Behavioral Problems in Sanfilippo Syndrome and How to Deal With Them

Children with Sanfilippo syndrome commonly have behavioral problems, which can have a negative effect on the lives of family members and caregivers.

Read on for more information about such behavioral problems, and ways to possibly manage them to ease their effects on your family and care team.

What is Sanfilippo syndrome?

Sanfilippo syndrome is a rare genetic disorder characterized by the buildup of a molecule called heparan sulfate. This buildup causes damage to a number of different organs in the body. The disease primarily affects the brain. This leads to symptoms such as developmental delays, seizures, and behavioral issues.

What behavioral problems might my child have?

Researchers have described several increasing behavioral problems in children with Sanfilippo syndrome. These include hyperactivity and restlessness, aggressive behavior, unusual laughing, screaming, or crying, destructiveness, mouthing and biting, lack of fear, and sleep disturbances.

A survey and follow-up study of children with different types of mucopolysaccharidosis received responses from 106 parents and caregivers of children with Sanfilippo syndrome. The behavioral problems of these children had devastating effects on the lives of the family.

It was reported that children were unduly aggressive, with some children striking people for no apparent reason. The researchers proposed that this may have been due to people invading the child’s social space. Many parents indicated that their children were restless and would wander around the house or when outside, and could not sit still. Children also would frequently mouth or bite objects. About 71% of children between the ages of 5 and 14 were still sucking their thumbs.

One of the most challenging behaviors discussed was sleep disturbances. Carers reported that many children stayed up all night, roamed around the house, and laughed or sang in the middle of the night.

How can we manage behavioral problems?

Successful management of behavioral problems is often reliant on knowing the underlying cause of the issue.

Some of the irritability and mood swings seen in Sanfilippo patients may be due to physical factors such as pain or increased pressure on the brain due to hydrocephalus – too much fluid around the brain. Painkillers can possibly help with pain, while a shunt can help reduce pressure on the brain.

Seizures also can sometimes result in changes in mood or abnormal behavior. Doctors can diagnose these effects using electroencephalography (EEG) to look for abnormal electrical activity in the brain. Antiepileptic medications may help reduce seizures in these patients.

Hearing loss also may be a factor in behavioral problems. Hearing aids or additional support for hearing impairments can help in these cases.

An altered circadian rhythm and natural melatonin levels may cause sleep disturbances. This may be treated with doses of melatonin. Consult your child’s healthcare providers to determine if this would be an appropriate treatment and to find the correct dosage for your child.

Sleep apnea also may play a role in sleep disturbances in some patients. Continuous positive airway pressure (CPAP) treatment may help. Medications to help with sleep also may help, but results in the literature have been variable.

Some practical interventions that parents can use to ease sleep disturbances are to remove extra furniture and objects from the child’s room at night. Using a video camera to watch the child, or sharing a room with children to supervise them also can be an option.

Other practical changes around the house can be to give the child a biting ring or toy to discourage mouthing of other objects or clothing. Try several models to determine what type and size aid works best. Also, limiting access to stairways and the outside of the home can help patients who want to wander to stay safe.

Behavioral interventions such as redirecting a child to another activity or rewarding him or her with praise or a prize, such as food or a toy, may help reduce negative behaviors and increase positive ones. Looking for patterns when children have behavioral problems may help determine the factors that are the possible causing issues. Your child may show distress or unpleasant feelings with a specific location or being in a new situation. Knowing such triggers can help parents and caregivers to address them.

Medications such as mood stabilizers and antipsychotic treatments have been used with partial success in Sanfilippo syndrome patients to control anxiety, aggression, and hyperactivity. In some female Sanfilippo syndrome patients who have already passed puberty, estrogen treatment may be helpful to reduce aggressive behaviors.

Last updated: Dec. 8, 2020

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Sanfilippo Syndrome News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.