first time. Delay in diagnosis and uncertainty regarding treatment are also avoided through this approach. Therefore, the purpose of this article is to inform providers on how to care for these patients in clinic. We will discuss how to identify orthostatic intolerance, how to explain orthostatic intolerance in a way that makes sense to families, what to do in clinic to move along care, what not to do (that if done will only hinder improvement), and finally how to best co-manage patients who you have referred to a specialist.

How to Identify Orthostatic Intolerance and POTS

How, amid a busy practice, can you identify orthostatic intolerance and POTS? Patients with poorly treated orthostatic intolerance tend to have a myriad of symptoms—dizziness, palpitations, syncope, nausea, exercise intolerance, shortness of breath, difficulty concentrating, tremor, anxiety, and insomnia among them—and taking a detailed history for each of these complaints is preclusively time consuming. Instead, we encourage the use of an intake form for patients with key chief complaints, particularly dizziness and syncope. Patients who are lightheaded when upright in particular should be identified. An example form we use in our clinic is available as Supplement Material 1. This form can be completed in the waiting room and is invaluable to review prior to seeing the patient (and cuts down on time needed to glean this information). The form is also helpful to gauge the severity of symptoms as well as contributing factors. When it comes to identifying orthostatic intolerance, all symptoms are not equally determinative. Fortunately for the busy physician, dizziness, especially when it is positional (worse with standing, for example with getting out of bed in the morning, and better with lying down) is both exceptionally sensitive and specific for the diagnosis. In an internal review of patients seen in our clinic from February to June 2024 who met criteria for POTS (based on a heart rate (HR) change of 40 bpm with standing vital signs or a single HR > 120 bpm during orthostatics), all of the patients had dizziness by report.

One exception to this “dizziness rule” is an eating disorder—in our experience an eating disorder can present with significant overlap in symptoms (including dizziness) and without the disclosure of food restriction it is easy to mistake this for orthostatic intolerance. Careful attention to weight over time, self-perception of body image, and nutritional intake in those not improving as expected is necessary to avoid this pitfall.

Dizziness is very helpful to follow over time as well, as improvement in dizziness heralds overall improvement. We tell families to think of dizziness quantitatively, and if one is making “40 units” of dizziness at baseline (in terms of severity and frequency of the symptom), seeing this dissipate week to week (down to 30 units, then 15 units, etc.) represents accurate feedback about following the plan sufficiently and being on one’s way to complete improvement (reaching zero units of dizziness). This is helpful for patients who are perceiving slow progress—something is improving and predicts further improvement to come. Finally, orthostatic vital signs can be done in clinic to confirm orthostatic tachycardia (as defined above), which when present is specific for POTS, however the test’s low sensitivity, especially in adolescents, means that this is not required for a diagnosis of orthostatic intolerance.^{1 -3} We often see teenagers with significant dizziness and other manifestations of orthostatic intolerance, but negative orthostatic vital signs. The orthostatics do not invalidate the patient’s history. For this reason, a tilt table test, which is additionally, but not adequately, sensitive for producing orthostatic tachycardia or vasovagal syncope, is also not required for a diagnosis of orthostatic intolerance in adolescents.^{4 -8} If orthostatic vital signs are to be performed, it must be remembered that there is no consensus on how to do these correctly. Our opinion is to obtain them over 3 min—at rest, then have the patient stand directly, then obtain HR and blood pressure at 1, 2, and 3 min. This strikes a balance between decent sensitivity for orthostatic tachycardia but also ability to move along care in clinic. When orthostatic tachycardia is present, we use this as part of counseling to families on confirmation of the diagnosis. It can also inform on severity of the diagnosis (meeting criteria for POTS—see below) and likelihood of requiring medications over time.

How to Explain the Pathophysiology to Patients and Families

Now that a patient with orthostatic intolerance is identified, how can the physiology and ultimately therapy be explained to families in a comprehensible way (a prerequisite for improvement in our experience, as well-informed patients are less anxious and more likely to follow a plan). Doing this well also fosters trust in the physician. This explanation is necessary in particular for patients who do not improve as quickly as they would like or see worsening of their condition along the way—do they abandon the plan or instead stick with the provider who explained their symptoms in an understandable way?

To begin, we often in clinic when talking to patients use the term orthostatic intolerance instead of POTS, as it covers the full spectrum of symptoms, from mild to severe, and ubiquitously includes lightheadedness/dizziness that occurs while upright and is relieved by recumbence. ⁹ Pediatric POTS is a typically more severe version of orthostatic intolerance, marked by (1) severe and daily symptoms of orthostatic intolerance, (2) ruling out other masquerading causes such as an eating disorder, (3) at least 6 months of symptoms, and (4) an upward change in heart rate by 40 bpm (or reaching a heart rate of 120 bpm) with changing from supine to standing, within 5 to 10 min of testing, using either a tilt table test or orthostatic vital signs. In our experience treating adolescents, there is significant clinical overlap in patients meeting criteria for POTS versus those with orthostatic intolerance, although the former might be more likely to require medication. Typical patients are adolescent girls, usually between 12 and 16 years old at time of presentation. ⁹ Interestingly, a high percentage of these patients will have hypermobile joints, which helps to explain the physiology.^10,11 Specifically, hypermobility in the joints marks one as having laxity in their connective tissue, and thus laxity in the support of their blood vessels, leading to increased gravitational pooling of venous blood in dependent parts of the body throughout the day (leading ultimately to decreased cerebral perfusion, the stimulus for orthostatic symptoms). Joint hypermobility is easily assessed in clinic utilizing the Beighton maneuvers and score.^12,13

Orthostatic intolerance is experienced when the patient is upright, sitting or standing. Gravitational effect leads to increased venous pooling of blood in the pelvis and lower extremities, resulting in decreased preload to the heart and therefore decreased cerebral perfusion. ¹⁴ This causes lightheadedness and dizziness in the patient. ¹⁵ In response, the sympathetic nervous system (SNS) is activated, with the aim of rectifying the drop in cerebral perfusion, specifically by inducing peripheral vasoconstriction and increasing cardiac output. This effort often is insufficient, however, so lightheadedness does not abate and instead the patient experiences the unpleasant effects of this “adrenaline surge,” both in the moment and throughout the day depending on how often the circumstances are met.^14,15 The excessive sympathetic activation then leads to the additional bothersome symptoms for the patient—palpitations, fatigue (excessive exposure to daily SNS activity), nausea and other symptoms of GI intolerance (gut under perfusion in a hyperadrenergic state), and eventually syncope if a vasovagal reflex is activated in response to excessive SNS activity.^16,17 Teenagers are particularly at risk for this due to their higher metabolisms and thus higher needs for fluid and salt intake to maintain adequate blood volume.

Anxiety about these symptoms potentiates the pathophysiology, as additional excessive adrenaline is made in response—drying out the patient, increasing fluid requirements, and cycling the pathophysiology. Explaining this to patients in this way takes the focus from anxiety as a primary cause, but still informs them that it is a contributor to symptoms and must be addressed just like the other parts of treatment. The Figure 1 below depicts this physiological model.

Figure 1.

Simplified pathophysiologic model of orthostatic intolerance to aid patient education.

Finally, families often want to know “What caused this?” or already have a pet theory, such as COVID-19 infection or a concussion. We often see patients whose histories fit this timeline, however not all patients with a concussion or an illness develop significant orthostatic intolerance. Instead, it is our hypothesis that many triggers (concussion, illness, becoming less active with sports among them) can unveil symptoms, but only in the predisposed patient who typically has a high metabolism and likely hypermobile joints as a marker of ability to significantly pool venous blood. This explanation takes attention from the supposed trigger (which just impedes progress) and instead refocuses on the lifestyle changes that must take place to regain health.

What to Do in Clinic

Once the problem of orthostatic intolerance is explained, an important first care step is to tell the patient and family that symptoms will improve. This is substantiated in the literature. Physicians at the Mayo Clinic reported their results in a 5-year follow-up survey of patients meeting criteria for POTS. ¹⁸ Of the 172 patients surveyed, 87% reported much improvement, only intermittent symptoms, or complete resolution over time. In our own previous internal review of patients with initial clinic visit from January to April 2019, 86% reported improvement by 6-month follow up (65% majorly improved, 21% mildly improved). Furthermore, average quality of life score, rated on a 10-point scale, improved statistically significantly from a mean of 5.3 ± 1.7 to 7.4 ± 1.8 (n = 70 patients, P < .01), and 87% of patients who were significantly out of school were able to resume it. Further improvement is typical as time and treatment progress. Providing this encouraging news up front is important in building patient trust, compliance, and perseverance, all of which are needed to ensure success.

School absenteeism, amongst other school issues, is a place where primary physicians can help. Families will report that they receive calls from the school often when their child is ever dizzy, not feeling well in general, or assuredly if they pass out, and this leads some families to choose to home school or do home-bound school instead. We encourage patients to return to school, but also know that having a strategic plan in place will make this more likely to be successful. This plan can be anything from a formal accommodation plan (i.e. a 504 Education Plan in the United States) to an informal letter to the school outlining how the child should be treated during the school day. We provide a letter for families that outlines the plan, which can be incorporated into a formal plan if needed. The letter specifically mentions being allowed to have a water bottle in class, salty snacks during the day, extra bathroom breaks while hydrating, salt tablets to be kept with the school nurse, and allowance to lie down when dizzy to avoid passing out (this includes not being made to exercise while dizzy during physical education class). Our experience is that schools are receptive to this plan once it is put in place, and having this plan keeps the school from being a place of consternation and impedance to overall patient progress. A copy of our letter is included as Supplement Material 2.

How much water and salt? This question comes up during every encounter and represents basic needs. Children who experience orthostatic symptoms need extra water and salt intake to ultimately increase their blood volume.^8,15,19 We recommend 64 to 72 fluid oz of water per day and 3000 to 5000 mg of sodium intake daily, for the typical teenager. This is in line with published recommendations.^8,19 Further recommendations include starting aggressively in the morning, with water in a thermos and salt tablets on the nightstand, in order to make up for the lack of intake overnight. We recommend buffered or coated salt tablets to improve palatability. Ultimately, total intake can be titrated individually, as patients can use the lack of dizziness and clear urine output throughout the day as feedback on salt and fluid adequacy.

We test for both iron and vitamin D deficiency, as these derangements worsen symptoms of orthostatic intolerance. The labs to send are a complete blood cell count to check for anemia, a 25-vitamin D level (normal > 30 ng/mL), and a ferritin (normal > 30 ng/mL). A ferritin is important to send in addition to checking for anemia, as iron deficiency even without anemia can worsen orthostatic symptoms.^{10,20 -22} Vitamin D deficiency has also been associated with orthostatic intolerance.^{22 -24} Completing this screening early into care is important, as it takes time for supplementation to adequately improve a patient’s blood work and thus should be started as soon as possible. For iron supplementation, we follow randomized-controlled trial-backed recommendations for every other day dosing to improve absorption and minimize side effects.^25,26 One final point to remember is that in adolescent females with iron deficiency, asking about menorrhagia is important as this can be a significant contributor to iron loss when present and is treatable via hormonal therapy.

Another area of focus is on encouraging exercise as therapy. A study that highlights the effectiveness of exercise comes from George et al, ²⁷ who reported results of 100 participants meeting POTS criteria who completed a program of mild-to-moderate intensity endurance training. At follow-up, 71% of the patients significantly improved to the point that they no longer met the diagnostic criteria for POTS. Perhaps even more encouraging is that this improvement remained true 6 to 12 months after the exercise intervention, based on clinical follow-up. Encouraging exercise makes sense, as through improving fitness and lower extremity muscle tone, the “skeletal muscle pump” is improved, which augments venous return to the heart and thus adequate cerebral perfusion.^15,28 Exercise also improves a patient’s blood volume capacity, thus creating a buffer between a patient and daily symptoms. ²⁹ Additional positive studies on the effects of exercise, from formal programs to informal counseling, are well-documented.^{30 -33} A key point in counseling exercise is to make sure patients are “reading their bodies” during exercise in order to avoid exercising in a volume-depleted state, which only leads to severe symptoms and even syncope as well as excessive fatigue afterward. We do this again by emphasizing dizziness/lightheadedness—is it not “normal” to be dizzy during exercise and instead indicates that the patient is exercising in an undehydrated instead. Instead, we counsel patients to be sufficient with salt and fluid intake (including salt tablets) especially 30 min prior to exercise, and then use dizziness during exercise as feedback on their plan. Exercising without being lightheaded leads to more steady progress physically and thus overall improvement with time. In patients who are slow to make progress on their own, we refer to physical therapy to provide oversight and guide initial improvement.

A final emphasis when treating patients in clinic is to identify cohabitating anxiety, but to do so in a way that does not alienate the family. As will be discussed in more detail below, many patients are told the symptoms are “all in your head” or solely due to anxiety. This is certainly not true (as indicated by the causative physiology covered above), but anxiety still contributes to symptoms when present. We explain this to patients by stating that anxiety about symptoms, while understandable, still has the “chemical consequence” of adding more adrenaline to the body, leading to even more symptoms. Our experience is that families are more receptive of this explanation, which then leads to better compliance with recommendations for counseling and even medications in some circumstances. A copy of an education handout (summarizing the above measures as well as other suggestions) that we give to patients in our clinic is available as Supplement Material 3.

The most important responsibility of the primary physician is to not abdicate your central role in caring for the patient. Be the overseer—specialists can help, but also can miss the broader picture while treating specific complaints. See the patient back—are they actually getting better? Do the labs need to be repeated? Was something missed—does this “POTS patient” have something else, like an eating disorder (is the patient losing weight)? Families trust their primary care providers, and knowing they are treating orthostatic intolerance as they have so many other ailments beforehand, is surely comforting, reinforcing, and ultimately therapeutic.

What Not to Do in Clinic

When treating patients in clinic, there are pitfalls to circumvent to guide patients expediently toward improvement. One scenario to avoid is multiple referrals for multiple complaints. As previously discussed, a patient presenting with orthostatic intolerance and not yet receiving proper treatment will likely have many complaints—tachypalpitations, shortness of breath, joint pain, nausea, diarrhea, headaches, insomnia, and anxiety among them. We often see patients who are referred to our pediatric cardiology office also being referred to pediatric gastroenterology, rheumatology, sleep medicine, neurology, and psychiatry as well. Some of these appointments are many months in the future, forcing patients to wait in limbo for the recommendations yet to come. This assuredly creates uncertainty for families—perhaps the rheumatologist to be seen 6 months from now will finally have the answers? Over utilization of medications also typically comes from heavy subspecialty referral. Instead, sifting through the complaints and identifying positional dizziness as both a specific and sensitive sign of orthostatic intolerance is helpful—this allows referral to your particular subspecialist (often a cardiologist) when this is desired.

A second pitfall is to over emphasize the centrality of a mental health disorder as a cause for symptoms. Many pediatric patients have anxiety, some significantly, but only certain patients with particular predisposition (as discussed above) develop orthostatic intolerance. Even so, labels of anxiety and other psychiatric diagnoses are likely to be applied to these patients along the way. In a survey of over 700 patients meeting criteria for POTS, 83% reported being given at least 1 psychiatric diagnosis prior to being diagnosed. ³⁴ This adds to delay in diagnosis, as in the same survey the average number of doctors seen prior to diagnosis was seven, and the average delay from time of symptoms to diagnosis was 50 months. Instead, address anxiety as a contributor to symptoms rather than the main issue present.

The pitfall of failing to communicate, both to the family and to the other treating physicians, should be avoided. As simple as it sounds, this is all too often a problem. Lab work checked with one provider is unnecessarily checked again with another. Medications are started by one specialist, stopped by another, and then restarted yet again. As the overseer of patient care, the primary physician can orchestrate this process to make sure treatment is proceeding in an orderly direction and not haphazardly. Communication to the patient is also important. Parents of course want to be involved in their child’s care, but an office visit spent mostly talking to the parents and not directly engaging the patient is a wasted opportunity. Instead, directly ask the patient how they are doing and gauge their level of understanding of orthostatic intolerance along the way. Our experience is that as patients themselves take more ownership of their diagnosis and treatment, overall patient improvement will follow.

Finally, avoid the trap of feeling like all of the treatment of the patient, including managing medications, must be within your scope of care. Once medications need to be initiated, this is often a good time for subspecialty referral if this has not already been enacted. However, overseeing all the medications, as will be discussed in the following section, is surely within the purview of primary care and becomes even more important as a regimen becomes more complex. This includes medications that were prescribed prior to an orthostatic intolerance diagnosis, such as stimulants for ADHD or anti-histamines for allergies. Knowing the deleterious side effects these medications can have on volume-sensitive patients, how necessary do they continue to be?

Coordinating the Care of the (Seemingly) Complex Patient Referred to a Specialist

Once a patient is referred to a specialist for care, it is tempting to turn over all the care to that physician. This is a mistake, as covered above, as the most effective care will be delivered via synergistic partnership involving the primary provider. We do have suggestions on how to make this partnership work well. The first is to simply see the patient back in the clinic. Communication with families only via phone or electronic medical record messages, or even worse solely through notifications from the specialist, ultimately leaves the primary physician out of the loop and deprives the patient of your expertise. By seeing the patient back in clinic, one can determine what is most important—is the patient actually improving? Not all specialists are equally adept at diagnosis and treatment, or sometimes a particular family and specialist will not mesh well, and thus the patient languishes. Identifying this predicament and directing care in a different direction is necessary in this situation. By seeing patients back in clinic, one can also gauge how well a family is understanding the diagnosis and treatment. Our experience is that orthostatic intolerance is not well cared for paternalistically, and instead must be done through education to best unlock potential for following a treatment plan. A family who returns from a specialist and has no further understanding of the diagnosis and treatment is a “red flag,” predicting failure to improve.

Co-management of a patient’s care can take various forms. Plans set in action by a specialist can be realized in the primary care office—for example with laboratory work. If a patient’s ferritin and 25-vitamin D level were low and supplementation started, have these supplements been adequate? Repeat labs can be done as soon as 4 to 6 weeks into treatment and changes made to the supplements based on these results.^35,36 Once started, supplementation is typically not completely stopped during the teenage years, with the exception being if iron deficiency was chiefly due to menorrhagia, which has subsequently been treated. Specialist plans can also be “double-checked.” This is particularly important with managing medications. Have too many medications been added? Polypharmacy tends to lead to overestimation of medical complexity (by providers and families) and even competing mechanisms of action of different medications for a single patient. Vigilance must be maintained for negative side effects of medications as well. We tell families that any medications that lower blood pressure, increase heart rate, or depletes volume status can potentially make symptoms of orthostatic intolerance worse. Typical medications we see on patients’ lists that fit this warning include stimulants for attention deficit, anti-histamines for allergic rhinitis, medications for sleep or nightmares (clonidine, prazosin), diuretics (acetazolamide for possible pseudotumor cerebri or spironolactone for acne), and many medications used for joint pain, headaches, and gastrointestinal discomfort (amitriptyline, gabapentin, oxcarbazepine, topiramate, etc.). Some of these medications may in fact be needed, but it is exactly the role of the primary physician to make this judgment by considering the entire clinical picture. Finally, continuing to address mental health is extremely important. Seeing specialists can sometimes only heighten anxiety; as an example, patients can take on the identity of a complex and chronically ill person, which by its nature can potentiate feelings of being unwell. We do well to keep such patients grounded—orthostatic intolerance is not a lifelong affliction and certainly not a diagnosis without hope and treatment. Primary physicians can foster this outlook in their patients and make sure mental health treatment is being given to those in need.

Conclusion

High quality care of orthostatic intolerance can be delivered in the primary care office by following particular strategies. These include identifying positional dizziness as a specific and sensitive symptom, explaining the pathophysiology well (to strengthen treatment compliance through understanding and alleviate anxiety about the condition itself), and finally emphasizing bedrock treatment strategies as described above. A copacetic specialist is sometimes necessary to further a patient’s improvement, but does not replace the primary physician as the patient’s most trusted provider.

Supplemental Material

sj-docx-1-jpc-10.1177_21501319241299527 – Supplemental material for How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office

Supplemental material, sj-docx-1-jpc-10.1177_21501319241299527 for How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office by Camden Hebson, Kelly Muterspaw, Alexander Kuo, Polly Borasino, Khalisa Syeda and Toren Anderson in Journal of Primary Care & Community Health

Supplemental Material

sj-docx-2-jpc-10.1177_21501319241299527 – Supplemental material for How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office

Supplemental material, sj-docx-2-jpc-10.1177_21501319241299527 for How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office by Camden Hebson, Kelly Muterspaw, Alexander Kuo, Polly Borasino, Khalisa Syeda and Toren Anderson in Journal of Primary Care & Community Health

Supplemental Material

sj-docx-3-jpc-10.1177_21501319241299527 – Supplemental material for How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office

Supplemental material, sj-docx-3-jpc-10.1177_21501319241299527 for How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office by Camden Hebson, Kelly Muterspaw, Alexander Kuo, Polly Borasino, Khalisa Syeda and Toren Anderson in Journal of Primary Care & Community Health