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IVIG infusion

At age 25 I had diagnosis of Allergic bronchopulmonary aspergillosis
At 59 my current age I had a diagnosis of CF by a CF doctor. I have a Delta F508 gene and the 5T variant.
I also have a Primary Immune deficiency diagnosed by an immunologist. I had no titers to the pneumonia vaccine.
In December of 2011 I had a pulmonary embolism. I was put on Warfarin. I am told I will be on Warfarin the rest of my life.
I also have neuropathy. I have a follow-up appointment with a Neurologist in December. I went back into the hospital after the sinus surgery because I fell several times. I hurt myself and there was a concern I would hit my head.
There was a concern that I had Multiple Myeloma. The Hematologist determined that I had MGus.
I have had sinus and pulmonary problems all of my life. In the last year I have been in the hospital 11 times for a total of at least 4 or 5 months. In October I had sinus surgery. Burkholderia Cepacia along with 2 additional bacteria was cultured from my sinus and lungs. In the last year I think I have had 11 different bacteria cultured. I have been on intervenes antibiotics for most of the last year. The Immunologist would like to start me on IVIG infusions which would be another thing to be on the rest of my life. I don’t think that antibiotics every 6 to 8 weeks is the way to go for the rest of my life. What are your thoughts on the IVIG infusion?
Dear questionner.

Thank you for your detailed and interesting narrative. You have clearly had a very challenging time with a range of illnesses, some of which are related to cystic fibrosis. Firstly you have a slightly unusual combination of mutations F508del and a 5T polymorphism. This combination sometimes causes cystic fibrosis though in other individuals it can result in very mild disease presenting only as congenital absence of the vas deferens. The occurrence of primary immune deficiency with cystic fibrosis is unusual, though from your letter it suggests that this diagnosis was made on the diagnosis of a lack of response to pneumococcal vaccine.

The pulmonary embolism is possibly unrelated to cystic fibrosis and I am not sure what the explanation for the monoclonal band is. You would be very young to have multiple myeloma.

The question regarding starting IV immunoglobulin on the basis of, I’m presuming only a poor response to pneumococcus is a difficult one. The key question is the borderline immune deficiency or cystic fibrosis causing you to be susceptible to infection or are indeed both contributing. My own approach would be to maximise therapy aimed at your cystic fibrosis first, as there is strong evidence for this, while immunoglobulin replacement in individuals who don’t respond to pneumococcus is less clear. In my clinic we have had a number of individuals in this sort of situation and I have found it best for the CF and immunology teams to discuss the patients together and come to a conclusion.

So my final advice is to go back to your doctors and encourage them to get their heads together and decide the best course of action for you.

Best wishes
Prof Stuart Elborn
The answer is edited by: Prof Stuart Elborn