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Fragile X Syndrome

Before discussing the specifics of fragile X syndrome, it is important that you understand the definition, various classifications and effects of mental retardation. Mental retardation is diagnosed before the age of 18 and can involve below-average intellectual functions and/or problems with adaptive skills. This type of learning disability makes acquiring and retaining knowledge difficult and time-consuming.

The Diagnostic and Statistical Manual of Mental Disorders (DSM IV) recognizes various levels of mental retardation. These levels are based on intelligence quotients. An intelligence quotient or IQ is a number determined by your performance on a standardized intelligence test. Your IQ is then compared to the average performance of others at the same age. IQ scores, however, do not tell the entire story. Even if you have a low IQ, you may still adapt well to your surroundings and function quite competently.

Furthermore, the validity of IQ testing has been questioned by many experts in the field. Higher or lower scores do not always correlate with performance in school and the test itself is considered biased toward those who come from a lower socio-economic class, those from more restrictive cultures and those who lack exposure to outside influences. Cultural minorities have a significant disadvantage when it comes to IQ tests. Despite these claims, IQ tests are and will likely remain the manner in which intelligence is measured.

A child with an IQ level between 50 and 70 is considered mildly mental retarded, while a child with an IQ level between 35 and 55 is considered moderately mentally retarded. A child with an IQ level between 20 and 40 is considered severely mentally retarded, while a child with an IQ level below 20 is considered profoundly mentally retarded.

The effects of mental retardation can range from mild to profound. Approximately 85% of cases fall into the mild category. When you are mildly mentally retarded, you may need help with the chores and daily functions, particularly when you are stressed. In addition, you may attend school and support yourself when you become an adult. Approximatley 10% of people who are moderately mentally retarded are able care for themselves, excel at vocational training and, with supervision, thrive in the community. Severely mentally retardated individuals require careful supervision. These individuals are able to function well when doing relatively simple tasks in a structured and sheltered setting.

If you have mental retardation, your speech may be impaired, but you will still be able to understand verbal communication. Approxmisately 1% of the general population suffer from profound mental retardation. If you are profoundly mentally retarded life is very challenging for you. In addition, you will need life skills training such as learning how to maintain your personal hygiene and/or learning how to communicate with others.

What is Fragile X Syndrome?

Fragile X syndrome is a genetic condition that occurs when a mutation on the end of the X chromosome, one of two that determine the sex of a child, either dangles or is broken off altogether. It is the second most common single cause of mental retardation. Some individuals merely carry the altered gene that causes fragile X syndrome and show no symptoms of the disorder. However, with each succeeding generation, the excessive genetic material at the area of fragility expands, increasing the chances that it will result in this disorder.

Girls with fragile X syndrome are usually very attractive, with lovely soft skin, while boys, typically have a long face, prominent and/or long ears and macroorchidism or large testicles. In addition, boys diagnosed with fragile X syndrome may have loose connective tissue, flexible joints and ankles, flat feet, heart murmurs, scoliosis and/or vision impairments. Both boys and girls may have hypotonia – low muscle tone that can cause difficulties sitting and walking. Hypotonia usually improves with age and physical therapy.

Males are more likely to have moderate mental retardation. However, roughly 10 to 15% of boys affected by fragile X syndrome have IQ’s ranging from borderline to low normal and learning disabilities that cause a short attention span and distractibility, impulsive behaviors, an inability to accurately process what they hear, restricted math skills and/or problems with abstract reasoning. In addition, approximately 30% of males admit that they remember bits of information found in their environment and facts on subjects that they have particular interest in.

Girls with fragile X syndrome have levels of intelligence bordering on normal and others having moderate mental retardation. With the exception of math, girls have less of a struggle with learning disabilities. Their language development and ability to express themselves verbally may take longer to develop and grasping the meaning of more complicated information may present a challenge. Overall, both boys and girls are slower than average, with a gap of about two and a half years between children with fragile X syndrome and other children at age 7.

Physical problems associated with fragile X syndrome include loose connective tissue, recurrent ear infections (especially in the middle ear), hernias, gastroesophageal reflux, cleft palates, scoliosis, flat feet and/or joint dislocation. Approximately 50% of adult men with this condition will experience heart disease (specifically mitral valve prolapse). Parents must be alert to possible vision problems such as strabismus, near and farsightedness. Seizures occur in one-fifth of boys.

In addition, children with this disorder may experience hyperactivity. Approximately 30% of teenagers and adults (mostly males) engage in aggressive or violent behavior. Girls, rather than boys, have a tendency to develop depression and/or anxiety, which can be treated successfully with the appropriate medications.

How Common is Fragile X Syndrome?

Approximately 1 in every 1000 boys and 1 in every 2000 girls develops fragile X syndrome.

What Treatments are Recommended for Children with Fragile X Syndrome?

The Individuals with Disabilities Education Act (IDEA) governs the ways in which states and public agencies provide services such as early intervention and special education from infancy through their college years. IDEA mandates that “a free and appropriate public education” is provided to every child of school age, including all that have special needs. Children with fragile X syndrome can expect transportation, therapeutic, counseling, vocational and mobility services.

Where Can I Find Additional Information on Fragile X Syndrome?

There are several resources that can provide more information on fragile X syndrome.

These resources include:

Children with Fragile X Syndrome: A Parents’ Guide by Jayne Dixon Weber and The National Fragile X Association (www.fragilex.org/html/home.shtml).

References:

Comer, R. J. (1996). Fundamentals of abnormal psychology. New York: W.H. Freeman and Company.

Kaplan, H. I., & Sadoc, B. J. (1996). Concise textbook of clinical psychiatry. Baltimore, MD: Williams & Wilkins.

Weber, J. D. (ed.). (2000). The Special needs collection: Children with fragile X syndrome: A parents’ guide. Bethesda, MD: Woodbine House.

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