Explaining Alpha-1 to Your Child
The following article is an Alpha mothers account of how she explained Alpha-1 to her 8 year old daughter.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?”
“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.
The following article is an Alpha mothers account of how she explained Alpha-1 to her 8 year old daughter.Special thanks to Jen (author) who gave us permission to publish this article.
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