By Jean McCaslin, RN, IgCN; Clinical Nurse Educator

I am proud to share that I have been a Registered Nurse since 1981 in a multitude of settings. For all that I have learned and continue to experience, my role as a patient has taught me the most.

Being one of 7 children, as you might imagine there was an overabundance of "togetherness". What I failed to know then, was that we were better "sharers" than we thought. This was particularly true when it came to germs. We passed illness through our modest home in an unforgiving way. As a family, we were nearly evenly divided into two groups; the almost always sick and the almost never sick. The ones with the environmental allergies, runny noses, bronchitis, and fevers and those without. I never paid attention to the fact that I was in the sickly crew, as was my Mom; it was all very normal to me.

While working in the Emergency Room, Intensive Care, and Cardiac Care Units I was exposed to a plethora of cooties and was often sick. I had my tonsils removed at age 21 and my strep throat and other infected sinus episodes appeared to subside. I discovered that some of my childhood vaccines needed to be repeated, as my work-mandated viral titer studies showed that I lost protection; it is possible that I never developed the proper protective titers in the first place.

In my early forties, I left bedside nursing due to repetitive back injuries. While employed in a Neurology office, I began to get sick again quite often. These illnesses ranged from sinusitis to bronchitis, to ear infections, to urinary tract infections, to fungal infections. Dietary and holistic alternatives helped somewhat, but not significantly. During this same time span, one brother developed pneumonia and one sister was treated for a uterine abscess after a routine surgical procedure.

I then left direct patient care and had some degree of respite from infection. I was working in the IgG sales and home-infusion nursing industries. Over the course of 15 or so years, I worked closely with the Immune Deficiency Foundation, Jeffrey Modell Foundation, and Clinical Immunology Society. I collaborated with immunologists and assisted in the care of primary and secondary immune deficient patients. I learned the warning signs of immune deficiency diagnoses and assisted in the IDF Zebra advocacy efforts.

How could I be so ill-informed? These symptoms sounded like me. These symptoms seemed like my siblings and my mother. I recognized these things in 2003 or 2004. I explained this to my PCP and to my family. It did not elicit much more than a groan or eye roll from any of them. Perhaps my flair for the dramatic had gotten the better of me? Maybe my college-day hypochondria (in Anatomy/Physiology class the signs and symptoms of many disorders seemed to fit me).

One immunologist I had known for many years pulled me aside in about 2013 or 2014. He asked me if I had ever been tested for an immune deficiency. He noticed that I had a multitude of infective illnesses in the years he had known me. I explained I had no more than an IgG level drawn after pushing my PCP to please at least order that; it was borderline low-normal.

Travel kept me busy and I put off scheduling a formal visit with this immunologist, who was as much a friend as a colleague. One day he requested that his staff "tie me down" to draw lab work. I followed up 2 weeks later for results. I had 3 significant Ig-subclass deficiencies and protection to only 4 of 21 pneumococcal strains. I hardly had any protection against tetanus although I had gotten a tetanus shot only 2 years earlier for a gardening accident. My IgG level was just above the acceptable level for my age.

After receiving both a tetanus vaccine and a pneumococcal-vaccine challenge, I achieved adequate responses to both. I was infection free for 3 years for the first time in my personal history. As sinus infections began again in late 2016, I again was vaccinated with a pneumococcal-vaccine; this time 2 injections were needed to achieve a more modest result. At this time, my immunologist prescribed IVIG; it was denied by my health insurance despite my IgG subclass deficiency diagnosis and recurrent infections.

I currently work as a Clinical Nurse Educator at Nufactor. A large portion of my time is spent training nurses as well as patients about IgG (IV and SC) administration as well as the disorders treated with it. In late 2018, I was hospitalized for pneumonia. In the summer of 2019, I was placed on prophylactic antibiotics for recurrent sinusitis and bronchitis. An immune panel was again drawn, and a pneumococcal-vaccine again administered. This time, I had a very poor response.

My immunologist suggested I be seen at the University Hospital practice where he was trained, and formerly worked as an attending physician. Subcutaneous immune globulin (SCIG) was prescribed and insurance authorization was near-immediate; I now self-administer weekly.

I very quickly learned that self-administration of SCIG is trickier from a patient perspective, than I had thought as an outsider looking in. Collaboration, trial and error, and communication are all critical. The truth is that SCIG can be painful, inconvenient, sometimes sites leak, and at times leaves welts. While I knew this as an instructor, the impact of it on my own quality of life was far more significant.

Before I was a SCIG patient, I advised patients to slowly work up to the least number of sites as possible so that less needle sticks were needed; I now feel otherwise. I thought that the pain sensation was equal in all sites; that too is not so. I thought that not feeling pain in my thighs, meant there would be no pain in my posterior hips/flank; also, not true. I also believed that every week my infusion would infuse in the same way. The truth is that every site infuses at a different speed and the medication disperses differently.

I am grateful that I can now speak to the procedure from a first-hand perspective; it has made me a better resource. While I consider myself compassionate and empathetic in general, being a SCIG patient, surgical patient, or pneumonia patient has blessed me with a greater level of understanding. I am a better educational resource than I might otherwise have been, had I not been a patient as well.

My family and personal medical history was taken during my first visit; it includes many skin disorders including poor wound healing in the absence of diabetes, eczema, psoriasis, skin cancers (basal, squamous, and melanoma), colon, uterine and breast cancers. This led to immunology laboratory testing for defects specific to skin and mucous membrane disorders; they were all normal except for a borderline abnormal T-cell study.

More extensive T-cell studies were ordered and were again borderline dysfunctional. Genetic testing was suggested and a referral to a geneticist was made. There is still so much that is poorly understood by medical science today; some things I may not want to know. This is particularly true today, as managed care organizations continue to lobby law-makers to exclude pre-existing condition coverage from health care plans. I, like many patients, are hopeful that future legislation encourages the testing and sharing of genetically-linked disorders such as PIDD and cancer risk.

The future holds promise for the treatment of immune deficiencies. Education and advocacy are extremely critical aspects of this promise. As an RN with nearly 40 years of experience, it haunts me to know that my journey to treatment was so elusive. Considering that someone like me who is so entrenched in the treatment of PIDD's would experience such delay in diagnosis and treatment. I can only imagine and be empathetic towards those less informed than me.

The IDF Signs of Primary Immune Deficiency Disorders should be widely shared (see below):

• Is it just an infection?
• You should be suspicious if you have an infection that is…
• Severe - requires hospitalization or intravenous antibiotics
• Persistent - won't completely clear up or clears very slowly
• Unusual - caused by an uncommon organism
• Recurrent - keeps coming back or if it
• Runs in the Family - others in your family have had a similar susceptibility to infection

If any of these words describe your infection, the Immune Deficiency Foundation (IDF) recommends that you ask your physician to check for the possibility of PI. Click here to read more about specific types of PI. If you have further questions, please contact IDF through Ask IDF or call 800-296-4433.