Preparation and Approach to Testing
Many preparations, strategies, and accommodations can be used to maximize the chances for a successful and valid clinical assessment of someone with Fragile X syndrome. These considerations are described in more detail below.
Unique aspects of Fragile X syndrome are anxiety, hyperarousal, and sensory processing difficulties. It is critical to consider these during testing. Many individuals are already anxious prior to the start of an evaluation. Eye contact, touch, unexpected noise, as well as the fatigue that may come with performing physically and emotionally difficult tasks can all lead to increased hyperarousal.
Paying attention to signs such as increased perspiration (especially in the palms), redness in the cheeks or ears, rapid breathing, increased gaze avoidance, or attempts to avoid the task with conversation (often seen in higher functioning or more verbal individuals) can help the assessor intervene earlier by minimizing eye contact, using side conversations, increasing breaks, offering rewards, etc., to reduce anxiety and hyperarousal.
In a comprehensive clinical assessment, various tasks can be interspersed in the evaluation with intentional breaks that include opportunities to move. The assessor should pace the testing session to maximize performance and maintain ease and engagement of the person being tested. Experienced assessors familiar with the needs of those with Fragile X syndrome are adept at taking proactive breaks to maintain the pace and flow and allow for a productive assessment experience.
Observing the child’s response patterns while attempting a task is valuable in assessing how the child solves a problem. This provides valuable diagnostic information when developing school strategies to improve learning.
In general, parents and other caregivers, and those conducting the assessment, should always be sensitive to the child’s need for breaks and rest, food and beverage breaks, and a comfortable environment free of distractions and neither too warm nor too cool.
Except for the very youngest of children, who may not understand verbal descriptions of a planned visit to a unique setting (if that is the case), children and adults with intellectual disability may benefit and have lessened anxiety if they receive an advance description of what will be happening. However, in some cases, such a description can actually increase anxiety. Therefore, parents will need to make a judgment call about how much and when they should begin to prepare their child for an assessment session.
Lastly, the assessor should allow appropriate time for the individual to become comfortable in the testing environment and to develop appropriate rapport prior to starting the assessment.
Presenting tasks with clear verbal and visual supports that denote beginning and end can be particularly useful when assessing individuals with Fragile X syndrome. For example, provide empty boxes that are crossed off (or filled with a sticker of their choice) after completing a task. For example: Telling them “when all the coins are in the box,” or after counting together to 10.
Consideration of the testing environment itself is also imperative, as sensory sensitivities and hyperarousal can impact performance. Reducing the amount of light, closing blinds to eliminate visual distraction, positioning the assessor between the individual and the door, and decreasing auditory interruptions (such as announcements over a speaker or loud fans or clocks) will help to encourage the best effort of the person being assessed and promote focus and sustained attention. Utilizing the expertise of an occupational therapist on the team to train the assessment team on how to support sensory needs and manage anxiety and hyperarousal can be very helpful.
Young children with Fragile X syndrome may be hyperactive and may be better able to respond to test items if they are not confined to a chair; others will benefit from the added structure and confinement of a seated position. These young children often need a lot of breaks, and all individuals with Fragile X syndrome may do best with testing spread across one or more days to minimize fatigue and frustration.
Many people with Fragile X syndrome respond well to humor or to comments about favorite interests, which can help to reduce anxiety and improve rapport.
Prior to the Test
The assessor should obtain information from the caregiver about expected challenges and past experiences with testing and should collect information about items or activities that may be especially motivating to use as rewards during the testing process.
It will be important to communicate with the caregiver about what to expect of the testing environment and schedule. Some assessors utilize a visual schedule that can be sent ahead of time to preview what will happen in order to reduce anxiety and increase predictability. To minimize anxiety and increase rapport, one idea is to send a friendly photo (or link to photos), or a short video of the assessor and testing environment before the testing day. People with Fragile X syndrome are often remarkable in their ability to recall names and faces and this can be used to ease anxiety, as they will often recognize the assessor immediately upon introduction.
It is important to establish whether the caregiver will be present in the testing room. Some younger children or particularly anxious individuals may need this to even enter the testing room, and thus will require the caregiver to be present throughout. Others will do much better without the caregiver present. Finally, a third group seems to do well with the caregiver initially present and then later excused.
Flexibility from the assessor is crucial, and the assessor may have to use different strategies throughout the process. For example, multiple breaks may be successful at the beginning of testing, but later may need to be reduced in order to maintain attention and motivation.
The assessment team may consider gathering information about interest areas and favorite topics so that they can personalize the materials and interactions, as familiarity can help to establish rapport.
To name just a few considerations, on test day the person being assessed should be:
- As rested as possible
- Adequately fed (and prepared with snacks)
- Equipped with any necessary vision correction (glasses, contacts) and communication devices
- Physically healthy
- Following their typical medication protocol
The amount of time for testing may be either much shorter than usual for a typically developing person (if the person with Fragile X syndrome is unable to progress very far on test items), or it may be much longer than usual (if the person is agitated, needs many breaks, etc.) so it is best to schedule more time than typically expected if possible, and to be flexible regarding breaks and pauses during the assessment.