CVID

So, currently my blood results are good. After months of not really knowing what’s been going on, and not getting the desired results. It’s a relief to say the least.

So really everything apart from platelets are fine. Looks like the Rituximab and steroids and cocktail of other pills seem to be doing the trick. As it is, my platelets stand at 15. Last week they were 5, so small victories...

I’m currently taking eltrombopag, which is a platelet booster and I’ve been started up on my sirolimus again, but only a small dose, which seems to have helped my platelets.

Got to lot having a rare immunity disorder, right?

Anyway, the real aim of this post is to enlighten people about CVID, which I realise I’ve never really talked about. Not like I have everything else anyway.

CVID, which stands for common variable immunodeficiency, is a primary immune disorder. There’s nothing common about it, as only 1 in 50, 000 people are diagnosed, though I’m sure that means there are plenty more who aren’t.

In a nutshell it means that my immunoglobulin levels are low. Lower than a “normal” person. Immunoglobulins are basically antibodies, which help to fight off infection; the baseline defense. There are five types; A, E, G, D and M, if I am not mistaken. Even though having CVID means all these are dangerously low, they only synthesise immunoglobulin G (IGG). My immunologist once told me that they were looking into making serums for the other 4 types, but I don’t know who these ominous “they” are, and I’m sure it would take years to develop.

Anyway, IGG is what they make. I say make, it’s actually a blood product, so it must come from a person somewhere, somehow, though I have no clue how. IGG is the most abundant type of antibody and so replacement therapy for this type is the best option when faced with low antibodies, I suppose. Its better than nothing.

When I was first diagnosed, the last thing I wanted to think about was treatment options, especially ones that involved more than swallowing some pills with water, but I didn’t have any other choice really. My immunologist gave me some time to think about it, and to see if the levels came back up, which he must have known they wouldn’t, and in the end the decision was made.

I would start the treatment, and continue to have it for the rest of my life. That’s a big step for a 20 something year old. I mean, I can barely commit to what I’m having for dinner. Yet, here I was.

The treatment can be given in one of two ways. Intravenously or subcutaneously. The former meaning that I would be subjected to monthly hospital trips, and through a cannula have several small bottles of IGG pumped into me. As I’ve mentioned in previous posts, my veins are shite. They’re small, and deep, and they move, so quite often when a phlebotomist has found one it disappears whilst they turn around to grab a needle. Wiley little things.

So, subcutaneous administration was looking like the best option. This is simply done at home. Which is fantastic in its own way. I don’t have to go up to London once a month, for IVIG that is, though I’ve been visiting more and more frequently for other health issues lately.

Baxter health provided me with everything I needed. Plasters, dressing, gauze, syringes, butterfly needles, electronic pumps, sterile swabs. I just had to provide my own antibacterial gel.

Once a week I am supposed to set everything up, and then allow it to pump into the fatty tissue of my stomach or thigh (not that I’ve ever used my thigh) and wait for it to be done. It takes time, and sometimes I really struggle to pluck up the effort to do it. At various stages, when I’ve had an autoimmune attack, my immunologist has instructed me not to do my infusions because I could get infections from the needles, and after my blood levels start to right themselves it takes tremendous effort to get back into the swing of home infusions again, but I’ll get there. I always do.

Below is a step by step of my infusions, if anyone is interested.

 Above is a picture of the various components I have to use during an infusion.

A)      The pumps – These are used to push the immunoglobulin straight into my stomach, or thigh if I wanted.

B)      Butterfly needles.

C)      Antibacterial gel, to kill off those pesky germs.

D)      Plasters, for the end, once the needles have been removed.

E)      Syringes – to draw up the immunoglobulin and attach to the pumps.

F)       Hizentra – this is the immunoglobulin.

G)     Sterile swabs, to clean my skin before inserting the butterfly needles.

H)     Sterile sheet, to prepare everything on at the beginning.

I)        What my sister and I call “stick sticks”, to secure the butterfly needles into place when attached to my stomach.

J)        Gauze, to help remove the needles at the end. It’s like your skin creates a sort of vacuum, because you need to press down on the site, with gauze, while pulling the needle out, otherwise it’s almost impossible.

The first step is to draw up the immunoglobulin into a syringe, in this case my product is hizentra, but I have used Subgam previously, which wasn’t doing the trick for me. I had a horrible breathing fridge on the landing for a while, but when they changed my product to hizentra the fridge was history, as this particular product doesn’t need to be stored in a fridge. There’s pros and cons to everything right?

The next is priming the line, which simply involves attaching the above syringe to a needle and line and pushing the liquid through.

And finally, inserting the needles into my abdomen, where there’s plenty of fatty tissue, securing it in place and starting the pumps. 

This takes an hour, with two pumps, to push 40mls of hizentra in. And once that's done, I prep one more lot and push them for another hour.

• It probably takes around 3 hours, all preparation included, and it sure beats sitting around in a hospital for several hours a month. 

Ok, thanks for reading. 

If you have any questions, feel free to ask.

Rachel.