Sickle cell disease (SCD) is a health condition that affects the red blood cells. In some cases, people with SCD may require blood transfusions to help treat and manage the condition and prevent complications.
SCD affects about 100,000 people in the United States and is more common among African American and Hispanic American people.
According to the Centers for Disease Control and Prevention (CDC), 1 in every 13 African American babies is born with sickle cell trait, which means that they have one sickle cell gene that they could pass on to their children.
Blood transfusion definition
A blood transfusion is a medical procedure that provides healthy blood and blood products to people who need them. It involves transferring blood through the veins. It can take place in a hospital, clinic, or private home.
People can receive different mixtures of blood and its components. These are whole blood, red blood cells (RBC), white blood cells, platelets, and plasma.
Before a blood transfusion, healthcare providers identify an individual’s blood type and take care to select donated blood that is a suitable match. For people with SCD, the emphasis is usually on transfusing RBCs, and the main types of blood transfusion are:
- Simple transfusion: The process adds donated blood into a person’s system without removing any of their blood.
- Partial exchange transfusion: In this procedure, a doctor removes a small amount of blood and replaces it with donated healthy blood. They do this to avoid removing too much blood at a time.
- Chronic transfusion therapy: Doctors frequently use this approach for people who are at high risk for stroke. It consists of simple transfusions that take place every few weeks.
- Erythrocytapheresis: This automated process exchanges RBCs, removing unhealthy, misshapen cells and replacing them with healthy ones. This procedure is safe for children, even toddlers, as well as adults.
SCD is a collection of blood disorders that affect RBCs. Healthy RBCs are round and move through blood vessels smoothly to transport oxygen around the body. In someone with SCD, a problem with the protein hemoglobin means that the RBCs are hard, sticky, and sickle-shaped.
Due to this, the RBCs may become stuck in the body’s smaller blood vessels, causing a lack of oxygen throughout the body. The possible effects include pain, stroke, acute chest syndrome, and infections.
When people have SCD, their RBCs also tend to die quickly, living only for 10–20 days, instead of 90–120. If the body cannot make new RBCs to replace them quickly enough, a person may develop anemia.
Prescription medications, stem cell therapy, and blood transfusions are the standard treatments for SCD. The disease tends to get worse over time, so the treatments that a person needs throughout their life are also likely to change.
Blood transfusions play a significant role in helping people manage SCD. These procedures allow healthcare teams to respond rapidly when people develop sudden severe symptoms and complications due to SCD, such as:
- anemia, especially when problems with the spleen make it worse
- some infections
- acute chest syndrome
- splenic sequestration, which is when the spleen swells suddenly and dangerously due to trapped sickle cells
- multiple organ failure
Transfusions can also prevent SCD-related problems, such as:
- complications before surgery and other medical interventions
- reducing the risk of stroke in people who have already had one
- reducing the risk of stroke in children who are at risk
Donating blood is a relatively painless and straightforward procedure. The American Red Cross note that while the process usually takes about 1 hour from checking in to getting refreshments afterward, the actual collection of whole blood only takes about 10 minutes. Donations involving apheresis will take longer.
Although it is also not too painful, aside from a pinprick for the intravenous (IV) insertion, it takes somewhat longer for an individual to receive blood, depending on the type of transfusion:
- Simple transfusion: A doctor adds donated blood to an individual’s system, and the length of time that it takes varies depending on how much blood a person needs.
- Partial exchange: This process goes in phases, removing small amounts of blood, refreshing it, and then returning it before removing another batch. This method means that a person undergoing the procedure is never missing more than a few ounces of blood at a time.
- Erythrocytapheresis: A doctor takes blood from a person with SCD and puts it into an apheresis machine, which removes RBCs and discards them. A doctor then mixes the individual’s plasma, platelets, and white blood cells with RBCs from a donor and adds an anticoagulant to prevent clotting. They then put the mixture back into the individual’s body.
Monitoring is very important after blood transfusions. In people with SCD, it can be particularly difficult to predict how and when they might start responding poorly to donated blood. As adverse reactions can be fatal, extra caution is necessary.
People with SCD may need to rely on blood transfusions throughout their lives. Although they are generally safe, blood transfusions can sometimes cause a few complications in people with SCD over time.
One of the risks of frequent blood transfusions is hemosiderosis, or iron overload disorder. Hemosiderosis can develop after frequent blood transfusions and may damage the heart, liver, pancreas, and other organs, unless the individual receives iron chelation therapy.
A type of blood transfusion called erythrocytapheresis, or automated red blood cell exchange, can also help reduce the risk of iron building up in the body.
Alloimmunization, in which an individual produces an immune response to donated blood, is another condition that can develop after multiple transfusions. According to a 2014 study, alloimmunization occurs in about 30% of people with SCD who undergo blood transfusions.
The area where a doctor inserts the needle may feel sore, numb, or tender. Bruising is possible, and some people may develop infections.
Delayed hemolytic transfusion reactions (DHTRs) occur when people start making antibodies in response to blood that they receive via a transfusion. DHTRs are likely to cause more than 4.2% of deaths due to SCD.
Other risks associated with blood transfusions include:
- low blood pressure
Sickle cell disease is a serious medical condition that can cause significant, life threatening damage to an individual’s health and quality of life.
Blood transfusions can be an effective form of treatment, although the procedure has significant associated risks, particularly with continued use.