Pediatric Hematologic Overview
Two themes run through every blood disorder: oxygen and bleeding.
The blood does two jobs the nurse watches most closely: red blood cells (RBCs) carry oxygen, and platelets and clotting factors stop bleeding. Several high-yield pediatric blood disorders are inherited — sickle cell disease, hemophilia, and thalassemia — while others, such as iron-deficiency anemia and ITP, are usually acquired. Many still show up early in life, so the family is part of the care plan. Sort each disorder into one of the two themes and the priorities fall into place.
If the problem is with red cells (sickle cell, iron-deficiency anemia, thalassemia), the danger is poor oxygen delivery — watch for pallor, fatigue, and tissue hypoxia. If the problem is with platelets or clotting factors (hemophilia, ITP), the danger is bleeding — and bleeding precautions become the priority.
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Sickle Cell Disease
Abnormal hemoglobin turns round red cells into rigid sickles.
Sickle cell disease is an autosomal recessive disorder (a child inherits the gene from both parents). It is most common in people with ancestry from sub-Saharan Africa, the Mediterranean, the Middle East, India, and parts of Central and South America and the Caribbean; in the U.S. it most often affects Black/African American patients. The child makes abnormal hemoglobin S. When oxygen drops, RBCs change from soft discs into stiff, crescent "sickle" shapes that clog small vessels and break down early — causing pain, tissue damage, and anemia. Symptoms usually begin after about 6 months of age, as protective fetal hemoglobin fades.
Triggers of Sickling
- Hypoxia (low oxygen) and high altitude
- Dehydration and infection / fever
- Cold exposure, strenuous exercise, and physical or emotional stress
Types of Crisis
- Vaso-occlusive (painful) crisis — the most common; sickled cells block blood flow, causing severe pain in the extremities, abdomen, or joints. In infants and toddlers it often appears as dactylitis (hand-foot syndrome) — painful swelling of the hands and feet.
- Splenic sequestration — blood pools in the spleen, which enlarges rapidly while blood pressure drops; this can cause shock and is life-threatening.
- Aplastic crisis — RBC production temporarily stops (often after a viral infection), leading to severe anemia.
Management — think "HOP" plus avoid triggers: Hydration (IV + oral fluids to dilute the blood and stop sickling), Oxygen for hypoxia, and Pain control with scheduled opioids. Add rest, warmth, infection prevention (prophylactic penicillin and pneumococcal/meningococcal vaccines, since the spleen works poorly), hydroxyurea to reduce crises, folic acid, and transfusions when needed.
Apply warm compresses to painful areas; cold causes vasoconstriction and more sickling. Treat pain aggressively with morphine (often PCA), but avoid meperidine (Demerol) — its byproduct can cause seizures. A fever is an emergency in these children because their spleen can't fight infection well.
Iron-Deficiency Anemia
The most common anemia in children — often from too much cow's milk.
Iron-deficiency anemia is the most common blood disorder of childhood. The classic toddler picture is a child who drinks large amounts of cow's milk (more than about 24 ounces a day): milk is low in iron, fills the child up so they eat fewer iron-rich foods, and can cause tiny amounts of GI bleeding. Adolescents are also at risk from rapid growth and menstruation. The RBCs become small and pale (microcytic, hypochromic).
Signs
- Pallor, fatigue, irritability, and poor feeding
- Tachycardia and, when severe, decreased activity tolerance
- Pica (craving non-food items such as ice, dirt, or paper)
Treatment: oral iron (ferrous sulfate), more iron-rich foods (meat, fortified cereal, leafy greens, beans), and limiting cow's milk in toddlers to under 24 oz/day.
Give iron between meals with vitamin C (like orange juice) to boost absorption, and not with milk (it blocks absorption). Liquid iron stains teeth — use a straw or dropper toward the back of the mouth and rinse after. Dark, tarry stools are expected. Keep iron locked away — an overdose is one of the most toxic poisonings in young children.
Hemophilia
A missing clotting factor means bleeding that won't stop.
Hemophilia is an X-linked recessive disorder, so it almost always affects boys, while mothers are usually carriers. A clotting factor is missing or low — factor VIII in hemophilia A (the most common form) and factor IX in hemophilia B. The blood doesn't bleed faster; it bleeds longer, because it can't form a stable clot.
Signs
- Hemarthrosis — bleeding into joints (knees, ankles, elbows) causing pain, swelling, warmth, and stiffness; the hallmark of hemophilia
- Easy bruising, prolonged bleeding after minor cuts, circumcision, or losing a tooth
- Nosebleeds and prolonged bleeding after dental work or surgery
Treatment: factor replacement (IV factor VIII or IX concentrate), given on demand for bleeds or as scheduled prophylaxis; desmopressin (DDAVP) may be used for mild hemophilia A. For a joint bleed, use RICE — Rest, Ice, Compression, Elevation.
Use acetaminophen, never aspirin or NSAIDs, which impair platelets and worsen bleeding. Avoid IM injections when possible (and rectal temperatures); give medications by mouth or subcutaneously. If an IM injection or vaccine is necessary, follow provider/hematology guidance and apply firm pressure afterward. Teach a soft toothbrush, electric razor, no contact sports (swimming is fine), protective gear, and a MedicAlert bracelet.
Beta-Thalassemia
Inherited anemia treated with transfusions — and the iron problem they create.
Beta-thalassemia (the severe form is called Cooley's anemia) is an inherited disorder, most common in people of Mediterranean, Middle Eastern, and Asian descent, in which the body can't make enough normal beta-globin for hemoglobin. The result is chronic, severe anemia with pallor, fatigue, failure to thrive, an enlarged liver and spleen, and bone changes (such as frontal bossing) as the marrow tries to keep up.
Treatment: regular blood transfusions to keep hemoglobin near normal.
Frequent transfusions slowly build up too much iron in the body (hemosiderosis), which damages the heart, liver, and endocrine glands. So these children also need iron chelation therapy (a medication such as deferoxamine that binds excess iron so it can be excreted). Transfusions treat the anemia; chelation protects the organs. Expect the urine to turn reddish or dark during therapy — that is the iron-deferoxamine complex being excreted, mostly in the urine. It is an expected, harmless finding, not a sign the drug is failing or causing harm. Tell families ahead of time so it doesn't alarm them.
Immune Thrombocytopenia (ITP)
The immune system destroys the body's own platelets.
Immune thrombocytopenia — formerly called idiopathic thrombocytopenic purpura (ITP) — is an autoimmune disorder in which the body destroys its own platelets, dropping the platelet count. In children it commonly appears after a viral illness and is usually acute and self-limiting, resolving on its own.
Signs
- Petechiae (tiny pinpoint red-purple spots) and purpura (larger bruised areas)
- Easy bruising, nosebleeds, and gum bleeding
- Otherwise the child usually looks well
Treatment: often just observation; IVIG, corticosteroids, or anti-D may be used if the platelet count is very low or active bleeding occurs.
While the count is low, prevent injury: no contact sports, no aspirin or NSAIDs, soft toothbrush, and a padded, fall-safe environment. Teach the family to recognize bleeding and to seek care for head injury — the most feared complication is bleeding in the brain.
Nursing Care & Bleeding Precautions
The shared priorities across pediatric blood disorders.
- Bleeding precautions (hemophilia, ITP) — soft toothbrush, electric razor, no contact sports, no aspirin/NSAIDs, avoid IM injections and rectal temperatures, pad the environment.
- Prevent infection (sickle cell, thalassemia, post-splenectomy) — keep immunizations current, give prophylactic penicillin when ordered, and treat fever as an emergency.
- Support oxygen delivery (the anemias) — balance rest and activity, conserve energy, and monitor for increasing pallor or fatigue.
- Hydration and pain control — the core of sickle cell crisis care.
- Family teaching & genetic counseling — most of these disorders are inherited; teach trigger avoidance, medication routines, and when to seek care.
Match the hallmark to the disorder: sickling pain + dactylitis = sickle cell (treat with HOP — Hydration, Oxygen, Pain); pallor + heavy cow's-milk intake = iron-deficiency anemia; hemarthrosis in a boy = hemophilia (no aspirin, no IM); transfusions + iron overload = thalassemia (needs chelation); petechiae after a virus = ITP.
? A boy with hemophilia has a swollen, painful knee after a fall. What are the nursing priorities?
Frequently Asked Questions
Common student questions on this topic.
What triggers a sickle cell crisis?
What is the priority treatment during a vaso-occlusive sickle cell crisis?
Why are aspirin and IM injections avoided in hemophilia?
What is the hallmark sign of hemophilia?
How should oral iron be given to a child with iron-deficiency anemia?
What is ITP (immune thrombocytopenia)?
Quick-Reference Cheatsheet
Every key point from this guide on a single printable page.
Pediatric Hematologic Disorders Cheatsheet
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