Pediatric Hematology/Immunology

What is the most common hematologic disorder in infancy?

Anemia

What are the consequences of anemia?

*decreased RBCs results decreased hgb which decreases oxygen available to the tissues
*Growth retardation is more commonly the result of chronic anemia (FTT rt anemia)

What causes anemia?

One of three things:
*Decreased RBCs
*Increased destruction of RBCs
*Blood loss

How will I know if a patient has anemia?

*Fatigue...Easily fatigued
Pallor
Some kiddos are asymptomatic. Look at the CBC.

What drug can be used to decrease pain in children receiving blood work?

Elamax or EMLA
Several tests are done in series instead of all at once. Several tests are ordered.

Teaching for anemia tests:

Encourage parents to stay with the pt.
Allow the child to play with a doll

Nursing Management for Anemia

Bed Rest
Diversional Activities: Kids will have a short attention span and irritability with anemia.
Plan activities to prevent boredom and withdrawal.

Anemic kids are at risk for what?

Infection

IRON DEFICIENCY ANEMIA

*caused by lack of iron in diet
*most prevalent nutritional disorder in the US
*most common mineral disturbance

Iron Deficiency Anemia

*cause a decrease in iron supply
*impair its absorption
*increase the body's need for iron
*affect the synthesis of Hgb

Signs and symptoms of IDA

Spoon fingers
Fatigue, HA, dyspnea, palpitations, pallor, dizziness
dyspnea on exertion, sensitivity to cold, decreased concentration.

Drug treatment for Iron Deficiency

Iron fortified foods
Oral iron supplements for 3 months
Vitamin C rich juice aids in absorption of iron. take with juice.

If hgb does not rise after one month of treatment for IDA, what will the nurse assess?

Is there persistent bleeding?
Iron Malabsorption?
Was the pt non-compliant with the medication?
Improper iron administration?

Why are IM/IV administrations of iron not used very often?

Expensive
Painful
Lots of allergic reactions

If a pt with IDA needs a blood transfusion, what will the nurse expect to see given?

PRBC 2-3ML/KG

What is a common tx for a pt with IDA in the hospital setting?

Oxygen therapy

What should the parents of a child with IDA be told?

Infants should never be fed cows milk. Causes anemia rt GI bleeding in immature GI system. Poor source of iron.

How should the parent give the iron supplement?

Two divided doses between meals when stomach acid is the greatest.
Should be given with Vit C juices: Never with cows milk which decreases absorption
Liquid preparations can cause temp. teeth stains-take thru a straw--brush teeth afterwards
Turns stool ta

Parents Education on IDA

Diet: Breast milk is poor in iron after 5 months.
introduce fortified cereals, semi-solids at 4 months.

Aplastic Anemia: Pancytopenia

Inability of the bone marrow to produce enough or ANY new cells. Low count in ALL three blood cell types.

Who does aplastic anemia affect primarily?

Affects all ages and genders.
Idiopathic and acquired.
Primary is congenital. Secondary is acquired.

If a pt has secondary aplastic anemia, what will the nurse know caused the anemia?

Bone Marrow Disease
Autoimmune Disorder
Drugs
Chemicals

What is the pathophysiology for aplastic anemia?

Bone Marrow stops producing cells.

Aplastic anemia is unique. It has s/s in each blood cell type. What are the s/s in the decreased RBCs?

Fatigue
Weakness
Headache
Dyspnea
Tachycardia
Pallor

Aplastic Anemia: decreased WBCs s/s

Frequent infections

Aplastic Anemia: Platlet s/s

Heavy menses
Prolonged bleeding
Skin rash
Bone pain
Nose bleeds
Bleeding gums
Easy and unexplained bruising

How is a definite dx of aplastic anemia acquired?

Bone Marrow Biopsy

What are the tx options for aplastic anemia pts?

Immunosuppresive therapy
Replace the bone marrow via transplant (suitable if donor exists)
ALG: anti.lympho.cyte globulin
ATG: anti.thymocyte globulin
Hematopoietic stem cell transplanation should be considered early in the course of the disease if a comp

The nursing care for AA is very comparable to that of leukemia pts. Why?

CVL education.
Preparation of child, family for procedures, complications, and emotional support.

If a mom asks "What will happen to my son after he gets treatment?", how will I respond?

He could have some nausea and vomiting. His hair will be lost, but will grow back about a month after treatments end. He will need extra gentle mouth care due to stomatitis.
(There is specialized tx for pts with stem cell transplants.)

Sickle Cell Anemia: Vaso-occlusive Phenomena

Sequestrian Crisis
Pooling of large amounts of blood...Hepatomegaly, Spleenomegaly, Circulatory collapse

S/S of SSA

Chronic and life-long disease
Lives are punctuated by periods of vaso-occlusive crises
Infarcts
Fever, malaise, and leukocytes
Average lifespan of 40 years

Sickle Cell Anemia complications

Acute Chest Syndrome:
Severe chest, back or abdominal pain
Fever of 38.5C or higher
Very congested cough
Dyspnea, Tachypnea
Retractions
Declining oxygen saturation

SSA AND Cerebral Vascular Accident (CVA, stroke)

Severe, unrelenting headaches
severe vomiting
jerking or twitching of the face, legs or arms
Seizures
Strange, abnormal behavior
Inability to move an arm or leg
Stagger or slurred speech
Weakness in the hands, feet, or legs
Changes in vision

Sickle Cell Anemia Dx Evaluation

Newborn Screenings are mandatory in most states.
If dx not at birth, s/s present in toddler or preschool years: normally GI or respiratory infection

How is a child diagnosed with sickle cell?

Finger stick: Sickle-turbidity test (Sickledex) Blood placed on card. (Saw in clinical)
Or Electrophoresis is done if the Sickledex comes back +.

Medical Management of the SSA Crises

Electrolyte replacement: hypoxia results in metabolic acidosis which promotes sickling.
Pain meds
Blood replacement to treat anemia and hydration to reduce the viscosity of the sickled blood
Abx to treat any existing infection

Special Considerations for Sickle Cell Pts

Kiddos with SCA need pneumococcal and meningococcal vaccines RT their susceptibility to infection.
Routine Immunizations rt aslpenia: Need yearly flu shot
Oral penicillin prophylaxis is also recommended by 2 months of age.

Prognosis for Sickle Cell Kids

Greatest risk is in kids under 5. Death rt overwhelming infection.
Hematopoietic stem cell transplantation offers the hope of a cure for some children.

Explaining Sickle Cell to parents:

Disease education
Early intervention for fevers!
Give penicillin as ordered.
recognize s/s of splenic sequestration
recognize respiratory problems that can lead to hypoxia
treat the child normally
emphasize the major importance of hydration!
Never ICE on