ATP Series: Case Studies

Making the Case for Standing

Stronger & Stronger

Child with Thrombocytopenia-absent radius syndromeThrombocytopenia-absent radius syndrome (TAR), of which there are two types, short arm and long arm, is an extremely rare condition impacting fewer than one in 100,000 newborns. Affected individuals have a deficiency of blood cells involved in clotting (platelets) and can require hundreds of blood transfusions every year. Children who survive infancy and do not have damaging hemorrhages in the brain usually have a normal life expectancy and normal intellectual development.

Mutations in the RBM8A gene cause TAR syndrome. The RBM8A gene provides instructions for making a protein called RNA-binding motif protein 8A. This protein is believed to be involved in several important cellular functions related to production of other proteins.

Some children with TAR also have malformations of the heart and kidneys — Kade, who came to the attention of Wendy Altizer, PT, ATP, doesn’t have those difficulties. But about half of children with TAR have allergic reactions to cow’s milk that may worsen the thrombocytopenia — and Kade does have that. He also needs the very frequent blood transfusions associated with TAR.

Child with Thrombocytopenia-absent radius syndrome in a standing deviceKade was hospitalized for one week after birth and sent home after three transfusions. He has had multiple Broviac ports placed in his left pectoral region to ease transfusions. He was referred into an education-based earlyintervention system in April 2015, and his outpatient referral for medical-based therapy began in May 2015.

At Kade’s initial assessment Wendy noted minimal hand movement, externally rotated lower extremities, and knee flexion contractures of 120° bilaterally, although Kade had the ability to achieve a neutral heel/foot position. Kade was very alert and visually attentive. He presented with right head tilt and left rotation, but no range limits. He was able to kick his legs using hip flexion movement.

Wendy uses lots of gait trainers, and Grillo and KidWalk are her favorites. She prefers the prone position of the Grillo and arm supports for her older children and those who need to lean forward. For more upright gait, she uses the KidWalk and appreciates the dynamic movement in all their planes of motion.

Recently, Wendy saw children with inseams of 6" and less. The KidWalk was just too high for them. She contacted Prime Engineering, and they told her about their extra-small earlyintervention model. Now she has all the features she loves for her itty-bitty clients!

Child with Thrombocytopenia-absent radius syndrome in a wheelchairKade’s therapy team at Milestones in Hurricane, W.V., used Hip Helpers to de-rotate his hips and start work on rolling, which he accomplished independently at 7 months. The team addressed range of motion, trunk strength and vestibular function, and used lots of strategies and equipment to encourage play and exploration, including a Bumbo chair, Jenx Giraffe chair, aquatics to initiate movement, Oball toys, switch toys, a self feeder, a SuperStand HLT EI and a KidWalk EI. Wendy choose the KidWalk Ei because of its ability to go very low, and she modified the soft trunk support to allow Kade’s hands to be free.

Kade loves walking, and it’s making him stronger and improving his endurance!

boy with spastic tetraplegic cerebral palsy in standing deviceOn His Feet Again

Now you can use a stander for a person with knee flexion contractures, while preserving hip range of motion. The new knee contracture brackets from Prime Engineering can be added to any SuperStand Stander. Traditional sit-to-stand devices link the amount of hip and knee flexion so that you cannot bend one joint without bending the other. This new system allows for full adjustments on all planes and can accommodate severe asymmetries.

My case comes from Los Angeles and involves Vicente Capati, PT, California Children’s Services, and Karen Hobson, PT, Richard’s Rehab Solutions. They were challenged to accommodate a 17-year-old boy with spastic tetraplegic cerebral palsy, level V on the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS V), Communication Function Classification System (CFCS V), and Eating and Drinking Ability Classification System (EDACS IV).

profile of boy with spastic tetraplegic cerebral palsy in his standerHe presented with bilateral knee flexion contractures (35° right, 30° left) and hip flexion contractures of 20° right and 30° left. When he was in kindergarten, he enjoyed being fed and watching TV while standing, and the family wanted to try to return to this.

The team quickly realized that a traditional standing frame would not accommodate his deformities. The knee flexion contracture brackets from Prime Engineering were large and padded, adjustable at both ends (tibia/femur) and allowed this teenager to stand. He was so happy and his family is able to use the stander regularly at home.

This article originally appeared in the August 2016 issue of Mobility Management.

In Support of Upper-Extremity Positioning