Living With Sickle Cell Disease:

An educational resource about transfusion therapy

Approximately 300,000 babies are born with sickle cell disease each year, according to the World Health Organization (WHO).1

In the U.S., sickle cell disease affects approximately 100,000 individuals.2

Only 20% of family physicians report feeling comfortable treating people with sickle cell disease.2

The number of people with sickle cell disease is expected to grow 30% globally by 2050.2

Sickle Cell Disease Basics

Transfusion Therapy for Sickle Cell Disease


Transfusion therapy is generally defined as receiving blood via an intravenous (IV) line and is a widely utilized treatment option for specific long-term and acute complications of sickle cell disease. However, there are multiple types of transfusion therapy to manage sickle cell disease. Learn about the different types of transfusion therapy and how you may better manage your disease.3

Transfusion therapy is not the only option for treating sickle cell disease and may not be appropriate for all sickle cell disease patients. Ask the right questions when you consult your physician about whether transfusion therapy may be an option for you.
Get a list of questions to ask your physician
Download the questions

Sickle Cell Trait


People who inherit one sickle cell gene and one non-sickle gene have sickle cell trait. People with sickle cell trait usually do not have any of the symptoms of sickle cell disease, but they can pass the trait on to their children.4

Sickle Cell Disease


Sickle cell disease is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has sickle cell disease, the red blood cells become inflexible and sticky and look like a C-shaped farm tool called a "sickle." The sickled cells die early, which causes a constant shortage of red blood cells. Sickled cells can also get stuck when traveling through blood vessels and clog the blood flow. This can cause pain and other serious complications such as infection, acute chest syndrome and stroke.

Why Transfusion Therapy?


Transfusion therapy is generally a safe and effective therapy for managing sickle cell disease. By giving you healthy red blood cells or exchanging sickled red blood cells with healthy ones, transfusion can allow oxygen to flow more easily through your body to help improve symptoms and reduce complications of sickle cell disease.5

Transfusion Therapy for Sickle Cell Disease


Transfusion therapy is generally defined as receiving blood via an intravenous (IV) line and is a widely utilized treatment option for specific long-term and acute complications of sickle cell disease. However, there are multiple types of transfusion therapy to manage sickle cell disease. Learn about the different types of transfusion therapy and how you may better manage your disease.3

Transfusion therapy is not the only option for treating sickle cell disease and may not be appropriate for all sickle cell disease patients. Ask the right questions when you consult your physician about wether transfusion therapy may be an option for you.
Get a list of questions to ask your physician
Download the questions
video thumbnail

Sickle Cell Trait


People who inherit one sickle cell gene and one non-sickle gene have sickle cell trait. People with sickle cell trait usually do not have any of the symptoms of sickle cell disease, but they can pass the trait on to their children.4

Sickle Cell Disease


Sickle cell disease is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has sickle cell disease, the red blood cells become inflexible and sticky and look like a C-shaped farm tool called a "sickle." The sickled cells die early, which causes a constant shortage of red blood cells. Sickled cells can also get stuck when traveling through blood vessels and clog the blood flow. This can cause pain and other serious complications such as infection, acute chest syndrome and stroke.

Why Transfusion Therapy?


Transfusion therapy is generally a safe and effective therapy for managing sickle cell disease. By giving you healthy red blood cells or exchanging sickled red blood cells with healthy ones, transfusion can allow oxygen to flow more easily through your body to help improve symptoms and reduce complications of sickle cell disease.5
Avoid Transfusion Confusion

Transfusion-Releated Safety Information  

Side effects of automated RBCX may include:
Anxiety, headache, light-headedness, digital and/or facial paresthesia (tingling sensation in the arms, hands, feet, legs, or face), fever, chills, hematoma, hyperventilation, nausea and vomiting, syncope (fainting), urticaria (hives), hypotension, allergic reactions, infection, hemolysis, thrombosis in patient and device, hypocalcemia, hypokalemia, thrombocytopenia, hypoalbuminemia, anemia, coagulopathy, fatigue, hypomagnesemia, hypogammaglobulinemia, adverse tissue reaction, device failure/disposable set failure, air embolism, blood loss/anemia, electrical shock, fluid imbalance and inadequate separation of blood components.18

Contraindications
Automated RBCX may not be an option for all patients with sickle cell disease. Talk to your physician about your transfusion options. Contraindications for the use of apheresis systems are limited to those associated with the infusion of solutions and replacement fluids as required by the apheresis procedure and those associated with all types of automated apheresis systems.

Reactions to blood products transfused during procedures can include:
Hemolytic transfusion reaction, immune-mediated platelet destruction, fever, allergic reactions, anaphylaxis, transfusion-related acute lung injury (TRALI), alloimmunization, posttransfusion purpura (PTP), transfusion-associated graft-versus-host disease (TA-GVHD), circulatory overload, hypothermia, metabolic complications and transmission of infectious diseases and bacteria.19,20
Restricted to prescription use only.
  • Operators must be familiar with the system’s operating instructions.
  • Procedures must be performed by qualified medical personnel.

1World Health Organization (WHO). Fifty-ninth world health assembly. Sickle-cell anaemia: report by the Secretariat. https://who.int. Published April 24, 2006. Accessed June 1, 2020.
2American Society of Hematology. Understanding the Impact of Sickle Cell Disease. https://www.hematology.org/education/patients/anemia/sickle-cell-disease. Accessed November 5, 2020.
3Danielson CF. The role of red blood cell exchange transfusion in the treatment and prevention of complications of sickle cell disease. Ther Apher. 2002;6(1):24-31.
4Centers for Disease Control and Prevention. Sickle Cell Disease. Data & Statistics on Sickle Cell Disease. https://www.cdc.gov/ncbddd/sicklecell/data.html#:~:text=SCD%20affects%20approximately%20100%2C000%20Americans,sickle%20cell%20trait%20(SCT). Accessed June 1, 2020.
5Tsitsikas DA, Sirigireddy B, Nzouakou R, et al. Safety, tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease. J Clin Apher. 2016;31(6):545-550.
6Dedeken L, Le PQ, Rozen L, et al. Automated red blood cell exchange compared to manual exchange transfusion for children with sickle cell disease is cost-effective and reduces iron overload. Transfusion. 2018;58(6):1356-1362.
7Kuo K, Ward R, Kaya B, Howard J, Telfer P. A comparison of chronic manual and automated red blood cell exchange transfusion in sickle cell disease patients. Br J Haematol. 2015;170(3):425-428.
8National Institute for Health and Care Excellence (NICE). Spectra Optia for automatic red blood cell exchange in patients with sickle cell disease. https://www.nice.org.uk/guidance/mtg28/chapter/5-Cost-considerations. Accessed November 2020.
9Singer T, Quirolo K, Nishi K, Hackney-Stephens E, Evans C, Vichinsky E. Erythrocytapheresis for chronically transfused children with sickle cell disease: an effective method for maintaining a low HbS level and reducing iron overload. J Clin Apher. 1999;14(3):122-125.
10Duclos C, Merlin E, Paillard C, et al. Long-term red blood cell exchange in children with sickle cell disease: manual or automatic? Transfus Apher Sci. 2013;48(2):219-222.
11Howard J. The role of blood transfusion in sickle cell disease. ISBT Science Series. 2013;8:225-228.
12Barth D, Sanchez A, Thomsen A, et al. Peripheral vascular access for therapeutic plasma exchange: a practical approach to increased utilization and selecting the most appropriate vascular access. J Clin Apher. 2020;35(3):178-187.
13Adams DM, Schultz WH, Ware RE, Kinney TR. Erythrocytapheresis can reduce iron overload and prevent the need for chelation therapy in chronically transfused pediatric patients. J Pediatr Hematol Oncol. 1996;18(1):46-50.
14Fasano RM, Leong T, Kaushal M, Sagiv E, Luban NL, Meier ER. Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients. Transfusion. 2016;56(7):1707-1715.
15Al-Salem AH. Medical and Surgical Complications of Sickle Cell Anemia. Springer; 2016.
16Cobianchi C, Fafoutis D, Roig J, Dierick K, Comasolivas N. Measuring health-related quality of life in individuals with sickle cell disease undergoing automated red blood cell exchange. Poster presented at: Sickle Cell Disease Association of America Annual Meeting: October 2018; Baltimore, MD.
17Barth D, Nemec RM, Cho DD, et al. The practical integration of a hybrid model of ultrasound-guided peripheral venous access in a large apheresis center. J Clin Apher. 2020; 35(4):328-334.
18Crookston KP. Therapeutic Apheresis: a Physician’s Handbook. 5th ed. Bethesda, MD: AABB/ASFA; 2017.
19AABB. Circular of Information for the Use of Human Blood and Blood Components. Bethesda, MD: AABB; 2017.
20European Directorate for the Quality of Medicines & HealthCare (EDQM). Guide to the Preparation, Use and Quality Assurance of Blood Components. 20th ed. Strasbourg, France: EDQM Council of Europe; 2020.