My life has changed in so many ways in the last two years, and writing fell to the wayside for a while. I hope to return to it soon. In the meantime, my dearest grandmother sparked my dormant writing muse, and I wrote this in early February for her as she was dying. She died on February 10th. I was able to visit her before she passed, and it was beautiful to see her being greeted by her loved ones on the other side. I hope it conveys how much she meant to me.
How do I put my feeling into words on a page? How do I qualify or quantify what you’ve meant to me? How do I really tell you how amazingly special you’ve been in my life? My instinct is to tell you what I learned from you. I feel that a person’s life is valued in their interactions with others. So here goes, Gram.
With each memory of you, I learned a life lesson. Honestly, I don’t think I truly realized those lessons until I became a mother and began my own teaching of the next generation. Coupled with their associated memories, here are 10 lessons I’ve learned from my time with you.
1. Some of my earliest memories are of you, with you. The fuzziness of early childhood veils your words then, but my memory is filled with heartwarming love. Your hushed voice whispering, lulling me in love and pulling me to dreamland. I wasn't an easy kid to put to sleep. I still use the memory of your whispers to help me sleep. I always wondered if you liked caring for a little girl after your three sons. I was all pink and frilly.
Lesson: Quieting the mind elicits sleep.
2. Your little yellow house in Wilmington, IL is a bit vague for me. What I do remember is the car ride from Milwaukee. I felt like it took forever to get to you and Grandpa. My life, at that point, was kindergarten and every other weekend in Wilmington. We'd gather around your table, beginning with a prayer, and enjoy each other's company. I'd beg to sit next to you on the step stool chair. I would try to sit like you, and often admired your dainty pink floral coffee cup. You made me feel special with my own set of fancy silverware and a tiny jelly jar glass. All the while, Grandpa would be telling me to eat my “wetchtables” as he had a glimmer in his eye. Sometimes, I’d catch the smiles between you two when you felt Kristen and I were being cute.
Lesson: Helping children feel that they are part of the adult world is empowering for them, and sitting around a table nurtures family togetherness.
3. There were other times when we would visit Wilmington, and we’d find you and Grandpa at the Ben Franklin store. I would literally twitch with movement in the car thinking about jumping on the black mat to magically open the glass door of the store. I’d sprint to the penny candy, dig in, and dump my loot into a white paper bag. I felt as if I owned the place, and loved it when the cashiers would smile and wave to me. Chewing on my latest treat, I’d run to the side of the store to the office. I’d climb up the steps and enter your work world. Your desk seemed oversized, and I gleefully punched the buttons on your adding machine pretending to be you. You greeted me with love, hugs, and grins. It was the best feeling for me. Your seeming unending love blanketed me.
Lesson: Girls can grow up to be working women, contributing to society and maintaining their independence.
4. Soon after, Grandpa and you sold the store and moved to Oxford, WI to be farmers. For me, the farm provided so many lessons and wonderful memories of time with you. The farm was solace for me. It was the one place in my childhood where I could go and be completely free of stress and worry. From the old wood stove burning and glowing orange from its cracks in the night to the expanse of old oak trees in the woods, I explored what felt like every inch of the farm. You would often take us out to the field in Big Foot to go visit Grandpa working or to pick choke cherries or berries for jam. I loved sitting directly next to you as you drove, and would wait to hear your infectious laugh as we’d bump along through the ruts in the road out to the field. I saw the glimmer in your expression as you’d accelerate to make each big bump even bumpier.
Lesson: Sometimes you have to accelerate to amplify the effect of living.
5. My childhood memories of you are often of sounds and textures I experienced. I can vividly hear Grandpa snoring on the couch for an afternoon nap while the latest football game was being called in the background. You often would keep us quiet at the kitchen table with rounds and rounds of Gin Rummy or Crazy 8s. Whispering directions as we played, keeping the cheating to a minimum, encouraging the following of rules, and sometimes losing so one of us could win. The too-big-for-me cards in my hand made me feel like I had made it in the world. I was a big girl, like you.
Lesson: Sometimes you win, and sometimes you lose. It really does matter how you play the game.
6. For this city girl, the farm provided me with immeasurable respect for the food I ate. It was where I learned that the grocery store didn’t magically make meat or offer fruits and vegetables. You and I spent hours picking strawberries in the patch, and gathering eggs from the coop. I never liked going in that coop, but you firmly guided me. “Just reach in and grab the eggs. The quicker, the better.” I loved holding the chicks each spring, and watching them sprout white feathers. You encouraged Kristen and me to name the cattle, or cows as we called them. Thus began the litany of cows with M names, inspired by 1970s TV shows: Mork, Mindy, Mickey, Minnie, Mandy, Maude, etc…. I vividly recall the day I realized that Mindy was on the table for dinner. I cried myself to sleep that night.
Lesson: The food chain is not fair, but life enabling.
7. The farm was a place to work, but where you could also have fun. I often climbed the ladder to the hay loft, and promptly became freaked out by the barn swallows scattering. You’d shout, “Be careful up there, Jenny.” I loved looking out the conveyor opening and feeling like I was above it all. I spent countless hours in the woods, dodging pies the cows left as I went, and touching every tree I could find. I stood teetering on fallen tree trunks, peering up at the green canopy, enjoying the shade in the summer’s heat. The rope swings Grandpa hung from the tree in the yard were where I’d watch you weeding the garden, digging up potatoes, or picking vegetables. Pumping my legs to go higher and higher, I can still feel the sun hit my face as I’d swing high enough to leave the cool shade of the tree. That swing was also where I’d sit mesmerized looking out over the fence lining the field and see the gorgeous wild flowers you’d plant on the fence line. The daisy pin wheel also spinning in the breeze, cast against the blue sky scattered with cottony clouds.
Lesson: Nature provides peace and calm to souls that are stressed, and even if you try your hardest, sometimes you step in a cow pie.
8. While visiting you at the farm, we’d end up at St. Ann’s on Sunday mornings in Brooks. With Father Kelly leading and Grandpa ushering, you’d bring little items to keep us entertained and quiet in our pew. Those pink and white circular mints you had buried in tissue inside your purse still make me think of you when I see them in stores. Kristen and I often vied to sit on your lap during mass. Kneeling next to you to pray was always comforting. I enjoyed seeing your rosary wrapped around the back of your hand. It was hard to resist the little girl urge to wear it as a necklace. Afterward, you often introduced me to your friends in the parish, “These are our granddaughters, Jenny and Kristy. They are here visiting from Milwaukee.” I ate up the attention it brought. “Oh what a pretty dress you have on! Look at all of that beautiful red hair!”
Lesson: Getting on your knees to ask for God’s grace is fulfilling, and church provides a sense of community.
9. Eventually, it was time for you and Grandpa to retire from farming and make the move to Rugby, ND. I think I was about 12 then. That last visit to the farm, I made a conscious effort to memorize its layout and touch all my favorite spots again. I climbed to stand on the lower rung of the white fence around the barn to peer inside at the cows drinking water. I stood in the breezeway between the old house and the new house to feel the wind wash over me on its way to the back pasture. I pumped my legs long and hard on that rope swing, and spun until I was dizzy in the tire swing. I ran to the rain meter to check out how much rain the crops had gotten. I walked down the road leading to the field to pick berries. I stood inside the barn and listened to the barn swallows tweeting and the cattles’ breath huffing. One last time, I heard Grandpa say, “Come boss. Come boss.” And, I heard your voice shout out to us from the breezeway, “Come on in. Dinner’s ready.” I was so sad to drive away from the farm that last time. I knew I’d miss it for the rest of my life, and that is still true.
Lesson: Life brings change. Enjoy what you have, when you have it.
10. Your move to North Dakota meant I didn’t see you as often. I had to rely more on my memories of you and the occasional phone call. Time and distance separated us, but you got to return to your home town, where you likely learned many life lessons too. During this time, I grew up, and went out into the world. Even with the distance, my love for you never waned. It has stayed with me, and will be forever a foundation upon which I built my family. So as you are preparing for the journey into never ending life, I want you to know that your influence, guidance, and love prepared me for my own life journey. I thank you for every hug and kiss I received, every scolding I needed, and every time you shared your wisdom and experience with me. Having you in my life is immeasurable to value. I love you, Gram.
Lesson: You can never have too much love.
Sunday, June 03, 2012
Monday, October 17, 2011
Meghan Lost Her First Tooth
At nearly 7 1/2 half years old, Meghan finally lost her first tooth on October 12th. She lost her right lower front tooth. She was very excited, although sad as she is a thumb sucker. We've been working with her to wean herself off as her adult teeth come in. I remember how it felt when I had to stop sucking my index and middle finger on my right hand. Poor Meggie! It is her way of self-soothing. (And to those who've said this...No, it isn't bad parenting. Children need all ways of self-soothing. Life for Meghan was extremely hard in the beginning as she was a micro-preemie. I vividly remember Megs inside her craddle head turned to the left with her thumb inside her mouth happily slumbering. It melted my mommy heart.)
Anyway, Meghan lost her tooth during a Brownie meeting. The tooth fairy brought her $5, plus $1 for her big sister, Grace, so she wouldn't feel bad. ;) Dad Charlie said that the tooth fairy found them just fine on their trip up to the South Shore of Lake Superior.
My baby...she is growing up so fast now.
Monday, January 17, 2011
Faith
"Faith is taking the first step even when you don't see the whole staircase."
~ Martin Luther King, Jr.
~ Martin Luther King, Jr.
Saturday, January 08, 2011
Ed Brailey In Tribute
When I met Ed in 2005, I was a newbie to the Alpha-1 community. He and I were elected to the board of the Association that year. Previously, I had read his story in the Foundation's Alpha 1-to-1 magazine.
I was touched by his amazing dedication to give back to the COPD, transplant, and Alpha-1 communities. As a mama to two beautiful little girls who have Alpha-1, I was always looking for inspiration. Ed was pure inspiration to me.
In fact, the day we met I will never forget. He sat down next to me and said, “Jen, tell me about yourself, and about those beautiful girls I’ve heard so much about.”
That conversation created a connection.
And that, to me, is the epitome of our precious Ed Brailey – connecting Alphas one-by-one using his subtle charm and unpretentiousness.
When Alpha-1 came into my life, I felt very alone. There were not many parents of Alpha-1 children that I could find at that time.
Ed helped reassure me as I doubted how much of an impact I could make or how much I could help.
I remember the smile that grew upon his face as he said, “Jen, keep up the great work. We need to bring the kids into our group. You being here will help draw out the other parents. We’ll do it together, Jen!”
I couldn’t help but smile in return.
Ed may not have realized how much he cemented my connection to our community, but I know one thing for certain.
I promise to continue the mission until every last Alpha gets the cure.
So thank you, Ed! Our world has an Ed Brailey-sized hole in it. Your dedication, perseverance, and amazing service will be missed.
Long live the Alphas!
God speed, Ed!
I was touched by his amazing dedication to give back to the COPD, transplant, and Alpha-1 communities. As a mama to two beautiful little girls who have Alpha-1, I was always looking for inspiration. Ed was pure inspiration to me.
In fact, the day we met I will never forget. He sat down next to me and said, “Jen, tell me about yourself, and about those beautiful girls I’ve heard so much about.”
That conversation created a connection.
And that, to me, is the epitome of our precious Ed Brailey – connecting Alphas one-by-one using his subtle charm and unpretentiousness.
When Alpha-1 came into my life, I felt very alone. There were not many parents of Alpha-1 children that I could find at that time.
Ed helped reassure me as I doubted how much of an impact I could make or how much I could help.
I remember the smile that grew upon his face as he said, “Jen, keep up the great work. We need to bring the kids into our group. You being here will help draw out the other parents. We’ll do it together, Jen!”
I couldn’t help but smile in return.
Ed may not have realized how much he cemented my connection to our community, but I know one thing for certain.
I promise to continue the mission until every last Alpha gets the cure.
So thank you, Ed! Our world has an Ed Brailey-sized hole in it. Your dedication, perseverance, and amazing service will be missed.
Long live the Alphas!
God speed, Ed!
Sunday, January 02, 2011
I'm a Preemie
"Meghan! Meghan! Earth to Meghan!" I said impatiently.
"Hey Megsy Rose! Are you listening to me? Can you please get your pajamas on?" I sighed as more attempts at saying her name in different cadences and tones met no response.
Silence from Meghan ensued as she continued scanning the bathroom with intense interest. I was standing right next to her. Her towel was wrapped around her as she peeked behind the closed shower curtain. I could feel my impatience growing.
In my thoughts, I heard, "Oh come on kid! Really? Seriously? You can't hear me at all. Really? Good grief!"
And then my thoughts leaped from my mouth, "Gosh, you have such A.D.D."
Of course, she suddenly heard that.
"I have what Momma?"
"Whoops," I thought.
"Um. Oh. Well, ADD stands for attention deficit disorder. It means you have trouble paying attention," I stammered.
And then my former micro-preemie, smart-as-a-whip, daughter said, "I'm a preemie, Mom."
As I searched my thoughts for a response, I couldn't believe that Meghan had grown up enough to deduce that former prematurity and ADD might go together. "Well, Megs, you used to be a preemie. If you were still a preemie, you would be living in a "box" in the hospital still growing. You are a big six-year old girl now. You don't live in a box (isolette)."
"Why was I in the box? I don't remember the box," she inquired.
"Well Meghan, when you were born 13 weeks early, you body wasn't quite ready to be born yet. The box kept you warm and safe. Your body still had a lot of growing to do, and the box was kind of like my tummy was for you. One thing that wasn't done growing yet was your lungs. We had to wait for your lungs to grow before we could bring you home." As I answered, I helped her into her pajamas to expedite the process.
"Oh. OK. Why did I come out early?"
"Remember that I was very sick. I had preeclampsia, which is kind of like having an allergy to having a baby in your tummy."
"Did you have a runny nose and sneeze, Mom?"
Her question elicited a smile across my face. "No, not an allergy like that. My blood went way too fast in my body (high blood pressure), and then my kidneys began to shut down."
I took my index finger and pointed to her lower back. "This is where you have kidneys. There are two in your body, and they clean the bad stuff out of your blood. That bad stuff gets kicked out through your pee. Anyway, when I had you in my tummy, my kidneys didn't want to work anymore. If my kidneys stopped working, I would have gotten too sick to keep you alive. So, the smart doctors said they would take you out of my tummy using surgery so my preeclampsia would go away."
"Oh, where did I come out again?"
As I pointed to my lower stomach, I said, "Right here."
"Where did Grace come out?" Her older sister was born by c-section due to preeclampsia as well, but just six weeks early.
"In the same spot, Megs."
"What did I do when I came out?"
"Well, I was having a surgery but your daddy told me that you mewed like a kitten. That was before they put the tube in your lungs to help you breathe," I responded.
"But I could breathe when I was born, right?"
"Yes, you could, but you were too little. You got so tired from breathing that they helped you with the breathing machine until your lungs grew stronger."
"Oh," she dryly remarked with what seemed to be understanding.
"Hey Mom! Do you think we could go visit where I lived in the box?"
"Sure, Megsy. We could do that."
"Yeah, I wanna see where I used to live. Do all the preemies have 'tension order too?"
"I don't know if they all have attention deficit disorder, but I remember your preemie doctor told us that it is really common for babies born 13 weeks early. You aren't the only one. Momma had ADD growing up, and Uncle Tim, too. People born not-too-soon can have ADD, too. I was born 1 week late."
"Oh."
"Just remember that having problems with paying attention does not mean you can't pay attention, Meghan. Momma learned to do it. Uncle Tim learned how as well. You just need to try to listen. Remember to listen. As you grow up, it will be easier."
"I know, Mom. You always tell me that kids are still learning how to be good people. I'm learning. I just need to practice."
God, I love that kid. Amazing...
"Hey Megsy Rose! Are you listening to me? Can you please get your pajamas on?" I sighed as more attempts at saying her name in different cadences and tones met no response.
Silence from Meghan ensued as she continued scanning the bathroom with intense interest. I was standing right next to her. Her towel was wrapped around her as she peeked behind the closed shower curtain. I could feel my impatience growing.
In my thoughts, I heard, "Oh come on kid! Really? Seriously? You can't hear me at all. Really? Good grief!"
And then my thoughts leaped from my mouth, "Gosh, you have such A.D.D."
Of course, she suddenly heard that.
"I have what Momma?"
"Whoops," I thought.
"Um. Oh. Well, ADD stands for attention deficit disorder. It means you have trouble paying attention," I stammered.
And then my former micro-preemie, smart-as-a-whip, daughter said, "I'm a preemie, Mom."
As I searched my thoughts for a response, I couldn't believe that Meghan had grown up enough to deduce that former prematurity and ADD might go together. "Well, Megs, you used to be a preemie. If you were still a preemie, you would be living in a "box" in the hospital still growing. You are a big six-year old girl now. You don't live in a box (isolette)."
"Why was I in the box? I don't remember the box," she inquired.
"Well Meghan, when you were born 13 weeks early, you body wasn't quite ready to be born yet. The box kept you warm and safe. Your body still had a lot of growing to do, and the box was kind of like my tummy was for you. One thing that wasn't done growing yet was your lungs. We had to wait for your lungs to grow before we could bring you home." As I answered, I helped her into her pajamas to expedite the process.
"Oh. OK. Why did I come out early?"
"Remember that I was very sick. I had preeclampsia, which is kind of like having an allergy to having a baby in your tummy."
"Did you have a runny nose and sneeze, Mom?"
Her question elicited a smile across my face. "No, not an allergy like that. My blood went way too fast in my body (high blood pressure), and then my kidneys began to shut down."
I took my index finger and pointed to her lower back. "This is where you have kidneys. There are two in your body, and they clean the bad stuff out of your blood. That bad stuff gets kicked out through your pee. Anyway, when I had you in my tummy, my kidneys didn't want to work anymore. If my kidneys stopped working, I would have gotten too sick to keep you alive. So, the smart doctors said they would take you out of my tummy using surgery so my preeclampsia would go away."
"Oh, where did I come out again?"
As I pointed to my lower stomach, I said, "Right here."
"Where did Grace come out?" Her older sister was born by c-section due to preeclampsia as well, but just six weeks early.
"In the same spot, Megs."
"What did I do when I came out?"
"Well, I was having a surgery but your daddy told me that you mewed like a kitten. That was before they put the tube in your lungs to help you breathe," I responded.
"But I could breathe when I was born, right?"
"Yes, you could, but you were too little. You got so tired from breathing that they helped you with the breathing machine until your lungs grew stronger."
"Oh," she dryly remarked with what seemed to be understanding.
"Hey Mom! Do you think we could go visit where I lived in the box?"
"Sure, Megsy. We could do that."
"Yeah, I wanna see where I used to live. Do all the preemies have 'tension order too?"
"I don't know if they all have attention deficit disorder, but I remember your preemie doctor told us that it is really common for babies born 13 weeks early. You aren't the only one. Momma had ADD growing up, and Uncle Tim, too. People born not-too-soon can have ADD, too. I was born 1 week late."
"Oh."
"Just remember that having problems with paying attention does not mean you can't pay attention, Meghan. Momma learned to do it. Uncle Tim learned how as well. You just need to try to listen. Remember to listen. As you grow up, it will be easier."
"I know, Mom. You always tell me that kids are still learning how to be good people. I'm learning. I just need to practice."
God, I love that kid. Amazing...
Tuesday, December 28, 2010
Thursday, December 02, 2010
All the Right Words?
During our nightly prayers, I added a new name to the very lengthy list of angels and those battling illness for which we pray. Meghan peered up at me from her bed with love on her face along with the call of sleep in her eyes.
“Megsy, let’s add a new name to our list for prayers tonight. Mommy’s Alpha friend, Ed, needs some prayers.”
“What happened to him, Momma?”
“Well, he is an adult with Alpha-1, like you. I think it was about 7 years ago that he received a new lung. They took out one of his old, tired Alpha-1 lungs, and gave him a new one. He liked it very much that he could breathe easier again. But now, his liver is pretty sick from his Alpha-1. He needs some prayers so maybe God will help his liver get better,” I replied.
As she listened, I could see the questions rising up inside of her. Her facial expressions revealed her brain was inquiring, pondering, and pensively beginning to analyze how she was like Ed. I thought, “Aha. There it is. She is beginning to realize what Alpha-1 might mean to her.”
“Mommy, what is wrong with Mr. Ed’s liver? What happened to it? I hope he gets better soon,” she whispered with a bit of fear in her voice.
“Well honey. Ed has Alpha-1 like you and Gracie. Remember how I told you that you have a liver on the right side of your body underneath your ribs?”
“Uh huh.”
“Well, when you have Alpha-1 Antitrypsin Deficiency…”
“What? Is that the real name for it?” she interrupted.
“Yup. It is. That’s a mouth-full, isn’t it?” As she nodded her head, I added, “All people have a liver which makes something called bile to help digest your food, but your liver also makes really important things called proteins and enzymes which are used in other parts of your body.”
“Oh yeah. I ‘member that Alpha-1 is a protein. You told me that a different time.”
“Yes, I did. So when you have Alpha-1 deficiency, there is something that happens inside your liver that isn’t so good. Your liver is really great at making the Alpha-1 proteins, but then something goes wrong. The way your liver makes your Alpha-1 proteins is not quite right, and they get stuck inside your liver. It is like being stuck behind a locked door that you can’t open or go through. Sometimes, though some of that Alpha-1 sneaks through the cracks around and under the door.”
“It is sneaky,” she exclaimed.
“Well Momma wishes it were sneakier. Gracie and you don’t have very many Alpha-1 proteins floating around in your blood where they should be. That is why we have to take very good care of your lungs. Remember that your blood should bring Alpha-1 proteins made by your liver into your lungs where they are important. Alpha-1 proteins are really cool little helpers in the lungs.”
“How do they help, Mom?” she asked as she wrinkled her brow and scrunched up her nose.
“I know we’ve talked about how our bodies are really great at kicking out the bad stuff that makes us sick. That is called your immune system. Well, Alpha-1 proteins are helper parts of your immune system.”
“How do they help my immune system, Momma?” I could feel my heart starting to pound a little harder as my anxiety about the topic began to increase. I thought that I had better put more of a smile on my face so I would not bias her learning or pass on some of my fears from her and her sister having Alpha-1.
With my right hand, I made a sign language letter C. “Well Megsy, when you breathe something yucky into your lungs like a germ or dust, your lungs “ask” a different kind of helper called a neutrophil to kick out that bad stuff. Neutrophils are like hungry little fish.”
I took my “chomper” hand and pretended to munch down on bad things in the imaginary land of the lung. “Neutrophils work like this. They eat up the germs and dust and keep your lungs really nice and clean so you can breathe. There is a whole army of neutrophils that clean up your lungs. That is normally a really good thing, but when you have Alpha-1 deficiency, there is a problem.” I took my right “chomper” hand and pretended to chomp down on my left fist.
“Munch. Munch. Munch. Alpha-1 is kind of like having an army of really big fish, bigger than the neutrophils. Those Alpha-1 “fish” go into your lungs when the neutrophils are done cleaning up the bad germs and dust in your lungs. Then, something cool happens again. (I outstretched my arms and pretended to use them to chomp up imaginary neutrophils in the air.) Alpha-1 takes its really big chomper arms and catches all the neutrophils before they eat up the good parts of your lungs that aren’t germs or dust. Does that make sense, Megs?”
“So Alpha-1s grab all the neu, neu, neu…”
“Yes, neutrophils. And do you remember what those neutrophils eat up in your lungs?”
“Yeah. They eat up the germs and yucky stuff we breathe.”
“Woohoo! You are a smart girl and good listener, Megsy Rose. But here is some bad news, and I wish I could change it for you. I wish I could take away Alpha-1 from you and all of our Alpha friends.”
“What’s wrong Momma?” she inquired with concern on her face.
“Well honey, your liver doesn’t make Alpha-1 proteins the right way. They get stuck behind the “door” of your liver and can’t get out except through the cracks a tiny bit. So, your lungs might get slowly damaged over many years. We really don’t know what will happen so we do our best to take care of your lungs and hope your liver doesn’t get very sick again. Both you and Grace had sick livers when you were babies, but then your livers got a bit better.”
“Why doesn’t my liver make Alpha-1s the right way?” she seemed to ask in protest.
“Because you were born that way. God made you and when you were made, you were given a liver that doesn’t make Alpha-1 the right way. But you need to remember something really important. Every person born has something wrong with them.”
“In their genes, Mom?” she guessed.
“Absolutely right Meghan Rose! You are so smart to remember that our genes are like maps for what might happen to our bodies as we grow. Not everyone knows what genes they have. In fact, we only know that you have the gene for Alpha-1, but we don’t know all of your genes. So we will do our best to take care of your lungs and liver because we know about your Alpha-1 gene.”
“Like stay inside on oze actshun days?”
“Yes. When it is an ozone action day, you and Grace must stay inside in the air conditioning so you don’t breathe that yucky air. If you did, the neutrophils would come marching into your lungs to eat up the yucky stuff, but then those neutrophils would keep eating and eating up the good parts of your lungs because too little Alpha-1 is in your lungs and can’t munch up all those neutrophils. So we just try really hard to keep those bad things from getting into your lungs in the first place.”
“The Alpha-1s can’t get that door open in my liver, huh?”
“Right Sweetie! It sometimes sneaks through the cracks of the door, but most of the time, it just stays inside your liver causing it to be just a little bit sick. Whenever the Alpha-1 gets stuck inside the teeny-tiny parts of your liver called cells, that Alpha-1 gets all mad and angry. It “throws a fit” and messes up that cell by making it swell up like water that goes into a water balloon. For some reason though, your liver is lucky. It seems to be able to do something called regenerate. That is a big word for fix itself. It keeps rebuilding your cells.”
“My liver is fixing itself when the Alpha-1 throws a fit?”
I could feel myself becoming elated that she was actually following along on the convoluted path that I had formed off the top of my head trying to explain Alpha-1.“Yes. It is. Gosh Meghan. Does this really make sense to you?”
“Yes. I think I get it Mommy, but I can’t ‘member all the right words yet.”
“Megsy Rose, you don’t need to remember all the right words. You just need to know that we love you very much, and that we’ll do our best to take care of you and your Alpha-1.” I felt myself take a very long, deep breath as I seemed to be holding it during most of the conversation.
“Ok, Mommy. So is Mr. Ed’s Alpha-1 throwing a fit inside his liver?”
“Yes, it is. Let’s pray his liver can fix itself again. Sometimes that doesn’t happen. He might need to get a new liver from someone who goes up to heaven and doesn’t need it anymore.”
“God bless Mr. Ed,” she concluded. I couldn’t help but feel enormous pride in her realization, but also some sadness as she figured out her Alpha-1 can cause bad things. God bless Meghan and her sister, Grace.
“Megsy, let’s add a new name to our list for prayers tonight. Mommy’s Alpha friend, Ed, needs some prayers.”
“What happened to him, Momma?”
“Well, he is an adult with Alpha-1, like you. I think it was about 7 years ago that he received a new lung. They took out one of his old, tired Alpha-1 lungs, and gave him a new one. He liked it very much that he could breathe easier again. But now, his liver is pretty sick from his Alpha-1. He needs some prayers so maybe God will help his liver get better,” I replied.
As she listened, I could see the questions rising up inside of her. Her facial expressions revealed her brain was inquiring, pondering, and pensively beginning to analyze how she was like Ed. I thought, “Aha. There it is. She is beginning to realize what Alpha-1 might mean to her.”
“Mommy, what is wrong with Mr. Ed’s liver? What happened to it? I hope he gets better soon,” she whispered with a bit of fear in her voice.
“Well honey. Ed has Alpha-1 like you and Gracie. Remember how I told you that you have a liver on the right side of your body underneath your ribs?”
“Uh huh.”
“Well, when you have Alpha-1 Antitrypsin Deficiency…”
“What? Is that the real name for it?” she interrupted.
“Yup. It is. That’s a mouth-full, isn’t it?” As she nodded her head, I added, “All people have a liver which makes something called bile to help digest your food, but your liver also makes really important things called proteins and enzymes which are used in other parts of your body.”
“Oh yeah. I ‘member that Alpha-1 is a protein. You told me that a different time.”
“Yes, I did. So when you have Alpha-1 deficiency, there is something that happens inside your liver that isn’t so good. Your liver is really great at making the Alpha-1 proteins, but then something goes wrong. The way your liver makes your Alpha-1 proteins is not quite right, and they get stuck inside your liver. It is like being stuck behind a locked door that you can’t open or go through. Sometimes, though some of that Alpha-1 sneaks through the cracks around and under the door.”
“It is sneaky,” she exclaimed.
“Well Momma wishes it were sneakier. Gracie and you don’t have very many Alpha-1 proteins floating around in your blood where they should be. That is why we have to take very good care of your lungs. Remember that your blood should bring Alpha-1 proteins made by your liver into your lungs where they are important. Alpha-1 proteins are really cool little helpers in the lungs.”
“How do they help, Mom?” she asked as she wrinkled her brow and scrunched up her nose.
“I know we’ve talked about how our bodies are really great at kicking out the bad stuff that makes us sick. That is called your immune system. Well, Alpha-1 proteins are helper parts of your immune system.”
“How do they help my immune system, Momma?” I could feel my heart starting to pound a little harder as my anxiety about the topic began to increase. I thought that I had better put more of a smile on my face so I would not bias her learning or pass on some of my fears from her and her sister having Alpha-1.
With my right hand, I made a sign language letter C. “Well Megsy, when you breathe something yucky into your lungs like a germ or dust, your lungs “ask” a different kind of helper called a neutrophil to kick out that bad stuff. Neutrophils are like hungry little fish.”
I took my “chomper” hand and pretended to munch down on bad things in the imaginary land of the lung. “Neutrophils work like this. They eat up the germs and dust and keep your lungs really nice and clean so you can breathe. There is a whole army of neutrophils that clean up your lungs. That is normally a really good thing, but when you have Alpha-1 deficiency, there is a problem.” I took my right “chomper” hand and pretended to chomp down on my left fist.
“Munch. Munch. Munch. Alpha-1 is kind of like having an army of really big fish, bigger than the neutrophils. Those Alpha-1 “fish” go into your lungs when the neutrophils are done cleaning up the bad germs and dust in your lungs. Then, something cool happens again. (I outstretched my arms and pretended to use them to chomp up imaginary neutrophils in the air.) Alpha-1 takes its really big chomper arms and catches all the neutrophils before they eat up the good parts of your lungs that aren’t germs or dust. Does that make sense, Megs?”
“So Alpha-1s grab all the neu, neu, neu…”
“Yes, neutrophils. And do you remember what those neutrophils eat up in your lungs?”
“Yeah. They eat up the germs and yucky stuff we breathe.”
“Woohoo! You are a smart girl and good listener, Megsy Rose. But here is some bad news, and I wish I could change it for you. I wish I could take away Alpha-1 from you and all of our Alpha friends.”
“What’s wrong Momma?” she inquired with concern on her face.
“Well honey, your liver doesn’t make Alpha-1 proteins the right way. They get stuck behind the “door” of your liver and can’t get out except through the cracks a tiny bit. So, your lungs might get slowly damaged over many years. We really don’t know what will happen so we do our best to take care of your lungs and hope your liver doesn’t get very sick again. Both you and Grace had sick livers when you were babies, but then your livers got a bit better.”
“Why doesn’t my liver make Alpha-1s the right way?” she seemed to ask in protest.
“Because you were born that way. God made you and when you were made, you were given a liver that doesn’t make Alpha-1 the right way. But you need to remember something really important. Every person born has something wrong with them.”
“In their genes, Mom?” she guessed.
“Absolutely right Meghan Rose! You are so smart to remember that our genes are like maps for what might happen to our bodies as we grow. Not everyone knows what genes they have. In fact, we only know that you have the gene for Alpha-1, but we don’t know all of your genes. So we will do our best to take care of your lungs and liver because we know about your Alpha-1 gene.”
“Like stay inside on oze actshun days?”
“Yes. When it is an ozone action day, you and Grace must stay inside in the air conditioning so you don’t breathe that yucky air. If you did, the neutrophils would come marching into your lungs to eat up the yucky stuff, but then those neutrophils would keep eating and eating up the good parts of your lungs because too little Alpha-1 is in your lungs and can’t munch up all those neutrophils. So we just try really hard to keep those bad things from getting into your lungs in the first place.”
“The Alpha-1s can’t get that door open in my liver, huh?”
“Right Sweetie! It sometimes sneaks through the cracks of the door, but most of the time, it just stays inside your liver causing it to be just a little bit sick. Whenever the Alpha-1 gets stuck inside the teeny-tiny parts of your liver called cells, that Alpha-1 gets all mad and angry. It “throws a fit” and messes up that cell by making it swell up like water that goes into a water balloon. For some reason though, your liver is lucky. It seems to be able to do something called regenerate. That is a big word for fix itself. It keeps rebuilding your cells.”
“My liver is fixing itself when the Alpha-1 throws a fit?”
I could feel myself becoming elated that she was actually following along on the convoluted path that I had formed off the top of my head trying to explain Alpha-1.“Yes. It is. Gosh Meghan. Does this really make sense to you?”
“Yes. I think I get it Mommy, but I can’t ‘member all the right words yet.”
“Megsy Rose, you don’t need to remember all the right words. You just need to know that we love you very much, and that we’ll do our best to take care of you and your Alpha-1.” I felt myself take a very long, deep breath as I seemed to be holding it during most of the conversation.
“Ok, Mommy. So is Mr. Ed’s Alpha-1 throwing a fit inside his liver?”
“Yes, it is. Let’s pray his liver can fix itself again. Sometimes that doesn’t happen. He might need to get a new liver from someone who goes up to heaven and doesn’t need it anymore.”
“God bless Mr. Ed,” she concluded. I couldn’t help but feel enormous pride in her realization, but also some sadness as she figured out her Alpha-1 can cause bad things. God bless Meghan and her sister, Grace.
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