Tag Archives: CVID

What’s Going On In There…?

For years I’ve fed the dogs and cats at exactly 7:20 every morning. An odd time? Yeah, but it’s how we do things I guess. Traditions start in strange ways… Years ago we used to kennel the pups until Wifey Dagmar left for work each morning. She’d leave at 7:18, the pups were fed at 7:20. It made sense. And even though Wifey hasn’t left for work in nearly two years now we still feed the dogs at 7:20.

This morning was pretty normal. The pups got restless around 5:30, I let them out to read their morning paper, they came in and snoozed whilst I worked until 7:19 at which point their inner alarm clocks went off with an uncanny accuracy that’s vaguely unnerving. I shooshed them out the door, got the kibble, “Okay, girls, calm,” I said as I unceremoniously dumped a cupful of dried nuggets into their bowls, the words forming clouds of icy vapor in the still winter air. They sat, Zoey by her bowl, Buttercup by the other, staring at me, quivering. “Free,” I said. By the time my mouth had started forming the letter “R” the girls were snout-down in the Heaven That Is Food.

I popped back inside to get another cup of food, this time for the cats. I often wished I had three hands so I only had to make one trip at feeding time. I put some nuggets on the table for Miss Mittens, opened the strongbox, nudged Nitty Kitty aside with my hand, and dumped the rest in the bowl. A few years ago Pops made us a smallish box with a kitty-sized hole in the front – I put the cat’s food in there so the pups can’t get to it. Every morning I smile a bit; as I feed the pups I’ll usually see two golden-green eyes blinking mysteriously at me from the depths of the shadowy kitty box, a small but fierce black kitty inkily biding her time in the safety of darkness… Nitty is the smallest of our family, a tiny cat. Yet she rules her kingdom with a firm, though adorable, black velvet paw – Miss Mittens (also known as The Lady Miss Waddlebottom) might be twice her size but Nitty always gets the food bowl first.

I stood outside for a moment and pondered the silent dawn creeping it’s way over distant frosted hills, the vibrant warm colors in the eastern sky giving false promise of comfort on a bitter blue December morning. A small bird, feathers fluffed out to double its size, sat in the pine tree a few feet away, staring towards the gathering light, hopeful.

Breaking the spell, the pups finished their food and eagerly traded places so each could examine the other’s bowls, hoping for an errant leftover nugget, snuffling and shuffling. I shooshed them back inside, Buttercup heading towards her kennel, Zoey to the other. Click, click, doors shut, I patter through the room towards my office as quietly as possible, but…

A muffled voice, “Honey? Can you shut my door please?” I turn back and peek into Wifey Dagmar’s room. A silent TV flickered away, merrily showing pictures to itself, absently casting light on a comfy lumpy bumpy pile of pillows and cushy blankets with a little nose poking out. The muffled voice spoke again from the pile of blankets. “I just fell asleep about half an hour ago… I vish these stupid meds wouldn’t keep me avake.” I obediently reached in to swing her door shut, soft snores from the blankets already competing with the quiet, ever-present whoosh-whoosh-pfffff of her oxygen machine. I’ve learned that her doorknob rattles, so I tend to simply give the door a bit of a tug with my finger and let it swing silently toward me, hinges oiled against squeaks. But today, “thunk.” The door hit Dagmar’s wheelchair. If her chair is pushed right up to her bed there’s about a quarter-inch of clearance to swing the door shut, but if there’s any gap between the wheelchair and the bed the door won’t shut. The snoring stopped as I stepped into the room and quietly pushed the chair an inch northwards, but resumed again as the door swung quietly shut, unobstructed.

Last summer I took the trim off the bottom two or three feet of her door so she could get the wheelchair in and out a bit easier – the wheels of the chair fit through the door, but barely. The extra inch of clearance makes a big difference, but it also means that when her door is closed there’s now an inch wide gap allowing light – and sound – into her sanctuary. It’s on my wish-list to widen her doorway and put a barn door (a door that slides back and forth on a rail outside her room rather than a normal swinging door) in someday so she can get her chair in and out easier – and park her chair in her room without worrying about it blocking the swinging door. But that’s a task for a different day.

I tippie-toed back across the living room to my office, eager to get back to work. But as I listened to the combination of gentle almost-snores and whoosh-whoosh-pfffff on the WIfey monitor I keep on my desk, I found myself almost haunted by a thought.

What does she think?

Most people enjoy a little alone time every now and then, but Wifey’s isolation takes “alone time” to completely new levels. Because of the immune system disorder not only is she always ill, but we’re always trying to avoid introducing her to any new germs… While we don’t turn people away at the door by any means, we also don’t particularly encourage visitors as Dagmar is invariably ill for days or weeks afterwards. And she never feels well enough to leave the house.

A photo of Dagmar swimming with children

Dagmar, before the illness. Happy, full days.

Her world consists of three rooms – bedroom, bathroom, and living room. Her social life consists of a television, a phone she can rarely use due to her pain, two dogs, and a husband who’s either working or sleeping. She’s usually awake all night, catnaps off and on through the morning, spending about twenty-two hours each day in her bed.

What does she think? What’s going on in there?

I can’t imagine the loneliness.

A Conversation


“Yeah, Little?”

“I think I’m old enough to start to figure this stuff out now.”

I stared at the embers for a moment. The warmth of a rare fifty-degree day in northern Iowa in November had lost it’s battle with the evening chill, but I was loathe to give up the day just yet. I pulled my coat a little tighter, wishing I could pull the picnic table a little closer to the fire pit.

“What stuff, Honey?” I took a sip of my beer, wondering if we’d reached the point where the cooler was keeping the last can cold or if it was by now keeping it from freezing.

There was a pause from the other side of the table, then, “I’ve seen this happen before. I know I don’t have the best attention span, but I have a good memory. This happens every year, doesn’t it?”

“What happens, Little?”

“The trees. They look dead. But they’re not, are they – they’re just sleeping. They’ll come back again, won’t they, Papa.”

“Yep, the trees will come back in the spring,” I answered. Bonfires give a person a sense of calmness and patience that is increasingly rare in today’s world. “They always do.”

We sat together for a few moments, listening to the sound of nothing, the darkness gathering its strength from the shadows. We’re normally content with a comfortably silent companionship, but after a few minutes Little stirred again. “Papa?”


Slowly, “When the trees come back in the spring… Do you think we’ll all be here to see them?” She stared at the fire.

Another sip of beer. “What do you mean, Honey?”

“I’m old enough, Papa. I think I get it, sometimes. Things die in the winter.” She glanced at me, then back at the fire. “The trees, they come back, but some don’t. The plants, they come back, but some don’t.” She paused. “Every year it’s different.” A longer pause, then, “Will we all be here? In the spring?”

I took my gaze off the fire and looked at Little Buttercup. “Pretty deep questions for a five-year-old.”

She looked back at me, brown eyes wide in the firelight, “Some would say I’m almost 35.”

Papa and Buttercup

Papa and Buttercup

We stared at each other for a moment. I blinked first. “Am I really talking about mortality with a Golden Retriever?” Little Buttercup looked back at the fire, her silence an answer.

Bonfires, even in the chill of late November, bring a contemplative calm to conversations. We enjoyed a moment or two of quiet, thinking our thoughts. Then, from the puddle of fur at my feet, “I’m serious, Papa. I see what happens. I’m not as silly as you think. I need to know, will we all be here in the spring when the trees come back?”

“Oh, of course.” The words were uneasy.

“What about Nitty-Kitty,” she asked, lifting her head from her paws. “I worry about her.”

“So do I, Honey,” I said, “but she’s a tough little kitty.”

“She is,” agreed Buttercup, putting her head back on her paws, staring at the crackling logs. “I try to play chase with her sometimes but she never wants to play.” The fire crackled. “She’s so very small, but she doesn’t know it. Every night she’s out in the woods hunting. I worry sometimes she’ll try to fight that raccoon that lives up in Butterfly Corner and she won’t come back.”

“Me too.” We picked up little Nitty from a shelter years ago knowing she was a barn cat. Her silky black fur is stranded with silver now, but the fierceness of youth is undiminished. “But what can we do? Nitty lives to prowl in the woods – it’s what she loves. If she’s not here in the spring we’ll be sad, but we have to know that she’s doing what she wants to do.”

“Nitty’s tough.” A pause. “I hope she’s here when the trees wake up.”

We stared at the fire, my Golden Retriever and me, letting time slip through us. Then…


“Yeah, Little?”

Little Buttercup never looked away from the fire. “Papa, why doesn’t Mama ever come outside?”

“What do you think?”

The pup was quiet for a moment. “I don’t know. I remember her coming outside once. You were working by the garage and Mama came outside with her walker to talk to you, but she fell in the driveway on the rocks and hurt herself. I tried to help her, but she cried and you ran over and were all upset.” She sat for a moment. “The only time Mama comes outside is when you push her out in her chair and she gets in the car and you take her away. Then when you come home she gets out of the car and goes straight inside again in her chair. She never stays outside to play.” Two big brown eyes looked up at me. “I think maybe being outside hurts Mama? But how can that be?”

“You’re pretty smart for a dog,” I said. I picked up my beer. Empty. I reached into the cooler for the last can, hoping it wasn’t frozen. A sip, then, “You’re right, sort of. Mama gets sick real easy and she always hurts, so it’s not easy for her to come outside. She can’t walk much because it hurts and sometimes standing is hard for her. She wants to come outside and play with you, but it hurts her too much and sometimes the air makes her sick.”

“How can air make someone sick? I breathe it all the time.”

Mama's Last Outing

Mama’s Last Outing

“Oh, she has some allergies,” I answered. A glance at the Goldie Treever was enough to realize she didn’t understand. “There are invisible things in the air that make her sick.” My mind ran ahead, trying to get the words right. “And she has a disease that makes her so gets sick real easy from other people. You and me can come outside any time, and we can play with other people if we want, but Mama can’t. If she is around other people the germs – more invisible things that everyone has – will make her very, very sick. Everyone has germs and they don’t bother most people, but they make Mama really sick every time.” I picked up my frozen beer and took a sip.

Buttercup picked herself up and moved closer, still not quite looking at me. “I worry about Mama.”

“I do too, Little Buttercup.”

She leaned her head on my knee so I could scritch her ears. “When you take me in the car we only go for a little ride,” she said. “But when you take Mama in the car you’re gone a long time and I get lonely in my kennel. If Mama gets sick being outside and she can’t be around people, where do you go?”

“She has to go to her doctors every once in a while,” I answered, pulling my coat a bit tighter.

“What’s a ‘doctors?'”

“Oh, I’m sorry, Cupsy. I forget sometimes… Doctors are people who try to make Mama so she’s not sick all the time.”

“They must not be very good. She’s always sick.”

“Well, they try,” I said. “They’re really very smart people, and they want to help her. But it’s complicated.” I stared at the fire as I continued, “They don’t really know why she’s so sick all the time, and they only have a few minutes to see her so they don’t have time to really think about it much. But they do the best they can.”

“I wish they could do better. I wish Mama wasn’t sick. I wish Mama could throw the ball for me like you do sometimes when you’re not busy.”

Nothing hurts quite like honesty from an innocent you love. She continued, after a pause, “I wish you weren’t so busy all the time. You’re like those doctors – you only have a few minutes to see me.”

She let that hang in the air while she scratched her ear and I sipped my beer as time whirled inkily about us.

“Yeah, I’m sorry, Honey,” I said. “I wish I could spend more time with you, but I’m just busy.”


“Why what?”

“Why are you too busy to play with me?”

“Well, someone has to pay the bills,” I snapped. “Those damned doctors aren’t cheap. I have to keep working to pay for this you know.”

She sat up sharply. “Wait. You mean to tell me that there are people who can help Mama, but they’re too busy – and you have to pay them? So you’re too busy to play with me?” She paused. “WAIT A MINUTE!”

I waited whilst my pup cogitated.



“Okay, Papa. Explain this. Mama’s sick. People can help her, but they won’t unless you give them money?”


“So you have to ignore me – and Mama too! – to work to make money to pay these people?”


“If they want to help her why do they need money? Why don’t they just help her?”

“I wish it were that way, Honey,” I said. “But they need equipment and tools to help her, and they had to go to school for a long time to learn how to help people and that’s expensive.”

She flopped down on the ground. “But you said they don’t have time to help her much.”

“They don’t – they only have a few minutes to see her. They have to help a lot of people every day to make enough money to keep helping people, so they can only see people for a few minutes. Mama’s illness is something they can’t really help, but they try.”

“Monkeys are confusing,” said the canine. “These ‘doctors’ must be really poor and live in tiny shacks if they have to work that hard. Why don’t people just give them more money?”

“Oh no,” I replied. “Doctors are usually very rich. They have much, much more money than we do and usually live in really big houses.”

“I don’t get it. If they have a lot of money, why don’t they help people like Mama so she can be healthy and play with me?”

“I know, Honey. It’s confusing.” I sipped the last bit of my frozen beer. “The doctors want to help, but they can’t. Mama’s illness isn’t something anyone really understands. It’s expensive, but we have to keep seeing the doctors because insurance makes us.”

At this point the Golden Retriever named Little Buttercup stood up and stared me right in the eye. “Okay, monkey, what’s an ‘insurance’ and why won’t THEY help Mama?”

This is going to be difficult… “Okay, Honey. Insurance is something people buy to help when they’re sick. You pay in a little bit every month even when you’re healthy, then when you’re sick they give the money back so you can pay the doctors. It’s like a savings account in a way.”

She stared at me with a steadiness that was unnerving. “So this ‘Insurance’ thing make you take Mama to the doctors even though the doctors are expensive and can’t help Mama?”

“It gets worse, Cupsy.” I replied. “When I take Mama to the doctors she’s around other people. Remember how I said being around other people makes Mama sick? So when I take her to the doctors, they don’t help her much AND it makes her sicker every time.” I shivered as the fire waned. “But the insurance people won’t give Mama her money if she doesn’t go to the doctors because if she doesn’t they think she must not really be sick. So she has to go to the doctors and get sick and I have to work to pay their fees so insurance will believe she’s sick and will give her her money.”

She blinked at me. “Who in the world made up this system?”

“Beats me.”

“But wait! You said these insurance people give Mama the money she paid them when she’s sick.”


“So why do you have to work so hard that you don’t have any time to spend with me or Mama?”

“Because the insurance people took Mama’s money and decided to keep it. They say she’s not really sick so they shouldn’t have to give her money back. So we have a lawyer to fight the insurance people for us, but we have to pay the lawyer. So I have to work to pay the lawyer to get the money from the insurance people to take Mama to the doctors that can’t help her.”



“My head hurts and I’m sad now. Can we go inside?”

I shook my empty beer can. “Yeah.” I put my hand on her head and scritched her ears. “I hate to say it, Little, but there’s more. Tomorrow we’ll talk about how people around us elected a government that wants to take a different kind of insurance away from us altogether so Mama can’t have any medicine.”

“I hate to say it, Papa, but I think you monkey-folk are really weird.” She stood up with a yawn, picked up her tennis ball, and headed for the house. “At least wolves look after their own and care for their sick.” A pause as she trotted up the stairs and stood by the door, waiting for someone with thumbs to let her in.


“Yeah, Little?”

“Do you think Mama will see the trees? Will Mama be here in the spring?”

“Yeah, of course she’ll be here.”


“Yeah, Little?”

“Why are you crying?”

“Let’s just go inside, shall we?”

Past Perfect Future Tense

Such a simple request… But those are often the most difficult.

“I just want four photos of our doggies to hang in my office,” Beloved Wifey said to me a few months ago. Her slight Germanic accent makes every word sound just a bit more matter-of-fact than normal. But I immediately knew this was going to hurt…

I work as a photographer. Our dogs are my test subjects quite often… I’ve got, literally, tens of thousands of photos of our critter-kids, none of them labeled or tagged in any way. This was going to mean some serious digging for me… And I know my Wifey – she doesn’t want the best photography I have, she just wants fun pictures of the dogs. Snapshots. Photos that are attached to memories. This is gonna hurt…

Sure enough, I started to dig through the photos I have on file. Finding photos of Little Buttercup, our now-two-year-old happy-go-lucky Golden Retriever, only took a few minutes. But to find photos of our older Zoey-doggie took the rest of the afternoon…

Zoey-Dog in February, 2012

Zoey, 2012

I worked my way backwards in time. Tons of photos of Zoey and Buttercup playing around on our acreage, but Zoey’s almost always looking down, her sniffer to the ground, following a neat smell around… “Chipmunk? No, this is eau de squirrel…” I smile my way back through Buttercup’s puppy days – she was SO damned cute! I start to get a bit of a catch in my throat when I see a photo of our kitty, Fruitloop, looking frail and thin… He only lived here at the acreage for a little less than a year before he passed. Diabetes.

The photos of the days and months right after we moved to our dream home out here on our acreage, what full memories! A picture of Beloved Wifey poking about hopefully at a garden we’d planted… That stabbed, somehow. It took me a minute to realize why. She’s not been healthy enough to go outside for a few years now other than to get back and forth to the car. I’d sort of forgotten that at one point she went outside a bit… It looked wrong, in the photo, to see her outside, so far from the house.

Backwards to our old house in Sioux City… I’m skimming through the photos, looking for photos of Zoey-Dog, but I couldn’t help but notice patterns. Right there. In this period right here, between 2009 and 2010. A transition. Before about 2009 all the photos of Zoey Dog included a smiling Beloved Wifey, and were entitled “A Day in the Park,” or “A Walk By the River.” A happy woman with a happy doggie in a happy park.

Dagmar and Zoey, 2008

Dagmar and Zoey, 2008

After that date, the photo albums were titled things like, “House,” or “Acreage,” and didn’t have Beloved Wifey in them. Just a dog.

I kept going back… Almost every day a happy Beloved Wifey took a happy Zoey Dog out for a walk, and almost every day I took pictures. I hated my life back then – I was working a dead-end job, playing in a band with tensions, living in a cozy little house in the bad part of town, watching the homeless and unfortunate folks walking by day after day, hoping I wouldn’t ever be one of them.

The happiest dog in the world is the one who just found a Forever Home.

The happiest dog in the world is the one who just found a Forever Home.

I took my photo tour all the way back to the first day we got Zoey. We have about twenty photos of that day of smiling people petting a trepidatious but hopeful doggie taken with a cheap camera… Tears rolling down my face I looked back at photos of Beloved Wifey proudly and nervously walking the dog for the first time. Outdoors.

She used to dance barefoot in the snow every New Years’ Eve, talking on the phone to her Mama and her relatives back in Austria. We used to go watch bands play and dance until two in the morning. She used to walk the dog every single day while I sat at my computer working. (Boy, could I kick myself in the ass now for missing those mundane moments.) They’d come home, stomping snow off their feet as I’d drop towels and offer warm drinks. When I was in a band, Beloved Wifey would come and watch me until closing time, sometimes sitting by herself for hours in dive bars, but always there…

I’d forgotten all that.

Life for Beloved Wifey now is to wake up around 5:30 each morning, do her medical treatments and routines until 7:15, then out the door to work. At 4:30 she leaves work, by 5:15 she’s home and in the shower. By six she’s in bed, doing more medical treatments, asleep by seven. I see her about twenty minutes a day – and I’m always engrossed in some work problem and can’t seem to find the time to really talk. Work. Dishes. Laundry. Work. Mowing. Something always must be done. I’ll see her face peering out her bedroom window sometimes, “I vas vatching you, it’s fun how you play with the doggies. The frisbee is behind the shed… The vind blew it back there while you weren’t looking.” Tubes trailing from her midsection to a plasma pump… “Be sure to give Miss Mittens some tuna. Nitty-kitty will steal it all if you don’t vatch her close.”

Dancing in Omaha in 2007

Dancing in Omaha in 2007

I see a picture of her dancing back in 2007. I’d forgotten about those days when we’d go places, see people, there were no face masks, no tubes.

I sort of hope she has, too.


A Few Facts About CVID

We found a rather concise explanation of CVID on Yoni Maisel’s blog. I’ve altered it just a bit, but he did the work:

What It Is: 
Common Variable Immune Deficiency – A rare, genetically determined, immune-system disorder. The cause remains unknown.

Is it common:
No. Approximately 1 in every 50,000 individuals have the disease according to the National Institutes for Health.

What People With CVID Lack:
Life-saving and life-sustaining antibodies. Those with CVID simply do not produce them in sufficiently effective amounts or at all.

Living in Fear:
People with CVID fear pathogens of all types: bacteria, fungi, parasites, protozoals, and more. They realize that pathogens which might only affect others mildly have potentially life-threatening repercussions for them.

Conditions Associated With CVID:
There are numerous secondary auto-immune diseases and a highly-increased predisposition to various types of malignancies associated with CVID. Lymphoma, for instance, has an 11-fold increase in CVID – that’s 1100%. Those with CVID often have respiratory problems as well, such as severe, uncontrolled asthma. 

Delay in Diagnosis:
According the the Immune Deficiency Foundation (IDF), there is an average delay in diagnosis of 12 years from the onset of symptoms, with nearly a quarter of patients experiencing a delay of over 20 years. This, unfortunately, represents a very protracted period of being ill with nobody knowing quite what is wrong, and can lead to permanent, irreversible damage. It usually takes a very perceptive and proactive physician or specialist to connect the dots and follow up with diagnostics.

What Keeps CVID Sufferers Alive:
Regular intravenous or subcutaneous infusions of immunoglobulin (antibodies).

How to Remain Healthy:
People with CVID depend upon antimicrobials: antibiotics, anti-fungals, anti-parasitics, etc. They tend to be on these agents quite often and for longer durations than our “normal” counterparts. (This can often lead to even more health issues.) 

Who Figured This All Out?
Dr. (Colonel) Ogden Bruton (1908-2003) – pediatrician and Chief of Pediatrics at Walter Reed Army Hospital. Although he discovered the related PI disorder “agammaglobulinemia,” Dr. Bruton was the first researcher to uncover Primary Immunodeficiency Disorders.

The Face of Immunodeficiency
David Vetter, the Original “Bubble-Boy”. Although what David suffered from, Severe Combined Immunodeficiency, is somewhat different than CVID, David has become the everlasting face of Primary immune Deficiency.

Daily Lifestyle:
Aside from suffering more frequent illnesses than the normal population, CVID patients probably don’t live much differently than most others… but with far more caution and awareness of risks and dangers to our immune-system health. When our immunoglobulin levels are high (post-infusion) we tend to be rather “normal.” When they are down, we tend to be fatigued and very susceptible to illnesses.

Life Expectancy:
According to the Mayo Clinic, with proper treatment a 20-year survival rate is 64% for male patients and 67% for female patients. 

Where Does The Future Lie:
Probably in adult stem-cell technology which has already made significant inroads in the treatment of the Primary Immunodeficiency Disorder Severe Combined Immunodeficiency (SCID).