Did You Know?

Now you know! What’s the difference!? A lot, mainly A PI is primary, meaning born with it. HIV is acquired (contagious) after birth. Except when a HIV positive parent passes along the HIV virus to the fetus. It’s still HIV not a PI. Serum testing can confirm

Only available to subscribers! Launch of PI Patient Piracy e-Book Coming Soon.
 Pi is not HIV or AIDS
Now you know! What’s the difference!? A lot, mainly A PI is primary, meaning born with it. HIV is acquired (contagious) after birth. Except when a HIV positive parent passes along the HIV virus to the fetus. It’s still HIV not a PI. Serum testing can confirm.

When Will This Headache Stop?

A constant, one-sided headache that does not respond to any treatment and last for.weeks.to.months. It’s not a Migraine, in fact typical Migraine Medications make it worse. Guess what? I will save you the legwork because their is a condition and treatment for this debilitating chronic headache! It’s another “rare” diagnosis–because we are lucky like that 😂. There is also ONE treatment that works, and once you find out about it, you will appreciate it. Research is slowly coming out, the new “herbal” supplement is claimed as Melatonin, nope, melatonin did not work for me. Read on…

When Will This Headache Stop?

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.

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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.

IN 4 Days: Skin on Elbow and Hand was Dead

In 4 days what first looked like a minor scrape, was a crater, quadrupled in size, the flesh from elbow and hand dead. The bacteria entered directly through skin, no entrance via cut, bug bite, scratch and spread rapidly

[contact-field label='Name' type='name' required='true'/][contact-field label='Email' type='email' required='true'/][contact-field label='Website' type='url'/][contact-field label='”Message”' type='”</p>' <p><strong>UPDATED /> What you see below may not be cellulitis. As I’ve been told by a trusted specialist that’s cellulitis does not continue to re occur in the same place. I have battled this excruciating condition since late May. I do not know what it is, it continues to reappear in the same locations, extremely painful, and as you can see is very rapid. I’ve been on several antibiotics since May and I continue to get these /> If anyone has experienced such, please help me to figure this out.</p> <p>As a person with a Primary Immune (PI)deficiency I am pretty “anal” about /> A germapobe, I am. Long before the PI diagnosis: I go out of my way to avoid any contaminates. Which is why the following story has me questioning: Where and how did I contract a skin eating bacteria.</p> <p>Four days ago, I mentioned to my son, “my elbow was really hurting”. A weird statement for me. As I endure a lot more pain than what appeared As a scrape on my elbow. </p> <p>The pain had bothered me for a while, I ignored it. When leaning on a hard surface I cradled my elbow with my palm. My son, had mentioned, “Yes, the small scrapes seem to hurt the most”. I agreed. A bit later, he said, “Mom, that does not look like a scrape”. </p> <p>Although, it was a small red area on the bony part of my elbow, I was unable to see it. So, he snapped a picture. Enlarging the pictures he said, “this looks like pieces of glass”. Indeed, I surprised myself how odd this “scratch” appeared. Maybe a form of psoriasis or /> [caption id='attachment_9271' align='aligncenter' width='768'/] That’s one weird scratch[/caption]


I then notice a slight irritation on my palm and finger. Weird. I applied some topical medication, and bandaged my elbow and hand and went to bed.

Day 2:
Removing the bandages. My little photographer was the first to see if this, “scrape” was improving. I was curious if it was at least scabbing. As the pain was pretty intense. The photos show no scabbing, but indentations. My concern began to mount. As my IGg levels are decreasing, I’m very susceptible to all bacteria, viruses, fungus. I’ve limited all activities, realizing that I am prone to serious infections. No scabbing, and complete change in appearance, concerns me.

The once broken glass appearance was now a sunken indentation.

The life-long patient that I am, I began researching what the heck this was. My husband, just realizing the situation, thought a possible spider bite. We ran through so many scenarios. He, and my kids suggested to go to the doctor. Absolutely NOT. As a chronic patient, going to the doctor exposes me to many more germs. As well, I have , learned how to take care of various illnesses. It’s exhausting explaining CVID to the medical field. Risking get very ill, and going in for a strange “painful-scrape-turned-glass-turned-crater” was not what I had the energy for. After researching for hours on dermatology, skin infections, insect or spider bites. There was not any connection.

Day 3:
The bandage pulled off, and the look on my child’s face was worrisome. I asked, really hoping, “does it look better”? The reply, “Mom go wash it off, looks like some cream is on it”
I did. I could see some type of transparent film covering the area. Thinking it was padding from the gauze. Ummmm, no. It was the skin.

Day 4: The Skin sloughing grew larger, peeling off layers as I took off the protective covering. I decided, I need medical advice.

*The Diagnosis: Cellulitis
*My Case: It is not unusual that I have complications that are not technically diagnosable. Again, I have a rare disease, and research is limited. My manifestations are certainly a concern. Although, typically persons contract Cellultis on their feet or legs. The bacteria travel through an open cut or bite. I had neither. Especially, the palm of my hand. The bacteria were able to infiltrate the layers of my skin without an actual entry point, as those with cellulitis contract it.
*Complications: The bacteria may travel to lymph nodes and into the bloodstream. This can lead to a blood infection or permanently damage lymph vessels, which are part of your immune system. Other complications can also develop. – See more at: https://www.aad.org/public/diseases/rashes/cellulitis#sthash.f0BhLYBR.dpuf
Causes: cellulitis is caused by two types of bacteria:
Streptococcus and staphylococcus
*Staphylococcus aureus is the most dangerous of all the many common Staphylococcus bacteria.
Causing the antibiotic resistant MRSA and Sepsis.
mrsa poster

Lesson Learned: For myself, family and others. Staph naturally lives in most people’s nose, and other body parts. If you blow your nose, etc., and touch any open access point (cut, blister, dry skin). The staph is then introduced into your body, potentially creating the above case. Keep all open wounds covered with an antibiotic cream and bandaged until scabbed over. Wash your hands frequently!
Don’t Open the Door to Germs

Tomorrow is Day 5: I have begun the antibiotic regime, and care of the site. If all goes well, This crater will heal. If it does not I will have intravenous antibiotics.

David Vetter’s Sacrifice

A conversation about David Vetter between his mother, Carol Ann Demaret, and his pediatric immunologist, Dr. William Shearer (SCIDs)

The Legacy Of The ‘Boy In The Bubble,’ One Of Houston’s Most Famous Patients

Attribution:
https://www.houstonpublicmedia.org/
Carrie Feibel; Originally Posted ON MAY 17, 2016