The parents of a child with sickle cell anemia (SSA) tell the nurse, "We have never had
any symptoms of SSA and do not understand why our child has this
... [Show More] problem." Which
information will the nurse include when teaching the parents?
1
SSA is caused by a random genetic mutation with no known cause.
2
People who are carriers of SSA may not have symptoms, but all of their children will
have SSA.
3
If both parents are carriers of SSA, there is a 25% chance that offspring will have SSA.
4
When a child is born with SSA, genetic testing of both parents is needed to determine if
they have sickle cell trait. - 3 If both parents are carriers of SSA, there is a 25% chance
that offspring will have SSA.
People who have 1 gene for SSA are described as having sickle cell trait or as carriers
of SSA and are usually asymptomatic for sickling-related complications. Sickle cell
anemia is not random; the gene must come from both parents. If both parents are
carriers, with each pregnancy there is a 25% chance that the child may have the
disease and a 50% chance that the child will be a carrier. Genetic testing is not needed
to establish the presence of sickle cell trait when two asymptomatic parents have a child
with SSA because SSA is an autosomal recessive inherited condition and both parents
must be carriers to have a child with SSA.
An adolescent child with sickle cell anemia is admitted to the pediatric unit during a
vaso-occlusive crisis. Which pathophysiology is correct?
1
Severe depression of the circulating thrombocytes
2
Diminished red blood cell (RBC) production by the bone marrow
3
Pooling of blood in the spleen with splenomegaly as a consequence
4
Blockage of small blood vessels as a result of clumping of RBCs - 4 Blockage of small
blood vessels as a result of clumping of RBCs
The red blood cells in sickle cell anemia are fragile. When hypoxia or dehydration
occurs, the cells take on a crescent shape; they then clump together and occlude blood
vessels. The platelet count is not severely depressed in vaso-occlusive crisis.
Diminished RBC production by the bone marrow is an aplastic crisis resulting in severe
anemia. Pooling of blood in the spleen that results in splenomegaly is known as a
splenic sequestration crisis.Which is the most appropriate nursing intervention for an adolescent child with sickle
cell anemia?
1
Teaching the family how to limit sickling episodes
2
Preparing the child for occasional blood transfusions
3
Educating the family about prophylactic medications
4
Explaining to the child how excess oxygen causes sickling - 1 Teaching the family how
to limit sickling episodes
To help prevent a crisis, the child and family must be taught to try to prevent sickling by
maintaining hydration, promoting adequate oxygenation, and avoiding strenuous
exercise. Blood transfusions are a common treatment rather than an occasional
occurrence. There are no prophylactic medications to prevent sickle cell crisis. It is a
lack, not an excess, of oxygen that contributes to sickling.
The parent of a child with sickle cell anemia tells the nurse that the family is going
camping by a lake in the mountains this summer. The parent inquires what activities are
appropriate. Which activity would the nurse suggest?
1
Swimming in the lake
2
Soccer with the family
3
Climbing the mountain trails
4
Motorboat rides around the lake - 4 Motorboat rides around the lake
Motorboating is a relatively passive activity that will not increase the child's oxygen
demands, which can precipitate sickling and a painful episode. Mountain lakes are
usually cold; temperature extremes can contribute to sickling, which may precipitate a
painful episode. Playing soccer may lead to increased cellular metabolism and
increased tissue hypoxia, which can precipitate sickling that could progress to a painful
episode. High altitudes should be avoided because the lower oxygen concentration of
the air might trigger a painful episode. [Show Less]