What to Expect the Toddler Years (259 page)

Hearing aids (which magnify all sounds) or assistive listening devices (which magnify selected sounds without background noises). There are many types of devices, and the type used will depend on the child’s age and the type of hearing loss. Some assistive listening devices are used with hearing aids, others are used independently.

Education. An education program should be begun as soon as hearing loss is diagnosed, and may include teaching a child to use devices that assist in learning to hear and/or to speak; cued speech, in which a system of manual cues is used to supplement speech (or lip) reading; a total communication program, which uses a combination of speech reading, signing, and finger spelling and may also emphasize listening skills and speech production. No one program fits all hearing-impaired children. The child’s pediatrician and hearing specialist can help parents find the program that best meets their child’s needs.

Whether a child with a hearing impairment is mainstreamed (that is, attends regular classes with hearing children) depends on the individual child, the programs that local schools offer, and the availability of special classes in speech and language development in the mainstream schools. When the hearing-impaired toddler is ready for day-care or preschool, the options should be discussed with his or her doctor and
audiologist as well as the school director and/or teachers at the program being considered.

Prognosis.
With proper treatment, children with hearing impairments can have successful, fulfilling lives. Some may be helped to hear and to speak; others will learn to communicate through signing.

What is it?
A pediatric connective-tissue disease, or actually, group of diseases. Fatigue, low-grade fever, loss of appetite, weight loss, and failure to grow are common at onset of the disease in children with moderate to severe JRA. Morning joint stiffness, or “gelling” after inactivity, and night pain are also common, but toddlers are often not able to verbalize these symptoms; instead they may just become increasingly irritable, seem to be protective of their joints, and limp and/or refuse to walk. The three most common forms of JRA are:
Polyarthritis
. In this, the most common form, five or more joints—most often those in the knees, wrists, elbows, and ankles, and possibly the hands—are involved, usually symmetrically (both wrists or both ankles are involved, for example). Joints may be tender and painful, though the child may not complain of the pain, and there may be neck stiffness and loss of rotation (the head can’t turn fully from side to side). Systemic involvement (generalized illness, fever; see below) is usually mild.
Oligoarthritis (pauciarticular disease)
. This form of JRA is almost as common; four or fewer joints are involved (sometimes just the knees or the ankles or even just one joint), and there may be chronic uveitis (irritation of the iris of the eye).
Systemic disease
. This form accounts for about 10% to 20% of JRA cases. A variable number of joints are affected, systemic involvement is prominent, and chronic uveitis is rare; systemic symptoms that include a spiking fever (over 102.2°F or 39°C) once or twice a day that quickly returns to normal or below and a salmon-colored rash that comes and goes are common and may precede joint symptoms by weeks or more. Intermittent episodes of fever may continue for years in some children; in others, severe chronic polyarthritis develops. The spleen and liver may be enlarged and a variety of organs, including the heart, may be affected.

How common is it?
1 in 250 children in the U.S. has arthritis or another rheumatologic condition.

Who is susceptible?
Though JRA can begin anytime during childhood, the median age is one to three. Polyarthritis is three times more common in girls than in boys, oligoarthritis (pauciarticular disease) is five times more common in girls, and systemic disease is about equally common in girls and boys.

What causes it?
The cause is unknown.

Related problems.
Uveitis (inflammation of the iris of the eye), especially with oligoarthritis; disturbances in growth and development.

Treatment/management.
A wide range of medications, including aspirin, steroids, glucocorticoids, nonsteroidal anti-inflammatory drugs (NSAIDs), methrotraxate, or combinations of these, and/or injections of a medication containing gold. (If, however, your child is taking aspirin for Juvenile Rheumatoid Arthritis and comes down with chicken pox, influenza, or any other suspected viral illness, stop administering the
aspirin and switch to an NSAID during the course of his or her illness—see page 597). Antibiotics are given for infectious arthritis. Splinting, physical therapy, and exercise are also important (though sometimes unappealing because of the associated pain). Joint replacement may be considered when damage is severe. Steroid eye drops and dilating agents are usually successful for treating uveitis. Treatment can often completely control JRA.

Some procedures (such as joint injection, open synovectomy, and arthroscopic synovectomy) are controversial. In older children, muscle relaxation, guided imagery, and meditative breathing may be adjunct therapies that help reduce pain and improve functioning.

Prognosis.
Polyarthritis
. Varies from poor to moderately good.
Oligoarthritis
. Generally excellent (since the disease is usually nondestructive), except for the risk of damage to eyesight (glaucoma or cataracts) from untreated uveitis.
Systemic disease
. Moderate to poor; the systemic disease is self-limiting, but the arthritis becomes chronic and destructive in half of children affected, causing disability. Most children with JRA recover and have normal lives. But JRA can sometimes lead to permanent damage to the joints and to deformities; treatment can often make a major difference in outcome.

What is it?
A condition in which there is a slower rate of learning and a limited capacity to learn. (Though some people prefer such terms as “intellectual disability,” the medical community still uses the term “mental retardation.”) Intelligence is below the normal range—usually defined as an IQ below 70 on standard scales. Only 11% of children in this group have IQs below 50 (making them moderately or severely retarded) or under 25 (making them profoundly retarded). The other 89% are mildly retarded and educable. (Retardation is sometimes assessed somewhat differently: mild retardation, an IQ of 52 to 67; moderate, an IQ of 36 to 51; severe, an IQ of 20 to 35; and profound, an IQ of below 20.) Most mildly retarded children develop large motor skills (sitting, crawling, standing, walking) at the appropriate age, unless they also have cerebral palsy or another motor disorder. Mild retardation may not be evident until it is clear that language is not developing well. More severely retarded children may not walk until very late, possibly because there is some motor involvement in their condition. Parents may notice overall developmental slowness, motor disability, language disorder, and behavior disturbances early on. Children who consistently do not reach social and language milestones by the time 90% of their peers do need to be evaluated, though they won’t necessarily turn out to have intellectual deficits.

How common is it?
About 1% to 3% of Americans are considered mentally retarded. An estimated 3 in 100 children have IQs below 70.

Who is susceptible?
Preterm or low-birth-weight infants, and children with any of the risk factors listed in “causes,” below. In general, boys are affected more often than girls (possibly because they are more susceptible to the fragile X syndrome).

What causes it?
It is estimated that the cause of some 30% to 60% of cases of retardation are unknown. The major known causes in the U.S. are Down syndrome and fragile X syndrome, both chromosomal abnormalities. Other causes include: the heavy use of alcohol
and possibly other drugs during pregnancy; intrauterine infection or infection during childbirth (toxoplasmosis or herpes simplex virus); exposure to lead and possibly other environmental toxins, pre-or postnatally; genetic factors (Hurler syndrome, neurofibromatosis); inborn errors of metabolism that go untreated (phenylketonuria, hypothyroidism, galactosemia); brain damage (due to trauma or lack of oxygen before or during birth); severe childhood trauma (such as nearly drowning or head injury); certain childhood diseases; pre- or postnatal malnutrition or placental insufficiency. In some cases, two or more factors may combine to cause the retardation. Sometimes what appears to be mild retardation is actually due to a lack of attention and inadequate physical and intellectual stimulation in infancy and early childhood or to a hearing or vision deficit. With adequate intervention, the IQs of these children can be brought up into the normal range.

Related problems.
Sometimes, other neurological deficits, most commonly CP (about 1 in 10 retarded children has CP). Less common, but not unusual, are hearing loss, vision deficits, language problems, seizures, disorders of sensation and perception, malformations of the brain and other organ systems, and motor disability.

Treatment/management.
Early intervention and training, both by professionals and by parents trained by professionals, can raise the IQs of mentally retarded children and give them a better chance for a more productive future. Often removal of causative factors (such as lead in the water) or treatment of a causative condition (such as phenylketonuria or a hearing or vision deficit) can increase IQs up to inborn potential. Improved nutrition and nutritional supplementation can also raise IQ in some cases.

Prognosis.
With optimum education begun early, children who are educable can learn basic academic (most often, to the fourth-grade level), social, and vocational skills, and are able to function independently as adults. Many marry, have children (who, unless a genetic disorder is involved, are no more likely to be retarded than other children), and make satisfying lives for themselves. Those who are trainable can rarely learn useful reading, writing, or spelling skills. With optimal early education they can, however, learn to speak and to complete routine tasks and take care of their personal needs. As adults they can usually function well in a sheltered environment (either within their family or in a group home). Those who are subtrainable (severely and profoundly retarded) are not able to achieve this level of learning and may communicate only nonverbally; their ultimate mental age is no more than six years and they are often unable to take care of even their simplest needs. Most, however, do learn to walk, unless they have CP. Profoundly retarded individuals require constant care and supervision throughout their lives.

What is it?
Duchenne-type muscular dystrophy (DMD) is the most severe form of progressive primary muscular degeneration. Though the condition, which generally affects only boys, is present at birth, symptoms don’t usually appear until sometime between ages three and five. Thereafter there is rapid deterioration and most children with DMD are confined to wheelchairs by the time they’re ten or twelve years old. (Other types of muscular dystrophy
are much less common; they are also less severe.)

How common is it?
About 1 in 3,500 to 5,000 boys is born with the condition.

Who is susceptible?
Mostly males, who inherit the faulty gene. Occasionally, a female child develops DMD, usually because of a chromosomal aberration.

What causes it?
X-linked inheritance, passed on from mother to son on the X chromosome. In about a third of cases there is no family history of the problem because the genetic defect is a mutation.

Related problems.
Cardiac and nervous system involvement; musculoskeletal deformities; respiratory failure.