What to Expect the Toddler Years (260 page)

Treatment/management.
Presently only supportive treatment is available, but much research is under way. One controversial study suggests that injecting immature muscle cells, called myoblasts, into the muscles of a DMD patient may improve muscle strength.

Prognosis.
At this time, the prognosis is poor. Muscular weakness becomes progressively worse; many children with DMD do not survive their teen years. Hopefully, medical research will find a way to improve the prognosis in the future.

What is it?
A metabolic disorder in which the affected individual is unable to metabolize a protein called phenylalanine. The buildup of phenylalanine in the bloodstream can interfere with brain development and cause serious retardation.

How common is it?
1 in 10,000 to 15,000 newborns in the U.S. is affected.

Who is susceptible?
The child of parents who both carry the trait has a 1 in 4 chance of being born with PKU. The incidence is low among Finns, Ashkenazi Jews, and those of African descent.

What causes it?
Autosomal recessive inheritance; both parents must pass on recessive genes for their child to have PKU.

Related problems.
Without treatment, children with PKU are irritable, restless, and destructive; they may have a musty odor, dry skin or rashes, and possibly convulsions. They are usually physically well developed, and often blonder than others in their family.

Treatment/management.
If the blood test for PKU, routinely performed shortly after birth, is positive, treatment with a diet low in phenylalanine (breast milk, cow’s milk and regular cow’s milk formula, meat, and the artificial sweetener aspartame are high in phenylalanine) will be instituted. It is recommended that the diet should be started as soon as possible after birth and be continued for at least twelve years to maximize IQ. Some studies suggest that continuing the diet through the reproductive years may be particularly beneficial for women. At any rate, the diet should be resumed when a woman with PKU becomes pregnant. Blood levels of phenylalanine are monitored periodically during treatment. Researchers are trying to find a medication that can help individuals with PKU metabolize phenylalanine. Until then, careful dietary control in childhood and pregnancy is critical.

Prognosis.
Usually, a completely normal life for those who are treated; serious retardation for those who aren’t.

What is it?
A form of anemia in which red blood cells contain hemoglobin S rather than normal hemoglobin (hemoglobin is a major component of the oxygen-carrying red blood cells). Hemoglobin S has the ability, especially when oxygen is short, to change the usually disc-shaped red blood cells to a sickle shape. These abnormal cells do a poor job of delivering oxygen to the cells throughout the body and often clump together, blocking blood vessels and causing a sickle-cell “crisis.” Children with sickle-cell disease may be chronically fatigued and have difficulty breathing. Painful swelling of joints, especially in fingers and toes, is common. There are several types of sickle-cell crisis; the most common type is characterized by excruciating pain. Carriers of the sickle-cell trait (those with just one gene for sickle cell) may sometimes show mild signs of the disease.

How common is it?
In the U.S., 1 in 500 children of African descent has sickle-cell anemia (as many as 1 in 12 carry the trait); the incidence is much lower in children from other backgrounds.

Who is susceptible?
Primarily children of African descent, but also those of Continental Indian /Middle Eastern/ Mediterranean heritage. Risk of sickle cell is 1 in 4 if both parents are carriers; however, if both parents have sickle cell,
all
their children will have the disease.

What causes it?
Autosomal recessive inheritance: Both parents must pass on recessive genes for a child to have full-blown sickle-cell anemia. Periodic crises can be triggered by infection, stress, dehydration, and inadequate oxygen (from overexertion or high altitude).

Related problems.
Poor growth, delayed puberty, narrow body, curved spine, and barrel chest; increased susceptibility to infection, particularly pneumococcal; eventual damage to organs denied blood during a crisis (including liver, kidneys, gall bladder); stroke.

Treatment/management.
Folic acid and other nutritional supplementation to help control the anemia and improve growth and development; pain relievers, oxygen, and fluids to keep the patient comfortable when a sickle-cell crisis occurs; transfusions of healthy blood in a severe sickle-cell crisis; prophylactic antibiotic therapy to help prevent the development of pneumonia in young children. Pneumococcal vaccine to prevent infection is useful in some adults and is now recommended by the CDC for use by children. Bone marrow and stem cell transplants are experimental. Ultrasound evaluation may be able to predict children who are at high risk for stroke, and allow for possible preventive measures to be taken. Other experimental procedures are currently under investigation, including high-dose IV corticosteroids to relieve pain.

Prevention.
Avoidance of factors that may reduce oxygen supply (including overexertion, cold, stress, and high altitude) may reduce the number of crises.

Prognosis.
Fair; while some do not live past young adulthood, others reach middle age. Those with high levels of fetal hemoglobin tend to do best.

What are they?
A group of hereditary anemias, in which there is a defect in the process necessary for the production of hemoglobin (a major component of the oxygen-carrying red blood cells). The most common form, thalassemia B, can range from thalassemia minima, which has no visible symptoms but can be detected through blood and genetic testing, to the very serious Cooley’s anemia. Even with serious illness, infants appear normal at birth, but gradually become listless, fussy, and pale, and begin to lose their appetites. Growth and development are slow.

How common is it?
Worldwide, more than 100,000 babies are born each year with severe thalassemia.

Who is susceptible?
Most often those from Mediterranean backgrounds.

What causes it?
Autosomal recessive inheritance; both parents must pass on a recessive gene for a child to have the trait.

Related problems.
Bone weakness and fractures; iron overload, which can cause a variety of problems.

Treatment/management.
Transfusions; bone marrow transplants may be warranted in severe cases; sometimes, prophylactic antibiotics to prevent infection; removal of excess iron through chelation therapy, when necessary.

Prognosis.
Excellent for those with minor forms of the disease; children with moderate disease also do well, though puberty may be delayed. Of those with severe disease, more children are now living into their teens and twenties, though the threat of heart failure and infection are still significant.

What is it?
An inability to see well, even with corrective lenses. See page 478 for clues to vision problems.

Who is susceptible?
Anyone who sustains an injury to the eye or suffers from a condition (present at birth or acquired later) that can damage vision.

What causes it?
Injury, infection, genetic disease, eye diseases (such as cataracts and glaucoma).

Related problems.
The myriad educational and social problems related to being unable to see.

Treatment/management.
Depends on the cause, but in all cases prevention is the best treatment. Sometimes surgery can restore partial vision. There are many modern approaches to educating those who are visually handicapped, including voice-activated computers and machines that translate print into voice. Controversial is the teaching of Braille, which has been dropping in recent years, but knowledge of which many experts insist is necessary for building a successful future.

Prevention.
Good eye care and proper safety precautions (see page 477); prompt treatment of infection or other eye problems, such as cataracts.

Prognosis.
Children who cannot see can, with the use of modern teaching technology, be educated and prepared for successful careers and fulfilling lives.

They can go from archenemies to best friends—and back again—in the space of a single morning; hurling insults at each other one moment, playing a companionable game of catch the next. They compete with each other, support each other, attack each other, defend each other, alternatively love, like, hate, and tolerate each other. They share the same toys, the same table, the same parent or parents, and often the same bedroom. They are siblings.

The relationship between siblings can be stormy, satisfying, or more likely, a confusing combination of the two. But when one (or both) of the siblings is a toddler, still short on social skills (including the ability to share, to compromise, to empathize, to cooperate) and long on egocentricity and possessiveness (the toddler wants center stage—and everything on it), the relationship can be more complex still.

Whether your toddler’s sibling (or siblings) is older, younger, or yet-to-be-born, there will be a variety of sibling-related issues to deal with. As parents, you’ll need to decide when to intervene, when to mediate, and when to turn your back and hope for the best. The following scenarios are a few of the most common that present themselves when a toddler is a sibling; use the advice as a guide to help you deal with the hundreds of others that are sure to come along.

“We want so much for our two daughters to be friends—that’s why we had them so close together. But we’re worried about sibling rivalry.”

Don’t worry about sibling rivalry, but do expect it. It’s one of those facts of life that’s pretty much inevitable; it isn’t so much a matter of whether, but of when and how much. Your daughters are probably the only two people in the house who
didn’t
choose to live together
(as, when they are older, they will probably remind you). And while they may very well grow to love each other, and even (this may be the hard part) to like each other, they may not grow to be best friends. The fact that they’re related doesn’t guarantee that they’ll ultimately have a lot in common besides family. (And even if they do, there can still be conflict—being too much alike can pose just as much of a problem in a sibling relationship as being altogether different.)

Remember, rivalry is the natural outcome when two people compete for the same prize—in this case, the attention and love of their parents. You can help minimize the competition, and thus the rivalry, by being sure to show love and appreciation for each child the way she is (by never comparing her—positively or negatively—to her sibling), by regularly spending time alone with each child, by making sure that policies and privileges are equitable (though not necessarily precisely the same), and by not showing favoritism (the child who feels her sibling is preferred is likely not only to feel the need to fight harder for parental attention, but to fight harder with her sibling, too). No matter how fairly you treat your two offspring, however, you can’t possibly eliminate all rivalry; it exists in even the best of sibling relationships. In fact, the only sure way to prevent sibling rivalry is to have just one child. (Keep in mind that there are many positive effects of sibling quibbling; see page 737.)