Riley-Day syndrome
Alternate Names : Familial dysautonomia, Hereditary sensory and autonomic neuropathy - type III (HSAN III)
Treatment
Treatment may include: - Anticonvulsant therapy if there are seizures
- Feeding in an upright position and giving textured formula to prevent gastroesophageal reflux
- Increased fluid and salt intake, caffeine, and elastic stockings to prevent low blood pressure when standing (postural hypotension)
- Medicines called anti-emetics, to control vomiting
- Medicines, including liquid tears, to prevent dry eyes
- Physical therapy of the chest
- Protecting the person from injury
- Providing enough nutrition and fluids
- Surgery or spinal fusion
- Treating aspiration pneumonia
Prognosis (Expectations)
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 complications occur in about 40% of patients with this condition: - Blotching of the face and torso
- Excessive sweating of the head and torso
- High blood pressure (hypertension) and rapid heart rate (tachycardia)
- Insomnia
- Irritability
- Mottling of the hands and feet
- Nausea/vomiting
- Severe difficulty swallowing (dysphagia), drooling
- Worsening of muscle tone
Calling Your Health Care Provider
Call your doctor if symptoms change or get worse. A genetic counselor can help clarify information about the condition and tell you how to contact support groups in your area.
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