Blood Disorders in children. For Nursing Students

1. Disorders of Blood
1. Anemia
2. Thalassemia
3. Hemophilia
4. Leukemia
5. Purpura

2. Anemia
It is defined as the reduction in
the volume of red blood cells
Reduction in the hemoglobin
concentration.
The Hb concentration goes below
the lower limit of the lower range
for age and sex

4. Classification
Based on Morphology Based on Etiology

5. Based on
Morphology
Microcytic Anemia:
Abnormally small RBCs
Normocytic Anemia:
Normal in shape
Macrocytic Anemia :
Abnormally large RBCs

6. Based on
Etiology
Anemia due to blood loss
Anemia due to impaired
red cell production
Anemia due to increased
red cell destruction

7. Anemia
due to
blood loss
Acute
posthemorrhagic
anemia
Chronic
posthemorrhagic
anemia

8. Anemia due to impaired red cell production
A. Deficiency of substances needed for erythropoiesis
- Iron deficiency
- Vitamin B12 and folate deficiency anemia
B. Disturbance of proliferation and differentiation of stem cells
- Aplastic anemia
- Aplasia of pure red cells
C. Disturbance of bone marrow disease or due to systemic disease
- Anemia due to infection - Anemia due to renal diseases
- Anemia in liver disease - Anemia in endocrinopathies

9. D. Anemia due to bone marrow dysfunction
- Myelosclerosis
- Multiple Myeloma
E. Congenital Anemia
Anemia due to impaired red cell production cont..

10. Anemia due to increased destruction of RBCs
A. Anemia due to intracorpuscular defect
- Sickle Cell Anemia
- Thalassemia
B. Anemia due to extracorpuscular defect
- HDN
- Effect of toxic agents
- Poison or venoms
- Burn/ Thermal injury
- Blood transfusion reaction

11. Diagnosis
Hemoglobin Estimation
Peripheral blood smear
Bone marrow assessment
MCV- Mean Corpuscular Volume
MCH – Mean Corpuscular Hemoglobin
MCHC- Mean Corpuscular Hemoglobin
Concentration

12. Thalassemia
• Thalasa: Sea (in Greek)
• Found in people living around Mediterranean Sea.
• It is an inherited blood disorder characterized by deficiencies in the
rate of production of specific globin chains in Hemoglobin.
• It is a group of inherited disorders of hemoglobin synthesis
characterized by reduced or absence of one or more of the globin
chains of adult hemoglobin

13. Hemoglobin
structure

14. Prevalence in India
• India has a huge burden with an
estimated 100,000 patients with β-
Thalassemia.
• Commonly involved communities:
Sindhis, Punjabis, Baniyas,
Gujaratis, Benglais, Gaurs,
Banushali and Rajputs
• States:
High: Punjab, Delhi, Haryana,
Gujarat- (10 to 20%)
Moderate: Rajasthan, UP, West
Bengal, MP (5 to 10%)

17. Alpha Thalassemia
• Occurs when some or all 4 genes that make hemoglobin are missing or
damaged.
4 types:
1. α- Thalassemia silent carrier
2. α- Thalassemia carrier
3. Hemoglobin H disease
4. α- thalassemia Major

18. Beta Thalassemia
•β- thalassemia is caused by damage or missing genes. Two
specific genes are involved in the forming the Beta chain.
• Types:
* β- thalassemia major (Cooley’s Anemia)
* β- thalassemia minor or trait
- Thalassemia minima
- Thalassemia intermedia

19. Inheritance of β-Thalassemia
R r r
R
R
R
R R r r
r r
Unaffected, 1:4 Unaffected carrier, 2:4 Affected, 1:4
Unaffected
carrier mother
Unaffected
carrier father
r Affected Gene
R Unafffected
Gene

20. Risk
factors
Family History of thalassemia in
parents.
Consanguineous marriage
Ancestry : African Americans,
Mediterranean and Southeast
Asian decent

21. Clinical Features
• Failure to thrive
• Anemia
• Jaundice
• Gall stones
• Hepatosplenomegaly
• Growth retardation
• Skin bronzing
Initial symptoms are exhibited between 3 to 6 months

22. Clinical features cont.
• Bone Abnormalities
- Frontal bossing
- Depression of bridge of nose
- Generalized osteoporosis
- Brittle bones leading to
fracture
- Deformed facial bones

23. Pathophysiological changes
•Severe anemia, ineffective erythropoiesis
•Iron overload resulting from transfusion and increased iron
absorption.
•Skin shows Pallor from Anemia and Jaundice from
hyperbilirubinemia
•Cardiac examination: Shows Heart failure and arrhythmias

24. •Abdominal examination: Shows changes in liver,
gallbladder and spleen
•Patients receiving regular blood transfusion may have
hepatosplenomegaly or chronic hepatitis due to iron
overload
•Gall bladder may contain bilirubin stones
•Splenomegaly: due to extramedullary hematopoiesis

25. Diagnosis
•Chorionic Villus Sampling at 11th
week of pregnancy to identify the
genes.
•Amniocentesis

26. Hemoglobin
types

27. Hb Electrophoresis

28. •Hemoglobin estimation
•Complete Blood Count
•X- rays

29. Treatment
1. Blood Transfusion
Lab Criteria: Hb < 7g/dl on 2 occasions, > 2 weeks apart
Lab with clinical criteria:
- Hb > 7g/dl
- Facial changes
- Poor growth
- Fractures

30. 2. Surgical Management
Splenectomy: decreases transfusion requirements
Cholecystectomy : To remove the bilirubin stones

31. 3. Iron Chelation : To treat Hemosiderosis (1 unit of blood = 200 mg of Iron)
- Excess iron is stored in various organs.
- Cardiomyopathy (70% of death in Thalassemia)
- It is the treatment through which excess iron from the blood is removed
- Iron build up occurs as a result of regular transfusions
- Removing this excess iron is very vital for your health.
Drugs which help in chelation:
- Deferoxamine (DFO)
- Deferiprone, 1998
- Deferasirox, 2005 - OD
The proper use of the three chelators will
improve the prevention and treatment of iron
overload, it will reduce complications, and
improve survival and quality of life of transfused
patients.

34. 4. Supplements
Folic Acid
Calcium
Zinc

35. 5. Stem cell or Bone marrow transplantation
- For severe thalassemia, it can eliminate
the need for lifelong blood transfusion and iron
chelation.
The cost of doing a BMT in India can vary from
₹ 15 lakhs to ₹ 40 lakhs

36. Bone marrow Transplant
• Done for children with severe Thalassemia as it can eliminate the
need for lifelong blood transfusions and iron chelation
• Types
- Autologous transplant
- Allogenic transplant
> Matched : Related or unrelated donors
> Half- matched or Haploidentical donors

38. Steps of BMT
Step 1: Preparation
•Finding the right
donor
•Collecting the stem
cells
Step 2: Conditioning
•Chemotherapy
or
•Radiation therapy

39. Step 3: Transplant
- Infusing stem cells into the patient using a central venous
catheter
e.g. PICC , Subclavian line, Femoral line, Internal Jugular line
- The transfusions are continued in intervals until target blood
count is achieved
Step 4: Waiting engraftment
- Cancer-free, healthy blood cells begin to grow.
- Regular blood tests and transfusion until full recovery.
- It takes 2 to 6 weeks to return to normal blood counts.

40. • Recovery after BMT
1. Allogenic transplant : 3 to 6 months
2. Autologous transplant: 12 to 18 months
• Precautions
- Regular follow up and blood test
- Always wear a mask
- Frequent hand washing
- Avoiding crowded places
- Healthy well-balanced diet
- Maintain personal hygiene
80%
of Bone
Marrow
Transplants
are Successful
In India

41. Living with
Thalassemia : A
Health Teaching
•Survival and quality of life has
improved with better
treatment options
Approaches to help cope
- Follow the treatment plan
- Get regular follow up

42.  Monthly CBCs
Blood iron every 3 to 6 months
Cardiac function test
Thyroid function every 6 months
 Growth and development
assessment

43. Maintenance of Hygiene and reduce Infection
Care of teeth
- Use soft brush and good tooth paste
- Brush your child’s teeth with short strokes
in morning and before going to bed.
- Wash mouth with ordinary water.

44. Care of Skin
- Take bath daily, preferably in the morning and after play
- Use soap which is not irritating to the skin
- Use soft towel to clean the body, don’t rub vigorously
- Use clean clothes every day

45. Care of nails and hands
- Cut child’s nail after bath as it becomes softer
- Wash hands before eating and after using toilet
- Promptly report any infection

46. 4. Nutritional Management
Food Items Rich source of Iron Low source of Iron
1. Cereals Bajra, Poha and Jowhar Rice, Maize, Makai
2. Pulses Bengal Gram, Soyabean, Rajma,
Green gram
Masoor, Red gram, Peas
3. Vegetables Green leafy vegetables: Spinach,
ridge gourd, drumstick, cauliflower
Cabbage, Sweet potato, Lady
finger, Pumpkin, Cucumber
4. Fruits Fig, Chickoo, Watermelon,
Pineapple
Pomegranate, grapes, amla,
apple, guava, orange
5. Non Vegetarian Meat, Liver, Yellow egg yolk Pomfret, Fish (Rohu)
6. Others Jaggery, Almonds, Dates, Dry Fruits Milk and milk products

47. What to eat? What not to eat?
At least 3 glasses of milk a day Avoid green leafy vegetables, black gram,
coriander, potato, pudina (mint), ginger,
mustard, pista, broccoli, spinach and peas.
Black tea or coffee after every meal. It
decreases iron absorption
Avoid red meat, raisins, yeast, germinating
foods, liver, pork, peanut butter
Calcium tablets after 10 years of age or give
lots of milk
White portion of boiled egg
Fruits such as papaya, apple, oranges or one
of any seasonal fruit per day.

49. Hemophilia
• It is a group of
hereditary genetic
disorder that impair
the body’s ability to
control blood
clotting or
coagulation.

51. SUMMARY OF CLOTTING PROCESS

52. History of Hemophilia
• Hemophilia is also referred to as Royal disease
• Because it was common among the royal
families of England, Germany, Russia and Spain.
• Queen Victoria of England is believed to have
been the carrier of Hemophilia B. She passed
the trait on to 3 of her 9 children.
• Her Son died of hemorrhage at the age of 30
years due to a fall.

53. Definition
Hemophilia is a hereditary bleeding disorder, in which there is a
partial or total lack of an essential blood clotting factor.
It is a lifelong disorder, that results in excessive bleeding and
spontaneous bleeding, which very often is internal.

54. Incidence
• Hemophilia A is the most
common form ( 1 in
10000 male births)
• Hemophilia B occurs in 1
in 35000 male births.
• It is a recessive sex
linked, X chromosome
disorder.

55. Etiology
•Genetic Mutation
Mutation of the genes that provide instructions for making
the clotting factor proteins need to form blood clot.
Theses genes are located on the X chromosomes.

56. Inheritanc
e
1

57. Inheritanc
e 2

58. Inheritance
3
(Very rare)

59. Types of Hemophilia
Hemophilia A or Classic Hemophilia
Hemophilia B or Christmas Disease
Hemophilia C

60. Hemophilia A or Classic Hemophilia
Low levels or completely missing factor VIII
80% of people with hemophilia have Hemophilia A
Usually manifested in Males
No family history in 30% of cases but spontaneous genetic mutation
Severe

61. Hemophilia B or Christmas Disease
Low levels or completely missing factor 9
20% of hemophiliacs
30% of cases of Hemophilia B is due to spontaneous
genetic mutation.
Moderate form

62. Hemophilia C
Low levels or completely missing Factor XI
May be present in both males and females
Mild form

63. Types based
on Severity

64. •80% of hemophiliacs are considered severe. They have
excessive bleeding and can have spontaneous bleeding
episodes.
•10% of hemophilia patients are considered moderate. They
may have spontaneous bleeding episodes and have
prolonged bleeding after an injury
•10% of hemophilia patients are considered mild. They may
have prolonged bleeding post injury or trauma or surgery.
Not discovered until adulthood.

65. Sign and Symptoms of
Hemophilia

66. General symptoms
Bleeding into joints/ muscles causing
pain and swelling
Frequent Epistaxis
Bleeding gums
Hematuria
Easily gets bruised

67. External bleeding which includes
- Bleeding in mouth from cut, bites or tooth loss.
- Nose bleed without a reason
- Heavy bleeding from small cuts
- Bleeding from a cut that occurs again after stopping.
Internal Bleeding
- Hematuria (Bleeding in kidneys or UB)
- Malena
- Large bruises

68. Bleeding in the joints
- Hemarthrosis (Knees and elbows)
- Tightness in joints ( due to bleeding)
- Arthralgia (Swollen, hot, painful joint)
Bleeding in the Brain
- Painful headaches
- Stiffness in neck
- Sudden loss of strength in arms and legs (Clumsiness)
- Double vision
- Convulsions

69. Diagnostic measures
•Family History
•Screening tests
- Complete Blood Counts
- Activated Partial Thromboplastin Time Test
- Prothrombin Time Test
- Fibrinogen test
•Clotting factor test

70. Treatment
1. Clotting factor replacement
- Infusion with clotting factor concentrate into a vein.
- Helps in treating bleeding episodes
- Prevent bleeding
- Prevent complications
2. Desmopressin (DDAVP) for HA
- Synthetic hormone for production of factor VIII

72. 4. Gene Therapy
-Researches are still going on to develop methods to
correct the defective genes.
-Clinical trials are going on

73. 5. Antifibrinolytics
- Medications are used with replacement therapy
- Help keep blood clot from breaking down
- Given before a dental procedure
- To treat oral, nasal or mild intestinal bleed
e.g. Tranexamic acid, Aminocaproic acid
6. Other medicines
- Analgesics
- Steroids
- Physical therapy

74. Prevention of complications and hemorrhages
• Avoiding IM Injections
• Avoid Contact sports
• Take appropriate measures to control bleeding
- RICE, Lean forward for epistaxis
• Prevent joint degeneration
- Immobilize joint during acute bleeding
- Progressive exercise
- Avoid prolonged immobility

75. Nursing
Diagnosis
Risk for deficient fluid volume related to
hemorrhage
Ineffective protection related to inability
of forming blood clot
Impaired physical mobility related to
bleeding into joints.
Acute pain related to bleeding into joints
and muscles
Ineffective family coping related to
disabling and life-threatening disease.

76. Leukemia
• Leukemia is a broad term given to a group of malignant disease of the
bone morrow and lymphatic system.
• Leukemia is an unrestricted proliferation of immature WBCs in the
blood- forming tissue of the body
• Leukemia demonstrates neoplastic properties

77. Etiology
•Exact cause is unknown
•Viruses: Human Papilloma Virus, Epstein- Barr Virus
•Radiations
•Exposure to chemicals
•Family history
•Chromosomal abnormalities

78. Classification
Acute Chronic
Acute Lymphoid
Leukemia (80%)
Acute Myeloid
Leukemia (10- 20 %)
Chronic
Lymphoid
Leukemia
Chronic
Myeloid
Leukemia
(2-3%)
T Cell B Cell Null
Cell
Pre B
Cell
Acute
Myeloblastic
Acute
Promyelocytic
Acute
Erythrocytic
Acute
Monocytic
Acute
Myelomonocytic

79. Pathophysiology
Uncontrolled Proliferation of leucocyte precursors
Competition for nutrients, infiltration of organs & replacement of normal cells by
leukemic cells
Bone marrow
dysfunction
Central Nervous
System
Reticuloendothelial
system
Generalized
Hypermetabolism
RBCs WBC Platelets
Anemia Haemorrhage
Infection
L. Meningitis
Enlarged
liver, lymph
nodes &
Spleen
Cellular
starvation

80. Acute Lymphoid Leukemia
•Most commonly diagnosed Cancer in Children
•80% of all childhood leukemia
•T cell : Poor prognosis (10-15%)
•B cell: Poor prognosis
•Pre B cell: Good prognosis
•Null cell: Most common (75%), better prognosis than others

81. Acute Myeloid Leukemia
• Abnormal proliferation of monocytes and myelocytes in bone marrow
• 15% children with poor prognosis
• Clinical features
- Recurrent infections
- Fatigue
- Lymphadenopathy
- Hepatosplenomegaly
- Bone – Joint pain
- Thrombocytopenia
- Frequent bruising

82. •Features of CNS involvement
- Headache
- Blurred vision
- Fundal haemorrhage
- Paresis

84. Diagnostic Evaluation
• Suspected from history, physical manifestation and peripheral blood
smear.
• Confirmation: Bone marrow examination
- 60-100% immature white blood cell
• Radiological finding: Mediastinal mass
• Liver Function Test
• Renal Function Test
• LP to assess CNS involvement
• Cytomorphologic studies to categorize

85. Management
Systemic Chemotherapy with or without irradiation
CHEMOTHERAPY : It is given in 3 phases
A. Induction Phase:
Reduction of leukemic cells to an undetectable level (Remission)
Takes 4 weeks to achieve
Drugs:
ALL: Prednisolone, Vincristine, L-asparginase, Doxorubicin
AML: Cytarabine and Daunorubicin

86. B. Consolidation Phase
Eradicating any residual leukemic cells
Started after remission is achieved
Drug: Metotrexate
High dose radiation treatment for 2 week

87. C. Maintenance Therapy
Aims at preventing recurrence and further reducing no. of
leukemic cells
Drugs
- 6 Mercaptopurine
- Methotrexate PO
Weekly CBC

88. Bone Marrow Transplantation

89. Side effects of Chemotherapy
•Nausea and Vomiting
•Anorexia
•Mucosal Ulceration
•Neuropathy
•Haemorrhagic Cystitis
•Alopecia
•Mood Changes

90. Assignment
Explain the Nursing management
for leukemia and the nursing
interventions during chemotherapy
and manging its side effects

91. Purpura
•It is a bleeding disorder
•Characterized by
- Petechiae
- Ecchymosis
•Bleeding from capillaries under
skin

92. Two types:
A.Idiopathic Thrombocytopenic Purpura
B.Anaphylactoid Purpura

93. Idiopathic Thrombocytopenic Purpura
• Acquired disorder
• Haemorrhagic condition involving skin, mucus
membrane and internal organs
• "Idiopathic" means the cause is
unknown.
• "Thrombocytopenia" means a
decreased number of platelets in the
blood.
• "Purpura" refers to the purple
discoloring of the skin, like a bruise.

94. Pathophysiology
Autoimmune disorder
Antibodies against platelets
Antibodies destroy the platelets leading to platelet
deficiency

95. Clinical Features
• Petechiae : prominent over legs
• Ecchymosis
• Bleeding from gums
• Epistaxis
• Anemia
• Internal hemorrhage : Hemetemesis, malena, hematuria
• Hemarthrosis
• Low grade fever
• Platelets < 5000/mm3 : Chances for intracranial hemorrhage

96. Diagnostic evaluation
•Platelet count
•Bleeding time and clotting time may be
increased
•Peripheral blood smear
•Bone marrow examination

97. Treatment
•NO need of treatment if platelet above 50,000/mm3
•Count < 20,000 / mm3 is an indication of treatment
- Steroids: Supresses the immune system (e.g. Dexamethasone,
prednisolone)
- Anti D antibodies: Immunoglobulin to destroy the antibodies
- Steroid sparing agents : for immunosuppression ( e,g, mofetil)
- Immunoglobulins: Costly and short term
- Platelet infusion

98. Anaphylactoid Purpura
•A disorder causing inflammation
and bleeding in the small blood
vessels (Age < 7 years)
•Symptoms include reddish-purple
spots on the lower extremities,
swollen and sore joints, abdominal
pain or bloody urine.
•Treatment: Steroids

99. Pathophysiology
Hypersensitive immunologic reaction
Vasculitis of small blood vessels in upper dermis, GIT, synovial join
and renal glomeruli
Extravasation of erythrocytes
Petechial haemorrhage

100. Clinical Features
• Erythromatous petechial rashes over buttocks and lower
extremities
• GI Symptoms
- Colicky abdominal pain
- Vomiting with or without hematemesis
- Intussusception
• Synovial membrane: arthralgia, hemarthrosis
• Renal : Hematuria, Proteinuria, Hypertension
• Nervous system: Convulsions, paralysis and coma (Rare)

101. Management
Symptomatic management
Antibiotics to prevent bacterial
infections
Short term steroid therapy –
Prednisolone
Analgesics

102. Goals of
Nursing
Management
Minimize chances of
trauma to the child
Control bleeding
Administering the
prescribed medicines

103. Thank you