Primary Immune Derficiency (PID)

Wow - well a lot has happened since I wrote this post. After I  saw my Immunologist a few times, I was diagnosed with Common Variable Immune Deficiency. This is an umbrella diagnosis and so far there are said to be over 250 known variations. Common because as as patients we have some common symptoms and we all have deficiencies in one or more type of antibody. Variable because each of us then has different deficiencies, different symptoms and different complexities with our immune system.

I started having Immunoglobulin Replacement Therapy soon after my initial post below (c March/April 2012). I was having IVIG (IV Immunoglobulin) every 3 weeks at the Wellington Hospital and then after 3 or 4 infusions moved to Royal Free in Hampstead. It used to be a whole day affair with the infusion running very slowly at first and it would take 7 or 8 hours plus the journey time and then the initial debacle of cannulating me in the first place. My veins eventually became impossible for the Specialist Nurses to cope with so I was trained to do subcut (SCIG), infusions at home. As it is a much smaller dose, it only takes a few hours and although it is quite painful sometimes (thick, gloopy liquid being infused just under the skin), I dont suffer half as badly with the after effects as I did on the IVIG, which used to put me out of action for 2 days after).

Now, it's almost the end of 2015 and from an immunology perspective I've had a relatively good year. I did however have a close call in February. I was at home and when Mummy couldn't wake me and I had an extremely high temperature, she called an ambulance. I remember the paramedics trying to get me out of bed and after that, nothing for 3 days. They took me straight into Resus, not unusual for me, but this time they sent Mum to the relatives room/sent her to get some tea whilst they 'worked' on me. They struggled to get access but eventually did and with a temp of 39c+, the blood tests showed that I had severe septicemia, blood poisoning. When I came round a few days later, the Lead Constant of Acute Medicine came to chat with me. I usually believe that I can do anything, even with this immune deficiency and believe I can bounce back from nasty infections but until he explained quite hw ill I had no idea how bad the situation was. He told me that when I arrived in Resus, the nurses and doctors who know me well didn't think I'd make it through the next 24 hours and that some of the nurses stayed after their shift had ended so that they could look afte me. I was on double dose IV antibiotics for 17 days in hospital. A few days after leaving there I felt .fine.!

The only other really nasty infection that I have had this year was in May when I had Pneumonia in both lungs and fluid in both too. Whilst I fele completely rotten during my 10 daus in hospital, I was OK and up and driving the nurses crackers in no time! Since starting this blog in 2012, I have had about 3 bouts of Pneumonia a year and touch wood I've only had 1 this year and we're in November. Always look on the bright side...

Whilst these have been the two major infections I've had this year, I have had countless minor infections and CVID has the tendency to exhaust you and wipe you out just when you least expect it. It also causes havoc with my diabetes. Very inconvenient when you have plans!!!

I will write more about this somewhere but I could have this far or dealt with this without my wonderful family but also without UKPIPS (UK Primary Immunodeficiency Patient Support). They have taught me so much about my condition and helped me with fighting to get the right treatment. I can't thank them enough.

Antibody Deficiency Disorder

Tonight (Tuesday, though now it appears to be Wednesday am) I went to see my Immunology Consultant in London. This is the big news that I have been waiting for. 

Although I was prepared for this and knew it was coming, it still took me by surprise when he was going through the various problems that I have. Hopefully it will reduce the number of infections that I have and radically reduce the amount of time spent in hospital (4 months in the last 8). 

I have 3/4 deficiencies of one type or another. Autoimmune diseases arise when the immune system mistakes some part of the body as a pathogen and attacks its own cells. My primary deficiency is with my antibodies. I am still taking it all in but will post here when I know more about it and have done some research. My white blood cell count is much lower than it should be, which means that when I have an infection my body is not fighting it effectively. This is apparently due to the fact that the few white blood cells that I have seem to have confused themselves and now do not have memory of previous infections, meaning it takes them quite a while to figure out that I'm ill and then what to do about it. 

Whilst I was there today, he also diagnosed another bout of pneumonia. Not ideal but it could be worse.

The plan of attack is to:

• Take 14 days of antibiotics to clear the Pneumonia
• Have the Pneumovax II vaccine
• Start on preventative antibiotics
• Go back to work 3 weeks after the vaccine if the Dr is happy to say I'm fit for work!

Fingers crossed this works - the next step is antibody infusions. For the first 3-6 months of these infusions i would need to spend a week in hospital every month, followed by maintenance therapy every 3-4 weeks after that. Keep your fingers crossed and hope it doesn't come to this!