Executive Functioning and PKU What it is, how it effects us, and what we can do about it. Rachel Kaup, M.Ed., C.ABA The Parent Helper

RBK, Who am I?? What do I know?? An adult diagnosed with CPKU Been officially on-diet since diagnosed Often non-compliant to treatment from pre-teen years into the 20’s Went to grad school but didn’t finish Worked in various jobs after college Moved many times until early 30’s Earned Master’s degree mid 30’s First started to consistently comply mid 30’s First started to recognize difference in Executive Functioning….last year

RBK, What is Executive Functioning? The Basics The executive functions all serve a "command and control" function; they can be viewed as the "conductor" of all cognitive skills. Executive functions help you manage life tasks of all types. For example, executive functions let you organize a trip, a research project, or a paper for school. Often, when we think of problems with executive functioning, we think of disorganization. However, organization is only one of these important skills. ~Cooper-Kahn and Dietzel (2008), LD Online

RBK, What is Executive Functioning? 12 Executive Functions: Flexibility – The ability to be adaptable, improvise, and shift approaches to demands. Flexibility Goal-Directed Persistence --The ability to perservere on tasks that require sustained effort. Goal-Directed Persistence Metacognition – The ability to self-monitor and observe. Metacognition Organization – The ability to use a systematic approach for achieving goals. Organization Planning – The ability to develop a set of strategies in order to accomplish a goal. Planning Regulation of Affect – The ability to manage one’s feelings effectively for decision making and task completion. Regulation of Affect

RBK, What is Executive Functioning? 12 Executive Functions (cont’d): Response Inhibition (self-control) – The ability to stop or delay an action rather than display impulsive behavior. Response Inhibition Social Thinking– The ability to respond appropriately to social conditions. Social Thinking Sustained Attention – The ability to maintain one’s focus and attention in the presence of distractions. Sustained Attention Task Initiation (getting started) – The ability to initiate a task without procrastination. Task Initiation Time Management (prioritization) – The ability to respond to things in a timely fashion. Time Management Working Memory – The ability to remember something while performing an activity on this memory. Working Memory

RBK, How does this issue effect us? High levels (or phenylalinine in the brain) directly effects the neurological system, which in turn influences our everyday functioning For school age people, EF skills are a crucial part of academic success For working adults, depending on your job and/or EF skills you are stronger in, could be the basis of your status of employment! (Take out your Executive Functioning Survey)

RBK, 20107

8 Executive Functioning Issues from Dr. Susan Waisbren’s Presentation at the NPKUA Conference 1/17/10: Everyday planning of diet or treatment regiment Remembering Phe intake for records/management purposes Remembering to take formula Internal reasoning of intake of foods not allowed on diet, monitoring blood Phe, and making adjustments accordingly Maintaining supplies: preplanning, not taken off guard with running out

RBK, My Experience Early years: good cognitive function, compliant with diet and formula Preteens: Still ok cognitive function, starting to “experiment”, ok grades Teen years: non-compliance to diet begins, slipping grades Early Twenties: Started graduate school, left program after certification Mid-Late Twenties: “Limbo” Early-Mid Thirties: Grad school, again, difficulty with planning/org.

RBK, My Experience (cont’d) : Changing my life and “remembering” my disorder 2009-Present: Aha!! My diet DOES effect my life and everyday functioning!!..It’s about time I figure that out…

RBK, What we can do about it Drink Formula / Take Protein/AA Replacement Comply to personal diet! (easier said than done) Keep a visual record: this helps when your working memory is being effected by your higher phe intake, test your blood!! Stop and consciously recognize what, why (peer pressure can be a factor)and how your eating It’s a viscous cycle, if you’re not complying to your diet/therapy: it effects your executive functioning, which in turn, inhibits your ability to comply! UGGH!! Gotta do it!!

RBK, Survey Results Activity Add up each of the sections. Identify your 3 lowest scores and then your 3 highest. Now, each section represents an executive skill. Every one stand up and sit down when your strength has been mentioned as one of your strengths.

RBK, Conclusion My goal is for you to walk away with: Knowing what the Executive Functioning Skills are. Knowing your strengths Knowing your weaknesses The motivation to address each and be aware of how the diet effects (good or bad) your ability to function every day.