Postural orthostatic tachycardia syndrome (POTS) is a type of dysautonomia, which is a malfunction of the autonomic nervous system (ANS). The ANS automatically controls our heart rate, blood pressure, gastrointestinal motility and other automatic functions of the body. POTS is the most common form of dysautonomia, affecting between 500,000 and 1 million individuals in the United States.
The cause of POTS is unknown. For some patients, symptoms start after the onset of puberty. Some patients develop the condition after having a virus, like mononucleosis (Epstein-Barr virus), or in association with joint hypermobility, where joints are very stretchy (sometimes referred to as “double-jointed”). Others have it after repeated brain trauma or concussions. And a small group of patients have POTS associated with a metabolic disorder, a disease in which abnormal chemical reactions occur in the body.
POTS is associated with many symptoms. Patients frequently have difficulty standing upright, resulting in a rapid heart rate with lightheadedness, nausea, fatigue, dizziness, or fainting. Because of these symptoms, many patients struggle with daily activities and may be unable to work, attend school or participate in recreational activities.
The symptoms of POTS can be unpredictable — they may come and go, appear in any combination and vary in severity. Often patients will have more symptoms after a stressor or a physical activity, but the exact triggers that cause the onset of POTS are unknown.
Symptoms of POTS vary widely from person to person and are often invisible to the untrained eye. Many POTS symptoms are also associated with other diseases and conditions, so it is important to realize that having these symptoms does not mean that your child has POTS. Consultation with your child’s doctor is important to accurately diagnose POTS.
Symptoms may include a combination of the following:
- Fainting or passing out (syncope)
- Fast heart rate (tachycardia)
- Chest pain
- Severe fatigue
- Difficulty concentrating (“brain fog”)
- Feeling worse in very hot or very cold temperatures
- Feeling worse with exercise
- Insomnia (difficulty getting to sleep or staying asleep)
- Nausea or vomiting
- Diarrhea or constipation
- Feeling full quickly with eating (early satiety)
- Blood collecting in the legs (venous pooling)
- Joint or muscle pain
- Worsening symptoms with bright light or loud sounds
For up to 75 percent of patients, POTS symptoms may improve, or even disappear, by age 21 to 25 years.
Obtaining a thorough patient history is an essential first step in diagnosing POTS.
Clinical criteria for diagnosing POTS includes orthostatic intolerance, which is the development of a faster than normal heart rate within 10 minutes of standing. The combination of patient history and the clinical criteria help to make the diagnosis of POTS.
Because the exact cause of POTS is unknown, the common treatment approach is to focus on managing the symptoms. Treatment options are highly individualized depending on your child’s unique needs.
Your child’s symptoms will help determine which specialists she should be referred to for further evaluation and care. For example, POTS can be associated with chronic headaches, connective tissue disorders, mitochondrial disorders, a history of concussion and gastrointestinal disorders, so patients may need to see physicians from a variety of specialties who treat each type of symptom.
Anxiety and depression can also be associated with having a chronic disease like POTS, so your child may need to work with a psychiatrist or behavioral medicine specialist.
POTS symptoms can be controlled using a combination of non-pharmacologic (drug-free) treatments and medications.
Your child’s care team may recommend that your child use a combination of the following drug-free interventions:
- Drink 60-80 ounces of fluid (water or milk) each day, and have salty snacks (pretzels, saltines, pickles). Salt tablets are also an option, but many patients prefer salty snacks because salt tablets can cause an upset stomach.
- Don’t skip meals.
- No caffeine — it’s a diuretic, so it makes you urinate and empty your “tank” of fluid.
- Raise the head of your bed on something firm, like cinder blocks, to help reduce dizziness in the morning when you get up.
- Have a consistent bedtime and amount of sleep (10 to 14 hours for adolescents). If you have trouble sleeping, insomnia can be treated with good sleep habits:
- Lower the lights one hour before bedtime.
- Engage in a relaxing activity before bed, such as reading under low light, massage, meditation, yoga, stretching or a warm bath.
- Turn off the television, computer and video games, and stop cellphone use.
- When it is time for bed, the room should be dark (no night lights) and cool, but not cold.
- Melatonin can be used as a sleep aid. Consult your doctor before taking melatonin.
- Avoid triggers that worsen POTS, including extreme heat or cold and alcoholic beverages.
- Start with a small amount of aerobic exercise, such as ten minutes per day, using a recumbent bicycle, rowing machine or swimming. Leg and core strengthening exercises are also important. The goal is to increase slowly until you can perform 60 minutes per day. For patients with joint hypermobility, using the Dallas POTS exercise protocol (a widely used approach developed by Benjamin D. Levine, MD, director of the Institute for Exercise and Environmental Medicine at Texas Health Presbyterian Hospital and professor of medicine at the University of Texas Southwestern Medical Center) in conjunction with physical therapy, may help you to stabilize and protect your joints. Exercise makes a large difference in overall well-being and may be one of the most significant factors in helping to resolve POTS. Exercise is also thought to play a role in possibly resetting the autonomic nervous system.
Compression stockings may help with dizziness and can also help keep you warm. These waist-to-toe stockings should be worn while awake and are available by prescription.
Patients who cannot tolerate the heat may find cooling vests helpful. One type has gel inserts that can be cooled in the freezer. There are also evaporative cooling vests. Some insurance companies cover these with a prescription.
Medications are used to reduce or manage the symptoms of POTS.
The goal is to match the medications with the symptoms that they cover, and to find the right doses and combinations that control your child’s symptoms. Some patients with POTS are very sensitive to medications, so it is prudent to start with low doses to avoid side effects.
Once the medications help to reduce the symptoms, the next part of treatment is exercise, as described above. The goal is to reduce symptoms in order to make daily life easier for patients so that they can work, go to school, and be an active part of their families.
It is important to let your child’s care team know about any side effects from therapies so that adjustments to treatment can be made as needed.
Coping with a chronic disease is stressful, and many families find counseling to be helpful. It is important to know that your child’s symptoms may vary from hour to hour, day to day, and week to week. Some patients also experience what is called the “October slide,” worsening as the autumn progresses.
It may be difficult for family members, friends or teachers to understand these changes, and some may question whether the symptoms, or the illness, are real. Your child’s care team may be able to provide education and resources to your child’s school or family members to help them understand POTS and how they can best support your child. Some patients even give talks in their schools to raise awareness about POTS.
This website offers educational videos, patient resources, information about research grants and patient recruitment, links to research publications, and ways to connect and get involved.
Dysautonomia Information Network
DINET is a volunteer run nonprofit organization that raises awareness of autonomic nervous system dysfunction and promotes dysautonomia education, support, and networking.
The Dysautonomia Youth Network of America
DYNA’s programs benefit all individuals impacted by dysautonomia conditions through medically-endorsed resources, programs, and events.