Irene's Case Study: Dementia, Down Syndrome and PBS Intervention

This dissertation examines the case of Irene, a 53-year-old woman with learning disabilities and Down syndrome, presenting symptoms of early-stage dementia. The study utilizes the Health Equality Framework (HEF) to analyze Irene's complexities and explores the application of a Positive Behavior Support (PBS) intervention to address her depression. The dissertation delves into the increasing prevalence of age-related depression, particularly among individuals with learning disabilities, and highlights the heightened risk of Alzheimer's disease within the Down syndrome population. It covers key terminologies, the use of national assessment frameworks, and evidence supporting PBS as a preferred intervention. The research further investigates the roles of RNLDs in implementing and observing PBS, and evaluates progress, challenges, and recommendations for future improvements. The study aims to provide insights into managing behavioral problems, improving patient and caregiver satisfaction, and preventing the need for institutionalization, ultimately aiming to enhance the quality of life for individuals like Irene.

Dissertation

Abstract

Introduction
The dissertation used the Health Equality Framework (HEF) to control Irene's study and
complexity analysis (see Appendices 1 and 2 for a full contextual and HEF study). The facility
allowed various experiments to think and motivate to avoid some opportunities that could clarify
the signs and side effects introduced by Irene. Irene was recognized as suffering from
manifestations of early stage dementia and PBS was chosen as an intervention to treat Irene's
depression, as there was strong evidence of vitality for people with learning difficulties. The
presentation theme continues to be important and crucial as age-related depression is increasing
in the United Kingdom (UK) and people with a low condition are currently living longer on
baseline a broad base of welfare and social consideration frameworks compared to the smallest
created countries on the planet. This makes these people dependent and helpless socially,
mentally and realistically, including positive social problems and welfare structures. It has been
reported according to the National Down Syndrome Association that 30% of people with a down
disorder who are 50 years of age have Alzheimer's depression and half of them are 60 years old,
these numbers are much higher faster than anyone other. Since depression is hopeless, its later
stages can be strong and difficult to cope with, which responds to the need to explore this topic
throughout the paper.
The main section quickly identifies and analyzes the main points related to the contextual study
and analyzes and analyzes the key words. Section one will continue with gaining knowledge of
HEF and applying to HEF the contextual analysis that would reveal the zone of need Irene
considers mediation, shaping an opening to part two to examine the selected intervention. The
next section looks at evidence that includes PBS (Preferred Intervention) using specific survey
information bases and clarifies how the writing was found and conducts a basic study of
appropriateness the writing by speaking practically and examining the idea and meaning of the
practice-based test. The third part examines the work and ideas of the RNLD in line with
reformed procedures, initiatives and officers and in the implementation and observation of the
PBS to Irene.
The final section explores the perception of progress and preparation for change and its
challenges, as well as building on ideas of change in which a model of change will be used to

drive and manage change and closes with addressing key concerns and making recommendations
for future improvements.

Chapter I. Exploring complexities
1.1. Introduction
Irene is a 53-year-old woman with learning disability and Down syndrome and lives with both
her parents. It was reported by her parents that Irene has been presenting symptoms similar to
what they think to be dementia, they express that Irene has been confused, muddled and change
in behavior like sad, fair and angry which is not normal for her. Irene’s parents are concerned as
they think Irene might need treatment in order to prevent the situation from worsening. The
RNLD was approached and Irene’s case was handed over. Chapter one will highlight and define
the key terminologies relating to Irene’s scenario to enable readers to understand and explore the
complexities compounded with learning disability-down syndrome and dementia making
reference to Irene (see appendix 1 for full case study) The section will proceed to introduce a
national recognized assessment framework used to identify the needs of people with learning
disabilities. Also a proposed intervention of either applying a drug or a non drug strategy will be
explored to respond to the needs of Irene in order to improve and enhance Irene’s quality of life.
1.2 Learning Disability – Down Syndrome
According to Kavale and Forness, Learning disability is a neurological problem. Essentially, the
inability to absorb is the result of a difference in the way an individual's brain is "chorded".
Children with learning barriers are just as rude or subtle as their peers. However, they can also
have problems with hearing, inventing, orthography, thinking, reviewing, or organizing data
when used to solve things without human help any other or when they are normally educated.
The learning disability cannot be alleviated or alleviated; it is a deeply rooted issue. With the
right help and intervention where possible, children with learning difficulties can take over
school and become success, often distinguished careers later in life. Guardians can help children
and learn the barriers to making such progress by strengthening their quality, experiencing their
shortcomings, understanding the learning framework and working with experts and educating
themselves on systems for dealing with specific problems.
Russell et al.(2017) elicits that a learning disability is often classified by IQ scores, as IQ tests
are supposed to test ones reasoning capacity measuring intellect, the degree of impairment and

its descriptors show the severity; a score of 50–70 is defined as a mild learning disability, 35–50
moderate, 20–35 severe, and < 20 as profound. Although used as a rough indicator to gain
insight into one’s cognitive ability it is not a favored way to define a learning disability alone
because it does not represent the functional abilities people with learning disabilities.
In regards to Irene, she has learning disability, Down syndrome and behavior issues. Antonarakis
et al. (2020) describes Down Syndrome as a long-term condition affected by chromosome
change and is associated with intellectual disability -where individuals experience cognitive
delays, a characteristically facial appearance that includes: a flattered appearance of the face,
small ears, short neck, weak or floppy muscle tone (hypotonia) and other birth defects like
cardiac defects, gastrointestinal abnormalities and delayed development in cognition and
behavior in infancy and throughout adulthood. These individuals are also at an increased risk of
developing several medical conditions as well as being prone to several ailments throughout their
life.
According to American Journal, 2020; Psychological symptoms and behavioral abnormalities are
common and unique features in depression. They include manifestations, such as depression,
anxiety, depression, restlessness, irritability, dehydration and exacerbation. Approximately 30-
90% of patients with dementia experience the negative effects of such social problems. There are
complex links between intellectual deficits, mental side effects, and behavioral disorders.
Routine, robust, and fully agreed-upon tools for detecting depressive behavior and psychiatric
symptoms have been developed to assess treatment potential. Neurodegenerative cycles occur in
different mental regions, particularly in the frontotemporal cortex and limbic locales, which
cause cholinergic, serotonergic and noradrenergic synapse disorders involving an organic pattern
of behavioral side effects, while variables Mind and character traits take on a changing task.
Countless pharmacological, psychoeducational, psychotherapeutic and social techniques have
been created to improve the personal satisfaction of patients and their parents.
According to Ravona-Springer, 2003; psychological decline and deficit in social well-being are
symptoms of progressive neurodegeneration, behavioral abnormalities are common and
important features of dementia. Alzheimer's disease (AD) is the leading cause of older
depression, so the accompanying study closely identifies this problem. It only affects about 15
million people around the world. A wide range of social disorders affect the anxiety of most

patients with dementia. Accelerated behavior, for example, oral or true urination, urinary
incontinence and exorbitant incontinence, are good remedies for immune problems and greatly
support the option of entrusting AD patients to prolonged systemic care. Social and mental skills
and resilience of the number of parents, just like the presence and well-being of social
organizers, largely determines whether a hysterical patient with social problems can survive. at
home or needing confirmation in a nursing home. However, previously, AD research relied
largely on early identification and elimination of psychological deficits, while social stress was
eliminated. Managing behavioral problems can improve patient and caregiver personal
satisfaction and can help maintain a strategic distance from unprecedented systematization.
1.3 Dementia
Duong et al. (2017) outlines that Dementia refers to a clinical syndrome characterized by
progressive cognitive decline that interferes with independent functioning ability and symptoms
are said to be gradual, persistent and progressive those affected are not aware of their deficits.
Individuals with dementia are said to experience changes in cognition, function and behavior
(Bender and Cheston, 1997). Duong et al. (2017) explains that the clinical presentation of
dementia differs among individuals but the cognitive deficit it presents are known to be short-
term memory loss, communication and language impairments, agnosia (inability to recognize
objects), apraxia.
Kumar et al. (2020) explains that there are different types of dementia: Lewy body, Vascular
dementia, Frontotemporal dementia and Alzheimer’s disease and it is possible to have more than
one type simultaneously. Weller and Budson (2018) suggest that the neurodegenerative disease
process is characterized classically by two hallmark pathologies: β-amyloid plaque deposition
and neurofibrillary tangles of hyperphosphorylated tau. Emmandy and Tadi (2020) explains that
the pathophysiological process of dementia is not fully understood, but most types of dementia,
except vascular dementia, are caused by the accumulation of native proteins in the brain.
Emmnady and Tadi (2020) added that certain genetic traits can also predispose an individual to
dementia. Cunningham et al. (2015) mentioned that the incidence of dementia rises with age and
this is not a normal part of the aging process nevertheless. According to Alzheimer’s research
UK, 2020; there are currently 850,000 people with dementia in the UK and this is projected to
rise to 1.6 million by 2040.

Within the Down Syndrome population, it is noted that they may develop symptoms of dementia
earlier than their counter parts. Also the risk of developing dementia is considerably higher
among people with Down Syndrome. According to Wiserman et al. (2018) people with Down
syndrome are born with an extra copy of chromosome 21 which is the carrier of The APP
(Amyloid Precursor Protein) gene. The gene creates some proteins called Amyloid Precursor
Protein (APP). The abnormal creation of APP leads to a build-up of protein clumps called beta
amyloid plaques in the brain. People with Down syndrome possess the plaques along with other
proteins deposits called tau tangles which are said to be the main cause of Alzheimer Disease.
The combination of these proteins is observed among people with Down syndrome by the age of
40 years, thus increasing the risk of early onset of dementia to this group. Understandably, Down
syndrome is said to be the biggest genetic risk factor and determinant of Alzheimer’s disease,
making individuals with Down syndrome become predisposed to dementia due to the similarities
in genetic construct. According to NDSS, 2020; 30% of people with Down syndrome who
(improper citation) are in their 50’s have Alzheimer’s dementia and 50% have it in their 60’s.
This evidence along with the above mentioned therefore, suggests that the down syndrome
population and the general population both suffer from cognitive decline as they age, however
the difference is seen within the Down syndrome population as they tend to present the
symptoms at an early age and can develop slowly and might worsen over time if not observed
early or managed as dementia is incurable.
Castro and et al, 2017; suggests that there are investigations being carried for potential therapies
that can target the amyloid in order to modify and treat Alzheimer’s disease among people with
Down syndrome. They reckoned that preventive treatment needs to start before the onset of
symptoms given that the neuropathology associated with Alzheimer’s disease starts early in
people with Down syndrome. However, to date, no such trials have been done in patients with
Down syndrome. Elliott-King, 2016; implies that it can be challenging to diagnose dementia
among people with learning disability. Diagnosing dementia among people with learning
disability is important as the real illness that affect the individual’s quality of life will be known
and the right course of treatment will be prescribed thus preventing diagnostic overshadowing
where the correct treatment could be missed.
In relation to complexity, Irene’s health is surrounded by learning disability, Down Syndrome
and a suspected dementia. As the terminologies have explained about the condition and illness, it

is important for health professional to recognize how each condition might affect Irene. There
are similarities in symptoms such short memories and other that could be misinterpreted or
misattributed to the learning disabilities thus resulting in unnecessary suffering which could be
alleviated by access to the right care and support. Therefore, a thorough assessment is needed
covering all aspects of health and social care as some of these areas are known to be missed and
not picked up earlier enough among people with learning disabilities. This would take form
using the Health Equality Framework (HEF).
1.4 The Health Equality Framework (HEF)
According to National development Team for Inclusion (2013) The Health Equality Framework
(HEF) is an assessment tool developed by the National Development Team for Inclusion (NTDI)
that helps in understanding the effectiveness of services provided to people with learning
disabilities as well as inform essential commissioning functions based on the determinants of
health inequalities affected by these individuals:
 Social determinants
 Genetic and biological determinants
 Communication difficulties and reduced health literacy
 Personal health behaviour and lifestyle risks
 Deficiencies in access to and quality of health provision
Each of these determinants has indicator statements and descriptors which define the scope of
interventions needed with their associated adverse consequences which is rated against a five
point impact scale.
National development Team for Inclusion (2013) claims that this framework is driven by the
health inequalities people with learning disabilities face and lack of adaptation to their needs
which is strengthened by many public health inquiries/reports to include the well known
Winterbourne report, a pertinent case review in learning disability detailing the shortcomings of
care and recommendations and implementation for future practice. There are a number of
national outcomes framework across public health, social care and the NHS all of which have
equality at the centre, the NHS outcomes framework has been a source of inspiration and
catalyse to the development of the HEF and HEF proactively focuses on prevention and

detection rather than reactive approaches generating the model to be used in nursing
interventions.
HEF is also a response to health inequalities as its tool focuses on the evidence base of the
known determinant of health inequality experienced by people with learning disability. Emerson
et al (2011) argued that HEF’s uniqueness relies on the way health professional view the urgency
for intervention that will lead them to plan the type and format of the intervention.
National development Team for Inclusion (2013) expresses that the framework can be used both
by service providers with the purpose of providing services that will enhance the quality of life
for people with learning disabilities and service users including people with learning disabilities,
for service providers there exists a more detailed through analysis manual and a learning
disability user friendly manual for those who wish to self -evaluate their situation
Atkinson et al (2014) states that HEF is based on a person-centred approach as it is fitted to
address the needs of the individual. The NDTI (2013) Articulates that HEF can be accessed on
the NDTI organization page online as an electronic template provided with a step by step
guidance to match the individual’s needs to the correct level. Moulster and Lorizzo (2014)
encourage the use of HEF as they discovered that presently there are no known national targeted
framework set to address the health inequalities people with learning disabilities face, therefore
underpinning the need to use HEF for Irene.
1.4.1 Advantages and disadvantages
Atkinson (2015) explains that the greatest advantage of using the HEF framework is that it can
be used as an evaluation tool to evaluate the efficacy and quality of services provided and that it
is person centered in approach, enabling the patient to be at the centre of their care with
everything collaborated at their convenience and in their best interest. Duff (2016) identifies that
this concept is permitted in healthcare and there is a growing push to continue to use the person
centered approach model in health and social care, a big cultural shift from a passive approach to
care, its prominence has been shown to improve patient satisfaction in turn improving health
outcomes and care quality. Atkinson (2015) outlines that the disadvantage to HEF is that it can
be problematic to classify individuals’ needs correctly within a multidisciplinary team of
professionals, due to variances in understanding relating to how best to categorize an individual,

making it a challenge to reach an agreed consensus however, to counteract this health
professionals are encouraged to work collaboratively to mitigate different understandings.
However, as per duty of care that requires health professionals to act urgently in responding to
the needs of patients under their care, HEF, as suggested by Emerson & Baines (2010) is the best
application to use as it determines the level of urgency need to intervene base on its way of
scoring.
1.4.2 Identifying and summarizing Irene’s complexities and needs through HEF
In analyzing Irene’s case, it is understood that Irene has health complexities and areas of need
which surround her life; to include learning disability and Down syndrome. Irene’s family
circumstance with both parents being elderly as explained in the case study make them unable to
continue to look after Irene due to the decline that comes with old age hence, these factors need
to be considered when addressing Irene’s needs. To effectively identify and address the needs of
people with learning disabilities, NDTI (2013) suggests that the completion of HEF can be done
by health professionals with the involvement of the individual’s carer who has known the
individual for a reasonable length of time or the service user considering they have the mental
capacity to understand.
On completion of HEF it is shown that Irene’s mental health was the most affected followed by
the physical health, hence scored high highlighting the need to be addressed and the need for a
clinical review. It was noted by the parents that Irene has become withdrawn and appears to like
isolating herself where she no longer shows interest in the things she used to enjoy and no longer
shows engagement with her parents. It was also reported that she has become quiet, forgetful and
a constant fear of falls due to re-concurrent falls resulting in a decline in her mobility and she
suffers from muscle and joint pain.
The last determinant of health to be affected by Irene is communication. It appeared that Irene is
unable to communicate her health needs for action to take place. This is one of the five health
equality indicators linked to communication. This point out the need to address possibilities as to
why Irene’s communication is affected. It is well established that in this population
communicating health problems is known to be either limited or impeded but looking into other
areas that may affect communication like patterns of changed behavior can be signs to address.

Irene was supported to go through her annual health check in order to see whether Irene is up to
date with her appointments and screenings, her pain was managed using different pain reliever,
dental check up attended, blood test and urine test were all done and all went well. However,
Irene was still presenting the same symptoms despite her annual check. Irene was referred for
some tests for dementia and it was concluded that Irene has an early onset of dementia.
The RNLD has come up with a plan for intervention to support Irene. It was agreed that Irene
would benefit from behavioral management therapy given her dementia is early stages not
warranting the need for medications to avoid polypharmacy and prescribing cascade. Reducing
the need for medication given Irene is on numerous medications is appropriate and according to
Rieckert et al (2020) the less need for medication reduces the risk of adverse health outcomes
affecting quality of life especially in the vulnerable groups like the elderly and people with
multiple comorbidities.
In conclusion, HEF in application to Irene has indicated that Irene’s mental health scored the
most thus needing urgent intervention to improve quality of life. This will enable the RNLD
(Registered Nurse in Learning Disability) to outline the areas of need pertaining to Irene to target
support and intervention to prevent deterioration in health. The need for an intervention
highlights a gap in care that needs to be filled, placing duty on the RNLD to respond to the needs
of Irene and in her best interest to improve her quality of life.