10 Warning Signs of a Primary Immunodeficiency (PI/CVID)

10 most “common” symptoms that present as symptoms of Primary Immunodeficiency (PI). PIs are complex and manifest differently, severely, and typically with unknown episodes/illnesses that are not as black and white as the warning signs. The warning signs listed are a good starting point for further evaluation.

Loading...

The following information displays the 10 most “common” symptoms that present as symptoms of Primary immunodeficiency (PI). Research tends to agree on these broad “symptoms”. PIs are complex and manifest differently, severely, and typically with unknown episodes/illnesses that are not as black and white as the “Warning Signs”. If one has any 2 of the 10 warning signs, follow-up with the specific blood work described below.

10-Warning-Signs—Illustrated–2-

10-Warning-Signs-2014-jfm
Primary-Immunodeficiencies—Know-the-Warning-Signs

Remember when stated, the 10 Warning Signs are not “black and white”? PIs are rare. Research is still in its infancy, and the Immune System is complex. I have witnessed children with debilitating arthritis and joint conditions, “strange” or “unknown” organ illnesses, enlarged spleens, heart and/or severe organ irregularities, and more. A PI is not a one size fits all. Neither are the comorbidities.
Comorbidites are the result (secondary or caused by) a Primary Immune Deficiency.
How can you confirm a PI? A simple blood test! The AAAI recommends, “An accurate diagnosis can be made through screening tests that measure immunoglobulin (IGg) levels or the number of B cells in the blood.”

I recommend saving (paper format) all serum testing, and filing in a safe place. The reason for saving previous serum or blood work, you can document decreases or increases, of the Immunoglobulin (IGg, IGa, IGm)or WBC (check the Albumin, Globulin, and the Ratios of A/G) of serum tests. This is especially true for children, as the immune system is not considered fully developed at younger ages. Which makes diagnosing a PI in young children more of a challenge. Even when children show definitive symptoms early in life. Another reason it’s nearly impossible to find IGg results 10 years later.
A PI is the umbrella diagnosis for all specific rare diseases that fall under the criteria of a PI. The second “umbrella diagnosis” under PI is Common Variable Immune Deficiency (CVID). CVID symptoms vary widely, and is less understood (as most PIs). The CVID diagnosis is confirmed if the IGg levels are significantly decreased in either IGg and IGa, OR IGg and IGm.

Further serum and clinical testing will involve what is called a, “Vaccine Challenge”. If the “Vaccine Challenge” confirms the inability for the person to create “memory” (B) cells to previous vaccinations, the diagnosis may be CVID. There may be other PI deficiencies, or genetic defects interacting, in addition to CVID diagnosis.

The rarity of a specific PI, or CVID, and diagnosing by medical professionals remains a challenge. Many physicians never see patients with CVID, and are unaware of the disease. It’s imperative to seek a Clinical Immunologist that is familiar with CVID or PIs if the IGg results are abnormal. You can request the immunoglobulin blood work at a primary doctor (get a copy!.

Posted below you will find reference points, of testing, to discuss with your primary/preferred physician.

Physician-Algorithm

When to Consider a PI in .pdf format for (Specialists):
Diagnostic-Algorithm-for-Specialty-Care-Physicians

If one is ultimately diagnosed with a PI, A patients decision of specialists, is a personal, private decision. The decisions involve many factors; Knowledge of the specialists, geographic location, experience, insurance, and your personal preferences.
I do NOT recommend sourcing any specialist from any one entity.

AAAI_1

AAAAI_2

*Disclosure: I am not a medical physician, nor pretend to be one. Consult with a medical physician with any questions, professional knowledge, follow-up and/or advice.

Attributions:
American Academy of Allergy Asthma and Allergy (AAAAI): https://www.aaaai.org/
JMF: http://www.info4pi.org/library/educational-materials/educational-books

***I do NOT endorse, am not employed, and have zero affiliation with the sources of the information presented. The pictorial information are public resources that helps the reader to understand basic warning signs of a PI.

Hypogamma-Globulinemia and Sjornes Syndromes: Updates

I happened upon a site today, and “rediscovered” this diagnosis. Say, what—-Hypogamma-globulinemia, think that we have almost every letter in the alphabet in that diagnosis. It was the beginning of the CVID diagnosis. I remember that I could not even pronounce the word. Moving onto Invisible Illness: Sjornes diagnosis, and recruiting patients for the aftermath of #AHCA to unite in order to survive.

Originally Published: 10/16; New Updates

499061352-562891public
One of the difficult obstacles, invisible illnesses.

Say, what—-Hypogamma-globulinemia, I think that we have almost every letter in the alphabet in that diagnosis. I happened upon a site today, and “rediscovered” this diagnosis. It was the beginning of the CVID diagnosis. I remember that I could not even pronounce the word. I can now, on rare occasions anyway.

I recently received my results for Sjornes disease, and I do not show any antibodies for Sjornes, but I have all of the symptoms. I do wonder if the Sjornes markers are accurate, as I receive tens of thousands of antibodies weekly. Sjornes is an antibody marker tool for diagnosis. Prior research (peer reviewed only) state 70% of people are diagnosed through the antibody and markers for Sjornes. Well, I know dog gone well, I’m so rare I certainly won’t show positive on those tests! Doc does suspect my symptoms may be due to another rare disease, but he stated that it’s  also difficult to diagnose. As well, as adding another specialist to my team. We will see.

***Update 6/2017: Sjornes Disease, an autoimmune disease, is in fact, a co-morbidity of a PI. My IGg (Immune system cells) levels have been decreasing and are well below ‘norms” with my current infusions. The IGg Infusions dosage has increased as a result, although still waiting on approval from insurance. According to the specialists, it seems that the CVID (PI) is progressing. Which is NOT what I wanted to hear. After a load of additional testing to rule out other means of IGg loss. The conclusion of IGg levels decreasing, despite IGg replacement therapy, is the “progression of the disease”. It makes complete sense that Sjornes has developed. Of course, my medical team has been treating all of the symptoms that are characteristic of this disease for years. I am not sure, what, if any treatment will be available, other than symptomatic relief. The morale here to others suffering, pay close attention to your body. I knew there was something else going on, although a vague marker test, was inconclusive. The fact that my IGg levels were declining, was a clear indicator that my immune system is now attacking itself in another, severe manner.


Originally Published: 10/16; New Updates:
Update: revamping my blog, it has been extremely difficult due to my eyes, vision and sandpaper feeling due to not making my own tears…hard to focus on anything.

The menu items are difficult, if not impossible, to move on most of WP themes. I know it’s confusing, I created this a year ago, as a personal journey, and don’t want to lose what I do have. As soon as I can tidy up, and make security changes, I will begin development of the PiPatients United (PiPUnited).

If anyone is interested a VERY rough draft of what is envisioned; a general interest  link will be posted  on THIS Blog, for a brief amount of time, with information on how to submit any interest. Keep an eye on this blog, the initial “rough draft proposal” will be released here, on this blog as a link, on Thursday (approximately) for a limited amount of time, one week. This is due to privacy issues, and the need to gather interest, and move  forward. Any responses that I receive will be followed up with, through a secure and private means. Again, any current volunteers for any other non transparent organizations will not be permitted to be involved.  PiP United’s sole focus is inclusive of PI patients and those with Invisible Illness and caregivers with intent to aid our fellow peers.  We will not be bound by any political or otherwise “rules” that ultimately decrease the quality of life for our PI and II peers. Our focus is patients helping patients in whatever capacity is needed and/or available.

Update 6/17: Still open to other individuals who are interested in building a community to help each other, regarding any and everything (gathering resources, supplies, etc) that we as patients will need due to upcoming #AHCA and the potential aftermath will leave many without medication, etc. Note: I was able to forsee the upcoming chaos in October, when this posted. Since that time, it is evident that I was correct. It was not a guess, I knew that several entities had betrayed many patients, and were doing so for personal profit. I hate that I was correct. I was, and this mission, to save lives, is needed now more than ever.

CONTACT ME

 
https://www.sjogrens.org/home/about-sjogrens/symptoms

Common symptoms that go Undiagnosed for Decades

Common symptoms of CVID and delayed diagnosis….by decades

Common symptoms that may help answer your questions about why your sick all the time:

https://primaryimmunepatientsunited.com/common-symptoms-that-go-undiagnosed-for-decades/?relatedposts_hit=1&relatedposts_origin=155&relatedposts_position=0

Common symptoms that go Undiagnosed for Decades

Common symptoms that may help answer your questions as to why your sick all the time

Illnesses, and symptoms, that I have experienced over the course of my life, pre and post diagnosis are briefly detailed here. I’m currently experiencing a horrible bout of chronic sinusitis, third course of antibiotics, and no end in site. Which is why I thought briefly detailing what seems to be common illnesses, may not be that “common” and if you experience the following, ask your doctor to run an immunoglobulin test its a simple blood test to see if your immune system is functioning correctly. Or contact the IDF for resources.

  1.  Chronic Sinusitis—takes longer to clear, does not clear in one course of antibiotics.  This is a big indicator that something is not *right*.  According to the latest statistics, “Prevalance of Sinusitis: Health care experts estimate that 37 million Americans are affected by sinusitis every year. Health care workers report 33 million cases of chronic sinusitis to the U.S. Centers for Disease Control and Prevention annually. (Source: excerpt from Sinusitis, NIAID Fact Sheet: NIAID)”

WHY is this important? Look at these numbers, 33 MILLION-cases a year, now, the patients are being treated, re treated, and continue to suffer DECADES, when they *may have an immune Defeciency, yet the patients are left to suffer, and are never informed there may be more to their Chronic Sinusitis.  I will touch more on this topic as the blog progresses.

2.  Bronchitis: NON smoker, or Smoker, wether brought on by a virus or bacteria, it always becomes a case of chronic bronchitis.  Symptoms include shortness of breath, especially with exertion, and a loose cough that produces large amount of phlegm or mucus, or deep cough without production of mucous  People with acute bronchitis may also have wheezing (a whistling sound made with breathing), *fever (some PI patients RARELY run fevers) fatigue, and chest tightness or pain that occurs with coughing. Inhalers, steroids, antibiotics are needed. Often the bronchitis cough lasts for weeks. There are many other symptoms, so please don’t rely on just these. If you tend to get bronchitis often, this may be an indicator of a poorly functioning immune system.

Prevalence of bronchitis Incidence (annual) of Acute Bronchitis: 4.6 per 100 (NHIS96: acute bronchitis); 14.2 million cases annually.  14.2 MILLION cases annually, again of this huge number, how many people are going misdiagnosed and or undiagnosed due to medical ignorance in the field of immune Defeciencies.

3. Ear Infections:  Also extremely common with those with immune systems that are not working properly.

4.  Pneumonia:  Many people with an immune Defeciency suffer from Pneumonia, and require invasive treatment to get well. Sending an immune compromised patient home with an antibiotic, will only land the patient in the hospital fighting for their life.

5.  Autoimmune Problems:  This can occur throughout any organ in your body. The bodies immune system does not recognize itself and instead, attacks and destroys any organ or system. Prior to diagnosis, my body destroyed my intestines, my thyroid, my lungs, my heart, and my joints, to name a few. I had all of these different ailments, no one could connect the dots. Lupus is also very common with CVID.

6. ZPacs are ineffective for a person who has an immune Defeciency.

7. You may not develop fevers, unless you are so ill, you may be near death before your body responds with a fever. This is variable for a lot of patients, but studies are coming out indicating most of us don’t run fevers very often.

8.  Often labeled a hypochondriac and referred to psychiatrists.

9.  Basic CBC bloodwork shows abnormal albumin or globulin (if so, this is a HUGE indicator that you need to find a clinical immunologist, do NOT believe what your trusted primary tells you. I did, and wasted two years believing her. If you basic blood work shows any abnormality in these two categories, insist on an immunoglobulin blood test.

10. Unusual and serious hospitalizations, without ever getting a true indicator as to your diagnosis.

11. Being misdiagnosed with sarcoidosis.

12. Extreme fatigue.

13. Gastrointestinal problems.

This is not an all exhaustive list, as there are many more factors, when actually diagnosed. However, these are the MOST common physical illnesses that I have encountered all of my life, and from others who also suffer from primary immunedfeciencies, or CVID.