1. Child with blood, blood forming organs, lymphatic system

2. Terms Alopecia Christmas disease Ecchymosis Erythropoietin
Hemathrosis Hematoma
Hematopoiesis Hemosiderosis
Lymphadenopathy Oncologists
Petechiae Purpura
Respite care Sickle cell
Sickledex Splenomegaly
Can ask any of these terms on any of the test that you may take in this class

3. Blood, blood forming organs, or lymphatic disorders
Blood dyscrasias or disorders occur when components of the blood do not form in the normal fashion
Blood, plasma and other cells are formed in the second week of gestation in the yolk sac and then by the
Liver
Spleen
Thymus
Lymph system
And bone marrow
Any problem with any of these can cause the blood not to form correctly. RBC formation is formed in the bone marrow of long bones such as the femur and tibia. At adolescence Hematopoiesis takes place in other location such as the ribs, sternum, pelvis vertebra , skull, clavicle and scapula. The RBC production is regulated by erythropoietin which in the fetus is from the liver but then after birth it transfers over to the kidneys. The lymphatic system is a collection of vessels and organs that help the body control infection and aids in the production of antibodies. They are not palpable as infants but are palpable in childhood (cervical, axillary, and inguinal)

4. Anemia Can result from many different underlying causes
A reduction in the amount of circulating hemoglobin (HGB) reduces the oxygen-carrying ability of the blood
A HGB level below 8 g/dl results in an increased cardiac output and a shunting of blood from the periphery to the vital organs

5. Iron-Deficiency Anemia
Most common nutritional deficiency of children
Incidence is highest during infancy (from ninth to twenty-fourth months) and adolescent
May be caused by severe hemorrhage, inability to absorb iron received, excessive growth requirements, or an inadequate diet
What foods are highest in iron Boiled egg yolks, liver, leafy green vegetables, cream of wheat, dried fruits, (apricots, peaches, prunes, raisins) dry beans, crushed nuts, and whole grain breads

6. Iron-Deficiency Anemia
Manifestations
Pallor
Irritability
Anorexia
Decrease in activity
Infants may be overweight due to excessive milk consumption
Blood tests
RBC count
HGB and hematocrit
Morphological cell changes
Iron concentrations
Stool may be tested for occult blood

7. Iron-Deficiency Anemia
Untreated, iron-deficiency anemia will progress slowly
In severe cases, heart muscle becomes too weak to function
Children with long-standing anemia may also show growth retardation and cognitive changes
Treatment
Iron, usually ferrous sulfate, orally 2 to 3 times a day
Vitamin C aids in absorption

8. Nursing Tip
Oral iron supplements should not be given with milk or milk products because milk interferes with iron absorption
Infants on a iron supplement may have green tarry stools, if this is not apparent this may be a sign of poor compliance by the parents

9. Sickle Cell Disease Inherited defect in the formation of hemoglobin
Sickling (clumping) caused by decreased blood oxygen levels may be triggered by dehydration, infection, physical or emotional stress, or exposure to cold
Membranes of these cells are fragile and easily destroyed
Their crescent shape makes it difficult for them to pass through the capillaries, causing a pileup of cells in the small vessels
May lead to thrombosis
Hemosiderosis (iron deposits into body organs) is a complication of the disease

10. Sickle Cell Disease Https://youtu.be/9UpwV1tdxcs
Sickle cell trait (asymptomatic)
Blood of the patient contains a mixture of Hgb A and sickle (Hgb S)
Proportions of Hgb S are low because the disease is inherited from only one parent
HGB and RBC counts are normal
Sickle cell anemia (more severe)
Both parents had the trait and passed it on to their child
Clinical symptoms do not appear until the last part of the first year of life
May be an unusual swelling of the fingers and toes
Symptoms are caused by enlarging bone marrow sites that impair circulation to the bone and the abnormal sickle cell shape that causes clumping, obstruction in the vessel, and ischemia to the organ the vessel supplies

11. Sickle Cell Disease HGB level ranges from 6 to 9 g/dl or lower
Child is pale, tires easily, and has little appetite
Sickle cell crises are painful and can be fatal
Symptoms: severe abdominal pain, muscle spasms, leg pain, or painful swollen joints may be seen
Fever, vomiting, hematuria, seizures, stiff neck, coma, or paralysis can result
There is a risk for stroke as a complication of a vaso-occlusive sickle cell crisis

12. Types of Sickle Cell Crises
Comments
Vasoocclusive (painful Crises)
Most common type : there is an obstruction of blood flow by cells, infarctions, and some degree of vasospasm
Splenic Sequestration
Large amount of blood pools in liver and spleen. Spleen becomes massive
Aplastic crises
Bone marrow stops producing red blood cells; a number of infections precipitate this usually viral in nature
Hyperhemolytic
Rapid rate of hemlysis is superimposed on an already severe process: rare condition

13. Nursing Tip
During sickle cell crises, anticipate the child’s need for tissue oxygenation, hydration, rest, protection from infection, pain control, blood transfusion, and emotional support for this life-threatening illness

14. Therapies and Goals
Erythropoietin and some chemotherapy regimens can increase the production of fetal HGB and reduce complications
Routine splenectomy is not recommended because the spleen generally atrophies on its own because of fibrotic changes that take place in patients with sickle cell disease
Prevent
Sickling
Dehydration
Hypoxia
Infection

15. Thalassemia
Is a group of hereditary blood disorders in which the body cannot produce enough adult red blood cells
The RBC’s are odd shape or to small in size and are rapidly destroyed
Two main types of thalassemia
Major
Minor

16. Thalassemia Minor (β- Thalassemia trait)
Usually in children that only receives the trait from one parent
Hemoglobin concentration is usually 2-3 g/dl lower than normal
Have iron deficiency diagnosis first
Patient is pale and has enlarged spleen
May lead a normal life span

17. Thalassemia Major (Cooley’s Anemia)
This is the patient that receives the trait from both parents
A progressive anemia develops within 6 months of birth
Child is hypoxic and pale has a poor appetite
Diagnosis is helped by a good family history

18. Treatment The goals of treatment is to
1 maintain hemoglobin counts above 9.5g/dL to prevent the overgrowth of bones
Provide for growth and development and normal physical activities

19. Hemophilia One of the oldest hereditary diseases known to man
Blood does not clot normally
Congenital disorder confined almost exclusively to males
Is transmitted by symptom-free females

20. Hemophilia Inherited sex-linked recessive trait
Defective gene is located on the X, or female, chromosome
Fetal blood samples detect hemophilia
Two most common types are
Hemophilia B (Christmas disease [a factor IX deficiency])
Hemophilia A (a deficiency in factor VIII)
A deficiency in any one of the factors will interfere with normal blood clotting

21. Hemophilia A
Caused by a deficiency of coagulation factor VIII, or antihemophilic globulin (AHG)
Severity dependent on level of factor VIII in the plasma
Some lives are endangered from a minor scratch, whereas others might just bruise more easily
Aim of therapy is to increase level of factor VIII to ensure clotting
This is checked by a blood test call partial thromboplastin time (PTT)

22. Manifestations of Hemophilia
Can be diagnosed at birth because factor VIII cannot cross the placenta and be transferred to the fetus
Usually not apparent in the newborn unless abnormal bleeding occurs at the umbilical cord or after circumcision
Normal blood clots in 3 to 6 minutes
In severe hemophilia, it can take up to 1 hour or longer

23. Manifestations of Hemophilia
Anemia, leukocytosis, moderate increase in platelets may be seen in hemorrhaging; may also be signs of shock
Spontaneous hematuria is seen
Death can result from excessive bleeding, especially if it occurs in the brain or neck
Severe headache, vomiting, and disorientation may be symptoms
Circumstances leading to diagnosis
A nosebleed that will not stop
Loss of a deciduous tooth
Hematomas develop at the injection site of an immunization
Hemorrhage into the joint cavity (considered a classic symptom)

24. Nursing Tip
A classic symptom of hemophilia is bleeding into the joints (hemarthrosis)
Drugs that contain salicylates are contraindicated for children with hemophilia
What else could you think of that may be something that you would look for

25. Treatment of Hemophilia
If a family history exists, a newborn may have certain procedures delayed to prevent bleeding and tissue injury
Principal therapy is to prevent bleeding by replacing the missing factor
Recombinant antihemophilic factor, a synthetic product, has eliminated the need for repeated blood transfusions
Desmopressin acetate (DDAVP) is a nasal spray that can stop bleeding
Prophylactic care must be provided before planned invasive procedures

26. Treatment of Hemophilia
Multidisciplinary approach to care assists families with developing healthy coping strategies to deal with a child who has a chronic illness
It is difficult for parents to not be overprotective
The struggle to protect these children and still foster independence and a sense of autonomy is important, therefore allowing the children to participate in decision making about their care and focusing on their strengths are helpful

27. Leukemia
Oncologists are challenged in treating children with cancer because of the effects the treatments may have on growth and development
Genetic and environmental factors play a role
Exposure of the fetus to diagnostic x-rays or therapeutic irradiation for brain tumors, the use of fluoroscopy, ultraviolet (sun) exposure, and some drugs have been associated with the increase in cancer

28. Leukemia
A malignant disease of the blood-forming organs that results in an uncontrolled growth of immature WBCs
Most common form of childhood cancer
Involves a disruption of bone marrow function caused by the overproduction of immature WBCs in the marrow
These immature WBCs take over the centers that are designed to form RBCs, and anemia results
Platelet counts are also reduced
Invasion of the bone marrow causes weakening of the bone, and pathological fractures can occur

29. Leukemia
Leukemia cells can infiltrate the spleen, liver, and lymph glands, resulting in fibrosis and diminished function
Cancerous cells invade the CNS and other organs
Drains the nutrients
Leads to metabolic starvation of the body

30. Leukemia
Leukemia's are classified according to the type of WBC affected
Acute lymphocytic leukemia (ALL)
Most common form in childhood
Acute nonlymphocytic leukemia (ANLL)
Acute myelocytic leukemia (AML)

31. Manifestations Initial phase of leukemia are Low grade fever Pallor
Leg and joint pain
Bruising tendency
Abdominal pain
Enlargement of spleen and liver along with the lymph glands
Petechiae and Purpura

32. Diagnosis
Is based on the history of symptoms and the results of extensive testing
Some of the testing that is done for Leukemia are:
CBC with white blood cell differentiation
Bone marrow aspiration
X-ray films to see long bone changes
Spinal tap after diagnosis is confirmed
Kidney and liver function testing are done to make sure that they are healthy enough to receive chemotherapy

33. Leukemia

34. Treatment of Leukemia
Long-term care is given whenever possible in an outpatient setting
Involves a multidisciplinary team
In chemotherapy, bone marrow suppression requires that the family be taught about infection prevention
Adequate hydration is emphasized to minimize kidney damage
Active routine immunizations must be delayed while the child is receiving immunosuppressive drugs
The body will not be able to manufacture antigens as expected
Nausea and vomiting are common side effects of chemotherapy and lead to decreased appetite, weight loss, and generalized weakness
Meticulous oral care is given

35. Treatment of Leukemia
Long-term care is given whenever possible in an outpatient setting
Involves a multidisciplinary team
In chemotherapy, bone marrow suppression requires that the family be taught about infection prevention
Adequate hydration is emphasized to minimize kidney damage
Active routine immunizations must be delayed while the child is receiving immunosuppressive drugs
The body will not be able to manufacture antigens as expected
Nausea and vomiting are common side effects of chemotherapy and lead to decreased appetite, weight loss, and generalized weakness
Meticulous oral care is given

36. Treatment of Leukemia Components of chemotherapy include
An induction period
Central nervous system prophylaxis for high-risk patients
Maintenance
Reinduction therapy (if relapse occurs)
Extramedullary disease therapy
Bone marrow transplant

37. Side Effects of Chemotherapy
Steroids can mask signs of infection, cause fluid retention, induce personality changes, and cause the child’s face to appear moon shaped
Certain chemotherapy agents can cause nausea, diarrhea, rash, hair loss, fever, anuria, anemia, and bone marrow depression
Peripheral neuropathy may be signaled by severe constipation caused by decreased nerve sensations to the bowel

38. Nursing Care of the Child With Leukemia
Encourage the child to verbalize feelings
Give permission to discuss his/her concerns, which will help clear up misconceptions and decrease feelings of isolation
Frequently observe child for infection
Monitor vital signs and for symptoms of thrombocytopenic bleeding (a common complication of leukemia)
Meticulous mouth and skin care

39. Hodgkin’s Disease
Hodgkin's disease is a malignancy of the lymph system
It may metastasis to the spleen, liver, bone marrow, and lungs
The appearance of giant multinucleated cells called Reed-Sternberg cells are diagnostic for this disease
Hodgkin’s disease is rarely seen before the age of 5

40. Hodgkin’s Disease Manifestations
Usually it starts with a non-painful swelling of the lymph nodes
It may progress to:
Low grade fever
Anorexia
Weight loss
Night sweets
General malaise
Testing confirms hodgkins by xrays, blood tests, body scans, and biopsy of the nodes look at table 27-2 for staging of Hodgkin’s lymphoma

41. Treatment of Hodgkin’s
Well established treatment plans include both radiation and chemotherapy
Long term prognosis for this disease is excellent

42. Nursing care plan
Nursing concerns are focused on the growth and development of the child
The family stresses that are there with a sick child and family education on precautions such as
Notify school nurse of child condition
Sun screen
And risk of long term infections related to splenectomy

43. Facing Death The nurse must understand:
The grieving process
Personal and cultural views concerning that process
The views of a parent losing a child
Perceptions of the child facing death
The response to a child’s death is influenced by whether there was a long period of uncertainty before the death or whether it was a sudden unexpected event

44. Facing Death The nurse must: Show compassion
Demonstrate a nonjudgmental approach
Be sensitive and effective in the provision of care
Facilitate the grief process by anticipating psychological and somatic responses while maintaining open lines of communication
Support the family’s efforts to cope, adapt, and grieve
Know that hostility is a normal response and may drive away those who do not understand its normalcy in the acute grieving process

45. The Child’s Reaction to Death
Cognitive development, rather than chronological age, affects the response to death
Children younger than 5 years of age are mainly concerned with separation from their parents and abandonment
Preschool children respond to questions about death by relying on their experience and by turning to fantasy
May believe death is reversible or that they are in some way responsible
Children do not develop a realistic concept of death as a permanent biological process until 9 or 10 years of age

46. The Child’s Awareness of His or Her Condition
Failure to be honest with children leaves them to suffer alone, unable to express their fears and sadness or even to say good-bye
Stages
Denial
Anger
Bargaining
Depression
Acceptance
Nurse’s role
Listen
Provide privacy
Provide therapeutic intervention
Provide information
Use appropriate phrases and open-ended statements

47. Questions
When a patient experiences apprehension and uticaria while receiving a blood transfusion, the nurse:
A. slows the transfusion and takes the patients vital signs
B. observes the child for further transfusion reactions
C. Stops the transfusion, allows normal saline to run slowly , and notifies the charge nurse
D. stops what he or she is doing and obtains the patients history
Answer is C

48. Questions
Which of the following principles should the nurse teach the parent concerning administration of liquid iron preparations to the child with iron-deficiency anemia
A. Allow the preparation to mix with the saliva and bathe the teeth before swallowing
B. Warm the medication before administration
C. Administer the medication between meals
D. Administer the medication in the bottle of formula
Answer is C