Top Five Questions to Ask Your Physician



Be prepared, be engaged and take control. Take this list of questions to your next doctor’s appointment to help lead the conversation about your treatment options.



1.

Is transfusion therapy an option for me? And if so, which type of therapy (manual, simple, automated) will best manage my sickle cell disease?


2.

If transfusion therapy is not an option, what treatment alternatives are available that could help me better manage my sickle cell disease?


3.

What are the safety concerns with the different transfusion therapy options?


4.

What can I do now to prevent future sickle cell disease-related health problems?


5.

What other resources are available to help me learn more about sickle cell disease and my treatment options?

1.

Is transfusion therapy an option for me? And if so, which one will best manage my sickle cell disease (manual, simple, automated)?


2.

If transfusion therapy is not an option, what treatment alternatives are available that could help me better manage my sickle cell disease?


3.

What are the safety concerns of the different transfusion therapy options?


4.

What can I do now to prevent future sickle cell disease related health problems?


5.

What other resources are available to help me learn more about sickle cell disease and my treatment options?


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Resources

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.