1. Pediatric Hematologic Disorders and Cancer
Presented by
Christina Hernandez RN, MSN

2. Hematologic System Adult Pedi Life cycle of RBC- 120 days
Cell production- marrow and spleen
RBC’s= 4.1 to 4.9 million/ml
Hemoglobin=
Hematocrit=
Life cycle of RBC- 100 days (neonate)
Cell production- red bone marrow (infant)
#RBC’s= 5million/ml at birth
Hemoglobin= g
Hematocrit= 45-50%
Life cycle of RBC- short in neonate/infant What condition does this contribute to in the neonate? Hyperbillirubinemia
When you compare adult with infant the volume is significant. Why do you think children require a higher Hct & Hbg?
Page 1491 WBC’s table 49-1

3. Iron Deficiency Anemia

4. Iron Deficiency Anemia
Cause
Signs and symptoms
Diagnostic tests
Nursing interventions
Oral supplements- What significant side effects does the nurse need to remember?
Dietary teaching- what specific foods?
Cause- p Neonates have extra iron supplied from the mother to last about 4-6 months, if an RBC lasts 120 days (3months) then IDA is not likely to manifest until after 9 months of age.
Infants don’t have adequate stores and lack dietary iron, and the short life cycle of the RBC, blood loss, increased internal demands (rapid growth) poor nutritional intake.
Iron deficiency anemia most common nutritional deficiency in children
Diagnostic tests- Hgb- 6 to 11 g/dL, and decreased Hct , serum iron, RBC count, reticulocytes (immature or newly released RBC’s) iron-binding capacity, serum ferritin. Microscopic analysis, RBC’s small in size and hypochromic (pale) in color
Treatments: iron supplements- constipation- give with food, assess for elimination, encourage fluids, for liquid iron supplements avoid contact with teeth (brush/wipe) avoid mixing in milk or feeding with calcium rich foods.
Dietary teaching- p iron fortified formula or breast milk and iron fortified food supplements for infants; meats, eggs, dried fruits, iron-fortified cereal

5. What Parents Want to Know: (p 1281)
Specific foods: (based on age of child)
Cream of wheat or iron fortified cereal
Apricots, prunes, raisins and other dried fruits
Egg yolks
Dark green leafy vegetables
Administration of Iron Supplements:
Give with vitamin C –rich fluids
Prevent staining from liquid iron supplements
Changes in stool patterns
Avoid mixing supplement with food/drink containing calcium

6. Sickle Cell Disease

7. Sickle Cell disease
Sickle cell trait- genetic disorders characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbg p
Precipitating factors (p 1284)
Signs and symptoms
Precipitating factors- hypoxia, low blood pH (acidosis); increased blood viscosity (dehydration), fever, and general stress
Signs & symptoms- episodes of acute pain in joints, may have abdominal or back pain; edema to extremities; headache

8. Three Forms of Sickle Cell Crisis
Vaso-occlusive
Acute sequestration
Aplastic

9. Types of Sickle Cell Crisis
Vaso-occlusive- most common effects
Pain
Hand and foot syndrome (dactylitis)
CVA- hemiplegia, aphasia, seizures, LOC changes, vision changes, and headache
Acute chest syndrome- chest pain, fever cough (leading cause of death in SCD)
Priapism
Hepatomegaly
Hematuria
Pain- joint and bone pain of extremities. Pain is often transient. R/T necrosis of tissue- child may refuse to move extremity, cry out when joint is touched or moved
Hand and foot syndrome (dactylitis)- painful edema of hands and/or feet, warmer to touch, May be first S&S of crisis often accompanied by fever
CVA- vaso-occlusion in the brain causes cerebral infarction with neurologic impairment.
Acute Chest syndrome- chest pain, fever, cough precipitated by pneumonia- assess for retractions, tachypnea, dyspnea
Priapism- persisitent erection of the penis when penile blood flow is obstructed- can lead to ishemia and necrosis
Hepatomegaly- enlarged liver with jaundice and hepatic coma
Hematuria- blood in urine from sickling in the kidneys

10. Types of Sickle Cell Crisis
Aplastic Crisis:
Decreased RBC production- S&S malaise, headache, pallor, lethargy, and fainting (precipitated by infection)
Splenic sequestration- life threatening S&S pallor, irritability, tachycardia, hypovolemic shock
Hyperhemolytic crisis- (not in text)- RBC’s destroyed more rapidly than usual (immature cells)
Aplastic Crisis- decreased RBC production
Splenic sequestration- Life threatening Death occurs in hours, caused by pooling of blood in the spleen. This leads to shock and hypovolemia.

11. Quick Review:
What is most common reason for admission to the ED for a child with SCD?
What precipitates a sickle cell crisis?
How does sickling effect the life span of an RBC?
what organs experience complications as a result of chronic sickling crisis?
*- pain most common reason for admission due to obstruction of blood flow to tissue, predominantly in joints and vertebrae.
*- organs most effected- spleen- more susceptible to infection, gradual scarring reduces spleen activity/asplenia – Autospleenectomy by age 7-8;
Liver- enlarge, firm, tender
Brain- single episode in brain can cause CVA, seizures
Heart- may become enlarged with murmur
Lungs- any infection causes pulmonary edema and stasis, leads to further sickling
Kidneys- micro infarcts cause hematuria, inabiity to concentrate urine
Bone infarcts- small bones of hands and feet swell and are painful- hand & foot syndrome

12. Diagnosis & Treatment
Cord blood testing if one parent is known to carry trait
Blood transfusions
Complications
Nursing interventions before/during/after
Cord blood testing- if one parent know to carry trait. Move for all NB’s to have a screen with why? Symptoms may not show up until 4-6 months of age, sickling is inhibited by high levels of fetal hemoglobin and the child becomes more mobile around 6 months of age. Early detection prevents complications
Blood transfusions increased blood cells provide oxygen for tissues and reduce pain. Always preceded by and followed by an IV of saline avoid glucose in IV complications– increased risks for reactions (p.1498) Hemolytic reactions: (mismatched blood) fever, chills, hematuria, headache, chest pain, progress to shock. STOP transfusion and continue IV saline- monitor VS and stay with patient—febrile or sepsis: from contaminated blood, notify primary healthcare provider for N&V, decrease BP, fever, chills, headache, leg and back pain– circulatory overload: excessive fluid or too rapid infusion rate (s&s= dyspnea, chest or low back pain, productive cough, rales, distended neck veins) * usually order diuretics

13. Treatment Patient/family teaching Medications
Immunizations- why important?
Patient/family teaching focus on prevention***- hydration- teach s&s of dehydration (weight loss, dry mucous membranes, sunken fontanelles)
Give specific instructions as to how many glasses of liquid daily. (what additional method for monitoring hydration- output) How does this impact a school age child? **Make sure family understands triggers** What are possible triggers??
Medications- prophylactic antibiotic therapy- why? Infection makes the child more susceptible to a crisis

14. Clinical Judgment:
Why are blood transfusions ordered for the patient in sickle cell crisis?
Can a neonate have a diagnosis of sickle cell disease?
What ethical issues relate to this diagnosis?

15. Hemophilia

16. What factor is missing or defective? Factor VIII
Hemophilia (p 1291)
X-linked trait
What factor is missing or defective? Factor VIII
Who is the carrier, and who is effected by this disorder?
If the mother is a carrier, and the father does not have hemophilia, each son has a 50% chance of getting hemophilia and each daughter has a 50% chance of passing on the trait to her offspring.

17. Diagnosis & Treatment When does diagnosis most commonly occur?
What specific laboratory tests and values?
What are signs & symptoms?
Diagnosis- if parent is known carrier amniocentesis or CVS (chorionic villi sampling) during gestation. If unknown, following circumcision. Most commonly diagnosed after 6 months when child becomes more mobile. Bleeding in the mouth (cut, bitten tongue, tooth), surface brusing- bleeding from trauma to soft tissues and muscles, (seen when begins walking). Hematuria- blood in urine; Hemarthrosis- bleeding into a joint, also deep tissue hemorrhage.
Diagnosed on basis of hx, physical exam, lab findings- PTT (partial thromboplastin time APTT, PTT)- evaluates factor VIII and clot formation. Normal APTT is seconds

18. Nursing Care:
Factor VIII- when should the patient receive this medication?
What does the family need to know about factor VIII?
Human plasma
Vasopressin (DDAVP)
Factor VIII- genetically engineered (artificial) factor VIII is an artificial blood product that is reconstituted with sterile water and given IV. Give immediately upon injury. Need to teach parents, school nurse, teachers how to safely administer Factor VIII
Human Plasma (a component of blood)- fresh whole blood, fresh or frozen plasma may also be used. One bag of concentrate per 5 kg of body weight is usually sufficient.
Vasopressin (DDAVP)- administered IV for mild hemophilia and causes a two to four-fold increases in factor VIII activity p.1292

19. Nursing Care cont…
What is the primary nursing goal for a patient with hemophilia?
Prevent or stop bleeding
What are specific interventions to achieve this goal?
Administer Factor VIII
Apply local pressure for minutes
Elevate the joint and immobilize
Apply cold compresses

20. Complications of hemophilia
Hemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common?
At risk for hemorrhage
Death
p assess for joint pain, edema, instruct family on risks once child is mobile. Caution to prevent falls.
School-aged children need an accurate hx with school nurse and teachers. Prevent unnecessary injury to the child during play. No contact sports. Keep what on hand?? Factor VIII
Fatal hemorrhage is rare with the use of Factor VIII and transfusion therapy.

21. The Child with Cancer

22. Childhood Cancers “…communication promotes understanding and clarity;
with understanding, fear diminishes;
in the absence of fear,
hope emerges; and in the presence of hope, anything is possible” (Stovall, 1995)

23. Childhood Cancer C- continual unexplained weight loss, fatigue malaise
H- headaches with vomiting (early morning)
I- increased edema or pain in joints
L- lump or mass, persistent lymphadenopathy
D- development of whitish appearance in pupil of the eye
R- recurrent or persistent fevers, night sweats
E- excessive bruising or bleeding
N- noticeable pallor

24. Acute Lymphatic Leukemia- ALL

25. What signs and symptoms would lead to the diagnosis of leukemia?
Fever
Pallor
Overt signs of bleeding
Lethargy or malaise
Anrexia
Large joint or bone pain
Petechiae, frank bleeding
Enlarged liver or spleen, changes in lymph nodes
Neurologic changes

26. Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis
Normal
Leukemia
Leukocytes < 10,000
Leukocytes> 10,000
Platelets ,000
Hemoglobin 7-11

27. Further diagnostic findings:
Bone marrow aspiration- iliac crest (why this site?)
How does the nurse prepare the child/family for this procedure?
What are the nurse responsibilities for this procedure?
Bone marrow aspiration via iliac crest- yields more bone marrow, less frightening, less painful.
Explain all aspects of the procedure to the child and parents. Obtain signed permit. Prepare the child/family for sedation and analgesia.

28. Treatment and Plan of Care: (p 1313/1321)
Chemotherapy: three phases
Induction phase
Consolidation
Delayed intensification
Remission and maintenance
Induction- most intense treatment. Large doses of antineoplastic drug administered in an effort to destroy as many proliferating cells as possible. Lasts 4-6 weeks about 95% successful
Consolidation- method of destroying leukemia cells in the cNS- for children who have CNS involvement or are high risk, given intrathecal
Delayed intensification- additional drugs to target the leukemis cells that survived
Remission maintenance- meds given at intervals If remain in remission for 2-3 years continue to remain in remission and appear to the bured

29. Nursing Plan of Care for a Child Undergoing Chemotherapy:
Myelosupression- protect from injury
Infection/sepsis (neutropenia)- protect from infection
Renal damage
GI disturbances
Metabolic emergencies
Myelosupression- monitor labs protect from injury, alert for signs of bleeding, petechiae or blood in vomitus or urine. Minimize needle sticks and report bleeding to primary care provider.
Infection/sepsis- (neutropenia) risk increases as WBC count falls; nurses must inform family/staff/primary care provider- may place child on protective isolation- when does the child wear a mask? When does the staff wear a mask?
Renal damage (from chemo)- strict I&O, notify if hematuria occurs. Many chemo agents are nephrotoxic and may damage kidneys. Avoid invasive procedures like cath if possible.
Gi Disturbances (n&V, stomatitis); maintain nutrition, high calorie dringks and foods that are tolerated by the child. Cold or room temp liquids more comfortable. Bedside medications may be administered by the parents that contain lidocaine 1% as a swish and swallow. Also disturbances in bowel patterns. Assess daily bowel habits, esp. if on opiates for pain or if immobile.
Metabolic emergencies (lysis of tumor cells) p caused by breakdown of malignant cells releasing intracellular components into the blood. Elecrolyte imbalance causess metabolic acidosis and serious abnormal values of lab. Hyperuricemia, hyperkalemia, hypocalcemia, cardiac arrythmias, impaired renal function, tetany and neurologic changes. Nurse should administer fluids, daily weights, adminsieer elecrolytes PRN, Trousseau’s and Chvostek’s sign for tetany. (tap on cheek)

30. Intrathecal Medication
Chemotherapy instilled into spinal canal
Assess and monitor for placement of intrathecal catheter and assess neuro checks

31. Cranial Radiation
Head and neck tumors are more sensitive to radiation than chemotherapy.
When would chemotherapy become an adjunct to radiation therapy?
Add chemo to radiation if metastasis occurs. Radiation is focused to irradiate the tumor but not healthy adjacent tissue.

32. Tumor Lysis Syndrome: What causes tumor lysis syndrome?
What are signs and symptoms of this complications
What nursing interventions apply to treatment?
p. 1528
Oncological emergency: S&S- 1) hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, 2) Cardiac arrhythmias, impaired renal funciton, tetany, neurologic and mental status changes.
Nursing interventions: Adminsiter fluids, careful I&O, daily weights, monitor for desired and side effects of chemo, Administer electrolytes, check for tetany, obtain lab specimens (urine and fecal- why?)

33. Bone marrow and Stem Cell Transplantation
Used to treat leukemia, neuroblastomas and some noncancerous conditions-aplastic anemia
Goal to administer a lethal dose to kill the cancer, and resupply the body with stem cells from the child’s own bone marrow, or a compatible donor
Autologous transplant – child’s own stem cells
Allogenic transplant- compatible donor
Major side effect– rejection of the donor marrow

34. Risk for alteration in bowel elimination Risk for GI distress
Develop a plan of care for the child undergoing treatment for leukemia:
Risk for injury:
Soft tissue/mucous membranes
Generalized trauma
Risk for infection:
Risk for alteration in bowel elimination
Risk for GI distress
Fluid volume delicate
Injury- take care with brushing teeth to prevent trauma to gums, soft bristle brush or sponge cleanser to clean mouth, tongue and teeth.
Prevent injury- risk for hemorrhage (bruising) child has low platelets and slightest injury causes severe bleeding
Infection- neutropenic (low WBC’s) need to limit visitors, avoid fresh fruits, house plants with soil, after discharge avoid public places and gatherings of people outside immediate family. Limits risk of exposure to pathogens. Teach family to assess for infections, and good hand washing.
Alteration in bowel elimination-if stomatitis, n&v and diarrhea from radiation, may become dehydrated or constipation. Use lidocaine 1% as prescribed to soothe mouth ulcerations.
Dehydration or alteration in renal function- some chemo agents are nephron toxic. Monitor I&O carefully for dehydration

35. Other Cancers of Childhood

36. Nephroblastoma- Wilm’s Tumor
Soft renal tumor - one or both kidneys
(p 1332) Metastasis or seeding spread by palpation
Nephrectomy treatment of Wilm’s tumor
Occurs most commonly outside the cranium, Smooth, hard, non-tender mass that can occur anywhere along the sympathetic nervous system
Frequently located in abdomen do not palpate!!! Altered bowel and bladder elimination patterns. Neck and facila edema from vena cava syndrome, Limp may occur if in long bone.

37. Nursing treatment of Wilms’ tumor:
Pain management
Frequent reposition
Noninvasive and pharmacologic pain interventions
Prevent circulatory overload
Weigh daily
I&O, urine for specific gravity
Prevent infection
Hand washing
Protective isolation
Homecare needs
p Assess site of tumor by observation and inspection
Palpation Is contraindicated!!! Document related functioning, bowel and bladder baseline, observe gait. Prepare family for chemo, radiation and surgery if necessary.

38. Clinical manifestations of Neuroblastoma (p 1327)
Smooth, hard, non-tender along sympathetic nervous system
Frequent location is abdomen
Neck and facial edema from vena cava syndrome
Increased ICP
Limp if metastasis to bone
Pancytopenia
Location- in abdomen causes alteration in bowel function- may be first indication- weight loss, abdominal distention, enlarged liver, fatigue and fever

39. Nursing Management
Assess by observation and inspection (not palpation)
Document bowel and bladder function
Record height & weight, observe gait
Chemotherapy, radiation, surgery
Teach parents S&S of infection. Why?

40. Osteosarcoma

41. Osteosarcoma- most common primary bone malignancy in children
Goal of treatment- remove tumor and prevent spread of disease
Biopsy Chemo Surgery Chemo (radiation=palliative pain control)
Promote self esteem
Side effects of chemotherapy
Amputation of extremity
Separation from friends and family
Biopsy of bone/tumor chemo (3 months) to prevent seeding and shrink tumor surgery to remove bulk of tumor followed by chemo (9 months) to kill remaining cancer
Osteo

42. Ewing Sarcoma- second most common bone tumor associated with children
Pain, soft tissue swelling
Anorexia, fever, malaise with metastasis
Diagnosis same as osteosarcoma
Management
Chemo
Surgery (decrease tumor bulk)
Radiation

43. Rhabdomyosarcoma

44. Pathophysiology and Manifestations
Most common soft tissue malignancy
Divided by young (<10 yrs) and older (adolescents) in location
60% have positive prognosis
Soft to hard, nontender mass (depends on location)
In pelvic tumors, may disrupt organ function

45. Diagnosis and Treatment
CT, BM aspiration and biopsy
Renal function and liver function tests
Treated with chemo, surgery and radiation

46. Retinoblastoma (p1333)

47. Retinoblastoma- rare malignant tumor of the neural retina
“cat’s eye” reflex seen as a white light in the pupil is the most common “leukocoria”
May have strabismus of involved eye
Red painful eye is late symptom
Staging based on extent of disease

48. Nursing care of the child/family with a malignant disease:
Initial focus on support of family members
Nurses facilitate the educational process to allay fears of unknown
Encourage family members to verbalize fears and questions
Postoperative care if indicated
Community resources (through the discharge planner, case worker)

49. Death and Dying:
Understanding of death according to developmental age:
< 3 years- no understanding/concept of death
3-5 years- afraid of separation from parents
5-9 years- understand death is permanent, irreversible and sad. Concerns for fear of pain, being left alone and leaving parents and friends.
Age 10> have adult’s concept of death
<3- child may perceive family’s anxiety and sadness
3-5 years- greatest fear is separation, They envision death as temporary and have little of adult’s fear of death. Thik of it as a long sleep not a final process. Nighmares increase. May feel pain/illness is a punishment for misdeeds or thought. May ask questions about death. In long-term illness, may simulate adult responses with depression, withdrawal, fearfulness, anxiety
5-9 years- begin to understand death is permanent, irreversible and sad. May think it is something that only happens to adults. Become aware of what is happening to them when their disorder has fatal prognosis. Concerns center on fear of pain, being left alone, and leaving parents and friends. May associate death with sleep and may be afraid to got to sleep without someone near them. May associate death with darkness and want light left on in room.
10> adult concept of death. Know death is end of life, Emotional outbursts may reflect anger. View death as fearsome and fascinating (increase in adolescent suicide). May feel immune to death and deny symptoms for longer. Some consider themselves alienated from peers and unable to communicate with their parents for emotional support feeling alone in their struggles.

50. Nursing Care and Grief
Child- encourage child to express feelings, allow choices, help maintain independence
Family- listen, answer questions, provide information, encourage expression of feelings and fears

51. For questions or concerns please contact Christina Hernandez RN, MSN References: McKinney, James, Murray, & Ashwill. Maternal- Child Nursing Third ed (2009). Saunders.