NURSING CARE OF A CHILD WITH

HEMATOLOGIC DISORDER

BLEEDING DISORDERS

HEMOPHILIA

Review of concepts:
Clotting factors
● makes sure that the platelet plug created by the
platelets is stable and won’t be dislodged
Hemophilia inheritance pattern
Coagulation cascade
● A series of steps to establish the platelet plug Types of Hemophilia

A B C

It is the most It is the second It is a mild form of

common type of most common hemophilia.
hemophilia. type of
hemophilia.
(Severe) (Moderate) (Mild)

X-linked Autosomal

It is also known as It was originally Deficiency of

factor VIII named “Christmas factor XI.
deficiency or disease”. Caused
classic hemophilia. by factor IX
deficiency.

NOTE: X-linked recessive traits - males are more

likely to manifest the disease while females
are the carriers
Autosomal recessive traits - can affect both
the males and the females
Illustration of the coagulation cascade process
● Hemophilia - A hereditary bleeding disorder, Clinical manifestations
that result from deficiency of specific clotting ● Children usually do not manifest sxs until after
factors 6 mos. of age
○ Without these clotting factors, there is a ○ The age when the child begins moving
problem with bleeding around, losing teeth, etc.
○ Hemophilia A aka Factor VIII is the most ○ Ex: Child starts crawling → stress on the
common type knees → manifestation of easy bruising on
○ 80% of people with hemophilia the knees
● Spontaneous bleeding
○ Ex: gum bleeding, epistaxis (common but not
severe)

2N8‌‌SAPALO‌‌ʕ•ω•ʔ‌
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● Hemarthrosis
○ Bleeding into joint space
○ Usually on hinged joints
■ Knees, elbows, ankles
■ Too much swelling → blood goes into the
synovial fluid → pain
● Deep tissue hemorrhage
Swelling in joints
○ Life threatening situation
○ Can cause bleeding in the brain and the neck
NOTE: Regardless of the type, they will all have the
→ compromised airway
same manifestations.
● Nosebleeds
● Ecchymosis
Treatment
○ Easy bruising
● PRICES
○ Dark purplish marks on the skin
○ Protect the affected area from further injury
● Hematuria
○ Rest the area through splinting or bandage
○ Fresh blood in the urine
but NEVER a cast
● Hematochezia
○ Ice is applied to promote vasoconstriction →
○ Fresh blood in the stool
lessen the bleeding and swelling
● Life threatening bleeding includes
■ Apply ice for only 15 mins to prevent the
○ Head/intracranial
rebound effect of cold therapy →
○ Neck and throat
vasodilation
○ Abdominal/GI
○ Compression
○ Iliopsoas muscle with decreased hip ROM
○ Elevation above the level of the heart
○ Support through putting a pillow underneath
the elevated body part
● Corticosteroids and NSAIDS
○ Corticosteroids ↓ swelling and inflammation
○ NSAIDS are actually contraindicated
because it can affect the coagulation cascade
but it is given to control the pain
○ NO aspirin!
● Goal to control bleeding by replacing the missing
clotting factor and prevent complication
S/Sx of Hemophilia
● Factor replacement therapy
○ On demand - STAT
○ Prophylaxis - has a scheduled frequency
○ Similar to IV medications
○ Powdered form → reconstituted with a
diluent → infused to the patient
● IV infusions consist of
○ Fresh frozen plasma
■ Thawed to about 40℃ before
administration
Sites of bleeding

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 ■ Usually not done anymore but when it is
infused, it should be thawed
■ Thawed, BUT NOT TO BE PUT IN THE
MICROWAVE!
○ Cryoprecipitate
■ Usually the last resort due to some issues
that would result from it
● Prophylaxis
○ Scheduled infusions of factor 2-3x/week
● DDAVP (Desmopressin acetate)
○ An analog of vasopressin, causes a 2-4 fold
increase in factor VIII
○ Nasal spray
Proper drug administration through nasal spray
● The position of the px should be upright
● Perform nasal care (clean the nose, check for any obstructions)
● Do a test spray first
● Wait 30 to 60 secs before administering the net dose (if more
than 2 doses)
● Make sure to occlude the opposite nostril before administering
the medication
● Complications of treatment
○ Inhibitors /antibody development
■ Amicar (epsilon aminocaproic acid) is
given via oral or IV instead of the
recombinant factor replacement therapy
○ Blood-borne illnesses
■ Hep B and C
■ HIV
Nursing Considerations
● Factor replacement given on time
○ Should be given STAT during emergency
cases to prevent complications
● Lab monitoring as ordered
○ Partial Thromboplastin Time (PTT)
■ The time it takes for the px blood to clot
○ Activated Partial Thromboplastin Time
(aPTT)
■ More reliable because it has an activator
that makes it more reliable and provides
a narrowed reference range
● Increase metabolic states will increase factor
requirements
○ Activities are encouraged but contact sports
are not recommended
● Factor coverage for invasive procedures
○ Procedures may result to excessive bleeding
○ Prophylactic treatment might be ordered
● Document infusion and response to tx
○ Px might show s/sx of an allergic rxn to the
therapy
● Avoid taking temperatures rectally or giving
suppositories
○ These procedures might cause bleeding
● Check BP by cuff as little as possible
○ Might result to bruising if the cuff is too tight
● Use only paper or silk tape for dressings
○ Other types of adhesives might cause
abrasions
● Perform mouth care w/ glycerin swab
○ Toothies are more appropriate for younger
children because these are more gentle to
the gums
● Limit venipunctures
○ Px laboratory procedures must be carefully
planned to limit venipunctures
● Do not give aspirin
○ These are platelet coagulants → px would be
more prone to bleeding
VON WILLEBRAND DISEASE
● Most common form of disorder is autosomal
dominant trait
○ Only one copy of the mutated gene is needed
for their child acquire the disease
● Disease can occur in both males and females
equally
● Factor VIII is also deficient and platelets may or
may not be affected
2N8‌‌SAPALO‌‌ʕ•ω•ʔ‌
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 Hemophilia Hemophilia VW Disease
A B

Bleeding Normal Normal Prolonged

Time

Prothrombin Normal Normal Normal

Time

APTT Prolonged Prolonged Prolonged

Factor VIII Low Normal Low or normal

Inheritance pattern of Von Willebrand Disease
Factor IX Normal Low Normal

Clinical Manifestations VWF Normal Normal Low

● Easy bruising
● Epistaxis DENGUE HEMORRHAGIC FEVER
● Gingival bleeding
● Ecchymosis ● Affects the platelets
● Increased bleeding w/ lacerations or during ● Caused by dengue virus
surgery and dental extractions ● Transmitted by Aedes Aegypti
● Menorrhagia - ↑ menstrual bleeding ○ Distinguished by their tiger appearance,
● GI bleeding white stripes
○ It only transmits the virus but the virus does
Treatment not come from it
● Similar to Hemophilia A ○ Can carry other types of viruses (ex. Zika
● Restore clotting factor and prevent virus)
complications associated with bleeding ○ Usually bites during morning and are usually
○ Infusion of vWB protein concentrate low-lying
○ DDAVP ○ May inhabit places with stagnant water
○ Amicar ● Affects all ages
○ Mostly 10-15 y/o
Nursing Considerations
● Monitor the px during delivery because they
might have a massive bleeding
● Similar to Hemophilia A

Hemophilia Von Willebrand Disease

Clotting factors are affected Clotting factors are affected,

but not the platelets specifically factor VIII, and
platelets may or may not be
affected

X-linked recessive and Autosomal dominant trait

Clinical manifestations of Dengue fever
autosomal recessive trait

Hemarthrosis is present Hemarthrosis is not common

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 Disease Progression Signs and Symptoms
● Depends on the phase that the px is in
● Febrile phase
○ N/V
○ High grade fever for 2-4 days
○ Severe headache
○ Retro-orbital pain
○ Arthralgia (joint pain)
○ Myalgia (muscle pain)
○ Bone pain
○ Maculopapular rash - petechiae or purpura
NOTE: Dengue fever is a.k.a. breakbone fever
because of the bone pain felt by the px who
has the disease.
Petechiae - flat, tiny marks
Purpura - larger than petechiae; bleeding
under the skin; slightly elevated

Petechiae and purpura marks on the skin

Disease progression starts with dengue fever and may progress to a ● Critical Phase
dengue hemorrhagic fever due to a phenomenon called capillary leak
syndrome or plasma leakage. There is increased permeability of the
○ Most px start to recover by this stage but if
capillaries. Plasma would seep out of the capillary, causing accumulation they don’t progress to recovery there could
of plasma in other areas of the body → pleural effusion, cardiac overload, be plasma leakage
ascites. This results in the Hct accumulating in the capillaries →
hemoconcentration. If not further addressed, it can lead to dengue shock ○ Begins after defervescence (24-48 hours)
syndrome. ○ Plasma leakage
■ A.k.a. Capillary leak syndrome
NOTE: Dengue fever has 4 serotypes or 4 strains.
■ Severe dengue
Once a px has already been infected with
○ Reappearance of fever
one serotype, they would be immune to that
■ Saddleback fever
serotype but not to other serotypes. And when they
○ Could lead to shock
are infected again for the 2nd time, the more severe
● Convalescent stage
it would be.
○ Hemodynamic stability
○ Normal hct and wbc
○ Watch out for thrombocytopenia
■ Mechanism is unknown

2N8‌‌SAPALO‌‌ʕ•ω•ʔ‌
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 ■ Virus has ↑ affinity to platelets → attach
to the platelets → replicate in the
platelets → attacks other platelets →
Thrombocytopenia
Treatment
● Supportive therapy
● IV fluids
○ Isotonic fluids or colloids to prevent
dehydration
○ Monitor px for circulatory overload due to
plasma leakage
● Monitor BP
○ Narrowed pulse pressure ( ↓ 20 mmHg)
○ Monitor sx of internal hemorrhage or shock
● No aspirin for fever
○ It is an anticoagulant → ↑ risk for bleeding
● Plasma expander and platelet concentrate
○ Platelet concentrate is administered when it
falls ↓ 20,000
● O2 PRN
○ Administered when there is already sx of
shock or plasma leakage such as pleural
effusion
● DAT Diet as tolerated (except dark colored
food)
○ Colored food might mask the bleeding
-Ma'am Magpantay’s Clarificatory-
● Blood is drawn everyday to monitor the px blood
● Monitor the px vital signs frequently (usually
every 15 min, 30 min, or every hour)
● Best management is prevention since it is
transmitted through a vector
● Prevent it through teaching the px in the
community regarding hygiene and prevent any
stagnant water
○ Common sites: alulod, tires, surroundings of
their house, water contained in a pail for a
long time (should be used and changed
regularly)
● Remind the parents of the px that this is not
transmissible from human to human
○ They may have been bitten by the same type
of mosquito
● Tourniquet test
○ You use a simple tourniquet or a bp cuff
○ There should be (in a one inch square) around
20 petechiae marks to be able to say that the
px is positive for dengue but the gold
standard is to check the cbc to check the
platelet count or the serum test
● Prevent bleeding in dengue
○ Sx of bleeding:
■ Epistaxis
■ Gum bleeding
■ Nose bleeding
■ Abdominal pain
■ Check for hematochezia or melena
● Early signs: increased HR
● Late signs: hypotension or ↓ BP
● Health teachings
○ Use soft bristled brush to prevent bleeding
○ No dark colored foods because these can be
mistaken as blood
■ Meats, beef, burgers
■ Pusit
■ Encourage them to eat tinola except the
leafy vegetables bc it’s dark
● Manage fever
○ TSB
○ Antipyretics PRN

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 ○ Eosinophils - degranulation → parasitic
WBC DISORDERS
death
● Never matures in Acute Myeloid Leukemia
Review of concepts: ○ Stays as myeloblasts

ACUTE LYMPHOCYTIC LEUKEMIA

● Cancer of blood forming cells

● Involves rapid proliferation of lymphoblasts
(immature lymphocytes)
● 2-6 y/o
○ Preschoolers and toddlers
○ Have good prognosis - responsive to
treatment
Agranulocytes
○ 80% of childhood leukemia is attributed to
● Lymphocytes
ALL
● Matured T lymphocytes, B lymphocytes
■ 95% would have remissions once therapy
○ Fights abnormal cells through releasing
has started
chemicals → cell death
■ *remission - absence of symptoms or
○ Important in fighting cancer cells
clinical manifestations
● Would only be able to function if they are
● Pancytopenia
matured
○ ↓ in all blood components
● Never matures in Acute Lymphocytic Leukemia
○ Leukopenia + Thrombocytopenia + Anemia
○ This causes easy proliferation of cancer cells
○ Stays as lymphoblasts
Diagnosis
● CBC
Granulocytes
○ ↓ blood components
● Matured basophils , eosinophils, and neutrophils
● Peripheral blood smear
○ Basophils - degranulation → releases
○ Results would show a lot of immature cells in
histamine → counteracts the effect of the
the blood
allergic reaction
● Bone marrow biopsy
○ Neutrophils - phagocytic; eats the pathogen

2N8‌‌SAPALO‌‌ʕ•ω•ʔ‌
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 ○ Would also reveal a lot of immature cells in ○ Fluid accumulation in the lungs → pleural
the bone marrow effusion
○ Site of aspiration is the iliac crest
○ Px is sedated
● Lumbar puncture
○ Done when there is suspected CNS
involvement
○ Site of aspiration is between L3 and L4
○ After the procedure, advise px to remain flat
to avoid csf leakage and spinal headache

Signs and symptoms of Lymphoblastic Leukemia

Management

Px position in lumbar puncture procedure

● Chemotherapy
○ Curing the patient
○ End goal: Complete remission for 5
consecutive years
Px position in bone marrow biopsy procedure 1. Induction Phase
■ Lasts for 4 weeks
Signs and Symptoms ■ Initiated once the px is diagnosed with
● Fever ALL
● Easy bruising - ecchymosis ■ Watch out for ↑ of uric acid due to the
● Easily fatigability rapid cell death
● Joint swelling ● Allopurinol is given - ↓ uric acid in the
● Bone and joint pain bloodstream and prevents kidney
● Lymphadenopathy damage
○ Lymph nodes are palpable ■ Goal: to have remission
● Skin rashes 2. CNS Prophylactic phase
● Severe cases may present ■ A.k.a. Sanctuary phase
○ CNS involvement → Seizures
○ ↑ of immature cells in the lungs → difficulty in
breathing
2N8‌‌SAPALO‌‌ʕ•ω•ʔ‌
Goodluck( •̀ᄇ• ́)‫✧ﻭ‬
 ■ Chemotherapeutic drugs are
administered intrathecally (via the spinal
canal, or via the subarachnoid space)
● Most commonly the Methotrexate
■ Ensures that the CNS is free from
leukemic cells
3. Consolidation Phase
■ Lasts for 28 weeks
■ Goal: eradicate leukemic cells that are
still in the bloodstream
Intensification Phase
(From the book)
■ Another set of chemotherapeutic agents
are given
■ Normal cells are protected through
Leucovorin
4. Maintenance Phase
■ Longest phase (2-3 years)
■ Prevent relapse
■ Goal: No regrowth of leukemic cells; no
presence of leukemic cells in the
bloodstream; complete eradication of
leukemic cells
NOTE: Different books may give different
terminologies for the phases of treatment
but it will all start from the induction phase
and end in the maintenance phase.
- If there would be relapse while treatment is
ongoing, the px would be a candidate for
bone marrow transplant.
Nursing Considerations
● Relieve pain
○ Tylenol, Paracetamol, Acetaminophen
● Prevent infection
○ ↓ blood cells + chemotherapy → ↑ risk for
infection
○ Implement reverse isolation
○ Px would wear mask, observe handwashing
and the nurse handling the px shouldn’t
handle a different px (if possible) or an
infectious px
○ Px diet: Neutropenic diet
■ No raw foods
○ No fresh flowers
○ Visitors should be limited
● Precaution in chemotherapy
○ Nausea
○ Vomiting
○ Anorexia - small frequent feeding
○ Neuropathy - encourage ambulation
○ Mucosal ulceration
○ Hemorrhagic cystitis
○ Moon face
○ Mood changes
NOTE: Alopecia is also a side effect of
chemotherapy. We should explain once the
px asks if alopecia is a permanent side effect
that this is just temporary. Hair would regrow
once treatment has been discontinued.
-Ma'am Magpantay’s Clarificatory-
● The blood cells remain immature
○ Cannot function as the soldiers of the body
→ px will be at high risk for infection
● Protect the px from infection
○ Avoid crowds
○ Perform handwashing
○ Prevent fresh fruits of fresh vegetable
○ Their diet should be well cooked
○ If they have a roommate in the hospital, they
should be non infectious
■ Px with pneumonia, tonsillitis shouldnt
be put in the same room as a px with
leukemia
■ Ideally they should be in reverse isolation
○ Let the px wear a mask
● Activities of the child should be managed
○ Bed rest
○ Tolerated activities
● Diet
○ Should be easy to chew, soft
○ Shouldn't make the px exhausted from eating
● Remember the normal ranges of blood
components
○ Remember them by heart ♡
ACUTE MYELOID LEUKEMIA
2N8‌‌SAPALO‌‌ʕ•ω•ʔ‌
Goodluck( •̀ᄇ• ́)‫✧ﻭ‬
● Granulocytes remain as myeloblasts
● Only 20% of childhood leukemias would involve
the myeloid
○ Commonly affects adults
○ Commonly affects adolescents in pediatric px
● Poor prognosis
○ Life expectancy: 5 - 10 years
● Manifestation is similar to ALL
● Has lesser incidents of lymphadenopathy, bone
and joint pain because the bone marrow is the
one mainly affected

AML ALL

Age Commonly Children

adults

Lymphadenopathy Less common More common

Hepatosplenomegaly Less common More common

Bone and Joint Pain Less common More common

Gum hypertrophy More common Less common

Blast cells Myeloblast Lymphoblast

Treatment
● Chemotherapy
○ Would also start with induction phase but no
maintenance phase
○ Ideally, when there is remission → proceed to
bone marrow transplant → complete
remission → ↑ life expectancy
● Bone marrow transplant

Bone marrow transplant

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Goodluck( •̀ᄇ• ́)‫✧ﻭ‬