Treatment:

Treatment may include:

• Protecting the person from injury
• Treatment of aspiration pneumonia
• Anticonvulsant therapy if seizures are present
• Medicines, including liquid tears, to prevent dry eyes
• Medicines called anti-emetics, to control vomiting

Postural hypotension (low blood pressure when standing) can be managed with increased fluid and salt intake, caffeine, and waist-high elastic stockings

Expectations (prognosis):

With advances in diagnosis and treatment, survival continues to improve. Currently, a newborn with Riley-Day has a 50% chance of reaching age 30.

Complications:

The following symptoms of "autonomic crises" occur in about 40% of patients:

• Excessive sweating of the head and torso
• Blotching of the face and torso
• Mottling of the hands and feet
• Hypertension (high blood pressure) and tachycardia (rapid heart rate)
• Nausea/vomiting
• Severe dysphagia/drooling
• Irritability
• Insomnia
• Worsening of muscle tone

Calling your health care provider:

Call your doctor if symptoms change or worsen.