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 antiemetics, 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

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 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.