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Primary immune deficiency.......anyone?

Discussion in 'General ME/CFS News' started by IntuneJune, Apr 22, 2012.

  1. IntuneJune

    IntuneJune Senior Member

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    Well, it has been awhile since I have been here and am having trouble finding my way around...again.

    A number of years ago, I was diagnosed with a primary immune deficiency, CVID, and started on IVIG. Almost a year and a half ago, I switched to subq Hizentra at home, much more comfortable.

    When attending the PIDD meetings, so many folks present with ME/CFS symptoms as well, we all "talk" the same. I was diagnosed with fibromyalgia over 30 years ago.

    Have others been diagnosed with PIDD? Receiving treatment???

    I was reading through posts and saw that Corinne was on Hizentra, but that was not the topic in the thread, she referred to her blog, but I could not find the blog.

    Wondering if others have been diagnosed. Thanks folks!

    June
  2. Jenny

    Jenny Senior Member

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    I have Mannose Binding Lectin deficiency - I am homozygous, so have none. Some others on this board have this too. The immunologist whom I see doesn't think there's any link to ME - I'm not so sure.

    There's a thread on it somewhere.

    Jenny
  3. satoshikasumi

    satoshikasumi Senior Member

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    Many patients would qualify for a diagnosis of primary immunodeficiency if appropriate testing were done. "CFS" is a diagnosis made by excluding certain other illnesses, while PI is a diagnosis made on the basis of certain positive findings. It is better to get a PI diagnosis because you are much more likely to get treatments paid for.

    The most widely recognized and serious form of PI, low IGG levels across the board, does not apply to most CFS patients and probably excludes the diagnosis.

    But, more minor kinds of PI are very frequently found in those with a CFS diagnosis initially:
    -IGG subclass deficiency, especially subclass 3 (B-cell dysfunction)
    -Failure to respond to pneumococcal vaccine or other vaccines by producing antibodies (B-cell dysfunction)
    -Mannose-binding lectin deficiency
    -reduced T-cell proliferation in response to mitogen stimulation (T-cell dysfunction)
    -gamma clonal T-cell gene rearrangement (T-cell dysfunction, pre-lymphoma)
    -NK cell deficiency, or reduced NK cell function + multiple recurrent herpes virus infections (T-cell dysfunction)

    Having a specific immune defect makes it much more likely that you can get insurance companies to pay for the more expensive treatments. They are especially sympathetic if you have B-cell disorders and your doctor is seeking treatment with IVIG or Rituxan.
  4. IntuneJune

    IntuneJune Senior Member

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    Jenny, thanks again for your reply. I actually answered a question you asked in a post back in the Fall of 2011 just a few hours ago. I am thinking also there is a link. CFS, FMS, autism, primary immune deficiencies, mitochronial disease have so much overlapping.

    Satoshikasumi, I have had, hummm, probably 4 1/2 years of treatment with immunoglobulins...the high test meds....and while I was being treated, was hoping my FMS symptoms would improve, but to no avail. Right now, my energy has really tanked, something is going on. Most of my subclasses improved. My IgG level is very good, however, the patient is not. My immunologist increased the dose of Ig.

    Off of Ig, I failed the vaccines. My CD4 and CD8 values are off also. Sed rate and CRP are always elevated, immunologist is sending me back to the rheumatologist AGAIN, so I am thinking I should be looked at with new eyes as my present rheumatologist is not impressed with the lab work.

    Thank you for your comprehensive response, I appreciate your time.

    Hoping to find others with similar experiences.

    June
    akrasia likes this.
  5. Ember

    Ember Senior Member

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  6. IntuneJune

    IntuneJune Senior Member

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    Thank you

    Thank you Ember, I will go take a look. June
  7. satoshikasumi

    satoshikasumi Senior Member

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    Some doctors have observed that IVIG doesn't change the course of the disease for most CFS patients with immune defects over the long term, even though it helps prevent secondary infections and can give short-term symptom relief. I am not surprised that you have not achieved remission with 4.5 years of treatment, or that you backslid. Note that there is a small minority of patients who have parvovirus infection who are practically cured by a course of IVIG (!)

    Some options that might help certain people with primary immune defects, other than IVIG are:
    --treatment for more than 6 months with strong antivirals if you have t-cell dysfunction and evidence of herpes infection. Options are Valcyte, cidofovir. (famvir and acyclovir may be useful but only at much higher than the maximum dose on the label). Symptoms take a year to improve. There is a patient assistance program to help people afford Valcyte, and insurance usually pays.
    --treating b-cell dysfunction with rituximab. This is a very new approach, nothing is known about long-term results. The theory here is that the b-cells are producing some type of toxic substance that makes you feel sick, which may be an autoantibody, or that they act as reservoirs for herpes viruses. Getting insurance companies to pay is more difficult than IVIG, but there is also a patient assistance program if you get denied and can't afford the medicine.
    --taking Ampligen for at least a year. Compared to IVIG, Ampligen can result in progressive improvements over time in immune parameters, physical activity, and cognition. Average results are modest but a minority gets full remission. This option is only covered by insurance if you live in Nevada.
  8. IreneF

    IreneF Senior Member

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    Like Kati, I am also being treated by Dr. Kogelnik with Rituxan. He diagnosed both of us with CVID on the basis of our low natural killer cell (NKC) function. His explanation is that our B cells kick out interleukins (ILs) in an attempt to stimulate the NKCs. These ILs have the unfortunate side effect of causing the symptoms we experience as CFS or ME.

    NKCs fight off herpes infections. I suspect that our B cells increase their production of antibodies to herpes and whatever else the NKCs would otherwise take care of, which is why we tend to show evidence of Mycoplasma, parvo, and other oddities. I wonder if this is an artifact of a redundancy in the immune system rather than evidence of infections that are harming us--the B cells, which produce antibodies, are in effect stepping up to the plate and doing the job when the NKCs are "out sick".

    Does this make sense?
  9. Jenny

    Jenny Senior Member

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    Talking of NK cell function, I've just got the following test results:

    T cell activation marker
    CD4+ CD45RA+ CD38+ 15.9 (29-69)
    CD8+ DR+/DP+/DQ+ 20.0 (5-20)

    NK phenotyping
    CD56dim 98.1% (control 92.4%)

    Can anyone interpret these, please?

    Jenny
  10. taniaaust1

    taniaaust1 Senior Member

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    Probably lots of us including myself, havent been tested for this. A few things thou said here are relevent with me..

    - Ive had issues with herpes (I had an outbreak inside my mouth over the roof of my mouth, only recently).

    - Ive had vaccines not take as I didnt produce antibodies like I was supposed to, so had to be redone more then they should of (wasnt tested again so dont know if it took then).

    - I responded very positively to something which had immunoglobulins in it.. it was like the whole ME/CFS was starting to improve.. feeling generally healthy in a short time while taking. At the time I put the improvement down to that the thing I was taking also had Glutathoine in it (which ME/CFS people are mostly meant to be deficient in) but this post now has me wondering. Its the only thing Ive ever taken which had immunoglobulins in it.

    - I havent ever been given any NKC function tests (but my WCC and Neutrophils are just on the normal border and would be out of range and low if any lower.. I dont know if its relevent but have heard that indicates long term chronic infection of some kind)

    Im really wondering now if I have this issue.
  11. Rooney

    Rooney

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    I just started Hizentra after my Dr. uncovered Igg subclass deficiency in subclasses 1 and 3. Interesting that have antibody to Parvo as well. He said minitor my NKC to track Hizentra's lab performance, not Igg as I will have other peoples immune system in me.



  12. IntuneJune

    IntuneJune Senior Member

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    Satoshikasumi, thank you again. As I was started on immunoglobulin therapy to augment my immune system, the treatment is considered a success as it has kept me out of the hospital with serious infections. Every infusion is considered temporary as overtime the replacement is depleted. Immunotherapy for CVID and other PIDDs is considered a life time adventure....at least at the present time.

    While I was hoping immunotherapy would help the FMS, fatigue, treatment for those diagnoses would be denied.

    Because I have been having malaise, sweating, feverish (without elevated temp which MANY PIDDers commonly do not run), other flu-like symptoms, my immunologist sent me to Infectious Disease, who after an extensive workup found nothing. I am wondering if my new-found immune system is now actually working on some bug, not-yet-discovered, and that is why I am feeling punk. Nice thought anyway.

    Satoshikasumi, you have a wealth of knowlege in this field and must admit, I am curious of your training.

    Thank you again. June
  13. IntuneJune

    IntuneJune Senior Member

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    Rooney, yes, to be tested to see how well YOUR immune system is working (or not) one must be off treatment for months, possibly six.

    Are you seeing an immunologist? In my State, many patients are treated by allergists, oncologists, or pulmonologists. I travel to Boston, MA to be seen by an immunologist.

    June
  14. IntuneJune

    IntuneJune Senior Member

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    IreneF

    I am only familiar with CVID being treated with immunoglobulins. When I get a chance, I am going to look into Rituxan. How long have you been on treatment and are you feeling better?

    As far as what you posted making sense, I find the whole darn immune system very confusing. It seems the more I read the more confused I get. Sorry I could not be of any help there.

    June
  15. IntuneJune

    IntuneJune Senior Member

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    Jenny:

    I am not good at interpreting lab results but can tell you my CD4 and CD8 and their ratios are not normal and this is an indication of an autoimmune problem.

    June
  16. SOC

    SOC Moderator and Senior Member

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    I don't have low B cells or low NK cells.
    Total B cells 230 (range 128-400)
    NK cells 232 (range 98-294)

    What I do have is low T-Cytotoxic/Suppressor CD3+CD8+ and CD5+
    T-Cytotoxic 160 (range 219-731)
    Total CD5+ 1019 (range 1022-1933)

    I have other whatsis (I don't know what they are) that look way out of whack. Does anybody know what these are or mean?
    CD8+CD95+ 5 (range 160-473) LOW
    CD4+CD38+ 484 (range 0 - 138) HIGH

    A bunch of cytokines are either high or low by no discernible pattern. IL-23 is VERY LOW. I can't figure out what that means.

    Any ideas? I'm with June on this -- the more I read, the more confused I get.
  17. Wally

    Wally Senior Member

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    Intune June,

    Have you ever consulted with an infectious disease specialist or immunologist who specializes in diagnosing/treating patients with ME/CFS? Three infectious disease doctors who are very involved with looking at viral pathogens in patients who fit a particular ME/CFS profile are Dr. Lerner (located in Michigan), Dr. Montoya (located in California), and Dr. Kogelnik (located in California). An immunologist who is actively involved with ME/CFS is Dr. Klimas (located in Florida). Another physician in the northeast who is also actively involved in the ME/CFS world is Dr. Enlander (he is located in New York). Not sure of Dr. Enlander's area of medical specialty, but he has a very impressive medical background including a four year fellowship at Stanford University with the subject of his research being EBV (herpesvirus) and cancer. It would be interesting to know if either the immunologist and/or infectious disease specialist that you have been in contact with is aware of the work of these physicians (as well as others) and if they have looked at any possible correlation between the two illnesses.

    Wally

    Here are links to the websites for the doctors noted above:

    Dr. Erlander - http://www.enlander.com/doctor.html

    Dr. Klimas - http://www.cfsclinic.com/About-Us.html

    Dr. Kogelnik - http://www.openmedicineinstitute.org/ and http://www.openmedicinefoundation.org

    Dr. Lerner - http://www.treatmentcenterforcfs.com/

    Dr. Montoya - http://chronicfatigue.stanford.edu/
    taniaaust1, merylg and SOC like this.
  18. heapsreal

    heapsreal iherb 10% discount code OPA989,

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    not just autoimmune but also persistent viral infections.

    cheers!!!
  19. heapsreal

    heapsreal iherb 10% discount code OPA989,

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    Do we think cfs/me or some of us just have a primary immune defiency, maybe nk dysfunction that is common in many of us is the type of immune defiency we have?? others like soc have t-cell or b-cell defiencies etc.
  20. SOC

    SOC Moderator and Senior Member

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    I'm beginning to think so. How many of us have had adequate immune testing to know whether or not we have some primary immune deficiency? Not many, all told.

    Certainly we've seen low NK cell function (Klimas, Bond Uni), and I'm betting I'm not the only one with CD deficiency given how many of us have persistent viral infections, chronic bronchitis, or chronic sinusitis.

    It's gonna piss me off if a diagnosable T-cell (CD8) deficiency, indicated by chronic bronchitis, multiple bouts of pneumonia, persistent ebv symptoms, and catching everything under the sun, was missed by my GPs all these years. I mean, I've been telling my GPs for decades that my immune system doesn't seem to be right and they just blew me off. I mean, jeez, what are we paying them so frickin' much money for?

    Can you tell I'm in a bad mood tonight? Baby didn't get her nap today. :D

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