Charlie’s Very Irritating Disease (CVID)
This is the first of 2 (so far) posts about my diagnosis with a primary immune deficiency (Common Variable Immunodeficiency) and my first treatments. This one is the lead up to, and including my first treatment. The second post follows on from this one up to my second treatment.
About a month ago, on the 15th of July, I got a pretty life-changing diagnosis. It was simultaneously terrible and amazing news.
I was told I have something called Common Variable Immunodeficiency (or CVID). Dubbed “Charlie’s Very Irritating Disease” by my Irishman. Pretty accurate.
CVID is pretty rare, and I’ve mostly read blogs by some incredibly sick people who weren’t diagnosed until they were much older or much sicker. They seem to have extreme fatigue, joint pain, auto-immune diseases and other serious ongoing medical issues. I thought I might be able to write about what it’s like to be diagnosed a bit earlier, at 27, with what appears to be no serious long-term organ damage. I’m obviously very lucky, but hopefully someone reads it and relates to it as I found it hard to find something that I could draw proper parallels with.
Otherwise this might just inform people about this random rare condition that they probably haven’t heard about and might explain to my friends why I don’t “die quietly” very well and just cough all the time instead.
CVID is a primary immune deficiency in which the immune system hasn’t developed or matured properly and doesn’t create enough immunoglobulins (or antibodies) to keep you healthy. Antibodies are required to fight bacterial and viral infections, so people with CVID often have recurrent infections. 20% of people with CVID get autoimmune diseases (their immune system starts attacking healthy cells – eek!).
In NZ, between 1 and 2 people in 50,000 have primary immune deficiencies (I’ve been told there’s probably about 120 people in NZ and 50 in Auckland, where I am). Because of its rareness, CVID is most commonly diagnosed later in life in people between 30 and 40 years old, although 1 in 5 will have had symptoms in childhood.
As people with CVID have recurrent infections, there is the risk of organ damage from them not being treated properly. Commonly, people with this immune deficiency will have chronic sinusitis and damage to the lungs, pneumonia is fairly common. There is also a good chance of skin infection, and joint and bowel issues.
There is no cure but there is treatment, although it’s ongoing and fairly intrusive. Unfortunately I can’t kick start my immune system with a bit of a boost as I had hoped, I have to constantly keep it topped up with antibodies for the rest of my life in order to attempt to be as healthy as a “normal” person.
So yea, not a great diagnosis. I’ve been an increasingly sickly person since childhood which got much worse in the last 3 years and really quite bad these last 3 months.
It’s a huge relief in a way to finally have an answer, and hopefully be able to get a fix. It’s also opened my eyes a lot to why my life has panned out as it has and answers a lot of questions!
If anyone has spent any amount of time with me, they probably know I’m kind of “that guy” when it comes to being ill. (I can especially see it in old Facebook posts)
In hindsight, I remember as a child having cold sores and regularly having mouthfuls of ulcers and ear infections. I’ve been prone to UTIs for as long as I’ve known (Yaaay! Girls know what I’m cheering about), but when I was little I underwent lots of tests to have this explained by the fact I have a duplex kidney (my right kidney is one kidney with the internals of two kidneys) and it was written off. Now we know that was probably a combination of my kidney and CVID.
I remember at high school being in the sick bay more often than all of my friends. The health nurse and I were tight and I went to my creepy doctor fairly regularly. To the point that sometimes I escorted people when they didn’t know where to go. I also recall getting strep throat all the time and other random illnesses, as well as getting the severe reaction to the Meningococcal vaccine (which was horrible).
If it’s going around, I’ll probably catch it. I’m buddies with my pharmacist. If I get run down, I get sick. Every time. And lately, if I get drunk, I get sick a couple of days later.
Actually, I’ve always had terrible hangovers… I wonder if this is why…
I’ve joked to my friends in the past that I always get the worst case scenario in health so I imagine if I got pregnant I’d have 9 months of morning sickness. And I’ve said to a few people that without modern medicine, I don’t think I’d have lived past age 6 and would have died of something gross like a bladder infection. People seemed to think that was far-fetched but it doesn’t really seem it now. In terms of health, my body isn’t really doing it’s part to keep me alive by itself! It’s a little scary to think in a Darwinian way and realise that I’m not the fittest so probably shouldn’t be surviving. Sorry, evolution.
I definitely recall that whenever I got sick, I couldn’t seem to budge it on my own. I’d try avoid doctors but I’d end up with about 3 things wrong with me and have to go. I pretty much never went for just one issue. They blamed my iron for a long time as a (mostly) vegetarian I was often anaemic, now I wonder if that was really the issue.
On one occasion I went to my doctor in Kingsland around 2011-2012 really sick and voiced my concern to him. “I get pretty sick all of the time and it’s usually lots of different things, I’m kind of worried there’s some kind of underlying issue that might be causing it.” My extremely competent doctor replied that “some people are just unlucky”. I kinda want to send that guy a strongly worded postcard now.
I just remembered – I got Swine Flu when everyone was panicking about it in NZ. Seriously!
Just over 3 years ago, I moved to the UK on my 2 year working visa. This is when it became most obvious that there was something not quite right.
Pretty much every place I lived in, I was that sick person.
Sick the entire time working at the London Olympics, glandular fever in Southampton, persistent cough and always getting random illnesses in Ringwood, Blackpool and Bournemouth (ask anyone I worked or lived with), then when I was in Ringwood for my last few months, I, amazingly, kicked the cough briefly for the last couple (which was weird).
Pretty much every place I travelled to, I was that sick person.
I got terribly sick at Oktoberfest when no one else did, moved myself to an empty hostel dorm in Prague so my coughing wouldn’t keep everyone up, was ill in Amsterdam, and felt like I was dying in Turkey. To be honest, it’s hard to remember how sick I was in the other European countries I visited as coughing was so much my norm it doesn’t stand out to me when I think back.
I coughed my way through Budapest, Krakow (Polish vodka helped. Seriously) and almost passed out in Berlin from trying to push through my sickness. Went out and watched the football final that night despite the feeling of death. Coughed, vomited and diarrheaed my way through China, Vietnam, Cambodia, Laos, Thailand, Malaysia, Singapore and Bali. Good tiiiimes!
That cough that pretty much started when I arrived in London in 2012 has stuck around on and off for about 3 years so far. It’s so persistent, and ranging from an annoying little cough to a full on one that develops into chest infections and other fun things when it feels like it. Turns out it’s potentially from chronic sinusitis (which I still have), but it’s never been fully treated and without recent diagnoses it just seemed like an annoying cough to the untrained eye.
Ha, what a hopeful fool.
However, it’s been what’s caused my bronchitis about 3 months ago, which took ages to (almost) go, then that developed into pneumonia which has now gone, leaving me just with chronic sinusitis and a chest infection (and still getting over the damn pneumonia). My body just won’t kick it despite the fact I’ve been given 7 different lots of antibiotics in those 3 months (and still going).
I even had shingles earlier this year. That’s what old people get! Also this year, my fingernails went weak and started peeling apart and my normally wavy hair is sad and lifeless. Dafuq body. Sort it out!
But no, it can’t.
I sure write about being sick a lot on Facebook…
Being some degree of sick, constantly, for years seriously lowers your quality of life.
It kills a degree of your social life. You can drink through sickness but you’ll pay for it dearly and that’s not ideal.
It hurts your work – I burnt through my sick leave in the last couple of month, plus two weeks on top of that. My career is really important to me and it kills me to feel like I’m not pulling my weight. I know I’m better than what I can show at the moment. My work is ridiculously supportive and understanding though and are just worried about me getting better so I am so grateful for that!
Relationships can suffer when you can’t do all the things that couples do when you’re sat in bed on a Friday night feeling sorry for yourself. I feel like I can’t be the fun, active girl that’s in me with the people I enjoy being with, and that’s not what I want at all. Luckily I have a huge amount of support from the people closest to me and we’re making the best of the situation.
It’s a bit depressing at times, and I’m not using the word lightly, I mean literally. I’ve had break-downs. Like, all-out, uncontrollable “why me?!?!” bawling my eyes out in the shower. What’s the point of life? What did I do to deserve this? I don’t think I’ve done anything that terrible, why is karma ruining my life? Why have I experienced a whole lot of trauma, only to get through it and be FUCKING SICK forever?
That particular day is potentially the lowest I’ve ever felt (it was pre-diagnosis – at least now I know the science of “why”). Luckily I was not alone that day, I had a confused boy holding me while I unleashed tears all over him, barely able to speak and tell him why I had left for the shower fine and come back in pieces, and a friend brought me lunch and a hug. He said he had never seen me look so low (a few weeks later, not that day haha).
But I come back up. It’s a rollercoaster.
I’m actually in essence a fairly healthy person. I eat pretty well, exercise regularly (when my lungs work) and try sleep 8 hours a day. In fact, I often have friends say “how are you always sick?! You’re so healthy!” Um, no, I’m not healthy. I have a fairly healthy lifestyle, but I am FAR from healthy.
In hindsight, there were a few signs that I don’t have the immune system of the average person.
My handbag is like a roving drug store. To the point that people know and will come to me if they need painkillers. I am currently carting around antibiotics, probiotics, an inhaler, paracetamol, decongestant tablets, 2 different bottles of inhalant decongestant herby stuff, dermatological moisturizing lip balm, and tissues. But I’m not that sick at the moment, so it’s much less than usual 😉
Speaking of tissues, I get anxious if I leave the house without them. I take 2 or 3 in every single pocket I have if I’m going to be out for a while, plus the ones in my bag and a few loose extras jammed in between the medicine. It can get dangerous laundry-wise. I’m as prone to white speckles over all of my clothes as I’m prone to UTIs.
I have a jam-packed medicine drawer next to my bed and when I travel, I have a special little toiletry bag chocca full of drugs. They cover all the basic things I often get as a ‘just in case’ to cover a weekend away, as well as enough paracetamol to keep me above ground if I need to be taking them for, say, a week straight.
And I’ll need to use something in it, guaranteed.
I buy paracetamol in 100 packs when they’re on special. Like, I get excited when I see that cos medicine is expensive yo!
I’ve literally only just realized that all of these things are really not that normal. I just thought I was a little different. But after talking to a couple of people, nope, girl be worrying about her health all the time when apparently other people don’t… Huh. Funny that.
Getting diagnosed was not great.
I had appointments with the doctor, a chest x-ray, an allergy specialist who also got me a sinus CT Scan and a blood test, and a respiratory specialist appointment lined up to kick off the search for why the hell I was sick all the time. I was getting over pneumonia so I wasn’t well at all.
The call came from the allergist telling me the CT Scan showed chronic sinusitis and it would require an aggressive treatment to get rid of it. Then a few minutes later, he called back having looked at my blood test results. His voice was much more serious so I knew something was wrong. He told me my immunoglobulins should be between 7 and 16, but they were only just over 1. I didn’t know what that meant, but I could tell that’s ridiculously low.
He then said “it looks like you have a primary immune deficiency, so it’s not AIDS, because it’s probably a genetic thing”. He literally compared my immune to that of a person with AIDS, then set me up with an appointment with an immunologist from his practice a couple of hours later.
The next couple of hours were terrible. I was so upset and confused I had to call up work and explained it to them that I couldn’t work from home that day, I couldn’t concentrate when I thought I could potentially be told I was dying in a couple of hours.
Google didn’t help. So I waited, talked to people, waited, talked to my parents, waited, sat and thought. Longest couple of hours ever.
Finally I got to my appointment and the immunologist asked me a bunch of awkward questions about my health and lifestyle, took my temperature, listened to my chest, felt all my lymph nodes (including in my armpits – gross), tapped me around my stomach, took my blood pressure and heart rate. Then just took me to his office to give me the paper he had already printed out that told me all about Common Variable Immunodeficiency. Gave me a brief run down about CVID and the treatment and asked if I had any questions.
What do you ask when you’re told you have something that you hadn’t heard of until 2 minutes beforehand?!
I optimistically asked if the treatment was ongoing for the rest of my life or just till I’m topped up with the goodness and he said “well…. That’s the bad news”. Ugh. I started crying.
I asked if I had a baby if they’d get it, that answer was more positive. There’s a chance but it’s pretty low because it’s so rare. Ok.
That’s all I remember asking. He booked me in an urgent appointment at the hospital immunology department (apparently 2 weeks’ time is considered urgent) to get a proper diagnosis and set up for treatment and sent me on my way.
I shouldn’t have driven home that day. I felt empty. Confused. I was crying but hadn’t packed enough tissues (which is unlike me). I called my parents and bought some McDonalds. That helped.
My glorious parents drove up to Auckland that day, took me out for dinner, stayed the night then took me home with them the next day to deal with life, recover from the pneumonia/chest infection/sinusitis and generally come to terms with what was going on.
I stayed there for about 2 weeks, bar coming up to Auckland for the respiratory specialist. He told me my lung function was fine but it seems like I might have some minor lung damage due to all of the infections I’ve had in my chest. Nothing to worry about thank goodness. Chest CT Scan is still lined up so we can see exactly what’s wrong with me.
Other than that, I obsessively googled, watched YouTube videos, read blogs, found websites, joined a Facebook group to find out what the hell was wrong with me, as sitting around waiting for a professional to tell me was going to take too long. I started a list of questions to ask the immunologist.
Most of the time I was sat on the couch, fatigued from being ill, and just sad. I felt like I was spending most of my energy trying not to cry. Like, what is life without health? What will life be if I rely on plasma infusions every month? How will it affect my work? How will it affect my travel? How will it affect my relationships? Can I ever live in another country again? Can I travel everywhere still? Do I need to travel for more than 3 months at a time? What if I got pregnant – how sick could that make me? Would I be a terribly sick mother? Can I keep a career for years? Will my health deteriorate faster than a normal person?
Can I keep drinking alcohol and coffee? That’s kind of a non-negotiable for me… I want the good things in life, but I don’t mind if it’s limited.
I was so distracted by these thought, I could be in the middle of a conversation and randomly just go, “Oh! Diabetes! I wonder if I’m more at risk… Anyway, keep talking.” Bit rude haha. Probably understandable though.
I think I was experiencing some mild depression. Just balancing on the edge of ‘just ok’ and ‘is there a hole I can sit in for the rest of my life?’
I had some weird mental ups and downs, and trying to stay optimistic (which is kinda my thing) was getting seriously difficult. I had a bit of a ‘moment’ when I found out that some of the people that I had told about the CVID had been telling our mutual friends.
I mean, of course they were! I don’t know what I expected them to do if someone asked why I was hiding at my parents’ house for 2 weeks. I was just confused that people knew such a personal, life-changing thing about me as second-hand information. Almost gossip. I felt like what was monumental for me was gossip for others.
And no one else had called or messaged me.
My life had flipped, people knew about it, but hardly anyone contacted me.
Now I understand – it’s weird. How could anyone know I was having a bit of an internal crisis. You can’t expect people to know what you want them to do, especially when you don’t actually know what you want them to do yourself. I was just a sad little mess who was confused and didn’t know how to deal with it. I got over it pretty quickly, it was just interesting the little things that seemed to get me upset for no real reason.
Also, no hard feeling to anyone who didn’t contact me – I get it. I probably wouldn’t have either. I’m ridiculously awkard in these situations. On that note, thanks to the people who messaged and called – that was really sweet 🙂
Anyways, I’ll fast-forward to July 29 for my first proper appointment at the hospital.
I managed to get there just on time. My parents were a little late so I was soooo grateful to have the Irishman with me to relieve the nerves so I didn’t throw up on myself or something.
Mum and dad arrived and we went in to chat to the immunologist. He was young and cheerful which was immediately calming and made me wonder what I was stressing about.
He didn’t immediately explain why I was there, just asked what I knew and what I wanted to know. I had a list of questions so I unloaded on him and what I got out of it was…
In general the answer was, if a normal person can do it, you probably can too (with some treatment under your belt). Technically, I’ll get sick like the average person, and go to the doctor like normal. Difference is, I’ll get longer prescriptions for medicine (thank god, because I’m ALWAYS under-prescribed), and I get the green light for having my own antibiotics at home to self-treat when I know I’m getting an infection (because I’ll know I have one after having so many in my life).
It’s free. Thank you New Zealand! <3 It would cost me tens of thousands of dollars a year otherwise I believe which does seem a bit ridiculous as a measure to just keep me alive.
He said that the people whose blogs I’ve read about on the internet were probably diagnosed later than me and have more health issues because of that. On the other end of the spectrum, kids that are diagnosed can often be in life-threatening and dire situations, so I’m one of the lucky ones in the middle. Those like me are probably going to be less likely to be writing about their ailments simply because, even though they’re probably sicker than the average person, once they’ve started treatment, ideally they’ll have a fairly normal life. And THAT’S why reading about CVID online can be a little intimidating.
I also apparently can’t get live vaccinations, which includes Yellow Fever. There are definitely countries in South America and Africa that are high risk for Yellow Fever, and many countries that won’t let you in if you’ve been to one of those countries without a vaccination. This obviously needs more research on my behalf, but besides being really sick and sitting in a hospital for a few hours a month, this is the worst news I’ve gotten with this diagnosis. I’ve been dying to go to South America for years now and was planning on learning Spanish especially. Hopefully there is potential for me to still go…
Treatment will be ongoing from now on. At the moment it will be every 3 weeks in the hospital. The first one will take 4-5 hours but after that, maybe around 2-3 hours. This may go to every 4 weeks depending how my health changes.
Treatment is an infusion of intravenous immunoglobulins (IVIG) which I believe is extracted from the plasma of thousands of blood and plasma donations and goes into an IV on my hand. It’s clear which I thought was weird. They use such a large pool of donors to get the range of antibodies to fight various viruses and bacteria, rather than just a couple of people’s very specific antibodies.
Although they give you some meds to try prevent it, there are likely to be side effects that range from common (headaches, aching muscles/joints, fatigue, low-grade fever etc) to rare (anaphylactic reaction, aseptic meningitis) depending on how your body reacts. Your body also reacts to the speed of the infusion so the time could be more like 1.5 hours, or closer to 5 hours each time but most people are about 2.5 hours.
It’s hard to know what to expect before going so you just dive into it and see how you handle it.
I had to get a bunch of blood tests and do a vaccination test to see how my body reacts to confirm what was a 99% kind of diagnosis anyway. Then I saw the infusion room, met the nurses and had more blood taken from me to check my blood type. There was a girl chilling in there with a magazine and an IV in her hand – she seemed pretty comfortable which is good.
The first appointment for treatment was made ‘urgent’ so I was booked in for the following Thursday and went on my merry way.
Infusion day! Thursday 6th August.
On the Wednesday I spent the entire day hydrating. Like, all I did was drink water, coconut water and green tea. This is important as the IVIG that they put into you is apparently the consistency of dishwashing liquid and your body needs all the water it can get to dilute it to help your body and organs handle it and it helps you avoid headaches which are common.
Naturally that meant a night of running to the loo, but I wasn’t taking any chances.
I slept through my alarm after a terrible night’s sleep but luckily my mum was early and I got up and ate a big breakfast with her (apparently the infusion kills your appetite), bought some lunch to eat in the middle of my 5 hour stint in the hospital and headed off.
I immediately regretted my choice of breakfast drink – coffee – even though I went cold-brew with soda water (for hydration :P) as it didn’t help the swarm of butterflies attacking my stomach when we got to the hospital. I must have been pale and nervous looking but the nurses were wonderful.
She put me in a comfortable chair in a spot that mum could stay with me if the other comfy chairs filled up with patients, gave me painkillers and antihistamines and set up my IV. I’ve never had that before so that was bad enough. Then she put some cold solution down it, into my arm and even though it went in the top of my hand, I could feel it in my vein under my wrist. Then she set up the bottle of IVIG hanging upside down like a drip through a little machine, connected me up at a low infusion rate and left me to it.
The side-effects weren’t too bad for me. I felt really quite drowsy and didn’t like having an IV in my hand. Other than that, it was only when she turned up the infusion rate a few times I started to feel a weird ache in my arm and got a little light-headed. Turns out that’s probably because the IVIG is cold compared to my body and it feels uncomfortable to some people. The nurse gave me a heat pack and that seemed to help. So overall, not terrible, although, I wouldn’t recommend it for a fun day out.
It took under 5 hours and almost 500mls of meds in my veins. Weird.
Afterwards I felt fine…
Until I walked for a few minutes, then I realized I was light-headed and tired and had to sit down. I just felt ditzy and a bit slow. Ate some food and went and sat around for the rest of the afternoon with my parents. I had to keep drinking water as I could feel headaches coming on and the fluids were keeping them at bay. The nurse recommended I drink about 1.5L that afternoon/night.
My lips also chapped up which was creepy. It was like I had wind burn without being outside or in the cold, and it only took a few hours for my poor lips to shrivel up.
I also got random aches. Like, in my left thigh it would just ache for a few minutes, then later it was a spot in my tummy, then my other leg. I knew it was nothing to worry about so we joked it was the IVIG going through my veins so slow that I could feel it getting to random parts of my body hours later.
As for the infusion being an appetite killer – yea right! They clearly hadn’t met me.
I had a big dinner that night and slept pretty well, except for waking up in the middle of the night and having a weird moment imagining the thick plasma that had come from other people’s blood squidging through my veins and trying to absorb in my organs. Not really a pleasant thought, so I can understand why people of certain religions and beliefs are funny about receiving human blood products.
The day after I was still shattered.
Just physically tired, and when I moved around too much like going for breakfast and the supermarket with my parents (to stock me up when they went home) I felt so light-headed. Lights were making me feel dizzy and sick, but as soon as I sat down it was fine.
Overall, I feel like I got the not-so-bad end of the spectrum when it comes to how I react to the infusions. There’s nothing great or fun about it, and “how did it go?” is a hard question to answer but I think “not terrible” is the most honest way.
I’m a little nervous about the next infusion in under 2 weeks as they’ll be upping the infusion rate and I’m not sure how that’s going to feel. Eek!
No doubt it’ll all get easier and my body will get used to it so hopefully even less side-effects and fairly quick infusion rate.
In the future there is also a possibility for me to start home infusions, although they take about 1.5 hours a week and you have to prick yourself. I need to wait about 6 months before I can get trained on this if I want to, and it’s not as straightforward as I was hoping it would be.
It could be a viable way for me to decrease peaks and troughs in health and energy that apparently is common in people receiving monthly infusions. It’ll also be good for if I want to travel for more than a month as I can get 3 months of meds at a time.
I guess I’ll just take it as it comes. All I can do now is eat well, exercise and focus on my body and mind and just hope the treatments do their job. It’ll probably take a few months for me to even resemble a normal person health-wise so we’ll just see how it goes 🙂
Sorry for the long post, I’m impressed you got this far!
Watch this space, there may be more exciting CVID updates! Woop!
This is post #1 of 2 following my CVID diagnosis and treatment.
Read post 2 “CVID #2 – Optimism, grief, energy, rebellion & panic attacks” here.
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Hi Charlie. Please can you email me? I so want to be able to link with you. You’re a very good author and we desperately need help writing for cvid patients. Also Jackie Murphy us setting g up a patient led organisation in Australia and definitely needs help and support from able people, even if it is only checking patient literature.
I’ve emailed you 🙂
Loved reading this. I’m also a well cvid er as I put it. Very thoughtful and truly correct piece of writing. Still hate the infusions but 5 years into it now. Good luck!!!
Thanks Dru! Ha, yea they’re no fun. I only have one under my belt. A lot more to go I guess haha.
Thanks for sharing your story. It is so like mine, except that I am 70 years old and have been have treatment like yourself for thew past 4 years and 9 months. My cough had been going on for nearly 4 years and took a lot of my energy away. Although, thank God I have a bit of relief from the cough over the past few months. Mainly I
I have the same condition as yourself. My CVID was diagnosed about 5 years years ago. I have my privigen injections every 3 weeks at the hospital, where it takes about 3 and half hours. I am 70 years old and find like you, my energy level is low. My cough went on for 4 years, but has eased off a bit the last few months. It does however come back at night when I go to bed and sometimes after I eat. This is the bronchectasis. (spellig?) Take care and thanks for your story. Liz Waters
Thanks for your comment Liz 🙂 I actually think I have possible bronchiectasis too (or that’s what the CT Scan on my chest is going to find out). It’s crazy to find people with the same health issues as me!
Charlie,
I hope things will continue to go more smoothly. I read your blog/post start to finish bc like you, my son is not as sick as most. He has multiple special needs but was diagnosed when he was 19, I believe. He had a history of continuous ear infections and had sinus infections fairly often. Everything for him was upper respiratory, never any pneumonia, thank the Lord!
He had tubes in his ears since right before turning 5 which would always be replaced as needed. His infections were attributed to physical anomalies with his ears – small, curved canals and eustachian tubes that remained much more flat, like a younger child’s are, rather than becoming more vertical as he aged so his ears could drain, as typically happens. So basically due to his unusual chromosomal disorder, little was known about that and his infections were written off as I said to physical anomalies.
Finally his pediatrician started growing suspicious and began running some tests. Certain #s were low like WBCs and she also gave him the pneumonia vaccine and then tested him for titers the next month. Of the 13 strains of pneumonia the vaccine is supposed to cover for, his body only produced a slight amount of titers to one of these. She then gave him the pneumonia vaccine given to very young children (babies?) but it only covers about 5 or 6 strains, however the body reacts differently as to how it produces immunity, so she was seeing what happened with this vaccine – nothing, again. She also tested most of the vaccines he had been given, showing few were protecting him. He was then referred to an immunologist.
From what I’ve learned since then, which isn’t that much, he has a much less severe form of CVID than most. Much gratitude to hear that, as his challenges are many already.
He did not do well with IVIG as his cognitive deficits really did not allow him to understand why he couldn’t move around for 4 whole hours & he didn’t want to be connected to the IV (Something he’s had many times as he’s had many surgeries/procedures under general anesthesia, requiring IVs). We had the first infusion done in the hospital to watch for side effects, but then since it went well, the rest were done at home scheduling a nurse to come every 3 1/2 – 4 weeks. This ended up being very difficult because the nurses were not always available within that time frame so it ended up becoming every 5 weeks or 5 1/2, way too often, plus if our son was having a bad day, it was near impossible to have him last through the whole infusion, so the stress was high!
We eventually felt we had no choice but to switch to the subcutaneous infusions. I could not imagine sticking a needle into my son’s stomach as medicine was as far from my field of interest as possible, but I had been thrown into it headfirst once he was born, hospitalized for almost 5 months, the NICU setting, and all the 40-60 dr appts a year that followed. Then my mom became severely ill with a rare, always fatal cancer that she lived with for a record 19 1/2 years so doctors and hospitals, treatment centers became very familiar and I also got cancer, went thru the works and have been 10 years post treatment cancer free.
However, what we had no clue was that instead of one needle there were at that time 6 needles for the infusion! We had to stick 6 tiny needles in his stomach. Oh my goodness. There was numbing cream tho, so he did not feel a thing. And in time, the actual immune globulin med changed and we now use hizentra and he has only 3 needles. They are tiny. He does not need the numbing cream. He tolerates these infusions very well. His take about 50-60 minutes, twice a week. I kind of slacked off during the summer, not intentionally, but am trying to get back on track. He is nonverbal and cannot communicate what he is feeling but I am quite tuned in to his nonverbal communication. He does not seem to have any ill side effects from the treatment.
(Having been through some major chemotherapy, I’d advise to not be too anxious to speed up your treatments. I know the drugs are of no comparison, but there was such a huge difference in the intensity & length of side effects from one of my chemo drugs when it was slowed down. I’d much rather spend an extra hour or so and not have the side effects from that drug torture me for the next two weeks. When the speed was lowered, the side effects were about 25% of what they were before & didn’t last near as long…Anyway, just a thought on the rate change – if you feel something, let them know, bc going fast isn’t always best.
And on the other hand from our experience, I’d much rather be infusing the sub-Q hizentra 2 x’s a week for an hour than doing the IVIG any day. Our son, now almost 28, does not mind these sub-Q infusions at all. He prophylactically takes 1 benadryl and 2 tylenol or sometimes I use ibuprofen. And if he has it later in the day, I often give him zyrtec instead of benadryl as he takes zyrtec daily for allergies. I have the anaphylactic kit if needed with the epi-pens, but we haven’t needed those. I keep a log of how he is each day I infuse – sick? slept well? on any new special meds? – just for myself. It also has the start/finish time, the lot #s from each of the two bottles I infuse – 40ml each time and I write the start/finish time as well as if anything happened during the infusion & how his sites looked afterwards etc.
I know they have come out with yet another way to infuse, a medication that is given either once a month or twice a month – so a lot more meds at once, infused at home, but something that you have to gradually build up to the full dose as it requires allowing a lot more immune globulin to get into the subcutaneous layers. I am not yet ready to try that. If it worked well, it would eliminate a lot of infusions, but I’m just not ready to try it yet!
So, just thought I’d tell you, you’re not alone with being younger and not as severely ill. Our son has been on so many antibiotics thru the years – hundreds, sad to say. He is allergic to two classes now and unfortunately seems to be getting more episodes of GI distress ( severe stomach pains) with more& more types of antibiotics which scares me. He can be fine on an IV antibiotic but the oral can be a huge problem. He has not had near the number of ear infections especially these past years, but just happens he is dealing with an ears/sinus infection now.
Good luck!!
Hi Lisa, thanks for your comment! Wow it sounds like you and your son have been through a lot – I wouldn’t wish to compare our situations, but I am definitely glad that you’re better and in terms of CVID he is at the milder end and you can relate to my post. No doubt with a Super Mum like you he’s getting the best support he could get as well. My parents are amazing too and my Mum is going out of her way to help me and support me – it makes such a huge difference for someone with health issues! It also must have been much harder to diagnose your son’s CVID with his other health issues so it’s great news that they caught it at a younger age 🙂
Thanks for the info about the different infusions as well – especially about not worrying about the speed of it, that’s a good point. The Sub-Q does look quite intimidating but judging by the way people talk about it I feel that I’m likely to get onto it as soon as I can (the nurses said 6 months – maybe they do it differently in NZ?) and also hopefully get over the idea of having to put needles in myself pretty quickly!
All the best to you and your son 🙂
My husband was diagnosed after years of lung infections and pneumonia and pretty much everything you’ve mentioned. It’s a life changer, no doubt about that! beung as positive as you are is the best way…
I learned to do his IV at home, every two weeks and we just blend it in to our lives…instead of having to go to hospital every two weeks. We’ve been doing it for 5 years now. He was a hill walker, outdoor mountaineering instructor in the army, black belt karate, boxing, rugby, you name it, he did it… and was pretty fit but had to give up a lot due to pain from arthritis, related or not, we’ll never know. He’s 49 now and retired early, mainly due to fatigue and latterly being an HGV driver and not being able to stay awake at the wheel on bad days. It’s not all bad though; gently paving himself is the answer. Walking and enjoying travel when we can. He may have passed that bastard elusive IgG deficiency to our daughter: she’s experiences some measure of symptoms but not enough to warrant treatment, yet! Such is life, we didn’t know back then that he had anything that might be passed on. We’re still doing what they call ‘watchful waiting’ with her. His poor dad (since passed) blamed it on ‘that bloody army stuff’… and it was inherited in his ‘onion’, so to speak. I knew he had CVID before any GP would refer him, but that’s another long story, it’s my psychotherapist’s nature to delve deep and I had researched all his symptoms several times over the years. I was poo poohed by a senior consultant when I suggested what he may have and dismissed and my poor man suffered more unnecessary lung scarring from further infection in the process. The consultant has since retired. I like your blog, it’s a fresh perspective for any newly diagnosed person and I wish there had been something to read like yours back when I was in a bubble with the jumble of it all in my head. It affects the people that loves someone who has CVID. But it’s the people that have to deal with infusions for the rest of their lives that need more support and awareness from others.
I just wanted to say to for your next infusion it’s always good to take an anti-histimine on the day, it helps keep headaches, aches and pains at a minimum. Warming yourself before the treatment and letting the treatment get to room temp before you infuse cuts out the ache from it going in your veins too. Good luck and keep blogging 🙂
Hi Karen, I’m glad you liked the blog – I hope it can be helpful for people who were in my position a month ago (and their families/friends). It sounds like your husband is making the most of his situation and is lucky to have you around! 🙂 It definitely does sound like positivity and optimism are the main things really. And thanks for the tip – I think warming will definitely help as the cold IVIG was so uncomfortable for me. I’m sure I’ll keep writing blogs and posting them on the CVID page if people respond positively!
Thanks for the article. It sounds so familiar to what went through for years. I was diagnosed 15 years ago after spending every month in the hospital with some illness. I have my infusions at home every two weeks and it takes approximately 5-[6 hours. It was important to find others with the same illness as I always felt it was only me. Stay as healthy as you can and thanks again for all your information.
Fantastic blog! I’m almost 30 and was diagnosed with specific immune-deficiency, but your symptoms and the effect of the illness resonate with exactly how I feel! Currently on preventative antibiotics while they’re monitoring whether I’m sick enough to get bloods. Thank you for sharing this – I was expecting your condition to be absolutely terrible and disabling, I’m glad to see there’s hope! Like you, I like being active, travelling and love my career!
I was diagnosed in 2011 in my 50’s. I was diagnosed with asthma when I was 21! But now I know it was CVID. Since starting tx. my asthma is completely gone. No more nebulizer tx., no more ongoing prednisone TX. my Dr. would give me 100 tabs. of prednisone 20 mg to medicate myself so I could breathe. Finally after seeing a pulmonolgist, infection control doc, internal med doc. nothing was helping my Dr. order a blood test to check my IGG level. It came back very low so was able to see a hematoligist and start tx. the same day. 4yrs later I am very healthy and doing well. I walk 3 miles a day just started that this year again. I had to go to PT for several months just to get my strength back in the beginning. I am receiving Gamma globulin tx. every other month now and my levels are staying up. I keep praying that a permanent cure would be found but nothing yet. I stay very active, but I have not gone back to being a nurse in the hospital and prob. won’t. Good luck to all who have this disease.
[…] is CVID post #2, following on from my last (pretty long) CVID post #1 where I talked about being sick all the time, diagnosis with a primary immune deficiency and my […]
Oh My God. I feel like I am reading my own life. Charlie, I hope you still check this page… How did you go about getting a referral? Just through your GP? I have the same thing with the FB posts, reminding me that I’ve been sick for YEARS with no break. Seeing this page has actually given me hope.
I am so sorry Alison, I didn’t see this comment! I’m so sorry to hear you’re struggling with your health and I hope you get some answers soon!
What you can do is request a blood test from your GP to check your immunoglobulin levels which should give you the fastest answer. The other check they do is take some blood, give you some vaccinations, then check your blood again in a week or so. People with certain Primary Immune Deficiencies don’t take to vaccinations (they don’t work) so they can test that too.
Good luck in your hunt! Feel free to email me on [email protected] if you want to know anything else – it might be faster in case I miss another comment x