What should I look for in a child with primary immunodeficiency disease?

  Many years ago, there was an American film that depicted the life of a boy with primary immunodeficiency disease who lived in a large transparent ball from birth and lived an “isolated” life where he could see the world through the ball but could not have direct contact with it. Through the film, we can see that children with primary immunodeficiency disease need to be careful, if not treading on thin ice, in their lives. So, what are the main things that these children should be aware of?  First, parents should take special care of these children, pay attention to the weather changes, add and remove clothes for children, and try not to have a cold, because for children with primary immunodeficiency disease, a small cold can be fatal. During the flu season, parents should try to prevent their children from being exposed to the source of infection and prevent infection by not living in a big transparent ball all the time, but it is better to have their own separate room and not to go to places with a lot of people and poor air circulation, such as supermarkets and shopping malls. If there are infected children around, you should let your child avoid them in time, or even go to a childcare facility or school for a while.  Second, as a basic national policy, every child is required to receive the necessary immunizations. However, children with primary immunodeficiency diseases have special immunization plans due to defects in their immune systems. Specifically, children with B- or T-cell immunodeficiency cannot be immunized with live vaccines or live vaccines (such as polio, measles, mumps, rubella, BCG) because these vaccines can cause serious vaccine or vaccine infections and even endanger their lives. In addition, children with primary immunodeficiency should avoid contact with individuals who have just received live poliovirus vaccination, as this may also result in viral infection.  Third, we introduce the concept of graft-versus-host disease (GVHD): when the recipient is immunocompetent or extremely immunocompromised, if the graft contains a sufficient number of immunoreactive cells, it will cause the donor’s immunoreactive cells to react with the recipient’s antigen, resulting in damage to the recipient’s organism. Based on the above concept, blood products that may contain intact lymphocytes should not be given to children with cellular immunodeficiency because of the risk of graft-versus-host disease (GVHD), so it is best to use banked blood and first irradiate it with X-rays to disable the proliferation of lymphocytes in the blood. The plasma also needs to be irradiated by X-ray or freeze-thawed 3 times to destroy the remaining lymphocytes in the plasma to prevent graft-versus-host disease.  Fourth, immunoglobulin or plasma should usually be avoided in children with selective IgA deficiency because the lack of IgA antibodies in the child’s body may produce anti-IgA antibodies after transfusion of IgA-containing immunoglobulin preparations, which may cause severe allergic reactions and even life-threatening reactions when IgA-containing immunoglobulin preparations or blood transfusions are used again. If necessary, the child may be given an asymptomatic selective IgA-deficient donor or the child’s own stored blood. In addition, the child must be transfused with blood products from a cytomegalovirus-negative donor. Children with megasplenomegaly should avoid strenuous exercise to prevent spleen rupture. Children with thrombocytopenia should avoid intramuscular immunoglobulin injections. Antibiotics should be given when the child undergoes surgery and dental procedures.  In general, children with primary immunodeficiency diseases require careful care to improve their quality of life and to prolong their life expectancy.