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Behavior Medications 2

This letter is regarding my 10-year-old son with CdLS and his behavior medication. Last year he was on Adderall, Risperdal and Clonidine. He has a hard time getting to sleep and staying asleep. We recently changed him to Clonidine and Paxil. Behaviorally, he has worsened, very angry, hitting a lot, hardly sleeping, eating poorly, and more aggressive. He is having behavior intervention at home but he is so angry and out of control it is impossible for us to work with him. We may try to get him admitted to a hospital-based program. Can you give me any advice or recommendations for a new medication?

Most of the medication trials you mention sound appropriate but I'm concerned that they are being assessed in too brief a trial period. These medications, which may produce effects within the first few days, may also take many weeks to establish a genuine response that reduces intermittent outbursts like your son's. The reason is that the neuropsychiatric issues involved include both short- and long-term changes in brain "pathways." Medications (like Risperdal) often continue to develop their effectiveness for many months when used in diseases like Schizophrenia - we can expect them to take a while to change overall behavior in other situations as well.

I like to use a step-by-step protocol to evaluate and treat intermittent aggressive or self-injurious behavior in people with CdLS - although there is no real evidence that this protocol should be lavishly followed by others. The protocol is a "first things first," common sense approach to medication. The principle is "first, do no harm," "second, make sure you address any underlying causes" and "third, progress from simple interventions to the more complicated (and potentially dangerous) ones."

The following suggestions should always be matched by attempts at behavioral and "psycho-social" interventions since many times the source of irritation that leads to the outburst can be recognized and avoided. I'll always remember the child with malformed arms who would become out of control whenever her parents tried to put her shoes on. As I spoke to the parents, the little girl reached up onto my desk and grabbed my pen with her toes and played with it. Clearly, her "agitation" was because they were trying to cover her "hands" with leather covers that rendered her unable to interact with her world. She was much better off without shoes - problem solved.

That said, here is a basic protocol:
1) Document the pattern, if there is one - Is this situational? Is the agitation or aggression always at some time, some place, or with a particular person or persons? Look for environmental interventions.

2) Look for physical problems. People with CdLS have many of them. Pain, GI upset, stiffness, physical limitations, and even seizures can all lead to irritability and outbursts. This is more common in the kids who have fewer verbal means to express themselves, but is not unheard of in the mildest cases. Address the problems with cause-specific medical treatments.

3) Look for neuropsychiatric symptoms. Mood problems like depression, affective issues like excessive anxiety, problems in agitation or even misperception which sometimes respond to the "atypical anti-psychotic" like Risperdal or Zyprexa. I like to start with an intervention that is likely to cause the least side effects - but in people with CdLS, that is anybody's bet. Where appropriate, drugs like Clonidine (for restlessness) or a selective serotonin reuptake inhibitor (SSRI) are usually well-tolerated. I try to give the prescription a minimum of six (6) weeks to work - if it is at all possible. Any trial shorter than this, not stopped because of intolerable side effects, should be considered inadequate. If one SSRI does not work, try another or try Remeron (a unique antidepressant) that can help with sleep as well as appetite disturbance. I avoid benzodiazepines, (and other addicting drugs) but this should not be an absolute rule.

Kids with ADHD-like symptoms may respond to stimulants, but I would also consider the serotonin/norepinephrine reuptake inhibitor (SNRI) bupropion (Wellbutrin). Remember that agitation and "hyperactivity" look similar but are likely very different biochemically and respond very differently to medication. Not all "hyperactivity" is the same either; some can be due to deficits in focus, but other cases seem to respond more like "mania" in bipolar disorder. Again, the guiding principle is observation and trial of the least potentially-injurious approach. I reserve Adderal, Ritalin, Concerta, and the like, to diagnostic trials (or last in line approach for long-term use).

Lastly, kids with pure agitation, "psychotic" symptoms, or extremes in aggressive or self-injurious behavior may respond to the atypical anti-psychotic drugs. The choices are Zyprexa (my favorite), Risperdal, Seroquel, and Geodon (formerly known as Ziprasodone). Doses should start very low, but "underdosing" is common so I would not be afraid to try higher doses - slowly. The trial time should, again, be many weeks, not just a few days. Document, document, document the behaviors since "impressions" about whether the child is better or worse are notoriously unreliable.

4) Try the less traveled road. Some newer approaches: Probably the most interesting angle is the opiate agonists and antagonists and the anti-seizure drugs (for kid s who have no formal diagnosis of seizure disorder), Revia (which blocks opiate receptors), and the agonist-antagonist opioid drugs that can help calm some kids who have developed self-injurious aggression. Remember, it is common to see kids switch between SIB (self-injurious behaviors) and aggression toward others. I make few distinctions in the two when considering the potential interventions. The anti-seizure drugs Tegretol and Depakote, other newer drugs Lamictal, Neurontin, and several others, can have some calming effect - and are already being used in bipolar disorder manic episodes. I don't have much experience with these newer agents and kids having CdLS yet, but Depakote and Tegretol can be useful despite their sedating effects. I would warn people that aggression can be a result of over sedation (like a fussy two-year-old who has been up too late).

5) Again, reexamine the behavioral and environmental interventions. These kids do sometimes "outgrow" the aggression. There are the two typical periods for this behavior to peak, early (0-5 years old) and late (11-20 years old or older). Token systems, rewards for more appropriate behavior, and structure all can help kids to modulate the aggression - even when they don't seem to work at first.

The last word - Stick with various interventions for longer trials and assess with the best documentation and "objective" observations that you can. These are solvable problems and, while complex, they can be figured out.

DS/
TK 7-13-10



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