Managing the First Years: Seizures, Feeding, and Growth

The first few years of life with Wolf-Hirschhorn syndrome (WHS) are often the most intensive in terms of medical management. During this time, the primary focus is on managing seizures, ensuring safe and adequate nutrition, and navigating the unique growth patterns of the syndrome.

Seizure Emergencies and First Aid

While many daily symptoms in WHS are not emergencies, Status Epilepticus (prolonged seizures or clusters without recovery) is a life-threatening medical emergency. High fevers and common infections are known to frequently trigger severe seizures in children with WHS ^[1].

Basic Seizure First Aid:

1. Time the event.
2. Protect from injury: Clear the area of hard objects and place the child on their side when safe to do so.
3. Do NOT restrain them or put anything in their mouth.
4. Follow your child’s written, individualized seizure action plan.

When to call 911 or seek emergency services:
You should always rely on the specific thresholds set by your neurologist in your written action plan. Generally, emergency services are needed if a seizure lasts longer than 5 minutes, if the child has difficulty breathing, if it is their first seizure, or if multiple seizures occur without the child waking up in between ^[2][3].

Managing Epilepsy

Approximately 92% of children with WHS develop epilepsy, usually beginning before 12 months of age ^[2][1]. The frequency of seizures often peaks around age one, and while they may decline in frequency later, epilepsy often persists and requires ongoing management ^[3].

Medication for WHS is highly individualized based on seizure type, age, and individual response. While observational caregiver reports show some medications (like clobazam or levetiracetam) are frequently used, there is no universal ranking, and medications like carbamazepine or phenytoin may still have a role in specific situations ^[4][3]. Never start, stop, or change an antiseizure medication without explicit guidance from your treating neurologist.

Feeding and Nutrition

Feeding difficulties are a hallmark of WHS, and most infants will need specialized support to grow safely ^[5]. Always consult with your feeding/swallow team before thickening feeds or altering food textures.

• Aspiration Risk: Because of low muscle tone and facial structures like a small jaw (micrognathia), many infants struggle with swallowing. This can lead to overt aspiration (coughing or choking) or silent aspiration (where food or liquid enters the lungs without any obvious coughing), potentially causing pneumonia ^[6][7]. An instrumental swallow study by a speech-language pathologist may be needed to properly assess this risk.
• Tube Feeding: When oral feeding is unsafe or inadequate for growth, a gastrostomy tube (G-tube) may be recommended. While a G-tube provides essential calories and hydration, it does not completely eliminate the risk of aspiration (such as aspirating one’s own saliva) ^[6][8].
• WHS Growth Charts: Standard CDC growth charts often do not accurately reflect the growth of a child with WHS. There are syndrome-specific growth charts that your doctor should use alongside standard charts and clinical nutritional assessments to monitor your child’s progress ^[9][10].

Anesthesia and Airway Safety

If your child requires any procedure involving sedation or anesthesia, extra precautions are necessary. Children with WHS often have a potentially “difficult airway” due to physical features like a small jaw, a tongue that falls back (glossoptosis), or a floppy airway (laryngomalacia) ^[11][6].

Before any surgery, you should arrange a preoperative assessment to review:

1. The increased risk of airway obstruction, requiring an individualized airway plan by an experienced pediatric anesthesia team ^[11].
2. Your child’s specific seizure medications and history.
3. Any existing heart defects, which require careful monitoring of blood pressure and heart rate during the procedure ^[12].