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Potential Less Invasive Scoliosis Treatment for Prader-Willi Syndrome Patients Receiving

Shereen
Richard
Growth
Hormone Therapy
Our Lady of the Lake College, Baton Rouge, Louisiana

Abstract

Hypothesis

Method

Prader-Willi Syndrome is a rare genetic


disorder that has the potential to cause many
medical issues. Much research has been
conducted on the effects of growth hormone
therapy on Prader-Willi Syndrome patients.
Growth hormone therapy is shown to reduce
obesity, increase muscle mass, increase height,
and improve overall body composition of these
patients. Growth hormone therapy has also been
correlated with an increase in the speed of
scoliosis progression, which is prominent in those
with Prader-Willi Syndrome (Bridges, 2014).
Invasive measures are usually taken to correct
the spinal curvature in these patients, but more
non-invasive measures, such as yoga or physical
therapy, could be more beneficial (Hagit, 2013;
Fishmen, Groessi, & Sherman, 2014).
I propose physical therapy may help PraderWilli Syndrome patients avoid scoliosis
progression while receiving growth hormone
therapy. This experiment will be conducted on
two groups of individuals who have Prader-Willi
Syndrome between the ages of two and
eighteen. Cobb angles will be measured by X-ray
before and after physical therapy intervention to
determine if physical therapy has an effect on
scoliosis
progression
in Prader-Willi
Syndrome
Prader-Willi
Syndrome
is a rare genetic
patients
hormone
therapy.
disorder receiving
caused bygrowth
a mutation
in the
paternal

Prader-Willi Syndrome patients who


participate in physical therapy while receiving
growth hormone therapy treatment will have less
scoliosis progression compared to Prader-Willi
Syndrome patients who participate in no physical
therapy while receiving growth hormone therapy
treatment.

In order to determine the effects of physical


therapy on the progression of scoliosis in PraderWilli Syndrome patients receiving growth
hormone therapy treatment, I will perform an
experiment involving 30 Prader-Willi Syndrome
subjects between the ages of 2 and 18 who
posses a Cobb angle greater than 10 degrees
and have never received growth hormone
therapy treatment during their lifetime. The
subjects who possess these requirements will be
chosen at random.
These 30 subjects will be divided into two
groups, each group containing 15 individuals:
Group A and Group B. The members of each
group will also be chosen at random. Both
groups will receive the same amount of growth
hormone therapy treatment at the same time
and will continue to be controlled throughout the
experiment. Group A will participate in physical
therapy 1 hour, twice a week for 6 months while
receiving growth hormone therapy treatment.
Group B will receive no physical therapy
treatment while receiving growth hormone
therapy treatment for 6 months. Group B will be
considered the control group. All subjects will be
X-rayed before the administering of the growth
hormone therapy treatment and after the 6
month period of physical therapy/no physical
therapy.
After X-rays are obtained from both groups
after the 6 months of intervention, Cobb angles
will be measured and compared to determine
how scoliosis progression was affected in each
group.
After the experiment is completed and the
data is collected from the two groups, a t-test
will be performed to determine the statistical
significance between the 2 groups. These results
will show if the results from the experiment are
biologically significant.
While receiving growth hormone therapy
treatment, glucose levels and sleeping patterns
will be monitored throughout the experiment to
avoid any participant harm.
If any participant feels the experiment puts
According
to the
research
his or
her health
or well
being found
at riskinorthe
simply
literature
I predict
followingfor
results
decides
toreview,
discontinue
the the
experiment
any
will occur:
reason,
he or she is free to withdraw from the
Group A at
willany
have
a significant Cobb angle
experiment
time.
meanAll
decrease
due to
the
physical will
therapy
participants
and
guardians
be
participation.
The
Cobbconsent
angle will
required
to sign
anmean
informed
to decrease
by approximately
50%. and will be well notified
participate
in the study
B will have
a slight Cobb
angle mean
thatGroup
all participant
information
remains
increase due to the lack of physical therapy
confidential.
participation. The mean Cobb angle will increase
by approximately 10%.

Introduction

copy of chromosome 15. Some of the major


medical issues that occur with this disease are
hyperphagia, childhood obesity, diabetes, late
development of psychomotor skills, short
stature, and severe spinal deformities (Greggi et
al., 2010). Recent studies have shown that
growth hormone therapy improves overall body
composition in these patients, but the rapid
increase in body mass from the therapy may be
correlated with accelerated scoliosis progression.
Many times, the increase in spinal curvature
must be corrected by invasive surgical means,
which can lead to postoperative complications.
The goal of this experiment is to find a less
invasive way to prevent scoliosis progression in
Prader-Willi Syndrome patients while receiving
growth hormone therapy. The question that is
being answered by my research is While being
treated with growth hormone therapy, what is
the effect of physical therapy participation on
scoliosis progression in Prader-Willi Syndrome
patients?

(Blanco, 2010)

Literature Review

Much research has been conducted involving


the effects of growth hormone therapy on PraderWilli Syndrome patients. Some of these studies
show positive effects on patients, while others
show negative effects.
Growth hormone therapy treatment is shown to
improve height, muscle bulk, exercise capacity,
and overall body composition in Prader-Willi
Syndrome patients, but overall, this is not
enough to totally eliminate obesity problems.
(Sipila et al., 2010; Bridges, 2014). Growth
hormone therapy is also correlated with
respiratory issues and glucose levels. Although
growth hormone therapy has not been proven to
be the cause of scoliosis, the rapid increase in
body mass may be correlated with a quicker
scoliosis progression (Bridges, 2014).
Research has been conducted on the effects
of growth hormone therapy and surgery on
Prader-Willi Syndrome patients with scoliosis.
Various surgeries and treatments are available to
treat scoliosis in these patients, but postoperative complication rates are high (Greggi et
al., 2010). Growth hormone therapy has also
been shown to decrease these surgical risks
(Nakamura, Nagai, Iida, Ozeki, & Nohara, 2012).
With the benefits that come with the growth
hormone therapy treatment in Prader-Willi
Syndrome patients, there are also
disadvantages. One of the major problems that
occurs in Prader-Willi Syndrome patients is
scoliosis. As stated above, the growth hormone
therapy treatment may not be directly related to
scoliosis progression, but it is shown to advance
the time of scoliosis progression (Bridges, 2014).
Scoliosis progression may also be influenced by
paravertebral muscle volume and age (Marukami
et al., 2012).
Studies have been performed regarding less
invasive ways to improve spinal curvature of
those with scoliosis. It is shown that using
physical therapy exercises, such as the Schroth
Method, improves the overall Cobb angle of
scoliosis patients (Hagit, 2013). Another method
used to reduce spinal curvature in patients with
scoliosis is yoga. Yoga poses, such as the sideplank, have been shown to significantly improve
Cobb angles in scoliosis patients as well
(Fishmen et al., 2014).

Predicted Results

Overall, I believe this will be a successful


and overall less invasive way to treat
scoliosis in Prader-Willi Syndrome patients
receiving growth hormone therapy
Group B
treatment.Group A
80

70

70

60

60
50
Cobb Angle in Degrees

50

40

Before 6 Months of
Physical Therapy
Intervention

30

After 6 Months of Physical


Therapy Intervention

40
Cobb Angles in Degrees

30
20

20
10

10

Participant Number

Participant Number

Before 6 Months of No
Physical Therapy
Intervention
After 6 Months of No
Physical Therapy
Intervention

Prader-Willi Syndrome is an extremely


uncommon genetic disorder. A limitation that
can arise with this research is the population
and sample size. Because there are so few
individuals affected by PWS, and even fewer
who are in the age range required for the
study, the sample size may be extremely
small resulting in little statistical power.
This research experiment was carried out to
discover the effects of physical therapy
participation on Prader- Willi Syndrome
patients receiving growth hormone therapy
treatment. Further studies should be
performed to evaluate which physical
therapy exercises are the most influential on
the patients scoliosis progression.
The overall goal of this experiment is to find
a less invasive way to treat scoliosis
influenced by growth hormone therapy in
Prader-Willi Syndrome patients. Further
research should be performed to find other
less invasive ways to treat scoliosis in these
patients such as yoga or Pilates.
This experiment was also limited to
adolescence. Further research should be
conducted
on an older age group.
Blanco. J. S., (2010). Early onset scoliosis: An overview. Retrieved from

References

http://www.hss.edu/conditions_early-onset-scoliosis-overview.asp
Bridges, N. (2014). What is the value of growth hormone therapy in Prader-Willi
Syndrome?. Archives Of Disease In Childhood, 99(2), 166-170.
doi:10.1136/archdischild-2013-303760

Your text would go here. List your information on


Fishman, L.M., Groessi, E.J., & Sherman, K.J. (2014). Serial case reporting yoga for
these
lines. Your
text Global
would
go here.Health
Listand
your
idiopathic
and degenerative
scoliosis.
Advancements
Medicine,
3(5). Retrieved from http://www.gahmj.com
information on these lines.

Greggi, T., Martikos, K., Lolli, F., Bakaloudis, G., Di Silvestre, M., Cioni, A., & ...
Giacomini, S. (2010). Treatment of scoliosis in patients affected with Prader-Willi
syndrome using various techniques. Scoliosis (17487161), 511-18.
doi:10.1186/1748-7161-5-11

Hagit, B. (2013). The effectiveness of the Schroth method of physical therapy for
treating and adult with adolescent idiopathic scoliosis (AIS) in a outpatient clinic
in the United States with third-party payer constraints: a case report. Scoliosis
(17487161), 8(Suppl 2), O10-O11. doi:10.1186/1748-7161-8-S2-O10

Murakami, N., Obata, K., Abe, Y., Oto, Y., Kido, Y., Itabashi, H., & ... Nagai, T.
(2012). Scoliosis in Prader-Willi syndrome: effect of growth hormone therapy and
value of paravertebral muscle volume by CT in predicting scoliosis progression.
American Journal Of Medical Genetics. Part A, 158A(7), 1628-1632.
doi:10.1002/ajmg.a.35429

Nakamura, Y., Nagai, T., Iida, T., Ozeki, S., & Nohara, Y. (2012). Growth hormone
supplement treatment reduces the surgical risk for Prader-Willi Syndrome
patients. European Spine Journal: Official Publication Of The European Spine
Society, The European Spinal Deformity Society, And The European Section Of
The Cervical Spine Research Society, 21 Suppl 4S483-S491. doi:10.1007/s00586011-2110-1

Sipil, I., Sintonen, H., Hietanen, H., Apajasalo, M., Alanne, S., Viita, A., &
Leinonen, E. (2010). Long-term effects of growth hormone therapy on patients
with Prader-Willi syndrome. Acta Paediatrica (Oslo, Norway: 1992), 99(11), 17121718. doi:10.1111/j.1651-2227.2010.01904.x

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