Evaluation of the AMICUS RBCx System in Sickle Cell Patients

The goal of Red Blood Cell Exchange is to remove a patient's red blood cells (RBCs) and
replace the blood volume removed with either healthy donor RBCs and/or colloid/crystalloid
solutions. Depending on the RF used, the procedure can be considered an RBC Exchange or RBC
Depletion/Exchange procedure. The RBC Depletion/Exchange procedure is a modification of the
RBC Exchange procedure. The AMICUS RBCx protocol provides the ability to use various RFs
including healthy donor RBCs or a combination of fluids, such as saline followed by RBCs. In
RBC Exchange and the exchange portion of the RBC Depletion/Exchange procedures, RBC units are
used as the RF, while colloid and/or crystalloid solutions are used as the RF in the
depletion portion of the RBC Depletion/Exchange procedures. A new operating protocol on the
AMICUS Separator that enables the device to perform Red Blood Cell Exchange (RBCx) procedures
in accordance with the replacement fluid(s) (RF) prescribed by a physician for patients with
sickle cell disease

Inclusion Criteria:

- Subjects with documented diagnosis of a type of sickle cell disorder who require RBC
Exchange or RBC Depletion/Exchange treatment.

- Medically stable subjects who have been previously treated for sickle cell disease
with RBC Exchange or RBC Depletion/Exchange.

- Subjects, or subject's legal representative, who have provided signed informed
consent, and assent when applicable, prior to participation.

- Adequate availability of sickle trait negative, leukoreduced, Blood type (ABO) blood
group, Rhesus factor D (Rh (D)) compatible, unexpired replacement RBC products.

- Subjects with sufficient vascular access to accommodate the RBCx procedure as
determined by the medical staff responsible for obtaining intravenous access.

- Subjects who are able and agree to report adverse events (AEs) during the required
reporting period.

Exclusion Criteria:

- Procedures that occur during acute hospitalization.

- Procedures prescribed within one week of discharge of a hospitalization.

- Subjects with altered mental status that would prohibit the giving and understanding
of informed consent, and assent when applicable, who do not have a legally authorized
representative.

- Drug abuse, alcohol abuse, or other factors that in the opinion of the investigator
could affect the ability of the subject to comply with the requirements of the
protocol.

- Subjects who have experienced a serious adverse event associated with an RBCx
procedure in the past.

- In the opinion of the investigator, subjects who have a life expectancy fewer than 30
days.

- Subjects who refuse blood products.

- Subjects who are pregnant.

- Subjects who fail to comply with site requirements for cessation of medication that
interfere or increase procedure risk.