[Sophie0513]

Recent bloodwork showed that my Gamma Globulin was 0.6L (range is 0.8-1.7 g/dl) consistent with hypogammaglobulinemia.

I have some questions related to this and I am hoping that someone can give some guidance :

1. Could this be the possible underlying cause to my rapidly developing neuropathy symptoms? (I did do an internet search of this and well I didn't understand what I was reading)

2. My pain started in my feet (early October) and has since progressed to full body burning. Originally, my podiatrist diagnosed RSD/CRPS and than sent me to a pain management doctor who also diagnosed RSD/CRPS. In mid November when the pain spread from my feet to my upper body and my PM doctor said that wasn't possible, I went to a neurologist in Philadelphia who specialized in CRPS and he said he suspected small fiber neuropathy and ordered bloodwork and a skin biopsy. I would like to stay with this neurologist however he is two hours from where I live. I would like to find a doctor closer in distance.
Should I go back to my primary/what type of doctor should I go to or do I need multiple doctors (neurologist/rheumatologist)?

Just not sure what to do and any guidance or suggestions would be helpful.

Thank you

[kiwi33]

It sounds like you might have Common Variable Immune Deficiency (CVID). Despite its name, CVID is not common, affecting about 1 in 25000 people. There is some information about it here http://primaryimmune.org/about-prima...ne-deficiency/.

CVID can lead to rheumatoid arthritis (seeing a rheumatologist could help here) and PN has been reported to be associated with it.

Your health care team should be able to advise you about this but IVIG is often effective in its management. IVIG is regular intra-venous infusion of pooled human antibodies

[ElaineD]

Hi, I have CVID. It is diagnosed through evaluation of four factors:

1. Levels of your (auto=this is wrong, sorry) antibodies (IgA, IgE, IgM and IgG). At least two have to be significantly deficient (well below the normal range).

2. Failure to achieve immunity protection from a high number of type of pneumonia from the Pneumovax vaccine. I had 6 protections out of 23.

3. A history of many and varied illness/very serious illnesses.

and sometimes;

4. A history of other Immune related conditions (eczema, asthma,Sjogren's, Interstitial Cystitis, etc).

IVIG is a very very costly treatment, and only addresses a deficiency in IgG. It takes between 1000 and 10000 donors to produce enough IgG for ONE treatment. With a treatment every 4 weeks (for the rest of my life) that is 13 treatments a year. The cost of EACH treatment is upwards of $20,000.

Since I began my treatment in 2013, I no longer have illnesses, which is wonderful. But I continue to have progression of my small fiber neuropathy.

Neuropathy has many causes but often for those of us with Immune Disorder it is the result of our Immune System attacking our own organs/systems. Usually with autoantibodies. About 20% of those with CVID ( a form of Primary Immune Deficiency Disorder) have Autoimmune Conditions.

The Immune Deficiency Foundation http://primaryimmune.org is a good source of more information and there is a patient forum: IDF Friends › Log In

There are many causes of neuropathy, genetic, drugs, toxic chemicals, alcohol, nerve damage, diabetes, chemotherapy, and so on. Be sure your medical team is aware of the complexity of your situation.

And I always advise keeping a daily log of your health, moods, things of note. It is surprising how much we can learn from ourselves when we keep track regularly.

Hugs, Elaine D

[en bloc]

Just want to note that Elaine is correct about several things, but I think made a minor error in the list of initial testing. The levels tested are for 'antibodies', not auto-antibodies...and she is correct, they do include levels of IgA, IgM, IgE, and IgG (all four subclasses).

Sophie, you need to have the skin biopsy done to confirm the SFN (you don't say whether you had it or not...only that the doctor ordered it). I'd like to note that 25% of those with autoimmune disease also have immune deficiency, so this should be considered for you (testing for autoimmune disease).

If you do have SFN and found to also have an immune deficiency, then autoimmune testing should be done. IVIG can not only help the immune deficiency, but higher doses can help neuropathy (SFN) caused by autoimmune disease.

You should definitely keep this neurologist and complete the testing he ordered...but also see a hematologist or immunologist for the immune deficiency and maybe a rheumatologist if they suspect an autoimmune disease process.

[ElaineD]

Thanks en bloc, my fingers have a will of their own, out of synch with my mind and my eyes, it seems!

Hugs, ElaineD

[Sophie0513]

Originally Posted by kiwi33

It sounds like you might have Common Variable Immune Deficiency (CVID). Despite its name, CVID is not common, affecting about 1 in 25000 people. There is some information about it here http://primaryimmune.org/about-prima...ne-deficiency/.

CVID can lead to rheumatoid arthritis (seeing a rheumatologist could help here) and PN has been reported to be associated with it.

Your health care team should be able to advise you about this but IVIG is often effective in its management. IVIG is regular intra-venous infusion of pooled human antibodies

Thank you Kiwi for the information! I appreciate the time you took to help me out! Thank you also for providing me with the link to CVID!

[Sophie0513]

Originally Posted by ElaineD

Hi, I have CVID. It is diagnosed through evaluation of four factors:

1. Levels of your (auto=this is wrong, sorry) antibodies (IgA, IgE, IgM and IgG). At least two have to be significantly deficient (well below the normal range).

2. Failure to achieve immunity protection from a high number of type of pneumonia from the Pneumovax vaccine. I had 6 protections out of 23.

3. A history of many and varied illness/very serious illnesses.

and sometimes;

4. A history of other Immune related conditions (eczema, asthma,Sjogren's,

Interstitial Cystitis, etc).

IVIG is a very very costly treatment, and only addresses a deficiency in IgG. It takes between 1000 and 10000 donors to produce enough IgG for ONE treatment. With a treatment every 4 weeks (for the rest of my life) that is 13 treatments a year. The cost of EACH treatment is upwards of $20,000.

Since I began my treatment in 2013, I no longer have illnesses, which is wonderful. But I continue to have progression of my small fiber neuropathy.

Neuropathy has many causes but often for those of us with Immune Disorder it is the result of our Immune System attacking our own organs/systems. Usually with autoantibodies. About 20% of those with CVID ( a form of Primary Immune Deficiency Disorder) have Autoimmune Conditions.

The Immune Deficiency Foundation http://primaryimmune.org is a good source of more information and there is a patient forum: IDF Friends › Log In

There are many causes of neuropathy, genetic, drugs, toxic chemicals, alcohol, nerve damage, diabetes, chemotherapy, and so on. Be sure your medical team is aware of the complexity of your situation.

And I always advise keeping a daily log of your health, moods, things of note. It is surprising how much we can learn from ourselves when we keep track regularly.

Hugs, Elaine D

Thank you for sharing your experiences with CVID! I believe I also saw a post from you regarding a medication change that brought you some relief!!! I hope this continues for you in 2017!! It is always good to read about people getting pain relief!

[Sophie0513]

Originally Posted by en bloc

Just want to note that Elaine is correct about several things, but I think made a minor error in the list of initial testing. The levels tested are for 'antibodies', not auto-antibodies...and she is correct, they do include levels of IgA, IgM, IgE, and IgG (all four subclasses).

Sophie, you need to have the skin biopsy done to confirm the SFN (you don't say whether you had it or not...only that the doctor ordered it). I'd like to note that 25% of those with autoimmune disease also have immune deficiency, so this should be considered for you (testing for autoimmune disease).

If you do have SFN and found to also have an immune deficiency, then autoimmune testing should be done. IVIG can not only help the immune deficiency, but higher doses can help neuropathy (SFN) caused by autoimmune disease.

You should definitely keep this neurologist and complete the testing he ordered...but also see a hematologist or immunologist for the immune deficiency and maybe a rheumatologist if they suspect an autoimmune disease process.

Thank you for your help! My skin biopsy is scheduled for Jan 3 and based upon my symptoms I won't be surprised if it comes back positive. Also, thank you for providing me with direction regarding doctors. This alone just made my situation seem more manageable to me and for that I am grateful!

[Sophie0513]

Originally Posted by en bloc

Just want to note that Elaine is correct about several things, but I think made a minor error in the list of initial testing. The levels tested are for 'antibodies', not auto-antibodies...and she is correct, they do include levels of IgA, IgM, IgE, and IgG (all four subclasses).

Sophie, you need to have the skin biopsy done to confirm the SFN (you don't say whether you had it or not...only that the doctor ordered it). I'd like to note that 25% of those with autoimmune disease also have immune deficiency, so this should be considered for you (testing for autoimmune disease).

If you do have SFN and found to also have an immune deficiency, then autoimmune testing should be done. IVIG can not only help the immune deficiency, but higher doses can help neuropathy (SFN) caused by autoimmune disease.

You should definitely keep this neurologist and complete the testing he ordered...but also see a hematologist or immunologist for the immune deficiency and maybe a rheumatologist if they suspect an autoimmune disease process.

I just realized that my bloodwork revealed a normal ANA..So does that rule-out an autoimmune/immune deficiency? Should I still pursue working with an immunologist?

[echoes long ago]

Originally Posted by Sophie0513

I just realized that my bloodwork revealed a normal ANA..So does that rule-out an autoimmune/immune deficiency? Should I still pursue working with an immunologist?

my ANA goes back and forth between being normal and abnormal. What was your abnormal ANA ratio?