Judith C. Bierman

Physical therapists (PTs) who work with children see a wide spectrum of clients or students. Even if one considers the management of only those children with developmental disabilities or with cerebral palsy (CP), the scope of practice remains wide. The focus narrows substantially when considering the examination of and intervention for children with CP who are severely involved. These children represent a small number and are especially challenging for clinicians to manage.

In trying to best prepare future therapists for the entire breadth of potential practice, professional educators tend to emphasize the most prevalent issues or those the clinician would be most likely to encounter. Because children with developmental disabilities who are severely involved represent such a small percentage of the total population, therapists may be less prepared to manage the special needs of this population. In addition, there is a general perception that therapy is less effective for individuals who are more severely involved because these children may be perceived as being unlikely to make marked progress. Therapists managing a busy case schedule may elect to and be directed to provide more intensive therapy for children who are more likely to make faster functional gains.

Social Function: Participation and Participation Restrictions

After the birth of a child with special needs, the parents are faced with raising a child without specialized training. Typically, parents anticipate sharing in the development of motor milestones, such as rolling over, sitting, walking, running, bicycling, or maybe even the Olympics. Instead, parents of children who are severely involved observe their child struggling to achieve even basic skills. Strategies to teach motor skills employed with siblings are not effective in improving the desired skills. If referred for physical therapy and asked what their goals are for their child in therapy, those parents usually answer, “I want my child to walk.” The parent may not have any idea of what is actually possible.

Often parents at the time of diagnosis report that physicians stress what is not likely or possible to occur for their child. The parent may be clinging to the hopes and dreams of all parents for their children. However, the more realistically achievable gross motor skills may be that these children will sit safely in a car seat, that they will do a standing transfer from the wheelchair to the bed, or that they will take a few supported steps with the assist of a caregiver. These are not activities that parents routinely include in goal statements nor are they routinely included in standardized lists of developmental milestones. They are, however, activities that can dramatically alter participation for both a child with severe involvement and that child’s entire family.

Brandon’s diagnoses (listed in approximately chronological order) include intraventricular hemorrhage (IVH, bilateral grade IV), bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP, stage III+; resolved), hydrocephalus, CP, cortical visual impairment, severe mental retardation, seizure disorder, oral motor dysfunction, gastroesophageal reflux disease (GERD), chronic sinusitis, chronic pneumonia, hypothyroidism/hypoadrenalism, scoliosis, gastrostomy tube (G-tube) for all nutrition, osteopenia, and bilateral hip dysplasia.

Social Function—Participation and Participation Restrictions

Brandon was always a full participant in family life. Once living at home, he shared a bedroom with his twin and slept at the same hours. As he became more medically fragile, he was moved into his own bedroom because his sleep and wake cycles were less consistent. His room was centrally located so that he could be a part of family life, and it was spacious enough to accommodate all of his equipment. His bedroom looked like a boy’s bedroom rather than a hospital room. Brandon’s family moved twice to better accommodate his needs in the home and to support his inclusion in family life. The family had meals together throughout Brandon’s life, even though Brandon was fed by a G-tube. They enjoyed music and had regular video nights at home. Brandon participated in all of these activities, whether in his wheelchair, his special activity chair in the family room, his stander, or in one of his parents’ arms.

Brandon and his brothers liked to play together, especially when he was upright in his chairs or stander. Brandon liked to shake bells secured to his wrist and did it best in supported standing. He and his brother would dance together in this fashion. His family life did change significantly when he was 5 years old with the addition of a younger brother. He had to learn to share his parents’ attention even more, to play with someone younger than himself, to wait for short periods, and to take turns. His home environment was now more active and complex.

Brandon initially attended a public preschool for children with special needs and then an elementary school in a self-contained class for children with severe and profound physical and cognitive impairments. He attended the same school as his brother for the initial years. It was not the neighborhood school, making it more difficult to build a convenient group of friends nearby. As the differences between Brandon and Blake’s educational needs widened, the boys attended different schools. Neither Brandon nor Blake could ride the regular school bus. Their mother chose to drive Brandon to school because it was difficult to position him safely in his chair for the long rides without her watchful eye. By 8 years of age, his health eventually prevented him from attending school, even in a small self-contained class, so educational services were provided on a homebound basis with a teacher coming in on a weekly basis.

Brandon initially participated in early intervention with home-based occupational therapy, physical therapy, and speech-language pathology services. His parents selected to have clinic-based outpatient therapy services where therapists were more familiar with a medical model required for his care and directed toward meeting his specific needs. At age 2, he began out-patient therapy services when his health permitted. He also attended frequent medical visits with a wide range of specialists, in both Augusta and Atlanta, Georgia. He lived at home whenever care could be provided there, even when it required multiple hours of home health nursing care. He required multiple trips to the hospital, however, for stays varying from several days to a month at a time. Hospitalizations were based on respiratory infections, one cardiac arrest, severe gastrointestinal (GI) problems, and several episodes of infections that were difficult to diagnose and treat. Most visits entailed PICU stays, with an occasional stay on the regular pediatric floor. As time progressed, the home environment was adapted to provide most of the needed medical equipment and supplies of a hospital room, and Brandon’s mother and regular in-home nurses were able to provide closer supervision. Brandon’s mother was also better able to detect and respond to his signs of distress or recovery compared with unfamiliar hospital staff. The entire family dynamics changed when Brandon was in the hospital. Blake especially missed his brother and would at times report that he had some unnoticed medical problem that required a trip to the hospital.

Individual Function—Activity and Activity Limitations

Brandon’s functional activities varied based on his health. He could usually reposition himself by rolling when uncomfortable if positioned in prone, supine, or side lying. He could, at different points in his life span, sit independently, either in a modified long sit position or in supported bench sitting with upper extremity (UE) support, and he enjoyed sitting in a wide variety of chairs outside his wheelchair. He also, at times, could assist with standing transfers between these chairs or when preparing to be picked up and carried if supported by a caregiver and when wearing his ankle-foot orthoses (AFOs). He could usually stand with support of his stander, a partial body-weight-bearing device (PBWB), or an adult to watch videos or play with his brother. He could take some assisted steps during his healthier periods. These skills would improve as his health did and then disappear for periods after each hospitalization or when his oxygen saturations remained very low. His desire to accomplish daily tasks never decreased, and he worked hard each time after a medical crisis to regain those skills.

Body Structure and Function: Integrities and Impairments

Multisystem Issues Related to Posture and Movement

Brandon required support in all antigravity postures. He could lie independently in prone, supine, or side lying but required support for sitting and standing. As a pre-schooler, he could roll quickly from prone to supine and developed head control for sitting and standing for short periods of time. In all postures, Brandon always assumed a large base of support and was asymmetrical. His head was usually rotated and laterally flexed to the right and hyperextended. He had a significant scoliosis. Surgical spinal stabilization corrected this spinal asymmetry and made sitting far easier for him. His extremities also demonstrated the asymmetry, the lower extremities (LEs) more so than the UEs.

He had a paucity of movement, especially when his O₂ saturation levels (SaO₂) were low. He tended to move in full flexion or extension, but the range of movement was altered by his surgeries. When feeling well, he could demonstrate reciprocal flexion and extension in the LEs and could reach with either arm. He could move his extremities but had difficulty after each surgery developing the control necessary to manage the new situation (e.g., the hip control in both sitting and standing after an osteotomy). He had the freest movement when supported in the PBWB.

Single-System Integrities and Impairments

Brandon had many system impairments that varied across time. For each system, a general status will be given along with trends across time for that system. The constant changes in different systems’ status, the interaction between the body systems and posture and movement impairments, and the influence of all of this on his functional activity were challenging for the PT to analyze and to make the necessary shifts in his plan of care and intervention.

At age 2, Brandon had difficulty with temperature control and could not go outside on very hot days. He had increased redness across the extremities and would also suddenly flush. His heart rate and SaO₂ levels demonstrated sporadic changes not based on activity or observable environmental stresses, reflecting poor central regulation of basic physiological systems. He always required more sleep than his brother and always had difficulty with constipation. He was initially more irritable than his brother, and if something went wrong in the day, the entire day could be a bad day.

By age 9, Brandon could demonstrate a wider variety of states. He could be happy, quiet, fussy, or angry. Changes in status were often related to SaO₂ levels. He wore a monitor at all times that sounded when his O₂ levels were below 85%. His heart rate fluctuated. He took melatonin as needed to aid in sleep.

Respiratory System

Brandon demonstrated greater impairments in the respiratory system with chronic pneumonia. He first required supplemental O₂ at night for sleep apnea and eventually the use of O₂ throughout most of the day. The spinal stabilization also immediately improved his SaO₂ levels. He eventually required a tracheostomy and required ventilation on several hospital stays. His baseline SaO₂ gradually dropped and was monitored constantly. After the tracheostomy, the shifts in SaO₂ were not significantly altered by changes in postures, allowing him to be positioned safely in prone, supine, sitting, or standing. He had ongoing difficulties with congestion and upper airway infections. He developed severe sinusitis. He frequently stopped breathing altogether during seizures, yet the physicians had difficulty with balancing sufficient seizure medication to control his seizures and maintain adequate O₂ levels because the medication also tended to decrease his respiratory drive.

His breathing pattern was primarily diaphragmatic without full expansion of the rib cage. Medications for the respiratory system included Singulair (Merck Sharp and Dohme Corp.), Nasonex (Merck Sharp & Dohme Corp.), Pulmicort (AstraZeneca), albuterol, Atrovent (Boehringer Ingelheim), and Clarinex (Merck Sharp & Dohme Corp.) daily. In addition, he took tobramycin and Septra (Pfizer, Inc.) to address the chronic respiratory infections.

Cardiovascular System

During the later stages of Brandon’s life, his heart rate was also monitored constantly by the oxygen monitors. His heart rate varied from the 40 bpm range when he was asleep to between 60 and 80 bpm when he was awake and alert, but it did occasionally jump to the mid to upper 90s. He did have one cardiac arrest during a long seizure when he was 8 years old.

Neuromuscular System

Brandon could initiate, sustain, and terminate motor unit activity but had difficulty with the control/gradation of muscle activation and the coordination or timing and sequencing of muscle activation for functional activities. He initially had the greatest difficulty in terminating motor unit activity and would depend on a caregiver or changes in the environment to inhibit muscle activation. Most often, the muscle activation was isometric or concentric in nature with almost no eccentric control demonstrated. He also demonstrated a restricted number of synergies for function, with limitations in isolated control. He moved in full patterns of flexion and extension or if his head was positioned more asymmetrically—one side of his body in full flexion and the contralateral side in extension.

His overall dynamic stiffness varied from being low to very high. The changes were based on intrinsic changes in physiological status as well as from changes in the environment. He demonstrated very low levels of stiffness and was more flail when his oxygen levels were the lowest. He demonstrated clonus more so in the left LE than in the UEs. At times, however, he could demonstrate clonus throughout both LEs, both UEs, and even at the jaw.

Brandon had multiple medical interventions that addressed his neuromuscular system with eight sets of Botox (Allergan) or phenol injections to the hamstrings, adductors, and gastrocnemius, as well as to the pectorals, sternocleidomastoid, and latissimus dorsi. He had a baclofen pump trial but did not have a pump inserted, so he took oral baclofen daily. His seizure medications included phenobarbital, Trileptal (Novartis), Keppra (UCB), and Diastat (Valeant Pharmaceuticals North America) as needed for uncontrolled seizures. These medications also influenced his neuromuscular status.

Musculoskeletal System

Skeletal deformities include leg length discrepancy with the left ~1 in shorter than the right. The legs also demonstrated asymmetry in circumferences, with the left calf being a quarter inch bigger than the right and the left thigh being 2 in smaller than the right. As mentioned previously, he developed a scoliosis that was surgically stabilized. He had right hip dysplasia with surgical correction. He had severe forefoot varus on the left. He had severe osteopoenia with a fracture of his left distal femur with titanium rod implantation and later removal. He had marked limitations in range of motion (ROM) in both the UEs and the LEs and had significant weakness in all four limbs.

Integumentary System

His skin overall was fine and fragile. This fragility was true particularly in his hands and feet and was perhaps related to impaired distal circulation. His compromised skin integrity made it more difficult to wear orthoses successfully and required ongoing modifications of fit and careful monitoring within the day and across time.

Gastrointestinal System

Brandon had severe GERD with subsequent G-tube placement and a Nissan procedure within the first months of life. Even after the Nissan procedure, vomiting of meals was common. Brandon had difficulties with drooling and with constipation that were related to intrinsic impairments as well as being associated with his medications for other body system issues. His medications for his GI system included Reglan (Baxter Pharmaceutical), Robinul (Caspar Pharmaceuticals), MiraLAX (Bayer), Prevacid (Takeda Pharmaceuticals USA, Inc.), and vitamin B complex drops.

Sensory Systems

Although Brandon was reported to have cortical visual impairments, he did see many things in his environment. His ability to focus or track items changed based on his health status. Brandon had good hearing. He loved vestibular input, and his swing was his favorite way to get that input. Brandon loved tactile input, including deep pressure, particularly with cuddling, and he enjoyed moving tactile input.

Contextual Factors—the Family, Health Care System, Schools, Equipment, and Brandon’s Health as an Individual

The family preferred country living and being outdoors. Brandon had excellent support of his family. When Brandon was 3 years old, the family moved to another state for better employment for the father, but the family found that they could not find adequate therapy services for Brandon and Blake, so they returned to the previous state to obtain those services.

It was stressful for the family to have two boys with very different special needs. Blake was ambulatory by age 2 but had a diplegia with a marked asymmetry. He had sensory impairments with difficulty attending to tasks and had been identified as being on the autism spectrum. The younger brother also had some health concerns with a cardiac defect that resolved spontaneously.

The analysis of the data gathered from annual examinations varied. As Brandon grew, the desired participation and activity outcomes changed. Brandon’s parents were critical in selecting outcomes around which intervention focused. Across the years during his annual evaluations, the parents consistently stated that they wanted one focus to be on sitting for a variety of functions as well as wanting him to stand and take assisted steps. There were, however, also time frames in which the family said that they had no idea as to what was possible due to the multitude of medical complications. The therapist educated the family as to what might be important skills to develop for the future and what were potential future complications that could occur based on Brandon’s overall status.

As Brandon’s health changed, the relative importance of each impairment changed. Initially, the family focused on outcomes that were more developmentally age appropriate. He learned to roll and to inch forward on the floor in a commando-style crawl.

The following is a summary of outcomes from his annual evaluations and the associated intervention objectives related to the highest-priority impairments.

• By age 2 to 3 years, the family wanted him to be able to sit and hoped for some form of ambulation, even if it was with assistance.

Therefore, the outcomes for the years when Brandon was 2 and 3 years old were that he would be able to do the following:

Sit on the floor in either long or tailor sitting consistently for 20 seconds for play at home.

Bench sit with elbows propping on a tabletop surface for 45 seconds at a time for educational activities and to watch videos or his brother at play.

Transfer from his wheelchair to other chairs with assistance of an adult to UEs and trunk but not LEs while wearing AFOs.

• By age 4, the outcomes were expanded to include the following abilities:

Sit for longer episodes (up to a minute at a time).

Transfer with assist of an adult to only the pelvis.

Stand with support of a surface while engaging in UE play.

Take 50 steps with assistance of an anterior support walker with support of the therapist at the pelvis.

• By age 5, the outcomes were that Brandon would be able to do the following:

Sit without support for 3-plus minutes at a time to watch a video or listen to a song.

Walk 10 feet in a PBWB system, advancing the limbs independently but with the adult controlling the speed and directionality of the movement of the device.

Transfer between chairs of uneven heights.

Roll independently to cross a room for play.

• For the first time, when Brandon was 8 years old, the functional outcomes began to include qualifiers concerning SaO₂. However, they kept a focus on skills in transferring, sitting, rolling, and assisted walking. They included Brandon being able to do the following:

Transfer through stand pivot with assist of adult.

Take five assisted steps in suspension-style walker.

Reposition himself in prone, side lying, or supine to improve respiratory pattern, SaO₂, or comfort level.

Participate at home or at school in at least six different positions.

• By age 9, the outcomes included being able to do the following:

Sit on a bench with support of a small table to his arms for 10 minutes with supervision for play with a family member.

Stand in stander for 30-minute episodes to complete a single educational activity.

Take 10 assisted steps in a PBWB device with SaO₂ remaining above 90%.

In order for Brandon to reach these outcomes, impairment related objectives were identified during each evaluation. Initially (age 2–3), the highest-priority objectives included that Brandon would need increased ROM in the LEs, increased dissociation between the trunk/pelvis/LEs, and increased dissociation between and within the LEs. It was also noted that Brandon would need more active rotation in the trunk and neck and would need increased strength and control in the LEs to meet the established outcomes.

When Brandon was 4 years old, the list of objectives expanded to include more consistent recruitment of the postural muscles of the trunk and neck with the continued emphasis on increasing isolated control in the trunk and extremities and on increasing soft tissue length and strength of the muscles.

By age 5 years, the impairment-related objectives included increasing postural holding through the head and trunk, increasing isolated control between trunk and extremities, increasing ability to perform lateral weight shifts through the lower body, increasing strength through all extremities and the trunk, increasing soft tissue mobility, and increasing symmetry.

At age 8 and 9 years, the plan of care also included mention of increased chest expansion during respiration, an increase in body symmetry, and increased respiratory support for gross motor function.

It is possible to track the therapist’s problem-solving process relating impairments to activity limitations and subsequently to the individual’s participation restrictions. The evaluation of Brandon not only outlines the relationships between the different domains but also lays the foundation of the individualized intervention plan.

Abbreviations: ADLs, activities of daily living; SaO₂, oxygen saturation.

Summary of Impairments Preventing Activity or Participation Related to Supported Sitting

Multisystem Impairments

• Decreased postural control of head, neck and trunk.

• asymmetry LEs > UEs; atypical alignment of the hips.

• Poor sitting balance.

• Slow and limited limb movement—predominantly to midrange movement.