Pedatric Hematology/Oncology

What are the 3 main hematologic Disorders

• Sickle cell anemia

• Hemophilia

• Iron deficiency anemia

What is Iron deficiency anemia and s/s

most common ind of anemia in the US caused by inadequate supply of iron that can be caused by any number of fcators

• normally children ages 12-36mnths and 12-16ys

s/s

• Shortness of breathe

• Pallor

• Fatigue, irritability, muscle weakness

• systolic herat murmur

• enlarged heart

• can lead to cognitive, behavoiral and motor impatrment if sever or left untreated

• low CBC, HGB, HCT and ferritin

What are the risk factors for Iron deficiceny anemia

• Premature Birth

• Excessive intake of cow’s milk

• Inadequate intake of iron

• Malabsorption disorders

• Increased iron requirements( esp for active kids)

• Chronic disorders

• Lead exposure

• Puberty

How is iron deficney anemia treated

• Iron supplement administration

• Educate about supplements

• Monitor milk intake

• Packed RBC’s if needed

• Dietary sources of fiber

• O2 needed for severe hypoxia

• Frequent rest periods

• Instruct to parents to return for follow-up

What is Sickle cell disease and s/s

A autosomal recessive genetic disorder when normal hemoglobin is partly or completely replaced by abnormal sickle hemoglobin leading to

• Increased blood viscosity

• Obstruction of blood flow

• Tissue hypoxia

s/s:

• Extreme pain

• shortness of breath

• Fatigue

• Pallor

• jaundice

• Hands and feet cool to touch

• Dizziness 

• Headache

what are the types of crisis caused by Sickle cell disease(4)

• Vaso-Occlusive/ Sickle cell crisis

• Aplastic

• Hyperhemolytic

• splenic sequestration

What is Vaso-Occlusive/ Sickle cell crisis and s/s

from the abnormal replacement of normal hemoglobin (Hgb A) by sickle hemoglobin (Hgb S), which causes the blood to sickle. This sickling leads to increased blood viscosity and the obstruction of blood flow.

This crisis can cause swollen joints and pain in the long bones, back, hands, and feet, and it typically lasts minutes to days

• Swollen joints.

• Pain affecting specific areas such as the long bones, back, hands, and feet.

• The presence of jaundice.

• The crisis typically lasts minutes to day

what is Aplastic Crisis

a sudden episode of severe anemia in patients with sickle cell disease (or other hemolytic anemias) caused by a temporary halt in red blood cell (RBC) production by the bone marrow.

• Decreased RBC production

• Extreme Anemia

• Typically triggered by an infection

(URI, B-19 Parvovirus)

What is Hyperhemolytic crisis

rare but severe complication of sickle cell disease in which there is accelerated destruction of red blood cells (RBCs), leading to sudden worsening of anemia.

• Increased rate of RBC destruction ->anemia->jaundice/reticulosis

what is splenic sequestration crisis

life-threatening complication of sickle cell disease in which a large number of sickled red blood cells become trapped in the spleen, causing sudden splenic enlargement and severe anemia.

• Excessive pooling of blood ->splenomegaly, and/or hepatomegaly

• Hypovolemia- due to reduced circulating blood volume-> hypovolemic shock

what complications are associated with sickle cell disease

• Stroke

• Acute chest syndrome

• overwhelming infection

what meds are used to treat sickle cell disease

• NSAIDS-> Add codeine

• Morphine or Dilaudid/ PCAs

• Avoid Demerol because it is CNS stimulant (they are at risk for seizures)

• SL Morphine at home

• Penicillin- Prophylactically due to high risk for pneumococcal infection

• Folic Acid- Helps make new RBCs

• Hydroxyurea- Chemotherapy agent that increases fetal Hgb and helps reduce complications (FDA approved for SCA – pain crises and helps ^ o2 to the cells).

• Vaccines- Pneumococcal, meningococcal, Hib

what are some important management points for sickle cell disease

• Oral or IV therapy to prevent further sickling events

• Electrolyte replacement to avoid metabolic acidosis which may heighten the sickling of cells

• Diuresis can lead to hypokalemia so need to monitor

• Temp greater than 101 should be evaluated

• Manage with opioid analgesics

• No fear of addiction/ high tolerance

• Medical emergencies

  • ACS, Priapism, Severe HA, Jaundice, Abd distention, Febrile

• Medical identification: School bracelet/Medical bracelets

What is Hemophilia

A severe inherited hemorrhagic disorder with a group of bleeding disorders, including Von-Willebrand’s disease.

• Mechanism: Caused by deficiency in clotting factors (not platelets), leading to difficulty controlling bleeding.

• Inheritance: Hemophilia A & B are X-linked recessive, mostly affecting males.

• Severity: Can vary from mild to severe.

Key Types:

• Hemophilia A: Deficiency of Factor VIII.

• Hemophilia B (Christmas disease): Deficiency of Factor IX.

• Note: Von-Willebrand disease also affects clotting; unlike Hemophilia A & B, it affects both males and females and is less common.

Signs and Symproms of hemophilia

• Excess bleeding and bruising.

• Joint pain and swelling.

• Decreased range of motion

how is hemophilia treated

Primary therapy is replacement of missing clotting factor

• Corticosteroids

  • Decreases swelling and inflammation from bleeds

  • Ice helps with hemarthrosis

• NSAIDs avoid

• Positioning & pressure for Bleeds

  • Cold compresses (not heat)

  • Elevation

  • Rest

• Amicar (amino caproic acid)

  • Prevents clot destruction and promotes clotting

• Physical therapy- Encourage Active ROM once healed or bleeding controlled

  • Swimming, bike riding , fishing, golfing, stretches

what causes Childhood Cancer

• normally is unkown in most cases

• can be from chnage sin cell’s DNA

• enviroment causes can play a part( from electromagnetci fileds, chemcials, pesticides, viruses, external stimuli)

• certain chromosome can predispose chidlren to cancer

  • down syndrome= 2-% higher risk for leukemia

  • missing geteic material on chromosome 13= higher risk for retinoblastoma

  • missing pt of chromosome 11= higher risk for wilms tumor

what is teh diff between peds and adult cancer

peds cancer:

• Faster growing (usually)

• Nonepithelial or embryonal cell types

• Usually embryonic or oncogenic in origin

• Child usually becomes ill within days/weeks

Adult cancer:

• Slower growing (usually)

• Epithelial in origin

• Due to prolonged exposure to toxins or resulting from oncogenic responses to stimuli

• May have it for years before showing symptoms

what is leukemia

cancer of boen marrow an dlymphatic systems

what is lymphoma

cancer of lymphoid and hematopoietic systems

how are solid tumors classified

by location

what are the most common children cancer

Leukemia: the most common cancer in children younger than 14

3 main types

• ALL (Acute Lymphoblastic or Lymphocytic)

• ANLL or AML (Acute Non-lymphocytic) or (Acute Myelogenous)

• CML (Chronic Myeloid)

what are the most common childhood cancers and when they normally start

Neuroblastoma- <10 yrs. of age

• Most common solid tumors

• Occur in the nerve tissues

Hepatoblastoma- infant-3 yrs. of age

• Arises in the liver

• Wilm’s Tumor (Nephroblastoma)- 3-5 yrs. of age

• Most common malignant renal and intrabdominal tumor, favors left kidney

Retinoblastoma- <5 yrs. of age

• Arises in the retina

• White glow

Non-Hodgkin’s Lymphoma- <14 yrs. of age

• Malignant tumors of lymphoreticular origin (found in internal framework of the lymphatic system)

Rhabdomyosarcoma- <5 yrs. of age most commonly seen, but up to 19 yrs. of age

• Most Common soft tissue sarcoma in children

• Tumors found in striated skeletal muscle

what are the most common adolecence cancers and when they normally start

Hodgkin’s Lymphoma- between 15-19 yrs. of age

• Most curable (95%-97% cure rate)

• Main symptom is firm, enlarged, non-tender lymph nodes

Osteosarcoma- peak age 15 yrs.

• Originates in bone

• Seen during growth spurts

Ewing’s Sarcoma- <30 yrs. of age

• Occurs in the shafts of the long bones and trunks

• Can be found in bone or soft tissue

What are the main general manifestaions of cancer

Pain: may be directly related to neoplasm or by indirectly affecting nerve receptors

• Tissue damage

• Obstruction

• Stretching of visceral tissue

• Invasion of tissue

What are some common s/s of Cancer

• Cachexia: syndrome characterized by anorexia, weight loss and early satiety from chemotherapy or chronic illness

• Anemia: iron is used poorly in chronic illness; bone marrow suppression (low red cell count) and causes fatigue, paleness and weakness

• Bruising: usually a result of bone marrow suppression (low platelet count)

• Infection: results from altered or immature immune system (neutropenia or low white cells), unexplained fever, frequent infections unresponsive to antibiotics

• Neurological symptoms: usually due to mass in the brain or invasion of the spinal cord by metastasis of cancer originating in another site.

• Palpable mass: due to primary cancer or metastasis

• Pallor

• Lethargy

• anorexia

• Pain

What Diagnostics are used for cancer

• CBC w/ differntial

• Bonemarrow aspiration

• Lumbar puncture

• magnetic resoance imaging

• computed tomography

• ultrasound

• biopsy of tumor

How is cancer treated

• Placement of central line/s- Portacath & Broviacs most common

• Chemotherapy & Biotherapy/Immunotherapy

  • Based on Type of Cancer, Cell Type, Location or spread

  • Phases:

    • Induction (remission)

    • Prophylactic (prevent CNS invasion)

    • Intensification or Consolidation (destroy remaining cells)

    • Maintenance (sustain remission)

• Radiotherapy (Radiation)

  • Irradiate the tumor or lymph glands, but not healthy tissue

  • Total dose is divided or several weeks

    • Skin care education is important

    • Time, Distance & Shielding less in children because external beam radiation is often used

• Steroids

• Bone Marrow Transplant:

  • Autologous (own cells)

  • Allogeneic (others, siblings best)

• Umbilical Cord Blood

  • Free, yet still some ethical concerns

• Surgery-Debulking or complete removal

• Supportive Care & Atraumatic Care

what are some complications of Post chemo

• Mucositis 

• Pain

• fever

• Infection

• Neutropenia

• Anemia

• Thrombocytopenia

• Nausea and vomiting

• alopecia

• tumor lysis syndrome

• spetic shcok

What is Tumor lysis syndrome and s/s

Rapid breakdown of malignant cell causing inadequate renal function causing

• Hyperuricemia

• Hyperkalemia

• Hyperphosphatemia

• Hypocalcemi

Associated with B-cell leukemia, Burkitt’s lymphoma (Non-Hodgkins), ALL & AML

Rapid onset with abdominal flank pain, oliguria, cardiac arrhythmias, altered level of consciousness, seizures

How is tumor lysis syndrome managed

• Hydration and Electrolytes (2-4x maintenance)

• Dialysis

• Sodium Bicarb (alkalizes urine), Allopurinol (converts metabolic by-products to uric acid), Furosemide (facilitates K+ excretion)

• ECG Monitoring

what is Mucositis

Gastrointestinal mucosal cell damage from Chemotherapy and/or Radiotherapy

• Stomatitis (Oral ulcers in mouth and pharynx)

• Ulcers along the alimentary tract (esophagus to rectal area)

• Pain

• Dehydration

• Anorexia

how is mucosistis treated

• Treat Pain: IV Morphine or Fentanyl via PCA pump

• IV Hydration or TPN until able to take P.O. fluids

• Oral Medication: Pink Magic (Benadryl and Maalox), Pepcid, Emend, Nexium, Protonix

• Antifungal troches or suspension (Nystatin)

• Use toothettes instead of toothbrushes for cleaning and to apply oral medications.

• Frequent mouth rinses with a Sodium Bicarbonate mouth rinse (no alcohol or viscous lidocaine)

• Anorexia: Bland, moist, soft diet (for severe mucositis, TPN is initiated)

what is the formula for ANC  and what do the numbers mean

Formula: WBC X (% of segs + % bands)/100

• ANC 1000-1500: mild risk

• ANC 500-1000: moderate risk

• ANC<500: severe risk for life threatening infections

• NADIR: 7-14 days after chemo

what are some important treatment for neutropenia

• Handwashing

• Avoid sick people and crowds (malls,movies,church, etc…)

• Limit fresh fruits and vegetables (harbor bacteria)

• Monitor temperature for fever-no rectal temps (>100.5)

• Oral Care-Nystatin/Bicarb wash, saline rinses

• Septra-prophylactically to prevent Pneumocystis carinii

• Neupogen (G-CSF)- increases bone marrow’s production of neutrophils

• Meticulous CVL care (sterile dressing, cap, and needle changes)

What is thrombocytopenia and s/s

Deficiency of platelets, less than150,000

• Platelet count < 50,000+ risk for bleeding

• Expect transfusion <20,000, b/c extra high risk of bleeding

• S/S: Petechiae, bruising, nosebleeds, bleeding from IV or incisional areas.

what precautions are used for thombocytopenia

• no rectal temps/avoid constipation/ no suppositories

• wear shoes/ slipper

• avoid pciking nose

• avoid valsava maneuver

• assess for chnages in neuro status if pt has severe thrombocytopenia

• soft toothbrush or toothettes

• longer pressure on sites of peripheral sticks

• assess for other s/s of bleeding

what is septic shock

Systemic response to pathogens and endotoxins usually associated with gram negative organisms

• Decrease perfusion, cellular hypoxia, death

• Risk factors are ANC< 100 and neutropenia > 7 days

• Poor skin integrity

• Severe mucositis

• Central Line , Port-a-Cath, PICC Line (possible infection)

what s/s of septic shock

Early signs

• tahcycardia

• tahcypnea

• Hyperthermia

• Poor perfusiion(cap refil more than 3 sec)

• Decreased blood pressure

Late sign

• bradycardia

• mental changes

• hypothermia

How is spetic shock treated

• Draw blood cultures FIRST

• Monitor blood pressure

• Bolus with Isotonic Solutions (NS/LR) PRIOR to antibiotics

• Administer antibiotics as soon as possible (post-bolus)

• IV Steroids & Vasopressors

What are some important probelms caused by radiotherapy

• Impaired bone growth-scoliosis, short stature, poor dental health, osteopenia/osteoporosis

• Endocrine disorders after radiation to head-hypothyroidism, SIADH, stunted growth

• Neurocognitive dysfunction-developmental delays, difficulty with math/school performance, behavioral changesPulmonary toxicity

• Sterility/Delayed puberty