Cvid diagnosis and scared

[DaisyBright]

after 4 years of poor health I have just been told I have cvid and need Ig therapy
i have no igA and very low IgM and IgG, no chest or lung problems, all sinus and ear infections, spleen etc all fine, I have an under active thyroid and inflamed sore ankle joints plus terrible fatigue 
i feel like I have been hit by a bus.
To be told I will not get better, I will need treatment for life, a return to my previous life/career/energy will not happen ... I am struggling to take it all in. 

I am in my late 30's 

I have so many questions so any advice or info would be great as there seems so little in the UK and my consultant immuno gave me nothing to read/direction , cvid does not even feature on the nhs website from what I can see

I have a very well known fear of needles, blood tests are a battle, the thought of inserting needles in myself is overwhelming - how big are they? How painful is it? What can I do to overcome this ? I have chosen to try subQ, IV and a cannula every few weeks is to much to consider for me.

can I travel with subQ, can you have extra one week if you are going on holiday or do you have to take it all with you? 

How long does the infusion take if it is weekly? 
Are there any side effects? 

i know IG therapy will help prevent infections but will I feel better day to day? I have fatigue daily, I struggle to sleep, I have sore swollen ankles daily, i feel really weak on some days

My consultant said his other cvid patients dont present with fatigue and this is not usual , reading these forums is seems common? 

Is this disease progressive - if I manage to keep infections at bay with Ig therapy and antibiotics , is this it or will this get worse? And if it is progressive what can I expect in 5 and 10 years? 

When I read about cvid all I see on the page is an increased cancer risk. It makes me scared and keeps me awake At night, I recently lost a good friend to cancer. Should I be worried, is it true. What is the risk? Is it for all people with cvid? Some forums say it is a matter of when not if? 

I have poor B cells they don't mature so are poor quality - is this the link to B-Cell lymphoma ? 

What screening is given to cvid patients in the UK to be vigilant about this and ensure if I do get it it is caught early? 

Does this effect fertility? I have not had children yet 

Has anyone looked into alternative Medicine, Chinese medicine, holistic therapies - any success if so? I have never used any of these before but I will try anything?

if my bone marrow makes B cells and my B cells are poor quality does this mean I have a problem with my bone marrow? Is a bone marrow transplant an option ? 

Thank you for any support, advice, direction for further reading, help - I have never heard of this before, I have never met anyone with an immune system problem 

thank you 

[s162216]

Hi DaisyBright and welcome to the forum,

Don't give up on yourself just yet! At first it can be a lot to take in, but it doesn't mean that your life as you know it is necessarily over. CVID is like any other long term condition, it can take a while to get right, but once it is right then nothing says it has to rule or ruin your life. Take a step back and try to think it through, not everything is doom and gloom just yet!

I strongly recommend looking on the website of Primary Immunodeficiency UK (PID UK) who are a UK wide charity that support those with primary immunodeficiency conditions. They produce a very good wide range of information materials, and I'm sure you'll be able to find what you need through their website; if you have any other further questions then you can contact them and they will do their best to help with your query. I'm also on their patient representative panel, so if you can tell me the name of your hospital then I can arrange for PID UK to send out information leaflets etc to be given out to new patients (I'll send you a private message so you can tell me it confidentially). CVID is a pretty rare condition (about 1 in 20,000 to 50,000 people in the UK) so expect to have to explain it to medical/nursing staff quite a lot as hardly anyone has ever heard of it in my experience; I'll get in contact with Susan Walsh at PID UK to see if we try the persuade the NHS to put a page about it on their website for future new patients.

As you've chosen to do subcutaneous IgG then there's nothing really to it as all you need to do is put the infusion sets in subcutaneous fat and infuse the IgG in. The needles are very small (about 6 to 10mm usually), usually pretty painless and easy to insert; you don't need to worry about getting it in a specific place etc like when having it IV or taking blood, just in the right area. Your immunology team will make sure that you are well trained and know what to do before you'll be allowed to do it yourself at home. Although the advice you get will differ depending on your own medical needs, generally most people can take a double dose of IgG before going on holiday or have a dose of IVIG in hospital beforehand (as that covers you for 3 weeks) or take their supplies with them if it is a longer break away. A lot of people have done that, even taking it abroad on flights and it's generally not too difficult to do.

The infusion time will depend on your dose, but most for most people they take about a couple of hours. Side effects are usually very minor, e.g headaches, feeling cold etc and can be easily avoided or reduced; as subcutaneous isn't such a big "hit" of IgG in one go like with IVIG then side effects are usually quite uncommon and minor in comparison. Whilst more serious side effects are possible like with any medication, they are extremely uncommon (to put it in context, at the infusion clinic I used to go to they had only had a single person having a major reaction in the last 10 years).

CVID is a highly individual condition, so I would recommend taking the experiences of others with a grain of salt as you'll find your own experiences may markedly differ or be similar. Whilst some people do suffer from fatigue or tiredness, not everybody does. Same with infections, some suffer from birth with recurrent infections that take a long time to go, others might go for many years before they start suffering such problems. For instance I'm in the latter category, I naturally have hardly any IgG but having the IVIG I don't suffer from very many infections at all currently. Personally I don't generally suffer from fatigue or tiredness, although I do sometimes and I did notice after a few months on IVIG that I felt a lot less tired than I did before. A lot of people do report feeling better after starting IgG, but it usually takes a few months to start making a noticeable difference so don't be surprised if you don't get an immediate effect.

CVID as a condition is actually a blanket term for many different immunodeficiency conditions that have similar clinical symptoms, but different underlying causes that are currently very difficult to distinguish between: whilst some people might have B cell production/function problems, others might have defects in the antibody production chain or with T cell production/function. Unfortunately because of this CVID is not treatable by bone marrow transplantation (it has been tried a few times in the past by a few centres worldwide, but has generally not been successful and the risks are seen as outweighing any benefits currently). At the moment the genetic causes are unknown in about 90% of patients, and unfortunately unlike other immunodeficiency conditions like SCID it's not a single mutation condition so it's unlikely a gene therapy cure will be developed in the near future.

Regarding complementary medicines, I don't have any personal experiences. Before taking any such treatments though I would recommend talking with your GP or immunologist to see if it is medically suitable for you.

Whilst it is known that CVID does present a slightly heightened risk of developing some cancers, it doesn't necessarily mean that you will. Your immune, haematological and general health will be regularly checked through blood tests every few months, which will help flag up any other potential medical problems. I would strongly recommend that you talk with your immunology consultant about your concerns; don't be afraid to ask them questions and if you don't feel your question has been answered then say so and ask them to explain it again until you do, don't be afraid to be forceful. I would also contact PID UK (see www.piduk.org/contactus/) with your questions that you've presented here as they have an excellent team of people and a world class medical panel who can help to give the most appropriate answers. Go on!

I had never met anybody with a primary immunodeficiency before either when I was very un-expectantly diagnosed, so I know exactly what you're going through. Despite being a very biological science orientated person, with the exception of severe combined immunodeficiency (SCIG) children who have to be kept in a sterile bubble as they have no immune system, I'd never heard of primary immunodeficiency. It was a learning experience, but it all worked out in the end for me and I'm glad I found out that I had it earlier than most people. My CVID story is actually on the forum in the member stories section, see primaryimmuno.proboards.com/thread/661/cvid-story-sam ; it is a little out of date now, but still gives the general picture.

Unfortunately we're not getting as much activity as we used to on the forum these days, if you want to find other patients to talk with then the facebook group "UK PID Patients" is very active and may also be of help. You are of course completely welcome to stay on the forum, we welcome any activity and hope we can be of some help as we have members from all around the world who each have their own experiences.

Thanks and if you need anything else then just post it!

Sam (Admin)

[gillie]

Hi Daisy33. Sam's already said most of what I'd have told you. Your experience of symptoms pre-diagnosis sound very similar indeed to mine. I hope it'll encourage you to know that I was diagnosed in 2002. Neither of my daughters has CVID. It took me several months of infusions to feel a whole lot better, so don't get downhearted when your treatment isn't a quick fix. The immunoglobulin that you're given will have quite a bit of bug mopping-up to do in your body, but after its done that, the infections you've been experiencing should be a thing of the past.
On the subject of cancer, we are more prone to B cell lymphoma, but it's curable. I've had it. it took the NHS nine months to pull me through, but I'm here still and I'm completely cured. The treatment I had is so new that I don't think that there are statistics for it yet, but there will be.
Every good wish,
Gillie.

[DaisyBright]

thank you for taking your time to help me I have felt so isolated by my illness and more recently my diagnosis.

Congratulations on winning your cancer fight I have seen first hand the strength it takes to fight it.
Did you have regular monitoring by the nhs to keep an eye out for it and catch it early? If so what should I ask for?

If you were diagnosed in 2002 I guess you are my future .... My worry is that this is progressive? Do you feel better, the same, worse than you did 10 years ago, have other symptoms arisen?
What choice of Ig therapy did you make - any advice? I have been told to go away and think which route I would prefer but there is no info, support groups, etc to go to in order to are this decision from the nhs, I feel like I am in the dark being asked to make decisions without the facts.

I asked my immunologist about the cancer risk and whether this disease was progressive, he was very dismissive, I am not sure if this was to stop me worrying or because they don't know? He said people with my set of results (failed vaccine test, baby/teenager B cells only, no igA, low levels of IgM and IgG, no lung/liver/spleen problems, under active thyroid) tend to not have issues, he said I would be at the lower end of "the scale"- is is true?
If our symptoms are the same ore-diagnosis are you proof this is possibly not the case and I should push for regular testing?

Did you have children after your diagnosis and whilst on treatment?

Finally do you do anything else that has helped improve your health and immune system - diet (my friend drank veg juices daily to boost her immune system whilst having chemo and thought it helped? ) tried any alternative medicine or therapies?

Thank you thank you thank you

[DaisyBright]

Sam thanks for your info I will absolutely look at the PID site if you recommend it I am always dubious about reading things online!
If I get any good info I will post for others to read.
Sorry I sound so gloomy I am just feeling isolated and overwhelmed at the moment - glad to know what is wrong with me (I have been made to feel like a hypochondriac for the last two years by some people, been told I am mentally ill and been asked if I have HIV) so I can try and do something about it, but scared about the future.
I have so many questions and no one to turn to who will understand.

Good to speak to someone who does subcutaneous IG......so once I get over inserting the neeeles...... I have read that people can get bid red lumps where the fluid is sitting under the skin which is painful and takes days to go down. Is this true?
Do you use the same area every time ? If so it is painful from repeated injections or effect the skin?
Do you have any side effects when you inject?

Thank you

[gillie]

Hi Daisy. I haven't suffered any progression of my immune deficiency. If anything, I feel a great deal better than just before I started on ivig in 2002 because I was feeling so rotten: no energy, two recent really bad ear infections which led to me having to wear hearing aids, but it's the chemo that's caused me problems in the recent past, not CVID. My treatment had to be so intense that it effectively killed the sciatic nerve below my knee on both legs, so my poor brain's never sure where my feet are! I'm so glad to be alive that I don't let things like that get to me any more. I'm fine. I have my immunoglobulin every fortnight at an outpatients department where I know all the staff, get brought cups of coffee during the 2.5 hours it usually takes, and I feel comfortable and well looked after. I tried subcut, but it didn't work for me. I see my immunology specialist 2x a year for a catch-up and the rest of the time I see my G.P if I feel the need. It's really helpful to me that I'm prescribed a one-a-day antibiotic which I take for 6 weeks before I swap over to a second one for the next 6 weeks. On the subject of so-called immune system booster, I'm very sceptical of all of them. I ask myself, if I haven't got a functioning immune system because my body's unable to make the right cells, is a change in my diet or having veggie drinks going to be able to make my body suddenly wake up and start producing the missing things? No. They might make a significant difference if my diet previously was poor, but only to my general well-being.
I hope that you're getting a clearer picture of where you are and what you can expect in the future. Good luck.
Best wishes,
Gillie.

[s162216]

Apr 27, 2016 9:33:22 GMT DaisyBright said:

Sam thanks for your info I will absolutely look at the PID site if you recommend it I am always dubious about reading things online!
If I get any good info I will post for others to read.
Sorry I sound so gloomy I am just feeling isolated and overwhelmed at the moment - glad to know what is wrong with me (I have been made to feel like a hypochondriac for the last two years by some people, been told I am mentally ill and been asked if I have HIV) so I can try and do something about it, but scared about the future.
I have so many questions and no one to turn to who will understand.

Good to speak to someone who does subcutaneous IG......so once I get over inserting the neeeles...... I have read that people can get bid red lumps where the fluid is sitting under the skin which is painful and takes days to go down. Is this true?
Do you use the same area every time ? If so it is painful from repeated injections or effect the skin?
Do you have any side effects when you inject?

Thank you

Hi,

Don't be afraid to sound gloomy, everybody goes through it when they find they have something they don't understand at first and are scared. With time as you understand more you'll start to feel more confident about yourself.

PID UK are actually currently releasing a series of additional leaflets for world Primary Immunodeficiency Day, so I would check here to see them: www.piduk.org/resourcesforpatientscarersandprofessionals. All of their resources are reviewed by the patient and medical representative panels for accuracy and in my opinion are very good in getting the information across in a simple yet informative manner. If you have questions then don't be afraid to contact them as they are always there if you need some help. If you have Facebook then that group I linked to above is very good for getting together with other UK patients.

I don't actually do subcutaneous myself (I do it IV), but I do know many other people who do it by that method and I do have a subcutaneous insulin pump which works on a very similar principle (but remains attached to me all the time unlike with immunoglobulin). It's not difficult to insert the needles, and whilst you will get some lumps under the skin where the product has to absorb in they should go down within a day or so - subcutaneous infusions are inherently like that as they work by the infused product going in the subcutaneous fat and then slowly being absorbed into the venous system, imagine it almost like having a bucket with a small hole in the bottom. Generally you'll try to rotate infusion sites to avoid overusing the same area too much; as I said above though you'll get extensively trained in it before they let you loose with it yourself and know you are confident in what to do. I'll try and see if john (our other admin) or glowing who both do it SC can pop in to comment.

The main advantage IgG of subcutaneous over IV is the reduced side effects from the lower doses and the more constant IgG levels from the more frequent dosing so you don't get the lower levels before each infusion like with 3 weekly IV (which can cause tiredness before the infusion, I certainly get that for a few days before my IV infusion).

Thanks,

Sam.

[jeanine49]

I've been treating for CVID for 18 months after being plagued with upper respiratory infections, sinus infections, bronchitis, pneumonia, etc. for the first 7 months I did the IVID treatment, once a month at my Immunologist's office. It took 4 hours and I had terrible migraines, nausea, fever & was very ill the next day. This was a problem at work , because I'd miss 2 days & felt drained for @ 5 days before. I would take Prednisone, Tylenol & Ibuprofen before & prescribed migraine meds afterward. I opted to try subcue weekly treatments at home. I had a fear of needles also. I've been doing this treatment for 10 months now. I don't get headaches or feel bad after treatment; just ready for bed & I feel a little warm. I trained for 8 weeks at my Dr's office before doing at home. It didn't take long to get over the needle fear. You have to do it, so knowing that helps. I use five 26 gauge needles & they aren't very long. There are various sites you can use. I've used my stomach but I prefer the sides of my thighs. I alternate every week. I've never had any red bumps. You do get 1 or 2 lumps from where meds go in, but they're gone in 24 hours or less. Depending on how many sites, needle gauge & your weight, it usually takes 80-120 minutes. I have only had 2 upper respiratory viruses since I've started. On the down side, I do not feel that much better. I am exhausted all the time. I see a Pulmonologist & I use a CPAP for oxygen at night. I've quit work and moved from TX to CA where my daughter can check in on me, especially when doing the infusions. I am extremely bloated, gaining 10-12 lb since starting treatments. My immunoglobulin levels remain good; I just have no energy. I am now going to be tested for liver & kidney problems & also going to see a rheumatologist for a possible immune attacking arthritis (not Rheumatoid). So I believe the CVID & treatment has led to additional problems but the CVID treatment is working & not everyone has extra problems. Try to stay positive. It's hard but taking your mind off it helps. I garden a little, sew and read, but only for a short time. When I go out, 2 errands are my max & I wash my hands thoroughly as soon as I get home. Hope this helps some. I will keep you up to date on my next tests.

[shannon]

Hi Jeanine
From what I've been reading, on line, in this forum and in info given to me by my Dr, it seems that virtually everyone who gets the IVIG causes all sorts of nasty side effects. The subcu is much better. My Dr put me directly on the subcutaneous one at 10cc 6x per week. The needle is very small and I've not had any real problems other than itching at the injection site and maybe a bit of redness but it goes away in an hour or so.
It might be that you need to inject less but more often. I know it means more needles but if you feel better it may be worth it. Maybe discuss it with your Immunologist and see if there's a way to work it out.
I've been on this only for 3 weeks and it's been starting to work. I have more energy than I've had in years. I'm finding that I don't mind the needles so much because I feel like I have some control of this crazy situation with the injections.
Keep at it and maybe you can adjust the frequency and amount to a level that doesn't effect you so much.
Take care!

[mali42]

Hi Daisy
Now you have been diagnosed and after you get on an established treatment system your health will improve, it will take a while to get your mind round your diagnoses and I remember thinking what did I think about before cvid :-)

I have been on infusions now for 12 years and my health is about the same as when I got better after starting ivig which is pretty good considering my immune system was virtually zero.

I am going to Australia later this year for 3 weeks and 2 days and plan to go for longer at some point because my youngest has emigrated. I have found out it is doable because I can take my gear with me but I need to read up a bit before I do that so holidays should not be a problem.

Malcolm.

[twinmom]

Wanted to reply to Jeanine49

Hi Jeanine

I am also in California (Sonoma County) and wondered if you were nearby to see if we could possibly share info about any local groups, doctors, etc. California is huge, so you could be far but just thought I would ask. Just did my 3rd infusion ( I do six sites every other week ). I've had so many other diagnoses before I finally got diagnosed with this. This seems to be the most accurate although I seem to also have other maladies probably due to the CVID.

Anyway, if you do reply, Jeanine, maybe it should be a new thread.

Thx.

[Deleted]

Daisy,

I am a 33 yr old female, diagnosed as a very sick child with a condition that no longer exists and now, just sits under the CVID umbrella. I remember when doctors thought it was avant garde to prescribe liquid iron (yes, you needed a prescription back then) but now have the world's best general physician with a perfectly appropriate obsession with endochrinology that I feel saved my life. I have had a long fight with bad veins then a wonderful portable catheter that lasted me ten years. I went from powdered immunoglobulin IVs that took 14 hrs, to liquid glucose based IVs that lasted 10, then 8, then 6, then 2, subq bi-weeklies that took about 4 hrs and now, to working very hard to avoid needing therapy all together. I remember being outside a doctor's office when I was 9 yrs old and hearing him tell my mother that I wouldn't make it past age 21. No doctor will tell you about progression because our bodies are organic and will change or adapt as the years go by. The question I think you ought to be asking is: what can I do to support the health the doctors and my loved ones wish for me? How do I best implement change to enhance my treatment? What is available to us now is amazing! I finished enough schooling to be a teacher, I have three lovely and HEALTHY children and am able to work from home. If wishing to speak more, don't hesitate to PM me,

Melissa