pattis - CVID New Diagnosis

[pattis]

I am a newly diagnosied CVID patient.  I have so many questions!  when I was diagnosed this week, I was kind of in shock and the doctor asked "do you  have any questions" and I couldnt think of much!  Now after reading alot about CVID, I do  have questions.   I was referred to a pulmonologist by my GP because my asthma was out of control.  For the as long as I can remember, I have had multiple respiratory infections one after an other all winter and early spring.  Going back to childhood.  Then 8 years ago, I was diagnosed with asthma which kicks up big time when I get an infection.   so I have asthma too all winter and early spring.  My GP referred me to a pulmologist to treat my asthma, and my pulmologist started wondering whether my immune system was compromised.  I got back my first blood tests and he showed them to me and I didnt understand much. Some of the levels were with in normal, but two were "low"  He said that indicated CVID.  He will run more blood tests (the same panel) in three months to confirm the diagnosis.  He did mention that I could get infusion therapy but said that it was expensive and maybe my insurance wouldnt cover it (it does since I have had severe asthma for so long) and perhaps "I don't want another person's antibodies".  Well, I think I DO want antibodies, so I would be glad to have what I can get - Im very open to the infusions.  Im VERY tired of being so sick every year.   Im 60 years old.  I want to ask whether the Ig infusions really work?  Do they really help people not catch EVERYTHING all the time?   I read that CVID is a genetic mutant and may be  hereditary.  My 26 year old daughter is also "sickly".  Should she be tested?   Does having CVID mean that a flu vaccine or pneumonia vaccine (that I get every year) will be ineffective since I  may not get antibodies from it?   Does CVID mean that  my childhood vaccines are and have never been effective?    What if any thing can I do to make my body stronger so that I don't get infections and if I do recover more quickly?  

[glowing]

Hi Pattis. Welcome to the forum. My story is similar to yours. It took over ten years of being constantly ill before I was referred and diagnosed by my immunologist because my asthma and bronchitis failed to respond properly to any treatment. I've been on replacement therapy for nearly four years. I started on intravenous infusions every three weeks, which I had to attend hospital for. About two years ago I began weekly infusions at home, which I prefer as I don't get the peaks and troughs, plus the headache and tiredness I felt after each three weekly treatment. Both types have kept me well though, give or take a small infection once or twice a year. For me the treatment has changed my life. I still have breathing problems from asthma and bronchiectasis but have not been hospitalised for infection since treatment began. It took months before I felt really well, but even the bad times weren't as bad as before. I was told that it takes time for your body to adjust and your immune system to clean up.
I would suggest to your daughter to get tested, as I did with my sister. My sister doesn't have cvid but she has bronchiectasis. A test will at least help to find out why your
daughter is poorly.

Please keep in touch and let us know how you and your daughter get on.

Glo

[hlh]

Welcome.

It is great that you finally found a doctor that took the time to think about your situation and started doing the right tests to figure out what is going on. It seems from this board that many people... like you... go undiagnosed for many years and suffer with infections. Finally you know what is going on. It also took my father awhile to get diagnosed, and it took years to find a doctor interested and experienced enough to help.

First, I strongly recommend that you look for a local Immunologist to help do any additional testing you may need and who will be the person to follow you long term. These doctors are the best people to prescribe and recommend IVIG and to recommend the appropriate vaccines you may need. A pulmonologist is a helpful specialist as well, but an Immunologist is also needed. We have found that it can be challenging to find this specialist that works with adults with Immunodeficiency. Start by asking your Pulmonologist, and searching the websites of the best academic hospitals near you.

Yes, IVIG may be what you need if you have CVID with frequent infections and worsening asthma. The fact that your pulmonologist is suggesting that maybe you "don't want another person's antibodies" tells me that this is NOT the right doctor to be managing this problem though. My Dad's doctor wanted him to start on IVIG immediately once he knew his diagnosis. This is because he understood his risks of infection. My father is 72 and we know very well that infections become more and more dangerous the older you are and you are more likely to wind up in the hospital.... which is also a dangerous place full of bacteria that you might pick up. I am also a little worried that your asthma has been getting worse because your recurrent infections have been affecting your lungs. You will likely need a high resolution chest CT scan to see if you have developed brochiectasis, which can happen for people with CVID who have frequent infections.

To answer your questions, yes the infusions do help decrease the frequency of infections for most of us. It will be important for your daughter to tell her doctor about your diagnosis for the doctor to keep it in mind, and if she begins to develop frequent infections she should be tested, but I don't believe that there are general recommendations that all family members should be tested. That is because for most of us it is not a medical problem that we simply inherited from a parent. So similarly, we don't simply pass it on to our kids.

For some people with Immunodeficiencies, vaccines wont be as effective. This is a good question for your immunologist. Your immunologist will probably check your blood to see if you have antibodies for things that you should have been immunized against in childhood, and then you will be told what to do if you don't have sufficient antibodies. You may need "booster" vaccines. For my father, he does not respond to the Pneumovax pneumonia vaccine, but he does respond to the Prevnar pneumonia vaccine. So I suspect that if you haven't gotten a Prevnar already, you will need one. I believe you are supposed to receive Hepatitis vaccine before receiving IVIG, since it is a blood product. My Dad has also gotten Zostavax and he gets a flu vaccine every year.

Good question about what to do to take care of your self overall to prevent infections. We are very militant about hand-washing and keep hand sanitizer by the front door so that everyone cleans their hands when they come in to my father's house - including visitors. We do try to prevent my father from being around a lot of sick people. So when his friends visit in the winter months, they don't bring their sneezing kids. For us, a simple cold can progress to pneumonia in no time. It is well known that getting sufficient sleep on a regular sleep helps both prevent and recover from infections. If you are at risk for lung infections, it is definitely good to make sure you get some simple cardio exercise regularly (walking is fine!) and to take big breaths and good coughs regularly to open up your airways. General good nutrition and keeping a healthy weight is good for all of us. You want to be in good general health. My Dad has found that with each infection, especially as he gets older, it is harder and harder to bounce back. So you want your general health to be as good as possible so that when you get sick, you have have a better chance of recovering.

While there isn't good evidence for this, my father also takes a Zinc supplement. While my father's Immunologist didn't promote this, his primary care doctor tested his zinc level, which turned out to be a bit low. So now he takes a supplement. You have to be careful though, because too much zinc is dangerous, so do not take supplements without asking your doctor to check your level and to monitor it after supplementation to make sure it doesn't get too high.

[gillie]

Hi Pattis. Welcome. Please be reassured that infusions will help a lot. I've been on two weekly infusions at the hospital since 2002 when I was diagnosed and until this month, I hadn't had a chest infection in all that time! This one is responding at last to antibiotic No. 3, so I'm feeling better again. I tried sub-cut at home, but it didn't work for me long-term. For some reason my trough levels kept going down until my immunologist called it a day and I went back to hospital treatment. As my hospital isn't more than a 20 minute drive away, I don't find it a problem.
I hope all goes well for you. Keep posting your questions. Someone'll answer.

[joem]

Jul 3, 2015 6:04:17 GMT pattis said:

I am a newly diagnosied CVID patient.  I have so many questions!  when I was diagnosed this week, I was kind of in shock and the doctor asked "do you  have any questions" and I couldnt think of much!  Now after reading alot about CVID, I do  have questions.   I was referred to a pulmonologist by my GP because my asthma was out of control.  For the as long as I can remember, I have had multiple respiratory infections one after an other all winter and early spring.  Going back to childhood.  Then 8 years ago, I was diagnosed with asthma which kicks up big time when I get an infection.   so I have asthma too all winter and early spring.  My GP referred me to a pulmologist to treat my asthma, and my pulmologist started wondering whether my immune system was compromised.  I got back my first blood tests and he showed them to me and I didnt understand much. Some of the levels were with in normal, but two were "low"  He said that indicated CVID.  He will run more blood tests (the same panel) in three months to confirm the diagnosis.  He did mention that I could get infusion therapy but said that it was expensive and maybe my insurance wouldnt cover it (it does since I have had severe asthma for so long) and perhaps "I don't want another person's antibodies".  Well, I think I DO want antibodies, so I would be glad to have what I can get - Im very open to the infusions.  Im VERY tired of being so sick every year.   Im 60 years old.  I want to ask whether the Ig infusions really work?  Do they really help people not catch EVERYTHING all the time?   I read that CVID is a genetic mutant and may be  hereditary.  My 26 year old daughter is also "sickly".  Should she be tested?   Does having CVID mean that a flu vaccine or pneumonia vaccine (that I get every year) will be ineffective since I  may not get antibodies from it?   Does CVID mean that  my childhood vaccines are and have never been effective?    What if any thing can I do to make my body stronger so that I don't get infections and if I do recover more quickly?  

I'll begin by saying I was sick as a child. Colds, flu, respiratory infections, mono, ears, etc. By age 28 I was told my immune system didn't produce antibodies. That was 1983 when I had an infection that required a month long hospital stay. I was on IV antibiotics for 28 days. The plura, lining of the lung, had to be removed. The lower left lobe was also removed. I was running a fever that wouldn't come down. A doctor said, "take him off the antibiotics and lets see what happens. My temperature dropped to normal in 24 hours and I was released the next day. It took three months to recover at home. I wonder what those antibiotic did to my immune system. In 1983 the blood supply was tainted with HIV and mad cow disease, just to name a few. My doctor was reluctant to put me on IVIG. I saw an immunologist at age 30. He started with allergy tests and followed with blood tests. He recommended IVIG. That was 1987. I'm now 60 and had a health setback three years ago. My liver was failing, a disease know as Nodular Regenerative Hyperplasia (NRH). This condition is related to CVID. Blood was not passing through my liver properly and causing damage and reduced function. I had a TIPS procedure, a non-invasion, vascular by-pass. I was retaining fluid in my abdomen. Prior to the TIPS procedure, 24 lbs. of fluid were removed. I had that procedure twice before the liver by-pass. I was 155 lbs four years ago. I weigh 120 today with significant muscle loss. I was overweight at 155 and had lost 15 lbs in the year before my recent illness. I been getting IVIG (28 years) every four weeks (30 grams). It works great. I always take an antihistamine before infusions to temper side effects. I'm encouraged. I can't do many of the things I use to, but who can at 60. Believe me, 60 is not the new 40. Keep an eye on those liver enzymes when you get blood work and mention it to your doctor. You'll be fine once your body adjust to the immunoglobulin.

[s162216]

Hi pattis, take it from me, the IVIG is very effective usually in most people in reducing the number of infections. For example, I used to get at least two chest infections and a few upper respiratory infections per year until I unexpectedly found out I had CVID and started IVIG. Since then (5 years ago), I've not had a single chest infection and at the most only get one or two upper respiratory infections per year. Don't expect any effects to be immediate though as it usually takes a good few months to start making a difference.

Having CVID does mean that it's unlikely you will form protective antibodies against a vaccine, so it is probable that your flu vaccine won't work, although it's impossible to test whether it does or not (and because of the changes in influenza strains per year IVIG doesn't protect against flu) and there is always a chance, so it's usually recommended to have one every year.

Regarding other vaccines that you've had, it is possible to test for immunity with some of them, so your doctor may do this. In most people though they either didn't form an immune system response originally or in the past they've lost the memory cells formed (that allow the body to remember infections for quick action). The IVIG will cover you against everything that is routinely vaccinated against - as your getting other people's antibodies - in addition other organisms that the plasma donors may have been exposed to. This gives you a very good level of protection against most infections.

Regarding trying to prevent getting infections, you can't really do much more than common sense approaches like avoiding those who are ill, washing hands, avoiding certain potentially hazardous foods (like pate, raw meat, unpasteurised milk) etc.

CVID is usually not known to be hereditary, and in most cases it is the result of a random genetic mutation, so it does not usually pass down to children. There are very many different forms of CVID though (as the term covers many many different conditions that present themselves clinically in a very similar way, but at the moment it is not really possible to identify the individual reasons why. This is why some people with CVID have characteristics that others don't e.g developing autoimmune conditions), and it is not unknown for children to rarely develop it as well. As there is no definite yes/no test for CVID either (for the reason above)it does tend to work on clinical symptoms and test results; all I can suggest it to keep an eye on your daughter and possibly have her tested if you're concerned.

Keep us posted on how you do, and good luck!

Sam.

Jul 4, 2015 22:41:09 GMT hlh said:

I believe you are supposed to receive Hepatitis vaccine before receiving IVIG, since it is a blood product.

Just to point out that you don't need to have a Hepatitis vaccine before having IVIG, A) because most people with PID will not respond to the vaccine anyway (I had it before a holiday shortly before I was diagnosed, and later found I had formed no immunity against it at all - pity as I had paid for it and still had to pay for the boosters even though I though I never had them as they would have been useless!), and B) it is incredibly unlikely that hepatitis or any other blood borne infection would be transmitted by IgG. Mostly this is because of improved screening measures against those who donate plasma, but also the last time anything was transmitted via IVIG was in 1993 when a few people in the UK developed Hepatitis C. Afterwards worldwide the whole manufacturing processes were revamped and massively tightened to prevent it from happening again. All IgG products go through multiple rounds of treatment to kill or inactivate any viruses or bacteria that may be there, and all products are extensively tested before leaving the manufacturer.

The bottom line is that really you don't need to worry about any kind of infection via IgG products.