Supporting Children and Families Diagnosed with Juvenile Batten Disease

AER International Conference 2012

Bellevue, WA July 20, 2012 S

Overview

S Introductions S Basic Information About Juvenile Battens S Supporting Children by Supporting Families S Supporting Educational Teams S Transition Considerations S Resources

Introductions

Marjorie Newell,

Parent

Kathryn D Botsford,

TVI & COMS University of Northern Colorado, National Leadership Consortium on Sensory Disabilities Fellow Follow-up and Contact Information: [email protected]

What is Juvenile Batten Disease?

S

Juvenile Batten Disease

S Juvenile Neuronal Ceroid Lipofiscinosis (JNCL) S One of several degenerative double-recessive genetic neurological diseases collectively referred to as Batten Disease S The disease was described in 1826 in Norway, and documented in 1903 by a British pediatrician

Symptoms

S Early childhood development is typical or advanced S Age of Onset: 5 to 8 years S Early symptoms: Subtle vision loss, emotional or behavior changes, performance at school less than expected S A Retinitis Pigmentosa diagnosis

Diagnostics

S Testing: S Less invasive: ERG, EEG, urine or blood tests S S More invasive: skin, eye, or lymph node biopsies Genetic screening is available for siblings, potential carriers

Disease ‘Milestones’

S S S S S S Progressive blindness Slowing mental maturity Seizures Speech and motor impairment Dementia Death

Unique Challenges for Schools

S Issues around loss/guilt/grief for families S Issues around loss/grief for children S Issues around long-term support for families & children S Consistency or continuity S Trust & long term relationships w/ child & family S Issues around confidentially & privacy S Issues around family values S Importance of letting kids be kids

Supporting Children by Supporting Families Issues for Families, Children, & Schools

S

Extreme Grief

S The TVI may be working with the family before the student is diagnosed S The parents will be grief-stricken and overwhelmed S Parents have different ways of coping and planning for their child’s future, educationally, medically, and spiritually S Families need schools to be patient

Plan for the worst… hope for the best

S Adults will be planning for an outcome that is different than what the child knows or hopes for S Adults must accept and face the realities of the disease in order to plan for the child’s needs S Adults must allow the child to have a hopeful, happy childhood S While research is being done, no cure is on the horizon S Batten Disease is always fatal

To tell or not…

S Many parents choose not to tell their child that they have Batten disease S If that is the parents’ decision, confidentiality can be maintained by not using the name of the disease at school S Rather, describe the symptoms expected in the next year or two and the expectation that full physical care will be needed in the future

“Retirement Years”

S Self-satisfaction S Social interaction S Enrichment S Maintaining failing physical & mental functioning S Not about future planning

Why School?

• • • • School is Normal, Familiar, Structured, Comforting Peers and Learning Activities Resources for Enrichment The Family Needs the Break S

Least Restrictive Environment

LRE

S School Oriented—Early Stage S Inclusion with vision support S Personal Interest Oriented—Early Stage S Inclusion with separate space S School Oriented—Late Stage S Contained with separate space S Personal Interest Oriented—Late Stage S Separate space with visitors (home or school)

Issues with Full-Inclusion

As Batten Disease progresses

Inclusive Settings: S S S S S Accentuate losses Become stressful and overwhelming Appropriate peers change Behavior becomes more disruptive Complications from anxiety

Intervention Strategies

S ‘ Alone skills’ are important for their future S Least restrictive environment is the opposite of conventional thinking S Student’s focus becomes very narrow S S coping skills diminish disruptive behaviors increase

Age Appropriate?

S Memory: Long- and Short-Term S Maturity Plateau & Regression S Perseverative or Obsessive Thoughts/Interests S Unable to learn new concepts. S Later Stages: Benefit from having the same activities and supplies at school as at home. S They still want to be treated their age in some ways.

Parent’s wish list for School

S Accept the fatal outcome, plan accordingly, and don’t dwell on it at school S Don’t talk around the kids S Dispel myths (parents used drugs, parents are too emotional, “spoiling”) by assuring school personnel that the IEP goals are appropriate for the student.

S Continuity and consensus on the IEP team S Build an environment of trust and respect between parents and school S Help with annual transitions (new teacher, new building, new placement, new Para – all difficult)

Wish List

S Respect confidentiality S Para – good match, continuity, trained for seizures, confidentiality, professional boundaries S Para/Teacher/Parent Communication facilitates student participation & elevates frustration S Careful placement. Most parents, in hindsight, felt they chose inclusion too long.

Role of the Vision Team

S

Supporting Educational Teams

S Overall goal: Enhancing the child’s well being and providing enrichment in an educational setting, while minimizing stress and anxiety

Individualized Education Plans

Early Stage S IEPs support academic goals to facilitate classroom participation with peers Late Stage S IEPs goals worded to direct adult service provision

Specific Goals Focused on:

S Skill Maintenance: using motor, cognitive, and communication skills in a daily routine of enjoyable, successful activities S Social and Emotional: activities with groups and important peers; requesting assistance, and accepting help as disease progresses S Behavior: goals that facilitate both their well-being and other program goals

Materials & Supports

S Specific accommodations depending on stage of disease & student interests S Personal Aide to assist with mobility, materials, transitions, communication, personal care, redirection, and continuity S Adaptive materials and equipment S Mobility instruction as appropriate

S Separate space with familiar adapted activities for redirection, calming S Use of separate space when group activity is inappropriate S Adjusted expectations, parallel activities, positive grades, and limited testing S Late start or adjusted schedule as requested by family S Careful consideration of transportation needs and field-trips

Symptoms and Strategies

Specific Intervention Areas

S

Vision Symptoms

S Unable to see things others see (white moon in a blue sky) S Resistance to reading S School saying they need a vision check S Eye Doctor saying their physical eye exam is inconsistent with vision tests S Central vision is lost first, and then increasing patches of damage to the retina cause functional, then total blindness

Vision Strategies

S Adapt Materials for Low-Vision Needs S Respect Child’s Adaptation and Processing of Their Loss S Braille S Assistive Technology

Seizures

Symptoms Vary Widely Intervention Strategies S Updated seizure plan S Safety training for school staff S Avoid student boredom

Speech Symptoms

S Hesitating Speech S Repetitive Speech S Stuttering-Like Speech S Slow, Incorrect, or Absence of Word Retrieval

Speech Strategies

S Patience S Prompting S Personal interests S Provide choices A Note on Communication Systems

Behavior

Symptoms Vary: Personality and Disease Process S Gender & Innate Personality Differences S Obsessive Thinking S Repetitive Speech S Loss of Maturity and Self-Control S Moody, Stubborn, Emotional S Outbursts and Tantrums

Behavior

S Inattentive & fidgety S Increased anxiety, problems with anticipation, more easily over-stimulated S Understand right from wrong, but lose the ability to control certain behaviors S Good times & bad times S Dementia is common and varies

Behavior Strategies

S School placement S Scheduling & Schedules S Separate workspace S Transitions S Stimulating not Over-stimulating environment S Stay flexible S Encourage with positive feedback

Medications & Behavior

S Medications may have unexpected effects and side-effects for a child with Batten Disease S May increase neurological symptoms S Effectiveness may change S Hard to tell side-effects from the increasing disease symptoms.

S Medication Cocktail

Medication & Behavior Strategies

S Collaborate / Consult School Nurse S Manage Environment S Active Positive Engagement S Limit Stress / Distress

Motor Symptoms

S Gradual loss of gross motor ability due to neurological impairment S Complicated by cognitive impairment S Limited ability to remember and adapt S Impaired motor planning S Loss of ability to tactually identify impairs small motor function

Motor Strategies

S OT/PT S O&M S Motor Goals S S S short term flexible best in daily activities and routines.

S Assistive equipment: for safety, accessibility, and comfort

Transition Considerations

Families Become Caregivers

S

Support for Families

S General Information: Batten Disease Support and Research Association (BDSRA) S Medical: insurance provider (special case manager), primary care provider, pediatric neurologist, and palliative care or hospice S Social Services: DSBVI, DDD, SSI, and Medicaid S Legal: for guardianship and will trusts, through a lawyer or court facilitator S Emotional: from those who emerge in relationships of trust S Respite

Global – End Stage Symptoms

S Extremely poor function due to increased seizure activity and medications combined with motor, sensory, and cognitive losses S Extremely narrow focus, repetitious behaviors, less awareness of environment, heightened anxiety, and occasional psychotic episodes where function abruptly declines S Poor sleep, GI problems, increasing health side-effect

Global – End Stage Strategies

S Familiar, comforting environment S Repetition and routine S Knowledgeable, competent caregivers S Assistive Equipment S Palliative Care or Hospice?

Conclusion: Why a TVI?

S TVIs support children with Batten disease S TVIs likely the first professional to serve the family S Their understanding of Batten disease, the issues involved, and possible strategies will enable them to choose, brief, and prepare other school personnel to work successfully with the student and family S They provide continuity from year to year, and can help families through the painful process of explaining the child’s condition, needs, and program to new personnel

Resources

General Information and diagnostic testing:

Batten Disease Support and Research Association, BDSRA

166 Humphries Drive Reynoldsburg, OH 43068 (866) 648-8718 http://www.bdsra.org/

Batten Disease Family Association (BDFA) U.K.

http://www.bdfauk.co.uk/index.html

• • • • • •

U.S. Medical Clinics and Consultation:

Batten Disease Centers for Excellence (contact information at bdsra.org) Oregon Health Sciences University, Doernbecher Children’s Hospital, Portland, OR The Bluebird Circle Clinic at Texas Children’s Hospital, Houston, TX Nationwide Children’s Hospital, Columbus, OH University of Rochester Medical Center, Rochester, NY Massachusetts General Hospital, Boston, MA

Palliative Care information and directory

For Parents: online at www.getpalliativecare.org

For Medical Providers: online at www.CAPC.org

Publications available from BDSRA

Bills, W. (2011). Teach and Be Taught: A Guide to Teaching Students with Batten Disease.

Retrieved from http://www.bdsra.org/wp-content/uploads/2012/01/Teach and-Be-Taught-2009.pdf

Teach and Be Taught, DVD Volume II: Positive Behavioral Support.

BDFA Library Resources

http://www.bdfauk.co.uk/library.htm

von der Dunk, M., & de Jong, C. G. A. (Eds.). (1992). Growing up to Dependence: Children and Young People with Batten Spielmeyer-Vogt Disease. Doorn, The Netherlands: Bartimeushage.