Thread: IgG IgA deficiency in 5yr old Heart Patient

Hello,
My name is somer. My daughter is 5 years old and just to give you a little background, she was born with several major congenital heart defects including hypoplastic right heart syndrome. 1st open heart surgery was 3 days old....she's had 4 now(so far).- All done bloodless in portland,OR.
We just found out that she has IgG/IgA deficiency. The doctors want to start infusions of human Immuno globulins. As Jehovah's witnesses, we have come across the blood issue many times with her medical history. We have really only come across the issue of whole blood though, so that is a black and white decision for us. There is no grey area when it comes to whole blood. When it comes to fractions however, I'm feeling uneasy. In the past we did give her EPO(erithropoatin). I was wondering how the fraction of EPO compares to the fraction of IgG. I hope I'm making sence here, I just want to make sure that this IVIG is a SMALL fraction. It will need to be given to her intravenously every 3 weeks for the rest of her life. If we decide not to do this they said that since her liver is already not doing so good because of her heart, it will likely cause a lot more problems in the future. Thanks for listening

You may have already looked this up but the 11/06 Km p3-6 explains fractions including what immunoglobulins are. It says that they are protein fractions that make up 3% of plasma.


P.S. I am not a doctor, I hope this helps. I take it you are at Emanual Children's Hospital.

Yes, this was exactly what we were looking for. We read this a long time ago, and just couldn't find it since. Thank You! Yes, we do go to legacy emanuel for all of her heart surgeries. In fact her first open heart was done by Dr. Albert Starr, many people know him. The rest have been by Dr. Iguidbashian (dr. john). They are amazing, and very understanding when it comes to the blood issue. For all of this though, we are going to Seattle Children's since this is where they always transfer her when she goes to the hospital here in Olympia.

Albumin is the blood fraction that is contained in the Epo injection. It is used as a stabilizing agent in this medication. It's extracted from the plasma portion of donated blood, which comprises about 55% of our total blood volume. The red blood cells make up about 45% of our blood volume, and the platelets and white blood cells make up the final 1%. A further breakdown of the plasma yields the following components:

Water- 91.5%
Proteins- 7%. These proteins are Albumin (55%), Immunoglobulins (38%), and Fibrinogen (7%)
Other substances (hormones, nutrients,electrolytes,vitamins)- 1.5%

The Epo injection contained approximately 2.5 mg of Albumin.

Albumin, along with Immunoglobulin preparations for replacement therapy(IVIG) are prepared from large pools of plasma obtained from thousands of donors. These pools are subsequently fractionated to separate out the various proteins. All donors are screened for potential infectious disease agents, including HIV and hepatitis B and C viruses. Following treatment with physical and chemical viral inactivating regimens, as well as nanofiltration, these preparations are remarkably free of transmissible infectious agents. However, because they are blood products, the risk of transmitting infectious agents is always present. The usual treatment dose of IVIG is 300-600 mg/kg every 3-4 weeks, so this would be a much higher dose of a blood fraction then what your daughter received with the Epo. But a point you might want to also take into consideration is that during pregnancy, Moms share some of their antibodies with the unborn fetus. If this didn't occur, once the baby was born, it would have no readily available defenses to fight off bacterias or viruses it might be exposed to in the first few weeks of life. Babies also receive antibodies in breast milk. Once again, this serves as a protect until their immune system is able to fight off these infections on its own.

The immunoglobulin deficiencies that your daughter has puts her at increased risk for ear, lung and sinus infections, just to name a few. Even though these infections can be treated with antibiotics, she would be required to take these antibiotics at higher doses and for longer periods of time then someone with a normal immune system. And frequent lung infections can have a detrimental effect on her heart.

Accepting minor blood fractions is a conscience matter. Since you have felt comfortable with accepting blood fractions in the past, try not to focus so much on the amount of the blood fraction that would be given. Hopefully, this explanation will help to make you more comfortable with your decision.

Originally Posted by somer

Hello,
My name is somer. My daughter is 5 years old and just to give you a little background, she was born with several major congenital heart defects including hypoplastic right heart syndrome. 1st open heart surgery was 3 days old....she's had 4 now(so far).- All done bloodless in portland,OR.
We just found out that she has IgG/IgA deficiency. The doctors want to start infusions of human Immuno globulins. As Jehovah's witnesses, we have come across the blood issue many times with her medical history. We have really only come across the issue of whole blood though, so that is a black and white decision for us. There is no grey area when it comes to whole blood. When it comes to fractions however, I'm feeling uneasy. In the past we did give her EPO(erithropoatin). I was wondering how the fraction of EPO compares to the fraction of IgG. I hope I'm making sense here, I just want to make sure that this IVIG is a SMALL fraction. It will need to be given to her intravenously every 3 weeks for the rest of her life. If we decide not to do this they said that since her liver is already not doing so good because of her heart, it will likely cause a lot more problems in the future. Thanks for listening

I have cared for several children with IgG and/or IgA deficiencies over the last 25 years. My approach is changing. Previously, I had favored the treatment that your doctors have recommended.

After almost losing a patient due to a severe anaphylactic reaction, I stopped his infusions. That was 10 years ago. He is actually doing quite well, despite still having low immunoglobulins. His IgM runs low as well.

After seeing how well he was doing, we stopped infusions on his older sister, who also had combined variable immune deficiency. She is also doing well.