Blood Pressure Management for Familial Dysautonomia
Frequent swings in blood pressure are a hallmark of familial dysautonomia. People who have a sudden increase in blood pressure may become agitated, flushed, and sweaty, and may have vomiting episodes. When blood pressure drops suddenly, it can cause lightheadedness, weakness, fainting, and difficulty seeing clearly. Over time, these blood pressure swings can damage the heart and kidneys.
Doctors at NYU Langone’s Dysautonomia Center create personalized treatment plans for people who have this condition to help keep blood pressure from becoming too high or too low.
Blood Pressure Monitoring
Because of the complexity of the condition, NYU Langone doctors monitor people with familial dysautonomia who are being treated for high or low blood pressure closely and frequently.
Our experts conduct several tests to identify fluctuations in blood pressure while maintaining different positions and doing various activities. They may measure blood pressure in NYU Langone’s autonomic laboratory, which allows our familial dysautonomia experts to see how well the reflexes that control blood pressure are working. Our doctors may also recommend monitoring blood pressure with a portable blood pressure cuff you can use at home. The portable device takes and stores blood pressure readings throughout the day and night.
Our doctors also measure blood flow in the brain with an ultrasound test called transcranial Doppler. This noninvasive test can reveal whether symptoms, such as dizziness when standing upright, are caused by a drop in blood pressure and reduced blood supply to the brain.
In this test, a small probe is fitted on a headband. The probe sends a signal that can detect and monitor the amount of blood that is flowing to the brain.
Depending on the results of these tests, our doctors may give you advice on how to change body position slowly to prevent sudden changes in blood pressure and how to adjust your sleeping position to prevent changes in blood pressure. They can also provide training in relaxation techniques that can help you manage stress and lower your blood pressure.
Blood Pressure Medications
Our doctors may also prescribe medications to treat high or low blood pressure.
Medications such as midodrine can combat low blood pressure that occurs when standing up, which is called orthostatic hypotension. Doctors may prescribe this medication for use during the daytime before physical activities or when standing up for long periods of time.
Some people may need medication to lower blood pressure when it becomes too high. In the past, doctors often used powerful sedatives, such as benzodiazepines, to reduce blood pressure in people who have familial dysautonomia. That’s because blood pressure rises suddenly in response to emotions, such as happiness, excitement, fear, and anger. However, sedatives can cause serious problems, such as dependence, suppressed breathing, and even respiratory arrest, in which breathing stops.
As an alternative, NYU Langone doctors may prescribe carbidopa to prevent vomiting episodes caused by spikes in blood pressure. This medication blocks the body’s ability to make catecholamines—substances released by nerves that raise blood pressure and increase heart rate. Because carbidopa’s activity occurs outside of the brain, it is a safe alternative to sedatives.
Our doctors can also provide medical care to help people with familial dysautonomia safely reduce their need for sedatives.
Treatment for Kidney Complications
In some instances, blood pressure problems can cause irreversible damage to the kidneys. Our doctors may recommend kidney dialysis, which uses a machine to filter the blood, or kidney transplantation for children and adults with severe kidney damage.
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