Immune System, HIV & AIDS

Immune System and HIV

• HIV infects CD4 T-cells (Helper cells), using them to replicate.
• This process leads to dysfunctional CD4 cells and immune system suppression.
• B cells are also suppressed in HIV, weakening the immune response and bone marrow's ability to produce infection-fighting cells.
• HIV causes AIDS (acquired immunodeficiency syndrome).

Perinatal HIV Transmission

• HIV can be transmitted from an infected mother to her baby in utero, during birth, or via breastfeeding.
• Interventions include antiretroviral drug therapy (e.g., Zidovudine) for HIV-infected mothers, elective C-sections, and education on avoiding breastfeeding.
• HIV testing is currently voluntary for pregnant women.

HIV in Children: Transmission and Progression

• In children, HIV transmission via sexual contact is rare and should prompt investigation into possible sexual abuse.
• In adolescents, sexual contact is the major mode of HIV transmission.
• Children may experience either rapid HIV progression with death by age 4 or slower progression with AIDS manifestation in adolescence.
• Common signs include HSM, lymphadenopathy, FTT, developmental delay, oral thrush, diarrhea, and parotitis.
• Recurrent infections, especially opportunistic infections, are common and may not respond to typical treatments.

Diagnosing HIV

• Western Blot and ELISA tests may produce false positives in infants due to maternal antibodies that persist for about 18 months.
• These tests are only reliable for children over 18 months old.
• Infants are diagnosed using Polymerase chain reactions (PCR) and p24 antigen detection tests, which can detect HIV in infants as young as 4 weeks old.
• An infant born to an HIV+ mother is presumed positive until proven otherwise.

HIV Treatment Goals

• Slow virus growth
• Promote normal growth and development
• Prevent complications
• Improve quality of life
• Prolong survival

HIV Pharmacology and Nursing Care

• Combination antiretroviral drugs are the most effective treatment, though they are not a cure but can slow viral progression.
• Adherence to medication schedules is critical but can be difficult due to taste and timing issues.
• Early recognition is key
• Nursing care includes infection prevention, nutritional support, pain control, psychological support, assessing disease progression signs, providing ethically safe care.
• Custody arrangements for the child if parents are infected, encouraging day care and school participation are also important.
• Education is crucial for prevention.

Immunizations and T-Cell Counts in HIV-Positive Children

• Immunizations are safe only for children with HIV when CD4 counts indicate no severe suppression.
• Immediate prophylaxis after disease exposure is recommended due to poor immune response.
• Memorize the chart of Tcell counts in notes.

SCIDS (Severe Combined Immunodeficiency)

• Some forms of SCIDS are autosomal recessive or X-linked.
• SCIDS is characterized by the absence of a functioning immune system.
• There is an overwhelming potential for life-threatening infection.
• SCT (stem cell transplant) is possible when there is a good match
• Preventing infections, prompt recognition and treatment of infections, and supportive care for the patient and family are priorities.

Hemophilia

• Hemophilia is a group of bleeding disorders caused by a deficiency in a clotting factor.
• It is an X-linked recessive disorder, with about 1/3 of cases resulting from genetic mutation.
• Hemophilia's most common forms are hemophilia A (Factor VIII deficiency) and hemophilia B (Factor IX deficiency).
• Christmas disease is named after Stephen Christmas, the first person diagnosed with the disorder in 1952.
• Prolonged bleeding can occur spontaneously or after trauma, even without major injuries.
• Petechiae is uncommon

Hemophilia: Labs

• Prolonged aPTT or PTT
• aPTT uses an activator, so results are faster
• Normal PT and INR
• Normal CBC**
• Low factor levels

Hemophilia: Nursing Care and Hemarthrosis

• Focus on preventing bleeding
• Replace missing factor immediately
• Mouth lacerations
  • Watch ABCs, may need to be NPO until managed
• Dental work/teeth extractions
  • Pre- and post-administration of antifibrinolytic agent
• Hemarthrosis (bleeding in joints) can be common
  • Signs include joint stiffness, tingling and pain, warmth, redness, swelling, and pain
• If hemarthrosis:
  • ICE pack
  • No weight bearing
  • Elevate affected limb above heart
  • Immobilization (NO PROM exercises until acute injury phase is done)
• Nursing care:
  • Planning/coordination for dental and surgical procedures
  • Teaching regarding disease awareness
  • Teaching/support for home infusion
  • Health promotion/prevention
  • Non-contact sport choices

Hemophilia: Home Treatment and Family Teaching

• Timely treatment (DO NOT DELAY THE ADMINISTRATION OF FACTOR!!!!)
  • Decreased joint damage & disabilities
  • Decreased hospitalizations
  • Increased school attendance
  • Increased normal family life
• Family teaching:
  • Teach venipuncture and factor administration
  • Stress that factor administration is NEVER DELAYED
  • Avoid aspirin, but Motrin is ok because the platelets are the problem
  • Physical activity and exercise are fine and encouraged when not bleeding (choose exercises/sports carefully)

Sickle Cell Disease (SCD)

• SCD is an autosomal disorder mainly affecting African and Middle Eastern/Mediterranean individuals.
• In SCD, hemoglobin A is replaced by hemoglobin S.
• #1 inherited disorder in US
• It follows an autosomal recessive inheritance pattern.

SCD: Sickling and Clinical Symptoms

• Sickling is related to the concentration of hemoglobin within the cell
  • Sickling occurs if there is an increased concentration of hemoglobin.
• Babies with sickle cell usually do not experience crisis
  • Experience it after 3 months they still have fetal hemoglobin in circulation
• Sickle cell changes are related to blood viscosity and red cell destruction
  • Viscosity is an internal property of a fluid that offers resistance to flow.
  • Higher viscosity= the stickier the blood is
• Insufficient oxygen = cells to sickle and become rigid and clump together obstructing blood flow.

SCD: Consequences of Obstructed Blood Flow

• Obstructed blood flow leads to ischemia
• All systems affected: stroke (CVA), circulation issues (thrombosis)
• Bone marrow is hyperplastic, but the rate of destruction exceeds the rate of production
  • Infarction,,,death,,,stroke-cva-circulation issues-thrombosis

SCD: Crisis

• Situations that can cause sickling, include fever, physical or emotional stress, or any condition that increases the need for oxygen or alters oxygen transport.
• The sickling response is reversible with oxygenation and hydration
  • The cell becomes permanently sickled after repeated sickling
• Health maintenance

SCD: Care and Crisis Types

• Comprehensive care
• Immunizations:
  • All recommended vaccines
  • Pneumococcal
  • Meningococcal
  • Annual influenza
• Penicillin prophylaxis
• Regular screenings
• Transition to adult care providers
• Types of Crises:
  • Vaso-occlusive/pain crisis (VOC)= most common and PAINFUL, results from clumping of sickled cells that obstruct blood flow
  • Acute chest syndrome (ACS)
  • Splenic sequestration= large amounts of blood pooled in the spleen causing drop in circulating blood volume, eventual shock
  • Aplastic= diminished RBC production, usually a result of a viral insult (parvovirus)

SCD: Nursing During a Crisis

• ABCS
• Maintain IV hydration (but don't overdo it)
• Administer O2 (humidified) and monitor respiratory assessment
• Treat any infections promptly
• Control pain
• Bedrest (want to decrease cellular metabolism)
• Treat anemia
  • Possible will need apheresis or exchange transfusions (differences between heme & onco kids)
  • transfuse with a hemoglobin or 8 or less (platelets of 10 or less) for kids with cancer
  • Kids with SCD usually "live" at a Hgb of sometimes 6-7

Sickle Cell Disease- Labs

• Hemoglobin electrophoresis- absence of hemoglobin A
  • Only accurate diagnostic test for SCD
  • This test tells us which types of hemoglobin are present and their concentration
• CBC- decreased Hgb/Hct
• Increased reticulocyte count
  • Measure of RBC production
  • Anemia stimulates grater RBC production
• Increased WBCs and platelets
• Increased total bilirubin
• Average Hgb below 7 and a white count over 13,700 in the first decade of life are indicative of a more severe course of the disease

SCD: Infection Risks

• #1 cause of death and hospitalization in kids with SCD:
  • INFECTION!!!! this is a medical emergency
• Types of infections in patients with SCD:
  • Bacteremia
  • Pneumonia (#1 cause of death is streptococcus pneumoniae sepsis)
  • Osteomyelitis
• #1 cause of hospitalization in kids with SCD= vaso-occlusive crisis!!!!
• Why infections?
  • Functional asplenia: no function by 6 months of age
    • Can be detected on CBC smear by presence of Howell-Jolly bodies
  • Ineffective antibody production
  • Increase susceptibility of infections
  • The spleen is a low oxygen environment tendency for RBCs to sickle there

SCD: infection/sepsis

• Temperature greater than 38.5C (101.3F)
• Sick appearance with or without fever
• Pneumonia
  • Cough, chest pain, respiratory distress
• Osteomyelitis
  • Localized bone pain, swelling, redness, warmth
• Treatment of infections:
  • CBC, retic, blood cultures
  • Consider CSF, throat and urine cultures if symptomatic
  • CXR if pneumonia is suspected
  • Aspirate or radiologic testing of bone if osteomyelitis is suspected
  • IMMEDIATE ADMINISTRATION OF ANTIBIOTIC (even if potential infection)

Post-Splenectomy Care in SCD

• The spleen filters blood in splenic sequestration, the red cells become trapped in the spleen causing splenomegaly which may lead to hypovolemia, anemia, and shock
• Functional asplenia= sickled cells are trapped in the spleen and get replaced by fibrous tissue
• In patients who need their spleens removed, they must be on prophylactic antibiotic coverage (Pen VK)
  • Because spleen filters blood and releases phagocytes which help fight bacteria
• If splenic sequestration:
  • PICU transfer
  • Immediate, conservative packed RBC transfusion (minimum of 10 cc/kg)
  • Raise Hbg to no higher than 8 to avoid autotransfusion

SCD: Splenectomy

• Antibiotics
• These children may rapidly deteriorate!!!
  • Blood trapped in the spleen can accumulate very quickly causing a rapid deterioration in the child's condition, placing them at risk for circulatory collapse
• Nursing care:
  • Close monitoring
  • Know risk factors (less than 5 years old, infection, history of sequestration)
  • Discharge teaching (splenic palpation, treatment to avoid recurrent episode (chronic transfusions, splenectomy))
• Post splenectomy, often penicillin will continue beyond the age of 5 years & child should receive pneumococcal and meningococcal immunizations if they do not have them

SCD: Vaso-occlusive Crisis-

• Nursing care of the child in vaso-occlusive crisis
• The #1 reason for ED visits in the SC population
• Results from:
  • Ischemia secondary to vaso-occlusion
  • Damage to vascular endothelium
  • Inflammation
• Under conditions of hypoxia or acidosis, sickling of the RBCs occurs
  • Example= bony infarcts or avascular necrosis

SCD: Pain Management

• How to know if it's pain:
  • Verbalizes pain (#1 indicator of pain crisis)
  • Tachycardia
  • Tachypnea, splinting, decreased respiratory effort
  • Hypertension
  • Crying, clinging, inconsolability
  • Refusal to be touched
  • DO NOT RELY ON PATIENT'S ABILITY TO BE DISTRACTED TO DECIDE AMOUNT OF PAIN!!!! Nursing care:
• Hydration (usually D5W .45 NS with potassium, 1.5x maintenance, not for every type of crisis!)
  • Careful I&Os
• Analgesics:
  • Opioid/NSAID combo (morphine, fentanyl, hydromorphone, ibuprofen, ketoprofen, ketorolac)
    • Ketorolac= PO or IV, use for less than 5 days/month
      • Side effects= gastritis, renal failure (gastric bleeding and renal impairment) -Adjuvants (diphenhydramine, hydroxyzine, lorazepam)
    • Appropriate dose
    • Appropriate time intervals -Only every give Demerol to kids if they are rigoring (shaking chills you can't get rid of)!!! Demerol should never be given to kids due to risk of seizures from byproduct of the drug!!!!
    • Antibiotics if infection is suspected
    • Oxygen is not routinely given unless there is an increased O2 demand/hypoxia

SCD: Managing Side Effects & and Other Treatments

• Managing side effects of pain management:
  • Sedation, respiratory depression= continuous pulse ox, less with small frequent doses
    • Sedation interventions: -Sleeping, easy to arouse= no intervention -Drifts off while talking= stimulate lower dose -Somnolent, minimal response= O2, discontinue opioid, give Narcan -N/V= antiemetics, evaluate for constipation -Pruritus= antihistamines (histamine release is NOT an allergy) -Constipation= stool softeners and/or laxatives -Withdrawal= more than a few days of opioids, taper off
• Other treatment options of pain management:
  • Psychological= distraction, imagery, education, hypnotherapy, psychotherapy
    • Behavioral= deep breathing, relaxation, exercise, biofeedback, behavior modification
    • Physical= hydration, heat, massage, PT, acupressure
• Consider development, family coping styles, cultural practices, and past pain experiences
• Adequate pain control:
  • ATC regiment should be instituted
  • Usually Ketorolac (Toradol), morphine, Motrin
  • Use PCA if needed
  • Use non-pharmacological techniques as well (heating pads, guided imagery, distraction)
  • Assess and reassess pain frequently
  • ADVOCATE
  • Monitor your own beliefs and listen to your patient!!!!

Sickle Cell Disease- Genetics and Patient Education

• Autosomal recessive inheritance pattern
  • Both parents must carry the mutation
• There is a 25% chance that a baby will inherit two genes for sickle cell disease or two genes for normal hemoglobin
• There is a 50% chance a baby will inherit one gene for sickle cell, and one gene for normal hemoglobin
• This inheritance pattern holds true with each pregnancy
• In cases where carrier states are known chorionic villi sampling (CVS) and amniocentesis are both useful in the prenatal diagnosis of sickle cell disease
• Individuals with the trait have the same defect, but only have about 35-45% of hemoglobin S most of them are asymptomatic
• Those with the disease have more hemoglobin S, and therefore, have the disease
• Patient education for home care of kids with SCD-fever management, managing pain
• Discharge teaching:
  • Assessment tools
  • Tapering PO analgesics
  • Side effects
  • Incentive spirometry, deep breathing
  • Tolerance vs. addiction
  • Keep pain journal

Adjuvant Medications in Pediatric Pain Management

• Pain management in children
  • Meds that are not pain meds but help manage pain (adjuvant medications- valium, Ativan-why would we give these if patient is in pain?)
• Adjuvant medications are used on an “as needed" basis to relieve concurrent symptoms, such as nausea or anxiety
• Side effects of opioids, such as constipation
  • Prevented and treated with laxatives and stool softeners, which should be ordered as scheduled adjuvant medications anytime an opioid is prescribed
• Diphenhydramine (antihistamine) treat pruritis
• Hydroxyzine (antihistamine) treat pruritus
• Lorazepam (antianxiety) treat anxiety

Anemia

• Signs and symptoms of anemia
  • Anemia= reduced RBCs
• Why anemia?
  • Decreased RBC production
    • Aplastic anemia, hemophilia, ITP, DIC, vWD
    • Pallor
    • Increased HR
    • Fatigue
    • H/A
    • Muscle weakness
    • Systolic murmur
    • Frontal bossing (prominent forehead)
  • Increased RBC destruction
    • SCD, thalassemia
    • Low BP
    • H/A
    • Increased HR
    • Jaundice
    • Icteric sclera
    • Dark urine
    • Fatigue
    • Hepatosplenomegaly
  • Increased RBC loss
    • Fatigue
    • Pallor
    • H/A
    • Increase HR
    • Cool skin
    • Decreased peripheral pulses
    • Low BP
• We expect reticulocyte count to be increased in anemia because it means the bone marrow is trying to make new RBCs
  • Anemia with high RC reflects an increased erythropoietic response to hemolysis or blood loss
  • Anemia with a low or normal RC reflects deficient production of RBCs (ex: a reduced marrow response to the anemia)
• Hemolysis or blood loss can be associated with a low reticulocyte count if there is a concurrent disorder that impairs RBC production (ex: infection)

ITP: Immune Thrombocytopenic Purpura

• ITP is idiopathic thrombocytopenia purpura
• Most common bleeding disorder in children!
• Acquired hemorrhagic disorder resulting from autoimmune destruction of platelets
• Most common bleeding disorder in kids
• Two forms: acute(which is self-limiting, ages 1-9) and chronic (ages 10+)
• ITP presents with petechiae, easy brushing, ecchymosis, and bleeding from mucous membranes
  • Other manifestations may be epistaxis, hematuria, menorrhagia, and melena
• About 50% of acute cases follow a viral infection

ITP: Immune Thrombocytopenic Purpura - Labs and Pathophysiology

• Lab data: low platelet count (less than 20,000) and antiplatelet antibodies, normal Hgb, normal differential, normal bone marrow (only done if something else is suspected)
• Pathophysiology:
  • Immune dysregulation leads to antibody mediated destruction of platelets by sleep and liver
  • In ITP, production isn't the problem, it's the destruction that's the problem!!
  • ITP is initiated when the immune system becomes dysregulated as a result of some stimuli, like a viral illness or vaccination as a result of dysregulation, the immune system becomes sensitized to platelets and proceeds to coat them with antibodies, this signals the phagocytic cells of mainly the spleen but also the liver to destroy the platelets, platelets are destroyed faster than they are produced, and a deficiency occurs

ITP: Treatment

• Treatment is NOT curative and is done with the purpose of treating bleeding complications
  • Aimed to prevent ICH (intracranial hemorrhage)
  • Includes:
    • Local measures
    • Steroids
    • Immunoglobulins (IVIG)
    • Anti D (winRho) in Rh+ patients only Platelet transfusions are RARELY given→ this is because the body is destroying it
• Splenectomy only in severe cases
• Most children recover with no long-term consequences

Iron Deficiency Anemia

• Worldwide most widespread nutritional deficit
• Most common childhood anemia
• Problems with supply, demand, absorption, or storage
• Rise in iron deficiency anemia among infants who are exclusively breastfed
  • By the age 6-9 months, infants deplete their maternal stores of iron (this occurs earlier for premature infants)
  • If iron is not coming from another source (formula, cereal, iron supplements), infants begin to lose the ability to produce RBCs
• Infants who drink a lot of cow's milk have an increased risk for anemia because it is difficult for young infants to digest cause microscopic loss of blood through Gl tract
• Adolescent females are also at risk for iron deficiency anemia because of iron loss through menstruation and poor dietary habits

Iron Deficiency Anemia: Labs

• What we will see: Pallor, weakness, fatigue Labs:
• Decreased Hgb & Hct
• Decreased RBCs
• Increased reticulocyte count due to blood loss low if inadequate intake What can I do?
• Make sure babies don't drink milk exclusively before 1 year iron fortified formula or breast milk + food after 6 months of age
• Treatment is 1 mg/kg/day of iron bid and continued 6-8 weeks after hemoglobin is back to normal
• Tips for giving iron supplements: Give with fruit juice as it increases absorption
• Do not give with milk as it decreases absorption
• When giving liquid iron, administer through straw or perform oral care after administration (iron stains their teeth)
• Monitor for constipation

Hematology: Counts and Parvovirus

• Know normal HGB and HCT counts
  • Hgb= infant: 10-18, child: 12-16
  • Hct= 36-48
  • Reticulocyte count= 0.5-1.5/100 RBCs or 0.5-1.5%
  • WBC= infant: 9-30, child: 4.5-13.5
• Parvovirus and staff education
  • Aplastic crisis is almost always a result from parvovirus
  • Human parvovirus, if contracted in pregnancy, can cause harm to the fetus
  • Staffing arrangements should be made to eliminate contact with these patients by all pregnant staff members Infection with parvovirus offers life-long immunity

Beta Thalassemia: Treatment

• Classified by the hemoglobin chain that is affected
• Most common form is beta thalassemia major or Cooley's anemia
• • Autosomal recessive (both parents must be carriers to produce a child with beta thalassemia) Highest incidence in Mediterranean, Syrian, Italian, and Greek descent Treatment is supportive and involves chronic RBC transfusions- but this is not without side effects
• The disease causes chronic hypoxia (H/A, bone pain, irritability, anorexia, listlessness) and the supportive care can cause iron overload (hemosiderosis) Iron overload, chelation therapy, Desferal (12 hours IV infusion daily) What will the labs reveal?
• Decreased Hgb
• Decreased MCV
• Hypochromic RBCs (and microcytic) -Increased reticulocyte count
• Hemoglobin electrophoresis is diagnostic (decreased Hgb A and increased Hgb H) Ferritin levels must be watched very carefully so that chelation therapy may be initiated or adjusted as needed to avoid the serious complications of iron overload
• Clinical presentation and complications: Prenatal diagnosis Newborn screening Infancy FTT/irritability Splenomegaly Pallor/severe anemia

Hemoglobin: Clinical Presentations and Treatment

• Older child: Bony changes Splenomegaly Iron overload Anemia Iron overload Cardiac disease (70%) Infections/sepsis Bone marrow expansion Bone deformities (facial changes, paraspinal deformities) Osteoporosis (fractures)
• If early diagnosis is not known, these symptoms of anemia and poor growth and development will become apparent in the first 6 months of life -If previously undiagnosed and not experiencing life threatening anemia, older children will have signs of transfusion needs not being met such as bone changes and splenomegaly (may also have iron overload secondary to increased iron absorption in the Gl tract) Treatment: Rbc transfusion (keep Hgb above 10) Folic acid Cheation Deferoxamine (Desferal) Deferasirox (Exjade) Splenectomy Bone marrow, cord blood, stem cell transplant (best treatment) Gene therapy in the works
• Do not give iron!!! iron overload!!!

Von Willebrand's Disease (vWD)

• Goals of nursing care:
• Educate and promote compliance with supportive treatments
• Support family and child with chronic nature of disease
• Monitor and manage blood transfusions
• Type I vWD:
• Quantitative issue -Gene leads to decrease in vWD production -- Genetic mutation causes an alteration in protein production of vWD generally leading to prolonged bleeding episodes Less protein available for primary hemostasis Autosomal dominant pattern

VWD Types II and III, Genetics, and Clinical Findings

vWD: -Qualitative issue Poor quality of vWD Autosomal recessive III vWD: Qualitative issue Absence of vWD (rare) Autosomal recessive

• Genetics: Autosomal dominant inheritance pattern Parent with vWD (50% chance in offspring) Type IIN & III (severe) are recessively inherited Clinical findings:
• Easy bruising -Recurrent, prolonged epistaxis
• • Problems with mucosal bleeding
• Menorrhagia → prolonged uterine bleeding, might have to go on birth control -Prolonged bleeding (from lacerations, dental work, IM injections or surgery)

VWD: Treatment and Nose Bleeds

Treatment: Treatment is given pre-operatively with surgery/dental work or bleeding episodes Desmopressin (DDAVP) Given to patients either intranasally, subq, or IV Acts to raise endogenous levels of vWF by stimulating release of stored vWF from endothelial cells vWF: FVIII concentrate (Humate P)

• reserved for serious surgeries, bleeding complications, or those unresponsive to DDAVP
• Made from a plasma-based product
• Other supportive measures: Anti-fibrinolytics: Tranexamic acid and aminocaproic acid inhibit antifibrolysis in order to keep the clot in place once it has formed Oral contraceptives: To control menorrhagia Topical hemostatic agents
• Treatment for vWD is given either in preparation (prophylactically) for procedures or for treatment of bleeding injury
• Management of nose bleeds Teach patient/parent to manage nosebleed by leaning FORWARD and apply continuous pressure to tip of nose with thumb and forefinger for at least 10 minutes Avoid all temptation to pick or blow your nose after a bleed

Neutropenia

• Low neutrophils→ body ability to fight off infection is low
• The ANC (absolute neutrophil count):
• Add the Segs (grown up neutrophils) and the Bands (immature neutrophils) together and multiple by the total WBC
• A person is considered neutropenic when their ANC is less than 1000-1500
• You get your ANC from the differential part of the CBC Automatic: done by computer, much better looking for NUMBERS Manual: lab technician is going to look at blood cells under microscope and DESCRIBE what they're seeing
• When you get a fever and you have neutropenia EMERGENCY!!!!!
• Susceptibility to infection increases Infection prevention is key!!!! (priority of care) Some may benefit from colony stimulating factors (G-CSF)
• Educate parents about “crowd control”, hand washing, and to immediately report any fever of 38.3 (100.4) or greater

Cancer: Risk Factors and TLS

• Prognostic factors / indicators in cancer -Type of malignancy
• -Age at diagnosis
• Primary site Extent of disease
• -Biologic/genetic markers
• Tumor Lysis Syndrome: definition, cause, treatment An oncological emergency that can happen with the induction of treatment As leukemic cells die, their intracellular contents leak into the blood
• Can be deadly this is why we do lysis labs, give allopurinol, and hyperhydrate with sodium bicarb
• In worst cases, kids need dialysis because they go into renal failure Patients at risk:
• Burkett's Lymphoma
  • ALL/AML -Management: Frequent labs Hydration/diuretics Electrolyte supplements

Leukemia: Bone Marrow

• Bone marrow aspiration/biopsy: nursing care of patient undergoing procedures, patient education
• Bone marrow aspiration and biopsy along with LP are done at diagnosis of ALL (acute lymphoblastic leukemia), and periodically throughout treatment to check progress of treatment
• Bone marrows are done at day 0, 8, 15, and 23
• Packed marrow only has one type of cell in it because the leukemia cells have overgrown and crowded out the normal cells
• Positioning for LP: you want to open up the intrathecal spaces need to curve the kid on their side with knees to chest so that they make a “c” shape, but tighter
• Initial CBC findings in ALL -WBC: increased Hemoglobin: decreased (pancytopenia)
• Platelets: decreased (thrombocytopenia) Neutrophils: decreased (neutropenia) Blast cells present Immature WBC (neutrophil)

Treating Leukemia

• IVF in initial ALL treatment
• -Induction: IVF, allopurinol
• -Used to prevent uric acid crystals along with bicarb in IVF
• IVF at 1 1½ maintenance for hydration and to flush our kidneys and again, prevent uric acid crystals
• Why do we give intrathecal chemo?
• -To treat any disease hiding in the CNS In between 3rd and 4th lumbar vertebrae Brain tumors: signs, symptoms and treatment
• Supratentorial:
• Structures- cerebrum, basal ganglia, thalamus, hypothalamus, pituitary, pineal, optic Symptoms- increased ICP, vision changes, headache, emesis, change in behavior, school difficulties, seizures, Parinaud's syndrome (paralysis of upward gaze) Infratentorial:
• Structures- cerebellum, brainstem
• Symptoms- ataxia, head tilt, nystagmus, cranial nerve palsy, increased ICP, morning emesis, back pain, loss of bladder & bowel function Morning emesis & bad headache in the morning that gets better as the day goes on= INFRATENTORIAL!!!! Clinical presentation: Localizing symptoms (depends on tumor location) If you know where the tumor is and what that area of the brain controls, it will help guide your assessment
• --Nursing care of kids with brain tumors: ABC become primary concern→ the ICP Neuro exam Safety Be complimentary Give yourself a break

Lymphoma and Tumors

Benign: Usually, one cell of origin Slow growing Localized Insidious/gradual S&S Malignant: Greater differentiation Fast growing Invades surrounding structures Mets outside CNS Sudden/intense S&S

Lymphoma Signs & Hodgkin's

• First line of treatment for brain tumor= surgery
• Hodgkin's disease: initial signs/symptoms, “B” symptoms
• Hodgkin's lymphoma: Peeks in adolescence Males more than females Cervical lymphadenopathy (painless, rubbery, firm, movable node) “B” symptoms: fever higher than 38 (100.4), unintentional weight loss greater than 10% body weight in 6 months, drenching night sweats Fatigue, loss of appetiteNon-Hodgins's lymphoma: Malignant, solid tumor of immune system Cells are undifferentiated lymphoid cells or their precursors Cells spread in a random, diffuse and aggressive pattern

Lymphoma Types

• Types include: Burkitt's, Lymphoblastic, and Large Cell
• Presentation: T cell lymphoma: mediastinal mass B cell: bone or nodes involved Burkitt's: jaw and abdomen(fastest growing pediatric malignancy) Anaplastic large cell: slowly progressive, includes nodes, bones, and skin Mediastinal mass, watch for respiratory symptoms, no sedation, cannot lie flat

Neutropenia- Mediastinal Mass

Nursing care:

• Watch respiratory status
• Positioning of patients CANNOT LIE FLAT!!!
• Theyre gonna struggle to breath Tumor lysis
• Anesthesia for these patients Very careful and monitored anesthesia
• Mucositis/Nausea and Vomiting: signs/causes/treatment Know emetogenic potential of meds N/V can be anticipatory, delayed, or chronic PREMEDICATE AND CONTINUE TO MEDICATE- you do not have to vomit to get meds!!! Mucosal damage along Gl tract= mucositis Bland diet (usually give nutrition in IV)

Mouth Care is Key

Mouth care:

• Soft toothette
• Frequent mouth rinses
• Local anesthetic/pain control SO PAINFUL!!!
• The clue (especially in babies) is refusing PO, drooling (because it hurts to swallow)
• It is drug dependent
• Nutritional concerns:
• Contributing factors:
  • N/V
• Tumor related Management:
  • TPN VS NGT feeds -Small, frequent meals -Nutritional supplemen ts -Assure caregivers Nursing care of kids with mediastinal masses Watch for respiratory symptoms NO SEDATION! CANNOT LIE FLAT!!!!

Radiation Therapy

• Care of kids receiving radiation therapy (labs, skin care): Premeds if needed Watch skin Be careful of dressings, lotions, constrictive clothing Watch for mucositis Watch for somnolence syndrome (educate caregivers) Went to sleep and never woke up still breathing and vitals are still being read, though Make sure Hgb is at least 10 (to promote efficacy of radiation with oxygen carrying capacity of cells

Patients receiving radiation

Nursing considerations for patients receiving radiation treatment: Short term side effects: Nutrition N/V Mouth sores Fatigue Reddened or itchy skin Hair loss Pancytopenia Changes in taste and smell Sleepiness (somnolence syndrome) Swollen parotid glands Decreased saliva Long term side effects: Cognitive function Radiation-site specific problems Delayed/absent puberty Fertility Secondary tumors

Complications and the Effect of treatment

Managing side effects and complications of tx: Myelosuppression Alopecia Infection/sepsis Bleeding Anemia Mucosal ulcerations Pain N/V Nutrition Tumor lysis syndrome Steroid effects Hemorrhagic cystitis Side effects of treatment: Bone marrow suppression Transfusion support (Hgb less than 8, transfuse normally) Keep platelets greater than 50 in neuronic kids (normally transfuse under 10 or active bleeding) No rectal temps for onco kids!!! Can cause infection in butt

Abdominal Tumors and Wilm's Tumors

Abdominal tumors (Wilm's/Neuroblastoma)- assessment and nursing care Wilm's tumor: Kidney tumor Most common primary malignant renal cancer in children 460 cases/year Peak age: 2-3 years Caregivers discover tumor while bathing, changing diapers, or tickling the child

• The higher the number of the stage, the more disease you have Neuroblastoma: Adrenal tumor Most common pediatric extracranial tumor 800 new cases/year Derived from neural crest cells (adrenal medulla and ganglia) 90% diagnosed under 5 years old Prognosis depends on age of child and stage of disease NBL presentation: Abdominal pain, fullness Respiratory difficulty Fatigue Fever Periorbital ecchymosis (“raccoon eyes”) indicates spread to the brain “blueberry spots" indicates spread to the skin

Neuroblastoma: Symptoms

• Opsoclonis-Myoclonis: rapid eye movements, involuntary twitching of extremities
• Horner's syndrome: damage to sympathetic nervous system, ptosis (drooping of upper eyelid), lack of sweat, miosis (pupillary constriction, one pupil is really big, one is really small)
• NBL staging: I- localized disease with complete resection Ila- localized with gross residual disease Ilb- localized disease with nodes III- spread across midline IV- disseminated disease IVS- distinct disease localized with spread to skin, liver, or marrow in infants under 1 year Pretty much magically goes away
• Nursing care of kids with abdominal tumors: Watch respiratory status Abdominal girth Palpation (don't want to palpate because we don't want to rupture the tumor) Monitor BP Watch urine output Why are some cancers treated with chemo first vs. surgery and vice versa? If the tumor isn't causing anything bad, chemo first to try to reduce it If tumor is causing harm (like brain tumor causes ICP) surgery to try to get it out faster If they can do chemo first, they will

Ewing Sarcoma

• Ewing's sarcoma signs and symptoms Bone tumor
• Presentation: Pain Pathologic fracture (fractures that result from activities that would normally not result in a fracture are highly suspicious) Back pain Fever, weight loss
• Affects long, flat bones (