Disease condition and management

1. Purpura, leukemia

2. PURPURA
DEFINITION
• Purpura is a bleeding disorder characterized by
petechiae & ecchymosis which may be due to either
deficiency in number or quality of platelets or defect in
vascular endothelium
• Purpura occurs due to bleeding from capillaries, under
the skin

4. Types
Purpura is of two types
A. Idiopathic Thrombocytopenic Purpura
B. Anaphylactoid Purpura

5. Idiopathic Thrombocytopenic
Purpura
Definition
• Idiopathic Thrombocytopenic Purpura, the most common
acquired thrombocytopenic disorder in children is a
hemorrhagic condition involving skin, mucous membrane
& internal organs.
• It is most likely an autoimmune response of the body
Incidence: 3-7 yrs of age

6. Pathophysiology
Normally the platelets are formed from megakaryocytes in bone
marrow
In 60% cases of purpura, the cause is autoimmune, with antibodies
against platelets
Most often these antibodies are against platelet membrane
glycoproteins and are of immunoglobulin G (lgG) type
After binding of the antibody to the platelet surface, circulating antibody
coated platelets are recognized by the Fc receptors or splenic
macrophages, ingested and destroyed
leading to platelet deficiency

8. Clinical Features
• The child with Purpura may present with:
• Petechiae (prominently over legs)
• Ecchymosis
• Bleeding from gums
• Epistaxis
• Anemia (proportional to bleeding)
• internal hemorrhage evidenced by hematemesis, melena and
hematuria
• Hemarthrosis (bleeding in joints)
• Low grade fever
• If platelet count goes below 5000/mm3,intracranial or intracerebral
hemorrhage may occur as a complication.

9. Diagnostic Evaluation
• Diagnosis is based on the following laboratory tests:
• Platelet count : platelet count may be below
20,000/mm3 (normal count is 1.5-4.5 lakhs/mm3)
• Bleeding time and clotting time may be increased
• Peripheral blood smear-Giant forms and deeply
stained platelets are seen
• Bone marrow examination: Abundant platelet
precursors are seen in bone marrow

10. Management
• There is usually no need of treatment for patients with a platelet
count above 50,000/mm3.
• A count below 20,000/mm3 is an indication for treatment includes-
1. Steroids
• The first line of treatment usually consists of steroids, which
suppress the immune system.
• The dose and route of steroid administration is determined by
platelet count and whether there is active bleeding.
• In emergency, infusion of dexamethasone or methyl prednisolone
may be used.
• Once the platelet count has improved, the dose of steroid is
gradually reduced while monitoring for relapses.
• Long term steroids are usually avoided, as they can cause
numerous side effects like cataract, diabetes and osteoporosis.

11. 2.Anti D antibodies
• Another line of treatment for Rh- positive patients is the
use of Anti D immunoglobulin, which is given
intravenously
3.Steroid Sparing agents
• Immunosuppressants like mycophenolate, mofetil and
azathioprine are being used now a days due to their
effectiveness.

12. 4. Immunoglobulins
• Intravenous Immunoglobulins may be infused in
some cases.
• It is costly and produces short term
improvement.
5.Platelet Infusion
• Platelet infusion may be done in emergency
situation, but is unsuccessful in producing long
term platelets count increase.

13. ANAPHYLACTOID PURPURA
• Generalized systemic vasulitis of unknown etiology is
known as Schonlein-henoch vasculitis.
• It is characterized by deposition of immune complexes
containing antibody lgA.
• Anaphylactoid purpura is a systemic inflammatory
disorder, seen primarily in children between 2-8 year of
age.
• Boys are affected twice as frequently as girls.

14. Etiology
• Children who develop anaphylactoid purpura have an
increased incidence of atopic disease and recent upper
respiratory infiction.
• Although the exact etiology is unknown, the distinctive
clinical manifestations suggest a hypersensitive
immunologic reaction to Group-A streptococcus resulting
in localized or widespread vascular damage.
• Possible precipitating causes include various drugs
(penicillin aspirin), food (chocolate, milk, eggs) insect
bite and bacterial or viral infections.

15. Pathophysiology
Hypersensitive immunologic reaction leads to vasculitis of
small blood vessels in upper dermis, GIT, synovial joints
and renal glomeruli.
Due to the inflammation of blood vessels, there is
extravasation of erythrocytes
petechial hemorrhage and other clinical features.

16. Clinical Features
• The onset of purpura may be acute or insidious.
• There is vasulitis in upper dermis, GIT, synovial
membrane and renal glomeruli.
• Clinical features are follows:
• Anaphylactoid purpura usually begins with
erythematous petechial rashes appearing symmetrically
over the buttocks and lower extremities that may
progress to large palpable ecchymotic areas.

18. • Gastrointestinal symptoms include-
• Colicky abdominal pain
• Vomiting with or without hematemesis
• Intussusception
• Synovial membrane vasculitis leading to
arthralgia is characterized by warm swollen and
painful joints.
• Usually knee and ankle joint are affected

19. • Renal manifestation include-
• Hematuria
• Proteinuria
• Hypertension
• Hemorrhagic complications involving the
central nervous system include seizures,
paresis and coma, but they occur rarely.

20. Diagnostic Evaluation
• Anaphylactoid Purpura is usually
diagnosed based on typical skin, joint and
kidney findings.
• Throat culture, urinalysis and blood test for
inflammation and renal function are used
to suggest the diagnosis.

21. Management
• Medical management of Schonlein-henoch vasculitis is
essentially symptomatic, since there is no specific
treatment
• Antibiotics are given if a specific bacterial infection is
identified.
• Short term steroid therapy (usually prednisolone) may
be give to suppress acute manifestations and relieve
joint and abdominal pain.
• Analgesics like Acetaminophen are give for pain relief.

22. Nursing Management
• Nursing management for patients with
Purpura focus on these aspects:
a. Minimize chances of trauma to the child
and provide safe environment
b. Control bleeding
c. Administer the prescribed drugs

23. A.Minimize chances of trauma to the child
• Safe environment should be provided to the child to
protect him from injury.
• The side rails of cradle should be padded.
• Avoid using hard tooth brush for the child. Use light
mouth wash or glycerin swabs for cleaning the child's
mouth.
• Provide soft and nutritious diet to the child.
• Make the child wear loose garments.
• Give smooth edged or soft toys to the child

24. • Do not give intramuscular or intravenous injection and
prevent frequent venipunctures.
• Avoid elective surgeries.
• B. Control bleeding
• First aid needs to be given during a bleeding episode.
• If these measured do not stop bleeding, other treatment
will be needed, following first aid measure may reduce
the amount of bleeding:
• R= Rest: Make the child lie down quietly until bleeding
episode ends.
• I= Ice: Apply an ice pack on the bleeding area.

25. • C= Compression: Apply pressure over the bleeding
site. A bleeding join may be wrapped with an elastic
compression bandage.
• E=Elevate: Position the child so that the bleeding area is
raised. Raise the area above the level of heart if
possible.

26. Administration of Prescribed Drugs
• In order to relive sever joint pain, Acetaminophen is
administered.
• Corticosteroids such as prednisolone may be give as
prescribed by the physician to minimize hemorrhagic
manifestations.
• Monitor the patient for side affect of steroid therapy like
cataract, increased body weight, hyperglycemia, etc.

27. Prognosis
• The prognosis of Purpura is good if
adequate supportive care is give and
complications (such as intracranial
hemorrhage) are prevented .
• Most of the patients recover within 2-3
months of onset.

28. LEUKEMIA

29. Introduction
Leukemia is the most common type of cancer in
children. All cancers begin in cells of the body,
and leukemia is a cancer that begins in blood
cells. Normally, cells grow and divide to form
new cells as the body needs them.
When cells grow old, they die and new cells take
their place. Sometimes, this process does not
work right. In cancer, new cells form when the
body does not need them, and old cells do not
die when they should.

30. LEUKEMIA
Definition
• Leukemia (Greek leukos "white"; aima "blood")
is a cancer of the blood or bone marrow
characterized by an abnormal increase of blood
cells, usually leukocytes (white blood cells).
• Leukemia is defined as uncontrolled neoplastic
proliferation of leucocyte precursors.

32. Incidence
• Leukemia is the most common malignancy
of children less than 15 years of age.
• The Peak incidence is at 4 years of age.
• Incidence in males is greater than in
females.
• It is twice more common in white than
black children.

33. Etiology
• The exact cause of leukemia is unknown but several
etiologic factors have been identified including:
• Viruses like Human papilloma virus, Epstein-Barr virus
• Radiations
• Exposure to chemicals and drugs like benzene and
dilantin
• Familial predisposition
• chromosomal abnormalities

34. Classification
Leukemia
Acute
Acute Lymphoid
Leukemia (80%)
T cell B cell Pre B cell Null cell
Acute Myeloid
Leukemia (20%)
Acue
Myeloblastic
Acute
Promyelocytic
Acute
Myelomonocytic
Acute
Monocytic
Acute
Erythrocytic
Chronic
Chronic Lymphoid
Leukemia (absent
in children)
Chronic Myeloid
Leukemia (2-3%)

35. • Leukemia can be classified according to predominant
cell type and level of cell maturity as:
• Lympho: It means leukemia involving the lymphoid
series
• Myelo: It means leukemia of myeloid (bone marrow
origin) series
• Blastic: It involves immature cells
• Cytic: It involves mature cells

36. Unknown aetiology
Somatic mutation in gene
Deactivate tumour suppressor gene
Malignant transformation of lymphoblast in
the bone marrow
Uncontrolled proliferation of lymphoblast in
bone marrow
Lymphoblasts replace the normal marrow
element

37. Uncontrolled proliferation of leucocyte precursors
Competition for nutrients, infiltration of organs & replacement of normal
cells by leukemic cells
Bone marrow reticuloendothelial Central nervous Generalized
Dysfunction system system hypermetabolism
RBCs WBC Platelets Enlarged liver, Leukemic Cellular
lymph nodes & meningitis starvation
Anemia Infection Hemorrhage Spleen

38. Acute Lymphoid Leukemia(all)
• Acute lymphoid or acute lymphocytic leukemia is the
most commonly diagnosed cancer in children, which
accounts for 80% of all childhood leukemias.
• Many etiologic factors have been implicated, that might
increased the risk of developing leukemia like viruses,
irradiation, exposure to certain toxic chemical and drugs
and a genetic predisposition.
• Acute lymphocytic leukemia develops when lymphoid
cell line is affected.

39. Types of ALL
A. T cell Leukemia
• It is seen in 10-15% cases of acute lymphocytic
leukemia.
• It is seen in older children, particularly males. It has the
following features-
• Mediastinal mass
• Hepatosplenomegaly
• High WBC count
• CNS involvement may be seen
• poor prognosis

40. • B- cell leukemia
• it is seen in 1-2% children with ALL . It is an aggressive
form and has poor prognosis
• Pre- B cell
• It has good prognosis and responds well to therapy
• Null cell leukemia
• it is the most common type of childhood ALL, occurring
in 75% cases. It has better prognosis than other types.

41. ACUTE NON- LYMPHOID LEUKEMIA/
ACUTE MYELOID LEUKEMIA(AML)
• Acute non- lymphocytic leukemia is abnormal
proliferation of monocytes and myelocytes in bone
marrow.
• It is present in approximately 15% children with
leukemia. It has a poor prognosis.
• It includes the following subtypes:
a. Acute myeloblastic leukemia
b. Acute promyelocytic leukemia
c. Acute myelomonocytic leukemia
d. Acute monocytic leukemia
e. Acute erythrocytic leukemia

42. Clinical Features
• The clinical features of AML are:
• Recurrent chronic infections
• Fatigue
• Lymphadenopathy
• Hepatosplenomegaly
• Bone or joint pain
• Pallor
• Frequent bruising
• Gingival hypertrophy may be present

44. • Feature of CNS involvement like headache, blurred
vision, fundal hemorrhage and paresis.
• Other life threatening problem like sludging and clumping
of leukemic cells in small cerebral capillaries and
hyperuricemia
• Thrombocytopenia is also present.

45. Diagnostic Evaluation
• History Collection
• Physical Examination & Clinical manifestations
• Peripheral blood smear showing immature leucocytes
frequently combined with low normal blood counts.
• Bone marrow Examination: A leukemic marrow is
hypercellular, with 60-100% immature white blood cell
reducing in normal marrow component .
• A finding of more than 25% of abnormal lymphoblasts is
diagnostic

46. • Blood investigations reveal an elevated serum uric
acid level due to increased turnover of malignant cells.
• Radiologic studies are done to evaluate presence of
mediastinal mass.
• Liver and renal functions tests are done to detect
leukemic cells infiltration in liver and kidenys.
• Lumbar puncture is done to assess CNS involvement.
• Cytomorphologic, cytochemical and immunologic studies
to categorize the disease into sub groups.

47. Management
i. Treatment of leukemia involves systemic chemotherapy
with or without cranial irradiation.
A. chemotherapy: it is given in three phases-
Induction Phase
• This is the phase that reduce leukemic cells to an
undetectable level, a state known as remission.
• In remission, there is no evidence of leukemia on
physical examination, bone marrow evaluation,
peripheral blood smear, CSF examination or
examination of other extramedullary sites.
• 95% children with leukemia achieve remission during
induction, within 4 week.

48. • Drugs used for induction in all are Predinisolone,
Vincristine and L-Asparginase with or without in
doxorubicin. In AML for induction drugs like Cytarabine
(ara-c) and Daunorubicin are used.
• Leukemic cells can cross the blood- brain barrier while
most chemotherapy drugs, do not cross this barrier.
• Children with ALL receive CNS prophylaxis with
chemotherapy drugs instilled intrathecally, into the
cerebrospinal fluid space during lumbar puncture.
• Children who present with CNS disease also need
radiation therapy.

49. Consolidation Phase
• The next phase of treatment is consolidation or
intensification that aims at eradicating any residual
leukemia cells.
• This phase of therapy begins once remission is attained.
• Treatment is directed at those anatomic sites which are
protected to some extent from systemic chemotherapy
like CNS (protected by blood- brain barrier) and testes
(that lie outside the body)
• This phase involves prophylactic treatment of CNS with
cranial irradition and or intrathecal administration of
Methotrexate.
• The therapy usually consists of daily high dose radiation
treatment for about 2 weeks or twice a week doses of
Methotrexate 3 total of 5-6 injections.

50. Maintenance therapy
• Maintenance therapy aims at preventing recurrence and
further reducing the number of leukemic cells.
• Maintenance therapy begins after successful completion
of induction and consolidation phase.
• Drugs frequently used in maintenance therapy are 6-
mercaptopurine and weekly doses of oral Methotrexate.
• During this phase weekly or monthly complete blood
count is done, to evaluate marrow’s response to drugs.
• If WBC count goes below 2000/mm3 or toxic side effect
occur, then therapy is temporarily stopped or dose is
reduced.

51. B. Bone marrow transplantation
• Bone marrow transplant have been used successfully in
treating some children with ALL and AML.
• In general bone marrow transplant is not recommended
for children with ALL because of excellent result possible
with chemotherapy.
• Marrow transplant is possible only when suitable donor
is available.
• Prognosis after transplantation depends on type of
leukemia.
• Long term survival after marrow transplant is seen in 25-
50% cases.

52. Nursing Management
• Nursing management focuses on managing the
problems of leukemia and side affects of chemotherapy.
a. Management of Problem of Leukemia
• Infiltration of bone marrow with leukemia cells, leads to
myelosuppression resulting in reduced RBC,WBC and
platelet production.
• This ultimately leads to anemia, infections and chances
of hemorrhage. Nursing management of these problems
is an follows-

53. i. Management of Anemia
• Nurses should assess the leukemia children for anemia
and its severity.
• Blood transfusion with packed red cells may be required
to raise hemoglobin level above 10gm/dl.
• Nurse should take all precaution to prevent problem
associated with blood transfusion like transmission of
blood bone infection to the patient through transfusion of
contaminated blood, transfusion reaction etc.
• Nurses should monitor the patient continuously during
blood transfusion

54. ii. Prevention from Infections
• Infection is the most frequent casus of death in
leukemia.
• Organisms most commonly infecting these children are
varicella, herpes zooster, MMR and polio virus, protozoa
like pneumocystis carnii and fungi like candida alibicans.
• Following measures must be taken to prevent infections-
• Broad spectrum antibiotics are used prophylactically
• Live vaccines should not be given to leukemic children,
as they are already immuno compromised.

55. • When the child is in hospital, universal precautions are
used along with isolation, barrier nursing, strict hand
washing and aseptic techniques.
• Fever is a sign of infection , so if fever occur blood urine,
stool and nasopharyngeal cultures are done, to identify
the cause and site of infection.
• An adequate protein and calorie intake provide the child
with better host defenses against infection and increases
tolerance to chemotherapy.

56. iii. Prevention and Management
of Chemotherapy
• Most of the bleeding episode can be prevented by administration of
platelet concentrates.
• Regular mouth care is essential since gingival bleeding is frequently
seen
• The child may be provide with soft tooth brush or only mouth wash
may be used to rinse the mouth.
• These children develop repeated episode of diarrhea, so they are
prone to rectal ulceration.
• Provide perineal care after each loose stool and keep the area
clean and dry.
• During a bleeding episode, the parents and child need emotional
support.
• Children are kept away from activities that might cause injury or
bleeding.

57. b. Management of side effect of
chemotherapy and problems of
irradiation
I. Nausea and vomiting
• For mild to moderate vomiting, antiemetics like
Promethazine (phenargan), chlorpromazine etc.
are used.
• Metacloperamide is administered for severe
vomiting.
• Antiemetics should be give before
chemotherapy is started (30 minutes to 1 hour
before chemotherapy) and then regularly at two
hours interval upto 24 hours.

58. ii. Anorexia
• Loss of appetite because of chemotherapy and radiation
therapy so:
• Give small frequent feeds to the child according to his
likes.
• Give soft and easily digestible food to the child
• Serve the food in an attractive manner.

59. iii. Mucosal Ulceration
• As side effect of chemotherapy, the mucosa of gastrointestinal tract
becomes ulcerated.
• Oral ulcers develop which make eating extremely uncomfortable so:
• Give bland, moist and soft diet to the child.
• Use soft tooth brush or cotton tipped applicator and mouth wash to
clean the child’s mouth.
• Frequent mouth wash may be provide with normal saline.
• Local anesthetics like lidocaine can be used to anesthetize oral
ulcers, before the child eats food.
• Liberal fluid intake is encouraged.
• Nasogastric feeds may be started in case of severe ulceration in
mouth.

60. iv.Neuropathy
• Vincristine and vinblastin can cause various neurotoxic
effect leading to foot drop, weakness and numbness of
extremities and reduced bowel movements
• Use foot board to prevent foot drop in bed ridden
children.
• The child suffers in from constipation.
• Regular bowel movement should be ensured by using
stool softeners and laxatives.
• Also fluid intake of the child must be increased.

61. V.Hemorrhagic Cystitis
• The drug Cyclophosphamide leads to hemorrhagic
cystitis.
• It can be prevented by:
• Liberal fluid intake.
• Motivate the child to void immediately on feeling the urge
to urinate.
• Administer chemotherapy drugs in the morning, to allow
for sufficient intake of oral fluids and frequent voiding or
urination.

62. vi. Alopecia
• Hair loss occur because of chemotherapy and cranial
irradiation, so:-
• Inform the parent and child about this side effect earlier.
• Encourage the parents to purchase a wig for child,
before hair fall occur.
• Child’s hair should be cut short and he should be made
to wear surgical cap to collect the fallen hair.
• Parents and child should be reassured that hair will grow
again after the treatment stops.

63. vii. Mood changes
• Shortly after starting of steroid therapy, children
experience mood changes which range from feeling of
wellbeing and euphoria to depression and irritability.
• Parents should be made aware of these behavior
changes.

64. C. Parents Support and
Guidance
• Nurses should continually guide, support and
help parent to adjust to this disease condition.
• Parents should be encourage to express their
feeling, fear, grief and concerns.
• Provide emotional support to the parents
continuously.

65. THANK
YOU