Two weeks after Jordan's Immunology appointment, I finally got a call from Dr. Wu, Jordan's Immunologist. Its definitely confirmed that he does have an immune deficiency called CVID.
Basically to give you a run down on what CVID is, so you don't have to google it (LOL), its a disorder that impairs the immune system. Patients with CVID are more susceptible to infections from bacteria and viruses and they can become recurring; also pneumonia is pretty common for CVID patients, so they can eventually end up with chronic lung disease if the infections continuously happens (luckily we have not dealt with any viruses or infections with Jordan). It can also cause symptoms we have dealt with since Jordan first started this journey, such as enlarged spleen, liver, and lymph nodes.
So as of now, I have no clear answers about the Rituxan, everyone is basically waiting for Dr. Gold (Jordan's hematologist) to give the go on that, on when we will start (or if). I am thinking he is waiting on this week's platelet count to determine on when we will start that treatment. Labs are scheduled to be drawn tomorrow afternoon (had to reschedule this afternoon's appointment). I am going to see if we can start the treatment the first week of November, this way Jordan is at school the next two weeks and will be there for his mid-term exams the last two days of October.
However, Dr. Wu has a plan as far as his CVID goes. Since his IGG (one of his immunoglobulins) are low (well they are all low, but we are concentrating on just the IGG), two weeks ago they were at 443, and she would like to see them maintained around the 800 area; she wants to start doing IVIG every 28 days for a couple of months (at the local hospital, through an IV) to see how his IGG levels do, and eventually transition his treatments to SubQ (Subcutaneous Immunoglobulin) therapy at home once a week. This will maintain his immunoglobulins levels, which will help his platelet levels, and he won't feel so fatigue all the time either. I will be trained on how to do the SubQ therapy at Chapel Hill, but after many hours of research and watching the videos on the websites for SubQ, I am fairly confident on giving him this treatment (I swear I need to go to school for this).
When we do travel to Chapel Hill for his first dose of Rituxan, Dr. Wu wants to do breathing tests on Jordan to check his lung function, she also wants to do some blood tests to check his thyroid levels and other things, since CVID patients are susceptible to other auto immune disorders , which is sounding to me like a domino effect. Start with ITP, move to ES, and then find out you have an immune deficiency, to which is the cause of you having ES. So my advice to those reading this, and been dealing with ITP/ES, go see an immunologist and get tested to see if you have an immune deficiency. Had I known this 8 years ago, when Jordan first started with ITP, I would have pushed for more testing, instead of waiting all these years for answers on WHY he has this.
It most likely is genetic (CVID), but no one has approached Brian or I on genetic testing to see which one of us carries the gene, and frankly I don't see a need to, we aren't having anymore kids, and our other two children are fine. So basically this is something Jordan needs to stay on top of, when he has a family of his own, to make sure these tests are done when his children are first born. Because Jordan has showed signs of low blood counts since he was a newborn, but they chalked it up to him just having severe jaundice (low hemoglobin counts), which is fairly common in babies anyway. So there was no need for alarm. So my personal thoughts, he has always had ES/CVID, and like with most patients, it just takes years to show symptoms, and I think that was the case with Jordan.
As far as life expectancy goes......yes, there is a chance we can lose him, but hell you can lose a loved one by them going to the grocery store; so we don't dwell on that, we are enjoying him in the moment. Patients with CIVD live well into their adulthood, it all just depends on the severity and frequencies of any illnesses he experiences; and we have been very lucky in that aspect, because he is rarely sick. So I fully expect for Jordan to outlive Brian and I. :)
Will update in a couple of days as soon as I get lab results and hear from Diana about what is next. As always, thoughts and prayers please.
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