Dempsey is seen by his school district’s Early Intervention team, with visits from the Physical Therapist twice per month as primary service provider with consultation from Early Childhood Special Education teacher and Service Coordinator.
Clients Name – Dempsey
Age – 10 months
Diagnosis – achondroplasia, cerebral palsy
Brief history – Dempsey’s mother experienced premature preterm rupture of membranes (PPROM) at 31 weeks, 3 days gestation and was hospitalized. Dempsey was delivered at 34 weeks, 1 day gestation with birth weight of 2400 grams. His Apgar scores were 3 at 1 minute and 8 at 5 minutes. He required positive pressure ventilation with oxygen and had ongoing care in the NICU for respiratory distress syndrome. MRI indicated severe hypoxic ischemic injury, multifocal cerebral, cerebellar, and intraventricular areas of microhemorrhage.
Dempsey has increased muscle tone through all four limbs. His hips remain abducted and externally rotated most of the time. Placing him in hip helpers not only moves him toward more neutral alignment, but makes him more alert and better able to organize and coordinate his movement. He sits with moderate support and rolls from supine to sidelying. His visual tracking has been inconsistent.
Why do they need to stand?
According to Wheeless’ Textbook of Orthopaedics, children with achondroplasia are typically delayed in reaching motor milestones, and are likely to be unable to walk independently until 2-3 years of age. It is important for bone and joint development to have Dempsey begin weight bearing around the same age as his peers. Dempsey is at increased risk for joint contractures and malalignment not just because of his diagnosis of achondroplasia, but also because of the spasticity he demonstrates due to his diagnosis of cerebral palsy.
We are primarily trying to achieve musculoskeletal development in good alignment to maximize symmetry and efficiency of movement as Dempsey develops motor skills, and to decrease the risk of fracture or the impact of degenerative joint disease as Dempsey ages.
Dempsey responds well to being placed in alignment with pressure through his joints, making standing an excellent position for keeping him alert for cognitive and social experiences.
Dempsey has demonstrated inconsistent visual responses, so providing standing with a tray places him in an excellent position for working on hand/eye coordination while minimizing other visual distractions.
By November 2015, Dempsey will pull himself to standing in his crib or on furniture in his home or daycare environments as observed by caregivers on 5 consecutive days.
Dempsey needs to develop a motor plan for standing and have appropriate alignment to be stable in this position.
By July 2015, Dempsey searches for, finds, grasps, and plays with a toy dropped or placed within his reach as observed by caregivers on 5 consecutive days.
Standing with hands in the visual field and a supportive play surface facilitates the awareness and motor skill needed for this task.
By September 2015, Dempsey transfers a small toy from one hand to the other during play with a caregiver at home or daycare as observed by caregivers on 5 consecutive days.
Dempsey is more likely to be successful with a task like this when his trunk is supported, as when using a standing frame, than when attempting to maintain sitting balance while performing a fine motor task.
What type of stander did you use, what options did they use? Why was this stander chosen over other types?
A supine stander is used because it is most readily available and can be transported to the family home for demonstration purposes most easily.
The Physical Therapist would prefer and will pursue a trial of an EasyStand Zing MPS, as it would allow for prone positioning and hip abduction to facilitate optimum development of the acetabulum and femoral head, as these are likely to be affected by both diagnoses of achondroplasia and cerebral palsy.
How often do they stand? How long do they stand? What is the standing protocol
The standing protocol is 60 minutes per day at least 6 days per week. The standing minutes need not be consecutive, as the family finds it more convenient for Dempsey to stand 30 minutes twice per day within their routines.
Where do they stand – rehab/school/home?
All standing is done at home.
ADLs or other Activities that they do while standing
Dempsey stands while his mother is working on meal preparation so that she can talk to him and he can work on visually and auditorily responding to her movements. He occasionally stands during tube feedings to minimize the reflux and vomiting he frequently experiences.
How were the (long or short-term) goals of standing met (medical benefits, physiological/emotional benefits, ease in daily living, etc.)
Dempsey is able to bear weight and receive proprioceptive input, making him much more alert. There is concern in children with achondroplasia regarding the development of either lordosis due to hip flexion contractures, or kyphosis due to chronic anterior compression of the vertebrae. Due to these spinal concerns, “This knowledge has led to the recommendation that sitting be restricted until the infant with achondroplasia is able to independently achieve this transition. Furthermore, parents of infants with achondroplasia should avoid pieces of equipment that reinforce this characteristic kyphosis such as baby slings or umbrella strollers.” (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4104450/) Dempsey is able to remain in a well-tolerated, neutral, supported position to experience the world in an upright, anti-gravity manner.
Seek information regarding anticipated developmental timelines for the diagnoses of the children you work with and learn about risk factors for musculoskeletal development. When musculoskeletal deformity, osteopenia/osteoporosis, hip dysplasia, abnormal muscle tone, and/or hypermobility are present or likely to occur, early standing in good alignment is an essential early intervention.
Stephenie Labandz, PT, received her MPT degree in 2002 and her DPT degree in 2009, both from the College of St. Catherine in St. Paul, MN. Her primary professional interests are neurological rehabilitation and assistive technology. She currently serves the children and families of Robbinsdale Area Schools through their Early Intervention and Early Childhood Special Education programs.