Hello,
could anyone tell me if they have had any lung issues
related to evans my son is going through testing in regards
to nodules found on ct scan we will be having a biopsy soon
their original thoughts have been fungal infection due to supressed
immune system but that has not been confirmed.
Thank You, Julie

Yes, my son is 15 and lung nodules were discovered on an xray and confirmed by CT scan last fall. He also had enlarged lymph nodes throughout his body. In December they decided to do a lymph node biopsy (under his arm) and bronchial lavage. The lavage came back clean and the lymph node biopsy showed follicular hyperplasia. The doctors feel the lung nodules are related to the lymphoproliferation that he is exhibiting in his lymph nodes and spleen, so they decided not to do an invasive lung procedure to investigate further. 

 

Scans were repeated in December, February and again last month, and they have shown that the nodules "wax and wane" - some disappear and new ones will appear. We are following him closely with pulmonary function tests as they feel this is the best indicator of his lung health. So far his PFT tests have been fine except for somewhat low diffusion results. This result has improved slightly over the last 6 months, so they are not concerned at this time. My son has been getting IVIG treatments every 3 weeks for the last 6 months in the hopes that it would help with the lung nodules (there are reports that it helps when lung nodules are related to certain immune deficiencies), but it does not seemed to have helped in this regard for my son. The regular treatments have helped stabilize his platelet count though.

 

I'm glad you wrote because I have been meaning to post an update on my son. He is being followed very closely by the NIH and they would like to find other patients like him. I told them I would reach out to this group. The lead researcher working on my son's case has a new web page describing her work, and specifically mentions Evan's patients, and lung nodules. They are looking for Evan's patients that also exhibit signs of immune deficiency. If your doctor has not run immune tests yet, her group will give advice on the types of tests to run. Here is a link to her page:

 

 

If you would like to find out more information you can contact Helen Matthews, the nurse coordinator for the study. She can be reached at matthewsh@.... Patients have to be referred by a physician to participate in a study at the NIH, but you can reach out to Helen Matthews for some preliminary information.

 

I hope some members of this group will consider looking into this. I am extremely encouraged by how interested the NIH seems to be in finding out what is causing my son's perplexing symptoms. It is a big change from only a few years ago when I couldn't interest anyone at the NIH in my son's case.

 

Julie, please feel free to post any questions you might have about my son's case to the list or email me privately. I know how scary all this can be.

 

Susan A.