The Effects of Strengthening Programs on Independence on Individuals with Down Syndrome: A Case Report and Evidence Based Analysis Jessica Seeley Physical Therapy Doctorate Program Class of 2013 University of New Mexico School of Medicine Advisor: Beth M. Jones, PT, DPT, OCS Table of Contents Abstract .............................................................................................................................................................. 2 Section 1: Background and Purpose ................................................................................................................. 3 Section 2: Case Description ............................................................................................................................... 5 Section 3: Evidence Based Analysis .................................................................................................................. 14 Discussion .......................................................................................................................................................... 32 Conclusion .......................................................................................................................................................... 33 References ......................................................................................................................................................... 34 Tables and Figures ............................................................................................................................................. 36 Appendix ............................................................................................................................................................ 39 1 Abstract Background/Purpose: Individuals with Down syndrome generally have deficits in motor skills and decreased strength throughout development. There are several strengthening interventions that can be beneficial for improving the decreased strength and motor skills including progressive resistance training, treadmill training, and balance and agility training. The purpose of this case study and evidence-based analysis was to determine the effect that various strengthening interventions can have on the level of independence and potentially quality of life for individuals with Down syndrome. Case Description: The patient was a 23-month-old female diagnosed with Trisomy 21, as well as acute myelogenous leukemia. She had decreased strength and overall gross motor delays. Outcomes: Following various strengthening interventions of 8 randomized controlled trials, the experimental groups showed significant improvements in upper and lower-limb muscle strength, as well as improved balance and agility as compared to the control groups. Discussion: After performing an extensive database searches and research article analyses, it was determined that various strengthening interventions can lead to significant improvements in upper and lower-limb muscle strength, as well as improved balance and agility were observed in individuals of all ages with Down syndrome. Therefore, progressive resistance training, treadmill training, and agility training are interventions that can be used in the clinic to promote strengthening and allow for improved functional, social, and vocational independence for individuals of all ages with Down syndrome. 2 SECTION 1: Background and Purpose Each year, 1 in every 660 babies is born with Down syndrome in the United States Individuals with Down syndrome generally have deficits in motor skills and decreased strength throughout their development and lifespan5. Infants and children with Down syndrome are typically slower to reach developmental milestones such as sitting, standing, creeping and walking1. Young children learn through interaction with their environment, which requires the ability to move and freely explore their surroundings. Therefore, the delays in gross motor development can decrease their independent functional mobility, play and social interaction. For adolescents and adults, these deficits can negatively impact social and vocational development, as typically their workplace activities emphasize physical rather than cognitive skills8. During a clinical rotation at the University of New Mexico Hospital, physical therapy was provided to a young girl who inspired investigation into this case study and evidence-based analysis. The patient was a 23-month-old female diagnosed with Trisomy 21, as well as acute myelogenous leukemia. She had decreased strength and overall gross motor delays. Inpatient physical therapy interventions focused on developmental milestone acquisition/skills, positioning and handling, and family/caregiver education. Direct intervention included activities and exercises for trunk stability and strengthening, bilateral lower extremity strengthening, transitional movements, unsupported standing and cruising along stable objects. Due to the inpatient setting, there was limitation to the types of interventions that could be provided to this patient, however, interest and curiosity grew as to what types of interventions could be beneficial for her and for future patients with Down syndrome that could be referred to physical therapy. Due to the prevalence of Down syndrome, it is important as a clinician to be familiar with and understand the evidence-based interventions that can provide beneficial outcomes for individuals of all ages who have this diagnosis. 3 There are several strengthening interventions that can be beneficial for improving decreased strength and motor skills, which are typically exhibited in these individuals. Such interventions used include progressive resistance training3, ,8 treadmill training2,9,10, and balance and agility training4. However, these specific interventions often times are not implemented in various rehabilitation setting. Some clinicians are unaware of the vast amount of research that has been conducted on the effects of these strengthening interventions for this population. Strengthening programs are commonly used in the rehabilitation setting for individuals of all ages without Down syndrome, but are often overlooked or forgotten about for individuals with Down syndrome. The purpose of this case study and evidence-based analysis was to answer the following PICO question: Will strengthening interventions increase the level of independence in individuals with Down syndrome? To determine this, evidence-based exploration will be conducted through extensive database searches and research article analyses. 4 SECTION 2: Case Description The setting for this case study was inpatient acute care, in the pediatrics department, at the University of New Mexico Hospital. The medical diagnosis that was the focus of this case was Down syndrome. Physical therapy evaluation and treatment was ordered for the duration of the patient’s acute care admission, in order to address her overall gross motor and developmental delays. EXAMINATION: History, Systems review, and Tests & Measurements The patient was a 23-month-old female diagnosed with Down syndrome. She was also diagnosed with acute myelogenous leukemia in April of 2012. Her past medical history included seizure disorder, hypothyroidism and status post AV canal repair. She was admitted to the University of New Mexico Hospital for her AAML0431 protocol with continuous infusion of cytarabine and etoposide. She tolerated the protocol well with some intermittent fevers. Past Medical History • Down syndrome. • Acute Myelogenous Leukemia; which was diagnoses in April of 2012. She was on chemotherapy protocol AAML0431. • Status post arteriovenous canal repair at 5 months of age. • Hypothyroidism. • History of myoclonic seizures; it was documented that they are well controlled. • Known documented allergies: lisinopril and losartan. Social and Family History The patient’s mother provided report of the patient’s social and family history. The patient lived with her mother in Santa Fe. Her parents were divorced, but the father was still involved. Other family members, 5 including her grandmother, are also very involved in the patient’s life and with her daily and medical care. The patient was an only child and did not currently attend daycare. There were home nurses that visited the home and took care of the patient when her mother was working. Mother stated that the patient was a very happy and active child at home. The patient engaged in social interaction and play with family members and other individuals, as evident by observation and mother’s report. Patient’s Values During the initial evaluation, the patient’s mother expressed a strong family value and said that she was very involved in the patient’s multidisciplinary care. The mother was very proactive and sought out the ways to best improve the patient’s well being. The mother was very receptive to physical therapy interventions and education for the patient during her admission, as well as for when the patient was discharged back home. Goals & Expectations The patient’s mother reported that her goals for the patient were to improve her functional abilities, including standing on her own and cruising along furniture. She also stated that she wanted the patient to be able to play more independently at home. Additionally, the patient’s mother had inquired about what she could to do, in conjunction with physical therapy, to improve the patient’s development during the inpatient admission, and once they were discharged back home to Santa Fe. Systems Review: An overview of systems review was determined through an extensive chart review, mother’s report at the time of evaluation, and patient observation/examination. • GENERAL: Fevers and fussiness were noted in the chart. Mother reported that there were no recent weight changes. • EYES/EARS/NOSE/THROAT: There was no eye drainage, ear pain, complaints of a sore throat or nasal congestion were reported or observed. 6 • RESPIRATORY: No coughing, shortness of breath or wheezing reported or observed. • CARDIOVASCULAR: No palpitations or chest pain were reported or observed. • GASTROINTESTINAL: Positive for vomiting as listed in the chart. No report of diarrhea or constipation. The patient does have some history of gas. • GENITOURINARY: No frequency or urgency problems, as well as no foul-smelling urine were reported or observed. • SKIN: No new rashes, lesions or discoloration on the skin were reported or observed. Test and Measures • Musculoskeletal Assessment: Musculoskeletal range of motion was observed and assessed for bilateral upper and lower extremities. o Right Upper Extremity: within functional limits. o Left Upper Extremity: within functional limits. o Right Lower Extremity: within functional limits. o Left Lower Extremity: within functional limits. • Posture & Alignment: Upon observation, the patient’s feet tended to pronate while she was in a supported standing position. The fourth toe tended to adduct and lie underneath the third toe. This presented bilaterally. • Functional Movements and Abilities: The patient’s functional ability and mobility was observed during the evaluation in prone, sitting and standing positions. Observations were also made as to the patient’s abilities and limitations with functional movement transitions. o Prone: Patient would creep up on her hands and knees with a reciprocal pattern. Her hips were slightly abducted while in a quadruped position. 7 o Sitting: Patient tucked her chin with pull to sit movement. She sat independently for extended periods of time. Patient was able to maintain a straight back and hold her head up at 90° while sitting unsupported, playing with toys. o Standing: Patient stood next to the crib rail without support. Her knees occasionally went into hyperextension. Both feet were pronated but not severely. Patient was just beginning to be able to cruise along furniture with some support. o Transitional Movements: Patient transitioned from supine and prone into a sitting position. She also moved well from sitting into a quadruped position for reciprocal creeping. She transitioned to standing with minimal assistance during the PT evaluation. Her mom reported that she would pull to a standing position on her own at home. • Muscle Tone & Strength: The patient’s muscle tone was overall hypotonic, as evident by palpation, passive range of motion, and observation of posture and stability in various positions. Through observation of functional mobility and functional transfers, it was determined that she had overall decreased strength in bilateral upper and lower extremities. • Fine Motor Skills: The patient demonstrated a pincer grasp with the right and left hand when picking up smaller toys. EVALUATION: Clinical judgments based on examination Diagnosis • Medical Diagnosis: Trisomy 21; Diagnosis Code: 758.0 • Physical Therapy Diagnosis: Decreased gross motors skills and developmental delays. Narrative Assessment The patient was a 23-month-old female diagnosed with Down syndrome. She was also diagnosed with acute myelogenous leukemia in April of 2012. Other past medical history included hypothyroidism, and 8 seizure disorder. The patient was status post arteriovenous canal repair at the age of 5. During the physical therapy evaluation, she was sitting independently for extended periods of time. She was also creeping on hands and knees with a reciprocal pattern. The patient could pull to stand with minimal assistance, but her mom stated that she would pull to stand on her own at home. She also had overall decreased strength of her extremities and was unable to cruise independently along furniture. She demonstrated overall gross motor and developmental delays for her age due to decreased strength and hypotonicity. After evaluation and observation, it was determined that she was functioning at around an 11 to 12 month old age level. The patient was motivated to participate in therapy activities and also had supportive family that was often present for therapy sessions. She had a good prognosis to benefit from physical therapy interventions to improve her strength and stability, transitional movements, and overall functional mobility. Problem List The patient presented with impairments and functional limitations that are typically seen in children with Down syndrome. In addition, the patient’s diagnosis of acute myelogenous leukemia and previous hospital admissions could have also contributed to the gross motor impairments and limitations that were observed. The following problem list was determined after the initial physical therapy evaluation and subsequent therapy sessions: • Impairments: 1. Patient had gross motor and developmental delays. 2. Patient had decreased strength in bilateral upper and lower extremities.. 3. Patient had overall hypotonicity. • Functional Limitations: 4. Patient was unable to pull to stand independently, due to decreased strength and hypotonicity. 9 5. Patient was unable to cruise along her crib rail independently, due to decreased strength and hypotonicity. Physical therapy recommendation for the anticipated discharge destination was for the patient to return home with family care. It was also recommended that she receive evaluation and treatment through an Early Intervention program at home to continue progressing with her gross motor skills. Short Term Acute Care Goals 1. Patient will pull to stand independently at crib rail 3 out of 4 trials, in 2 weeks. 2. Patient will cruise 4ft in each direction, while holding onto her crib rail, in 2 weeks. Long Term Acute Care Goals 1. Patient will stand independently for 1 minute, with contact guard support, in 4 weeks. 2. Patient will cruise 4ft, with supervision assist, in 4 weeks. Plan of Care It was determined that the patient would receive inpatient physical therapy 2-3 times per week throughout her acute care admission. She was admitted to the University Hospital for approximately 10 days, and was seen by physical therapy for an evaluation and treatment sessions 4 times. Prognosis The patient was happy and motivated to participate in therapy activities and play. She also had very supportive and involved parents and family members. Family was present and very engaged in the therapy sessions. Due to these factors, the patient had a good prognosis for improving her gross motor skills with physical therapy intervention. INTERVENTIONS The patient was seen by physical therapy throughout her inpatient admission for developmental exercises. Physical therapy interventions focused on developmental milestone acquisition/skills, positioning 10 and handling, and family/caregiver education. Direct intervention included activities and exercises for trunk stability and strengthening, bilateral lower extremity strengthening, transitional movements, unsupported standing and cruising along stable objects or furniture. Physical Therapy Evaluation: During the evaluation, the patient was assessed for musculoskeletal active and passive range of motion in bilateral upper and lower extremities and overall muscle tone. Her functional abilities and/or limitations were assessed in prone, sitting, and standing positions, as well as with transitional movements. During the evaluation of these areas, therapeutic interventions were also performed on the patient. Gentle, passive stretching into supination was performed on feet bilaterally, in sitting and supine positions. The patient then worked on pull to sit and spent time in unsupported sitting while using bilateral upper extremities to reach for and play with various toys. She was then assisted into standing at the rail of her crib. Physical therapist worked on assisting her with standing in this position without her knees going into hyperextension. The patient’s mother and grandmother were both present during the evaluation. They were educated on what the physical therapy plan of care would be throughout the remainder of her admission. Family was also educated on ways to assist the patient with developmental activities, outside of therapy session times. Physical Therapy Session #1: The patient worked on creeping in order to retrieve toys to play with, she tended to half creep and/or scoot herself along at times. She worked on pulling to stand and stood next to furniture for extended periods of time. The patient worked on standing without hyperextension of her knees. Her feet still tended to pronate, but the alignment looked better today than when she was standing in the crib during the evaluation. Her mother and grandmother were educated and instructed on how to assist the patient in standing, while supporting at her knees to prevent hyperextension. 11 Educational materials were also provided to the patient’s mother on various activities and exercises to facilitate gross motor development. PT discussed and demonstrated the information that was provided to the family. Additionally, educational materials, as well as two published articles were provided to the mother. These materials discussed information on the effects of foot orthoses for foot pronation in children with Down syndrome Physical Therapy Session #2: The patient worked on unsupported sitting at the beginning of the session, while engaging in activities and playing with toys. To further encourage trunk stability and strengthening, the toys were held out in front of her at various positions for her to reach for and retrieve with bilateral upper extremities. She practiced sit to stand transitions several times at the rail of her crib, with minimal assistance. The patient stood for 5 minutes while holding onto the crib rail and would initiate a bouncing motion, weight bearing equally through bilateral lower extremities. PT supported at her knees to inhibit hyperextension while she was in this standing position. She initiated cruising two steps to the left along her crib rail, with support from therapist at her hips. Physical Therapy Session #3: The patient worked on transitioning from sitting to quadruped and then would creep on hands and feet with a reciprocal pattern, for a distance of 5 feet to retrieve various toys. She would initiate reaching for the toys with alternating upper extremities while in the quadruped position. The patient also transitioned from sitting to standing at crib rail with contact guard assist. She was given minimal assist to keep her knees from hyperextending in standing. She stood for extended periods of time while weight bearing evenly through lower extremities. She continued to have slight bilateral foot pronation, but demonstrated overall improved alignment in standing. The patient was then prompted through the use of toys and her mom to cruise along the bed rail. She took two steps two the left and to the right, with minimal assistance at the pelvis and bilateral knees. 12 Outcomes: Physical therapy services were terminated because the patient was discharged from her inpatient stay once she had finished her acute myelogenous leukemia treatment protocol. Physical therapy was ordered and provided for the duration of her admission. The physical therapy goals that were set for the patient were not achieved, due to her discharge prior to the anticipated time frame for achieving the goals. However, the patient was progressing towards achieving each of the goals. She had made improvements with developmental and gross motor skills, as well as functional mobility. Inpatient physical therapy recommended that the patient be discharged to home with family care, and be evaluated and treated by an Early Intervention program at home. The patient would benefit from continued physical therapy at home to achieve further improvements and continue with her gross motor skills development. 13 SECTION 3: Evidence Based Analysis Methodologies of Search A comprehensive evidence-based literature search was performed to find research studies related to strengthening interventions for individuals of all ages with Down syndrome. Searches were performed using PubMed, CINAHL Plus, and PEDro databases (Table 1). • For the PubMed database, an advanced search was performed including the following keywords: o [Title Field]: Down syndrome (AND) o [All Fields] Different combinations of: Strength training, resistance training, treadmill training, agility training, strengthening, and exercise. o These searches yielded 202 articles. • For CINAHL Plus database, an advanced search was performed including the following keywords: o [TI Title]: Down syndrome (AND) o [TX All Text]: Different combinations of: Strength training, resistance training, treadmill training, agility training, strengthening and exercise. o These searches yielded 97 articles. • For the PEDro database, an advanced search was performed including the following keywords: o [Abstract & Title]: Down syndrome (AND) o [Therapy]: Strength training o [Therapy]: Fitness training o [Title Only]: Treadmill Training o [Title Only]: Exercise • These searches yielded 48 articles. 14 Evidence-Based Research Articles The following 8 research articles were reviewed and analyzed: 1. Shields N. and Taylor NF. A student-led progressive resistance training program increases lower limb muscle strength in adolescents with Down syndrome: a randomized controlled trial. Journal of Physiotherapy. 2010; 56:187-193. 2. Shields N, Taylor NF, Dodd KJ. Effects of a community-based progressive resistance training program on muscle performance and physical function in adults with Down syndrome: a randomized controlled trial. Archives of Physical Medicine and Rehabilitation. 2008; 89:1215-1220. 3. Lin H-C, and Wuang Y-P. Strength and agility training in adolescents with Down syndrome: A randomized controlled trial. Research in Developmental Disabilities. 2012; 33: 2236-2244. 4. Gupta S, Rao BK, SD K. Effect of strength and balance training in children with Down syndrome: a randomized controlled trial. Clinical Rehabilitation. 2011; 25: 425-432 5. Rimmer JH, Heller T, Wang E, Valerio I. Improvements in physical fitness in adults with Down syndrome. American Journal on Mental Retardation. 2004; 109(2):164-174. 6. Carmeli E, Kessel S, Coleman R, Ayalon M. Effects of a treadmill walking program on muscle strength and balance in elderly people with Down syndrome. Journal of Gerontology: MEDICAL SCIENCES. 2002; 57A(2): M106-M110. 7. Ulrich DA, Ulrich BD, Angulo-Kinzler RM, Yun J. Treadmill training of infants with Down syndrome: evidencebased developmental outcomes. Pediatrics. 2001;108(5). DOI:10.1542/peds.108.5.e84. 8. Ulrich DA, Lloyd MC, Tiernan CW, Looper JE, Angulo-Barroso RM. Effects of intensity of treadmill training on developmental outcomes and stepping in infants with down syndrome: a randomized trial. Physical Therapy. 2008; 88(1):114-122. 15 Research Article #1 Shields N. and Taylor NF. A student-led progressive resistance training program increases lower limb muscle strength in adolescents with Down syndrome: a randomized controlled trial. Journal of Physiotherapy. 2010; 56:187-193. Level of Evidence (Oxford scale): 1b Purpose: To determine whether a progressive resistance training (PRT) program leads to increased muscle strength and improved physical function in adolescents with Down syndrome. Methods: This was a random controlled trial, in which participants were randomly allocated to the experimental or control group using a concealed method, with block randomization. Group allocation was prepared and performed by a researcher not involved in recruitment or assessment. • Participants o 23 adolescents with Down syndrome aged 13 to 18 years. Inclusion criteria: able to follow simple verbal instructions in English, and fit and well enough to participate in the training. o 7-item Physical Activity Readiness questionnaire was given to parents. The level of intellectual disability of each participant was documented. o Exclusion: participation in a PRT program in the 6 months prior to the trial. • Experimental Group Intervention (n = 11) o Progressive resistance training program led by physiotherapy students, twice per week for 10 weeks. The intervention was completed at a community gymnasium. o Consisted of 6 exercises on pin-loaded weight machines. Three UE exercises: lat pull-down, seated chest press, seated row. Three LE exercises: seated leg press, knee extension, calf raise. o 3 sets of 12 repetitions, or until fatigue, with a 2-minute rest in between each set. 16 • Control Group Intervention (n = 12) o Participants continued with their usual daily activities, which may have included leisure and sporting activities, but did not include a PRT program. • Outcome Measures o Both groups were assessed at baseline and after the 10-week intervention phase. o Muscle strength was assessed using 1RM chest press and leg press. Minimally Clinically Importance Difference: 40% increase 1RM leg press o Lower-limb physical function was measured using the Timed Up and Down Stairs test. Upper-limb physical function was measured using the Grocery Shelving Task. Results: The experimental group increased lower limb muscle strength compared to the control group (MD 36kg; 95% CI, 15-58; SMD 0.7). The lower limb strength increase represented a 42% increase in baseline strength, which was clinically significant. There were no significant differences between the groups for upper limb muscle strength or upper and lower limb physical function. Critique & Conclusion: One of the main strengths of this study was that it was the first randomized controlled trial to assess the affects of progressive resistance training (PRT) for adolescents with Down syndrome. This study design also had high internal validity with random, concealed allocation of subjects to the groups, blinding of therapists administering the intervention, and an intention to treat analysis. However, the subjects and assessors were not blinded to which group was the experimental. Another weakness to the study was that there was no follow-up to determine if the effects were maintained long term. The data of this trial showed that PRT was effective in improving the strength of the quadriceps and hip extensors, which are the two major antigravity muscles. In conclusion, this type of student-led PRT was a feasible, safe, and inexpensive intervention option for adolescents with Down syndrome that can lead to improvements in lower-limb muscle performance. 17 Research Article #2 Shields N, Taylor NF, Dodd KJ. Effects of a community-based progressive resistance training program on muscle performance and physical function in adults with Down syndrome: a randomized controlled trial. Archives of Physical Medicine and Rehabilitation. 2008; 89:1215-1220. Level of Evidence (Oxford scale): 1b Purpose: To determine if a community-based progressive resistance training (PRT) program improves muscle strength, muscle endurance, and physical function in adults with Down syndrome. Methods: This was a randomized controlled trial, in which participants were randomly allocated to either an intervention or control group using concealed allocation, block randomization method. Participants were assigned to their allocation by a physiotherapist not involved in the study after baseline measures. • Participants o 23 adults with Down syndrome aged 20-49 years. Inclusion criteria: able to follow simple verbal instructions in English, and fit and well enough to participate in the training. o 7-item Physical Activity Readiness questionnaire was given to parents. The level of intellectual disability of each participant was documented. o Exclusion: participation in a PRT program in the 6 months prior to the trial. • Experimental Group Intervention (n=9) o PRT program supervised by 2 accredited fitness trainers, twice per week for 10 weeks, at a community gymnasium. o Consisted of 6 exercises using weight machines. Three UE exercises: shoulder press, seated chest press, seated row. Three LE exercises: seated leg press, knee extension, seated calf raise. o 2-3 sets of 10-12 reps, or until fatigue, with a 2-minute rest in between each set. 18 • Control Group Intervention (n=11) o Participants continued with their usual activities, which included employment, leisure and sporting activities, but did not include a PRT program. • Outcome Measures o Both groups were assessed at baseline and after the 10-week intervention phase. o Muscle strength was assessed using 1RM chest press and leg press. o Muscle endurance was measured by counting the number of reps completed at 50% 1RM for the seated chest and leg press. o Lower-limb physical function was measured using the Timed Up and Down Stairs test. Upper-limb physical function was measured using Grocery Shelving Task. Results: The intervention group showed significant improvement in upper-limb muscle endurance compared with the control group (MD # of chest press reps at 50% 1RM, 16.7; 95% CI 7.1-26.2). There was also a trend towards an improvement in upper-limb muscle strength (MD chest press 1RM, 8.6kg; 95% CI, -1.3 to 18.5kg) and in upper-limb function (MD grocery shelving task, -20.3s; 95% CI, -45.7 to 5.2s). No significant differences between groups for lower-limb muscle performance or physical function measures. Critique & Conclusion: One of the main strengths of this study was that it was a randomized controlled trial that conformed to the training guidelines of the ACSM for adults with Down syndrome. Another strength was the high internal validity due to random, concealed allocation of subject, blinding of all assessors measuring at least one key outcome, excellent program compliance and no withdrawals. and an intention to treat analysis. However, weaknesses of the trial included lack of therapist blinding, relatively small sample size of participants, as well as a short duration and lower frequency of the intervention. Overall conclusion is that PRT in a community gymnasium was a feasible, safe, and inexpensive intervention option for individuals with Down syndrome that can lead to improvements in upper-limb muscle performance. 19 Research Article #3 Lin H-C, and Wuang Y-P. Strength and agility training in adolescents with Down syndrome: A randomized controlled trial. Research in Developmental Disabilities. 2012; 33: 2236-2244. Level of Evidence (Oxford scale): 1b Purpose: To evaluate the effectiveness of a combined strength and agility training program in adolescents with Down syndrome, by using treadmills and Wii game protocols. Methods: This was a random controlled trial, in which participants were randomly assigned to either the experimental or control group using stratified random sample. • Participants o 92 adolescents, aged 13-18 years currently enrolled in high school. o Diagnosed with DS by board-certified physician, able to follower simple instructions, and had written consent indicating agreement to participate. o Exclusion: subjects with associated cardiovascular conditions, blindness, deafness, or previous neurological impairments. Received PT or OT in the year preceding the study. • Experimental Group Intervention (n = 46) o 6-week intervention consisting of three 35-minute sessions per week. Home programs were not provided to the parents/caretaker to minimize possible confounding effects. o Treadmill protocol: 5 minutes, with an average start speed of ~2.0kph and end spend ~3.0kph. The therapist determined the treatment protocol for each child in advance. o VR: 20min Wii game-like exercise with demands for muscle strength and agility performance. Most common chosen sports: boxing, bowling, table tennis, frisbee and golf. • Control Group Intervention (n=46) o Participants continued with their normal daily activities. 20 • Outcome Measures o Handheld dynamometer was used to measure lower-limb muscle strength. o Bruininks-Oserersk Test of Motor Proficiency (BOT-2) strength and agility composite (SAC) subtests: standing long jump, push-ups, sit-ups, wall sit, and V-up, used to measure agility. Results: Analysis between the groups revealed a significant group difference in terms of strength in all muscle groups following the intervention. Level of significance and comparison of mean change (in pounds) between the control and experimental group from pre and post intervention measures were as follows: • Hip Flexors (p = 0.010): 0.94 change in the intervention group vs. -0.08 change in the control group • Hip Extensors (p = 0.018): 0.64 change in the intervention group vs. 0.13 change in the control group • Hip abductors (p = 0.004): 1.57 change in the intervention group vs. 0.13 change in the control group • Knee Flexors (p = 0.029): 1.60 change in the intervention group vs. 0.17 change in the control group • Knee Extensors (p = 0.031): 1.42 change in the intervention group vs. 0.19 change in the control group • Ankle Plantarflexors (p = 0.011): 1.17 change in the intervention group vs. 0.30 change in the control group Moderate to large effect sizes were obtained for all muscle groups as well. There was also a significant difference found when comparing the pre-post measures on the BOT-2 SAC. (p = 0.02). Critique & Conclusion: Strengths of this study include excellent compliance with no withdrawals, random allocation to groups, blinding of assessors, and intention to treat analysis. However, one weakness of this study was the lack of blinding of therapists who administered the treatment. Another weakness was the study’s results did not generalize to group with severe and profound ID, and participants were not monitored for other physical exercise or VR-based activities in addition to the intervention. In conclusion, despite the limitations of this study, this type of intervention was found to be an effective, reasonable, and relatively inexpensive intervention that can result in improvements in strength and agility for adolescents with Down syndrome. In addition, a combination program of treadmill and VR-based activities might be even more effective in achieving these strength and agility improvements. 21 Research Article #4 Gupta S, Rao BK, SD K. Effect of strength and balance training in children with Down syndrome: a randomized controlled trial. Clinical Rehabilitation. 2011; 25: 425-432 Level of Evidence (Oxford scale): 1b Purpose: To determine the effect of a strength and balance training program in children with DS. Methods: This was a randomized controlled trial, in which the children were randomly divided into the experimental and control group using stratified random sampling. • Participants o 23 children with Down syndrome, aged 7 to 15 years o Ability to understand simple instructions and ability to stand and walk independently. o Exclusion criteria: associated cardiovascular condition, loss of functional vision/hearing. • Experimental Group Intervention (n = 12) o Strength and balance program 3 times per week, for 6 weeks. o Progressive resistance exercises using sandbags for lower limb: hip flexors, abductors, extensors, knee flexors, knee extensors, and plantarflexors. 2 sets of 10 reps for each muscle group. Strength training was started at 50% of the 1RM. Resistance increased by 1.1lbs when the child was able to complete the sets with ease. o Balance training program: Each balance activity was initially 10 performed repetitions and increased by 5 reps when the child could do it with ease. Activities included the following: jumps, vertical jumps, one leg stance with eyes open, tandem stance, walking on a line, walking on a balance beam, and jumping on a trampoline. 22 • Control Group Intervention (n=11) o Participants continued with their activities that were being followed in the school, which included classroom studying and play activities. . • Outcome Measures o Handheld dynamometer was used to measure lower-limb muscle strength. o Balance subscale of Bruininks Oserersk Test of Motor Proficiency to assess balance. Results: Children in the intervention group showed a statistically significant improvement (p<0.05) in the lower-limb strength of all the muscle groups assessed. Level of significance and comparison of change between the control and experimental group from pre and post intervention measures were as follows: • Hip Flexors (p = 0.001): 1.74 change in the intervention group vs. 0.01 change in the control group • Hip Extensors (p = 0.002): 2.37 change in the intervention group vs. -0.50 change in the control group • Hip abductors (p = 0.001): 0.95 change in the intervention group vs. -0.22 change in the control group • Knee Flexors (p = 0.03): 1.39 change in the intervention group vs. 0.24 change in the control group • Knee Extensors(p = 0.01): 2.54 change in the intervention group vs. 0.54 change in the control group • Ankle Plantarflexors (p = 0.02): 0.38 change in the intervention group vs. 0.22 change in the control group There was also significant improvement in scores of the balance subscale (p = .0001). • BOTMP: There was a 1.0 change in the control group (8.0 pre to 9.0 post) vs. a 9.5 change in the intervention group (10.5 pre to 19.5 post) Critique & Conclusion: Strengths of this study included concealed, random allocation of subjects to the groups, as well as excellent compliance to the intervention with no withdrawals. A main weakness was no blinding of subjects, therapists or assessors. Another limitation was the small sample size. Overall, a 6week exercise training program was sufficient to signigicantly improve lower-limb muscle strength and overall balance in children with Down syndrome. The exercise training program implemented in this study was a safe and feasible intervention for improving strength and balance in children with Down syndrome. 23 Research Article #5 Rimmer JH, Heller T, Wang E, Valerio I. Improvements in physical fitness in adults with Down syndrome. American Journal on Mental Retardation. 2004; 109(2):164-174. Level of Evidence (Oxford scale): 2b Purpose: To evaluate the effects of a combined program of strength and cardiovascular conditioning in a cohort of adults with Down syndrome. Methods: This was a controlled clinical trial conducted at a major university. • Participants o 52 adults with DS aged 30 to 70 years. Mean age: 39.4 years. o Eligibility criteria included: sedentary for the past year or longer, reside within a one-hour commute of the intervention site, written permission from primary care physician, and able to understand instructions and complete all physiological testing. • Experimental Group Intervention (n = 30) o Four 12-week exercise sessions were conducted with 7 to 8 participants in each repetition. o 30-45 min of cardiovascular exercise on one or more: recumbent stepper, stationary cycle, treadmill, and elliptical cross-trainer. 15-20 min of muscular strength and endurance. o 5-20 min of muscular strength and endurance. Initiated at 70% of the participants’ 1-RM for one set of 10-20 reps, and increased by 10% when they could perform 20 reps for two consecutive sessions with proper lifting technique. Bench press, seated leg press, seated leg curl, triceps push-down, seated shoulder press, seated row, lat pull-down, and biceps curl. • Control Group Intervention (n=22) o Participants continued with their normal daily activities. 24 • Outcome Measures o Cardiovascular fitness: measured by a symptom-limited graded exercise test (peak VO2) performed on an electronically braked upright stationary cycle, with a Metabolic Cart. o Strength: assessed by 1-RM on the bench press and seated leg press machines. Handgrip strength was assessed using a handgrip dynamometer. o Body composition: skinfold measurement was taken with a skinfold caliper at the chest, abdomen, and thigh locations for men and at triceps, suprailiac, and thigh locations for women. The sum of the three measures was used as the actual score. Results: There were significant gains in all measures of cardiovascular function for the treatment group’s participants as compared to the control group. This included peak VO2, peak heart rate, time to exhaustion, and maximum workload. (p<.01). Improvements of cardiovascular fitness ranged from 14.1% in peak VO2 to an increase of 27.1% in max workload. Significant group differences were observed for bench press and leg press (p<.0001). Improvements in strength ranged from 39% and 43% for lower and upper-body strength respectively. There was also a significant difference between treatment and control groups for body weight (p<.01), but there was not a significant difference with respect to BMI or total skinfold score. Critique & Conclusion: Strengths of this study include the large sample size of adults with DS for this randomized controlled trial to test muscular strength and endurance, random allocation of the subjects into groups, as well as good compliance and follow-up with the subjects. One of the main weaknesses of this study was the lack of blinding for subjects, therapists and assessors, which could have potentially led to bias in treatments and/or results. In conclusion, a combination of strengthening and cardiovascular exercise was a feasible and safe intervention that can improve muscular strength and endurance in older individuals with Down syndrome. Further research should be conducted to determine these effects with blinding of therapists and assessors. 25 Research Article #6 Carmeli E, Kessel S, Coleman R, Ayalon M. Effects of a treadmill walking program on muscle strength and balance in elderly people with Down syndrome. Journal of Gerontology: MEDICAL SCIENCES. 2002; 57A(2): M106-M110. Level of Evidence (Oxford scale): 1b Purpose: To evaluate the changes in isokinetic leg strength and balance ability and the benefits of a 6month walking program on improving leg muscle strength of elderly MR individuals with Down syndrome. Methods: This was an RCT, in which participants were randomly assigned to groups through a coin toss. • Participants o 26 individuals with Down syndrome, aged 57 to 65 years. o All participants were residents of a foster home in Hadera, Israel for at least 4 years. o Diagnosed with mild mental retardation, requiring minimal supervision for daily activities. o Subjects of the study were not taking drugs, such as sedatives or narcotics, which might have hindered balance or strength performance. o Exclusion: pre-existing conditions that could interfere with results or lead to comorbidity. • Walking Group (WG) (n = 16) o Participants walked on the treadmill three times per week, for 25 consecutive weeks. Initially for 10 to 15 minutes as tolerated and then gradually for as long as 45 minutes. Walked at a speed below threshold of breathlessness, but as fast as they could tolerate. Individually prescribed low-endurance walking at 0% incline. o Participants walked only between 9:30 and 11:30am indoors under controlled conditions. o Heart rate, blood pressure and respiration rate were recorded before and after each training session for monitoring purposes. 26 • Non-walking Control Group (CG) (n=10) o Participants were instructed not to change their current levels of physical activity. • Outcome Measures o Knee extension and knee flexion strength were measured bilaterally, using a medical isokinetic system. Data were collected for peak torque (greatest single value of 3 maximal efforts), peak torque % body weight, and average power (expression of work per unit time). o Dynamic balance and gait speed assessed using the TUAG test (a modification of the Timed Up And Go). Each participant was asked to rise from an armchair, walk 6m, and return to the chair. Target time for a good level of IND was between 26 and 30 seconds. o 95% confidence intervals of difference were determined. The critical valued for statistical significance was assumed at an alpha level of p < 0.05. Results: Following the walking program, peak torque, and average power of quadriceps and hamstrings were significantly higher than the pre-walking values. Results of the TUAG test for the WG after training were significantly faster in comparison with pre-walking speeds. They needed 9.1% less time to complete the test (p<0.05). All walking performances improved significantly as a result of the treadmill program. Critique & Conclusion: One of the strengths of this study was that it was the first published study showing a relationship between leg strength and functional ability in aged individuals with Down syndrome. Other strengths include random allocation of subjects to groups, blinding of all assessors, participants reported that they enjoyed the treadmill walking, and none withdrew from the study. Lastly, it was a longer intervention of 6-months. Weaknesses included lack of blinding of subjects and therapists, which could have led to bias in interventions and/or results. Overall, it was concluded that a regular treadmill walking protocol led to improvements in leg strength, walking function, and balance for elderly adults with Down syndrome, which in turn can potentially increase daily independence and improve functional capabilities. 27 Research Article #7 Ulrich DA, Ulrich BD, Angulo-Kinzler RM, Yun J. Treadmill training of infants with Down syndrome: evidence-based developmental outcomes. Pediatrics. 2001;108(5). DOI:10.1542/peds.108.5.e84. Level of Evidence (Oxford scale): 2b Purpose: To test the impact of treadmill practice, administered in the homes by parents, on the rate of onset of walking in infants with Down syndrome. Methods: This was a 4-year randomized controlled trial, in which families of infants with Down syndrome were randomly assigned to the intervention or control group. • Participants o 30 infants with Trisomy 21 were recruited from support groups and DS clinics. o Infants began participating in the study when they demonstrated the ability to sit independently for 30 sec. o Throughout the course of their participation in the study, all infants received biweekly pediatric physical therapy. • Experimental Group Intervention o Custom-engineered treadmills were placed in their homes. Parents were trained to position their infants appropriately and implement the treadmill protocol. o Parents administered the treadmill intervention. Infants gradually worked up to 8 minutes per day, 5 days per week, until the infants demonstrated the ability to walk independently (item 62 of BSID-II). Treadmill belt speed was set at .2 meters per second. o Parents maintained a logbook and researchers visited bi-weekly, and videotaped monthly. • Control Group Intervention o Did not participate in a treadmill protocol, but did receive the biweekly physical therapy. 28 • Outcome Measures o Motor performance was assessed with the Bayley Scales of Infant Development. o Items monitored during each biweekly follow-up session included: raises self to standing position, walks with help, and walks independently (3 steps). o Parent-reported milestone achievement was also verified at each biweekly visit. o A significance level of p = 0.05 was used for all analyses. Effect size of .5 was identified as representing a meaningful treatment. Results: Infants assigned to the treadmill group acquired the three behaviors faster than the control group. • Raises self to stand: No statistically significant difference (p = .09). Moderate effect size of (.61). • Walks with help: Statistically significant difference (p = .03). Large effect size statistic (.80). • Walks independently: Statistically significant difference (p = .02). Large meaningful effect size (.83). o Infants in the treadmill group demonstrated the ability to walk independently 101 days earlier than those in the control group. o Infants assigned to the treadmill group walked on average at a chronological age of 19.9 months, while the control group walking on average at a chronological age of 23.9 months. Critique & Conclusion: One main weaknesses of this study was the lack of blinding of subjects, therapists and assessors, which could have led to potential bias in interventions and outcomes. Another weakness was the decreased control of environment and protocol variability, due to the intervention taking place in the infant’s home. However, having the intervention performed in the home allowed for the parents to be involved in their own child’s intervention and development, which could also be considered a strength. With training and support, parents could use treadmills in their homes to help their infants walk earlier than they typically might. In conclusion, regularly scheduled physical therapy could be supplemented with in-home treadmill training, performed by parents, to facilitate significantly earlier onset of independent walking. 29 Research Article #8 Ulrich DA, Lloyd MC, Tiernan CW, Looper JE, Angulo-Barroso RM. Effects of intensity of treadmill training on developmental outcomes and stepping in infants with down syndrome: a randomized trial. Physical Therapy. 2008; 88(1):114-122. Level of Evidence (Oxford scale): 2b Purpose: To test the effects of individualized, progressively more intense treadmill training on developmental outcomes in infants with Down syndrome. Methods: This was a randomized controlled trial, in which infants were randomly assigned to the higherintensity, individualized training group or the lower-intensity, generalized training group. • Participants o 30 infants with Down syndrome. o Eligibility criteria included: presence of a seizure disorder, non-correctable vision problems, and any other medical conditions that would severely limit a child’s participation in the treadmill intervention. o A criterion for starting the treadmill intervention was the ability to take a minimum of 6 supported steps in a given minute on the treadmill. Most of the infants began the intervention at 10 months of age. • (HG) Higher-Intensity, Individualized Training Group (n = 16) o Training protocol included 8 minutes per day, 5 days per week, at a belt speed of 0.15m/s. o As infants progressed, ankle belts were added, belt speed was increased, and daily duration was increased in an effort to maximize the stepping response. These conditions were initiated once the infants displayed the ability to take 10 steps per minute and increased when they were able to take 20, 30, and 40 steps per minute. 30 • (LG) Lower-Intensity, Generalized Training Group (n=14) o Training protocol included 8 minutes per day, 5 days per week, at a belt speed of 0.15m/s. • Outcome Measures o Motor performance was assessed with the Bayley Scales of Infant Development. o Items monitored during each biweekly follow-up session included: moves forward using pre-walking methods, raises self to standing position, walks sideways,/cruises, walks with help, stands alone, walks alone and walks alone with good coordination. o Parent-reported milestone achievement was also verified at each biweekly visit. Results: Analysis of variance of the change in the numbers of alternating steps taken from study entry to onset of independent walking revealed significant time (p<.0001) and interaction (p<.05) effects for the HI group. Only moves forward using pre-walking methods and raises self to standing position reached statistical significance (p=.01 and p=.05, respectively). There were no significant differences between the HI and LG groups for the other milestones. Statistical power was low for each of these statistical tests and was influenced by the loss of 6 infants during the study. • Infants in the LG group walked independently, on average, at 21.3 months. • Infants in the HI group walked independently, on average, at 19.2 months. Critique & Conclusion: One of the most significant weaknesses of this study was the lack of blinding to subjects, therapists and assessors, which could have led to biases in he intervention results. Another weakness was insufficient follow-up for analysis, due to 6 infants being lost during the intervention. A strength of this study was the ability of parents to be personally involved in implementing their child’s intervention and assisting them to potentially walk earlier than they typically might. In conclusion, regularly scheduled physical therapy can be supplemented with treadmill training, including high intensity, individualized training, to facilitate a significantly earlier onset of independent walking 31 Discussion After reviewing the selected research articles testing progressive resistance training, strength training, agility training and treadmill training, it was found that such interventions have beneficial effects on individuals of all ages with Down syndrome (Table 2). In infants, Ulrich found that regularly scheduled physical therapy could be supplemented with treadmill training implemented by parents in the homes to facilitate a significantly earlier onset of independent walking9,10. A treadmill walking program, three times per week for 25 consecutive weeks, was also found to significantly improve muscle strength and balance in aged adults2. In regards to strengthening programs, one study found that a 12-week program with a combination of strengthening and cardiovascular exercise had a significant effect on upper and lower body strength, as well as cardiovascular fitness in adults with DS6. In addition, 10-week progressive resistance training programs were found to improve upper-limb muscle endurance and strength in adolescents7 and lower-limb muscle strength in adults8 with DS. A 6-week progressive resistance exercise program was also shown to improve lower-limb strength and overall balance in children with DS3. Finally, a study conducted by Lin and Wuang (2012) found that lower extremity muscle strength and agility performance in adolescents with DS improved significantly after a 6-week exercise program with a combination of treadmill and virtual reality game protocols4. These findings are significant because individuals of all ages with Down syndrome often experience decreased upper and lower limb muscle strength. Among motor impairments demonstrated by individuals with Down syndrome, muscle strength is one of the essential abilities in order to achieve effective and functional movements4. These improvements can facilitate an earlier onset of walking, allowing for greater independence with functional mobility, as well as interaction with the environment and play. Improvement in strength has also been associated with positive changes in functional activities in adults with Down syndrome2 and in work-related skills in people with intellectual disability12. 32 Conclusion The results of the research studies provided an answer for my posed clinical question. It was found that various strengthening interventions, such as progressive resistance training, treadmill training, and agility training, have the potential to lead to improved level of independence in individuals with Down syndrome. With improved strength, these individuals are better able to perform activities of daily living and carry out their social and vocational roles, leading to decreased dependency on others, and in turn, potentially improving their overall quality of life. If I had the opportunity to work with my case study patient for a longer duration and outside of an acute care setting, I would include treadmill training as a part of my interventions and plan of care. At my next pediatric rotation and in the future as a physical therapist, I will be incorporating these types of interventions for my patients. They are feasible, relatively inexpensive, safe, and most importantly have evidence to show that they can improve the strength for individuals with Down syndrome. 33 References 1. American Academy of Orthopaedic Surgeons. Down Syndrome: Musculoskeletal Effects. American Academy of Orthopaedic Surgeons. http://orthoinfo.aaos.org/topic.cfm?topic=A00045. October 2007. Accessed February 10, 2013. 2. Carmeli E, Kessel S, Coleman R, Ayalon M. Effects of a treadmill walking program on muscle strength and balance in elderly people with Down syndrome. Journal of Gerontology: MEDICAL SCIENCES. 2002; 57A(2): M106-M110. 3. Gupta S, Rao BK, SD K. Effect of strength and balance training in children with Down syndrome: a randomized controlled trial. Clinical Rehabilitation. 2011; 25: 425-432 4. Lin H-C, and Wuang Y-P. Strength and agility training in adolescents with Down syndrome: A randomized controlled trial. Research in Developmental Disabilities. 2012; 33: 2236-2244. 5. Palisano RJ, Walter SD, Russell DJ, Rosenbaum PL, Gemus M, Galuppi BE, et al. Gross motor function of children with Down syndrome: creation of motor growth curves. Archives of Physical Medicine and Rehabilitation. 2001; 82:494-500. 6. Rimmer JH, Heller T, Wang E, Valerio I. Improvements in physical fitness in adults with Down syndrome. American Journal on Mental Retardation. 2004; 109(2):164-174. 7. Shields N. and Taylor NF. A student-led progressive resistance training program increases lower limb muscle strength in adolescents with Down syndrome: a randomized controlled trial. Journal of Physiotherapy. 2010; 56:187-193. 8. Shields N, Taylor NF, Dodd KJ. Effects of a community-based progressive resistance training program on muscle performance and physical function in adults with Down syndrome: a randomized controlled trial. Archives of Physical Medicine and Rehabilitation. 2008; 89:1215-1220. 34 9. Ulrich DA, Lloyd MC, Tiernan CW, Looper JE, Angulo-Barroso RM. Effects of intensity of treadmill training on developmental outcomes and stepping in infants with down syndrome: a randomized trial. Physical Therapy. 2008; 88(1):114-122. 10. Ulrich DA, Ulrich BD, Angulo-Kinzler RM, Yun J. Treadmill training of infants with Down syndrome: evidence-based developmental outcomes. Pediatrics. 2001;108(5). DOI:10.1542/peds.108.5.e84. 11. Zieve D, Eltz DR, Slon S, Wang N. Down Syndrome. Medline Plus Medical Encyclopedia. http://www.nlm.nih.gov/medlineplus/ency/article/000997.htmm. Updated May 16, 2012. Accessed February 10, 2013. 12. Zettz R, Horvat M, Langone J. Effects of a community based progressive resistance training program on the work productivity of adolescents with moderate to severe intellectual disabilities. Education & Training in Mental Retardation. 1995; 30:166-178. 35
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