THE CHILD WITH § Indications of capillary bleeding

HEMATOLOGIC DISORDERS o Large spleen

• Blood component: § Dead RBCs go here
o Erythrocytes – for oxygen transport o Spoon-shaped nails
§ 4.9-5 million § Secondary to chronic peripheral
o Hemoglobin hypoxia
§ 11-16g/100ml blood o Weakness (refuses to play)
o Bilirubin § Lack of oxygen to tissues
§ 7 mg/100 ml blood o Irritable
o Leukocytes (WBC) § Lack of blood supply to the brain
§ 5,000 – 10, 000
o Thrombocytes (platelets) ETIOLOGY OF ANEMIA
§ 150,000 – 300,000/mm3 NORMOCHROMIC (normal color), NORMOCYTIC
(normal size) ANEMIAS
ASSESSMENT • Decreased or impaired production of erythrocytes
• Check table 44.2 o Bone marrow failure: congenital or
• History acquired
o Fatigue • Excessive blood loss
o Blood loss at birth o Hemorrhage
o “picky-eater” • Destruction (hemolysis) of erythrocytes
o family history – inherited • Infection induced
• Physical appearance • Renal disease cause
o Obese • Related to neoplastic disease
§ Overfed with milk
o Pale MANAGEMENT
o Facial bossing • Discontinue the cause
§ Thalassemia o Factors from the outside
§ Facial bones enlarge by o Radiation, chemicals, drugs
producing more blood • PRBC transfusion
o Petechiae
o Severe anemia
o Packed Red Blood Cell not fresh whole BEST MANAGEMENT: PREVENTION
blood • Use only breastmilk or Iron fortified formula
• Erythropoietin administration • Introduction of complementary feedings at 6
o Renal problems months
o To stimulate RBC factor in the individual o Iron-rich foods:
• Oral corticosteroid § Iron rich cereals, egg yolk, dark
o Related to leukemia to decrease the green leafy vegetables, mashed
immune response of the body potato, dark meat, snacks with
• Hematopoietic stem cell transplant raisins
• Limit milk intake to 1 liter/day
HYPOCHROMIC ANEMIA • Supplemental iron preparation (FeSO4, 1
IRON DEFICIENCY ANEMIA mg/kg/day) given with vitamin C to promote
• More common to 9 months to 3 yr old due to absorption
overfeeding of milk • Parenteral iron for children with iron
o “milk babies” maximum allowable – less malabsorption
than 32 oz/day • Blood (PRBC) transfusion PRN (2-3 ml/kg/bwt)
o 3 glasses
o maternal iron stores already used up ANEMIAS CAUSED BY INCREASED
• also to adolescent females rapid growth, weight DESTRUCTION OF RBC
reduction diet and vegetarian diet/heavy menses 1. Thalassemia
o chronic blood loss is the most frequent 2. ABO/Rh Incompatibility
cause of iron-deficiency anemia caused by 3. Sickle cell anemia
gastrointestinal tract lesions such as
polyps, ulcerative colitis, Crohn disease, HYPOCHROMIC MICROCYTIC ANEMIA
protein-induced enteropathies, parasitic 1. THALASSEMIA
infestation, or frequent epistaxis. • Hereditary as recessive trait
• Without adequate iron, hemoglobin cannot be o Both parents are asymptomatic carrier
incorporated into RBCs
 • Types • Without D antigen – RH negative
o T. minor o Blood can be received from an RH
§ T. trait negative person only
§ With mild microcytic anemia o O–
§ Asymptomatic o Antibodies can be produced if blood is
§ No treatment received from a positive individual
§ Normal life expectancy • Universal Blood Type is O-
o T. Major
§ Cooley anemia/Mediterranean
Anemia
• Partial or complete deficiency in the synthesis of
the B-chain of the hemoglobin molecule causing
production of very fragile hemoglobin that is
unstable
o Easily hemolysis
• Bone marrow compensates by producing more
blood causing aesthetic problem due to
hypertrophy
o “Bossing”
• Symptoms: table 44.3 • Utero-placental barrier prevents the mixing of the
• Diagnosis: At birth – Blood spot test mother’s blood and the baby’s blood
• Hence, the first baby is not affected
MANAGEMENT
• They should not be given iron supplements
• Blood transfusion to maintain Hemoglobin bw 10-
12g/100nml
o Given every 2-4 weeks
• Problem with iron hemosiderosis due to
accumulated iron in the organs and tissues due to
frequent transfusion
• Problem with potential to blood carried conditions
like Hepatitis and AIDS
• Requires CHELATION THERAPY with Fe
DEFEROXAMINE IV or SQ with vitamin C
• ORAL chelators – DEFERASIROX or • When the placenta detaches, the barrier is now torn
deferoxamine for the developing hemosiderosis • Blood in the placenta (RH+) is absorbed in the
• Splenectomy PRN maternal sinuses
o reduce discomfort and also to reduce the o RH+ accidentally absorbed through
rate of RBC hemolysis and the number of mother’s circulation (RH-)
transfusions needed o Antigen triggers the response on
antibodies à The mother produces Anti-
2. BLOOD INCOMPATIBILITY positive antibodies

• D antigen – RH positive
o 99% • 2nd baby is RH+
o RH negative blood can be received • The barrier does not allow the blood to mix
o O + or O- o The barrier allows the antibody to cross
 o The mother gave the baby IgG when she
was pregnant: Pinocytosis
• When the anti-positive antibodies from the
previous pregnancy cross the barrier to the 2nd
RH+ baby, will destroy the baby’s blood and
might die = Hydrops Fetalis

MANAGEMENT
• If mom and baby are not compatible:
o After 1st baby, COOMB’s TEST (baby)
§ Determines presence of maternal
antibody on the baby’s blood
• If mother is not compatible with baby and
Coomb’s is negative, then RhIg (RHOGAM) is
given to mother within 72 hours after delivery or •
abortion of an incompatible fetus

MANAGEMENT AFTER BIRTH OF THE SECOND

BABY IF AFFECTED
• Coomb’s Test is positive
o Early feeding
§ Promote urination and defacation
to eliminate bilirubin
o Phototherapy
o Exchange transfusion
§ Removal of baby’s blood and
replacement with fresh whole
blood (Rh negative)

BLEEDING DISORDER
HEMOPHILIA •
• Deficiency in Factor VIII (antihemophilic factor)
SIGNS AND SYMPTOMS
• Factor VIII is an intrinsic factor of coagulation; its
• Signs of external bleeding
absence causes the intrinsic system for
o Bleeding in the gums and mouth
manufacturing thromboplastin to be incomplete.
o Epistaxis/Nosebleed
• Transmitted as X-linked from carrier MOM to
o Heavy bleeding from a cut
AFFECTED SON
§ Bleeding from a cut that resumes
• Daughter gets it as a trait from carrier mom after stopping for a short time
• AFFECTED SON gives it to DAUGHTERS as • Signs of Internal Bleeding
TRAIT only o Blood in urine
• AFFECTED SON will have ALL NORMAL o Blood in the stool
SONS o Unexplained bruises
o Hemarthrosis
§ The joints most often damaged by
bleeding are the hinge joints.
§ These are the:
• Knees
• Ankles
• Elbows
• These hinge joints have
little protection from
side-to-side stresses. As a
result they bleed more
often
§ The ball-and-socket joint, which
are better supported, bleed less
often. These are the:
• • Hip
 • Shoulder If in shock
o Bleeding in the brain • Circulatory failure
§ Worst kind of bleeding • Pleural effusion: accumulation of fluid in the lungs
• Ascites in severe cases
MANAGEMENT
• Medical: Vasopressin (DDAVP) The WHO definition of dengue hemorrhagic fever has been
o Goal: stimulation of the production of in use since 1975; all four criteria must be fulfilled:
clotting factors to prevent bleeding 1. Fever, constant headaches (retro-orbital), severe
o Expensive = why it is called the royal dizziness and loss of appetite.
blood disease 2. Hemorrhagic tendency (positive tourniquet test,
• Transfusion of factor VIII – AHF concentrate spontaneous bruising, bleeding from mucosa,
Corticosteroids/NSAIDS gingiva, injection sites, vomiting blood, or bloody
o To support the production of factor VIII diarrhea)
• Prevent bleeding 3. Thrombocytopenia (<100,000 platelets per mm3)
o Wear devices such as helmets and pads 4. Evidence of plasma leakage (hematocrit more than
o Do not give aspirin 20% higher than expected)
o Gentle brushing of teeth
o Contact sports that are aggressive should TREATMENT: SUPPORTIVE THERAPY
be prevented • Prevent bleeding
o No piercings o Do not get blood through the use of
• Bleeding Management: PRICES capillary puncture
o Protect o Use venipuncture
o Rest • Fluid & electrolyte replacement (oral/IV)
o Ice: 15 minutes o Replace natural replacement with oral
o Compress: stops capillary bleeding • Monitor VS: especially BP, narrow PP (less than
o Elevate: above the heart 20 mmHg)
o Support: Doctors, Nurses o Normal PP is 40 mmHg
• Prevent crippling effects of bleeding o Less than 20 mmHg = circulatory collapse
o Active ROM after bleeding episodes • Proper fever control
§ Patient decides to the exercise o Avoid aspirin
§ Knows when to stop o Give paracetamol/acetaminophen
o Weight control • Plasma expanders, platelet concentrate
o Below 20,000
DENGUE HEMORRHAGIC FEVER • Oxygen PRN
ETIOLOGY • DAT (No dark colored foods)
• Dengue virus o Remove peeling for red apples
• Aedes aegypti o White chocolate can be given
o Domestic day-biting mosquito
o Vector LEUKEMIA
• Affects all ages mostly among 10 -15 year old, • Most common form of childhood cancer
both genders • Malignant disease of the bone marrow and the
• Capable in transmitting it to another individual lymphatic system
• Immature WBC’s (lymphoblast) not capable of
SYMPTOMS phagocytosis is formed
• Sudden onset • Very vulnerable to infection
• 2 -7 days fever • Intractable
• Muscle and joint pains
o break-bone fever or bonecrusher disease FORMS
• Malaise • ALL (Acute Lymphocytic Leukemia)
• Irritability o Lymphoblastic - WBC
• Headache with retro-orbital pain (behind the ears) o The abnormally proliferating cells are so
• Anorexia immature that they may be identifiable as
• Nausea &Vomiting an immature “blast cell.”
• Skin rash (Herman’s) o More common to children
o Tourniquet test shows petechiae • ANLL/AML (Acute non-lymphoid leukemia or
• Abdominal pain acute myelocytic leukemia)
• Bleeding o More complicated
o All components of blood are effected
THREE MAIN CONSEQUENCES
1. Anemia
2. Infection
3. Bleeding tendencies

DIAGNOSIS:
• peripheral blood smear
• definitive BONE MARROW BIOPSY to confirm
• lumbar puncture to determine CNS involvement

MANAGEMENT GOAL: CURE

Four Phases
1. Remission Induction
o IV (systemic chemotherapy)
o May be leukemic but the symptoms are
controlled
o Reverse isolation in his own private room
o PCG can stay inside
o IV is covered with a dark cloth because it
is photosensitive
2. CNS Prophylactic Therapy/Sanctuary
o Intrathecal chemotherapy
o Aimed at achieving the protection of the
bone marrow from being invaded
o Prevents it from spreading to other organs
o Given directly to the spinal column
3. Intensification or consolidation therapy
o Regular systemic and intrathecal
chemotherapy
o Goal: Remission à sent home
4. Maintenance therapy
o Combined drug regimen with periodic
CBC
o Do not bring the child in places where
there is infection
• Most Ideal
o Hematopoietic stem cell transplant =
BONE MARROW TRANSPLANT
o Not easy, expensive, finding a compatible
donor
o In the PH: regular chemotherapy
o Food should be well cooked
o No crowded areas

NURSING CARE
• Assist in diagnostic test
• Relieve pain
o Non-pharmacologic
o Pharmacologic (narcotic by PCA)
• Prevent complication of myelosuppression-
infection (reverse isolation), hemorrhage, anemia
• Precaution in chemotherapy
o Nausea & vomiting
o Anorexia
o Mucosal ulceration
o Neuropathy
o Hemorrhagic cystitis
o Moon face, mood changes
• Physical & emotional support