Hematologic Disorders - PEDS

Define Epistaxis

Short, isolated occurrences of nosebleeds, common in childhood.

Epistaxis - Risk Factors (7)

1. Trauma - Picking/rubbing nose cause fragile & vascular mucous membranes in nose to tear & bleed.

2. Low humidity

3. Allergic rhinitis

4. Upper respiratory infection

5. Blunt injury

6. Foreign body in nose

7. Medications or underlying bleeding disorders

Epistaxis - Nursing Care

• Have child sit up with head tilted slightly forward to prevent aspiration of blood. 

• Apply pressure to lower nose with thumb & forefinger for at least 10 min.

• Do not pack cotton/tissue into nostril or ask child to blow their nose because this could displace the clot.

• Apply ice across bridge of the nose if bleeding continues.

Epistaxis - Client Education

Bleeding usually stops within 10 mins

If it continues take to ER

Define Iron Deficiency Anemia (3)

• Most prevalent anemia worldwide, commonly from an inadequate dietary supply of iron

• Adolescents at risk - poor diet, rapid growth, menses, activities, obesity

• Results in decreased hgb levels

Risk Factors - Iron Deficiency Anemia (3)

• Premature birth resulting in decreased iron stores

• Excessive intake of cows’ milk in toddlers

• Milk is not a good source of iron & it takes the place of iron-rich solid foods.

Expected Findings - Iron Deficiency Anemia

• Tachycardia

• Pallor

• Brittle, spoon-shaped fingernails

• Fatigue, irritability, & muscle weakness

• Systolic heart murmur

• Cravings for non-nutritive substances (ice, dirt, paper)

Nursing Care - Iron Deficiency Anemia (4)

• Recommend iron-fortified formula for infants when solids are introduced.

• Modify infant’s diet to include high iron, & vitamin C.

• Limit formula intake to 32 oz (960 mL) per day.

• Encourage intake of iron-rich foods.

Iron Supplementation - Iron Deficiency Anemia (5)

• Give 1 hr before or 2 hr after milk, tea, or antacid to prevent decreased absorption.

• GI upset (diarrhea, constipation, nausea) is common at start of therapy & decreases over time.

• Give with vitamin C to increase absorption & best given on an empty stomach if tolerated

• Expect stools to turn tarry green if dose is adequate

• Brush teeth after to minimize staining

Iron-Rich Foods - Infants (2)

• Iron-fortified cereals

• Formula or exclusive breastfeeding

Iron-Rich Foods - Children (5)

• Dried beans & lentils

• Peanut butter

• Green, leafy vegetables

• Iron-fortified breads & flour

• Poultry & red meat

Define Sickle Cell Disease (SCD)

• Group of diseases in which abnormal sickle hemoglobin S (HbS) replaces normal adult hemoglobin (Hgb A)

• Cells look like crescent moons that stack & clump together

Define Sickle Cell Anemia (4)

• Homozygous & most common form of SCD

• Result of RBC sickling, which leads to increased blood viscosity, obstruction of blood flow, & tissue hypoxia

• Tissue hypoxia causes tissue ischemia, which results in pain.

• Increased RBC destruction

Expected Findings - Sickle Cell Anemia (7)

1. Reports of pain

2. SOB & fatigue

3. Pallor & pale mucous mebranes

4. Jaundice

5. Cold hands & feet

6. Dizziness

7. Headache

Define Sequestration - Sickle Cell Anemia (2)

• Excessive pooling of blood primarily in the spleen (splenomegaly), & sometimes in the liver (hepatomegaly)

• Reduced circulating blood volume results in hypovolemia & can progress to hypovolemic shock (thready pulse, decreased output, hypotension, & tachycardia)

Nursing Care - Sickle Cell Anemia (8)

1. Promote rest to decrease oxygen consumption.

2. Administer oxygen as prescribed if hypoxia is present.

3. Provide intense hydration therapy while maintaining fluid & electrolyte balance.

4. Monitor I&O & give oral & IV fluids

5. Administer blood products - packed RBCs

6. Observe for manifestations of hypervolemia & transfusion reaction.

7. Treat & prevent infection - hand hygiene, antibiotics

8. Administer pneumococcal conjugate vaccine, meningococcal vaccine, & Haemophilus influenzae type B vaccine.

Pain Management - Sickle Cell Anemia (4)

• Treat mild to moderate pain with acetaminophen or ibuprofen & manage severe pain with opioid analgesics.

• Apply comfort measures (warm packs to painful joints).

• Schedule administration of analgesics to prevent pain.

• Opioids - Codeine, morphine sulfate, oxycodone, hydromorphone, & methadone

HOP A - Sickle Cell Anemia (4)

H - Hydrate, dilutes to moved clumped cells, large volumes of fluid

O - Oxygen to address hypoxia

P - Pain (address pain)

-

A - Antibiotics if needed

Complications - Sickle Cell Anemia

Acute Chest Syndrome

Life-threatening

Common in adolescents

Increases risk for pnuemonia due to decreased oxygen to lung tissue

SCA Acute Chest Syndrome - S/S (7)

• Chest, back, or abdominal pain

• Fever of 101.3° F or higher

• Cough

• Tachypnea

• Dyspnea, wheezing

• Retractions

• Decreased spO2

SCA Acute Chest Syndrome - Education (2)

• May need blood transfusion

• Take antibiotics as prescribed

Define Hemophilia (2)

• Group of bleeding disorders characterized by difficulty controlling bleeding.

• Deficiencies in the clotting factors.

Types of Hemophilia (3)

• Hemophilia A - Most common 80%, X-linked recessive, low VII

• Hemophilia B - X linked recessive (men cannot pass down to sons, common in woman), low IX

• Von Willebrand - Inherited factor causing inability of platelets to aggregate

Hemophilia - Findings (4)

• Active bleeding - Bleeding gums, epistaxis, hematuria, &/or tarry stools

• Hematomas &/or bruising, even with minor injuries

• Joint pain & stiffness, loss of ROM, deformities (hemarthrosis)

• Headache, slurred speech, and a decreased level of consciousness

Hemophilia - Nursing Care (4)

• Administer injections via the SubQ route instead of the IM route when possible.

• Monitor urine, stool, and nasogastric fluid for occult blood.

• Do not administer aspirin or any products that contain aspirin.

• Acetaminophen is an acceptable substitute for aspirin.

• Elevate and apply ice to the affected joints. (RICE for joint symptoms)

Hemophilia - Nursing Considerations

No circumcisions in infants until hemophilia is ruled out

Hemophilia - Meds

1-deamino-8-d-arginine vasopressin (DDAVP) - synthetic form of vasopressin that increases plasma factor VIII

For mild hemophilia A ONLY

Hemophilia - Client Education (5)

• ​​​​​​Environment should be made as safe as possible to prevent injury.

• Engage in low-contact sports (bowling, swimming, fishing, golf)

• Soft toothbrushes

• Regular exercise & PT

• Control bleeding episodes using the RICE method.

Hemophilia - Complications

Joint deformity

Most often elbows, knees, ankles

Hemophiila - Joint Deformity Actions

Notify HCP

Repeated episodes of hemarthrosis (bleeding into joint spaces) lead to impaired ROM, pain, tenderness, & swelling, which can develop into joint deformities