Living With PI: Portrait of a Killer (Guest Post)

Apr 24, 2018 | Patient Blog

It was a sunny California-style autumn day in November 2005, when I woke to find that I could not get out of bed. For months, I had been dragging myself around feeling sick all over, but not sick enough to stay in bed all the time; I was hovering just above the point of complete debilitation. On this day, however, my illness had taken another step toward my demise: I no longer had the strength to get out of my bed. I was unable to eat or sleep, and I was suffering from severe dehydration. Some of my ailments were so strange that even my doctors couldn’t give them names. I weighed less than 100 lbs., and on my 5’4″ frame I looked like a scarecrow! I felt like one, too, not having enough energy to hold my arms and my head up.

Hopelessness and Helplessness

My doctors looked the other way and just kept prescribing antibiotics, pain pills, and antidepressants for me, but nothing worked. I steadily went downhill; now I had become bedridden. My friends and family, having been supportive in the beginning, increasingly became judgmental and hostile as no one could figure out the reason for my illness. Rumors about my having an emotional or mental problem, or a moral problem, started occurring, due to the lack of a positive diagnosis and a “good reason” for my behavior.

The people I counted on to be there for me, the people I loved, became distant and hostile, believing outrageous theories about me as “truth”. I was finally completely ostracized, and I found myself alone and dying from something that, at that time, had no name.

A Sunny Life Eclipsed By Darkness

In my career as a human resource specialist, I have worked for Intel Corporation as well as Sacramento State University, Sacramento; jobs I loved very much. In 1998, I finally graduated from the University of Phoenix with a BS in Business Management, and my husband and I had just bought a brand- new house, and my children were thriving. I had no discernible reason to feel depressed, sick or exhausted. Yet, there I was, day after day, wasting my life away for no apparent reason because I was physically unable to get well and get out of bed.

The Dawn of My Death Sentence

I was finally diagnosed with Primary Agammaglobulinemia in 2010, exactly 5 years after I became completely disabled. This disease is genetic, therefore not contagious, and it had been dismantling my immune system’s strength, undetected, since my birth. I had always been a “frail” child, having pneumonia on a regular basis, also bronchitis, the “flu”, and common colds, as well as developing the general gamut of childhood diseases. Friends and family told me to “eat more”, “stay indoors”, think “positive thoughts”, and my personal favorite: “clean your room!”, in misguided efforts to help me.

As I grew older, my condition, (called Primary Immune Deficiency Disease, or PIDD—it’s the more “user friendly” nomenclature of Primary Agammaglobulinemia), progressed. As I became increasingly vulnerable to bacterial and viral infections, my doctors seemed completely unable to figure out what was wrong with me. I did not realize I was never given the right blood test. It’s a simple test, measuring one’s immunoglobulin levels (we all have IGG, IGA, and IGM which are the building blocks of our immune systems.) When a person’s blood count is low in these categories, the patient may have an immunodeficiency problem needing treatment. For those of us who have a confirmed immunoglobulin deficiency, such as mine, our prognosis is fatal without treatment: we require regular infusions of immunoglobulin to stay alive. I am one of those patients.

Portrait of a Killer

This disease affects approximately one person out of 1,200 in the U.S., according to J. Michael Boyle and Rebekah Buckley in “Population Prevalence of Diagnosed Primary Immunodeficiency Diseases in the United States”, Journal of Clinical Immunology 2007. In their article, which represents recent theory; previously our medical community has assumed that PIDD is a “rare disease”, therefore not something one must test for on a regular basis, Boyle and Buckley write about recent figures which indicate this disease is much more prevalent than previously assumed. Unfortunately, the word has not spread far enough into everyday medical knowledge to justify regular testing. For instance, during my worst time with PIDD, before I’d received regular IGG infusions, I was told by an emergency room physician that I “could not possibly have PIDD because it is much too rare.” Therefore, I received no treatment for my disease and was discharged to find help on my own.

Diagnosis Does Not Mean Treatment

After I had been diagnosed correctly in 2010, my own health insurance company quickly dropped me because now I had a “pre-existing condition” they didn’t want to insure (this was just prior to President Obama’s health care initiatives, when insurance companies were allowed to do such things.) I applied to every health insurance company I could find, but none would insure me. At the same time, I had been referred by the diagnosing physician to a specialist, a hematologist, who confirmed his diagnosis, but then told me, “You have PIDD, and you require immunoglobulin treatment, but I’m not going to give it to you because your job doesn’t pay enough, and you have no health insurance. Go look somewhere else.” I managed to hold my tears until the door closed on her office. I wouldn’t give her rabid hostility the benefit of knowing she’d hit her mark.

Without Treatment We Die

It was a horrible time for me: Due to an untreated infection, I lost the sight in my left eye permanently, and all my hair fell out. I also had numerous operations on my sinuses and teeth, also due to untreated infections. I developed two faulty valves in my heart, the atrial and mitral valves, requiring either surgical correction or lifelong medication, or both.

Also, due to untreated infections, I developed permanent scars in my bronchial sacs and lung tissue, as well as intestinal scars that prevent food from absorbing correctly. My skin is subject to autoimmune disorders like psoriasis because PIDD causes the immune system to become unbalanced, and sometimes our healthy cells come under attack from our confused immune system, which can lead to serious consequences if not dealt with immediately. Without appropriate health insurance coverage, I was doomed to die a slow, painful, lingering death.

Never Say Die—At Least Not To Yourself

I didn’t give up: I kept asking questions of my diagnosing doctor, and he pointed me in the direction of the Immune Deficiency Foundation, the only organization in existence devoted entirely to information and encouragement for those of us with PIDD. I found a wealth of knowledge there, and was very lucky to run across a reference to Patient Services, Inc. PSI is the ONLY organization that meets a medical disaster where it hurts PIDD victims first: financially. Without their financial assistance, I would not be writing this letter. I would be dead (again!)

Our medical system of delivery still requires that only those with a blessed financial status are able to afford the immunoglobulin infusions and constant doctor’s supervision necessary. While I had enjoyed a healthy financial life prior to my disease’ becoming acute, I had lost everything through paying my medical bills on my own without regaining my health. I lost my house, my marriage, my kids and their college futures, my family and friends, and my dignity. All that was left was me, my faith in God, and organizations such as PSI.

Meeting My Unexpected Saviors

With God’s help, and that of a very astute and knowledgeable disability judge and his medical consultant, I was awarded “early” (I am not 65 yet) Medicare in 2012. If the judge had not ruled in my favor, I would be dead. I thank God whenever I think about him because he saved my life that day. Since then, my life has turned around for the better because I now receive intravenous immunoglobulin (IVIG) treatments on a regular basis. I have a future to look forward to because of the unfailing efforts of organizations like the Immune Deficiency Foundation and Patient Services, Inc., or PSI, as well as our own Social Security Disability Insurance program.

Deliverance From Death Is Expensive

Even though I have Medicare, as we all know, Medicare covers 80% of our medical costs that qualify for payment according to their rules. It is necessary for me to have a supplemental insurance plan that will cover the other 20% of uncovered medical costs. In my case, I would not be able to receive my IVIG treatments without it. PSI pays the $550 per month supplemental insurance premium that I cannot afford. Without PSI, I would be unable to get the treatments I need to survive. PSI gives me hope in the future, as no other organization has done. I currently live with my mother (she’s 88) and we take care of each other. If I die, my mother will have no one and nowhere to go. My children will lose their mother and grandmother, and my dog will be orphaned. Each of our lives touch so many other lives!

Life, Faith and Helping Others

My battle’s been worth it! There have been so many who’ve encouraged me to overcome serious adversity; people who have helped me when they didn’t have to, (for instance, the wonderful people at PSI), that I feel very grateful and driven to “pay it forward”; to do what I can to help others in need.

PSI: The Guiding Light In Our Storm

I’ve been volunteering for the Immune Deficiency Foundation helping those diagnosed with PIDD by encouraging them to keep trying, keep believing; that there is hope when organizations like PSI exist to help us on our very rocky road. I can tell you that without the existence of PSI and their very real “hand up”, I would have no hope. It is a very lonely, terror-driven place to live.

Keep Our Light Shining

Please help us protect, and keep, our PSI. It’s our only beacon of hope in coping with a financially forbidding treatment. Please stand up for life today (and PSI!) and contact your congressional representatives to add your voice to our most crucial battle. Together, we can win the fight for life!

Sharon, Patient Services Inc., PIDD Patient

Health Care Charities Like PSI Under Threat

Recent guidance from the U.S. Department of Health and Human Services Office of Inspector General (OIG) restricts the ability of charities to communicate with pharmaceutical donors about new therapies and patient needs. This guidance has become so constraining that it is impairing charities’ ability to operate, making it difficult to develop new programs and obtain funding for existing programs that many patients rely on.

What Can You Do?

If you or someone you know has been diagnosed with Primary Immune Deficiency Disease, PSI wants you to know that you are not alone. As a matter of fact, you can learn more about what we do by visiting our patients page and read about the various programs available based on your type of insurance.

Diagnosed With Primary Immune Deficiency Disease: A Portrait Of Hope

At PSI, we want to thank Sharon for so courageously sharing her story with us all. Primary Immune Deficiency Disease for certain is a portrait of a killer, but living with PI need not be.

To all of you battling PIDD, you are our portrait of hope for your courage, strength, belief and resilience. Each and every day we wake up and come to work with you on our mind and your stories deep within our hearts.

Dear Department of Justice and Department of Health and Human Services, on behalf of the many battling Primary Immune Deficiency Disease, #LetCharitiesBeCharitable!

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