System/concern | Manifestation | Treatment |
Gastrointestinal | Vomiting and gastroesophageal reflux disease (GERD) | - Maintainence of upright positioning during and after feeding
- Consideration of proton pump inhibitors in those age >1 year
- Nissen fundoplication with gastrostomy tube (G-tube) placement
|
Dysphagia | - Modifying texture and thickness of foods using commercial thickening agents can help with swallowing difficulties
- Swallowing ability may be improved by providing tiny tastes of food or juice several times per day in nonfeeders
- Consideration of nasogastric tube or gastric tube placement
|
Constipation | - Maintain appropriate fluid intake
- Consideration of osmotic laxatives and/or use of stimulant medication in those with refractory constipation
|
Neurologic | Spasticity | - Baclofen and clonazepam may improve global spasticity
- Botox injections at specific sites may be considered; spasticity typically decreases for 3 to 5 months after each injection
|
Neuropathic pain; seizures | - Standard antipileptic medications; monotherapy is preferred, if possible
- Gabapentin may decrease neuropathic pain and can be used for control of seizures in some
|
Musculoskeletal | Contractures | - Positioning devices (wedges, rolls, and cushions) to decrease spasticity and prevent contractures
- Physical therapy
|
Respiratory | Excessive airway secretions | - Chest physiotherapy
- Postural drainage
- Suctioning device
|
Genitourinary | Urinary tract infections | - Bladder massage (Crede maneuver) to encourage complete bladder emptying
- Intermittent catheterization
|
Eyes | Delayed pupillary response, difficulty with upward gaze and palpebral weakness | |
Corneal ulcers | - Dark glasses to help reduce photophobia
- Eye lubricants or protective ointments
|
Dental | Delayed dentition | |