Explanation: Hemophilia is a bleeding disorder caused by a deficiency in coagulation proteins.
Clients with classic hemophilia, or hemophilia A, lack factor VIII. Clients with hemophilia B
CONFIDENTIAL
(Christmas disease) lack factor IX. When injured, clients with hemophilia should be monitored
closely for external as well as internal bleeding. The most frequent sites of bleeding are the
joints (80%), especially the knee. Hemarthrosis can occur with minimal or no trauma, with
episodes beginning during toddlerhood when the child is active and ambulatory. Over time,
chronic swelling and deformity can occur.
(Option 1) Heart valve injury is common with rheumatic heart disease not hemophilia.
(Option 2) Intellectual disability in children is commonly seen with fetal alcohol syndrome,
Down syndrome, hypothyroidism, and lead poisoning. In rare cases, hemophilia can cause life-
threatening intracranial bleeding. However, isolated intellectual disability is not seen.
(Option 4) Recurrent pneumonia is commonly seen with cystic fibrosis not hemophilia.
Educational objective:
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,Clients with hemophilia are at risk for permanent joint destruction due to frequent bleeds
into the joint spaces. Assisting clients with decreasing the incidence of bleeding episodes and
prompt treatment when bleeding occurs can help minimize joint destruction.
A A A
The mother of a 6-year-old child with cystic fibrosis (CF) has received instruction on the use of
pancreatic enzymes. Which statement made by the mother indicates a need for further
teaching?
1. "I need to monitor the total amount of this medication that I give to my child every
day." [6%]
2. "I should give this medication with or just before my child has a meal or snack."
[10%]
3. "It is okay for my child to chew this medication." [61%]
4. "It is okay to open the capsule and sprinkle the medicine on a tablespoon of
applesauce." [21%]
Explanation: In CF, unusually thick mucus obstructs the pancreatic ducts, preventing pancreatic
enzymes (amylase, trypsin, and lipase) from reaching the small intestine. The result is
malabsorption of carbohydrates, fats, and proteins; the inability to absorb fat- soluble vitamins
(A, D, E, and K) is of particular concern. Gastrointestinal signs and symptoms of CF include
flatulence, abdominal cramping, ongoing diarrhea, and/or steatorrhea.
Nutritional therapy includes the administration pancreatic enzyme supplements with or just
before every meal or snack (Option 2). These enzymes are enteric-coated beads designed to
dissolve only in an alkaline environment similar to that of the small intestine. They must not be
mixed with a substance that would cause them to dissolve prior to reaching the jejunum.
Capsule contents may be sprinkled on applesauce, yogurt, or acidic, soft, room-temperature
foods with pH <4.5. Capsules should be swallowed whole and not crushed or chewed; chewing
the capsules could cause irritation of the oral mucosa.
Excessive intake of pancreatic enzymes can result in fibrosing colonopathy (Option 1).
P a g e 2 | 446
,(Option 4) This is a true statement; some children have difficulty taking a whole capsule.
Capsule contents can be sprinkled in acidic substances such as applesauce. Capsules should
not be taken with milk as they can cause it to curdle.
Educational objective:
Pancreatic enzyme supplements are used to aid the absorption of carbohydrates, fats, and
proteins in a child with CF. They are taken with or just before every meal (not as needed);
should be swallowed whole or sprinkled on an acidic food; and should not be crushed or
chewed. They should not be taken with milk. Excessive intake could result in fibrosing
colonopathy.
A A A
The nurse is caring for a pediatric client with end-stage leukemia who is on comfort care and is
unresponsive. The child's parent asks, "How can you tell if my child is in pain?" Which of these
would the nurse describe as signs of discomfort? Select all that apply.
1. Blank facial expression
2. Facial grimacing
3. Groaning
4. Knees bent up near chest
5. Lying still
The nurse will provide teaching on signs that should prompt the parent to administer as-
needed pain medication to the child.
(Option 1) A child who is comfortable will usually have a neutral facial expression. A child in
pain is likely to exhibit grimacing, frowning, or clenching of the jaw, based on the FLACC face
assessment.
P a g e 3 | 446
, (Option 5) A child who is comfortable will be lying quietly. A child who is squirming and
moving is more likely to be in pain, based on the FLACC activity assessment.
Educational objective:
It is difficult to assess for pain in the nonverbal client, particularly if the person is unresponsive
at the end of life. The FLACC scale is an accurate method of assessing pain in the nonverbal
child. This tool should be used to teach parents how to promote comfort for their nonverbal
child
A A A
A 2-month-old infant is brought to the pediatric emergency department due to vomiting and
diarrhea for 4 days. Assessment findings include lethargy, poor feeding, sunken fontanel,
temperature 100.4 F (38 C), heart rate 134/min, and respiratory rate 28/min. Which
prescription from the health care provider would be the priority?
1. Acetaminophen elixir 50 mg by mouth every 6 hours [1%]
2. Intravenous (IV) ampicillin 240 mg every 12 hours [2%] 3.
IV normal saline bolus 20 mL/kg over 1 hour [92%]
4. Obtain a stool culture [4%]
Explanation: Infants and young children have a higher body water percentage than older
children and adults. As a result, they become dehydrated quickly with fluid losses caused by
vomiting and diarrhea. Signs of severe dehydration include lethargy, sunken fontanel, poor
feeding, increased heart rate, and increased respiratory rate.
When an infant's basic vital signs are intact and there is severe dehydration, the priority is to
rehydrate. Normal saline is the fluid of choice for a bolus in an infant. Electrolytes may be
added to the maintenance IV fluids if needed.
(Option 1) A temperature of 100.4 F (38 C) is a mild fever in an infant and may indicate the
need for acetaminophen. However, hydration of the infant takes priority over this action.
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