OB/PEDS - EXAM 2

Pediatric Hematologic Considerations

• Production of blood cells begins at 8 weeks in utero

• Fetus receives iron through the placenta

• Erythropoietin is derived primarily from the liver in the fetus

• Kidneys are the primary source after birth

Iron-Deficiency Anemia

• Iron deficiency is most common nutritional disorder in the world

  • Adolescents and children go through rapid periods of growth and require more iron (at risk)

  • Peak prevalence in children 12-24 months (off formula) and then adolescence

• Disproportionately impacts children and pregnant women

• Occurs when the body doesn’t have enough iron to produce hemoglobin

Iron-Deficiency Anemia - Etiology

• Impaired absorption

• Increased requirement

  • Runners

• Chronic blood loss

  • GI bleed

• Dietary lack

  • Vegan - need beans, greens (have iron)

  • Adequate dietary intake of iron required for body to make enough hemoglobin

Iron-Deficiency Anemia - Risk Factors

• Prematurity

  • Immature gut and kidneys (poor absorption)

• Exposure to lead

  • Old homes have lead based paint (children ingest) or parents in the workforce

• Exclusive breast-feeding beyond 4 months old without iron supplementation

  • AAP recommends 1st 6 months for breastfeeding

• Cow’s milk without adequate iron intake

  • Fill up and not eat or microhemorrages in gut = slow blood loss

  • Switch at 12 months to cows milk

• Low socioeconomic status

  • Poor access, poverty

• Use of medications that decrease iron absorption

  • PPIs, H2 blockers, antacids, thyroid, antibiotics

Iron-Deficiency Anemia - Diagnostic Criteria

• For child 6 months to < 5 years:

  • Ferritin (blood test) < 15 micrograms/L

  • AND

  • Hemoglobin < 11 g/dL (for children 0.5 to 5 years)

REMEMBER THIS ONE

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• For children 5 to < 12 years:

  • Ferritin < 15 micrograms/L

  • AND

  • Hemoglobin < 11.5 g/dL (for children 5-12 years)

Iron-Deficiency Anemia - Clinical Manifestations

• Mild cases: often no symptoms

• Reduced growth in prolonged cases (decreased iron and hemoglobin)

• Common symptoms (d/t poor circulation of O2): pallor, fatigue, SOB, weakness, headache, dizziness, irritability, + murmur (blood is thinner)

• Other possible symptoms: pica (eat odd things; ice, dry wall (child), rocks (child)), unsteady gait, muscle weakness, difficulty feeding

Iron-Deficiency Anemia - Nursing Assessment

• Obtain thorough history

  • Ask about availability of iron-rich foods

  • Chronic: special diets (vegetarian)

• Inspect mucous membranes and skin for pallor (pull lip/eyelid in AA)

• Assess nails for spooning (chronic)

• Auscultate for murmur

• Assess spleen for enlargement (splenomegaly)

• Patient education on what foods to eat

Iron-Deficiency Anemia - Nursing Interventions

• Goal - determine and treat the underlying cause

• Breastfeeding mothers should increase their daily intake of iron (like red meat)

  • > 4 months need iron supplementation if just exclusively breastfeeding

• Formula-fed babies should have iron-fortified formula

• Limit cow’s milk intake to < 24 ounces/day for children > 1 year of age

  • Decreases risk of microscopic GI bleeding and increases appetite for other foods

  • Cows milk = decreases absorption of iron from foods in GI tract

• Avoid NSAIDS when possible

  • Can cause GI bleeding, irritation, and iron loss

Iron-Deficiency Anemia - Caregiver Education

• Give iron 1 hour before meals to maximize absorption

  • With syringe behind teeth or straw (d/t staining) in older children

  • Don’t give with milk

  • Citrus increases absorption

• Stools may be black in color

• Constipation may occur - increase fiber and water intake

  • Stool softener → miralax or apple juice

Common Sources of Iron

• Red meats (iron from this source is easiest for body to absorb)

• Tuna

• Eggs

• Enriched grains

• Dried beans

• Leafy green vegetables

• Iron-fortified cereals

• Dried fruits

Picture: heme iron (blood source) vs. non-heme iron

Sickle Cell Disease

• Inherited hemoglobinopathy (autosomal recessive)

  • Both carriers ~25% chance of offspring with disease

• Vaso-occlusive (temporary blockage of vessels - pain) phenomena and hemolytic anemia are the clinical hallmarks

• 3 most common types:

  • HbSS (homozygous) - symptoms are most severe (“so severe”)

  • HbSC - defect causing a structural variant in B-globin gene; causes deficiency and structural variations but milder features

  • HbS beta thalassemia - have a decreased production of normal hemoglobin

    • Thalassemia minor (B-thalassemia trait) - mild microcytic anemia (no treatment needed)

    • Thalassemia intermedia - child needs blood transfusions

    • Thalassemia major - ongoing medical attention, blood transfusions, and iron removal (chelation therapy)

Sickle Cell Disease - Diagnosis

• Newborn screening in all 50 states

  • Doesn’t distinguish between sickle cell disease and sickle cell trait

  • Electrophoresis confirms diagnosis of sickle cell disease

Mechanism of Vaso-Occlusion

• Normal RBC

  • Round shape

  • Soft/flexible to allow laminar flow of blood

  • Average lifespan 120 days

• Sickled RBC

  • Crescent shape

  • Sticky/rigid, inhibits laminar blood flow

  • Average lifespan 20 days

What happens in vaso-occlusion?

• Stress or traumatic event triggers sickling of cells

  • Physical (dehydration) + increased emotional state

• Blood becomes more viscous as sickled cells clump together

• Normal blood flow to tissues in that area is blocked (can be anywhere)

• Local tissue hypoxia followed by ischemia and possible infarction

• Pain due to decreased circulation in the area

• Increased metabolic need from pain causes tachycardia and tachypnea which leads to further sickling

Morphine (serious pain meds) and hydrate with fluids

Sickle Cell Disease - Nursing Assessment

• Skin - pallor, breakdown, lesions (where blockages are), jaundice (d/t breakdown of RBCs)

• Oral mucosa - pallor, dry/cracked

• Auscultate chest - murmur, adventitious or decreased breath sounds (acute chest = blockage in lungs)

• Palpation of extremities and abdomen - warmth/tenderness/ROM joints, abdominal tenderness, organomegaly

• Vital signs - elevated temperature, HR, RR, and hypotension (d/t decreased perfusion and dehydration)

Sickle Cell Disease - Dactylitis

Severe swelling and tenderness that affects fingers and toes (“sausage fingers”)

Sickle Cell Disease - Acute Chest Syndrome

• Severe lung complication of SCD

• Creates a pneumonia like illness

• One of leading causes of morbidity, hospitalizations, and death in children and adults

Picture - dark = air

Sickle Cell Disease - Nursing Care

• Prevention

  • Vaso-occlusive crises

  • Infection

  • Hydroxyurea

    • Preventive med

      • Increasing amount of fetal hemoglobin and makes RBCs increase, rounder, and more flexible

      • Done by increasing certain type of hemoglobin (F) or fetal hemoglobin because it is found in newborns

  • Penicillin

    • Preventive med

      • Decreased risk of infection (which would trigger a crisis)

• Managing Sequelae

  • Pain management

  • Treat underlying condition

  • Increase fluid requirements

  • Supplemental O2 PRN

  • No ice packs (may use heat; ice causes vasoconstriction while heat on affected joints unoccludes and vasodilates)

• Education + Support

  • S/S of vaso-occlusive events

  • Health maintenance w/ PCP and specialist

  • Med administration

  • Genetic testing

Hemophilia

• Inherited, blood borne disorder

• Disorder of “secondary hemostasis”

• Fibrin clot formation is too unstable to adequately stop bleeding

• Factor VIII (8) and factor IX (9) both play key roles by assisting in the formation of a fibrin clot

• A defect in either of these factors prevents secondary hemostasis by causing the fibrin clot to be “jelly-like” and unstable → delayed clotting → spontaneous bleeding

Clotting Cascade

• Series of steps in response to bleeding - each step activates the next and ultimately produces a clot

  • Hemostasis - bleed stopping

• Coagulation cascade involves the activation of a series of clotting factors

  • Once each protein is activated it is then able to catalyze the next reaction

Hemophilia A

• X-linked recessive disorder (think males)

• Inherited deficiency of factor VIII (“A = 8”)

• Most common hereditary disease associated with life-threatening bleeding

• Seen most often in males (late teens-20s)

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• Females may rarely be affected (affected father and carrier mother; genetic mutation (mild forms))

• Higher incidence of detection in 2nd and 3rd decades of life due to milder diagnosis

Hemophilia B

• X-linked recessive disorder (think males)

• Inherited deficiency of factor IX (“B = 9”)

• Also called Christmas disease

• Seen most often in males

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• Females may rarely be affected (affected father and carrier mother; genetic mutation (mild forms))

• Higher incidence of detection in 2nd and 3rd decades of life due to milder diagnosis

Describe how hemophilia is classified by severity (mild → severe)

• Mild: prophylactic factor before surgery or procedure (like dental)

• Severe: need factor daily → weekly

  • More likely for bleeding episodes

  • Bleed - vessels constrict and platelet plug forms (but because of deficient factor will not solidify = bleed continues)

Hemophilia - Clinical Manifestations

• Severe: more likely to have spontaneous bleeding, severe bleeding, and diagnosis at earlier age

• Mild-moderate: only have bleeding in response to injury or trauma

• Immediate or delayed bleeding after trauma is common

  • Has decreased with prophylactic use of factor replacement

• Infants: common bleeding site include extracranial, heel stick (red flag if bleeding doesn’t stop), venipuncture, and circumcision sites

• Children: bruising and joint bleeds once ambulating, forehead hematomas

• Older children/adults: bleeding/enlarged joints, muscles, GI tract, CNS, mouth, nares, bruising, forehead hematoma

Hemophilia - Nursing Care

• Manage bleeds

  • Admin factor replacement and/or desmopressin (DDVAP; stimulates stored factor in body) as ordered

  • Apply pressure to areas of external bleeding for a long time

  • If bleeding is inside a joint → ice cold (constricts, clumps) compress and elevate injured extremity, may need splint

  • AVOID NSAIDS (increases risk of bleeding), can give Tylenol for pain PRN

• Provide support

  • Lifelong illness

  • Medical alert bracelet/necklace

  • Refer to support groups - National Hemophilia Foundation

  • Help provide resources for financial strain of diagnosis

  • At home factor infusions (training, access - expensive, insurance)

• Prevent and educate

  • Provide prophylactic factor when ordered

  • Protect the toddling child with helmets/kneepads, soften corners

  • Regular exercise and activity to strengthen joints and muscles

  • Avoid activities with high likelihood of injury

  • Good oral hygiene to prevent hum/dental disease (soft toothbrush, rinses)

  • Teach caregiver how to admin factor replacement

  • Educate patient and family on when to seek expert care

NO IM INJECTIONS AND RECTAL TEMPS

Cancer in Adults

• Histologic Type

  • Epithelial tissues, carcinoma

• Mechanism

  • Environmental exposures (lifetime - smoking + drinking)

• Affects

  • Organs - lung, liver, intestines

• How its discovered

  • Early through screenings - mammograms

• Environmental/lifestyle factors

  • 80% preventable with lifestyle modifications

• Latent period

  • Long (> 20 years) - cells replicate overtime

• Metastasis

  • Less often at diagnosis

• Response to treatment

  • Less responsive

Cancer in Children

• Histologic Type

  • Embryonal, leukemia, lymphoma

• Mechanism

  • Cellular growth and development (not lifestyle)

• Affects

  • Tissues

• How its discovered

  • Incidental or accidental (presents as pain)

• Environmental/lifestyle factors

  • Little influence

• Latent period

  • Short

• Metastasis

  • Often present at diagnosis d/t it taking awhile to discover as symptoms may suggest something else (delayed diagnosis)

• Response to treatment

  • Very responsive and little resistance

Detecting Pediatric Cancer

• Can mimic growing pains, etc… (which is usually bilateral, but one extremity is concerning)

• Often difficult to detect early on (concerns with unusual presentations - certain tumors; different cancers have S/S that make it easier to detect early on vs. later)

  • Symptoms have an insidious onset and are nonspecific

  • Symptoms often resemble more common pathologies

  • ~85% of peds cancers have symptoms at presentation

• Obtain a thorough history: ask specific questions surrounding the chief complaint

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• Average time to diagnose:

  • 2-5 weeks - neuroblastoma

  • 3-5 weeks - acute leukemias (29%/year)

  • 7-10 weeks - lymphomas

  • 8-15 weeks - bone tumors

  • 8->20 weeks - brain tumors (26%/year)

Detecting Pediatric Cancer - Early Warning Signs

• Unexplained pallor and loss of energy

  • Anemic - decreased EBC, H+H, platelets

• Unusual lump, mass, or swelling

  • Large, fixed, hard lymph nodes

  • In abdomen needs further investigation

• Sudden unexplained weight loss

  • Kids usually grow

• Unexplained fever or illness that doesn’t go away or cough

  • Doesn’t get worse or better

  • Common (doesn’t have to high - >100.4F)

  • Need CBC (blood work - WBC, platelets, H+H)

• Easy bruising or bleeding

  • Nose bleeds/gum bleeding concerning

  • Common in healthy kids d/t increased activity

  • Hematomas in lower extremities or abdomen concerning

• Prolonged or ongoing pain in one or more areas of the body

  • Kids don’t typically have pain

• Limping, refusal to bear weight

  • Bones! → x-ray needed

• Frequent headaches, particularly if occurring in the morning and associated with vomiting

  • Very concerning for mass in head (laying down increases ICP)

• Sudden eye or vision changes

  • Want a red light reflex (mass causes white light)

Acute Lymphoblastic Leukemia (ALL)

• Most common childhood malignancy (kids on spectrum at most risk)

• Not familial linked; children with trisomy 21 have increased risk

• Immature lymphocytes replace normal cells in bone marrow, cannot maintain RBC, WBC, and platelets - pancytopenia (everything low)

  • Presence of “blasts” in blood smear → immature WBCs

Acute Lymphoblastic Leukemia (ALL) - Presentation

Hepatomegaly/splenomegaly, fever, lymphadenopathy, petechiae (d/t decreased platelets, MSK pain, fatigue, weight loss

Acute Lymphoblastic Leukemia (ALL) - Diagnosis

Peripheral blood smear, bone marrow biopsy, lumber puncture (CNS involvement; cancer cells in CNS)

Acute Lymphoblastic Leukemia (ALL) - Management

• Chemotherapy, allogenic stem cell transplant (HCT)

• Tx lasts 2-3 years, 5 year survival rate 90% for children < 15 years

Non-Hodgkins Lymphoma

• Includes all lymphomas except Hodgkins - common in primary immunodeficiencies

• B and T lymphocytes mutate then proliferate rapidly, effects lymph nodes or bloodstream

• Progression is quick and symptoms appear early

Non-Hodgkins Lymphoma - Presentation

Pain, lymphadenopathy, abdominal mass, mediastinal mass

Non-Hodgkins Lymphoma - Diagnosis

Lymph node and bone marrow biopsy, CXR, CT scan (extent of disease)

Non-Hodgkins Lymphoma - Management

• Chemotherapy, CNS prophylaxis (give chemo through a spinal tap intrathecally), possible BMT

• 70-90% survival rate

• Autologous bone marrow transplant - a procedure where healthy stem cells (blood-forming cells) are collected from the blood or bone marrow before treatment, stored, and then given back to patient after treatment

Hodgkins Lymphoma

• Most common in teens and young adults

  • Rare in children < 5 years; in preadolescents, more common in boys than girls

• Hallmark sign: Reed Sternberg B cells - malignant cells in lymph tissue, lymphadenopathy

• Epstein-Barr Virus (mono) infection associated with many cases

Hodgkins Lymphoma - Presentation

Weight loss, fever, night sweats, anorexia, fatigue, enlarged lymph nodes, mediastinal mass

Hodgkins Lymphoma - Diagnosis

Lymph node biopsy

Hodgkins Lymphoma - Management

• Chemotherapy, radiation, BMT/Hematopoietic Stem Cell Transplantation (HSCT) for relapse or unresponsive to treatment

• 90-85% cure rate

Medulloblastoma

”Brain tumor”

• Most common malignant brain tumor of childhood

• Primarily occur in cerebellum (cause ataxia)

Medulloblastoma - Presentation

Symptoms of increased ICP - headaches, vomiting, dizziness, blurred/double vision, ataxia, seizures, weakness, behavioral or developmental issues

Medulloblastoma - Diagnosis

Head CT, brain MRI, PET scan (a nuclear medicine imaging test that measures metabolic and cellular activity in your organs and tissues), surgical resection

Medulloblastoma - Management

• Corticosteroids initially for increased ICP

• Surgical resection of tumor, multimodal treatment; radiation after resection and chemotherapy

• 72% 5 year survival rate < 14 years old

Osteosarcoma

Bone

• Most common primary malignancy of bone (~60)

• Often occur in long bones - femur, tibia, humerus

Osteosarcoma - Presentation

Intermittent pain at site, limping, gait changes, soft tissue mass on exam that is tender to palpation

Osteosarcoma - Diagnosis

MRI of bone, bone biopsy, staging workup (x-ray initially)

Osteosarcoma - Management

• Tumor resection (removal; amputation vs. limb sparing), chemotherapy, radiation

• Reattach lower leg to upper leg (foot as knee) then prosthetic (rotationplasty)

Ewing Sarcoma

• Most common in long bones (femur) and pelvis

• Second most common bone malignancy (~30%)

Picture: allograft - reinforce bone with metal; prosthesis - metal replaces bone

Ewing Sarcoma - Presentation

Trauma may start symptoms, intermittent pain to site for weeks/months, pain worsens with activity and at night, erythema/edema over mass

Ewing Sarcoma - Diagnosis

Xray, MRI, CT scan, bone biopsy

Ewing Sarcoma - Management

• 4-6 months chemo cycles before local therapy (resection over radiation) and chemotherapy post local treatment

• 70-80% survival rate if metastasis is 20-30% only

Retinoblastoma

• Most common intraocular malignancy

  • 96% 0-14 years old

• Can metastasize to CNS through optic nerve

Retinoblastoma - Presentation

Leukocoria (white pupil reflex), strabismus (eye crossing), nystagmus (eye jitter)

Retinoblastoma - Diagnosis

Dilated opthalmoscopic exam, no biopsy required d/t risk of tumor seeding (spreading)

Retinoblastoma - Management

Severe - chemotherapy, ophthalmic artery chemosurgery, laser photoablation, enucleation (remove eyeball) if large tumor present or progression into optic nerve

Neuroblastoma

Along neural crest

• Tumor originates from neural crest during fetal development

• 90% of cases are diagnosed before age 5

• Can arise anywhere throughout the SNS; adrenal gland and abdomen most common

Neuroblastoma - Presentation

(Not neurological symptoms) Non-tender abdominal mass, abdominal pain, constipation, back pain/weakness from spinal cord suppression, fever, weight loss, anemia, bone pain, bowel/bladder dysfunction

Neuroblastoma - Diagnosis

Biopsy of tumor, bone marrow biopsy, urine catecholamines, CT, MRI, PET

Neuroblastoma - Management

• Surgical removal, chemotherapy

• 50-95% survival rate depending on risk group (low-risk to high-risk)

Wilms Tumor

• Most common renal malignancy (95% diagnosed before age 10)

• Most are solitary tumors surrounded by pseudocapsule (not an inherent part of an organ's structure; it is a protective border or layer of compressed tissue and fibers that forms around a slow-growing mass, tumor, or cyst as it pushes against surrounding healthy tissue)

• Metastasis is rare

• Once suspected… abdominal palpation should be limited; if capsule ruptured, tumor spreads rapidly + bleeding occurs

Wilms Tumor - Presentation

Firm, smooth abdominal mass, abdominal distention (can be one sided), abdominal pain, hematuria (blood in urine; tumor around kidney), fever, hypertension

Wilms Tumor - Diagnosis

Abdominal US, CT, MRI

Wilms Tumor - Management

• Stage I and II - resection and chemotherapy (may loose kidney)

• Stage III and IV - radiation also

• 90% survival rate

Diagnosing Pediatric Cancer

• Thorough history of child and family

• Current symptoms the child is having

• Head-to-toe exam

• Vital signs

• Initial screening tests:

  • CBC with diff

  • Coagulopathies (PT, PTT, INR)

  • CMP (check to see if kidney and liver are okay for medications)

  • Blood cultures

  • Lumber puncture (checks cancer cells in CSF; sedation)

  • Imaging - xray, US, CT scan, MRI

  • Bone marrow aspiration (sedated)

Treatment Modalities for Pediatric Cancer

• Surgery

  • Tumor debulking, tumor resection, CVL placement, etc…

• Chemotherapy

• Radiation

• Biotherapy

• BMT/HSCT

• Supportive therapy

Cancer - Surgery: Postop Nursing Care

• Admin pain/nausea meds and antibiotics as ordered

  • May have epidural (site checks) and/or PCA

• Assess surgical site - bleeding (increases risk), signs of infection

• Frequent physical assessments and monitoring VS closely

• Strict I&O’s

• Alert provider immediately of critical lab values

• Incentive spirometry

• Early ambulation

Radiation Therapy

• Damages or kills cells using gamma or particle radiation

• Affects all rapidly growing cells

• Can be curative, palliative, or adjuvant therapy

• Provide comfort measures and treat adverse affects as they occur

• A/E: fatigue, nausea/vomiting, mucositis (mucosa inflammed), myelosuppression (bone marrow suppression), damage to skin

• Phonton beam radiation = goes through to the other side (look for burns; skin assessment)

• Proton beam = doesn’t pass through

Chemotherapy

• Need training to admin

• Alter or destroy cancer cells based on specific points of the cell cycle

• Chemo drugs separated into classes based on actions or effect on cell cycle

• A/E are determined by the regimen that is given

  • Myelosuppression, infection, constipation, tooth damage (soft toothbrush), hearing loss, vision changes, altered growth, neuropathy

• 3 phases of treatment:

  • Induction - short and intensive, lasts a month

  • Intensification (consolidation) - intensive, lasts a few months

  • Maintenance - post-consolidation; decreases intensive, lasts 2 years

Common Side Effects of Cancer Therapies

• Infection

• Pain

• Anemia or blood loss (no rectal temps and IM injections)

• GI symptoms: anorexia, N/V, diarrhea, constipation

• Damage to oral mucosa

• Damage to skin

• Alopecia

Neutropenic Precautions

Absolute neutrophil count (< 1000 = neutropenia (risk of infection))

• Private room, closed door, regular room cleaning

• Frequent hand hygiene

• Avoid rectal temps, enemas, and invasive procedures like catheterization

• No fresh fruits/veggies, flowers, or plants in room

• Visitor restrictions

• CHG baths daily

• Mouth care

Cancer - Infection Prevention

• Regular assessments for potential infection(s)

  • Pain

  • Keep mucosa moist (oral hydration, ice chips if not NPO, petroleum jelly to lips)

    • Salt water rinses/mouthwash (no alcohol) q1-2h for hygiene

    • Soft toothbrush (prevents gum irritation/bladder)

    • Mucositis = magic mouthwash (order)

    • Avoid spicy/acidic foods, pain meds (order)

• Admin prophylactic antibiotics as ordered (increases risk of other infections)

• Promote and enforce proper hand hygiene

• Promote nutrition (decreased taste) and periods of rest

• Educate caregivers surrounding steps to prevent infection

  • Limit visitors, avoid sick contacts and crowds, encourage vaccinations (no live vaccines) once approved by provider

• Monitor for signs of infection (no rectal temps) and notify oncologist or bring child to ED if febrile

• Neutropenic precautions if indicated

Hematopoietic Stem Cell Transplant (HSCT)

• Bone marrow transplant (BMT)

  • Necessary for leukemias (body making abnormal cells → giving them so their body can make normal cells)

• Purging of abnormal/all bone marrow cells followed by transplant

• Can be autologous or allogenic

• Extensive evaluation and preparation

  • Evaluation for transplant

  • Family planning

  • CVL placement

  • HLA (human leukocyte antigen) matching

  • Stem cell harvest

  • Myeloablative therapy

• Prepare family for long hospitalization (need caregiver, no visitors)

HSCT - Pre-Transplant Care

Prevention of infection:

• Isolation in negative pressure room

• Limit visitors (caregivers)

• Admin prophylactic antibiotics and antifungals

• Frequent hand hygiene

• Regular CVL site care

• Daily CHG bathing

• Admin blood products PRN (d/t anemia)

• Provide child and family education and support

Prevention + Monitoring of Graft-Versus-Host-Disease (GVHD)

• Signs: maculopapular rash, diarrhea, abdominal pain

• Administer prophylactic antibiotics

• Monitor for maculopapular rash (skin checks)

• Administer steroids if development of GVHD occurs

• Manage GI symptoms (zofran; diarrhea → need electrolytes)

• Infection prevention

• Blood products PRN

• Admin G-CSF (type of growth factor; used after chemo to help WBCs recover after treatment) for autologous HSCT recipients

Tumor Lysis (Breakdown) Syndrome

• Therapy begins

  • Rapid lysis of tumor cells

  • Can be chemo, radiation, or glucocorticoids

• Intracellular contents released into systemic circulation

  • Potassium increases

  • Phosphate increases

  • Uric acid increases

• Metabolic consequences

  • Hyperkelamia (increased potassium)

  • Hyperphosphatemia (increased phosphate)

  • Hyperuricemia (increases uric acid)

  • Hypocalcemia (decreases calcium

  • AKI

Tumor Lysis Syndrome - Clinical Manifestations

• N/V/D

• Anorexia

• Lethargy/syncope

• Muscle cramps (increased K+ + uric acid)

• Dysrhythmias

• Hematuria

• Seizures

Tumor Lysis Syndrome - Prevention

• Admin allopurinol prophylactically

• IV fluids at 2x maintenance (d/t AKI)

• Frequent monitoring of serum electrolytes, urine output, and serum uric acid

Tumor Lysis Syndrome - Treatment

• Electrolyte replacement as needed

• Hemodialysis for severe symptoms

Pediatric Cancer Survivorship

Clinic

• Survivors are followed for many symptoms and late effect of treatments

  • Anxiety, depression, stress

• Monitored for symptoms of secondary cancers or relapse

• Transition into adult care when age appropriate

• All patients have a survivorship care plan with info about entire treatment regimen and follow-up recommendations

• Patients and families should be given resources for support groups

• Watch growth of child

Cancer - Supportive Care for EOL

• EOL care is focused on comfort, minimizing suffering

• Symptom management

  • Pharmacologic, nonpharmacologic, or both

    • Are in a lot of pain, makes them tired, determine goals

• Preparation for death (hospice/palliative)

  • Family decide on location

  • Discuss changes that occur during process

  • Assist family in obtaining memories and keepsakes: hair, handprints, photographs

  • Encourage cultural practices

• Bereavement

  • Check in with family periodically

  • Provide info for caregivers and siblings when appropriate

  • Grief counseling

• The process is on the family’s timeline (as much as possible)

• Palliative goal → live as well as possible for as long as possible

• When death occurs → give family as long as they need

Anterior Pituitary Disorders - Growth Hormone Deficiency

• S/S

  • Short stature

  • Poor growth

  • Delayed puberty and facial development

• Diagnosis

  • Rule out other reasons (genetic, FTT)

  • Wrist x-ray to assess “bone age”

  • End-organ blood testing (IGF-1)

• Treatment

  • Daily SQ GH injections (earlier is better)

  • Address underlying causes (tumor, head injury)

Anterior Pituitary Disorders - Precocious Puberty

Gonadotropin-releasing hormone (GnRH)

• Normal puberty

  • Girls 10-12

  • Boys 11-14

• Precocious puberty

  • Before 7-8 in girls

  • Before 9 in boys

  • In young patients - concern for pituitary issue

• Secondary sex characteristics - pubic and axillary hair, breast and testicular development from premature release of sex hormones

• May also see voice changes, rapid growth spurt, acne, body odor

• Always needs medical evaluation

• Diagnosis

  • Hormone levels

  • Imaging

• Treatment

  • Medical therapy

    • GnRH agonist to work on feedback loop to stop GnRH release

  • Address underlying cause

  • Emotional support

Posterior Pituitary Disorders

• ADH - antidiuretic hormone released by posterior pituitary

• Causes kidneys to retain water

• Disorders can cause fluid/electrolyte imbalances

ICP affects posterior pituitary (post brain injury) → concern for DI (diabetes insipidus → “dry inside”) and SIADH (syndrome of inappropriate ADH hormone → “soaked inside”)

Thyroid Disorders

• Metabolism control center (how food is converted to energy)

• Affects EVERYTHING

  • Weight gain or loss

  • HR

  • Temp

  • Speed of digestion

Congenital Hypothyroidism

• Tested on Newborn Screen

• Can cause irreversible intellectual disability and FTT if not treated early

• Requires lifelong daily levothyroxine given in the morning

Coarse facies = facial abnormalities

Diabetes

Normal blood glucose 70-110 mg/dL

Diabetes - High Blood Sugar

• Caused by carbohydrate intake, infection, stress, steroid intake, growth spurts, etc…

• Fatigue, 3 Ps (increased urination, thirst, hunger), can progress to DKA

• Treated with insulin or other meds

• Causes damage over time

Diabetes - Low Blood Sugar

• Caused by fasting, insulin overdose, exercise, etc…

• S/S: shaky, sweaty, headache, irritability, can progress to changes in LOC and even seizure

• Treated with rapid acting carbs (orange juice, glucose tabs) or IM glucagon if unconscious

• Can cause rapid decompensation - treat FIRST

Type I Diabetes

• Autoimmune destruction of pancreas = insulin deficiency

• Peak diagnoses at age 4-7 and 10-14

• Urinalysis, BG, A1C useful in diagnostics; many present in DKA

  • Fasting glucose (8 hours) > 125 on 2 separate occasions

  • Random glucose > 200

  • A1C > 6.5

• Major S/S: 3 Ps, weight loss, bedwetting (in previous potty trained kid)

Type I Diabetes - Management

• Frequent blood glucose checks (before meals, at bedtime, if symptomatic)

• Lifelong insulin dependence (nasal-bolus)

  • SQ injections

  • SQ pump

• Carb counting

  • Patient/family education is important!!

• Interdisciplinary team

Type I Diabetes - Treatment

• Tight BG management with minimal lows

• Goal A1C < 7.5

• Allow kids in run higher BG than adults

  • Hypoglycemia recognition can be more difficult

  • Immediate risk of hypoglycemia

Insulin

• Often take long acting once daily then rapid acting with meals based on carb intake and sliding scale

• Will be ordered uniquely to each child by provider

Type II Diabetes

• Characteristics

  • Insulin resistance (pancreas is producing insulin but cells are resistant)

  • Not autoimmune

  • Less likely linked to DKA

  • More common adults although peds diagnoses increasing

• S/S

  • 3 Ps

  • Obesity

  • HTN and hyperlipidemia

• Management

  • BG control

  • Lifestyle adjustments!!

    • Exercise and healthy diet

    • BG control

    • Cook at home

  • May add metformin, GLP-1, or insulin depending on A1C

Pediatric Diabetes Considerations

TEST

• Infants - educate the family, watch for lethargy or poor eating

• Toddlers - picky eaters, temper tantrums, routine

• Preschoolers - may begin to participate, awareness of DM

• School-age - socialization, more participation

• Adolescent - body image, independence, peer acceptance, consistency

Diabetic Ketoacidosis (DKA)

• Blood glucose > 250 (usually more like 400-800)

• Acidosis (pH < 7.3, usually closer to 7; bicarb < 15)

• Ketones in blood and urine

  • D/t keto diet, prolonged N/V, eating d/o

• Medical emergency!!!

DKA Management

• ICU admission

• IV fluids

• IV insulin

• Don’t want to drop BG too quickly (electrolyte shifts)

• Assess LOC, blood sugar, K+

Hypoglycemia

• BG < 70

• Priority treatment

• Can cause LOC, seizure, brain damage, and death if not treated

Hypoglycemia - Management

• Mild - 15 g fast acting carbs and recheck in 15 mins

• Severe - glucagon injection

• Educate patient to always have these on hand