Peds 5, Hematological Lecture

January 27, 2014

Case #1
An eight-month-old is admitted to the pediatric unit with the following findings:
Fatigues with feedings, pale skin color, Grade II/VI cardiac murmur
Lives in a single-parent home with her mother and grandmother and two
older siblings. The infant has started baby foods recently and has been
given cows milk since 1 month-of-age
Vital Signs-
Temp: 37.5 C axillary
HR: 180 @ rest
RR: 60
BP: 110/P
SaO2: 90%
Nutritional Assessment
Weight Appropriate for age at 50
th
%
Laboratory Results:
Hgb-8 g/dL, Hct-24%,
SIC-60g/dL,
TIBC 450g/dL,
MCV 65m
Admission Orders:
Oral Ferrous Sulfate
Vitamin C supplements
Oxygen by nasal cannula
Iron fortified Formula and Rice Cereal
Questions Related to this Case
What is this childs diagnosis?
How can the childs weight be appropriate for age with this diagnosis?
How are the infants clincal manifestations and iron deficiency anemia
related?
How are the infants laboratory findings significant?
Why is she receiving oxygen
What teaching or other care is required for this infants mother?
Nursing Diagnoses:
Altered growth and development related to inadequate weight gain
Goal
Projected Outcomes
Altered Nutrition: Less than body requirements related to reported
inadequate iron intake (< than RDA); knowledge deficit regarding iron
rich foods
Goal
Projected Outcomes
Proper Administration of Iron Supplement
Give in divided doses for maximum absorption
Give in between meals to increase absorption
Peds 5, Hematological Lecture
January 27, 2014

Administer with fruit juice or with multivitamin preparation Vit C
appears to facilitate the absorption of Iron
Do not administer with milk or antacids these will decrease
absorption
In cultures that drink tea iron should be given with another liquid
Tannin in tea binds with Iron to form an insoluble complex
What color are stools when dose is adequate?
May stain teeth administer to infants via syringe. Older children can
use straw. Brush or wipe off teeth after administration
Only 1 month supply should be kept in home why???

Therapeutic Management to Prevent Iron Deficiency Anemia
Breast milk or commercial formula for the first 12 months of life
Begin iron supplement (Iron fortified formula or rice cereal) for breast fed
full-term infants by 4-6 months and pre-term infants by 2 months-of-age
Administer Ferrous Sulfate @ 2-3mg/Kg to breast fed preterm infants after 2
months-of-age and give Iron fortified cereal to full-term infants after 4
months-of-age
Limit the amount of milk to <1 liter/day


Altered parenting related to inability to form normal maternal
attachment



ANEMIAS CAUSED BY IMPAIRED OR DECREASED PRODUCTION OF
RED BLOOD CELLS
Causes
Bone marrow fails to produce RBCs
Leukemia or other malignancy
Chronic renal disease
Collagen diseases
Hypothyroidism
Aplastic Anemia
-All formed elements of the blood are simultaneously depressed
pancytopenia
-Hypoplastic anemiaprofound depression of RBCs but normal WBCs and
platelets
Etiology
Primary (congenital)
Secondary (acquired)
Diagnostic evaluation
Therapeutic management
Bone marrow transplant
Peds 5, Hematological Lecture
January 27, 2014

Stem cell transplant (HSCT)
Immunosuppressive therapy
Nursing considerations

Sickle Cell Anemia
A hereditary hemoglobinopathy
Ethnicity
Occurs primarily in African-Americans
Occurrence 1 in 375 infants born in United States
1 in 12 have sickle cell trait
Occasionally also in people of Mediterranean descent
Also seen in South American, Arabian, and East Indian descent
Etiology of Sickle Cell
In areas of the world where malaria is common, individuals with sickle cell
trait tend to have a survival advantage over those without the trait


Autosomal recessive disorder
1 in 12 African-Americans are carriers (have sickle cell trait)
If both parents have trait, each offspring will have 1 in 4 likelihood of
having disease
Peds 5, Hematological Lecture
January 27, 2014


Pathophysiology
Partial or complete replacement of normal Hgb with abnormal hemoglobin S
(HgbS)
Hemoglobin in the RBCs takes on an elongated (sickle) shape
Sickled cells are rigid and obstruct capillary blood flow
Microscopic obstructions lead to engorgement and tissue ischemia
Hypoxia occurs and causes sickling
Autosomal, recessive inherited disorder
Valine in 6
th
amino acid position
When hemoglobin deprived of O2
Clumping
Agglutination
Disease not usually seen until 6 months of age because HbF does not sickle
Sickled Hemoglobin
Large tissue infarctions occur
Damaged tissues in organs lead to impaired function
Splenic sequestration
May require splenectomy at early age
Results in decreased immunity
Prognosis
No cure (except possibly bone marrow transplants)
Peds 5, Hematological Lecture
January 27, 2014

Supportive care/prevent sickling episodes
Frequent bacterial infections may occur due to immunocompromise
Bacterial infection is leading cause of death in young children with sickle cell
disease
Strokes in 5% to 10% of children with disease
Result in neurodevelopmental delay, cognitive disabilities
Sickle Cell Crisis
Precipitating factors
Anything that increases the bodys need for oxygen or alters transport
of oxygen
Trauma
Infection, fever
Physical and emotional stress
Increased blood viscosity due to dehydration
Hypoxia
From high altitude, poorly pressurized airplanes,
hypoventilation, vasoconstriction due to hypothermia
Acute exacerbations that vary in severity and frequency
Types
Vaso-occlusive (VOC) thrombotic
AKA painful event/ painful episode
Most common type of crisisvery painful
Stasis of blood with clumping of cells in microcirculation
ischemia infarction
Signsfever, pain, tissue engorgement
Splenic sequestration
Life-threateningdeath can occur within hours
Blood pools in the spleen
Signs
Profound anemia
Hypovolemia
Shock
Aplastic Crises
Diminished production and increased destruction of RBCs
Triggered by viral infection or depletion of folic acid
Signs include profound anemia, pallor
Acute Chest Syndrome
Similar to Pneumonia
VOC or infection results in sickling in the lungs
Chest pain, fever, cough, tachypnea, wheezing, and hypoxia
Repeated episodes may lead to pulmonary hypertension
Diagnosis of Sickle Cell
Cord blood in newborns
Newborn screening done in 43 states
Genetic testing to identify carriers and children who have disease
Peds 5, Hematological Lecture
January 27, 2014

Sickle turbidity test
Quick screening purposes in children older than 6 months
Psychosocial Needs
Coping mechanisms
Support with genetic counseling
Financial needs?
Caregiver role strain
Living with chronic illness in the family
Medical Treatment Of SCD
Transfusion/ hypertransfusion
Prevention of stroke
Monthly transfusions to keep Hbg S at 20-30 %
Risks
Iron overload
Development of antibodies to foreign red cells
Contamination with infective agents hypervisosity of
blood
Transfusion reactions
Time-consuming
Hydroxyurea
Bone Marrow transplant
Of vaso-occlusive crisis
Fluids
O2
Pain Rx
Opioids
Need large doses of Opioids to control pain. Should be
on PCA pump.
DEMEROL (MEPERIDINE) IS DANGEROUS DUE TO
METABOLITES (NORMEPERIDINE) AND SHOULD NOT
BE USED !!!!
Hydromorphone (Dilaudid) and Morphine are
considered the best drugs for pain management
NSAIDS
Ketorolac is used if serum creatinine is okay (<1.5)
No ASA May Acidosis
Nursing Management
Monitor childs growthwatch for failure to thrive
Careful multisystem assessment
Assess pain
Observe for presence of inflammation or possible infection
Maintain HYDRATION (oral or other as needed)
Carefully monitor for signs of shock
PC/ Nursing Diagnoses
Ineffective individual coping r/t stress of chronic illness
Peds 5, Hematological Lecture
January 27, 2014

Body image disturbance r/t physical manifestations of SCD
Impotence
Growth retardation
PC/ Nursing Diagnoses
PC: Hypoxia
? transfusion
Pain r/t ischemia 2 vaso-occlusion
Risk for ineffective management of therapeutic regimen r/t lack of
knowledge, stress of managing chronic illness.
Pneumovax, flu vaccine
Treat infections promptly
Maintain fluid intake
Penicillin prophylaxis
HTSC Transplant

Human tumor stem cell (HTSC)
Potential cure for patients with SCD
Difficult decision for HTSC transplant
Child may face death without the transplant
Preparing the child for transplant places the child at great risk
No rescue procedure if complications follow HTSC transplants
Nursing considerations
Other HSCT Modalities
Umbilical cord blood transplantation
Haploidentical transplants
Nonmyeloablative conditioning regimens
Sibling donor protocols
DO CASE STUDY

Sickle Cell Crisis Case Study and SBAR
4 year old Jared was admitted to room 812 at SLCH 2 days ago with the
diagnosis of Sickle Cell Crisis. He was diagnosed with SCD at the age of 8
months and has been hospitalized with the dx: Sickle Cell Crisis, 4 times since
initial diagnosis.
He takes Hydroxyurea and low dose Penicillin on a daily basis.
Wgt: 13.5 kg (3%-10 %) Hgt: 96 cm (10
th
%) BMI 14.6 (10-25%)
He has been receiving D51/2 NaCl with 2 mEq KCL/100 mls (added after
urine output determined) @ 60 mls/hour
0800 Assessment: HR 100,RR 30, BP 88/50, SaO 92%, Temp 38.4 C Ax
1000 nurse called to room by mother saying somethings happening to
Jared! At the same time the monitor alarms go off. Upon entering the room
nurse the notes that the telemetry monitor shows the following: HR 160 RR
60, SaO 86%. BP is taken and is 102/70 and Temp is 39 C Ax. Upon
auscultation nurse verifies monitor readings.
Peds 5, Hematological Lecture
January 27, 2014

Jared is noted to have increased WOB with intercostal and supraclavicular
retractions noted. Capillary refill 3 secs. He appears to be clutching his
chest and crying.
WHAT IS THE MOST LIKELY DIAGNOSIS??????


Hemophilia: Case Study
KL is a 1 year old male infant who was admitted to your hematology unit
after his mother noted a swelling in his left knee. She stated that KL fell this
afternoon when he was attempting to walk from the couch to the table. She
noted no obvious injury immediately after the fall but when KL woke up from
his nap his left knee was obviously swollen and painful. She reports no
previous episodes of frank bleeding but states that KL seems to bruise easily-
-in fact she points to a bruise on his left hip that she attributes to the rubbing
from his diaper pin. Mrs. L denies any family history of bleeding problems.
Laboratory tests reveal a prolonged PTT, a normal PT, normal bleeding time,
normal platelet count, normal factor VIII antigen and low levels of factor VIII
coagulant level. His medical diagnosis is Hemophilia.

Hemophilia
A group of hereditary bleeding disorders that result from deficiencies of
specific clotting factors

Types of Hemophilia
Hemophilia A
Classic Hemophilia
Deficiency of factor VIII
Accounts for 80% of cases of hemophilia
Occurrence1 in 5000 males
Hemophilia B
Also known as Christmas disease
Caused by deficiency of factor IX
Accounts for 15% of cases of hemophilia
Etiology of Hemophilia A
X-linked recessive trait
Males are affected
Females may be carriers
Degree of bleeding depends on amount of clotting factor and severity of a
given injury
Up to one third of cases have no known family history
In these cases disease is caused by a new mutation

Manifestations
Bleeding tendencies range from mild to severe
Symptoms may not occur until 6 months of age
Peds 5, Hematological Lecture
January 27, 2014

Mobility leads to injuries from falls and accidents
Hemarthrosis
Bleeding into joint spaces of knee, ankle, elbow, leading to impaired
mobility
Ecchymosis
Epistaxis
Bleeding after procedures
Minor trauma, tooth extraction, minor surgeries
Large subcutaneous and intramuscular hemorrhages may occur
Bleeding into neck, chest, mouth may compromise airway

Clinical Therapy
Can be diagnosed through amniocentesis
Genetic testing of family members to identify carriers
Diagnosis on basis of history, labs, and exam
Labslow levels of factor VIII or IX, prolonged PTT
Normalplatelet count, PT, and fibrinogen

Medical Management
DDAVP (1-deamino-8-D-arginine vasopressin AKA -desmopressin)
IV
Causes 2 to 4 times increase in factor VIII activity
Used for mild hemophilia
Replace missing clotting factors
Transfusions
At home with prompt intervention to decrease complications
Following major or minor hemorrhages

Prognosis
Historically, most died by age 5 years
Now those with mild to moderate hemophilia live near-normal lives
Gene therapy for the future
Infuse carrier organisms into patient; these act on target cells to
promote manufacture of deficient clotting factor

Interventions
Close supervision and safe environment
Dental procedures in controlled situation
Shave only with electric razor
Superficial bleedingapply pressure for at least 15 minutes and ice to
vasoconstrict
If significant bleeding occurs, transfuse for factor replacement

Managing Hemarthrosis
During bleeding episodes, elevate and immobilize the joint
Peds 5, Hematological Lecture
January 27, 2014

Ice
Analgesics
ROM after bleeding stops to prevent contractures
PT
Avoid obesity to minimize joint stress

Nursing Care Plan
PC: Hemorrhage
Risk for injury r/t bleeding episodes
Impaired physical mobility r/t bleeding into the joint 2 hemophilia
Immobilize joint while bleeding
Apply ice
After bleeding stopped, gentle, progressive ROM
Risk for ineffective management of therapeutic regimen r/t lack of
knowledge of disease and treatment of hemophilia
Importance of early treatment
Administration of factor replacement
Health promotion
Immunizations
Dental hygiene
Safe (not overprotective) environment
Dietary supplementation
Support groups

Which of the following patients who have hemophilia demonstrates a good
understanding of the disease?
A. Tim has decided to play on an intramural soccer team.
B. Tom wears gloves while doing yard work.
C. Ted takes Excedrin when he has a headache.
D. Todd avoids going to the dentist for routine check-ups.


Idiopathic Thrombocytopenic Purpura (ITP)
An acquired hemorrhagic disorder characterized by
Thrombocytopeniaexcessive destruction of platelets
Purpuradiscoloration caused by petechiae beneath the skin
Normal bone marrow with usual increase in large immature platelets
Peds 5, Hematological Lecture
January 27, 2014


ITP Forms
Acute, self-limiting
Often follows URI or other infection
Chronic (more than 6 months duration)

Diagnostic evaluation
Platelet count is < 20,000/mm - patient will have abnormal bleeding
time, tourniquet test and clot retraction time
Therapeutic management
Primarily supportive
Restrict Activity during initial period of low platelets why??
Treatment may be based on symptoms Prednisone, IV
immunoglobulin (IVIG) and anti-D antibody
Prognosis
Most children have a self-limiting course without major complications
Nursing Care of the Child with ITP
Supportive
Education:
About activities during low platelet count <100,000 mm
Seeking medical evaluation if the child sustains a head or abdominal
injury
Avoid Aspirin, Aspirin Containing Products and NSAIDs


Sample test question
A patient with a history of iron-deficiency anemia who has not taken iron
supplements for several years is experiencing increased fatigue and dizziness. The
nurse would expect the patients laboratory findings to include
A. Hct 38 %
Peds 5, Hematological Lecture
January 27, 2014

B. RBC 4, 500, 000/l
C. Hgb 8.6 g/dl
D. Normal RBC indices

During discharge teaching for the patient with sickle cell anemia, the nurse
instructs the patient to avoid precipitating factors for sickle cell crisis, such as
A. Caffeine & alcohol intake
B. Excessive intake of dietary iron
C. Overexertion and dehydration
D. Altitudes below sea level

Your Pt. who is receiving a unit of packed RBCs is having an acute transfusion rxn.
In what order should the following steps be taken?
A. Notify the blood bank & the health care provider.
B. Treat symptoms per physician order.
C. Stop the transfusion.
D. Complete transfusion reaction report.
E. Maintain a patent IV with NS.
_____, ______, _______, _______, ______