Vous êtes sur la page 1sur 74

Common Hematological Disorders

in Pediatric

Thandar Soe @ Sumaiyah Jamaludin


12/10/2015

sumaiyah@iium.edu.my

Words of Wisdom
Blood is just red to the child, but to the
nurse blood is life

12/10/2015

sumaiyah@iium.edu.my

Course Outline
Indentify major haemotologic disorders
that affect children
Determine priority physical examination
for children with hematologic disorders
Provide appropriate nursing diagnosis for
the child and family with hematologic
disorders
Discuss priority nursing intervention for
children with hematologic disorders
12/10/2015

sumaiyah@iium.edu.my

Introduction
The hematologic system consists of blood
and blood-forming tissues of the body.
These typically function together in a
balance that affects the metabolism of
the body
Three categories of cells: erythrocytes
(RBC), Thrombocytes (Platelets) and
Leukocytes (WBC)

12/10/2015

sumaiyah@iium.edu.my

Function of the main three cells


RBC

Platelets

WBC

12/10/2015

Transport nutrients & O2 to the body tissue


Waste products from the body tissue

Responsible for clotting

Responsible for fighting infection


Divided into granulocytes (neutrophils,
eosinophils and basophils) and agranulocytes
(lymphocytes and monocytes)

sumaiyah@iium.edu.my

Variation in Pediatric Anatomy and


Physiology
8th weeks of gestation: production of blood cell begins.
Primarily form in the liver
3rd timester shift to bone marrow ( in Premature occur
Extramedullary Haematopoiesis)
In infants and young children all of the bone contains red
marrow (so-called because of its color from formation of
erythrocytes).
At the end of adolescence, only the ribs, sternum,
vertebrae, and pelvis continue to produce blood cells. The
remainder of the bone marrow becomes yellow from deposition of
fat.
12/10/2015

sumaiyah@iium.edu.my

Classification of hematologic
disorders
1. Anemia
A.

Nutritional cause of Anemia


I.
II.
III.

Iron Deficiency Anemia


Aplastic Anemia
Hemoglobinopathies

Sickel cell disease


Thalassemia
Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

2. Clotting Disorders
A.
B.

Idiopathic Thrombocytopenia
Hemophilia

3. Neoplastic Disorder (Childhood cancer)


A.

leukemia

12/10/2015

sumaiyah@iium.edu.my

1. Anemia

12/10/2015

sumaiyah@iium.edu.my

Anemia
A condition in which the blood fails to
supply the body's tissues with sufficient
amounts of oxygen.
OR
A condition in which the number of RBCs
and/or the hemoglobin concentration is
reduced below normal:
0-14 days - Hb<145 g/L
15-28 days - Hb<120 g/L
1month-1yr - Hb<110 g/L
12/10/2015

sumaiyah@iium.edu.my

Common Causes of anaemia


.
Defective bone marrow
A shortage of iron, the main ingredient of haemoglobin
A shortage of the vitamins B12 or folic acid, both
needed to produce red blood cells( fruit, wholemeal bread,
beans, and lean meat)
Loss of blood, through heavy menstruation, or internal
bleeding, which may be caused by disease
Infections such as malaria
Prolonged treatment with aspirin and similar
nonsteroidal anti-inflammatory drugs

12/10/2015

sumaiyah@iium.edu.my

10

A. Nutritional Cause of Anemias

12/10/2015

sumaiyah@iium.edu.my

11

I. Iron Deficiency Anemia

12/10/2015

sumaiyah@iium.edu.my

12

Iron Deficiency Anemia

Causes
- inadequate supply of iron
- impaired absorption
- blood loss
- excessive demands for iron required for
growth
- inability for form of Haemoglobin

12/10/2015

sumaiyah@iium.edu.my

13

Iron Deficiency Anemia


The iron requirement of children
among different age groups.
6 months-2 years
15mg/day
4-10years
10mg/day
11-18years
18mg/day

12/10/2015

sumaiyah@iium.edu.my

14

Iron Deficiency Anemia

Signs and Symptoms: due to tissue


hypoxia > lack of energy, easy
fatigability, pallor and crackel of the
lips
Nails become thin, brittle + flat.
Longitudinal ridges appear on nails,
which may become spoon shaped and
concave.
Diagnosis: CBC, iron studies, physical
exam

12/10/2015

sumaiyah@iium.edu.my

15

Iron Deficiency Anemia

Nursing Assessment and


Interventions:
- educate parents about nutrition &
proper administration of iron preparations,
caution about high toxicity of iron
- explain laboratory testing

12/10/2015

sumaiyah@iium.edu.my

16

Treatment
The underlying cause of iron deficiency should be treated
Iron therapy (Medical treatment) & Counseling
Oral: ferrous sulphate/ ferrous gluconate/ ferrous succinate
Elemental iron 3-6mg/kg PO in three divided doses
Iron should not be given just after the milk feeds or after food.
Oral iron therapy should be continued for at least 6-8weeks after
the Haemoglobin has reached normal level to replete the iron
stores.
Diet counseling.

12/10/2015

sumaiyah@iium.edu.my

17

Nursing assessment
Assess
the cardiovascular system
Childs responsed to iron therapy
Childs activity level
Childs developmental level
Nursing diagnosis
Activity intolerance
Fatigue
Nutrition less than body requirement
Altered growth & development

12/10/2015

sumaiyah@iium.edu.my

18

Nursing management

For drug side effect


Take iron w/t fluid or juice to prevent discoloration
of teeth
Rinse the mouth
Increased fiber & water intake to minimized
constipation

For diet
Reduce milk intake but continue breast milk(BM
iron is well absorbed)
Increased meat & protein
Whole grain &leafy vegetable
12/10/2015

sumaiyah@iium.edu.my

19

Prevention
Children fed purely on milk diet are prone to develop anaemia.
To prevent anaemia, supplementary foods rich in iron should
be given to the child from 4 months of age.
(Diet rich in iron: beans, peas, green leafy vegetables).
Preterm and L.B.W infant with low iron stores should receive 1015 kg of elemental iron daily.
Iron supplements are necessary during adolescence for preventing
anaemia of puberty.
Iron availability in the diet can be improved by:
Increasing the ascorbic acid in the diet to increase iron
absorption.
Increasing the iron intake
Hookworm infestation should be managed with antihelminthicus
Children should be encouraged to wear shoes while going to the field
to prevent infestations with the infective form of hookworm larvae.

12/10/2015

sumaiyah@iium.edu.my

20

II. Aplastic Anemia

12/10/2015

sumaiyah@iium.edu.my

21

Aplastic Anaemia
Disorder of BM failure resulting in depletion
of all marrow elements
Causes
Primary congenital (unknown)
secondary
Autoimmune basics(SLE)
Antineoplastic or cytotoxic agent
Radiation therapy
Certain drugs
Chemical-----insecticides
Viral diseases(HIV)

12/10/2015

sumaiyah@iium.edu.my

22

Signs & Symptoms

12/10/2015

sumaiyah@iium.edu.my

23

III. Hemoglobinopathies

12/10/2015

sumaiyah@iium.edu.my

24

1. SICKLE CELL ANEMIA


A hereditary
disorder in which
normal hemoglobin
(HbA) is partly or
completely replaced
by an abnormal
hemoglobin (HbS)
causing distortion and
rigidity of red blood
cells under condition
of reduced oxygen
tension.
12/10/2015

sumaiyah@iium.edu.my

25

SICKLE CELL ANEMIA


Sickle cell anemia
is an autosomalrecessive
disorder.
Sickle cell anemia
is found primarily
in the black race,
although
infrequently it
affects whites.
12/10/2015

sumaiyah@iium.edu.my

26

SICKLE CELL ANEMIA


Manifestations:
General:
Growth retardation
Chronic anemia
Delayed sexual maturation
Marked susceptibility to sepsis
Other signs and symptoms.
weakness; anorexia; joint, back, and abdominal
pain; fever; and vomiting.
Chronic leg ulcers are common in adolescents and
adults and are thought to be the result of thrombosis and
decreased peripheral circulation.
*** Take health history, especially regarding any evidence of
sick ling crisis and history of the disease in family
members.

12/10/2015

sumaiyah@iium.edu.my

27

Sickel Cell Anemia

12/10/2015

sumaiyah@iium.edu.my

28

2. Thalassaemia
Is syndrome characterized by lack of or
decreased synthesis of either
(Alpha) globulin or (Beta) globulin
chain.
Types
Thalassaemia major
Thalassaemia Minor
Silent Carrier state
Thalassaemia trait
12/10/2015

sumaiyah@iium.edu.my

29

Pathophysiology of beta thalassaemia


Diminished synthesis of
beta globulin chain
Decreased amt of RBC
in Hb, Ineffective
erythropoiesis
Produce short life span &
fragile RBC which easily
destroy
Increased erythropoitic
activity

12/10/2015

sumaiyah@iium.edu.my

30

Pathophysiology Con
Iron deposit in the organ
hemosiderosis (systemic
overload of iron) resulting
in decreased function of
cardiac, liver and skin
(hemocromatosis): Bronze
pigmentation
Endocrine abnormalities
Delay development of
secondary sex characters

12/10/2015

sumaiyah@iium.edu.my

31

Clinical features of Thalassaemia syndrome

The infant are born normally


Anaemia and jaundice
Hepato-splenomegaly
The children usually have thalassaemia facie characterized by:
bossing of skull
prominent frontal and parietal eminence with flattened vault
straight forehead
hypertrophy of mandible
prominent malar eminence
depressed bridge of the nose
Teeth are malformed.
Reduced activity, and physical growth.
Irregular fever and intercurrent infections
Pathological fracture of long bones in the untreated cases.
The course of the disease depends on the severity of the disease
Death may be due to severe anaemia, cardiac or liver failure.

12/10/2015

sumaiyah@iium.edu.my

32

12/10/2015

sumaiyah@iium.edu.my

33

12/10/2015

sumaiyah@iium.edu.my

34

Management

Frequent & regular bld transfusion


maintain Hb>10g/dl ( used packed RBc)
Blood transfusion 10-15ml/kg (body
weight)
Iron chelation therapy with
deferoxamine(Desferal)-reduced toxic side
effect of iron excretion by feces & urine
12/10/2015

sumaiyah@iium.edu.my

35

Complications
Complication due to intramedullay erythropoiesis
e.g. frontal bossing, prominent malar bones (cosmetic effects) etc.
Complications due to haemosiderosis

Iron deposits in major organs liver, heart, pancreas etc. Lead


to cirrhosis, liver, cardiac cirrhosis, diabetes mellitus respectively
and heart failure
Anaemic heart failure
Stunted growth; fractures of long bones.
Complications due to repeated blood transfusions and
splenectomy
Risk of transmission of viral infections like (HIV, hepatitis B &C,
cytomegalovirus)
Post splenectomy sepsis is common below 6 years of age.

12/10/2015

sumaiyah@iium.edu.my

36

Nursing DX

Altered tissue perfusion


Chronic pain
Activity intolerance
Risk for infection
Knowledge deficit
Body image disturbance
Ineffective family coping

12/10/2015

sumaiyah@iium.edu.my

37

3. Glucose-6-phosphate
dehydrogenase enzyme deficiency
G6 PD deficiency
Common in male
Numerous cell enzyme
deficiency
S/S
Jaundice
Hemoglobinuria

12/10/2015

sumaiyah@iium.edu.my

38

G6PD Deficiency Anemia

12/10/2015

sumaiyah@iium.edu.my

39

Acute Intravascular Haemolysis in G6PD


deficiency
The agents causing acute intravascular haemolysis (in G6PD
deficient patients)
Antimalarial drugs
eg. Quiolones, Primaquine, Pamaquine, chloroquine, Quinacrine
Antibacterials
eg Chloromphenicol, Nalidixic acid
Analgesics, antipyretics
eg Salicylates, NSAIDS
Chemical
eg. Phenylhydrazine, Benzene, Napthalene mothballs
Illness
eg.Deabetic acidosis, Hepatilis
Miscelloneous
eg. Synthetic Vitamin K, Feva Bean-like beans, Phenacetin,
Methylee blue.
Clinical features of acute intravascular haemolysis.
Passing high coloured urine (haemoglobinuria, jaundice, and
pallor)
12/10/2015

sumaiyah@iium.edu.my

40

2. Clotting Disorder

12/10/2015

sumaiyah@iium.edu.my

41

A. IDIOPATHIC THROMBOCYTOPENIC PURPURA


(ITP)
Reduced the number of
circulating platelets in BM
Associated with the
immunologically mediated
destruction of platelets
Acute ITP is found in children
after viral infection
(antiplatelet
autoantibodies)platelet count
return to normal within 6mo
Chronic ITP is destruction of
platelet result from the
present of platelet
autoantibodies pls count
persists longer than 6mo
**Peak

aged 2-5 yrs


,male & female equal

12/10/2015

sumaiyah@iium.edu.my

42

Clinical features
Easily bruise and
subcutaneous haemorrhages
occur spontaneously or
following minor trauma.
Skin bleeding Petechiae,
purpura, Ecchymoses.
Bleeding from the mucosal
surfaces Hematemesis,
melena and haemorrhages in
the brain may occur
Anemia is proportionate
to the degree of bleeding.

12/10/2015

sumaiyah@iium.edu.my

43

Laboratory investigation
Isolated thrombocytopenia below
<100,000/ mm3 in a complete blood
count.
Bleeding time is prolonged
Platelet antibodies can be demonstrated in
70-90 % of children
Bone marrow reveals normal or increased
number of megakaryocytes.
Megakaryocytes show diminished budding.
12/10/2015

sumaiyah@iium.edu.my

44

Treatment
Acute ITP

No specific therapy in children with platelet counts above 40,000/mm3.


The disease is usually self-limiting in nature (skin bleeding only, no mucosal
bleeding.)
Supportive
Avoidance of aspirin and related drugs
Intramuscular injections should be avoided during the acute
phase.
Platelet count < 20,000/mm3 should be admitted as they are at higher
risk of serious bleed.
Fresh blood/ platelet transfusions may be given in life threatening
situation or prior to surgery in presence of severe thrombocytopenia
Corticosteroids
Immediate steroid therapy is indicated when there is widespread
bleeding manifestation with a low platelet count below 25,000/mm3
They inhibit platelet antibody production, interaction between the platelet
and antibodies, prolong platelet survival and improve vascular stability.
Prednisolone: 1-2 mg/kg/day for 2-3 weeks followed by tapering doses
over next 1-2 weeks, regardless of the response.

12/10/2015

sumaiyah@iium.edu.my

45

Treatment
Spleenectomy.
Indications: Patients having chronic ITP
uncontrolled bleeding or those not responding to
steroids or IV IgG therapy.
Splenectomy should be under taken after 6 years of
age.
Prophylatic penicillin is given to prevent gram positive
infections
All children should receive meningococcal, H.
influenzae, and pneumococcal vaccine 3 weeks prior to
splenectomy.

12/10/2015

sumaiyah@iium.edu.my

46

Nursing diagnosis

Fluid volume deficit


Pain
Risk for injury
Delay growth & development
Knowledge deficit
Ineffective management for therapeutic regimen
Impaired physical mobility
Care giver role strain
Altered family process

12/10/2015

sumaiyah@iium.edu.my

47

Nursing action
Monitor

Reponses to treatment
Adaptation to pain & action of drugs
Further bleeding
Further injury

Dental care
Educate for longterm T
Provide aged appropriate play&explanation
Emotional support

12/10/2015

sumaiyah@iium.edu.my

48

B. Hemophilia

Factor VIII (Hemophilia A) or factor IX


(Hemophilia B or Christmas disease)
deficiency
prolonged bleeding any where in the body!
Cause: X-linked recessive disorder, defects in
platelets and clotting factors
Diagnosis: history of bleeding episodes,
evidence of x-linked inheritance, labs
Medical Management: Factor VIII concentrate
(vasopressin)

12/10/2015

sumaiyah@iium.edu.my

49

Hemophilia A

12/10/2015

sumaiyah@iium.edu.my

50

Hemophilia
Nursing care:
Prevent bleeding
Recognize and control bleeding (RICE)
- Rest
- Ice
- Compression
- Elevation
Prevent crippling effects of bleeding
Client education
12/10/2015

sumaiyah@iium.edu.my

51

3. Neoplastic Disorder

12/10/2015

sumaiyah@iium.edu.my

52

Leukemia
account for 1/3 of all childhood cancer
Primary disorder of bone marrow
(normal element are replace with
abnormal WBC).
Normal: Lymphoid cell lymphocytes
myeloid RBC, granulocytes & Platelets.
Leukemia may develop at any time during
normal development of lymphoid or myeloid
12/10/2015

sumaiyah@iium.edu.my

53

12/10/2015

sumaiyah@iium.edu.my

54

Classification of Leukemia
1. Acute Lymphoblastic Leukemia
2. Acute Myelogenous Leukemia

12/10/2015

sumaiyah@iium.edu.my

55

Acute lymphoblastic leukemia

80% of leukemias
Girl to- boy ratio is 1: 1
Peak incidence 2 5 years
Incidence in white children is twice as high as in nonwhite children

12/10/2015

sumaiyah@iium.edu.my

56

Acute myelogenous leukemia


Heterogeneous group of malignant hematological
precursor cells of the myeloid, monocytic, erythroid or
megakaryocytic cell lineage
Epidemiology: 15-20% of all leukemias in children
Frequency remains stable throughout childhood with
slight increase during adolescence
No difference in incidence between boys and girls

12/10/2015

sumaiyah@iium.edu.my

57

Leukemia -Clinical Manifestations


Relate to problems caused by:
Bone marrow failure
Overcrowding by abnormal cells
Inadequate production of normal marrow elements
Anemia, thrombocytopenia, number and function of WBCs

Leukemic cells infiltrate patients organs

12/10/2015

Splenomegaly
Hepatomegaly
Lymphadenopathy
Bone pain, meningeal irritation, oral lesions (chloromas)

sumaiyah@iium.edu.my

58

Leukemia - Diagnostic Studies


To diagnose and classify
Peripheral blood evaluation (CBC and blood
smear)
Bone marrow evaluation

To identify cell subtype and stage


Morphologic, histochemical, immunologic, and
cytogenic methods

12/10/2015

sumaiyah@iium.edu.my

59

Leukemia -Collaborative Care


Goal is to attain remission (when
there is no longer evidence of cancer
cells in the body)
Chemotherapeutic treatment
Induction therapy
Attempt to induce or bring remission
Seeks to destroy leukemic cells in the tissues,
peripheral blood, bone marrow
Patient may become critically ill
Provide psychological support as well

12/10/2015

sumaiyah@iium.edu.my

60

Leukemia -Collaborative Care


Chemotherapeutic treatment (cont.)
Intensification therapy
High-dose therapy
May be given after induction therapy
Same drugs at higher doses and/or other drugs

12/10/2015

sumaiyah@iium.edu.my

61

Leukemia - Collaborative Care


Chemotherapeutic treatment (cont.)
Consolidation therapy
Started after remission is achieved
Purpose is to eliminate remaining leukemic cells
that may not be evident

Maintenance therapy
Lower doses of the same drug

12/10/2015

sumaiyah@iium.edu.my

62

Leukemia - Bone Marrow and Stem Cell

Transplantation
Goal
Totally eliminate leukemic cells from the
body using combinations of chemotherapy
with or without total body irradiation

12/10/2015

sumaiyah@iium.edu.my

63

Leukemia - Bone Marrow and Stem Cell

Transplantation
Eradicates patients hematopoietic stem cells
Replaced with those of an HLA-matched
(Human Leukocyte Antigen)
Sibling (is a brother or a sister; that is, any person
who shares at least one of the same parents )
Volunteer
Identical twin
Patients own stem cells removed before

12/10/2015

sumaiyah@iium.edu.my

64

Nursing Management
Planning
Overall goals
Understand and cooperate with the
treatment plan
Experience minimal side effects and
complications of disease and treatment
Feel hopeful and supported during the
periods of treatment, relapse, and remission

12/10/2015

sumaiyah@iium.edu.my

65

Nursing Management
Many physical and psychological needs
Evokes great fear
Family also needs help

Balance demanding technical needs with a


humanistic, caring approach

12/10/2015

sumaiyah@iium.edu.my

66

Nursing Management
Patient empowered by knowledge of the
disease and treatment can have a more
positive outlook and improved quality of
life
Nurses face special challenges when
meeting the intense psychosocial needs of
a patient with leukemia

12/10/2015

sumaiyah@iium.edu.my

67

Nursing Management
Ongoing care is necessary to monitor for
signs and symptoms of disease control or
relapse
Teach patient and significant other
Diligence in disease management
Need for follow-up care
When to seek medical attention

12/10/2015

sumaiyah@iium.edu.my

68

Nursing Management
Goals of rehabilitation
Manage
Physical
Psychosocial
Social
Spiritual
Delayed effects

Support groups

12/10/2015

sumaiyah@iium.edu.my

69

Nursing Management:

Evaluation
Cope effectively with diagnosis,
treatment regimen, and prognosis
Attain and maintain adequate
nutrition
Experience no complications
Feel comfortable and supported

12/10/2015

sumaiyah@iium.edu.my

70

References
Hockenberry M.J., Wilson D., Winkelstein M.L. & Kline
N .E. (2007) Wongs Nursing Care of Infants and
Children. Missouri, Mosby.
Kyle, T. and S. Carman (2014). Essentials of Pediatric
Nursing, Lippincott Williams & Wilkins.
Hockenberry, M. J. and D. Wilson (2013). Wong's
Essentials of Pediatric Nursing, Elsevier Health
Sciences

12/10/2015

sumaiyah@iium.edu.my

71

Exercise 8
Shelly, 10 months old is admitted to pediatric ward after
being brought to the clinic by her father for a small
laceration that he thought needed stitches. Her father
states that I didnt think the cut was very deep but I
was supervised by how long it bled.
What additional health history & physical
examination you should obtained?
Describe two priorities nursing diagnosis depend
on your assessment and discuss nursing
intervention followed by health education

12/10/2015

sumaiyah@iium.edu.my

72

Istighfar moments
Dont forget to use the last 5-10 minutes
before Fajr prayer to ask Allah for
forgiveness and making lots of istighfar so
that you are among those Allah mentioned
in the Quran: "And in the hour of early
dawn, they (were found) praying for
Forgiveness"
(Quran, Chapter 51, Verse 18)

12/10/2015

sumaiyah@iium.edu.my

73

Thank you

Any Question?
12/10/2015

sumaiyah@iium.edu.my

74