Prevalence of Dysphagia in People with Intellectual Disability: A Systematic Review

Introduction

The purpose of this study is to summarize international research that has been conducted on the prevalence of dysphagia in people with intellectual disabilities.

Methodology

The authors reviewed studies that were published from 1990 to July 2016. The studies were identified through using databases including Medline, Cinahl, PsyINFO, and Web of Science. Further information was obtained through email requests and cross-citations.

Through this method, twenty studies were identified. The study selection required that the studies were peer reviewed, and samples of people where at least 50% had an intellectual disability

Findings

  • Dysphagia is common in people with an intellectual disability and my be under-recognized.
  • Improved recognition and management of dysphagia may reduce the occurrence of associated health conditions and reduce hospital admissions and premature death
  • Organizations that are providing services and supports to individuals with an intellectual disability need access to resources that provide comprehensive information on topics relating to dysphagia.

 

Reference

Robert, J.; Chadwick, D.; Baines, S.; Emerson, E.; Hatton, C. (2017). Prevalence of Dysphagia in People with Intellectual Disability: A Systematic Review. American Journal of Intellectual and Developmental Disabilities. 55(6). 377-391

Williams Syndrome Awareness Month

Date: May 1- May 30, 2022

May is Williams Syndrome Awareness Month.

What is Williams Syndrome?

Williams Syndrome also known as Williams-Beuren syndrome was discovered in 1961 by J.C.P. Williams, a Cardiologist from New Zealand. Williams Syndrome is a rare disorder with a prevalence of in 7,500 to 20,000 caused by the deletion of genetic material from chromosome 7. Williams syndrome symptoms include heart problems, low birth weight, l problems and developmental delays. 75 are diagnosed with mile to moderate intellectual disabilities or a learning disability.

Facts and Statistics
  • It is a genetic condition that is present a birth.
  • It is a developmental disorder
  • Tend to have a mild or moderate intellectual disability.
  • It is also known as Beuren Syndrome and Williams-Beuren Syndrome.
  • The symptoms were first described by John C.P. Williams in 1961.
  • A year later, German Physician, A.J. Beuren described three new incidents of patients with similar facial features.
  • It is caused by the spontaneous deletion of 26-28 genes on Chromosome #7
  • The deletion is caused by either the sperm or the egg.
  • The deletion is present at the time of conception
  • The most common symptoms of Williams Syndrome includes unusual facial features and heart defects.
  • The diagnosis is typically confirmed after identifying facial features and genetic testing.
  • An individual with Williams Syndrome has a 50% chance of passing the disorder on to their children.
  • Williams Syndrome affects 1 in 10,000 people worldwide.
  • An estimated 20,000 to 30,000 people in the United States are affected.
  • It occurs in both males and females equally
  • It is found in every culture
  • Individuals with Williams Syndrome tend to be overly friendly.
  • People with Williams Syndrome often have difficulty with visual-spatial tasks
  • Congenital heart defects (CHD) occur in approximately 75 percent of children
  • By the age of 30, the majority of individuals with Williams Syndrome have pre-diabetes or diabetes.

Physical characteristics include:

Musculoskeletal

Almond shape eyes

Broad forehead

long neck

Longer upper lip

Puffiness around the eyes

sloping shoulders

Small chin

Small upturned nose

Wide mouth

Learning Characteristics

During Williams Syndrome Awareness Month, You can create a fundraising page or team

Download awareness event planning tools

purchase an awareness t-shirt

For more information: Awareness Month | Williams Syndrome Association (williams-syndrome.org)

What is Cerebral Palsy?

What is Cerebral Palsy?

Cerebral Palsy is a collection of motor disorders resulting from damage to the brain that can occur before, during and after birth. Congenital cerebral palsy indicates that a person developed cerebral palsy at birth which is the case of the majority of people with cerebral palsy. According to the Centers for Disease Control and Prevention (CDC), Cerebral Palsy (CP) is a group of disorders that affect a person’s ability to move and maintain balance and posture. It is the most common motor disability in childhood. It is estimated that an average of 1 in 345 children in the U.S. have cerebral palsy. For many years, it was thought cerebral palsy was due to lack of oxygen. Studies show this only accounts for 19% of all cases.

Prevalence and Characteristics
  • Around 764,000 people in the United states have at least one symptom of cerebral palsy
  • Around 10,000 babies are born each year with cerebral palsy
  • Boys are diagnosed more often than girls
  • Cerebral palsy is the mot commonly diagnosed childhood motor disability in the United States
  • Over 77% of children with cerebral palsy have the spastic form
  • More than 50% of all children with cerebral palsy can walk independently
  • African American children with cerebral palsy are 1.7 times more likely to need assistance with walking or be unable to walk at all
  • Around 41% of babies and children with cerebral palsy will have limited abilities in crawling, walking and running.
  • Around 41% children with cerebral palsy in the United states have some form of a cognitive disorder
  • Behavior problems are common in children with cerebral palsy including social skills and anger issues.
  • Seizures are a common associate disorder of cerebral palsy and can range from mild to extreme severe.
  • There is no known cure
What Causes Cerebral Palsy?

Studies show that about 10 to 20 percent of children with cerebral palsy acquire the disorder after birth. This includes through infections, jaundice, RH incompatibility and severe oxygen shortage in the brain.

Types of Cerebral Palsy

Ataxic- indicates the muscle tone is too low or too loose

  • affects 5 to 10 percent of people with cerebral palsy
  • movements are unsteady and shaking
  • have difficulty making quick movements

Spastic- refers to the inability of  muscle to relax

  • is the most common type of cerebral palsy
  • 70-80% of people have spastic cerebral palsy
  • will have difficulty moving from one position to another

Athetoid-uncontrolled twisting movements

  • Affects 10 to 20% of people with cerebral palsy
  • often have difficulty holding themselves in an upright position
  • muscles move involuntarily causing limbs to twitch

Spina Bifida Factsheet

 

Download here: Spina Bifida

Down Syndrome and Sleep Apnea

Obstructive Sleep Apnea Syndrome (OSAS) is considered one of the conditions affecting 2% to 4% of adults with Down syndrome and as they get older, the prevalence increases to 37% of men and 50% of women.

What is Obstructive Sleep Apnea?

It is a common disorder due to repetitive episodes of different breathing while sleeping due to upper airway collapse. The obstruction occurs when the muscles in the back of the throat fails to keep the airway open.

Signs and Symptoms

Signs of obstructive sleep apnea in individuals with Down syndrome include:

  • Snoring
  • Gasping
  • Excessive daytime sleeping
  • Daytime mouth breathing

According the Down Syndrome Association, the following techniques will help with sleeping during the night:

  • a nightly routine at bedtime
  • a bedroom that is free of distractions (e.g. cut out any unwanted light or noise)
  • regular sleeping hours
  • regular exercise and activities
  • avoidance of caffeine and other stimulants in the evening
  • avoidance of exercise in the evening.
Resources

Obstructive Sleep Apnea and Down Syndrome– NDSS

Obstructive sleep apnea in children with Down syndrome– Children’s Hospital Boston

Obstructive sleep apnea in children with Down syndrome– Massachusetts General Hospital

Obstruction sleep apnea in patients with Down syndrome: Current perspectives- NCBI

Sleep apnea confirmed common in children with Down syndrome– Cincinnati Children’s

Sleep problems in people with Down syndrome- Down’s Syndrome Association