By: Helene M. Dumas
Published in Hindawi Journal
December 24, 2012
Activities of daily living (ADLs) include feeding, bathing, toileting, dressing, grooming home/environment management, and home, school, and work activities. ADLs vary by age, gender, care setting, disease limitations, and family expectations. Children dependent on mechanical ventilation face unique challenges in gaining and/or maintaining independence with these self-care activities, while providers are met with the challenge of how to intervene and measure changes in daily function [49, 51].
Studies examining activities of daily living that include children with dependence on long-term mechanical ventilation indicate a reduction in self-care independence and ability. In a study assessing the functional status of children across all types of Spinal Muscular Atrophy (SMA), all children with SMA type I were dependent on a ventilator and all required assistance with self-care activities . In another study of 30 infants and children with varying diagnoses but who required oxygen and/or ventilator support upon admission to an inpatient pulmonary rehabilitation program, admission and discharge scores were noted to be on the lower half of the self-care continuum, indicating deficits in self-care function, despite increases in scores from admission to discharge . The Functional Independence measure has been shown useful for detecting changes in function from admission to discharge for adults in pulmonary rehabilitation programs .
Independence with self-care activities for a child dependent on long-term MV will vary based on primary diagnosis, age, cognitive and physical impairments and may require modifications including adaptive equipment, assisted set-up, altered procedures to conserve energy, and emergency problem-solving. It has been recommended that design improvements of medical technology for use at home are needed .
For infants and young children, feeding and playing are primary ADLs and dependence on caregivers is otherwise expected. Infants who are dependent on MV however, have experienced altered positioning, handling, and oral motor experiences. Oral aversion becomes a concern as oral intake may have been limited in the early weeks or early months of life. Often, oral intake is then inadequate to meet the caloric needs of a growing infant dependent on MV. In addition, infants are at risk for aspiration and as such, a nasogastric tube may be placed for short-term use . Eventually, a gastrostomy tube may be needed to replace the nasogastric tube . For older children, oral intake still may not be adequate to meet caloric and nutritional requirements and the continued use of a gastrotomy tube will be required .
For children with genetic and central nervous system disorders, bladder and/or bowel function may be impaired . Regardless of the primary diagnosis, the use of mechanical ventilation may restricttoileting activities because of the restrictions of the ventilator tubing and environmental obstacles. As with any child or adult requiring environmental modifications, equipment such as grab bars, a raised or adapted toilet seat and/or a commode may be needed. Along with the child and family, rehabilitation team members can consult with durable medical equipment vendors to explore options for each of the child’s care environments (e.g., school, home) for toileting.
Bathing options, similar to toileting options will be directed by the child’s cognitive and physical impairments due to the underlying disease or disorder, the child’s physical care environment as well as because of the child’s ventilator dependence. Some children may spend short periods of time off of the ventilator and can bathe when off of the ventilator while other children may not.
Adaptive equipment is available for children who are physically able to independently dress or participate in dressing and grooming activities. Equipment such as long-handled reachers and extended handle hair brushes and combs are available. These devices allow rehabilitation providers and family members to encourage participation in activities of daily living and minimize energy expenditure for each child. It has been recommended that clothing that blocks the tracheostomy or access to any tubing be avoided. A myriad of home modifications and adaptive equipment options are available and should be individualized based on the child’s needs and environment .
The percent of children who were ventilator dependent and engaging in daily home, school and work activities was documented by Lumeng et al. . Results indicate that more than 97% of children who were ventilator-dependent listened to music or watched television daily while approximately 74% attended school, and 53% participated in computer activities. Frequent suctioning of the tracheostomy tube has been reported as a persistent interference with ADL independence , promoting ongoing dependence on a caregiver.
While children dependent on prolonged MV do not share a common diagnosis, there are many physical impairments common across diagnoses and conditions that may impact functional mobility and exercise participation and capacity. Skeletal muscle weakness, limited muscle-tendon flexibility, and skeletal and joint deformities due to a musculoskeletal or neuromuscular disorder or disuse, in addition to the cardiopulmonary impairments and potential cognitive limitations, consequently decrease functional mobility [81, 82].
In a study of 30 infants and children with varying diagnoses but who required oxygen and/or ventilator support upon admission to an inpatient pulmonary rehabilitation program, mean changes in functional mobility scores from admission to discharge were significant. Similar to the self-care scores noted earlier in this same study, scores were at the low end of the scoring range, indicating limited mobility function .
Exercise training is a foundation of pulmonary rehabilitation programs for adults with chronic obstructive pulmonary disease (COPD), pulmonary hypertension and heart failure [83–85]. A review of mobility interventions for adults on prolonged MV revealed, however, that most studies introduced mobility intervention upon patient transfer to rehabilitation but not in the ICU during the earlier stages of illness and recovery. The review showed support for mobility interventions to improve weaning outcomes in patients on prolonged MV but a limited number of studies examine a variety of interventions (e.g., electrical stimulation, upper extremity exercise, inspiratory muscle training) and there is no report of improvement in functional mobility . As with any diagnostic group, exercise limitations and restrictions should be determined prior to the start of an exercise program and children should be supervised closely to avoid musculoskeletal injury, excessive increases in heart and respiratory rate and dislodgement of tubing.
Ventilator dependence often dictates limited movement and mobility if the child must remain close to a non-mobile ventilator. Additionally, young children often spend little to no time in a prone position and this often only occurs during therapy. Exercise and activity in a prone position is useful as it provides an opportunity for strengthening of neck and shoulder girdle musculature. Rotational movements also are often constrained by the child’s equipment limitations and/or impairments but should be encouraged as they are important components of mobility activities including creeping and ambulation. For children of all ages, upright positioning in sitting or standing is also important for physiological function and bone density and should be encouraged when developmentally and medically appropriate. Activities such as pull-to-stand and cruising may only occur in a crib if a child is in a hospital with limited opportunities for play time on the floor.
For children with ventilator dependence, functional mobility training may include bed mobility, transitional movements such as getting up from the floor or transfer training including getting in and out of bed and in and out of a wheelchair. There are no studies detailing motor or mobility interventions and their effectiveness for children dependent on prolonged MV. One could assume that the dependence on MV would have an impact on developmental motor skills if for no other reason than because of the child’s restricted and constrained movement due to the ventilator and its tubing. It is unclear however, if there is a relationship between ventilator use, ventilator weaning outcomes and motor and mobility performance. As well, there are few reports of weaning outcomes that include motor/mobility skills. Reports of developmental motor outcome in survivors of bronchopulmonary dysplasia (BPD) are inconclusive as there is conflicting evidence demonstrating a relationship between the length of time a child is ventilated and developmental outcome. Several studies report that the shorter time an infant with BPD uses a ventilator, the better the developmental outcome [86–88]. Others however, show no significant correlation between duration of MV and overall developmental prognosis, hypothesizing that the impact of other medical complications (e.g., hypoxic episodes, low birthweight) have a greater effect on developmental outcome than MV .
In a recent case study report, achievement of independent ambulation while a child was using CPAP is reported. The progress made when an 18-month old with chronic lung disease and tracheobronchomalacia was provided a new CPAP device that allowed compressed air to be delivered via trachesotomy tube with extended tubing is described. It was hypothesized that the shorter, heavier tubing of the traditional CPAP device had severely restricted the child’s mobility but application of the new CPAP device afforded the child the additional space to move (up to 10 meters) .
Ventilator settings may be influenced by the child’s physical response in areas such as strength, functional skills and endurance, provided these findings are communicated to the medical care team. It has been noted that the observations and ongoing clinical assessment made by rehabilitation therapists can be critical factors in weaning decisions and these individuals can be a valuable resource in clinical decision making . It has also been the experience of this author that therapy participation may be limited when children are being actively weaned from the ventilator. Infants may sleep more and older children often do not tolerate the same level of physical activity until they accommodate to the increased demands on their respiratory system. It is essential that the team coordinate the management of these children at all levels of their care. Additionally, it is essential that therapists be skilled in the assessment and monitoring of exercise capacity and limitations and that outcome measures reflect these parameters .
Appropriate equipment can minimize caregiver dependence and assist a child to attain independence with functional mobility. Orthoses may be needed to manage joint contractures, maintain joint flexibility, or provide proper alignment of the trunk, feet, legs, hands, or arms. For a child who may require a trunk orthosis secondary to weak or paralyzed axial musculature or those being treated for scoliosis, it is important that the orthosis allow for full rib cage expansion. Adaptive equipment may also be used to minimize the chance of secondary impairments due to the underlying disease process and/or immobility. Young children may require specialized adapted strollers with ventilator carrying capability. Older children may require manual or power wheelchairs adequately outfitted to transport a ventilator. The ventilator and other equipment must be safely secured to the seating system. Children who are independent ambulators may carry their ventilator in a traditional backpack, a rolling backpack, in a pushcart, or be accompanied by a caregiver who transports the ventilator using any of the listed options. For those children who require a wheelchair, multiple options are available for either manual or power wheelchairs and an experienced clinician and durable medical equipment vendor can help the child and family determine the safest, most energy efficient, and feasible seating and mobility system .
In addition to considering the functional mobility of children dependent on long-term MA, transporting children dependent on MV in a motor vehicle presents a challenge for caregivers. In a study by Lumeng et al.  examining quality of life of children who are ventilator dependent, it was reported that children rode in a car between zero and 30 times per month. In 1999, the American Academy of Pediatrics released guidelines for transporting children with special health care needs including recommendations for children with tracheostomies. These recommendations include use of a rear-facing care safety seat with a three-point harness or a care safety seat with a five point harness and avoiding the use of a harness-tray/shield combination. The American Academy of Pediatrics has published a policy statement and guidelines for the transport of children with special health care needs. These guidelines include securing electrical equipment to the floor or seat of the vehicle . In addition, when transporting a ventilator, it has been our typical practice for the child to have a spare tracheostomy tube, ambu bag, oxygen tank, portable suction unit with sterile catheters, and an external battery for the ventilator. More recent and specific guidelines can be found for many states and individual towns and counties within the United States including the use of battery power, tie-downs, and securing the ventilator in a car seat.
As noted earlier, children dependent on long-term MV have had a tracheostomy placed to provide a stable attachment for the ventilator tubing. Placement of a tracheostomy tube re-directs the natural inspiratory and expiratory flow of air from the nose and oropharynx to the path of least resistance (the artificial opening of the tube) resulting in minimal airflow across the vocal folds. This diminished airflow reduces vocal intensity to inaudible levels, altering a child’s ability to vocalize and negatively affects the development of expressive language skills in children .
Naturally, it would be expected that infants and young children with a tracheostomy and subsequent reduced vocalization miss out on many of the critical steps toward speech development and early forms of communication including cooing, babbling and crying. For the older child who has undergone tracheostomy placement, there is often a loss of the ability to communicate verbally. Reports indicate that tracheostomy affects speech and language development in children with and without neurological disorders. Established factors influencing speech and language development in children without neurological involvement include the child’s age at the time of tracheostomy placement and the duration of the tracheostomy until decannulation .
Children with long-term MV via tracheostomy may need to use multiple methods of communication depending on their age, cognitive development and neurological status . In a study examining quality of life for children using mechanical ventilation at home, the communication modalities of the 35 child participants were noted to be as follows: oral communication (71%), oral communication combined with other modalities (9%), eye blinking only (6%), communication device (3%), combination of communication modalities without oral communication (3%), and other methods (9%) .
In another study that included 35 children who were ventilator dependent and recruited to describe their health and quality of life, six children required the help of a caregiver to assist with communication, one child had no speech or established method of communication (parent read movement of lips), and the remaining 22 children had difficulty communicating, such as having speech that was affected by the ventilator and/or their tracheostomy .
Speech can be facilitated in patients with a tracheostomy tube who are breathing spontaneously by use of a talking tracheostomy tube, by using a cuff-down technique with finger occlusion of the proximal tracheostomy tube, and with the use of a cuff-down technique with a speaking valve . A cuff is a soft balloon around the distal end of the tube that is inflated to provide an adequate seal to allow for mechanical ventilation.
Attachment of a one-way speaking valve to the external hub of the tracheostomy tube allows expiratory flow of air around the tube to instead exit up through the larynx and vocal folds rather than the tracheostomy tube promoting phonation. Improved verbal communication has been documented with the use of tracheostomy speaking valves in adults  and children . In a study by Hull et al. , all 10 children who tolerated a speaking valve achieved phonation. Vocalizations included audible crying, non-specific vocalizations, word approximations, single words, and short phrases.
A recent survey of speech-language pathologists in the United Kingdom indicated that the majority (71%) were confident managing patients with a tracheostomy while still seeking professional development opportunities .
11. Psychosocial Concerns
Findings on the psychosocial impact of long-term MV for children and their families are important for all rehabilitation providers to consider regardless of role or care setting. Integrated, supportive services are required to help counter the challenges and distress endured by children and their families. Home ventilation offers a viable option for children’s psychosocial development, social integration, and quality of life [10, 39, 52]. Many children who are ventilator-dependent can attend school and participate in family activities . Unfortunately, negative consequences for families exist including a physical burden, emotional turmoil, and financial hardship [59, 62, 100]. Time demands of care routines limit participation in employment and social opportunities for family caregivers.
Differences exist between family expectations and what professional care providers are able to provide . Home nursing support, in particular night nursing, is important for the health and well-being of familial caregivers of children dependent on long-term MV . Parents report regular disruption to their sleep  and the high demands related to the care of a child with long-term MV has been shown to be a significant risk factor for poor mental health outcomes of those mothers providing care at home . The need for improved collaboration and communication between families and caregivers has also been cited as a source of stress for parents [57, 105]. There continues to be a lack of trained care providers but sample training courses have been published to improve the quality of home care for children who are dependent on technology [56, 106].
Summary: This article explains some of the more daily struggles of children on mechanical ventilation. Considering I want to make the medical experience for these kids as least invasive as possible, it is important to understand how the ventilator fits into their daily life. After reading this article, I understand that it is a medical goal for the kids to be as independent as possible.