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Poor weight gain
| Feeding therapy | The treatment of feeding issues in infancy & childhood depends on the cause (e.g., disordered suck & swallow, textural aversion, gastroesophageal reflux, hypercalcemia). |
Developmental delay /
Intellectual disability
| Developmental disabilities should be addressed by early intervention programs, special education programs, & vocational training. Recommended therapies include speech-language therapy, PT, & OT. Consider hippotherapy (use of equine movement during speech-language therapy, PT, &/or OT). Verbal strengths can be used to assist in learning spatial tasks. Phonics methods are recommended to teach reading. 1 Mastery of ADL contributes to adult well-being & should be encouraged.
| See also Developmental Delay / Intellectual Disability Management Issues. |
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Behavioral & psychiatric manifestations
| Treatment per psychologist &/or psychiatrist Behavior in young children may be addressed using techniques based on applied behavior analysis. 1 Behavioral counseling & psychotropic medication are often used to manage behavior issues, esp ADHD & anxiety, which require pharmacologic treatment in ~50%. 2, 3 Self-calming techniques can help manage anxiety.
| See also Developmental Delay / Intellectual Disability Management Issues. |
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Sleep disturbance
| Mgmt per sleep specialist | Consider melatonin therapy. 4 |
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Cardiovascular
| Mgmt of SVAS & other cardiovascular disease per cardiologist |
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| Hypertension is usually treated medically. 5 | |
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Risk of adverse events w/sedation or anesthesia
| Anesthesia consultation for surgical procedures Electrocardiogram prior to surgery Use of a center equipped for cardiopulmonary resuscitation
| Guidelines for sedation & anesthesia risk assessment & anesthetic mgmt for WS have been published. 6 There is ↑ risk for myocardial insufficiency & cardiac arrest in persons w/biventricular outflow tract obstruction, esp during induction of anesthesia.
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Eyes
| Hyperopia is treated w/corrective lenses. Strabismus is treated w/patching of 1 eye or surgery. Standard treatments for lacrimal duct abnormalities
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Hearing
| Recurrent otitis media may be treated w/tympanotomy tubes. Hypersensitivity to sounds may be treated w/ear protection when ↑ noise levels can be predicted. Hearing aids may be helpful per otolaryngologist.
| Community hearing services through early intervention or school district |
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Dental issues
| Dental care may require assistance w/daily brushing & flossing. Dental cleaning frequency should be ↑ to every 4 mos in adolescents & adults. Orthodontic referral for treatment of malocclusion.
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Gastrointestinal manifestations
| Constipation treatment usually incl dietary ↑ in water & fiber followed by osmotic laxative treatment. | Constipation must be aggressively managed at all ages due to ↑ risk for early-onset diverticulosis/diverticulitis. |
| The treatment of abdominal pain in children & adults depends on cause (e.g., gastroesophageal reflux, hypercalcemia, hiatal hernia, &/or diverticulitis). | Severe abdominal pain may indicate diverticulitis &/or intestinal perforation, which may occur at a young age in WS. |
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Urinary tract abnormalities
| Investigation of lower urinary tract (voiding cystourethrogram) in those w/febrile urinary tract infections to direct treatment Mgmt of nephrocalcinosis, persistent hypercalcemia, &/or hypercalciuria per nephrologist
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Musculoskeletal manifestations
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Hypercalcemia
| Avoid vitamin supplements w/vitamin D, esp in young children. Assess hydration status & ↑ water intake as indicated. Adjust diet w/nutritionist to maintain calcium intake no higher than 100% of RDI. 7 If serum calcium remains ↑, dietary calcium should be ↓, but serum calcium must be monitored. Parents should be counseled not to restrict dietary intake of calcium w/o medical supervision. Refractory hypercalcemia may be treated w/oral steroids. Referral to endocrinologist &/or nephrologist for treatment of persistent hypercalcemia, hypercalciuria, &/or nephrocalcinosis
| If vitamin D deficiency is suspected, check vitamin D levels prior to initiating therapy, & monitor calcium levels during treatment. Absorption of calcium from the gut is ↑ in WS (cause unknown) & vitamin D promotes calcium absorption. Intravenous pamidronate has been used successfully to treat infants w/severe symptomatic hypercalcemia. 8
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Early puberty
| Treatment w/gonadotropin-releasing hormone agonist 8 | |
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Hypothyroidism
| Oral thyroxine therapy | Subclinical hypothyroidism typically is monitored but does not require treatment. |
Insulin resistance /
Diabetes mellitus
| Exercise & balanced diet Mgmt per endocrinologist
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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.
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