Mucolipidosis Type IV

Mucolipidosis Type IV

What is MLIV?

What Does MLIV Look Like?

• Rare lysosome storage disorder
• Mutation in the MCOLN1 gene that produces a hormone which transports other proteins.
• Genetically inherited.
• It effects 1:40,000 people with 70% of from Ashkenazi Jewish ancestry.
• Affects males and females equally.
• Developmental delays and will have issues related to their eyes.
• Two types of ML IV:
• Typical (95%) - more severe
• Atypical (5%) - mild

Adam Manishewtiz

• Psychomotor delays (connection
• between the brain and movement)
• Developmental delay
• Cognitive age of 12-15 months
• Low muscle tone
• Crossed eyes
• Clouding of white area of eye
• (cornea)
• Retinal degeneration - blind by
• teenage years
• Iron deficiency
• Heightened gastric levels in
• stomach

• 12 years old
• Developmental age 14 months
• Residential student
• Self-contained classroom
• PT, OT, ST, VT, & RT

What Causes MLIV?

Questions?

Adam

• Motor
• Wheelchair
• Arm/Head Movement
• Communication
• Non-verbal
• "Yes/No" gesture
• Augmented Communication Device
• Social/Emotional
• Enjoys large groups
• Cognitive
• 14 months
• Behavior
• Great Kid
• Family
• Very supportive

Music Therapy Evaluation

Goal #3

Individual Evaluation

• Hello experience - engaged through smiling and laughing
• Vocal Improvisation - vocalize after several prompts and overly exaggerated the vocalizations
• Instrument playing - adapted mallet was used to play the drum the drum with the mallet. Adam would smile if the music was in time with his playing, however the majority of his playing was erratic
• The goodbye song - help adjust Adam back into a grounded sound environment

Group Evaluation

• Observed within his special education classroom
• Appeared disengaged and required heavy prompting
• When the students gathered, Adam’s began to smile, laugh, and turn his head toward the voices of his peers.

Goal 3: To increase peer interactions, Adam will sustain an individual instrumental part (following hand over hand assistance) with at least 80% accuracy during an Orff arrangement.

Objective 3-1: Given cueing and hand over hand assistance, Adam will sustain his musical part with at least 50% accuracy during an Orff arrangement by November 2015.

Objective 3-2: Given cueing and following hand over hand assistance, Adam will sustain his musical part with at least 50% accuracy during an Orff arrangement by January 2017.

Objective 3-3: Given cueing and following hand over hand assistance, Adam will sustain his musical part with at least 70% accuracy during an Orff arrangement by April 2017.

Objective 3-4: Given cueing, Adam will sustain his musical part with at least 80% accuracy during an Orff arrangement by June 2017.

Goal #2

Goal 2: To develop self-confidence, Adam will appropriately vocalize/play instruments (with no more than 5 verbal prompts) within an improvised music making experience, in at least 20 sessions

Objective 2-1: Given (no more than) 8 verbal prompts, Adam will vocalize/play an instrument within an improvised music making experience, in 5 sessions by November 2015.

Objective 2-2: Given (no more than) 7 verbal prompts, Adam will vocalize/play an instrument within an improvised music making experience, in 5 sessions by January 2017.

Objective 2-3: Given (no more than) 6 verbal prompts, Adam will vocalize/play an instrument within an improvised music making experience, in 5 sessions by April 2017.

Objective 2-4: Given (no more than) 5 verbal prompts, Adam will vocalize/play an instrument within an improvised music making experience, in 5 sessions by June 2017.

Music Therapy Services

• 2 individual sessions/week for 30 minutes

• 1 classroom group session/week for 30 minutes

Goal #1

Goal 1: To enhance gross motor skills, Adam will maintain a steady rhythm while playing an instrument (following hand over hand assistance) for a minimum of two minutes during an improvisational experience in five consecutive sessions.

Objective 1-1: Given 4 physical prompts (following hand over hand assistance), Adam will maintain a steady rhythm playing an instrument for 30 seconds in 5 consecutive sessions by November 2016.

Objective 1-2: Given 3 verbal prompts (following hand over hand assistance), Adam will maintain a steady rhythm while playing an instrument for 1 minute in 5 consecutive sessions by January 2017.

Objective 1-3: Given 2 verbal prompts (following hand over hand assistance), Adam will maintain a steady rhythm while playing an instrument for 1 minute and 30 seconds in 5 consecutive sessions by April 2017.

Objective 1-4: Following hand over hand assistance, Adam will maintain a steady rhythm while playing an instrument for 2 minutes in 5 consecutive sessions by June 2017.

Methodology

• Modified Nordoff-Robbins approach
• Role of music
• Improvisation
• Orff arrangement

Songs Used

Purposeful Play

“Catch the Beat” – Richard Thompson

Themes for Therapy - Michele Schnur Ritholz

& Clive Robbins

Free Vocalization

“Matilda the Gorilla” – Mary Rice Hopkins

Instrument Playing

“Let Me Hear You Beat That Drum!” – Michele Schnur

Ritholz

Themes for Therapy - Michele Schnur Ritholz

& Clive Robbins

Data Collection

• Observation recording
• Duration recording
• Frequency recording
• Video decoding
• Duration recording

Progress Note

Taken Mid-Year

Goal #1

• 3-4 physical prompts
• 45 seconds - 1:00+
• Achieved 3 consecutive sessions of gaining over 1 minute
• Adapt the goal

Goal #2

• Experience preference
• 7-8 prompts to initiate vocalizing
• “Ah” and “Eh”
• Vocalized/played during 11 sessions.

Goal #3

• Continual progress to
• Difficulty at first
• Heavy prompting and hand over hand assistance
• Reduction beginning Currently 50%-60% accuracy.

- Timothy Doak, MT-BC

Instrumental Arrangement

"This Little Light of Mine"

• Guitar (MUTH)
• Conductor (MUTH)
• Xylophone (3)
• Bass Bar
• Drum - Adam