saturation by pulse oximetry will be closely monitored according to the 
schedule in Table 2. 
Infusions will be performed every 6 weeks for a total of approximately 3 
administrations. The second and third infusions may be performed on 
an outpatient basis at the discretion of the PI. For outpatient infusions, 
patients will be treated in the 8th floor clinic of the Clinical Center or in 
the 1 1th floor day hospital. The infusion will be administered over 1 
hour, and the patient will be observed for 4 hours thereafter. Patients 
will return to the Clinical Center for outpatient evaluation daily for the 
first week after infusions, then weekly or less frequently (to be guided 
by the data produced) for the first 24 weeks, and then monthly to 
complete 6 months of follow-up beyond the last lymphocyte infusion. 
Comprehensive safety and immunologic evaluations will be performed 
according to the schedule in Table 3. 
We plan to administer the transduced T cells within approximately 2 
weeks of apheresis. This is designed to minimize the potential for 
clonal overgrowth in culture and therefore the development of a 
disproportionate mix of immune specificities that might be significantly 
different from those present in the fresh blood sample. Experiments in 
immunodeficient mice transplanted with human cells (including gene 
treated ADA(-)SCID cells) have shown that 10-20 million cells contain 
a broad repertoire of immune specificities (44,45). Experience in the 
ADA deficient SCID children demonstrates that this procedure is 
effective and is not associated with clonal disproportion. 
5.4.5 Treatment of Infusion Related Reactions 
If a patient develops fevers, chills, or muscle aches, he/she will receive 
the appropriate dose of either aspirin, ibuprofen, or acetaminophen. 
Acetaminophen will be used if the patient does not tolerate aspirin or 
ibuprofen. The development of a rash will be treated initially with 
diphenhydramine PO. Patients who experience reactions to the 
infusion such as fever, muscle aches, and chills will be premedicated 
30 minutes prior to future infusions with either aspirin, acetaminophen, 
or diphenhydramine. Meperidine will be used for more severe chills 
and muscle aches that do not quickly respond to antipyretics and 
antihistamines. Cell infusions will be slowed or discontinued 
depending upon the severity of the reaction. In the event of a severe 
reaction, emergency life support measures will be immediately 
undertaken. 
Recombinant DNA Research, Volume 16 
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