ATP Series: Case Studies
Making the Case for Standing
- By Ginny Paleg
- Aug 01, 2016
Stronger & Stronger
Thrombocytopenia-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.
Kade 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
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
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
Kade’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!
On 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).
He 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
This article originally appeared in the August 2016 issue of Mobility Management.