10 Ways Primary Immunodeficiency Affects Me Daily


Did you know that April was Primary Immune Deficiency Awareness Month, and April 22-29 was World PI Week? Yep. Most people didn’t. However, around the globe, individuals and organizations held activities and posted on social media to spread the word about PI.

Even though it tends to be spoken of with the collective “PI,” there are actually hundreds of types of primary immune deficiencies, which manifest in various ways and affect different aspects of the immune system. Primary immune deficiencies are thought to be genetic – some genes have already been identified – and while some types of PI can be detected in infancy, others don’t peek out of the double helix until later in life. Many types of PI have far-reaching effects on various organ systems and can lead to serious complications, ranging from respiratory disease and autoimmune conditions to B-cell cancers.

No, I don’t live in a bubble. No, I’m not “allergic to the world.” You know how there are about a million Imperial stormtroopers in the battles in the Star Wars films, they’ve got a lot of heavy armor and scary-looking blasters, and none of them seem to be able to actually hit the enemy? That’s my immune system right there. They’re more likely to hurt themselves than an intruding army of bacteria.

 

Many chronic and acute illnesses and conditions and medications can affect an individual’s immune system or response to infections, so many of these concerns aren’t exclusive to PI. However, as a person with a form of PI, they’re often on my mind.

1. I have a zebra-striped ribbon on my backpack.

Doctors are often taught, “When you hear hoofbeats, think horses, not zebras.” In other words, the ethos is that a patient’s symptoms are far more likely to stem from a common cause than from something unusual. The trouble with this philosophy is that when doctors don’t think outside of the box, it often leaves individuals with chronic or rare conditions undiagnosed and untreated.

Many patients with PI aren’t diagnosed for decades, even when they have frequent infections. This was true for me, too. I was sick a lot as a small child, teenager and college student, and had unusual infections like Rocky Mountain spotted fever and scarlet fever, but nobody ever thought to do the simple blood work that would have pointed toward an immune deficiency. I was only diagnosed with PI as an adult, and only after I insisted that my doctors needed to pay attention to the fact that I seemed to have a lot of ear, throat and respiratory infections.

The motto of the Immune Deficiency Foundation in the USA is “Think Zebra!” If doctors considered more uncommon causes of illness – such as PI – patients could be diagnosed and treated more quickly. Other rare conditions that often fly below the radar for doctors, such as Ehlers-Danlos syndrome, also invoke and identify with zebras.

2. I wash my hands (and things I touch) a lot.

I wash my hands a lot and try to avoid touching my face. I always have a bottle of hand sanitizer in my bag, and I use it many times a day. I wash my hands as soon as I get home. Extreme fatigue makes it difficult for me to do as much home cleaning as I’d like, but the things I touch often – for instance, the doorknobs, faucets, light switches and computer keyboard –are frequently disinfected with Lysol wipes. I often clean the screen on my phone, too. When I run my dishwasher, I always choose the high-heat drying cycle. It might seem extreme, but these are simple, everyday measures that help reduce my exposure to germs.

3. I try to avoid using communal items.

I won’t share makeup, earphones, straws, utensils or cups, and I try to avoid touching things that are handled by a lot of other people. Sometimes it’s unavoidable – for instance, if I’m on an escalator I want to hold the handrail – but if there’s any way to get around it, I will. If I have to fill out a form at the doctor’s office or somewhere else, for instance, I will use my own pen instead of the one on the counter.

4. If you have the flu, a cold or any sort of throat or respiratory infection, I want you to let me know and postpone our plans.

The same holds true if there’s something going around your child’s class at school, if they’re sick or if they’ve just come down with an interesting looking rash. I once caught strep after being around someone with it for five minutes, so when I say it’s very easy for me to pick up whatever you have, I’m really not joking. That cold might seem minor to you, but it could hit me hard, lead to a secondary infection or cause my other chronic conditions, such as fibromyalgia, to flare even more. Let’s not risk it.

5. I might need to wear a mask.

I might opt to wear a surgical/germ mask when I’m in crowded, enclosed spaces, such as a bus or waiting room. If I have to do this when we’re out together, please don’t be embarrassed. Yes, I know people are probably staring, and I’ve heard the comments about it. However, we all have to do what is best for ourselves. My immunologist supports the use of masks in specific situations, and I listen to him and my own instincts on the matter, not the hecklers.

6. I use a lot of antibiotics.

Yes, I know they have side effects. Yes, I know that doctors are trying to curtail antibiotic use because of concerns about resistance. The game’s a bit different for me since my immune system doesn’t pick up the slack. That’s why I have been prescribed antibiotics – ear drops, pills and ointments – to keep on standby in case I need them. There are certain criteria for using those antibiotics, the same way there are specific symptoms that prompt use of my rescue inhaler or Epi-Pen. It saves me from having to run to the doctor every time infection sets in and ensures that the tools I need are immediately available.

7. I don’t like going barefoot.

I kick off my shoes as soon as I get home, but I immediately put on “house socks” or flip-flops. If I go swimming, I wear flip-flops until I’m near the edge of the pool. I don’t walk around with bare feet anywhere if I can possibly help it. Part of that has nothing to do with PI; I tend to have very cold feet and toes. However, it’s also about not wanting to cut my feet or pick up any sort of fungus.

8. When I hear about outbreaks, I inwardly stress out.

When I hear about outbreaks of anything infectious – measles, whooping cough, norovirus, the flu, you name it – it triggers anxiety for me. I’m fully vaccinated, but my immune system isn’t competent. It forgets what it’s been taught and doesn’t react as it should, even when it’s been previously exposed to an illness or vaccine, and even when my titers appear to be high.

9. That “immune booster” you bought at the health food store won’t help me, so I will politely decline it.

Like a lot of people, I take vitamins and supplements. However, that tincture from the health food store, which promises to “boost immunity,” won’t help me even a little. If only it were that simple! I could discuss switched memory B cells and low immune globulin production, but let’s suffice to say it’s a very complex genetic condition that doesn’t have an easy solution. There are only a handful of accepted medical treatments for some forms of PI, and even those might not necessarily stop complications.

10. I get annoyed about inaccurate depictions of people with PI in the media.

The only positive representation of PI in mainstream media I’ve come across has been the Jeffrey Modell Foundation’s wonderful series of public service announcements. Other than that, there isn’t much out there. As far as I know, PI hasn’t even made it onto the medical drama shows like House and Grey’s Anatomy.

So what do we have? There’s a film from the 1970s called “The Boy in the Plastic Bubble,” which starred John Travolta. Travolta’s character was loosely patterned after two patients: David Vetter, who was born with SCID and lived in a sterile environment for his entire life, and Ted DeVita, who entered hospital isolation as a preteen after fighting aplastic anemia. A major character portrayed by Amandla Stenberg in the recent film “Everything, Everything” also ostensibly has SCID, is “allergic to everything” and is confined to her house.

Both film characters eventually just stroll out of isolation to pursue romantic relationships. One character is found not to have SCID at all, but to have been a victim of Munchausen’s by proxy. In reality, David Vetter died at the age of 12, Ted DeVita at 17. The gold standard for SCID right now is bone marrow transplantation, not isolation for a lifetime. Yes, we all know that Hollywood takes dramatic license and isn’t always painstakingly factual. Still, when there aren’t any other dramatic depictions out there, that’s the public frame of reference, and it can be very damaging.

There are better ways to depict uncommon conditions on film. For instance, the 2001 movie “The Others” featured two characters with a serious photosensitivity disorder – said to be xeroderma pigmentosum, although it was never defined straight-out in the actual movie. The DVD included a documentary about the XP Society, Camp Sundown and a young girl with XP, so viewers could learn some factual information on the condition. I wish we would see a film with a character with PI handled so respectfully and accurately.

Most of the time you’re not going to notice any of the everyday actions I take to manage my primary immune deficiency, since it’s an invisible condition for me. However, if you notice me washing my hands a lot or breaking out the hand sanitizer, believe me, it’s for my own good.

We want to hear your story. Become a Mighty contributor here.

Thinkstock photo via Sasiistock.

TOPICS
, Contributor list
JOIN THE CONVERSATION

Related to Primary Immunodeficiency

screenshot of everything everything with amandla stenberg

To the Author of 'Everything, Everything,' From a Teen With a Primary Immunodeficiency

“Everything, Everything” is a best-selling novel turned movie telling the story of a teenage girl who has severe combined immune deficiency (SCID). It shows her as “allergic to the world” and unable to leave her house, with the only people she has ever met was her mom and nurse. She then falls in love with [...]
nurse holding a woman's hand and checking her pulse

How a Nurse Helped Me Feel Less Alone During Treatment

After contracting aseptic meningitis from my treatment, I laid in bed hurting and aching with an intense feeling of isolation. I wondered if I would ever be able to reach my goals. This didn’t feel fair, and I was angry at the world. I didn’t understand why I was seemingly the only person to have [...]
Woman enjoying a concert.

4 Tips for Being Adventurous With a Primary Immunodeficiency Disorder

Live music, tattoos, heels and makeup – these are a few of my favorite things. However, I have a primary immunodeficiency disorder, which along with the lack of a functioning immune system, comes a host of symptoms that make being spontaneous impossible, but being adventurous doable. Well, doable if I prep and remember some hacks [...]

Life as a Mother With a Primary Immunodeficiency

“I don’t know why I can’t get this IV,” the emergency room nurse said last Saturday after trying for the third time to start an IV. “I just stuck a 98-year-old across the hall and you’re the young and healthy one.” Since I have what we call an invisible illness at 35 years old, which is [...]