Margaret Spring: Occupational Therapy Goals for Rett Clinic Patients

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Setting Goals and Achieving Function:

Margaret Spring, MS OTR/L

Occupational Therapist

Childrens Hospital Colorado

I have no financial relationship or

any commercial interest related to

the content of this presentation.

Disclosures

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Inspiration

“When you have exhausted all

possibilities, remember this:

You haven’t.”

-Thomas Edison

• Children with Rett Syndrome (RS) have:• severe intellectual disability

• progressive decline of adaptive behaviors

• withdrawal, isolation, and passivity

• minimal positive interactions with their environment

• reduced sensory stimulation and deprivation

• This negatively affects quality of life.

• In 3 girls with RS, assistive technology aids:• increased occupational opportunities, engagement and choice

strategies

• reduced stereotypic behaviors

• improved self-control on environmental inputs

• provided a variety of preferred stimuli

• positively affected patient moods

Why we are here…

Stasolla F, Res Dev Disabil, 2015; 36:36

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It’s all about quality of life and finding ways for each child to participate in their environment on

a daily basis.

So how do we go about doing this?

TRUTH!

• Identify common motor difficulties associated with

RS and the best therapeutic approach

• Identify how to assess hand function to help establish

treatment goals

• Identify common feeding difficulties associated with

RS and how to establish goals for feeding

• Identify adaptive community activities for your child

Objectives

Gross Motor Development

Specific Motor Challenges:• Hypotonia (low muscle tone)

• Ataxia (limited coordination of voluntary muscle movements)

• Apraxia (difficulty performing learned purposeful movements)

• Spasticity

• Difficulty developing or the loss of transitional movements

• Loss of hand function

• Scoliosis (asymmetrical spinal posture)

• Seizures

• Alterations in foot postures

• Low cardiovascular capacity

• Spatial disorientation

Therapy Approach

• Paradigm shift: Having a relatively clear picture including clinical features and course carries the inherent risk that we would limit our expectations and, in turn, our investments. It is vital to invest in the strengths of individuals with RS and aim toward their highest potentials.

• What is the game plan? Ongoing and multidisciplinary treatment interventions are important. Know your opponent – RS imposes neuro-motor obstacles (ataxia, apraxia,

dyspraxia, altered tone, etc.), which disrupt the progression and retention of motor development.

Practice – Motor development is driven by practice and meaningful repetition is key.

Keep practicing – Regression is a risk.

The ball is always in play – Every environment which an individual engages is an opportunity for meaningful practice. Every individual, with whom your child interacts with on a consistent basis, is a potential team member.

You are the coach – Communication is vital to create and implement an effective plan.

• The clinical picture is dynamic: Some individuals demonstrate the ability to regain skills, which appeared previously to be lost.

Therapy Approach

• The individual with RS is, in some ways, controlled by

her or his body instead of being in control of the body.

Fear can be a significant obstacle to movement.

Building and supporting trust as a foundation for treatment is vital.

• Psychologist Abraham Maslow suggested, “Therapy

may be conceived as a miniature ideal society of two.” Therapies seek to create a miniature ideal environment for

success in achieving specific movements.

Clinical treatment offers the opportunity to practice the successful movements and create strategies for generalizing these to a variety of environments.

Invite community team members (i.e., teachers or caregivers) to attend therapy sessions; therapists to visit the home or community environment

• Successful treatment interventions: Invest time in bonding initially to build trust.

Develop familiar routines and emphasize the beginning and end of sessions.

Verbal direction should precede/accompany physical guidance.

Encourage the individual with RS to guide the session.

Allow for a delayed response time. Patience is key!

Praise for all attempts at functional movement and respond to vocalizations.

Be aware that an individual’s ability to perform motor tasks will fluctuate based on time and environment.

Employ task analysis to break down challenging motor skills into manageable components.

Repetition is key.

Pair positive experiences with practice sessions.

Be actively aware of how you as a therapist or parent are feeling and recognize the impact of your nonverbal communication.

Siblings are natural therapists!

Therapy Approach

• Objectives of therapy interventions: Monitor and advance joint range of motion; bracing may be beneficial

Counter the effects of apraxia with repetitive functional practice

activities

Promote effective balance strategies

Improve body awareness through deep proprioceptive input

Promote stability in transitional postures or those which require

midrange control

Promote functional hand use and provide opportunities for practice

Support optimal foot position to limit deformity and retain functional

mobility

Promote trunk strength and postural awareness to limit the

progression of scoliosis

Promote volitional mobility as a priority

Therapy Approach

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Dyspraxia - Difficulty motor planning new and unfamiliar activities

Apraxia - Inability to coordinate thoughts and movements

Ideation - Knowing what to do-requires goal directed sequence of movement

Stereotypical Hand Movements - There are a variety of stereotypical hand movements that interfere with participation in activities

Handedness - Know which hand is the dominant hand of the child to get the best function

Mutation - Correlation between mutation and hand function

Motivation - Know what motivates the child. You can build skills from this.

Factors Impacting Fine Motor Skills

Assessing Hand Stereotypies

Downs J, Dev Neurorehab, 2014; 17:210

Assessing Hand Function


Algorithm for the Management of Hand Stereotypies


Motor Learning Therapy to Improve Hand Function


• Get a baseline of function and stereotypical

hand movements before beginning treatment

to make sure your treatment and use of

equipment are truly effective.

• Start with what motivates the child.

Research shows feeding/eating is extremely

motivating to children with RS.

• Use a variety of strategies including

behavioral/learning theory, hand over hand

assistance/guidance, adaptive equipment,

and splints.

Ways to Facilitate Functional Hand Use

Goal Example Strategies

• Hope will participate in 50% of the mealtime with use of adaptive

equipment and guided eating strategies in 4/4 sessions

The goal of guided eating is to improve participation and engagement

of the child during mealtime.

Guided eating includes verbal and physical prompting by the person

assisting with the meal.

The child with RS is an active participant in the process.

• Adaptive equipment

Examples: mother’s third arm to hold drinks; cup with straw; adaptive

utensils (e.g., universal cuff, wrap around spoon/fork); Kinesio tape to facilitate wrist extension; scoop bowls/plates; suction cup bowls/

plates; curved utensils; built-up handled utensils

Arm bracing: research shows minor improvements in finger feeding

with placement of an elbow splint on the non dominant arm.

Restraining decreases stereotypic movements.

Assistive technology: to help make choices during meals

Use of Hand and/or Arm Splints

Guided Eating

Modifying/Adapting the Task

• Decrease the motor requirements necessary

• Begin by working on maintaining grasp on an object

• Gradually increase the amount of time the child needs to hold the

item

Transfer of One Skill to Another Skill

• Limited research on transfer of skills from

feeding to other fine motor tasks

• Best to find one component of the task that

is similar to feeding and work on that part

first

• Example: If the child is able to maintain a

gross grasp on an object, transfer this to

maintaining grasp on a swing.

• Keep handedness in mind.

Rett Syndrome: Feeding Concerns

Oddy W, J Pediatr Gastroenterol Nutr, 2007; 45:582

Rett Syndrome: Feeding Difficulties

Things to consider:

• Reduced muscle tone can affect head and neck control

• Breathing disturbances which may interfere with swallowing ability

• Seizure activity requiring medications which may affect appetite, increase oral secretions, and cause sedation

• Possible need for enteral nutrition support via gastrostomy or nasogastric tube as well as supplemental formulas to boost calorie consumption

• Difficulties with growth, hydration and nutrition

• Oromotor and oropharyngeal dysfunction

• Sensory deficits

• Reduced tongue mobility

• Difficulty with higher food textures

• One or all phases of swallow may be impacted

• Oral apraxia

• Movement of tongue: rapid, repetitive - can

disrupt or interfere with oral preparation,

transport, transfer of bolus

• Incomplete or nonexistent bolus manipulation

Common Feeding Difficulties

Rett Syndrome: Recommendations

• May benefit from an individual treatment plan that

describes food and beverage consistencies and

portion sizes

• Consult with nutrition about how to get the most

calories in the least amount of effort

• Consult on positioning during feeding,

• Use of selected utensils and adaptive equipment

• Consider need for alternative feeding methods.

• Assessment of swallowing function at onset of the

following symptoms- choking, decreased control of

secretions, upper or lower respiratory tract

infections, weight loss

Strategies for Prolonged Meal Times (30+ min):

• Offer most advanced texture that matches oral motor

skills at the beginning of the meal

• Make sure foods match oral motor skills

• Offer lower texture foods to get volume and variety of

intake (easier textures should result in shorter meal

times)

• Boost calories of foods offered (work wort your

dietician to sort out how)

• Offer high calorie alternatives to food


Strategies for Sensory Deficits:

• Provide a couple of minutes of sensory warm up

(alerting activities) prior to the meal time

• Can change temperature of foods (such as make

colder to provide more input)

• Add high contrast flavors to foods -you can do this

with the addition of dips or spices (ranch, BBQ, honey

mustard, ketchup)

• Can offer very crunchy high flavor meltable solids

(freeze dried strawberries or raspberries), veggie

straws (variety of flavors), Pirates Booty (a variety of

flavors).



Strategies for Poor Muscle Tone:

• Consider positioning needs (hips and knees

at 90 degrees and feet fully supported)

• Head neck and trunk in midline

• Head and neck in slight chin tuck position

• Upright and fully supported

• Child should not be fed lying down unless an

infant and is developmentally appropriate

Strategies for Difficulty with Higher Food

Textures:

• Modify textures to match oral motor skills

• Start slow and work on quality of skill with the

just rite challenge. Work on skill for 5 minutes

at a time. If child is not at a level to work on

more advanced textures due to oral motor

impairments and risk for choking there is no

pressure to do this.


Additional Treatment Strategies

• Work on the actual task of feeding

• Focus on quality not quantity

• A dietitian will help to increase caloric

density of foods consumed to decrease

meal length


Mealtime Recommendations

• Have child participate in family meal times

• If g-tube fed, can have tube feeds during family

meal times

• Can pair g tube feedings with oral feedings

• Make sure child is properly positioned

• Limit meal times to 20-30 minutes

• Offer choices during meal time for best

participation

• Colorado Therapeutic Horseback Riding

• 11968 Mineral Road Longmont Co

• 303-652-9131

• Safe Splash Swim School

• 4151 E Colfax, Denver, CO 80220

• 303-799-1885 ext. 700

• Sera School Music

• Music Therapy-(866) 611-7558

• Access Gallery

• 909 Santa Fe Drive, Denver CO 80204

• 303-777-0797

Therapeutic Community Activities

Downs et al. (2009) Guidelines for Management of Scoliosis in Rett Syndrome Patinets

Based on Expert Consensus and Clinical Evidence. Spine. 34(17) 607-617.

Horsha et al. (2009) Brain Metabolism in Rett Syndrome: Age, Clinical and Genotype

Correlations. Annals of Neurology, 65(1) 90-96.

Larsson, G., Engerstrom, I. (2001) Gross motor ability in Rett syndrome – the power of

expectation, motivation and planning. Brain and Development. 23, S77-S81.

Lotan, M., Isakov, E., & Merrick, J. (2004) Improving functional skills and physical

fitness in children with Rett syndrome. Journal of Intellectual Disability Research

48(8), 730-735.

Lotan, M. (2006) Rett Syndrome. Guidelines for Individual Intervention. The Scientific

World Journal. 6, 1504-1516.

Percy, A. (2008) Rett Syndrome: Recent Research Progress. Journal of Child

Neurology. 23(5) 543-549.

Rossin, L (1997) Effectiveness of therapeutic and surgical intervention in the treatment

of scoliosis in Rett syndrome. Thesis. University of Duquesne, Pittsburg, PA.

Volkmar, F., Cook, E.H., Jr., Pomeroy, J., Realmuto, G., and Tanguay, P. (1999)

Practice parameters of the assessment of children, adolescents, and adults with

autism an dother pervasive developmental disorders. J, Am. Acad. Child Adolesc.

Psychiatry 38(12 Suppl), 32S-54S.

Works Cited

Works Cited

Budden S, Meek M, Henighan C. Communication and oral motor function in Rett syndrome. Developmental Medicine and Child Neurology. (1990) 32(1):51-55.

Carter P., Downs, J., Bebbington, A., Williams, S., Jacoby, P., Kaufmann., Leonard, H. Stereotypical hand movements in 144 subjects with Rett syndrome from the population-based Australian database. Movement Disorders. (2009) Vol 00., No. 00.

Downs, J., Bebbington, A., Jacoby, P., Williams, A., Ghosh, S.,Kaufmann, W., Leaonard,H. Level of purposeful hand function as a marker of clinical severity in Rett syndrome. Developmental Medicine and Child Neurology. (2010). 1-7.

Piazza C, Anderson C, Fisher W. Teaching self-feeding to patients with Rett syndrome. Developmental Medicine and Child Neurology. (1993). 35: 991-996.

Qvarfordt I, Engerstrom I, Eliasson A. Guided eating or feeding :three girls with Rett syndrome. Scandanavian Journal of Occupational Therapy. (2009). 16:33-39.

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Thank you very

much for joining

us.

Intervention Strategies By Stage

• Stage one – “Onset” (6-18 months)

Characteristics include:

• Delayed progress in developing motor milestones

• Reduction in volitional hand function with possible hand wringing

• Reduced play skills and diminished communication.

Therapeutic emphasis: Maximize progress, especially regarding transitional skills, standing and upright mobility. Individuals who attain walking in stage one often retain the ability for some time.

• Stage two – “The rapid destructive phase” (1-4 years old)


• Loss of some acquired motor milestones

• Stereotypical hand movements fully present

• Possible onset of medical concerns including: seizures, irregular breathing and spinal asymmetry.

Therapeutic emphasis: Employ relaxation techniques with gentle handling to support mobility and gross motor skills. Important to establish training for all possible team members to improve understanding of RS and strategies to promote function.

• Stage three – “Plateau” (2-10 years old)


• Relatively calm period with limited progression of the existing symptoms

• Apraxia is present and interferes with purposeful movement

• Concern for development of contractures and postural deviations (feet and spine)

Therapeutic emphasis: Reasonable to aim toward building transitional, mobility and

gross motor skills. Implement strategies, including equipment, to limit postural

deviations, promote standing activities and, if possible, walking.

• Stage four – “Late motor deterioration” Characteristics include:

• Reduction in mobility

• Weakness with increased muscle tone and rigidity

• Concern for the development of postural deviations and circulatory changes

Therapeutic emphasis: Provide support for improving range of motion, transfer skills

and positioning (seated and upright as able) as well as provide resources for families

to assist in care and transfers.

Intervention Strategies By Stage

Margaret Spring: Occupational Therapy Goals for Rett Clinic Patients

Health & Medicine

Transcript of Margaret Spring: Occupational Therapy Goals for Rett Clinic Patients