Hi, I have a few comments on selective IgA deficiency. First, as you said, it is pretty common at 1 in 500 people. It is rare in peoples of Asian descent. Second, if you search literature for IgA deficiency and something, you get not only selective IgA deficiency but also other reasons for immunoglobulin deficiency including common variable immunodeficiency as well as many more. This confuses associations with other diseases because non-selective immunodeficiencies are associated with more diseases that selective deficiencies are. Also many people lower than normal in IgA are called IgA deficient when they really have rather a lot–just not the normal amount. The fact that you have anti-IgA antibodies argues that you are really very low. I work at a children’s hospital with children with cancer and immunodeficiency diseases. I have always heard that the risk of anaphylaxis to blood products (including immunoglobulin) infusion is 1 in 20,000 so being IgA deficient is not enough nor is it to have anti-IgA antibodies since most anti-IgA antibodies are of the IgG1 subtype and it is very rare for them to be of the IgE type. I saw a good paper on IgA deficiency “Clinical significance of immunoglobulin A deficiency” in Annuals of Clinical Biochemistry, 2007, vol 44, p131-139. It lists a lot of autoimmune diseases–17 of them associated with IgA deficiency–especially celiac disease, but GBS/CIDP are not on the list. Myastenia gravis was the only neuromuscular disease. It also states that having IgA deficiency and anti-IgA antibodies are not reasons to exclude the possibility of immunoglobulin infusion. Here we test IgA levels before the first immunoglobulin infusion for which an alternative might be available and also as a precaution. If you were to get immunoglobulin, you are a person that should have it had a place where anaphylaxis could be immediately treated and with special precautions. The point of this is that you could get it, it just should be considered carefully and you might want to get subcutaneous immunoglobulin instead of IV. the problem with subcutaneous immunoglobulin is that it is not as studied in GBS/CIDP. There are also products of immunoglobulin that are lower in IgA levels and you would likely want one of these so your anti-IgA antibodies formed fewer immune complexes. If plasmapheresis works well–great–but if it does not alternatives including possibly immunoglobulin given carefully could be considered.
WithHope for a cure of these diseases
Willie, welcome to this forum. I have a few comments to add. It sounds like, thank God, you have a relatively mild case of CIDP at this time if your primary manifestations are the “stomping” gait after a while of walking and trouble carrying your son upstairs. This is good. The reason that people advocate treatment is that you want to do everything to keep it mild and limit damage and manifestations. For every person, the course is different in how fast things change, how bad they get, and what parts of the nervous system are involved. Also what you feel inside is often much, much more sensitive to the damage that what others can see or even neurologic testing can show. My neurologist says “the view is different from inside the plane than from 30,000 feet below.” Since this is a demyelinating process in which the insulating coating around the nerves is typically affected so that conduction of the signals is smaller or slower, the longer the nerves, typically the more affected they could be. So, most people start with weakness or sensory changes in the feet which have the longest nerves running to them. The stomping is called drop foot and is a slapping gait in which one has trouble raising the toes and rolling the foot as compactDisc describes.
It takes seven times as much energy to climb stairs as to walk flat (and twice as much to go down) and there is additional “work” to balance–especially if you are carrying something/someone as precious as your son and because people especially larger people are in the best of times, mechanically hard to carry (it shifts center of gravity so that the efficiency of muscle use is not optimal and if the muscles are not quite up to usual because the nerves are lapse in comminicating with them, it will show more).
Gabrielle is probably referring to the thread entitled “Steroids Better for CIDP says Dr. Parry” which was last commented on a few days ago. You can see it on the first page on the CIDP Forum or search for this title or keywords. Steroids are an acceptible treatment option. You will want to be careful to try to keep active and keep muscle strength with recoverable activity since steroids can make muscles weak. Steroids are a lot less expensive and, it seems, that pulses of steroids may tend to work better and cause less side effects.
Each person is different in how severe CIDP gets and you may not need prolonged treatment with IV IgG. Do not necessarily feel like this is going to be something that might be “required” for years. One problem with this disease is that NO ONE can reliably predict this so you have to advocate for yourself as if this is going to need to continue or continue to need steroids. Many people, especially men, are reluctant and quiet about talking about financial concerns regarding health care, but it is very important part of today’s world and you should discuss it with your doctor, but try not to make a decision about treatment based on this fact alone. You want the best possible containment of the CIDP because if you stop damage from continuing now, you limit future problems and also medical expenses. Part of the problems of this illness is that is is hard to tell exactly when ALL ongoing damage has been stopped and remains dormant because nerves heal so slowly, manifestations are altered in different situations (such as how tired you are, how steep the stairs are, etc).
WithHope for cure of these diseases.