Clinical Problem Solving II: Case Presentation - PowerPoint Presentation

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Clinical Problem Solving II: Case Presentation FALL 2017

Jake HillyardSlide2

Clinical Details and Patient IntroClinical experience at a local Acute Care Hospital spending majority of time on Cancer treatment and Pediatric units

15 yr. old male

S

coliosis secondary to Friedrich’s ataxia

Reason for Admission: Posterior Spinal Fusion performed to correct scoliosis (T2-L4)Slide3

Friedrich’s Ataxia

Inherited non-congenital autosomal recessive (FXN gene)

Mutated gene inhibits creation of protein

frataxin

Frataxin helps transport iron and is crucial to mitochondria functionSpecific spinal nerve cell degeneration- primary cause of noticeable symptoms“GAA” repeats in DNA sequence (966-699)Curefa.orgSlide4

Friedrich’s Ataxia cntd.

3 Major manifestations

: Scoliosis(Y), Hypertrophic Cardio Myopathy(N), Diabetes(N)

Most commonly diagnosed between 5-18 yrs. Late onset less common

Common signs and symptoms:

Impaired coordination, fatigue, diminished wound healing, vision impairment, hearing impairment, slurred speechMental capacity remains intact

Most early diagnoses result in mobility aids such as walker or wheelchair by late teens to early twenties.

Curefa.orgSlide5

Initial Evaluation post op day 1

Subjective Findings:

Prior level of function: Independent with frequent falls

“Walks like a drunken sailor” –per mom

Home situation:

2- story private residence5 steps to enterLives with mom and siblings

No current DME used

“I can’t do this” at initiation of movement

Pain: 10/10 – rib cage, constantSlide6

Initial Evaluation post op day 1 cont’d.

Objective Findings:

ROM: Generally decreased, functional

Strength: Generally decreased, functional

Tone: Abnormal- increasedSensation: IntactCoordination: Markedly decreased

Bed Mobility:

Rolling- Max assist

Supine to Sit- Max assist

Scooting- Max assist

Transfers:

Sit to Stand: Mod assist

Stand to sit: Min assist

Stand Pivot: Assist x2

Gait:

5 feet to chair- assist x2

Widened stance, valgus knees, ataxicSlide7

ICF Model

Impairments

Pain

Increased tone

Decreased coordination

Decreased strengthDecreased motor controlImpaired balance

Activity Limitations

Abnormal gait due to pain and ataxia

Difficulty transferring

Difficulty with bed mobility

Participation Restrictions

Difficulty with mobility at school

Altered social life

Dependent on others for transportationSlide8

Treatment PlanBed Mobility

Transfer training

Gait training

Endurance

Patient/Family Education

Back precautionsHome management

Activity pacingSlide9

Treatment GoalsWithin 4 days, patient will be able to:

Move from supine to sit and sit to supine with min assist

Perform sit to stand with supervision/set-up

Ascend/descend 5 stairs with 1 handrail with min assist

Verbalize and demonstrate back precautions accuratelySlide10

Treatment post op day 1 p.m. sessionGait Training: Ambulated 15 ft. with two-hand hold mod assist in front with stand by assist from behind.

Mod assist with sit to supine

Complained of nausea and pain 7/10Slide11

Treatment post op day 5Ambulated 50 ft. (10, 10, 30) with mom providing two-hand grasp from front

Ascended/Descended 3 stairs with min assist x1

Bed Mobility with mom provided assistance:

Supine to sit- mod assist

Sit to supine – stand-by assist

Scooting – stand-by assistSlide12

Clinical QuestionFor a

15 year old male patient with Friedreich’s Ataxia, would motor training and coordination exercises performed post-operatively improve functionality and gait following a posterior spinal fusion?

Hypothesis: Yes, I believe that motor training and coordination exercises following a posterior spinal fusion will improve my patient’s functionality and gait as his body adjusts to it’s new anatomical alignment.Slide13

Systematic Review

Motor Training in Degenerative Spinocerebellar Disease:

Ataxic-Specific Improvements by Intensive Physiotherapy and

Exergames

Synofzik

, M. Winfried, I.Published in BioMed Research International2014. Vol. 2014. Article ID 583507Slide14

Inclusion Methods

Time Period of January 1, 1980 – December 18, 2013

Articles were included if they met the following criteria:

Original report

Prospective clinical trial examining physiotherapy interventions

High-intensity training over an extended period of timeControl designPatients with spinocerebellar degeneration (not secondary)

Originally n = 578, only 3 met criteriaSlide15

Article #1

Cerebellar Ataxia Rehabilitation Trial in

Degenerative Cerebellar Diseases

Ichiro, M.

Mizuki

, I. et al.Published in Neurorehabilitation and Neural Repair2012. Vol. 26 (515-522)Slide16

Study Details

Design

Randomized Controlled Trial and Observational Study

2

Intervention groups- immediate and delayed

All participants received same intervention for 4 weeks- PT focused on improving balance and gait1 hr of PT + 1

hr

of OT on weekdays, 1

hr of either on

weekends

Data collected at 0, 4, 12, and 24

wks

after intervention

.

Participants

N = 42 (21 in each group)

Mean age 62.5

Outcome Measures

SARA- Scale for the Assessment and Rating of

Ataxia (0-40)

FIM

Also gait speed, falls, cadence, FACSlide17

Results- Short term (RCT)Slide18

Results – Long TermSlide19

Conclusion/Limitations

Intensive rehabilitation interventions showed significant improvements in SARA scores, gait speed, FIM, and fall frequency in patients with spinocerebellar ataxia in the

short term

. Unfortunately when the therapy was discontinued, these gains were lost as outcomes mostly returned to their pre-intervention levels.

Limitations

Lack of specificity in type of cerebellar ataxia (afferent or cerebellar)Age Average!

No video examination to ensure complete blinding

High number of interventions

Increased motivation in weeks post-intervention?Slide20

Article #2

Video

g

ame-based coordinative training improves

a

taxia in children with degenerative ataxiaIlg, W. Schatton, C. Schicks, J. Giese, M. Schols

, L.

Synofzik

, M.

Published in Neurology

2012 : 2056-2060

Class III evidenceSlide21

Study Details

Design

Prospective Cohort Study

8

wks

coordinative training on Microsoft XBOX Kinect (2 wks in lab, 6 wks at home)Assessments performed at 4 intervals, E1-E4.

2 weeks prior

Immediately prior

After 2 week lab training

After 6 weeks home training

Patient’s acted as own control

Participants

N = 10

Mean Age: 15.4

4/10 had FA

Outcome Measures

SARA

DGI

ABC

Slide22

Study Details cntd.

XBOX Kinect Training

Table Tennis

Light Race

20,000 Leaks

Movement Goals

Goal directed limb movements

Dynamic balance

Whole-body coordination

Rapid reactions

Recalibrate movement predictions

4 -1 hour training sessions/

wk

Data gathered via VICON MX motion capture system for movement analysisSlide23

Results

- Children with FASlide24

Results cntd.Slide25

Conclusions/Limitations

Children with degenerative ataxia were shown to make improvements in dynamic balance activities such as gait as evidenced by improved SARA scores, DGI, and quantitative movement analysis. Video game-based coordinative training provided a fun, inexpensive, and motivating method for potential long term retention of measured gains.

Limitations

Small sample size

Varying ataxia subsets (afferent vs cerebellar)

Potential practice bias between E1-E2

Subjectivity of practice effort at homeSlide26

Final ConclusionsFor a 15 year old male patient with Friedreich’s Ataxia, would motor training and coordination exercises performed post-operatively improve functionality and gait following a posterior spinal fusion

?

Yes, I do believe that coordination/motor-control based rehabilitation would be beneficial for my patient. Although the evidence showed limited effect on gait, overall functionality was improved if the interventions were sustained for an extended period of time.

If I were to treat a patient similar to this in the future, I would not hesitate to initiate balance and coordination training in addition to motor control activities post surgery.Slide27

References

Synofzik

, M., &

Ilg

, W. (2014). Motor Training in Degenerative Spinocerebellar

Disease: Ataxia-Specific Improvements by Intensive Physiotherapy and Exergames. BioMed Research International, 2014, 1-11. doi:10.1155/2014/583507

Miyai

, I., Ito, M., Hattori, N.,

Mihara, M.,

Hatakenaka

, M.,

Yagura

, H., . . .

Nishizawa

, M. (2011). Cerebellar Ataxia Rehabilitation Trial in

Degenerative

Cerebellar Diseases. 

Neurorehabilitation and Neural

Repair,26

(5

), 515-522.

doi:10.1177/1545968311425918

Ilg

W,

Schatton

C,

Schicks

J, et al. Video game-based coordinative training

improves

ataxia in children with degenerative ataxia. Neurology.

2012;79:2056–60

.Slide28

Questions?