Hello, I was diagnosed in 2008 with CVID (specifically low IgG antibodies), autoimmune hives, autoimmune hearing loss and unrelated SVTs in the heart. My 3-year old nephew is being tested for periodic fever disorder. In doing research, I ran across Muckle-Wells and was astonished to see sensorineural hearing loss, recurrent hives and joint pain all linked to one disorder, plus that amyloidosis could cause conduction problems in the heart. Everything I've read says that the hiving related to Muckle-Wells is not itchy. Mine is not horribly itchy, but is somewhat itchy. Does anyone on here with this diagnosis have itchy hives? I have an appt with my hematologist next month and will bring the possibility of Muckle-Wells up. Thanks.

Hi, My name is Karen Durrant, and my middle son has a form of Cryopyrin-Asociated Periodic Syndrome (CAPS) called NOMID. I also run The NOMID Alliance. My son used to have itchy areas when he had flares of the rash, which is the same rash for all CAPS, whether MWS, NOMID or FCAS. Most people do not feel itchy, but some have an itchy or even occasionally a burning sensation with the rash, during flares of symptoms. Please look at our website for more info, and please share the link with your nephew's famil as well. We can be of help. www.nomidalliance.net Also on our site there is a downloadable pdf of a medical guidebook co-authored my leading doctors on CAPs, and myself at this link: http://www.nomidalliance.net/downloads/finalCAPSbrochure_web.pdf You may find the chart in the center spread of the booklet helpful. I would bring the pdf with you to the doctor's visit, as you may have a form of CAPS, but you will need to get connected with a rheumatologist to help with the diagnosis. Do you have elevated C-reactive protein, ESR, or other abnormal labs? People with CAPS can have high C-reactive protein, especially during flares, but it can be normal at other times if they are not flaring in the most mild forms, such as a mild form of Familial Cold Autoinflammatory Syndrome (FCAS) that is triggered by cold or cooing temperatures. Muckle Wells and NOMID can be triggered by the cold, but often they have flares of symptoms that are frequent, and can be ever-present. Contact me if you have more questions at site_manager@nomidalliance.net Good luck, and I hope that you find some answers soon! The good news is that if you have CAPS, there are some wonderful and very effective medications that will change your life for the better, so there is hope for you and your nephew. Karen Durrant

Thanks Karen for the information. I haven't had my CRP or ESR tested in a while, but they were high prior to starting IVIG therapy for the CVID. My platelet count and neutrophils were high for 6 months then dropped to well within normal ranges for a few months until this last month where they are now at the top end of normal. I believe this can indicate inflammation. Overall WBC is normal. I've been fighting mild hives for the past couple days, ear ringing and just feeling pretty crappy. I have my next infusion and lab work tomorrow. Cold doesn't seem to be a trigger. Unfortunately my hearing is already badly damaged. My main concern is amyloidosis and if it's connected to my heart problems. I'll check out your links. Thanks again.

My son had high neutrophils (commonly seen in patients with MWS, NOMID) and also had very high platlets before going onto the right medications for this syndrome. Cold is not a trigger, or noted in the more pronounced forms of CAPS, such as some with MWS and most with NOMID, but can be a factor. I never noted cold correlation with my son, until he was on medications and so healthy, but at the point of needing a bit higher dose, He then would occasionally have cold-induced flares, but it has to be really long cold exposure, and was only noted once he was under good control 99% of the time for his NOMID. It was like since he had such good control of his disease in general that when he got a breakthrough flare, it was more like FCAS (the least inflammatory CAPS condition) versus the huge flares he used to have all the time with NOMID. There are labs to have done to test Serum Amyloid A (SAA) but the test would need to be sent out to a special lab. The NIH in Bethesda, MD could see you and do all the tests, if you were interested. They are the best we know in the US for dealing with these rare diseases, and would be able to determine if you have MWS on top of something else, or just MWS. I know one lady that was on IVIG and prograf by PICC line for over a year, then someone finally figured out she has CAPS ,and got her onto anakinra. She is now free of the IVIG, prograf, PICC line and living a great life for the first time ever! She wishes that someone had figured out sooner that she had MWS. Good luck, Karen

I'll keep Bethesa in mind. Fortunately, I'm in the Dallas/Ft. Worth metroplex and have access to many specialists...have already seen just about every type...and had previous genetic testing done, but not for CAPS. My bloodwork yesterday was platelets very high and neutrophils high, but I'm feeling great today after the IVIG infusion. I get Decadron with it and wonder if that's toned down the likely autoimmune issues from the last couple of weeks. I have an appt scheduled with my hematologist and will ask for the ESR, creatnine and SAA tests. Thanks again for your input. -Rebecca