Prader-Willi behaviours include self-injurious behaviour, such as

Prader-Willi Syndrome (PWS) is a rare neurodevelopmental disorder that
affects 1 in 22,000 people, and it currently has no cure. It is characterised
by an increased appetite (hyperphagia), weak muscles, delayed growth and sexual
development, and learning difficulties. Children with PWS exhibit symptoms
similar to those seen in obsessive-compulsive disorder, autism spectrum
disorder, and attention-deficit hyperactivity disorder (Dimitropoulos &
Schultz, 2007, Wigren & Hansen, 2005). They are also more likely to suffer from
psychological disorders compared to regular developing children (Skokauskas et
al., 2012). In this proposal we will specifically be looking at anxiety and its
symptoms in individuals with PWS.

            Studies
have shown that 6%–28% of individuals with PWS displayed psychotic symptoms (including
anxiety) starting from 13 to 26 years old, and they have a prevalence rate
higher than individuals with other forms of intellectual disabilities (Verhoeven et al., 2003,
Beardsmore et al., 1998). Maladaptive
behaviours are the result of the attempt to reduce anxiety; these behaviours
include self-injurious behaviour, such as skin picking (“How Does a Person With
PWS Think”, 2014).

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A study has found in their sample of 62 individuals with PWS that self-injury
was reported for 81% of individuals (Symons, 1999). Furthermore, research has
found that the frequency of skin picking and other maladaptive behaviours
increased with age, which may due to new stressors such as leaving school and
starting work (Dykens et al., 1992, Dykens ,2004). Although many studies have
established the effectiveness of growth hormone treatment for physical
characteristics of PWS such as short stature and poor physical health (Whitman
et al., 2002, Coupaye et al, 2013), little research has gone into treatment for
anxiety and its self-injurious symptoms. An article on the clinical management
of behavioural characteristics in PWS states, “behavioral treatment of self-injurious behavior among
individuals with PWS has not been well documented” (Ho & Dimitropoulos,
2010). This could be due to the fact that other symptoms of PWS are seen as
more life threatening, such as hyperphagia (as it leads to obesity and diabetes
in many PWS individuals), and therefore need to be treated more urgently (Ho
& Dimitropoulos, 2010).

            The aim of this study
is to examine whether cognitive behavioural therapy (CBT) is an effective
treatment to decrease anxiety in individuals with PWS. CBT is the chosen form
of therapy because it has shown to be a popular and effective way in reducing
anxiety (Hopkinson et al., 2017, Mayo-Wilson & Montgomery, 2013,
Wuthrich & Rapee, 2013). It is effective in treating childhood anxiety disorders as well
(James et al., 2015), which is important to take into consideration because
individuals with PWS have lower mental abilities and IQs, much like regularly
developing children. It is also recommended by many health agencies including
the NHS. A study into this topic is necessary in order to strengthen the
argument that CBT is an effective therapy for many types of anxiety, and not
just anxiety in regularly developed people. Furthermore, the effective
application of CBT for individuals with PWS may decrease the presence of
harmful maladaptive behaviours, most notably skin picking and self-injurious
behaviour. Our hypothesis is that based on previous research on the
effectiveness of CBT for general and child anxiety, CBT will be an effective
method to decrease anxiety and subsequently maladaptive behaviours in
individuals with PWS.

Method

            A sample of 100
individuals with a diagnosis of PWS will be recruited through opportunity
sampling, and individuals with PWS (or their carers on their behalf) will be
willing to take part. These individuals will need to meet the DSM-IV criteria
for anxiety, as well as exhibit self-injurious behaviour.

            PWS individuals and
their primary carers (if they have one, depending on their ability level) will
be invited to the university to take part in the study. Individuals will have
to bring medical proof that they currently exhibit self-injurious behaviour,
such as a note from a doctor. They will first have to sign consent forms
ensuring the confidentiality of their data and letting them know their rights
as a participants, such as the right to withdraw at any point from the study
without reason. After obtaining informed consent, participants will then complete
the Anxiety Disorders Interview Schedule for DSM-IV to determine whether they
have anxiety. On a severity rating scale of 0-8, a rating of 4 and above would
classify them as meeting the diagnosis criteria. Primary carers are able to complete
the interview on behalf of the PWS individual, as they would have the sufficient
knowledge from a daily basis of contact. The interview will be conducted by
three trained psychology students trained to ensure an accurate diagnosis.

Participants who meet the threshold for anxiety (meaning they obtained a score
of 4 or more) will then complete a Generalised Anxiety Disorder Assessment
(GAD-7) questionnaire (Spitzer et al, 2006). Then, CBT will be administered to
each individual, lead by three psychology graduates also trained in CBT. 10
2-hour sessions over the course of 10 weeks (1 session each week) will be
administered. During the sessions, generic CBT skills including
problem-solving, goal-setting, identifying negative thoughts, cognitive
restructuring, and graded exposure will be taught. After the 10-week trial,
participants will be asked to fill out another GAD-7 questionnaire.

            A one-sample Wilcoxon
signed rank test will be carried out in SPSS to determine whether anxiety
levels decreased after CBT. This test will be carried out because there is one
independent variable (the administration of CBT) and one dependent variable
(level of anxiety). The dependent variable is also interval by nature; therefore
we do not need to assume it is normally distributed.

            Ethical concerns in
this study mainly concern the use of participants with an intellectual
disability. There is the question of whether they are mentally capable to
participate, and whether they would be able to understand what they are
consenting to. Our study addresses this concern by allowing the carers of
individuals who have more severe symptoms to participate. There is also the
issue of using participants that we know are currently exhibiting
self-injurious behaviours. To address this concern, we require medical proof
that participants are self-injuring themselves, which lets us know that a
medical doctor is already aware of the situation.

Expected Results

         The proposed research study would have
considerable effects on the lives of individuals with PWS and their families. If
our hypothesis is found to be significant, CBT would have support to be
efficient in decreasing anxiety levels in PWS individuals, which in turn may
lower the frequency of maladaptive behaviours, in particular self-injurious
behaviours. A decrease in anxiety may also allow PWS individuals to focus on
other areas of improvement, such as in their emotional regulation and learning
abilities. Since anxiety is the most common factor that leads to challenging
behaviours, a decrease in their anxiety through the use of CBT would prove to
be helpful in their everyday lives.