Child and Preadolescence: Conditions and Interventions

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Child and Preadolescence Conditions and
Interventions

• Brown Ch 13

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Special Needs Growth

• May be different than growth for child without
  special needs
• Special Growth Charts List of charts Table 13.1
• Down Syndrome http//www.growthcharts.com/
• Prader-Willi syndrome
• http//www.pwsausa.org/postion/HCGuide/HCG.htm
• http//depts.washington.edu/nutrpeds/fug/growth/sp
  ecialty.htm Also has Noonan Syndrome and others

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Noonan Syndrome

• Support Group http//www.noonansyndrome.org/
• Information about Noonan Syndrome
  http//www.emedicine.com/PED/topic1616.htm

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Noonan Syndrome

• Noonan syndrome was first recognized as a unique
  entity in 1963 when Noonan and Ehmke described a
  series of patients with unusual facies and
  multiple malformations, including congenital
  heart disease.
• Symptoms similar to Turner syndrome (sex
  chromosome abnormality in females with one X
  chromosome missing or partly missing)
• Noonan Syndrome The pathophysiology not fully
  understood. May be autosomal dominant gene on
  chromosome 12. May appear spontaneously
• Karyotyping (looking at the chromosomes)
  important to distinguish between syndromes

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Noonan Syndrome

• The cardinal features of Noonan syndrome are
• unusual facies (ie, hypertelorism(widely set
  eyes), down-slanting eyes, webbed neck)
• congenital heart disease (in 50)
• pulmonary stenosis
• short stature
• chest deformity
• Approximately 25 of individuals with Noonan
  syndrome have mental retardation
• Bleeding problems may be present (50 of pts)
• Skeletal, neurologic, genitourinary, lymphatic,
  eye, and skin findings may be present to varying
  degrees.

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Noonan Syndrome Incidence

• In the US The incidence of Noonan syndrome is
  estimated at 1 in 1000 to 1 in 2500 live
  births..Males and Females equally affected.
• Similar world wide

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Noonan Syndrome Dx

• Growth parameters
• Size at birth is usually within the reference
  range.
• Short stature at adulthood is present in up to
  80 of patients. Average adult height for males
  is 5'5" and for females is 5'.
• The disorder is present from birth, but age
  impacts upon the facial phenotype. Infants with
  Noonan syndrome can be difficult to recognize by
  facial appearance alone.

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Noonan Syndrome Treatment

• No restricted foods
• If eating problems exist related to heart
  disease, pulmonary stenosis, or other related
  problems, nutrition support may be necessary
• Monitoring Growth small size consider Noonan
  Syndrome

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Case Study Cloud 6
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Growth Assessment Signs or Red Flags

• A plateau in weight
• A pattern of gain and then weight loss
• Not regaining weight lost during an illness
• A pattern of unexplained and unintentional weight
  gain

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Assessment of Growth Affected by Many Factors

• Onset of problem Example of a child who suffers
  from seizures
• If seizures start early in life versus later in
  life.
• Eg is started in middle school, growth patterns
  may already be established vs
• In toddlerhood where growth patterns may not have
  been established

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Body Composition and Growth

• Special Needs children may or may not be
  typically proportioned in size of body and body
  components
• Down syndrome kids need to be plotted on Down
  syndrome growth charts
• If not will show up as low tile on regular chart
  when their development would appear to be regular
  using Down syndrome chart

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Down Syndrome toddler

• 28 month old girl with Down Syndrome
• 32 inches and
• 23.5 pounds
• Plot on CDC growth chart
• Plot on Down Syndrome Growth Chart
• How do they compare?

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Special Conditions Cystic Fibrosis

• Genetic Disorder 1 in every 1500-2000 live
  births
• Long arm of Chromosome 7
• Mal-absorption of certain nutrients
• Resulting in lower rates of growth and higher
  energy needs due to lung infections
• Multi-disciplinary teams of RD, respiratory
  specialist, pulmonary specialist, and social
  worker are used in some sites.

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Cystic Fibrosis

• CF is an inherited (genetic) condition affecting
  the cells that produce mucus, sweat, saliva and
  digestive juices. Normally, these secretions are
  thin and slippery, but in CF, a defective gene
  causes the secretions to become thick and sticky.
  Instead of acting as a lubricant, the secretions
  plug up tubes, ducts and passageways, especially
  in the pancreas and lungs. Respiratory failure is
  the most dangerous consequence of CF.

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Cystic Fibrosis

• 3,200 white babies born Each year with CF. Much
  less common among black and Asian-American
  children.
• Most diagnosed by age 3, although mild forms of
  the disease may not be detected until the third,
  fourth or fifth decade of life.
• In all, about 30,000 American adults and children
  are living with the disorder.

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Cystic Fibrosis

• Defective gene alters a protein that regulates
  the normal movement of salt (sodium chloride) in
  and out of cells.
• Results in thick, sticky secretions in the
  respiratory and digestive tracts, as well as in
  the reproductive system. It also causes increased
  salt in sweat on the skin.

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Cystic Fibrosis

• Recessive Trait Need one gene from each parent
  to show trait
• CF carrier test available for prospective
  parents. IF CF runs in family, good idea to test
  both potential parents. Sample of blood or
  saliva, can help determine whether parents carry
  the gene for CF. Approximately 10 million
  Americans are CF carriers.

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Cystic Fibrosis Diagnosis

• Sweat test
• Measures the amount of sodium or chloride in a
  person's sweat.
• A small amount of an odorless sweat-producing
  chemical is applied to a small area on the arms
  or legs.
• An electrode attached to the area stimulates a
  very weak and painless electric current, causing
  a tingling or warm feeling.
• After several minutes, sweat is collected from
  the stimulated area and sent to a laboratory for
  analysis.

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Cystic Fibrosis

• The main goal is to prevent infections, reduce
  the amount and thickness of secretions in the
  lungs, improve airflow, and maintain adequate
  calories and nutrition.

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Cystic Fibrosis Treatment

• Antibiotics
• Mucus-thinning drugs
• Bronchodilators. Use of medications such as
  albuterol (Proventil, Ventolin) may help keep
  open the bronchial tubes by clearing thick
  secretions.
• Oral enzymes and good nutrition Often more
  calories needed to support poor digestion
  Individualized to meet needs for growth

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Cystic Fibrosis Future Treatment

• Insert copies of the normal gene into cells of
  the respiratory tract
• Researchers are developing innovative gene
  delivery systems -- or vectors -- to determine
  the best way to deposit healthy genes into the
  airways of people with CF. These "healthy" genes
  are manufactured in a laboratory using
  state-of-the-art biotechnology. Modified viruses
  that target the airways and compacted DNA are
  among the vectors being refined for potential
  gene transfer. Drissel virus containing cocktail
  down airway for virus to infiltrate the airway
  lining cells
• Other Treatment Lung Transplant

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ADHD

• 3 to 5 of children
• Chaotic meal pattern due to inability to sit down
  for a meal
• Treatment may be one of two approaches
• Structured behavioral approach that included
  mental health counseling and support
• A psychostimulant approach that includes
  medication such as ritalin or adderal
• These may reduce appetite and have a negative
  affect on growth

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ADHD

• Nutrition intervention Adding snacks when meds
  are not at peak blood levels
• Eg. Peak blood levels sought during school hours
  to control behavior. This includes lunch time.
• Breakfast and after school and evening snacks may
  help stabilize intake of Kcal for growth and
  development.

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Case Studies Case 13.1 Child with Spina Bifida
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Case Study 13.2 Dealing with Food Allergies in
School Settings