1. 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

2. 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

3. 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 16 14 JJ 15 13 SM
2009
XLA 2 FV 52
2008 51
therapy in hospital SV
XLP 19 17
refused s.c.therapy

4. Small infusion pump for scig administration
NOT refunded from insurance !
WE lend this pump from company

5. 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)

6. 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

7. 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

8. Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy.
MK *2007

9. Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy.
MK *2007

11. 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

12. Thank you for your attention!
ESID Prague Spring Meeting 2009