This week was my 59th birthday.  A bit of a milestone in my mind.   My older sister died from non-hodgkins lymphoma with undiagnosed PIDD when she was 58.  I did not want to be 58.  I feared I would follow in her footsteps.  I have been much more fortunate.  I was diagnosed when I was 55, and have been receiving Sub Q treatment with Vivaglobin.  This drug, combined with additional drugs that treat my inflammatory problems from my autoimmune disorders, have given me a quality of life my sister was not afforded.   I also focus STRONGLY on the benefits of eating wisely, exercising regularly, resting when necessary, being involved in extremely rewarding work (working with PIDD patients and their physicians), having loving family and friends around me, and a team of doctors who really care.    Laughing also is very beneficial.

This is an RX I plan on following as long as I possibly can.

I highly recommend that you follow a similar plan of attack on your own PIDD.  Don’t forget to ‘Carpe Diem’ as well.

Looking forward to 60 now.

This past week-end, I had the opportunity to hear some terrific speakers, including Dr Rebecca Buckley and Dr Joseph Roberts both of Duke University Medical Center.   I took copious notes, and thought I might share some of them with you.  

The first should not be anything surprising, but as far as a clinical presentation, most of us appear normal.   It isn’t until frequent use of antibiotics begins to show a pattern that most physicians suspect a PIDD.

The following notes refer to CVID:

There are reduced levels of IgG (IgA and/or IgM)

Impaired ability to produce specific antibodies

Can’t produce antibodies when needed - as in when an invader appears

Often diagnosed as an adult, and often following a triggering event; e.g. Epstein Barr, Mono, HPV, etc

Frequent GI problems

Multiple Bacterial infections

More prone to autoimmune diseases

Sinus problems

Arthritis

Enlarged lymph nodes in chest or GI tract

Lymph node malignancies in females in 5th and 6th decade

Granulomas or lymphocytic interstitial lung disease

Testing for siblings recommended- it may manifest itself in different ways- e.g.; autoimmune disease, allergies, IgA deficiency

Selective IgA Deficiency can often progress to CVID

MYTHS about PIDD

It’s probably not a rare disease anymore

Patients are not always chronically ill

Not all PIDDS have increased susceptiblity to infection.

Hope my notes help.

If I can sort through more of them, I’ll add to this list - but they look a lot like my old college notes- and I’m having a difficult time deciphering them.

I attended an IDF meeting at Duke and heard some of the top doctors in the field of PIDD speak.  One of the latest findings is there are now 150 types of PIDD!!  There are so many that it may not be classified as a rare disease in the future.

Who Knew?  There is a week dedicated to awareness of illnesses like ours.  So, I guess we are supposed to get out there and tell everyone about PIDD this week.   Let’s get going.
If you Google Invisible Illness Week- you will find tons of helpful tools as well- such as disablility info, etc.

Enjoy our week - no one seems to ever want to celebrate PIDD- but now we get to.

I attended an IDF meeting in Boston, and was thrilled to hear Dr. Francisco Bonilla speak.   He is from Boston Childrens Hospital.

I will share my ‘notes’ with you:

There are 4 types of treatment for PIDD:

They include:

Bone Marrow Therapy (for SCID, HIGM, XLP and a few other types)

Thymus Transplant (primarily for DiGeorge Syndrome)

Gene Therapy (for SCID and other VERY specific  PIDDs) - where they actually replace the defective gene

IgG Therapy - which most of us know about.

CVID is not open to the first 3 types of therapy as we have so many differences in disease, and differences in how it manifests itself.

He also talked about how adult onset PIDD usually has some triggering mechanism.   It is often Epstein Barr that triggers it.  (That was my trigger).  According to Dr Bonilla, our immune system did not handle the triggering event correctly, and thus the immune system was never repaired.

This Dr really gets it too.   I was very impressed by him.

I met some terrific patients and patient families at this meeting as well.

Hope my ‘notes’ help you.

I’m leaving shortly for a patient meeting outside of Boston.   I hope to meet lots of patients and hear their stories.   Watch for a couple of patient profiles here- make some new friends. A couple of doctors will be speaking,  and I’ll be sharing that information with you as well.

Here are some things I’ve learned from attending conferences.

This comes from the national IDF conference and is a compilation of several sessions I attended.

Recommended once a year:

Spirometry

CBC/Biochem panel, IgG trough levels/blood born virus check

Lung function test  - CT SCANS - Base line and follow-up as needed

Liver and Kidney function tests

ENT - Once per year

Follow-up with Clinical Immunologist 2 X/ year

I hope these recommendations help you work more closely with your physician.  This is a good check list for both of you to use annually.