Behavioral Issues in TSC
Is It Me or Is It TSC?
Often times, as a parent you may wonder if the behaviors you are witnessing are “normal” age-expected behaviors or an ominous sign of TSC. You may also wonder if it is a matter of “can’t” vs. “won’t.” In other words, “Is it that my loved one can’t follow through with my request or he/she just won’t?” These are natural questions important in deciding what you can do now.
In many cases, individuals may exhibit both age-expected and TSC-associated behaviors. Similarly, there may be some things they can’t do yet and there may be others they won’t do. Careful evaluation across multiple settings (home, community and the office) is the key to deciphering these complex situations. The results of this evaluation are critical to crafting a comprehensive treatment plan that targets:
- the “age-expected” and “can’t” behaviors with time/patience/waiting it out and counseling,
- the “won’t” behaviors with behavioral intervention, and
- the TSC-associated behaviors with surveillance screening and targeted medications if indicated.
Autism spectrum disorder, ADHD, anxiety, social withdrawal, aggression, self-injurious behavior and mood swings may develop in individuals with TSC. How these behaviors differ due to the presence of TSC has not been the focus of many research studies; however, our clinical observations suggest some behaviors may be directly related to mechanisms that underlie the development of TSC, possibly making them more resistant to conventional interventions. For example, we reported the case of a young man who exhibited severe aggression and self-injurious behavior. His aggression was most responsive to behavioral intervention; however, self-injury decreased with the addition of everolimus (a targeted therapy for TSC). We have also observed individuals with TSC-associated anxiety whose symptoms are actually worsened by first line anti-anxiety agents. Systematic screening for these conditions is warranted. This can be achieved by using the TSC-Associated Neuropsychiatric Disorders (TAND) Checklist. More data is needed about the usefulness of targeted therapies and the effectiveness of traditional interventions for these conditions.
What Can I Do Now?
Both medication and non-medication options are available for behavioral treatment. The first step for parents to take is not a step at all: it’s just respite. Rearing children without medical conditions is not an easy feat; therefore, rearing those with medical and behavioral challenges can be a Herculean effort. Respite, for those not accustomed to taking breaks, may provoke intense emotions, such as guilt, worry, and/or fear. A slow start may help. Maybe allow someone to watch your loved one for 30 minutes to 1 hour in your presence in your home while you read a book or watch TV. Gradually, increase the duration and observe the interactions of the caregiver with your loved one until it feels natural. Once you are comfortable, set a goal to do something enjoyable for at least 1-2 hours weekly. A next step might be four weekend getaways a year. A week’s independent vacation in addition to a family vacation every year could be an ultimate goal.
Counseling for the individual and the family using individual or group therapy is an option that has been extremely helpful for those who have been willing to commit long-term. Engagement with family, friends, the local community and the TS Alliance has helped many families develop a strong support network. For example, one family was successful in using social media to obtain full financial support for epilepsy surgery.
Expectation/ability matching ensures the appropriateness of expectations and can be a critical component of non-pharmacological intervention. Imagine a child who only speaks English being placed in a Russian-only classroom. At first, the child may become bored and tune out or display hyperactivity causing a well-meaning provider to diagnose him with ADHD. Later, he may become disruptive as he realizes throwing chairs provides a quick and efficient means of escape. Similarly, expectations set well below an individual’s ability may result in undesired behaviors. The aim is to establish a set of expectations in the home and community that challenge an individual based on a clear understanding of his or her abilities and interests. Battle picking requires decision-making about which behaviors to target. Decide how the behavior affects the individual. Does it place him or her at risk for harm? Is it impeding their opportunity to learn? Is it prohibiting them from fully participating in the community? How are the behaviors affecting the family?
Safety Interventions to protect the individual from harming themselves or others is a top priority. Interaction with authorities may benefit from advanced planning. A visible medical alert bracelet and/or necklace can warn a first responder that your loved one has a medical condition. Another idea is provision of a medical letter to local authorities, especially police officers and firefighters. Ideally, these authorities could keep this information on file and work with your family to create a strategic, peaceful plan for engagement should the need arise. Potentially, a visit could be scheduled to the headquarters of these local authorities to allow them to get to know him or her and importantly, to allow him or her to understand the helpful role of these authorities. Hopefully, this familiarity could prevent worrisome outcomes from an escalation of behaviors coinciding with an escalation of force. Finally, access to a 24/7 crisis line could be very helpful. A range of services including care coordination, phone counseling and in-person response teams with behaviorally trained staff may be available.
Targeting behavioral difficulties in TSC is a challenge. Our approach is to employ a 3 in 1 (functional, symptomatic and organic) model for evaluation and treatment. A functional evaluation seeks to find environmental factors that may worsen or reinforce a behavior. By symptomatic evaluation, we refer to the diagnosis and treatment of co-morbid medical conditions, such as epilepsy, ADHD, anxiety or bipolar disorder. Organic evaluation and treatment, in this model, refers to TSC-related medical conditions. To exemplify this model, we published a case report of a young man with severe aggression and self-injury associated with TSC. Functional assessment, completed using direct behavioral observation revealed that aggression was maintained (reinforced or made worse) by escape and access to attention. No environmental factors were associated with self-injury. Epilepsy, bipolar disorder and intellectual disability were his symptomatic conditions. Organically, a non-resectable subependymal giant cell astrocytoma (SEGA) was present in the frontal lobe of his brain. His 3 in 1 treatment plan included a structured behavioral intervention to target his desire to escape from demands and access to attention (functional); antiepileptic medications for epilepsy; lithium and asenapine for bipolar disorder (symptomatic) and everolimus for the SEGA (organic). He responded exceedingly well to this treatment and was able to successfully transition to his residential school.1
In another example, a young lady with TSC began to experience decreased school performance and an intense aversion to going to school. The reason (function) for her resistance to attending school was to avoid peer interactions that resulted in painful teasing and exclusion. Symptomatically, she was diagnosed with generalized anxiety disorder and pragmatic language impairment. Her language impairment hindered her interaction with peers, and she had difficulty with information processing. Her epilepsy was well managed with carbamazepine. Organically, we did not identify any new or worsened features of TSC. Her treatment program included citalopram, a medication identified as particularly helpful in a population of individuals with TSC.2 Behaviorally, she engaged successfully in individual and group therapy to target her fear of social interaction. Currently, she is now able to engage in her previously enjoyable activities of performing as a singer and dancer. Therapists involved in her care benefitted from an understanding of her neuropsychological and language profiles to include modifications specific to her needs while providing cognitive behavioral therapy, including yoga in teaching relaxation skills, and in facilitating communication barriers that presented in group therapy. In addition, her mother was provided with occasional supportive therapy to process her feelings and frustrations, and to problem-solve when her daughter encountered difficulties while interacting with others. Provider and family investment in forming a team around the patient to ensure that the 3 in 1 approach is evenly and consistently implemented is essential in establishing effective communication about care and support of the patient. In our clinic, we have the benefit of interdisciplinary team members providing care within one system.
Screen early. One way to do this with your physician is by either providing them with or bringing a completed TSC-Associated Neuropsychiatric Disorders (TAND) Checklist 3 with you to your appointment.
Peel the layers. Be sure your providers are considering the multiple facets of behavior – organic, symptomatic and behavioral.
Explore targeted interventions. Discuss the potential clinical or research use of these medications with your provider.
Pace yourself. Remember there is a lot to be done, but it doesn’t have to be done in a single day.
Celebrate! When you see your child or loved one doing something well, applaud them enthusiastically. Every bright moment is worth noting.