Acute acidosis
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| Avoid lactate containing solutions (e.g., Ringer lactate). |
Epilepsy
| Treatment w/ASM byexperienced neurologist |
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Dystonia
| Benzhexol, baclofen, tetrabenazine, & gabapentin may be useful, alone or in various combinations. | An initial low dose should be started & gradually ↑ until symptom control is achieved, or intolerable side effects occur. |
Botulinum toxin injection has been used in persons w/LSS & severe intractable dystonia. | Use w/caution given potential side effects of exacerbating muscle weakness; & do not use in head & neck because of risk of adverse events (e.g., impaired swallow). |
Pulmonology
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Feeding/Nutrition
| Caloric & nutritional supplementation as needed (incl micronutrients) Feeding therapy as needed Gastrostomy tube placement may improve nutritional intake for those w/persistent feeding issues, choking, or aspiration risk due to dysphagia.
| Low threshold for clinical feeding eval &/or radiographic swallowing study when showing clinical signs or symptoms of dysphagia |
Development/Cognition
| See Developmental Delay / Intellectual Disability Management Issues. | |
Myopathy &/or ataxia
| PT & OT | Equipment to prevent falling & maintain independent mobility as appropriate |
Eyes
| Standard treatments for eye movement disorders as advised by ophthalmologist | |
Cardiomyopathy
| Anticongestive therapy or antiarrhythmic therapy may be required & treatment should be managed by cardiologist. | |
Gastrointestinal
| Stool softeners, laxatives for constipation as needed | |
Liver
| Treatments for liver failure as advised by hepatologist | |
Renal
| Electrolyte monitoring & replacement for renal tubular losses per nephrologist | |
Hearing
| Hearing aids or cochlear implants for sensorineural hearing loss Referral to speech therapy Referral to hearing support services
| Counsel to avoid excessive noise exposure, which can exacerbate hearing loss. |
Endocrine
| Treatment for diabetes mellitus or adrenal insufficiency managed by endocrinologist | |
Psychosocial
| Standard treatments of anxiety &/or depression | |
Family/Community
| Ensure appropriate social work involvement to connect families w/local resources, respite, & support. Coordinate care to manage multiple subspecialty appointments, equipment, medications, & supplies.
| Ongoing assessment of need for palliative care involvement &/or home nursing |