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Frequently Asked Questions
Sherry Smith is a 6-month-old infant being seen in the clinic for a routine well-child
checkup. While documenting her data (length, weight, and occipital-frontal
circumference [OFC]), you note her skin and her sclera are pale, and she is lethargic
and somewhat irritable. You ask her mom about her feeding and sleeping. Mrs.
Smith reports that Sherry sleeps more than her other children, continues to
breastfeed, and has become more irritable than usual. A blood test reveals a
hemoglobin level of 10 g/dL. What additional information can be collected from
Mrs. Smith to help confirm a diagnosis? Why is this important?
Mrs. Smith reported that she is breastfeeding Sherry. You should make further inquiries
about how frequently Sherry feeds and for how long each time. You should also discuss
Mrs. Smith’s diet, particularly her intake of iron-containing foods. Mrs. Smith reports
that she decided to become a vegetarian a few weeks after Sherry was born. This piece of
information provides a clue to a possible diagnosis of iron deficiency anemia. Iron
deficiency anemia, the most common hematological disorder in infancy and childhood, is
associated to a great extent with poor or inadequate food choices accompanied by growth
of the infant or child. Dietary iron is absorbed in the small intestine and eventually
through different processes combines with other components of hemoglobin in the bone
marrow. Failing to ingest adequate amounts of iron coupled with the loss of fetal
hemoglobin lead to lower hemoglobin levels, manifested by the symptoms Sherry has
demonstrated.
Mr. and Mrs. Brown are expecting their first child. They are of African-American
descent. Mr. Brown has a brother and nephew with sickle cell anemia. Although
neither of the Browns have sickle cell anemia, they are concerned that their infant
may have it. What information can you provide without having genetic testing
results?
Sickle cell anemia is an autosomal recessive disease. In order for the disorder to be
present, both parents must have at least one recessive gene. For more information, the
Browns must be tested for the presence of the recessive gene. If neither parent has the
recessive gene it is most likely that their baby will not have sickle cell anemia. If one
parent has the recessive gene (is a carrier for sickle cell disease), there is a 25% chance
that the baby will be a carrier. If both parents have the recessive gene, there is a 50%
chance that the baby will be a carrier and 25% chance that he or she will have sickle cell
disease.
Mrs. Enderson has brought her 8-month-old daughter to the clinic, worried because
Amanda has been acting very unusual for the last month. Fighting a cold, Amanda
has suddenly developed difficulty breathing. Upon your assessment, you notice that
her sclera is yellow and her nail beds pale with prolonged capillary refill time
(CRT). The physician notes that Amanda’s liver is slightly enlarged and when her
feet, ankles, and arms are moved, she whimpers with pain. Lab work is ordered,
and the results indicate that Amanda is anemic. The physician is considering sickle
cell anemia. Mrs. Enderson doesn’t understand why this would even be a
consideration. Amanda has not experienced any health problems since her birth
except for her recent bout of respiratory illness. Why didn’t Amanda demonstrate
any symptoms of sickle cell disease at or shortly after birth? Why would the
symptoms suddenly begin now?
Infants with sickle cell anemia usually do not exhibit signs and symptoms of the disease
at birth or for the first few months of life. This is because of the presence of fetal
hemoglobin (Hb F), which does not sickle. Gradually as sickle hemoglobin (Hb S)
replaces Hb F, sickling increases and symptoms begin to develop. This replacement
begins immediately, but is mostly completed by the time the infant is about 6 months old.
Barry is 6 years old and has hemophilia type A, an inherited coagulation disease
with deficiency of factor VIII. Barry is going to school for the first time. His mother
seeks your help in planning Barry’s first school experience. What suggestions would
be helpful for Barry’s teacher, classmates, and the school staff?
A very important preparation for Barry’s first school experience will be education
regarding preventing and managing bleeding episodes. Teachers and staff need education
about how to identify a bleeding episode (e.g., open cut that does not coagulate, a knee or
elbow that swells and becomes painful) and what emergency care consists of
(compression or pressure for 10 to 15 minutes, elevation and immobilization of the
extremity, ice, and factor replacement). Barry should also wear a medic alert bracelet to
help identify his condition and list an emergency phone number. This information should
also be kept in the office and with the school nurse (if there is one). It will also be
important to provide education to Barry’s classmates, not to isolate him but to engage the
classmates in helping to make his environment and activities safe and to participate as
appropriate in his emergency care (calls for the teacher, brings the ice, etc.). Barry may
want to share some of his feelings and information personally. He should be supported in
doing this.
Cassandra is brought to the emergency department late one Saturday evening. Her
mother is worried because Cassandra has suddenly developed several bruises and
funny, small, reddish purple spots over her trunk. Her mom has also noticed dried
blood in Cassandra’s nose the last few mornings. Cassandra’s medical history
reveals normal growth and development, no changes in her bowel or bladder
functions, and no history of long unexplained fevers. She had an upper respiratory
infection about a week ago, but recovered quickly and easily from it. Cassandra is
diagnosed with immune thrombocytopenia (ITP). What is Cassandra’s prognosis?
Cassandra’s prognosis is excellent. Most cases of ITP resolve without treatment in 3 to 4
months. If treatment is ordered, it will most likely be supportive. The nurse will need to
provide education to the family on the importance of preventing injury and how to
control bleeding. For Cassandra, this may mean providing toys and food without sharp
edges, childproofing her bed, and being alert to surroundings for potential concerns. If the
physician determines that treatment should include administering prednisone, the nurse
will need to include additional teaching on administering the dosage as ordered,
identifying side effects, and informing the health care providers regarding behavior
changes that might occur (e.g., increased hunger and eating).
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