Pediatric Hematologic Disorders and Cancer

Pediatric Hematologic Disorders and CancerPresented by Marlene Meador RN, MSN, CNE1Hematologic SystemAdultPediLife cycle of RBC- 120 daysCell production- marrow and spleenRBCs= 4.1 to 4.9 million/mlHemoglobin=Hematocrit= Life cycle of RBC- 100 days (neonate)Cell production- red bone marrow (infant)#RBCs= 5million/ml at birthHemoglobin= 17-18 gHematocrit= 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 WBCs table 49-1

2Iron Deficiency AnemiaCauseSigns and symptoms Diagnostic testsNursing interventionsOral supplements- What significant side effects does the nurse need to remember?Dietary teaching- what specific foods?Cause- p 1492-1493- Infants dont 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- Hct, serum iron, RBC count, reticulocytes (immature or newly released RBCs) iron-binding capacity, serum ferritin. Microscopic analysis, RBCs small in size and hypochromic (pale) in colorTreatments: iron supplements- constipation- give with food, assess for elimination, encourage fluids, Dietary teaching- p 945-946- meats, eggs, dried fruits, iron-fortified cereal3Sickle Cell diseaseSickle cell trait- genetic disorders characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbg. p. 1494-1503 Precipitating factors (p. 1496)Signs and symptomsPrecipitating factors- hypoxia, low blood pH (acidosis); increased blood viscosity (dehydration), fever, and general stressSigns & symptoms- episodes of acute pain in joints, may have abdominal or back pain; edema to extremities; headache4Types of Sickle Cell CrisisVaso-occlusive- most common effectsPainHand and foot syndrome (dactylitis)CVA- hemiplegia, aphasia, seizures, LOC changes, vision changes, and headacheAcute chest syndrome- chest pain, fever cough (leading cause of death in SCD) PriapismHepatomegalyHematuria

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 movedHand and foot syndrome (dactylitis)- painful edema of hands and/or feet, warmer to touch, May be first S&S of crisis often accompanied by feverCVA- 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, dyspneaPriapism- persisitent erection of the penis when penile blood flow is obstructed- can lead to ishemia and necrosisHepatomegaly- enlarged liver with jaundice and hepatic comaHematuria- blood in urine from sickling in the kidneys

5Types of Sickle Cell CrisisAplastic 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 shockHyperhemolytic crisis- (not in text)- RBCs destroyed more rapidly than usual (immature cells) Aplastic Crisis- decreased RBC productionSplenic sequestration- Life threatening Death occurs in hours, caused by pooling of blood in the spleen. This leads to shock and hypovolemia. 6Quick Review:What is most common reason for admission 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, tenderBrain- single episode in brain can cause CVA, seizuresHeart- may become enlarged with murmurLungs- any infection causes pulmonary edema and stasis, leads to further sicklingKidneys- micro infarcts cause hematuria, inabiity to concentrate urineBone infarcts- small bones of hands and feet swell and are painful- hand & foot syndrome

7Diagnosis & TreatmentCord blood testing if one parent is known to carry trait

Blood transfusions-Cord blood testing- if one parent know to carry trait. Move for all NBs 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 patientfebrile 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 8TreatmentPatient/family teaching-MedicationsImmunizations- 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 crisis9HemophiliaX-linked traitWhat factor is missing or defective? Factor VIIIWho 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. 10Diagnosis & TreatmentWhen does diagnosis most commonly occur?

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 30-40 seconds 11Nursing Care:Factor VIII- when should the patient receive this medication? What does the family need to know about factor VIII?Human plasmaVasopressin (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 VIIIHuman 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.150512Nursing Care contWhat is the primary nursing goal for a patient with hemophilia?Prevent or stop bleedingWhat are specific interventions to achieve this goal?Administer Factor VIIIApply local pressure for 10-15 minutesElevate the joint and immobilizeApply cold compresses 13Complications of hemophiliaHemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common?

Death- at risk for hemorrhagep. 1505- 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 VIIIFatal hemorrhage is rare with the use of Factor VIII and transfusion therapy.14Childhood CancerC- continual unexplained weight loss, fatigue malaiseH- headaches with vomiting (early morning)I- increased edema or pain in jointsL- lump or mass, persistent lymphadenopathy D- development of whitish appearance in pupil of the eyeR- recurrent or persistent fevers, night sweatsE- excessive bruising or bleedingN- noticeable pallor15What signs and symptoms would lead to the diagnosis of leukemia?FeverPallorOvert signs of bleedingLethargy or malaiseAnrexiaLarge joint or bone painPetechiae, frank bleedingEnlarged liver or spleen, changes in lymph nodesNeurologic changes 16Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis NormalLeukemiaLeukocytes < 10,000Leukocytes> 10,000Platelets 20-100,000Hemoglobin 7-11

17Further 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.18Treatment and Plan of Care:Chemotherapy: four phasesInduction phaseConsolidationDelayed intensificationRemission and maintenanceInduction- 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% successfulConsolidation- method of destroying leukemia cells in the cNS- for children who have CNS involvement or are high risk, given intrathecalDelayed intensification- additional drugs to target the leukemis cells that survivedRemission maintenance- meds given at intervals If remain in remission for 2-3 years continue to remain in remission and appear to the bured

19Nursing Plan of Care:Myelosupression- protect from injuryInfection/sepsis (neutropenia)- protect from infectionRenal damageGI disturbancesMetabolic emergenciesMyelosupression- 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. 1526- 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, Trousseaus and Chvosteks sign for tetany. (tap on cheek)20Intrathecal MedicationChemotherapy instilled into spinal canalAssess and monitor for placement of intrathecal catheter and assess neuro checks21Cranial RadiationHead 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. 22Tumor Lysis Syndrome:What causes tumor lysis syndrome?What are signs and symptoms of this complicationsWhat nursing interventions apply to treatment? p. 1528Oncological 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?)

23Bone marrow and Stem Cell TransplantationUsed to treat leukemia, neuroblastomas and some noncancerous conditions-aplastic anemiaGoal to administer a lethal dose to kill the cancer, and resupply the body with stem cells from the childs own bone marrow, or a compatible donorAutologous transplant childs own stem cells

Allogenic transplant- compatible donor

Major side effect rejection of the donor marrow24Develop a plan of care for the child undergoing treatment for leukemia:Risk for injury:Soft tissue/mucous membranesGeneralized traumaRisk for infection:Risk for alteration in bowel eliminationRisk for GI distressFluid 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 bleedingInfection- neutropenic (low WBCs) 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 dehydration25Wilms TumorSoft tumor frequently along sympathetic nervous system(p.1552-3) Metastasis or seeding spread by palpation Nephrectomy treatment of Wilms tumorOccurs 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.26Nursing treatment of Wilms tumor:Pain management Frequent repositionNoninvasive and pharmacologic pain interventionsPrevent circulatory overloadWeigh dailyI&O, urine for specific gravityPrevent infectionHand washingProtective isolationHomecare needs

p. 1551 Assess site of tumor by observation and inspectionPalpation Is contraindicated!!! Document related functioning, bowel and bladder baseline, observe gait. Prepare family for chemo, radiation and surgery if necessary.

27Clinical manifestations of neuroblastoma:P. 1549-1552Smooth, hard, non-tender along sympathetic nervous systemFrequent location is abdomenNeck and facial edema from vena cava syndromeIncreased ICPLimp if mets to bonePancytopenia Location- in abdomen causes alteration in bowel function- may be first indication- weight loss, abdominal distention, enlarged liver, fatigue and fever28Nursing Management Assess by observation and inspection (not palpation)Document bowel and bladder functionRecord height & weight, observe gaitChemotherapy, radiation, surgeryTeach parents S&S of infection. Why?29Death and Dying:Understanding of death according to developmental age:< 3 years- no understanding/concept of death3-5 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 adults concept of death 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. 30Nursing and GriefChild- encourage child to express feelings, allow choices, help maintain independence

Family- listen, answer questions, provide information, encourage expression of feelings and fears31For questions or concerns please contact Marlene Meador RN, MSN, CNE Email mmeador@austincc.edu 32