Peds Final Exam: Ch 47 Hematologic
acute sequestration crisis
A sudden enlargement of the spleen that can be life-threatening; Blood volume pooling in the spleen, hypovolemic shock (often children aged 6mo to 4yr); Manifestations: Decreased hemoglobin level, acutely ill-looking child, pallor, irritability, tachycardia, enlarged spleen,
Leg ulcers in the patient with sickle cell anemia
(older children and adults) lack of blood flow, painful and very slow healing
medication for sickle cell anemia: Hydroxyurea
(usually used to treat cancer) drug creates fetal hemoglobin which decreases sickling episodes and help with anemia (decreasing the need for blood transfusions), medication causes a drop in white blood cells, teach patients good hand hygiene and to avoid infection
What vaccines are reccomended for a child with sickle cell disease due to the child's weakened immune system?
-Pneumococcal polyvalent vaccine (PCV13) is routinely recommended for children at ages 2, 4, 6 and 12-15 months of age. -Routine vaccine against H. influenzae -Immunization against hepatitis B. -Influenza vaccine annually -Immunization against meningococcal disease after 2 years of age
which population of people is most likely to be affected by sickle cell anemia?
African Americans, this is because (according to CDC) 1 in 12 African Americans have sickle cell trait so it can easily be passed on to offspring (other populations affected: middle eastern, Asian, Caribbean, Indian and eastern Mediterranean)
Sickle cell anemia is part of screening in most hospitals: Dithionite (sickledex) (-screening test)
A blood test that assesses for abnormal hemoglobins, it doesn't differentiate between sickle cell anemia and sickle cell trait so a hemoglobin electrophoresis will need to be done to confirm diagnosis
Vaso-occlusive Crisis: Priapism
Persistent erection of the penis, Occurs if penile blood flow becomes obstructed, painful treatment: Analgesics; hydration Avoid hot and cold packs If prolonged, transfusion therapy
what are life-threatening complications and long term complications of hemophilia?
life threatening: bleeding within the skull (intra-cranial hemorrhage) and bleeding into the soft tissue around important organs long-term: chronic, debilitating joint disease may develop
Which factors below can increase the patient's risk for developing sickle cell crisis? Select all that apply: A. Shellfish B. Infection C. Dehydration D. Hypoxia E. Low altitudes F. Hemorrhage G. Strenuous exercise
B, C, D, F and G. (Sickle cell crisis can occur when the body experiences low amounts of oxygen in the body. Therefore, infection, dehydration, hypoxia, HIGH (not low) altitudes, hemorrhage, or strenuous exercise can lead to a sickle cell crisis.)
Q 4: Because of the risks associated with administration of antihemophilic factor (recombinant), the nurse should teach the child's family to recognize and immediately report which problem? A) Yellowing of the skin B) Constipation C) Abdominal distention D) Hives
D) Hives *Administration of antihemolytic factor is a biosynthetic preparation of factor VIII that carries the risk of severe allergic reaction. Signs include hives, difficulty breathing, tachycardia, chills and fever.
Q 3: A child with hemophilia presents with a burning sensation in the knee and reluctance to move the body part. The nurse collaborates with the care team to provide factor replacement and: A) Administer an aspirin-containing compound. B) Institute rest, ice, compression and elevation (RICE). C) Begin physical therapy with active range of motion. D) Initiate skin traction.
B) Institute rest, ice, compression and elevation (RICE). *The child is displaying symptoms of bleeding in the joint and factor replacement is indicated. The RICE method is used additionally as a supportive measure to help control the bleeding.
Q 2: A Diagnosis of hemophilia A is confirmed in an infant. Which instructions should the nurse provide the parents as the infant becomes more mobile and starts to crawl? A)Administer one-half of a children's aspirin for a temperature higher than 101 F (38.3 C). B) Sew thick padding into the elbows and knees of the child's clothing. C) Check the color of the child's urine every day. D) Expect the eruption of the primary teeth to produce moderate to severe bleeding.
B) Sew thick padding into the elbows and knees of the child's clothing. *As the hemophilic infant begins to acquire motor skills, falls and bumps increase that risk of bleeding. Such injuries can be minimized by padding vulnerable joints.
Q5: The mother tells the nurse she will be afraid to allow her child with hemophilia to participate in sports because of the danger of injury and bleeding. After explaining that physical fitness is important for children with hemophilia, which activity should the nurse suggest as ideal? A) Snow skiing B) Swimming C) Basketball D) Gymnastics
B) Swimming *Swimming is an ideal activity for a child with hemophilia because it is a noncontact sport. Many noncontact sports and physical activities that do not place excessive strain on joints are also appropriate.
You're providing education to a patient with sickle cell anemia who is taking Hydroxyurea. You will make it priority to tell the patient to? A. Consume foods high in calcium and potassium B. Avoid sick people and maintain strict hand hygiene C. Take this medication with at least 8 oz of water D. Monitor your blood glucose level daily
B. (This medication can lower the white blood cell count. The nurse should make it priority to tell the patient to avoid infection by avoiding sick people and performing hand hygiene regularly.)
Which type of hemoglobin is present in a patient who has sickle cell anemia? A. Hemoglobin AA B. Hemoglobin AS C. Hemoglobin SS C. Hemoglobin AC
C. Hemoglobin SS
Q 1:What is the most appropriate method to use when drawing blood from a child with hemophilia? A) Use finger punctures for lab draws. B) Prepare to administer platelets. C) Apply heat to the extremity before venipunctures. D) Schedule all labs to be drawn at one time.
D) Schedule all labs to be drawn at one time. *Coordinating labs to minimize stick reduces trauma and the risk of bleeding.
Acute Chest Syndrome
manifestations: chest pain, cough, fever, low o2 sat, new chest x-ray infiltrate; VERY DEADLY, monitor respiratory status. happens due to infection like pneumonia or embolism or sickled cells blocking perfusion to lung tissue
treatment of acute sequestration crisis
Emergency treatment to restore circulating blood volume with crystalloid and colloid (blood) infusion Long-term transfusion therapy if recurrent Eventual splenectomy in cases of persistent recurrence
Which of the following is NOT a treatment for this condition? A. Blood transfusion B. Stem cell transplant C. Intravenous fluids D. Iron supplements E. Antibiotics F. Morphine
D. (Iron supplements are not prescribed because this type of anemia is not caused by low iron levels, and patients who take iron supplements with sickle cell disease are at risk for building up too much iron in the body, which will damage the organs.)
During an outpatient well visit with a patient who has sickle cell anemia, you make it PRIORITY to assess the patient's? A. hemoglobin A1C level B. heart rate C. reflexes D. vaccination history
D. (They are at risk for infection because of spleen compromise. Vaccination history is very important. The patient should be up-to-date with the flu, pneumococcal, and meningococcal vaccines.)
How are cases of hemophilia A normally seen?
Hemophilia A can be mild, moderate, or severe. 50 to 60 percent of patients with hemophilia A have the severe form.
Treatment of acute chest syndrome
IV hydration (1-1½ times maintenance), antibiotics, oxygen, blood transfusion, analgesics
Nursing interventions Hemophilia
monitor injury for bleeding, measure circumference of an injured joint, apply gentle pressure, administer factor replacement as ordered and monitor factor levels, avoid RECTAL temperatures (bleeding)
Vaso-Occlusive Crisis: Stroke
Manifestations Hemiparesis or monoparesis, aphasia/dysphasia, seizures, alteration in level of consciousness, vomiting, vision changes, ataxia, headache; Treament: Long-term RBC transfusion therapy or erythrocytapheresis and possibly chelation therapy, Sometimes requires extensive rehabilitation
Nursing Interventions (sickle cell anemia) Pain Control
Opioid (Morphine) and NSAIDs. Use in combination for painful crisis. Opioid: provide systemic relief, NSAIDs act locally to decrease inflammation to the site of vaso-occlusion. Guided imagery . (opioids given around the clock)
Treatment of vaso-occlusive crisis
Oral analgesics initially and, if ineffective, IV opioids, usually morphine (intermittent or continuous infusion), PO or IV NSAIDs, PO and IV hydration, incentive spirometry use (10 breaths q2hr when awake), assess pain, nonpharmacologic pain management
Prophylactic therapy for Sickle cell disease
Prophylactic daily: penicillin V therapy (given twice a day) is recommended for all children with suspected or actual diagnosis by age 2 months and is continued until at least age 5 years.
why does a person with sickle cell disease experience anemia?
RBCs with hemoglobin S are very weak and easily rupture. They only live 20 days while normal RBCs live 120 days; the bone marrow cannot keep up with production of RBCs so anemia occurs
factors that cause sickle cell crisis "SICKLE"
S- significant blood loss, trauma, surgery I- Illness, Infection C- Climbing or flying high altitudes K- keeping continued stress (physical/mental) L- Low fluid intake (dehydration) E- Elevated temperature (fever, strenous exercise) Extreme temp changes -(cold weather, swimming in cold water)
How may a baby present who the nurse suspects has sickle cell anemia?
The baby may be suddenly extremely fussy, crying like it's in pain, feverish, has unusual swelling in the hands and feet called dactylitis (hand-foot syndrome), the baby's cells are beginning to sickle and cause extreme pain, sickling decreases blood flow to the extremities and organs, parents describe change in baby's behavior
which organs are most affected by sickle cell disease?
The brain, lungs, and the spleen, the vessels are also affected (increase risk of stroke)
Aplastic crisis in sickle cell disease
The halt of RBC production or rapid destruction of RBCs, bone marrow cannot keep up with producing RBCs so the patient will cease in making RBCs, patient will be anemic Manifestations: Pallor, lethargy, headache, fainting Treatment: RBC transfusions and treatment of symptoms (associated with viral infection such as 5ths disease)
Why is a patient with sickle cell disease at risk for infections, especially pneumonia?
The spleen is enlarged, full of blood, and not functioning to filter out foreign invaders putting the child at an increased risk for infection
Nursing interventions: Sickling of RBCs occuring
focus hydration, oxygen, pain, at risk for infection, monitor respiratory status, neuro checks, watch for acute chest syndrome, prevention of future crisis episodes, medications, blood transfusions
Nursing interventions Sickle cell anemia
around the clock opiod pain control, oxygen therapy, increase fluids, prophylactic penicillin, increased hydration, blood transfusions, bed rest, elevate extremities, remove restrictive clothing (can cause sickling), warm compresses not cold (cold causes sickling), folic acid supplements, Not iron, these kids can have iron toxicity
Hemophilia A
associated with deficiency of coagulation factor VIII
Where does bleeding often develop in children with severe hemophilia?
bleeding develops in the muscles, and joints (hemarthrosis), especially in the knees
signs and symptoms of hemophilia
bleeding occurs after surgery or trauma in all children with hemophilia, in children with severe disease bleeding occurs spontaneously and for no reason, bruises easily, episodes of nose bleeds, some blood in urine, bleeding with: loss of baby teeth, injections and minor lacerations
Manifestations of sickle cell
chronic hemolytic anemia, pallor, jaundice, fatigue, cholelithiasis (formation of gallstones), delayed growth and puberty, avascular necrosis of the hips and shoulders, renal dysfunction and retinopathy.
Signs and symptoms of sickle cell anemia
dactylitis, pain (severe, back, joints, chest etc), anemia, fussiness, tired, tachycardia, jaundice, or pale skin, delayed growth, SOB, gallstones, stroke, eye problems, abnormal clotting, acute chest syndrome, leg ulcers
Hemophilia B
deficiency of coagulation factor IX, symptoms similar to hemophilia A
Hemophilia C
deficiency of factor XI, results in only mild bleeding tendencies
Prophylactic therapy of hemophilia (prevents joint problems)
demopressin acetate intranasal spray: a vasoconstrictor that stops bleeding; used in mild hemophilia
medical treatment of hemophilia
depends on the person, aims to prevent excessively bleeding and tissue damage by supplying the body with CLOTTING FACTORS, recombinant antihemophilic factor (not from human plasma but instead an isolated gene, reconstituted with sterile water b4 given IV) which eliminates the risk of virus transmission from blood products, children avoid activity that induces bleeding
diagnostic work up of hemophilia
determining prothrombin time (PT), partial thromboplastin time, bleeding time, fibrogen level, platelet count, qauntitative immunoelectrophoretic assay, factor VII and IX assays
Nursing Interventions (sickle cell anemia) IV and Oral fluids
dilutes the blood and helps blood flow to the kidneys to enhance kidney function
vaso-occlusive crisis (most common and most common reason for hospitalization)
fever, pain, bone and joint pain, edema and the hand and foot (seen in babies), RBCs are sticking together and blocking blood flow which can lead to decreased circulation to organs; infarction, stroke and renal issues can occur, priapism, acute chest syndrome can occur
What is the RBC with the hemoglobin S like?
hemoglobin S causes the RBC to be stiff, sticky, inflexible and form a sickle shape (half moon), (especially when there is a low amount of oxygen in the body which causes a sickling episode); sticky cells cause circulation problems
Hemophilia
hereditary bleeding disorder from deficiency of clotting factor, the ability of the blood to clot is severely reduced, sufferer bleeds severely from even a slight injury
what is the biggest health concern for a patient with hemophilia?
if the patient has a severe deficiency of clotting factor protein there is concern for deep bleeding inside of the body; internal bleeding damages organs and can be life threatening
In vaso-occlusive crisis, pain is precipitated by
infection, cold, stress acidosis, local or generalized hypoxia
Dactylitis (hand-foot syndrome)
inflammation of the fingers and/or toes, pain and warmth in area, early sign seen in babies at 6 months old; during crisis can be seen in children 6 months to 4 years old
How is hemophilia inherited?
it is an X-linked autosomal recessive disorder, carrier females pass it on to males, females are rarely born with the disorder, the gene can be silently passed on for generations, but there is usually a family history
How is sickle cell diagnosed?
it is diagnosed around 4-6 months of age, this is because prior to 6 months the baby has fetal hemoglobin, at this point the baby's hemoglobin S is starting to take over
where are common sites of deep bleeding in a patient with hemophilia?
knees, ankles, and elbows
medication for hemophilia A or B: Aminocaproic Acid (Amicar) or tranexamix acid (cyklokapron)
oral medications that stablize oral clots and can also sometimes stop nosebleeds
Why do the parents of a child with sickle cell disease normally not show any signs or symptoms?
parents have sickle cell trait (which means they're carriers), they have one normal hemoglobin gene and one hemoglobin S gene. People with sickle cell trait rarely show symptoms because they have enough normal hemoglobin to prevent sickling
recurrent bleeding in children with hemophilia
recurrent bleeding commonly occurs in the same joint in severely affected children, joint will be swollen, painful, bleeding, and stiff
RICE
rest, ice, compression, elevation; low temperatures impair platelet function so cold should be applied for no more than 15 to 20 minutes at a time
what is the most common and severe form of sickle cell disease?
sickle cell anemia
What is sickle cell anemia?
sickle cell anemia is a genetic blood disorder that effects the shape of the RBCs, the person has abnormal hemoglobin S (opposed to normal hemoglobin A), this type of hemoglobin is very sensitive to changes in oxygen which causes the RBCs to change their shape, which can lead to sickle cell crisis
why would a patient with sickle cell anemia experience gallstones?
sickled RBCs are constantly breaking down and releasing bilirubin, which leads to gallstones because there is too much bilirubin for the gallbladder to manage (chest pain, heart burn, pain in shoulder)
diagnosis of hemophilia
sometimes diagnosed after circumcision, because most common sites of bleeding are the muscles and joints, diagnosis can be delayed until toddler years when the child becomes more active, most children have had an episode of persistent bleeding from a mild laceration by preschool years
cure for sickle cell anemia
stem cell transplant, RARELY DONE because there needs to be a matched donor, bone marrow would make healthy Red Blood cells
family teaching hemophilia
teach family the need for safety precautions, correct response to injury, medication administration methods, importance of following treatment plan, teach how to promptly recognize and respond to bleeding, teach family how to reconstitute and give infusions of clotting factors by IV (depends on type of venous access)
when does a patient with sickle cell show symptoms of the disease?
the patient doesn't usually show symptoms until they are in a crisis episode
Normally the spleen filter bacteria in the blood, why would this be hindered in sickle cell anemia?
the spleen of a child with sickle cell does not function properly much beyond age 5 years because of repeated microvascular occlusion and damage.
is there a cure to hemophilia?
there is no cure to hemophillia and it is a life long disorder
how do people get sickle cell anemia?
they're born with it, it is autosomal recessive and the person had to receive a hemoglobin S from each parent to have the disease, offspring have a 25 percent chance of having disease if both parents are carriers
ways to prevent sickle cell crisis
vaccines, no high altitudes (climbing, flying), prevent infection, hydration, decrease stress, no smoking, don't over do exercise, hand hygiene, avoid extreme weather
types of sickle cell crisis:
vaso-occlusive, acute sequestration, or aplastic.
Why can vision problems occur in sickle cell disease?
vision issues due to the blockage of blood flow to eye vessels from sickled cells, eye checks regularly
What happens when a person is experiencing a sickle cell crisis?
when the RBCs sickle due to a lower oxygen level, the abnormal shape causes the RBCs to become stuck in vessels (blocking blood flow) so organs and tissues suffer, which leads to symptoms and pain