Overview and treatment of behavioral problems in Smith-Lemli-Opitz Syndrome Elaine Tierney MD Kennedy Krieger Institute Johns Hopkins Universtiy School of Medicine Baltimore, MD 4/4/2019

-Increase communication abilities: Sign language, pictures, or objects Behavioral techniques can be powerful Physical restraints can help self-injury.   4/4/2019

Sometimes behavior is because of the child's mood or obsessions or the cause may not be known. Cholesterol is needed for serotonin "keylocks" (receptors) and other keylocks to work properly in the brain. 4/4/2019

You can consider medicine if behavioral treatment isn’t working and a child has one of the three criteria. The child is 1. suffering 2. a danger to herself or others 3. impaired in school or at home 4/4/2019

All medication trials are “trials " All medication trials are “trials ". It is up to the parents to decide if they see enough benefit that they think is worth the risk of using a medicine. 4/4/2019

I ask questions about: sleep, behavior, energy, appetite, moodiness, irritability, crying spells, concentration, self-injury (including skin injury, eye problems), aggression, “obsessions”, repeating behaviors. 4/4/2019

I also ask 1) what medical and psychiatric illnesses run in the family, 2) medical illnesses the child has – including allergies, sinus infections, ear infections, GE reflux, headaches and seizure disorder. 4/4/2019

Thyroid problems can cause mood and sleep problems so I get a thyroid screen blood test (TSH) 4/4/2019

Sleep Sleep problems are seen in half of individuals with SLOS  Sleep Sleep problems are seen in half of individuals with SLOS. Behavioral techniques: bright light/sunlight in the morning limit napping during the day . 4/4/2019

Clonidine can be helpful Benedryl can be used (but can agitate) Clonidine can be helpful Benedryl can be used (but can agitate). Melatonin up to 9 mg 1 hour before bed 4/4/2019

Medicines in the family of "neuroleptics" have a side effect of sleepiness but also appear to help correct the sleep pattern: These medicines are used to treat bipolar disorder, schizophrenia, and confused states when ill. 4/4/2019

Geodon (ziprasidone), Abilify (aripiprazole), Zyprexa (olanzapine) Haldol (haloperidol), Risperdal (risperidone), Seroquel (quetiapine), Latuda (lurasidone) 4/4/2019

Neuroleptics all can cause: a temporary muscle stiffness (EPS) a movement disorder that might not go away (Tardive Dyskinesia) Neuroleptic Malignant Syndrome 4/4/2019

You can obtain “blood levels” of neuroleptics such as Risperdal (risperidone) and Zyprexa (olanzapine). 4/4/2019

Treatment for self injury and aggression: Self-injury can happen with ear infections, allergies, and sinus infections. Neuroleptics help self injury and aggression. 4/4/2019

Behavioral techniques can be very helpful Behavioral techniques can be very helpful. Head hitting can damage the ear drum and eyes (retinal detachment with the back of the eye comes loose). 4/4/2019

Obsessions and compulsions: An obsession is a thought that comes to you for you to do something when you don't want to do. A compulsion it is the act of doing what you're thinking of. 4/4/2019

Compulsions: opening and closing doors and drawers, turning light switches on and off, emptying out bottles, repeating behaviors, throwing things away, touching things and collecting things. 4/4/2019

Medicines for obsessions and compulsions and anxiety Medicines for obsessions and compulsions and anxiety. Medicines in the family of Prozac are used for OCD. The SSRI Lexapro (escitalopram) is a good choice. A neuroleptic or lithium can be added to the SSRI to help treat the OCD. 4/4/2019

Pervasive developmental disorders: autism, PDD, Asperger syndrome Autism describes symptoms seen together: social difficulties, communication difficulties, and unusual or repetitive behaviors. It is not a separate disorder.   4/4/2019

Anxiety Disorders and Selective Mutism. -Anxiety is often seen, -Often it is socially related anxiety -Selective mutism is when a person can speak in some settings but does not speak in other settings (often speaking little at school). 4/4/2019

-Cognitive behavioral therapy [CBT] can help; -Antidepressants –Prozac, Lexapro; Celexa, Zoloft, Paxil -Benzodiazepines - Valium (diazepam), Ativan (lorazepam) can help anxiety and maybe selective mutism (but they are not often used in children). 4/4/2019

Pervasive developmental disorders: autism, PDD, Asperger syndrome Autism describes symptoms seen together: social & communication difficulties with unusual or repetitive behaviors. It is not a separate disorder. 4/4/2019

1 out of every 10 we can find the cause of the autism. Causes are fragile X syndrome, Rett syndrome, chromosomal abnormalities, and SLOS. Half of individuals with SLOS have been diagnosed with autism. 4/4/2019

Autism classrooms are smaller in size, emphasize visual instructions. You can use a picture board that shows the activities of the day. An autism school program usually has social skills training. 4/4/2019

Stressful or loud events: traveling and haircuts: DVD, favorite music Benadryl (diphenhydramine). Ativan (lorazepam); highest dose is usually 0.025 mg per kilogram (0.0125 mg for every pound for each dose). Needs at least 4 hours between doses. 4/4/2019

MOOD disorders: Medicines for depression are antidepressants (Prozac, Wellbutrin, Celexa, Lexapro, etc.) help to correct brain chemistry that is abnormal from SLOS.   4/4/2019

Exercise can also be helpful for depression. For people who feel depressed in the winter (have seasonal depression) light boxes can be helpful (has side effect of worsening bipolar disorder). Exercise can also be helpful for depression.   4/4/2019

Bipolar disorder is an illness in which people can feel angry irritable and at other times overly happy. Many people with SLOS look like they have bipolar disorder. This is even when they don't have a family history of bipolar disorder 4/4/2019

Medications for Bipolar disorder: lithium, Depakote (divalproax sodium), Depakene (valproic acid), Trileptal (oxcarbazepine), Tegretol (carbamazepine), Lamictal (lamotrigene). 4/4/2019

Attention deficit hyperactivity disorder -attention problems & hyperactivity -or just attention problems. -Can be looking & not attending well. -Girls tend to have attention problems without as much high activity. -May be due to delayed processing time  4/4/2019

- -Methylphenidate family (Ritalin, Metadate, Concerta, Focalin) -dextroamphetamine family (Dexedrine, Adderall [mixture of dexedrine salts],Vyvanse -clonidine -guanfacine (Tenex- is short acting), Intuniv is long acting) -Strattera - 4/4/2019

Can make it harder to see the vein. Consume a lot of water in the a.m. For Blood tests, vaccines, procedures: Gebauer Pain Ease or othertopical skin refrigerant spray, or Emla (lidocaine/prilocaine) cream can numb the skin of the arms. Can make it harder to see the vein. Consume a lot of water in the a.m. 4/4/2019