Riley-Day syndrome
Alternative Names
Familial dysautonomia; Hereditary sensory and autonomic neuropathy - type IIITreatment
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
Outlook (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.
Possible 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
When to Contact a Medical Professional
Call your doctor if symptoms change or worsen.
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