Transcript Subcutaneous immunoglobulin replacement therapy in

Subcutaneous immunoglobulin
replacement therapy for primary
immunodeficiency in children and
young adults.
Radana Zachová
Department of Immunology
University hospital Motol, Charles University
Prague, Czech Republic
ESID Prague Spring Meeting 2009
Immunoglobulin replacement therapy in primary
immunodeficiencies
Life –long therapy for patients with PID
Possible administration ways – intravenous, subcutaneous
Historically - intramuscular
Disorders:
CVID
X- linked agammaglobulinemia
Specific antibody deficiencies
ESID Prague Spring Meeting 2009
Subcutaneous immunoglobulin replacement therapy
Patient
Diagnosis
Age
(years)
Subcutaneous
therapy
started
Age at
beginnig of
therapy
Results
ZP
CVID
29
2007
27
Home therapy
MK
CVID
16
2007
14
Home therapy
JJ
CVID
15
2007
13
Home therapy
SM
CVID
16
2009
16
Home therapy
MK
XLA
2
2009
2
Home therapy
FV
CVID
52
2008
51
therapy in hospital
SV
XLP
19
2007
17
refused s.c.therapy
Small infusion pump for scig
administration
NOT refunded from insurance !
WE lend this pump from company
Subcutaneous immunoglobulin replacement therapy in 1,5 year
old boy.
Case report:
MK *2007
History: boy, 1. pregnancy, unrelative parents, born in term,
3100g,
anorectal atresia, surgical treatment after delivery ,
3 month - pyelonephritis
11 month - bilateral bronchopneumonia
Immunoglobulin levels IgG, IgA, IgM – very low
CD 19-1,1% CD 20-0,5%
BTK mutation –pR28C missence mutation
Family history: grandmother´s brother ( mother´s side)
died at 18 years ( cerebral absces, recurrent
bronchopneumonias)
Subcutaneous immunoglobulin replacement therapy in 1,5 year old
boy.
MK *2007
Therapy:
Immunolgobulin substitution therapy was indicated
Weight : 10 kg
after first application of IVIG mother refused intravenous
application of immunoglobulins
Intramuscular administration was started 1x in 14 days - painfull,
uncomfortable - mother refused I.M. application too
↓
We started subcutaneous application of immunoglobulins
Subcutaneous immunoglobulin replacement therapy in 1,5 year old
boy.
Thinks taken into consideration
parents reffused IV and IM immunoglobulin substitution completelly
subcutaneous therapy ?
Disadvantages for SCIG
low BMI – small subcutaneous tissue - big local swelling???
scar on abdominal wall after surgical treatment - only one side of
abdominal wall can be used for replacement therapy
2 adult persons have to apply the therapy ( 1 has to fix the child)
Actually:
subcutaneous replacement therapy in home administration
Subcutaneous immunoglobulin replacement therapy in 1,5 year old
boy.
MK *2007
Subcutaneous immunoglobulin replacement therapy in 1,5 year old
boy.
MK *2007
Our experience with SCIG therapy
SCIG replacement therapy is not limited by age
Patients pretreatet with IVIG may refuse SCIG
SCIG is suitable for patients with previous
adverse reactions to IVIG
Thank you for your attention!
ESID Prague Spring Meeting 2009