Treatment Options - CVID & Lung Inflammation

[derrja]

Hi, I'm a brand new member, diagnosed with CVID last year and commenced immunoglobulin infusions in December.

My CVID comes with, amongst a host of other things, inflammation in my lungs which has gradually gotten worse over the past two years. This has left me at point where I was constantly fatigued and where minor physical activity would completely exhaust me. It has also deeply affected my family's quality of life as I've become more dependent on my wife doing things as I become incapable of doing things that I previously could do.

As a result of this my haematologist prescribed prednisolone in March which has had a massive impact and has turned me into the Duracell bunny, I can just keep on going now . My quality of life has jumped immeasurably as has that of my family. It’s also helped other aspects of my condition by reducing the size of my spleen which seemed to have been causing persistent back pain and restricting the amount I could eat.

My hope was that I would be left on a long term maintenance dose in but having seen my immunology consultant recently he wants to take me off them and only use them when needed. Having had a couple of courses of prednisolone over the past few years to treat other conditions and knowing the effect they have had on my lung function I know l that coming off them will cause a relapse of the lung inflammation and put me on a rapid downward trajectory. If I can avoid it I don’t want to be constantly going through a cycle of feeling rough, feeling brilliant, feeling rough, etc as the treatment stops and starts. I just want to feel ok-ish most of the time.

I know that continued long term use of prednisolone increases the risk of a host of nasty side effects but there don’t seem to be many alternatives and I as can mitigate a lot of those risks through lifestyle choices I'm willing to accept them. Hydroxychloroquine was also mentioned as an alternative but appears to come with a risk of blindness which is a risk I’m not sure I want to take.

Does anyone else have similar symptoms? If so what treatment options have you received? Is there anyone out there who is taking a long term maintenance dose of steroids and if so what steps are you taking or have you been asked to take to minimise the side effects.?

I'm due to see the immunologist again in July and want to go armed with as much information as possible to support staying on steroids (or about other options if they look more palatable) so any views or experiences would be greatly appreciated.

Thanks


Jason

[glowing]

Hi Jason.

Firstly, welcome to the forum. I can't help in the way of supporting you for staying on steroids. I'm just replying with my own experience of over ten years of prednisolone treatment. Up until I was diagnosed with CVID, in August 2011, it was a struggle to stay well and I spent most of the time on prednisolone, antibiotics and a host of Asthma treatments. Just as you described, each time the course of steroids finished I had, if I was lucky, usually about two weeks before my breathing became bad again. Things improved when I started immuno treatment. I'm only ill four or five times a year, when I have to have steroids again. Whilst on prednisolone my arthritis is far less painful and it's great to walk around without wheezing. What I hate about them is they make me hungry, bloated and I've put on weight and struggle to lose it. I agree the extra energy is great but trying to sleep soundly is difficult. My skin is very thin and scarred because it tears really easily. Last year my GP arranged for me to have a bone density test because of the dangers to our bones. I was very relieved that my bones are okay.

I'm sorry that my experience with steroids isn't any help to you but I wish you good luck in finding an appropriate treatment.

Glo

[aryani]

Hi Jason,

Tough choices. I can only tell you my own experiences. I'm diagnosed with specific antibody deficiency and amongst other things I've got lung problems as well. Getting a proper diagnosis and receiving IVIG, has improved my quality of life enormously. It has helped with my lung problems and lowered my use of corticosteroids.

I'm taking corticosteroids about 28 years now. Like Glo, I suffer from the side-effects: hangry (and not quite the figure of a model), weight gain, thin skin, retaining fluids, unwanted hair grow, stomach problems and beginning osteoporosis. I'm complaining the most about the weight gain and the unwanted hair grow. More serious are the stomach problems (other medication did not help either, so I'm not sure that is only due to the corticosteroids) and the osteoporosis. I'm taking medication to counter those side-effects. And, as you write yourself, lifestyle choices can help counter the side-effects. But....given the choice between no corticosteroids or a daily dose, I prefer the latter! Without them, I'm constantly ill, tired, etc. Not a roller coaster, but a downward spiral. Even now, I've got a few courses a year. But, the total amount of corticosteroids is less then when I take them only if needed.

So, my experiences are a bit different from Glo. What's wise and what's not? I hope you will get proper treatment.

Aryani.

[derrja]

Glo and Aruyani thanks for your responses, sorry to read that you’ve both had what sounds like pretty poor experiences with steroids.

After extensive consultations with Dr Google I don’t seem to have many options. I think it will just be question of trying to agree a plan with the consultant that minimises the peaks and troughs and also try to find the best balance between dosage and benefit.

Thanks again

Jason

[aryani]

Hi Jason,

Maybe my English just isn't good enough..... (either in writing or in understanding your message). Yes, I don't like the side-effects. But although I'm using them already 28 years, I do realise that the side effects are relatively harmless. My quality of life has improved enormously from a daily dose! And, yes, it is balancing between possible side effects and benefits.

I hope you will find a strategy that works for you!

Aryani.

[mothergoose]

I know this post was written a few months ago now.
I too am having lung problems/breathing problems.
I have been testing positive for pseudomonas for the last 3 years. At times it has made me very very sick, other times just sick enough to make me miserable.
I would get treated for it,(I don't think ever properly) symptoms would improve, then a couple of weeks later the symptoms would return.
Mostly I cough and choke a lot, I bring up 2 types of phlegm, one thicker white one that is constant. I think due to this I am exhausted, and it heavily impacts my life. All the coughing and clearing of my throat often gives me really sore throat, but Dr.'s can never see anything.
After each new out brake of pseudomonas, a couple of weeks later I cough up chunks sticky and thick like glue, that usually are round, darker in the middle, sometime almost black, then getting lighter in color in rings around the dark center, till it was red, then pink and lighter pink, and always ended in a ring of clear to opaque stuff. These range in size from eraser head to the size of a quarter. I can always tell when my body is trying to get one of theses out of my lungs. Sometimes it takes days to get them out they are so thick and sticky. Often when I am trying to get theses bits out my throat is extremely sore, and the throat pain goes away almost as soon as I get the bits out. When tested, they always tested positive for pseudomonas.
The only time steroids helped this was when I was given them with IV antibiotics.
It is thought now from having pseudomonas this long, that I may have a type of Cystic Fibrosis from it. My Dr's are hoping that now being on IGIV that my body will be able to kill this bug off it's self.
My symptoms did improve the first 5 days after my 1st treatment.
I don't feel like I have pseudomonas or at least that it is activity growing at this point, making me sicker. But my breathing has been much worse, the choking a bit better, but now I have spells when I can't breath in and cough and can't catch my breath, I rarely had this before but I think I just started breathing shallower and shallower, now that I make myself to breath deeper and do breathing exercises to loosen the phlegm, I think that is what has changed my breathing problems.
Hoping my next treatment things will improve move.
I don't like steroids, I find they only help when I am really sick and they give me a bit of a pick me up.
Anybody else had anything like this happen to them.
I am slattered to see a respertoryoligist, have another MRI of my lung, and possibly have a biopsy of my lungs.
Any ideas would be appreciated.
Mothergoose

[aryani]

Hi Mothergoose,

People with Cystic Fibrosis and are colonized with Pseudomonas are usually treated with a daily dose of antibiotics (in some form, I'm not familiar with the brands in the UK). Maybe that is an option, even if you do not have Cystic Fibrosis, but opportunistic infections.
To my knowledge, there is a test for Cystic Fibrosis. Have you had that test already? Otherwise, I would recommend it, because Cystic Fibrosis gets an complete different kind of treatment than a Primary Immune Deficiency.

Best Regards,
Aryani.

[mothergoose]

I have PID so that is why I am being treated for that, they figure I never got over the lung infection with multiple different kinds of treatments because of the PID, and possibly a defect in my lungs, that allows this bug to present like Cystic Fibrosis.
I have had many many other hard to cure infections along the way, so it is not just this bug or my lungs causing me problems.
I am waiting for dates for a CT scan and to see a respiratory ologist to see what she thinks. She will decide what tests she wants to do.
It is just hard to wait when you are so close to maybe getting answers.
Also to add to the problem the disease specialist sent a referral for a CT scan and then went on holidays for 2 weeks, the CT place said he never sent the referral request or I would have had that done by now.
As far as Cystic Fibrosis gets an complete different kind of treatment than a Primary Immune Deficiency, they already have it figured out nothing of my treatment will be straight forward.
Thank you for the information
Mothergoose