15.1 Anemia, Sickle Cell Disease, Coagulopathies & Blood Product Administration
Key Takeaways
In sickle cell disease, a temperature of 38.5°C or higher is treated as an emergency with prompt blood culture and parenteral antibiotics such as ceftriaxone, because functional asplenia raises the risk of overwhelming infection.
Acute chest syndrome is a new pulmonary infiltrate with fever or respiratory symptoms in a child with sickle cell disease; treatment includes oxygen, antibiotics (a cephalosporin plus a macrolide), analgesia, incentive spirometry, and transfusion (exchange for severe cases).
Splenic sequestration causes a rapid hemoglobin drop with an enlarging spleen and hypovolemic shock; transfusion is given in small aliquots because trapped cells return to the circulation as the spleen releases them.
For life-threatening bleeding in hemophilia, factor replacement comes first, before imaging or procedures: about 50 IU/kg of factor VIII in hemophilia A, and a product-specific dose of factor IX (often about 100 IU/kg) in hemophilia B.
Packed red cells at 10–15 mL/kg typically raise hemoglobin by about 2–3 g/dL; blood is infused only with 0.9% saline, and suspected transfusion reactions are managed by stopping the transfusion and supporting the patient.
Anemia, Sickle Cell Disease, Coagulopathies & Blood Products
Anemia in the Transport Patient
Oxygen delivery equals cardiac output × arterial oxygen content, and oxygen content depends mostly on hemoglobin (Section 5.4). Children compensate for anemia with tachycardia and increased stroke volume, until they cannot.
| Type | Examples | Transport concerns |
|---|---|---|
| Acute blood loss | Trauma (Section 13.3), GI bleeding, subgaleal hemorrhage (Section 9.4) | Hypovolemic shock; hemoglobin falls late; replace blood, not just crystalloid |
| Hemolysis | Sickle cell crisis, G6PD deficiency with oxidant exposure, autoimmune hemolytic anemia, hemolytic uremic syndrome | Hyperkalemia, hyperbilirubinemia, kidney injury; autoimmune hemolysis makes crossmatching difficult |
| Decreased production | Iron deficiency, aplastic crisis (parvovirus B19), leukemia, bone marrow failure | Often chronic and well compensated, but high-output heart failure can occur |
Severe chronic anemia (for example, hemoglobin 3–4 g/dL from iron deficiency in a toddler with heavy cow's milk intake) causes compensated high-output circulation. A rapid large-volume transfusion can cause heart failure. Transfuse slowly in small aliquots (commonly about 5 mL/kg at a time), sometimes with a diuretic, while watching for pulmonary edema.
Altitude matters: Anemic patients have little oxygen reserve. Provide supplemental oxygen, and consider cabin altitude restrictions for severe anemia (Section 3.2).
Sickle Cell Disease (SCD) Emergencies
Deoxygenated hemoglobin S polymerizes. Red cells sickle, occlude microvessels, and hemolyze. Children with SCD develop functional asplenia early in life.
| Emergency | Key features | Transport management |
|---|---|---|
| Fever | Temperature ≥38.5°C (NHLBI threshold); high risk of pneumococcal and other encapsulated-organism sepsis | Blood culture and prompt parenteral antibiotics (for example, ceftriaxone) without waiting for results; treat shock |
| Acute chest syndrome (ACS) | New infiltrate on radiograph plus fever and/or respiratory symptoms (chest pain, cough, tachypnea, hypoxemia); can progress rapidly | Oxygen to keep saturation normal, antibiotics (cephalosporin plus macrolide), analgesia (splinting from pain worsens atelectasis), incentive spirometry, cautious fluids (avoid overload), simple or exchange transfusion for severe or progressive disease |
| Vaso-occlusive pain crisis | Severe pain in limbs, back, chest, or abdomen | Rapid analgesia with parenteral opioids within about an hour of arrival (NHLBI), hydration without overload, monitor for ACS |
| Splenic sequestration | Sudden pallor, weakness, enlarging spleen, hemoglobin drop of about 2 g/dL or more below baseline, hypovolemic shock; usually young children | Volume resuscitation and careful transfusion in small aliquots; as the spleen releases trapped cells, hemoglobin can rise more than expected (overshoot increases viscosity) |
| Aplastic crisis | Parvovirus B19 halts red cell production; severe anemia with very low reticulocyte count | Transfusion; droplet precautions (parvovirus is contagious to pregnant staff and other SCD patients) |
| Stroke | Focal deficits, seizures, altered consciousness | Exchange transfusion (target HbS below about 30%) at a specialty center; avoid delays; neuroimaging |
| Priapism | Prolonged painful erection | Analgesia, hydration, urology consultation (time sensitive) |
Transport-specific points:
- Hypoxemia promotes sickling. Give supplemental oxygen when saturation is low, and consider altitude effects during air transport.
- Avoid hypothermia and dehydration, both of which promote sickling. Avoid overhydration, which worsens ACS.
- Avoid raising hemoglobin above about 10 g/dL with simple transfusion, because high viscosity can trigger vaso-occlusion and stroke.
- Transfused SCD patients need phenotypically matched blood when possible to prevent alloimmunization. Let the blood bank know the diagnosis.
Coagulopathies
Hemophilia
- Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) are X-linked. They cause joint, muscle, and potentially life-threatening intracranial, airway (neck, tongue), and iliopsoas bleeding.
- Factor first: For suspected intracranial hemorrhage or other life-threatening bleeding, give factor replacement before imaging or procedures:
- Hemophilia A: about 50 IU/kg of factor VIII, which raises activity to roughly 100%
- Hemophilia B: a product-specific dose of factor IX (plasma-derived products are often dosed at about 100 IU/kg for 100%)
- Follow the patient's hematology plan and home product whenever available.
- Avoid intramuscular injections, arterial punctures where possible, and NSAIDs.
- Patients with inhibitors or on emicizumab prophylaxis need their hematology team's specific plan. Emicizumab also makes the aPTT unreliable.
Von Willebrand disease
The most common inherited bleeding disorder. Mucosal bleeding. Desmopressin (DDAVP) helps responsive types. Watch for hyponatremia after DDAVP, especially in young children. Factor concentrates containing von Willebrand factor are used for major bleeding.
Immune thrombocytopenia (ITP)
Sudden petechiae and bruising in a well-appearing child, often after a viral illness, with isolated thrombocytopenia. Life-threatening bleeding is rare. When it occurs (intracranial hemorrhage, severe GI bleeding), treat with IVIG and corticosteroids plus platelet transfusion. Platelets alone are consumed quickly. Avoid IM injections and contact activities.
Disseminated intravascular coagulation (DIC)
Consumption of clotting factors and platelets with microvascular thrombosis. It occurs in sepsis (especially meningococcemia with purpura fulminans), trauma, heat stroke, and malignancy. Treat the cause. Replace products for bleeding or before procedures: platelets, fresh frozen plasma, and cryoprecipitate for low fibrinogen.
Vitamin K deficiency bleeding (VKDB)
Newborns have low vitamin K stores. VKDB can cause GI, skin, and intracranial bleeding in the first weeks to months, especially in exclusively breastfed infants who did not receive vitamin K at birth (0.5–1 mg IM prophylaxis). Treat with IV or subcutaneous vitamin K and fresh frozen plasma (or prothrombin complex concentrate per protocol) for active bleeding. Always ask whether vitamin K was given.
Blood Product Administration in Transport
| Product | Typical pediatric dose | Expected effect / notes |
|---|---|---|
| Packed red blood cells | 10–15 mL/kg | Raises hemoglobin about 2–3 g/dL; slower, smaller aliquots in chronic severe anemia |
| Platelets | 10–15 mL/kg (or 1 unit per 10 kg of whole blood-derived platelets) | Raises count roughly 50,000–100,000/mcL; less effective in ITP |
| Fresh frozen plasma | 10–15 mL/kg | Replaces clotting factors |
| Cryoprecipitate | About 1 unit per 5–10 kg | Fibrinogen, factor VIII, von Willebrand factor, factor XIII |
Safety essentials:
- Two-person identity check at the bedside, and verification of the product, expiration, and compatibility.
- Only 0.9% saline runs with blood. Dextrose solutions cause hemolysis, and calcium-containing solutions (such as lactated Ringer's) can cause clotting with the citrate anticoagulant.
- Use a blood filter, and a warmer for rapid or large-volume transfusion (Section 6.4).
- Irradiated products prevent transfusion-associated graft-versus-host disease in at-risk patients (preterm neonates, intrauterine or exchange transfusions, immunodeficiency, 22q11 deletion). Leukoreduced (CMV-safe) products are standard for neonates.
- Massive transfusion causes hypocalcemia (citrate), hyperkalemia (older units), hypothermia, and dilutional coagulopathy. Check ionized calcium and potassium.
Transfusion reactions: stop the transfusion first.
| Reaction | Clues | Action |
|---|---|---|
| Acute hemolytic (ABO incompatibility) | Fever, chills, hypotension, back or flank pain, hemoglobinuria, DIC; in sedated patients only hypotension and bleeding | Stop, keep IV open with saline, support circulation, maintain urine output, notify the blood bank, return the unit |
| Febrile non-hemolytic | Fever or chills without instability | Stop, exclude hemolysis and bacterial contamination, antipyretic |
| Allergic or anaphylactic | Urticaria, or bronchospasm and hypotension | Antihistamine for mild; IM epinephrine for anaphylaxis |
| TRALI (transfusion-related acute lung injury) | Hypoxemia and bilateral infiltrates within 6 hours, often with fever and hypotension | Respiratory support; diuretics are not helpful |
| TACO (circulatory overload) | Hypertension, pulmonary edema, and volume overload | Stop or slow the transfusion, give diuretics, sit the patient up |
A 7-year-old with sickle cell disease has fever to 39.1°C, cough, chest pain, tachypnea, and SpO2 90%. The chest radiograph shows a new right lower lobe infiltrate. Which diagnosis and management are most appropriate?
Uncomplicated viral bronchitis; give oral fluids and discharge
Aplastic crisis; start droplet precautions only
Splenic sequestration; give a 40 mL/kg fluid bolus
Acute chest syndrome; give oxygen, a cephalosporin plus a macrolide, analgesia, incentive spirometry, and transfusion as needed
A 12-year-old with severe hemophilia A falls from a bicycle and hits his head. He is alert with a mild headache. What should the transport team do first?
Obtain a head CT, and give factor only if bleeding is seen
Give factor VIII concentrate (about 50 IU/kg) promptly, before imaging
Give ibuprofen for the headache and observe
Give intramuscular vitamin K
Twenty minutes into a packed red cell transfusion, a sedated, intubated child becomes hypotensive with new oozing from IV sites and pink-tinged urine. What is the first action?
Increase the transfusion rate to correct the hypotension
Give acetaminophen and continue the transfusion
Stop the transfusion, keep the line open with 0.9% saline, support circulation, and notify the blood bank
Switch the carrier fluid to lactated Ringer's
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