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NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC

Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN


■ If one is affected then the other one will
BLEEDING DISORDERS
compensate.
ANATOMY & PHYSIOLOGY REVIEW ■ Ex. Even though we have a decrease of factor 8
Platelets that amount is sufficient to control the bleeding or
● responsible in creating a seal when blood vessels are damaged to finish or complete the entire coagulation
○ Damaged blood vessels → send signals to the cascade
bloodstream → attract the platelets to go there → ● Hereditary bleeding disorder that result from deficiency of specific
create a seal clotting factors
● Hemophilia A aka Factor VIII is most common type (and most severe)
Clotting Factors ○ The missing/deficient clotting factor is Factor 8
● Important to make sure that the seal created by platelets would ○ Also known as Classic hemophilia
be stable and not dislodged ○ 80% of people with hemophilia
● Proteins present in the bloodstream that would prevent the
platelet plug to be dislodged, therefore, the bleeding that would
occur in our patient would be controlled or would be stopped
● Involved in bleeding disorders; different from platelets
● Without these clotting factors (such as in hemophilia) there would
be a problem with the controlling of bleeding

Coagulation Cascade
● Series of steps that are activated so that there will be stabilization
of the platelet plug
● When there is damage to blood vessels → chemical signals sent
by the bloodstream → first clotting factor that would be activated
is the factor 12. Like lock and key mechanism that would activate TYPES OF HEMOPHILIA
the factor 11 → factor 9 → factor 8 → factor 10 → factor 5 →
A B C
thrombin → fibrinogen
○ The fibrinogen, when it is divided into several It is the most common It is the second most It is a mild form of
components, it becomes fibrin type of hemophilia common type of hemophilia
○ Fibrin is responsible for making the platelet plug stable (severe) hemophilia (moderate) (mild)
(added layer of security for our platelet plug)
● If one of the clotting factors would decrease or would be missing X-linked recessive X-linked recessive Autosomal recessive
on the cascade then there is no fibrin that would result in that disorder (only males will disorder (only males disorder (need 2 copies
cascade. manifest s/sx) will manifest s/sx) of the gene for disorder
○ Sometimes, on the thrombin level, it would already to manifest; affects both
stop → nothing will make that plug stable causing the males & females)
uncontrollable bleeding in hemophilia It is also known as It was originally named Deficiency of factor XI
factor VIII deficiency or “Christmas disease”.
classic hemophilia Caused by factor IX
deficiency
80% of the cases would
be the classic The first person to have
hemophilia this disease is Stephen
Christmas → christmas
disease

NOTE: All types would have the same manifestations

Hemophilia
CLINICAL MANIFESTATIONS
● Has a missing clotting factor depending on the type of hemophilia
● Children usually do not manifest s/sx until after 6 months of age (begin
● X-linked recessive disorder
moving around, losing teeth)
○ X chromosome would be affected that would contain the
○ Because at 6 months of age, the child begins to crawl, walk,
mutated gene → only the males will have the manifestation
climb stairs
of the disease while the females are the carrier
■ Ex. in crawling, the knees are stressed → easy
○ Only males would have manifestations because they only
bruising on the knees.
have one X chromosome (same with baldness because it is
■ Ex. mild bump of the elbow on the table → easy
also x-linked recessive disorder)
bruising on the elbow or sometimes swelling
■ Has a significant decrease of clotting factors that
● Spontaneous bleeding
would affect the coagulation cascade resulting to
uncontrollable bleeding ○ In some countries or even in the PH, early circumcision is
○ Females are more of a carrier because they have 2 X being practiced. So as soon as the baby boy is born →
chromosomes

1 SACRAMENTO | SANTOS, C. | SANTOS, Z | SAPALO | SAUL | SERRANO| SUMABAT


NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
circumcision → too much bleeding on the circumcised part → NOTE: Life threatening because it is painful and these are major organs (like
prompt the report to the primary caregiver the brain & neck) that’s why when this happens, the patient really need to do
○ Ex. simple activities like brushing of the teeth that would recombinant factor infusion (mainstay treatment for patient with hemophilia)
also result to uncontrollable bleeding of the gums
■ Bleeding will stop temporarily and it would bleed TREATMENT
profusely again ● PRICES
● Hemarthrosis (bleeding into joint space) ○ Protection - protect the affected area from further injury
○ Mostly the hinge joints ○ Rest - apply splint to immobilize the area or bandage
■ Elbows, knees, ankles ■ NEVER a cast as it may cause bruising because of
■ These hinge joints has a soft spots and not that its tightness and we may not be able to see
what’s happening under it
protected on the sides
○ Ice - to promote vasoconstriction that would further prevent
■ If it swells → bleeding go to the synovial fluid →
the bleeding episode
swelling → painful
■ Should only be done under 15 mins because
longer periods may cause a rebound effect of cold
therapy (vasodilation)
■ Book: 5 minutes ice, 10 minutes rest, 5 minutes
ice, 10 minutes rest, and so on
○ Compression - could be manual compression to stop the
● Deep tissue hemorrhage bleeding
○ That would involve the joints and muscle ○ Elevate - above the level of the heart to decrease the
○ Life threatening situation pressure → decrease bleeding
○ Affects the brain → bleeding in the brain, in the neck that ○ Support - ex. when you elevate the arm/legs, you may put a
would compromise the airway pillow underneath to make the patient comfortable
● Nosebleeds (Epistaxis) ● Corticosteroids and NSAIDs
○ But not severe compared to other bleeding disorders) ○ Corticosteroids decrease the swelling or inflammation
● Easy bruising (ecchymosis) especially if there is already hemarthrosis
○ There would be dark purplish marks on the body that would ○ NSAIDs are actually contraindicated as these may affect the
not go away coagulation cascade but there is no choice but to control the
○ Sign of bleeding that would alarm the parent & should be pain
reported to healthcare practitioners ○ Do NOT give aspirin because it is an anticoagulant
● Hematuria ○ Goal to control bleeding by replacing the missing clotting
○ Fresh blood in the urine factor and prevent complications
○ Or Hematochezia - fresh blood in the stool ● Factor Replacement Therapy
● Life-threatening bleeding includes: ○ Depends on the type
○ Head/intracranial ○ Ex. Hemophilia A - recombinant factor VIII will be replaced
■ Internal bleeding ○ Not a blood transfusion; similar to the usual IV medication
○ Neck and throat ○ Powder form reconstituted with a diluent and infused to the
■ Bleeding in neck compromises the airway patient
■ Considered as an emergency ○ Patients may infuse the medication themselves at home as
○ Abdominal/GI this is a lifelong disorder
■ Evidenced by hematochezia ○ Nursing Responsibility: Teach them how to administer their
○ Iliopsoas muscle with decreased hip ROM medications at home to lessen the hospitalization
■ Illopsoas muscle connects the femur to the lesser ○ But for toddlers and preschoolers, some moms are hesitant
trochanter to do this that’s why they still go on an outpatient basis for
■ The pain would be at the hip area → decreased this recombinant factor
movement or range of motion of the hips ○ 2 Therapies involved in factor replacement:
a. On demand
■ Ex. the patient is already bleeding then we
have to give the recombinant factor ASAP
b. Prophylaxis
■ Preventing further occurrence of massive
bleeding
■ Scheduled infusions of factor 2-3 times/week
○ It depends on the case of the patient whether we are going
to give on demand (ASAP/STAT) or prophylaxis (lifelong
treatment or there is already a time frame when we are
going to give it for example 2-3x a week)

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NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
● IV infusions consist of: NURSING CONSIDERATIONS
○ Fresh frozen plasma ● Factor replacement given on time
■ Infusion of choice for traumatic injury that ○ There is a certain schedule to give it
would result to massive bleeding ○ But for emergency cases, give it ASAP to prevent
○ Cryoprecipitate complications of bleeding in patient with hemophilia
■ According to Ma’am’s research, it is the last ● Lab monitoring as ordered
resort because of the certain issues and ○ Best laboratory test for hemophilia is PTT (partial
concern that would result to cryoprecipitate thromboplastin time) or aPTT
○ NOTE: The difference of fresh frozen plasma to ○ aPTT is more reliable than PTT because we add activator on
cryoprecipitate is we thaw the fresh frozen plasma to about that component so that there would be a narrow reference
40ºC before we administer it to the patient. range → more reliable when it comes to the detection of
● DDAVP (Desmopressin acetate) hemophilia
○ Deamino D-arginine Vasopressin ○ aPTT is prolonged for patient with hemophilia
○ An analog of vasopressin, causes a 2-4 fold increase in factor ● Increase metabolic state will increase factor requirements
VIII ○ Promotion of rest → decrease in metabolic rate → decrease
○ Stimulate the endothelial factors to release factor VIII in the in clotting factor requirements
bloodstream ○ Limit activities specially contact sports or competitive sports
○ Given intranasally (nasal spray) so that it would stimulate that would lead to injury
the release of factor VIII from the endothelial cells ○ Activities are encouraged but not contact sports or
○ To deliver the nasal spray: competitive sports
1. Position of the patient should be upright and not ● Factor coverage for invasive procedures
lying ○ Invasive procedures may result to excessive bleeding
2. Perform nasal care first to make sure that nose is ○ If the patient has surgeries or procedures, we really have to
clean (blow the nose, check if there is any plan carefully because we have to do prophylactic treatment
obstruction) of recombinant factor infusion so that the patient would not
3. If spray is used for the first time, do a test spray experience or will have a decrease risk of having a massive
away from the patient and if you noticed that there is bleeding
some medication released from the nasal spray ● Document infusion and response to tx
bottle then you can use it already ○ Because these medications are extraneous recombinant
4. If you are going to deliver 2 sprays per nostril, wait factor so there is a chance that patient may be allergic to
for 30-60 seconds before delivering the next nasal them → monitor the patient for reactions
spray ● Avoid taking temperatures rectally or giving suppositories
5. Make sure to occlude the opposite nostril or one ○ Might damage the rectal mucosa → bleeding
nostril before delivering the medication ● Check BP by cuff as little as possible
○ NOTE: Do not interchange the one that is being used for the ○ Make sure it's not too tight because it might result to bruising
treatment of nocturnal enuresis and diabetes insipidus ● Use only paper or silk tape for dressings
because it is different although it is also DDAVP ○ Tegaderm or tape used to secure IV site may cause abrasion
● Amicar (epsilon aminocaproic acid) → bleeding
○ the problem with frequent clotting factor treatment ● Perform mouth care w/ glycerin swab
(recombinant or infusion), because it is extraneous (foreign & ○ The most appropriate for smaller kids is the use of toothies
lab-made; not natural to the body to have them) → the body because we are sure that these tootsies are gentle in the
would eventually create antibodies against it. That antibody gums
is known to be inhibitors. ○ Ex. in the market, we check for soft bristle brush but when
○ A complication of this treatment is called inhibitor we try, it’s still harsh in the gums → bleeding (massive for
development and if that would happen, the recombinant hemophilic patients; it would stop for a moment and after
factor would be ineffective → Amicar is given some time, it would bleed again profusely)
○ Can be given orally (liquid or tablet form) or intravenously ○ Instead of toothbrush, use toothies or glycerin swab
(IV) if the patient is hospitalized to have a faster result ● Limit venipunctures
○ Schedule laboratory procedures wherein only one blood
COMPLICATIONS OF TREATMENT extraction would be done instead of several extractions
● Inhibitor/antibody development ○ As nurses, we make sure that lab exams are properly
○ Amicar is given to be part of the treatment instead of the scheduled
recombinant factor ● Do NOT give aspirin
● Blood-borne illnesses ○ Aspirin is an anticoagulant → inhibit platelets aggregation →
○ Because of massive bleeding, patient may be receiving blood prone to bleeding
components or blood product → blood-borne illness
○ Hep B and C NOTE: Platelet counts of patients with hemophilia are normal. The platelets
○ HIV are not affected, only the clotting factors are. The best lab procedure to do is
the PTT or aPTT (more reliable)

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NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
● Bleeding time would be normal in patients with hemophilia because
Von Willebrand Disease platelets are not affected. In VW, platelets may be decreased (platelets
● It’s good to compare Hemophilia with this disease aggregation might be affected) → bleeding time might be prolonged
● An autosomal DOMINANT disorder (only one copy of mutated gene ● In aPTT (most reliable lab result for hemophilia), it is prolonged in
would be needed to have this disorder) patient with hemophilia and VW disease
● If one parent is affected, there’s 50% chance that offspring will have the ● In factor VIII, depending on the type, if it’s hemophilia A, it would be
disease low. Normal for hemophilia B, but it’s low again in VW because in VW,
● Factor VIII will also be deficient plus platelets may or may not be factor VIII is also deficient
affected ● Factor IX is low for hemophilia B and normal for Hemophilia A and VW
● low levels of von Willebrand factor ● VW factor is low for VW disease and normal for Hemophilia A & B
● Most common form of disorder is autosomal dominant trait
● Disease can occur in both males and females equally Dengue Hemorrhagic Fever
● Common in the Philippines
MANIFESTATIONS ● Affects the platelets
● Easy bruising ● Caused by Dengue virus transmitted by a vector mosquito (Aedes
● Epistaxis Aegypti)
● Other clinical manifestations include: ○ Can easily be distinguished from other mosquitoes due to
○ Gingival bleeding their tiger appearance (white stripes)
○ Ecchymosis ○ Patients can have manifestations 4-5 days after being bitten
○ Increased bleeding w/ lacerations or during surgery and ○ Not all Aedes Aegypti can transmit dengue, but it is a good
dental extractions vector (can also transmit Zika virus)
○ Menorrhagia (increased menstrual bleeding) ○ Bites in broad daylight but can also bite at night (esp. well-lit
■ Pregnant patients may undergo massive bleeding areas); low-lying mosquito
during delivery ○ To minimize DHF, we need to remove areas where water can
○ GI bleeding accumulate (tires, pots, vases) since they like to dwell on
dark areas & stagnant water
NOTE: Hemarthrosis is not common in Von Willebrand unlike hemophilia and
○ Dengue virus has 4 serotypes/strains
the reason is unknown
■ If you have been infected by one serotype, you
will be immune to that but not to the other three
TREATMENT
■ If get infected again by dengue, it will have a more
● Similar to hemophilia A
severe manifestation
● Restore clotting factor and prevent complications associated w/
■ Patient is more at risk to develop severe dengue
bleeding
or DHF the second time they are infected
○ Infusion of vWB (Von WilleBrand) protein concentrate
● Affects all ages mostly 10-15 years
○ DDAVP
DIAGNOSTIC TEST
■ Stimulate the endothelial cells to release clotting

factor VIII in the bloodstream; intranasal
● Tourniquet test using the BP cuff
○ Amicar
○ take the BP
■ In cases of inhibitor development due to
○ take average/midline of systolic and diastolic
prolonged used of recombinant factor infusion
○ rest
○ inflate sa makukuhang values
NURSING MANAGEMENT
○ leave for 5 minutes
● Similar to Hemophilia A
○ check if there are signs of petechiae
● PRICES - Protection, Rest, Ice, Compression, Elevation, Support
MANIFESTATIONS OF DENGUE FEVER
● Fever
Hemophilia A Hemophilia B VW disease ● Rash
● Muscle & joint pain
Bleeding time Normal Normal Prolonged
● Nausea & vomiting
Prothrombin Normal Normal Normal
Time NOTE: Dengue fever can progress to Dengue Hemorrhagic Fever because of
aPTT Prolonged Prolonged Prolonged capillary leak syndrome or plasma leakage wherein there’s increased
permeability of capillary so plasma will sip into the capillary causing
Factor VIII Low Normal Low or normal
accumulation of plasma in other areas of body (heart, lungs) →
Factor IX Normal Low Normal
hemoconcentration → if not addressed can lead to Dengue Shock Syndrome
VWF Normal Normal Low

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NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
● Hemodynamic stability
● Normal Hct and WBC
○ Since it’s a viral infection, WBC is decreased but
during this phase WBC recovers

TREATMENT
● Supportive therapy
○ Nurses address the symptoms patient is experiencing
● IV Fluids
○ Dehydration due to plasma leakage → provide isotonic IV
fluids and sometimes colloids
○ Monitor patient because of increased risk for circulatory
SIGNS AND SYMPTOMS
NOTE: Signs and symptoms of DHF depends on which phase the patient is in overload due to plasma leakage
1. Febrile Phase ○ Plasma leakage can cause dehydration and circulatory
overload at the same time
● N/V ● Bone pain
● High grade fever (2-4 ○ DHF also called ● Monitor BP (narrowed pulse pressure)
days) breakbone fever ○ Patient may be experiencing internal hemorrhage or shock
● Severe headache because of the pain ○ Narrow pulse pressure: <20 mmHg → check if patient is
● Retro-orbital pain ● Maculopapular rash experiencing other symptoms of shock
● Joint pain (arthralgia) ○ Petechiae (bleeding or ● No aspirin for fever
● Muscle pain (myalgia) bursting within
○ Drug of choice for fever is paracetamol
capillary) or purpura
○ Aspirin is anticoagulant → increases risk for bleeding since
rash (sign of bleeding
under the skin, the platelets for patients with DHF are already low
palpable, elevated) ● Plasma expander and platelet concentrate (below 20,000)
○ Nursing responsibilities are the same with blood transfusion
■ Stay with the patient
■ Monitor VS every 15 mins, every 30 mins, and
every hour
■ Check for transfusion reactions
2. Critical Phase
○ We transfuse platelet concentrates when platelet value falls
● Most patients would recover but if patient doesn’t, they can
below 20,000
have plasma leakage (usually happens when fever subsides or
● Oxygen PRN
defervescence)
○ Administered if there are signs of shock or plasma leakage
● Begins after defervescence (24-48 hours)
has affected lungs (pleural effusion)
○ After 24-48 hrs of defervescence, patient may
● DAT (except dark colored food) - Diet As Tolerated
recover completely or progress to a more severe type
○ Dark colored foods may mask the internal bleeding
of dengue called DHF which would lead to plasma
○ Ex. Patient ate tocino/pusit → vomit is dark → we may not
leakage or capillary leak syndrome
recognize if it’s the food or blood
○ The exact mechanism of the increased permeability
● Best treatment is PREVENTION
of capillaries is not known, but it may be due to the
○ Instruct patients to remove stagnant water
reactions of the capillary walls to the enzymes
released by the virus
● Plasma leakage - severe dengue Leukemia (Blood Cancer)
● Cancer of blood-forming cells that usually involves bone marrow and
● Reappearance of fever (saddleback fever)
lymphatics
○ On and off fever
● There are many types but the most common in pediatrics: Acute
● Could lead to shock
Lymphocytic Leukemia (ALL) and Acute Myeloid Leukemia (AML)
NOTE: In capillary leak syndrome, only the plasma leaks out of the
capillary, the RBCs will stay in the capillary → plasma goes out to
ANATOMY & PHYSIOLOGY REVIEW
lungs, heart, peritoneal membrane causing accumulation of fluid in ● Stem cells gives rise to different blood components
these organs → pleural effusion, cardiac overload, ascites → end ● Before they become mature, stem cells produce the immature cells
result is hemoconcentration (high hematocrit, but capillaries are full ○ megakaryoblast (immature) → platelets (mature)
of RBCs) ○ erythroblasts → RBC
NOTE: In DHF, platelets are affected so patients also have ○ myeloblast (immature granulocyte) → eosinophil, basophil,
thrombocytopenia. The exact mechanism of this is also unknown, neutrophil, monocyte
but one explanation is the virus has high affinity to platelets → ■ In AML, there’s increased number of myeloblast in
attach to platelets and replicate there → platelets with virus attacks bloodstream (no mature granulocytes → no
normal platelets → death of platelets → drastic drop of platelets function)
○ Lymphoblast → lymphocyte
3. Convalescent Stage
● Patient is recovering

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NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
■ Lymphocytes give rise to B lymphocytes (cells cells (it can’t differentiate mature & immature cells so it
that mature in bone marrow), T lymphocytes thinks the body has enough cells)
(mature in thymus), and Natural Killer Cells ● 2-6 years of age
■ In ALL, they stay as lymphoblast and never ○ Toddlers & preschoolers
develop to B cells, T cells, and NK cells ● Pancytopenia
■ Lymphoblast is immature and not able to carry its ○ Decrease in all blood components
function ○ Includes leukopenia, thrombocytopenia, & anemia
● Good prognosis; very responsive to treatment; high life expectancy
○ 80% of childhood leukemia is attributed to ALL, of this,
95% will have remission of s/sx (absent manifestations)
once chemotherapy is started
● Acute lymphocytic leukemia is the most common cancer in young
children

DIAGNOSIS
● CBC
○ All blood components are decreased
● Peripheral Blood Smear
○ Checking the number of a blood component in relation to
other blood components & morphology
○ Reveals a lot of “-blasts” or immature cells
● Bone Marrow biopsy
○ Reveals a lot of immature cells in the system but specifically
Agranulocytes (affected in ALL) in the bone marrow
● lymphocyte and monocyte ○ Normal site: iliac crest (based on book)
● Lymphocytes aka Agranulocytes (B lymphocytes, T lymphocytes, ○ However, pediatricians often use sternum as site of
NK cells) are important in fighting abnormal cells aspiration
○ When they detect an abnormal cell in bloodstream, these ■ Ideally should be on iliac crest so that it won’t be
mature cells release chemicals that cause cell death on traumatic on child bc in sternum they can see
abnormal cells what’s happening
○ After killing it, they will proceed to finding more abnormal ○ Child is sedated
cells and completely remove them from the bloodstream ● Lumbar puncture
○ They can only function if they are mature ○ Aka lumbar tap or spinal tap
○ NK cells are important in detecting and fighting tumor or ○ If suspected of CNS involvement
cancer cells → since this is missing/deficient, it is easy for ○ Patient in side-lying knee-chest position to widen the space
cancer cells to proliferate in leukemia (ALL) between the vertebrae
■ Easier to locate the site to aspirate CSF (between
Granulocytes (affected in AML) L3 & L4)
● Myeloblast will not develop into mature granulocytes (basophil, ■ Spinal cord ends at L2 and we don’t want to hit
eosinophil, neutrophil) the spinal cord when we aspirate CSF
○ Basophils - largest granulocyte cells important in immune / ■ Can also be L4 & L5 or L5 & S1 but L3 & L4 most
allergic reactions of the time
■ These granules release histamine to counteract ○ Side effect: spinal headache
effects of allergic reaction ■ Advise patient to remain flat and still to avoid
○ Neutrophils - responsible for phagocytosis spinal leakage
■ When they detect a pathogen, they extend a part
of their cell to phagocyte the pathogen →
resolves invasion of pathogen
○ Eosinophils - responsible for parasitic infections,
inflammation
■ Through degranulation (release of granules), they
are able to kill the intestinal worm
SIGNS AND SYMPTOMS
Acute Lymphocytic Leukemia ● NOTE: S/sx are related to pancytopenia
● Involves lymphoblasts (immature lymphocytes) ● Normally, the first report of parents is on and off fever
○ Rapid proliferation of lymphoblasts (acute because they ● Extreme fatigue
rapidly multiply in bloodstream) → outnumber normal cells ● Headache
(RBC, WBC, platelets) → bone marrow stops producing ● Seizures
○ CNS involvement; a lot of immature cells in brain

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NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
● Frequent infections ○ Even if the patient is on remission, some leukemic cells may
● Anemia be present and we would like to make sure they are gone in
● Bruising (ecchymosis) & bleeding the consolidation phase
● Coughing & breathing difficulties
○ A lot of immature cells in lungs → may lead to fluid 4. Intensification Phase
accumulation in lungs → pleural effusion ○ Give another set of chemotherapeutic agents & we would
● Lymphadenopathy (swelling of lymph nodes) like to protect normal cells
○ Lymph nodes are palpable for pediatric patients ○ Recovering medications are given to protect normal cells
● Swelling on different parts of the body from harsh effect of cytotoxic/chemotherapeutic agents
● Lack of appetite
● Stomachache 5. Maintenance Phase
● Weight loss ○ Longest phase of chemotherapy
● Joint swelling ○ Making sure there’s no regrowth of leukemic cells and no
● Bone and joint pain presence of leukemic cells in bloodstream
● Skin rashes ○ So that there’s no chance of relapse (reappearance of s/sx
NOTE: Lymphatics (lymphatic system) are greatly affected in ALL, rather than that are already treated)
the bone marrow → lymphadenopathy, swelling, bone & joint pain ○ Complete eradication of leukemic cells
○ Complete remission for 5 consecutive years = patient is
MANAGEMENT considered cured
Chemotherapy NOTE: Different books have different phases but it will always start with
● End goal is to cure the patient meaning complete remission (free induction phase and end with maintenance phase and end goal of
from s/sx) for 5 consecutive years chemotherapy is CURE from cancer (5 consecutive years)
● Initiated once leukemia is diagnosed NOTE: For patients who have a relapse during the chemotherapy, they can be
a candidate for bone marrow transplant
1. Induction Phase
○ Lasts for 4 weeks
○ Goal is to have remission or subside the manifestations
experienced by patient
○ Initiated as soon as patient is confirmed to have ALL
○ NR: Watch out for increased uric acid in bloodstream
■ Caused by the rapid death of leukemic cells
○ Patients may be receiving allopurinol
■ This decreases amount of uric acid in bloodstream
→ prevent accumulation of uric acids in kidney →
prevent kidney damage NURSING CONSIDERATIONS
■ Not an anti-gout for pediatric patients ● Relieve pain
■ For a patient to receive chemotherapy, they should ○ Because of lymphadenopathy, muscle and joint pain
have good kidney function since chemotherapeutic ○ Give tylenol, paracetamol, or acetaminophen
drugs are nephrotoxic ○ Avoid aspirin & NSAIDS (ideally, but no choice sometimes)

2. CNS Therapy or CNS Prophylactic Phase or Sanctuary Phase ● Prevent infection


○ Started once there is remission or improvement of condition ○ Patient already has pancytopenia + chemotherapeutic
○ Administration of chemotherapy via intrathecal route (spinal drugs → more risk for infections
canal, subarachnoid space) ○ Implement reverse isolation (wear masks for both nurse &
■ To make sure that brain is also free from leukemic patient, strict handwashing, limited visitors and no fresh
cells flowers)
○ Even if the patient doesn’t have manifestation because of ○ If possible, only the nurse assigned to the patient should be
successful induction phase (95% of patients respond to the nurse of the patient (not allowed to handle other
induction phase) infectious patient)
■ Cancer cells are deceiving so we have to make ■ Only patients receiving chemotherapy would be
sure that they are not present in other parts of the our patients
body especially the brain ■ We do not want to cause infection to our
○ Usual drug: methotrexate immunocompromised patients
○ Neutropenic diet
3. Consolidation Phase ■ No raw foods
○ 28 weeks of treatment ■ Thoroughly wash vegetables for salads
○ Eradication of leukemic cells that could still be present in the ■ Careful in sushi & raw steak
bloodstream or any part of the body ● Precaution in chemotherapy (N/V, anorexia, neuropathy, mucosal
○ To eradicate all stubborn leukemic cells that regrow or were ulceration, hemorrhagic cystitis, moon face, mood changes, alopecia)
hidden and not targeted before ○ N/V - Antiemetics are given most of the time
○ Anorexia - Small frequent feeding
○ Neuropathy - Encourage ambulation

7 SACRAMENTO | SANTOS, C. | SANTOS, Z | SAPALO | SAUL | SERRANO| SUMABAT


NURSING CARE OF A CHILD WITH HEMATOLOGIC DISORDER PEDIA LEC
Ma’am Jaclyn Charmaine J. Magpantay, RN, MAN
○ Alopecia - Common concern of patients is if alopecia is ○ Ideally, after the patient responds well to induction phase &
permanent (no, it is temporary) there is remission of the manifestation → bone marrow
■ Because it targets rapidly dividing cells (such as transplant should follow so no maintenance phase to
hair follicles) patient with AML
■ Nurses should emphasize that it is temporary &
hair will regrow after stopping chemotherapy
once they have complete remission

Acute Myeloid Leukemia


● Granulocytes are affected
○ They remain as myeloblasts (bigger than lymphoblasts)
● Only 20% of childhood leukemias
○ Most of patients affected by AML are adults or adolescents
● Poor prognosis; life expectancy is 5-10 years (based on research)

● Punnett square - shows the percentage or the possibility that the


DIFFERENCE BETWEEN ALL AND AML person with hemophilia will have offsprings with hemophilia
● Examples:
AML ALL A male adolescent with hemophilia asks you what’s the percentage that he
Age Common adult Children will transfer it to this son
● He will only transfer it to his daughters (they will be carriers)
Lymphadenopathy Less common More common
Can a female with hemophilia transfer it to her sons?
Hepatosplenomegaly Less common More common ● Yes
● 25% if 4 children
Bone and joint pain Less common More common
● 50%
Gum hypertrophy More common Less common Can a female with hemophilia transfer it to her CHILDREN?
Blast cells Myeloblast Lymphoblast ● Yes. 25% (1 out of 4 children)

● The manifestation of ALL and AML are the same (attributed to Can a female with hemophilia transfer it to her SON?
pancytopenia of the patient) ● Yes. 50% because one of the 2 son’s chromosome will be affected
● But since ALL involves the lymphatics, there’s more incidence of
lymphadenopathy and bone and muscle joint pain
● With AML, since what’s greatly affected is the bone marrow, and the
occurrence of lymphadenopathies is decreased and not the common
compared to ALL
● According to age, ALL are common among children while AML is more
common in adults or in adolescents
● Hepatosplenomegaly is less common in AML because of the
involvement of the lymphatics. When there is an involvement of the
lymphatics in ALL, hepatosplenomegaly is more common to them and
because of hepatosplenomegaly in patient will ALL, they have this loss
of appetite (anorexia)
● Gum hypertrophy is more common in AML because the bone marrow is
affected. When there is bone marrow hyperactivity or increased
stimulation of the bone marrow, the bones (esp. facial bones) are
affected → gum hypertrophy
● The cells that are rampant or increase in AML would be myeloblast or
the myeloid while in ALL the increase would be lymphoblast

TREATMENT
● Chemotherapy
○ In ALL, there are different phases of chemotherapy:
induction, consolidation, and maintenance
○ In AML, the chemotherapy starts in induction phase but
there is no maintenance phase
■ Once there is remission after the induction phase,
→ proceed with bone marrow transplant ideally
so that complete remission could happen → life
expectancy will increase and the prognosis would
be good
● Bone marrow transplant

8 SACRAMENTO | SANTOS, C. | SANTOS, Z | SAPALO | SAUL | SERRANO| SUMABAT

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