immune, hematologic, and neoplastic disorders

immune and allergic disorders:

conditions affecting body's defense mechanisms and hypersensitivity responses

- HIV

- allergy

-allergy anaphylaxis

The nurse is caring for a pt w/ HIV and is providing edu about antiretroviral meds. What should be included in the teaching plan?

the meds can be used for pre-exposure prophylaxis for HIV

3 multiple choice options

HIV vs. AID's

preventing HIV transmission from mother to baby

pediatric HIV/ AIDS

virus attacks CD4 and T lymphocytes = immunodeficiency

pediatric HIV/ AIDS: clinical symptoms

- retrovirus targeting CD4 & t cells, leading to immune suppression

- fail. to thrive, chronic diarrhea, opportunistic infections

pediatric HIV/ AIDS: nursing assessment

- hx of maternal HIV/ risk factors, growth and development (watch for delays/ stagnation)

- frequent/ severe infections

- nutritional status

pediatric HIV/ AIDS: testing

- PCR testing (infants)

- Elisa/ western blot (older children)

- CD4 count and viral load mo nonitoring

pediatric HIV/ AIDS: nursing interventions

- assess growth, dev. milestones, infection signs

- promote adherence to ART

- prevent infection: hand hygiene, immunizations, safe feeding

- support coping and confidentiality

meds:

- antiretroviral therapy (ART): combo of NRTI's, NNRTI's, and protease inhibitors

antiretroviral tx

- when teaching clients about ART, emphasize:

- adherence is critical to prevent viral resistance

- ART DOES NOT CURE HIV, but allows people to live longer healthier lives

- PrEP and PEP use similar meds for prevention in HIV-negative individuals

- PrEP

- prevents HIV infection before exposure

- taken daily on a long term basis for ongoing risk

- PEP

- prevents HIV infection after a possible exposure

- MUST BE started w/ in 72 hrs of exposure

PrEP

- prevents HIV infection before exposure

- taken daily on a long term basis for ongoing risk

PEP

- prevents HIV infection after a possible exposure

- MUST BE started w/ in 72 hrs of exposure

allergy and anaphylaxis: clinical symptoms

- rash

- sneezing

- watery eyes

- wheezing

- asthma exacerbations

- urticaria

-hypotn

- angioedema

allergy and anaphylaxis: nursing assessment

- exposure hx

- skin and respiratory symptoms

allergy and anaphylaxis: testing

- skin testing

- serum IgE level

- elimination diets

allergy and anaphylaxis: nursing interventions

- identify and avoid triggers

- rapid assessment of ABC's (presence of urticaria, angioedema, resp. distress, shock)

- emergency management of airway

- teach proper use of epi pens

- edu parents on allergen labels, emergency plans

meds:

- antihistamines, corticosteroids, leukotriene modifiers, epi auto injectors

allergy anaphylaxis: pathophysiology

severe, systemic hypersensitivity → massive histamine release → vasodilation, airway edema → bronchospasm

allergy anaphylaxis: clinical manifestations

- sudden hypotension

- urticaria

- wheezing

- laryngeal edema

allergy anaphylaxis: dx

clinical dx (no time for labs)

allergy anaphylaxis: meds

- IM epi (first-line)

- antihistamines, corticosteroids, bronchodilators

allergy anaphylaxis: nursing interventions

- immediate admin of epi

- airway management, O2, IV fluids

epinephrine auto injector: generic name

epinephrine (epi pens)

epinephrine: primary eff/ MOA

- stimulates a and b adrenergic receptors

- vasoconstriction, bronchodilation, ↑HR, ↓mucosal edema

hematologic disorders

conditions r/t the blood and blood forming tissues (excluding primary malignancies)

ex.

- iron deficiency anemia

- sickle cell anemia

- hemophilia

- lead poisoning

iron deficiency anemia (IDA)

decreased iron intake, absorption, or loss → reduce hemoglobin synthesis

iron deficiency anemia: clinical symptoms

- pallor

- fatigue

- tachycardia

- koilonychia "spoon nails"

- poor feeding

- pica

- developmental delay

iron deficiency anemia: nursing assessment

- dietary hx

- assess energy level

- growth and dev.

- signs of pallor or fatigue

- vital signs (tachycardia, SOB, breathlessness)

iron deficiency anemia: testing

- CBC: red cell indices- low hgb & hct, low mean corpuscular vol (mcv)(microcytic anemia)

- low serum ferritin (primary indicator of iron stores)

- screening @ 12m, repeat between ages 1-5yr for pt at risk

iron deficiency anemia: nursing interventions

medications

oral ferrous sulfate or other iron supplements

- give iron with vitamin C; avoid milk around dosing

- give through straws, brush after (stains teeth)

- edu parents on iron rich foods

- monitor for constipation or GI upset

s/s of iron deficiency anemia in children

- fatigue

- cold hands or feet

- slowed growth or development

- poor appetite

- usual craving for non nutritive substances (ex. dirt, starch, ice, etc.)

- abnorm. rapid breathing

- behavioral problems

sickle cell anemia

autosomal recessive; HbS (hemoglobin S) → sickling → vaso- occlusion, ischemia, hemolysis

sickle cell anemia: clinical symptoms

- pain crisis

- anemia

- acute chest syndrome (ACS)

- splenic sequestration dactylitis (rapid blood trapping in spleen → causing severe anemia and hand and feet are swollen due to vaso occlusion

- splenomegaly

- stroke risk

- delayed growth

sickle cell anemia: nursing assessment

- pain episodes

- family hx

- growth delays

- physical exam

sickle cell anemia: testing

- hemoglobin electrophoresis

- sickledex

- CBC (low hgb/ hct), high reticulocyte count

sickle cell anemia: nursing interventions

- pain controls

- hydration

- respiratory status (watch for ACS)

- spleen precautions

- educate on avoiding triggers (cold, dehydration, stress)

- infection prevention

- psychosocial support

- blood transfusion management

H: hydrate

O: oxygen

P: pain meds

S: support

meds

- hydroxyurea

- pain management

- flic acid

hemophilia

X linked recessive deficiency of factor VIII (A) or IX (B)

hemophilia: clinical symptoms

- spontaneous bleeding/ prolonged bleeding

- hemarthrosis (bleeding into joints)

- bruising/ hematomas

hemophilia: nursing assessment

- hx of bleeding

- hemarthrosis

- neurological assessment (for intracranial hemorrhage)

hemophilia: testing

- coagulation studies

- factor assays

hemophilia: nursing interventions

- bleeding prevention

- RICE for hemarthrosis

- avoid aspirin/ NSAID's

- safe environment/ activity promotion

- educate on factor administration

meds

- replacement therapy

- DDAVP (desmopressin)

- aminocaproic acid (amicar)

- cryoprecipitate

8 steps of blood transfusion

1. verification

2. inspection of unit

3. pt identification

4. baseline vitals

5. administration

6. monitoring

7. duration

8. post transfusion

blood transfusion: verification

two nurses verify → correct pt, blood type, Rh factor, expiration date, unit #, consent

prevents fatal transfusion errors

blood transfusion: inspection of unit

check for discoloration, clots, bubbles, or leaks

ensures product integrity

blood transfusion: pt identification

confirm name, DOB, MRN w/ wristband and unit label

correct pt verification per protocol

blood transfusion: baseline vitals

record immediately before transfusions

detects changes indicating a reaction

blood transfusion: administration

start infusion SLOWLY (≤2mL/min) for first 15 min, remain w/ pt

early detection for a reaction

blood transfusion: monitoring

reassess vitals every 15 minutes, then every 30, then at completion

tracks for fluid retention or fluid overload

blood transfusion: duration

complete transfusion w/ in 4 hrs MAX

prevents bacterial growth

blood transfusion: post transfusion

flush line with Ns; document total volume and patient response

ensure complete delivery and accurate record keeping

lead poisoning (plumbism)

toxic accumulations of lead in the body, particularly in bones, teeth, and soft tissues affecting multiple organ systems

lead poisoning (plumbism): clinical symptoms

- neuro status, seizures, encephalopathy, coma

- vomiting, weight loss

- anemia

- bone pain, delayed growth, skeletal deformities

- developmental delay, poor attention,, learning deficits

lead poisoning (plumbism): nursing assessment

- health hx

- physical assessment

- environmental assessment

lead poisoning (plumbism): testing

- blood lead level (BLL): primary screening test\

- serum iron ferritin

- abdominal x ray

- neuro developmental screening

lead poisoning (plumbism): nursing interventions

- assessment and monitoring

- regular BLL screening for at-risk children

- monitor growth, neuro status, and learning milestones

- environmental control

- nutrition support

- encourage food high in iron, calcium, vit C

- medication administration

- chelating agents

- family edu

- teaching parents about lead sources and prevention

early signs of lead poisoning: neuro

- irritability

- lethargy

- headache

- learning difficulties

- behavior changes

early signs of lead poisoning: GI

- anorexia

- nausea

- vomiting

- abdominal pain

- constipation

early signs of lead poisoning: hematologic

mild anemia (inhibited heme synthesis)

early signs of lead poisoning: renal

- proteinuria

- impaired renal function

early signs of lead poisoning: musculoskeletal

- bone pain

- delayed growth

early signs of lead poisoning: developmental

- speech delay

- poor attention

- learning deficits

late/severe signs of lead poisoning: neuro

- seizures

- encephalopathy

- coma

late/severe signs of lead poisoning: GI

- weight loss

- colic

late/severe signs of lead poisoning: hematologic

severe anemia

late/severe signs of lead poisoning: renal

chronic nephropathy

late/severe signs of lead poisoning: musculoskeletal

skeletal deformities

late/severe signs of lead poisoning: developmental

- cognitive impairment, decreased IQ

chelation therapy

MOA

- binds to lead to enhance urinary excretion

indications

- if BLL is ≥45

nursing consideration

- monitor renal and hepatic function; ensure hydration

succimer (DMSA)

MOA

- oral chelating agent

indications

- mild-moderate poisoning (BLL 45- 69)

nursing considerations

- oral; monitor for neutropenia, GI upset

chelation therapy: supportive therapies

indications

- iron, calcium, and vitamin C supplements

nursing considerations

- reduce lead absorption and enhance secretion

neoplastic/ oncology disorders: pathophysiology

uncontrolled cell growth in various tissues

neoplastic/ oncology disorders: assessment

- fatigue

- weight loss

- unexplained bruising

neoplastic/ oncology disorders: clinical man.

varies by cancer type

neoplastic/ oncology disorders: meds

- chemotherapy

- targeted therapy

neoplastic/ oncology disorders: nursing interventions

- supportive care

- monitor for side effects

- family edu

childhood cancers

often arise from embryonal or blood forming tissues; tend to be rapidly proliferating

childhood cancers: symptoms

varies by cancer type

- fatigue

- weight loss

- unexplained bruising

- unexplained lumps/ swelling

- persistent pain

- unexplained fever

- pallor (anemia)

childhood cancers: testing

- CBC w/ differential

- lumbar puncture (checks CNS involvement)

- biopsy-definitive diagnosis

- CT, MRI, PET scans

childhood cancers: nursing interventions

- supportive care

- pain management

- monitor for side effects

- family edu

meds

- chemo (multi-phase), corticosteroids, antibiotics, antiemetics

- radiation

- surgery

acute lymphoblastic leukemia

malignant proliferation of lymphoblasts

acute lymphoblastic leukemia: clinical symptoms

- fever, pallor, bleeding

- bone pain

- anemia, thrombocytopenia, hepatosplenomegaly

acute lymphoblastic leukemia: nursing assessment

- pale skin

- petechiae

- ecchymosis

- lethargy

- signs of malnutrition

- s/s of infection

- enlarged lymph nodes

- hepatosplenomegaly

- bone tenderness/ joint swelling or pain

- headaches

- CNS involvement

acute lymphoblastic leukemia: testing

- CBC, bone marrow biopsy

acute lymphoblastic leukemia: nursing interventions

- meds

- chemo (multi-phase), corticosteroids, antibiotics, antiemetics

- monitor for infection, bleeding, mucostitis

- manage chemo side effects

- support nutritional and emotional needs

neuroblastoma

patho/ clinical manifestations

- tumor of SNS; often adrenal origin

- S/S: firm, irregular abdominal mass, crosses midline

dx

- urinary catecholamines

- imaging

- biopsy

nursing focus

- support surgery/ chemo

- monitor for mets

- poor prognosis → family support is crucial

Wilms tumor (nephroblastoma)

patho/ clinical manifestations

- renal tumor; peak 2-5 yrs

- S/S: painless abdominal mass, hematuria

dx

- ultrasound, CT, biopsy (AFTER SURGERY)

nursing focus

- DO NOT PALPATE ABDOMEN

- prep for nephrectomy

- monitor renal function

- protect remaining kidney post op

ewings sarcoma

patho/ clinical manifestations

- bone tumor (pelvis, long bones)

- S/S: pain, swelling, fever

dx

- bone biopsy, MRI

nursing focus

- pain management

- limb protection

- support during chemo/ radiation

- physical therapy!!

rhabdomyosarcoma

patho/ clinical manifestations

- soft tissue sarcoma (head, neck, GI tract)

dx

- biopsy, imaging

nursing focus

- post op care

- airway support (if head and neck)

- fam teaching

child receiving chemo

pathophysiology

- cytotoxic effects on rapidly dividing cells

clinical manifestations

- nausea, fatigue, neutropenia

nursing assessment

- side effects

- nutritional status

- infection signs

dx

- CBC, liver/ kidney, function tests

meds

- antiemetics, growth factors

nursing interventions

- infection prevention

- manage side effects

- emotional support

radiation therapy in children

pathophysiology

- ionizing radiation changes DNA of cancer cells

clinical manifestations

- skin changes

- fatigue

- site specific effects

nursing assessment

- skin integrity, fatigue, localized symptoms

dx

- imaging for planning

meds

- symptom management

nursing interventions

- skin care

- fatigue management

- emotional support

pediatric cancer strategies: preoperative

- assess baseline VS, labs (CBC, coagulation, electrolytes), nutrition

- screen for infection or neutropenia (delay surgery if febrile)

- age-appropriate edu and emotional support

- verify informed consent and child assent

- maintain NPO as ordered

pediatric cancer strategies: intraoperative

- verify correct sit/ procedure ("timeout")

- maintain strict asepsis

- monitor VS, oxygenation, and fluid balance

- assist anesthesia and maintain airway

pediatric cancer strategies: postoperative

- monitor VS, pain, incisions, drains, and output

- maintain IV fluids, nutrition, and sterile wound care

- watch for infection, bleeding, poor healing

- provide pt management and family support

- educate on home care and follow up