Rehabilitation and Recovery

Rehabilitation and Recovery in the ICU

1. Early involvement of PM&R, physical therapy, and occupational therapy is recommended.

• Early initiation of physical, occupational, and speech therapy services is essential in the intensive care unit, to address issues related to positioning, progressive and stepwise mobilization, splinting/bracing, communication, and swallowing.
• Early initiation of electrical stimulation therapy may be helpful to reduce disuse muscular atrophy
• Early mobilization may help to promote functional improvement, reduce complications of immobility, and mitigate the effects of muscle disuse. Strengthening programs can often begin in the ICU.

Inpatient rehabilitation

After the acute phase of AFM, medically stable children with significant residual neurologic deficits should transfer to an inpatient rehabilitation program with a multidisciplinary team.

• Intensive rehabilitation should include short-term goals to facilitate developmentally-appropriate functional independence and use of compensatory devices, while simultaneously working towards long-term goals for recovery of function and avoidance of musculoskeletal complications including muscle atrophy, bone mass loss, joint contractures, hip and/or shoulder subluxation, limb length inequality and scoliosis.
• Intensive activity-based therapy interventions should be tailored to each individual patient and can include some of the following:

1. 1. Weight loading of upper and lower limbs
      • Weight loading across a joint with the goal to promote proper joint alignment and muscle co-contraction surrounding the joint can be achieved in the upper limbs via propping on extended arms or forearms while in sitting, standing, prone, side-lying, quadruped, or kneeling; lower limbs weight-bearing is completed by standing, kneeling, FES cycling, or quadruped positions.
      • Patients who are unable to weight bear on their own can be provided with assistance from a therapist, bracing, or other external supports. Supported static and dynamic standers, standers with glider components to allow reciprocal upper and lower extremity movement, and body weight supported standing in harness systems can be utilized where necessary, and depending on the patient’s age and abilities
   2. Locomotor gait training (LT)
      • Locomotor gait training includes interventions aimed at retraining neural patterns to improve walking by providing specific sensory cues.
      • LT focuses on proper gait kinematics, full joint loading, and avoiding compensatory motions or devices.
      • LT is shown to improve trunk strength and neuromuscular capacity in pediatric patients after spinal cord injury (even in non-ambulatory children). However, there is a lack of evidence regarding the potential benefit or otherwise of LT in patients with AFM.
      • LT sessions can include facilitation over the treadmill, overground, and carryover activities intended for the patient to practice outside of therapy.
   3. Task-specific and massed practice with high count repetitions
      • Task-specific practice (the completion of specific motor tasks to improve motor learning) should be goal-directed and be associated with massed practice (high number of repetitions).
      • Children participate in task-specific practice activities including activities of daily living (ADL), instrumental activities of daily living (iADL), transfer training, developmental transitions, and mat mobility skills daily in therapy. -The tasks should be repeated multiple times a day, over multiple days, to improve strength, range of motion, and increase independence as cortical reorganization responds to non-use as much as it does to use and training.
   4. Functional electrical stimulation (FES).
      • FES can be used in conjunction with the above therapeutic modalities to facilitate muscle contraction in weak or partially denervated muscles. The motor response to electrical stimulation is noted to be decreased in patients with AFM due to lower motor neuron injury.
      • The most successful applications of FES for this kind of injury are the use of a low frequency and long pulse width, which allows for time for the slower moving motor units to respond with greater refractory periods.
      • Frequency can typically range from 20-40Hz; beginning at a high frequency and decreasing as tolerated by the patient.
      • Pulse width can be optimized by the motor response, up 3000 microseconds in some software programs; typical home units will have a maximum pulse width of 300-400 microseconds.

• Activity-based therapy principles can also be applied in the aquatic setting and can include developmental play and positioning, standing, kneeling, quadruped, and sitting. Aquatic LT is possible with the use of an underwater treadmill and/or assistance from a therapist
• Consider orthotic devices, mobility equipment, assistive technology, identification of home care needs, a plan for school and community re-entry, psychosocial support, and education for the child and family.
• In low resource settings with limited access to skilled therapy, education of the patient and care-givers regarding home-based activities is essential.

Outpatient rehabilitation and Long-Term Management

• Because AFM predominantly affects young, growing children who continue to improve over time, after discharge, continued rehabilitation with periodic bouts of skilled activity-based therapy should be provided to aid the acquisition of developmentally appropriate milestones and functional independence.
• Interdisciplinary reevaluation with a review of medical management and rehabilitation goals should occur every 3-4 months during the first year, and every 6-12 months during subsequent years. This includes a review and update of orthotic devices, trials of new equipment for mobility, progressing age-appropriate ADLs and communication, and updates to home/community rehabilitation programs.
• Ongoing specialty care is also needed and includes neurology, physiatry, and orthopedic surgery. It is focused on the prevention and management of musculoskeletal conditions including muscle atrophy, joint and soft tissue contractures, scoliosis, shoulder and/or hip subluxation, limb length discrepancies, and loss of bone mineral density.
• The children with significant residual bulbar paralysis and impaired breathing may require pulmonology, otolaryngology, and speech-language pathology care for prevention and management of complications including respiratory insufficiency, recurrent pneumonia, sleep-disordered breathing, ventilator weaning/transition to non-invasive ventilation, and speech dysfunction.
• Educational and developmental transitions, age-appropriate self-advocacy skills, increasing independence in self-care, and responsibility for medical management will aid the successful transition to adulthood.

Psychosocial Support for Children and Families

Psychosocial support is critical for the emotional and behavioral well-being of the child and caregivers. Distress behaviors can directly impact participation in therapies and cares and therefore should be addressed and in the center of the care plan.

Below are ways in which psychological support can be introduced, but psychology consultation is encouraged when available:

1. Incorporate preferred interests and items into therapy sessions.
2. Provide intermittent opportunities for control through choice such as allowing the child to choose the order of therapy activities.
3. Utilize differential attention (i.e. positive praise for adaptive behaviors and planned ignoring of maladaptive behaviors) for behavior management. Targeted reinforcement programs may be considered.
4. Procedural anxiety may occur during various interventions such as tracheostomy changes, ventilator weaning, PMV and capping trials, electrical stimulation, etc. Utilize desensitization to medical and therapy equipment, pre-determined and structured goals, and developmentally appropriate procedural education.
5. Separation anxiety may include crying and frequent requests for the caregiver, repositioning, suctioning, etc. Educate caregivers on intervention including notification of departure and anticipated return, a brief separation process, and return at a pre-determined time. Systematically increase the frequency and duration of separation over time.
6. Provide relaxation and coping skills training (e.g. diaphragmatic breathing, guided imagery, brave statements) for distress. For younger children, include caregivers so they can assist with prompts and generalization of strategies throughout the day.
7. Provide support for caregiver coping and adjustment as they grieve the sudden and chronic changes in their child’s functioning. Specialized education and peer support may also be beneficial for caregiver coping.
8. Teach families how to use modifications to play such as adaptive equipment or hand-over-hand support to engage in games and activities. Teaching healthy siblings new and adapted ways to play with their affected sibling may positively impact sibling relationships.
9. Encourage caregivers to consistently schedule individual time with healthy siblings.
10. Provide brief education regarding AFM to the child and their siblings in developmentally appropriate terminology. In preparation for return to home, school, and community environments, consider developing social scripts for the child to talk about their condition and hospital admission.

Nerve Transfer Surgery

• Children with a poor recovery in an affected muscle group or diaphragm greater than 3 months after onset should be considered for potential nerve transfer surgery by a center experienced in the relevant procedures. The appropriate timing for nerve transfer surgery is uncertain, but a delay in consideration may result in a missed window of opportunity as muscle viability wanes with extended periods of denervation.
• Tendon transfer surgery – which is not time-sensitive – may be considered months or years after the initial onset of AFM.