Skip to main content


Showing posts from March, 2015

Pulmonary Arteriovenous Malformation

I talked a lot about what Pulmonary Arteriovenous Malformations is doing to me emotionally but today, I wanted to post about what it actually means.

First of all, it's not really 'holes' in the lungs.  They are more like lesions.  But we'll get to that.

Basically, it is a very rare and complicated vascular disease.  It can strike anywhere in the nervous system, brain, lungs and liver.  We don't know how extensive the disease is in Sam but we do know that he has multiple lesions throughout both lungs.  This kind of AVM is quite rare.  The connection between the pulmonary arteries and veins are faulty and bypass the capillary system altogether.

Normal arteries carry blood from the heart to the lungs and from there gets sent out throughout the rest of the body, taking with it rich, oxygenated blood as it passes through the capillaries.  The blood is then returned to the heart by way of the veins. The capillaries job is to give up the oxygen and remove the waste.

In …

Trampled Upon

So, this is a slight deviation from the medical life we lead...though not entirely.

It's been a pretty crazy weekend.  Shelbie had her IVIG on Thursday.  It went well, nothing out of the ordinary in the infusion.  Friday was fine too, even Friday night.

At around midnight on Friday, the headache started for her.  By early Saturday morning, we were in the ER trying to get pain relief that about a dozen remedies at home couldn't quite catch hold of.  They did the usual cocktail for these horrible headaches and after about 3 hours or so, we headed home and Shelbie was feeling better.  That's usually the end of it but that darn headache came back with a vengeance and we ended up back in the ER by late in the evening.  I was pretty miffed with the ER doc the second time around.  She was in more pain than I have ever seen her in before.  It radiated from the top of her head down to the bottom of her spine. They gave her  Toradol and Steroids since we were now treading in the M…

What IF...

I find this whole process of living and dying so fascinating.  I don't think it would be so, if I was someone who had never really had to face anything too difficult or at least anything that messed with my foundation of mortality.

I find it fascinating to experience firsthand, how you can go from just fine... to not... in seconds, without an ounce of warning and with a toilet paper commercial tipping you off your precarious position of balance.  I hate it actually, but fascinating all the same.

I posted earlier this week about the patterns of grief I've been noticing, but then I deleted the post.  I hadn't fully completed my thoughts and accidentally published it. There are definite patterns though.

Overwhelm and shock are one of the first things you feel, mixed with a healthy dose of denial.  Then comes acceptance; at least in some degree.  I don't think acceptance is something you resolve just once and then move on.  It think it can be an hourly experience.  Afte…

The Current

When you are in the thick of things, it's hard to see beyond what is happening.   When you just live each day, doing what needs to be done, the subtleties of life get lost on you.  Probably because your focus is elsewhere.

It hasn't been a bad week.  It's been okay.  There were some uncomfortable moments but for the most part, I would say, I 'endured it well.' as the old saying goes.  We actually had some pleasant moments, moments when I felt somewhat normal.  Basically, I have felt to have been in a good place.

This morning, I met with a friend to help her with a remodeling project she is doing.  We don't talk a lot but when we run into each other every few months, we just pick up where we left off.  We usually do a ton of laughing because she is awesome like that!  Their whole family is awesome and our boys are friends and both serving missions.

We enjoyed our usual good time together.  Working on plans and laughing some.  When I was getting ready to leave,…

Somewhere in the telling

Having a blog, or several blogs, over the past few years has been an interesting experiment for me.

It's evolved from a blow by blow menagerie of current events and report of 'weather' types of things meant just for my family, to something with a little more meat.  It has been one of my greatest challenges.

Despite my seemingly public display of sarcasm on social media, I am a very mysterious person; private too but I think mysterious describes me better.  I am a chameleon of souls, fitting in whenever and wherever and acting out whatever the moment dictates. I can be in the farthest depths of sadness, yet no one would be the wiser.

To write my blogs with more honesty and less self censoring is one of the hardest things I have done and I still don't do it well. I read with jealousy, other people's blogs who just lay it all out there.  All the good, the bad and the horribly ugly stuff.  The feelings you never want to admit to a soul, not even your favorite friend…

What are you dying to do?

Yesterday, somewhere between listening to a talk on the mystery of ants to a seminar on Pulmonary Arteriovenous Malformations, I heard a man say, "What are you dying to do?"  That struck me as an interesting statement we hear all the time.

"I'm dying to..."

From day to day, we say it all the time, mostly in relation to trivial matters like that hot fudge sundae we are dying to taste, the big sale on the Coach bag you've been dying to get or a restful trip to Cancun.  We die for any number of things.  But, when the rubber meets the road and you realize that maybe you are dying, what is it you are going to do with this one very precious life you have been given?

What then, will you be dying to do?

It's been a week of struggles.  Yes, It's only Tuesday morning but the last three days have been a big struggle; on so many fronts.  Shelbie continues to feel rotten and I continue to feel rotten because there is nothing I can do to make her feel better.  W…

Different Than I Thought It Would Be

I thought, that the day the doctor told me my kids had a fatal lung disease, would be different.

Actually, I didn't really think about that day at all.  Ever. I thought about that day for other mothers; how they might feel if they heard those words. Those are the words reserved for the ears of another, not mine.

So, I carried on, not really believing anything too serious was wrong with my kids, even feeling guilty some days that they weren't sicker because surely, by all logical standards, they should have been.  People die from this disease dyskeratosis congenita.  Horrible things happen and they die.  My kids were flourishing in the long shadows of this disease.

It was by chance they found the heart and lung problems that divided my world between then and now.  Maybe the increased lung pressure was just a small hole in the heart. Devastating, but patch it and let's get on with living.  Then, it became more puzzling.  Maybe the increased pressure in the heart was just a cou…

A Little Too Much

Today, I'm dedicating this post to Shelbie. My sweet angel daughter!

This week, she is fighting both a bacterial pneumonia and a viral infection at the same time.  Because of the the other heart testing, she needs within one week, we have had to move forward with some really hard tests.

Today, she had her heart stress test, echocardiogram and an EKG.  She had run at a 14% incline at 3.5 mph!  She has a hard time catching her breath at best but it was even harder today.  I felt so sad as I watched her take on that challenge without complaining and I know she did her best.

When the tech and I waited out in the hall for her to change, the tech said, "She's awfully young to have these heart problems.  She was out of breath 5 seconds into it.  Does she have asthma?  Something is not right with her."

I just nodded in agreement because I knew if I opened my mouth, out with the words would tumble my tears.

When we left, we went to grab some lunch because she had to do th…

Pneumonia and Neutropenia

Second time I've missed the signs of Pneumonia!! What is wrong with me?  I'm losing my edge.

Ahh...Shelbie started feeling bad Saturday night, Sunday a little worse but went to church and stuff.  Monday, worse still so I ran her down to the clinic.  Got some antibiotics for an infection and then felt pretty confident it would work and that would be the end of that.

Monday night, she was having a hard time breathing, her chest was really tight and a dry cough pursued her through the night.

Tuesday, she was really in pain and suffering so, by the evening,  I ran her back down, knowing that today we had a bunch of tests to get through. They did some x-rays and her lungs were crackly and infected!  Bleh!  They gave her a shot of Rocefin and hoped that combined, with the oral antibiotic, she would turn the corner.  I'm thinking we caught it pretty early.

The very strange thing is that she hasn't been running a fever at all.  Not even a low grade fever.  Nothing.  She ha…

Today Sucks!

Today is not my favorite day of all time!  It just sucks!  I don't have any particular reason for it to suck, it just does.

I hate how my mind ricochets between just fine to...holy cow, I'm a mess.

It's already been a rough morning.  Lots of endless tears and now I have an unbelievable headache.

 The more I learn about Pulmonary AVM's the more terrified I get and the more I can't believe a doctor hasn't addressed the future with me, really explained what this means.  I am convinced that when it's all said and done, Shelbie has it too.  I hope I'm wrong.  Maybe in the next few posts, I will have the strength to actually explain what is happening to Sam's lungs.

Shelbie is still not feeling well.  She has all the signs of a major infection except a fever.  She has chills, body aches, then super hot but no fever. Swollen glands, sore throat... She is having a pretty hard time breathing too.  I almost want to get her on the nebulizer but I don't h…


Today was all sorts of fun at the urologist's office.

However, before we made our way down there, Shelbie woke up sick!  Infected eyes again, sore throat, a barky cough, swollen lymph nodes, the whole works.

I cleaned a house all morning, came home, raced Shelbie down to the clinic, raced back, picked up Sam, raced down to the city and get the picture.  A very busy day!

Shelbie is on antibiotics and hopefully feels better soon.

The urologist was about how I thought it was going to be.  He is not a doctor who is into small talk in any way!  He wasn't even interested in hearing about Sam's health history.  I think he is just so use to seeing old guys with kidney stones because all the paperwork we filled out was just about past kidney stone history.

I had told Sam that there was no way he would need an 'awkward' exam.  Wrong!

The doctor said, "Mom, please leave the room." As he snapped the rubber glove now on his hand.  I looked over at Sam …


I've been experiencing a series of moments.  Moments of pure joy as I watch Sam bound up the stairs to my office after school, proclaiming that he has decided what he is going to do with his life...raise Bullfrogs!   He learned in Aquascience class of a man who works 2 hours a day raising frogs and makes $100,000 a year.


That moment when I leave for work as he is coming home from being with friends at the park and he can't wait to tell me all about the new trick he learned slack-lining.


That moment when I see a picture that Shelbie has taken and it is the most stunning work of art.


That moment when I see her laugh without reserve, without a guarded thought...just an outburst of pure happiness and, when she calls her grandpa on her own to tell him she loves him and will be praying for him as he begins his battle with cancer.


That moment when my Elder writes home with excitement for the gospel that I have never seen before in my son and I know, without…

Family History Part 2

My Grandma P passed away 3 years ago.  I have her picture right in front of my computer so I see her beautiful face every day.

I asked her the same questions I asked my other grandma and here are her answers.

What did you imagine your adult life would be like?

"I always thought that if I lived to be 50 years old, I would be an old woman.  You know, I received a letter from 'a little old woman', she was 50 years old.  When I turned 50, I thought, 'I'm a little old lady now!'.   I always thought I would have a little boy and grandpa wanted a boy so badly he would babysit his sister's little boy.  All we had were girls but then, at last, Grandpa got his little boy and he was so happy."What has been your favorite tradition? "Singing to my grandchildren."   I would have to agree!  I loved it when my grandma babysat us and she would always sing. What was your greatest accomplishment as a mother? "Sewing, Candy making, Crocheting...all skills I le…

Family History

I have only slept for about 6 hours since Monday.  I'm kind of tired but I've also got this weird energy going on and I've been cleaning out my filing cabinets.  I figure, when I die, no one is going to want to sort out stuff that is decades old.

While I was sorting, I came across my folder when I was all over family history!  In that file were interviews I gave my grandmas.  Both grandmas.  I wanted to record the questions I asked and their answers here so I have them forever! ;)

My Grandma F...she is still alive!  Close to 100 years old and this interview was done nearly 10 years ago.  Never in a million years would I have thought my grandma would have lived this long but she is not yet done her purpose here on earth.

What did you imagine your adult life would be like?

"I always wanted to be a beauty parlor lady.  I never pictured myself as anything else.  I always knew I would do that.   I also knew I would be involved in music."  My grandma did both of those th…

Cardiology updates

Today, we had our cardiology appointments for both Shelbie and Sam.

As we drove down to the city, the kids were laughing, joking and seemed to be doing well.

Me...on the other hand was trying to keep up with my racing heart.  I think my heart actually made it to the clinic before I did...It was running fast!

I'm not sure why either...I pretty much knew what our two options were going to be and I have had weeks to mull it all over.

Starting with Sam, he has Pulmonary Arteriovenous  Malformation or, as I will now refer to it...Pulmonary AVM.  Basically, he has multiple holes in both lungs.   These 'lesions' are causing the increased pressure in his lungs and causing oxygenated blood to shunt into the wrong side of his heart. This is increasing the pressure in the right chamber of Sam's heart.

Unfortunately, there is no known cure for this disease and no treatment.  It is a progressive disease but it's anybody's guess how slow or fast it will progress.  I wasn&…


I'd like to tell you that things will  get better tomorrow
But...I've been through so many tomorrows and nothing has changed.
I read this poem many, many months ago and tonight, seemed like a good night to share it.

I keep telling myself tomorrow will be better.

I keep telling myself that there is going to be an end very soon to all this chaos we have been dealing with; that resolve is just around the next corner and like the summer afternoons spent with my grandpa walking to the corner store for a penny candy treat, I can see it.  I can see the next corner and I am sprinting towards it, wanting so desperately to turn it so that I can run right into the face of 'normal'.  Oh how wonderful that word rolls off my be normal. What is that like? To live each day without the lonely looming thoughts that loop subliminally through your head.  Still, I walk through my day, watching people pass by, rushing to strike another thing from the 'To Do' list or have…

At 5:15

If it doesn't make you crazy first...our minds are really amazing and beautiful.

Our minds can absorb so much and either terrify us to death with the catastrophic places it can take us, or create scenarios of beauty and peace and often out of really bad situations we find ourselves in.  It's pretty amazing if you think about it...with your mind.

Yesterday, was sort of a laid back, get work done kind of day.

I realized at about 5:15 that I hadn't had one terrifying thought all day.

At 5:15, I realized that I forgot my kids were sick.At 5:15, I realized that I was sitting at a stop light ...c.o.n.t.e.n.t.At 5:15, I realized that I was breathing, just normal, deep breaths not the shallow panting that keeps my shoulders wrapped around my ears.At 5:15, I looked over at Shelbie and we seemed normal!At 5:15, I realized that Sam has been sneaking off to gym class twice a week...and I let him!

At 5:16...panic struck.

At 5:16, I realized that Shelbie's lungs are filled with dama…

Today's Appointment

The appointment I was dreading actually ended up being positive, as far as the interaction and communication goes.  It was much better than our last appointment when we found out the boys have Dyskeratosis Congenita.

Basically, I just wanted to make sure that the large lymph nodes showing up everywhere weren't the beginning of lymphoma.  He doesn't feel like they are.

Clearly, her body is reacting to a systemic infection of some sort.  She has battled Adenovirus for many years now and because her marrow struggles and she has a chronically low white count and neutrophils, she just can't kick it.  He thinks this is likely the problem. He also thinks this virus could be responsible for the nodules and scarring in her lungs but he wants the pulmonologist to weigh in on that theory.

So, what do we do?  Well, there aren't many options since it's viral.  All we can basically do is continue with plasma transfusions.  For now, the treatments aren't making her feel bett…

Things are about to get hairy

Well, well, well...

Two kids.

Two hearts.

Two holes.

Four malfunctioning valves.

Two sets of bad lungs

Congenital they say.  Just a fluke thing...they say.

Bologna...I say!

I can't officially say I have three kids with heart and lung problems but without a doubt, I know Spencer has heart and lung problems.  We started cardiac testing just before he left and they found abnormalities but we just ran out of time to finish testing.  I bundled that big heart of his back up and handed it over to God to take care of and He has!!  Spencer hasn't passed out like he use to, had uncontrollable asthma attacks...nothing, at least that he has told me about!  He has done so well! For that reason, I wish he could serve a 10 year mission...except I could really use a big hug from that boy of mine!

I picked up the official report on Shelbie's bubble echo study today.  Positive for an Atrial Septal Defect.  Additionally, her tricuspid valve is not working properly and blood is shunting bac…

The weekly re-cap

It was a big week and we survived!  Barely but we survived.

Wednesday was Shelbie's testing which I already reported on.  Thursday was IVIG which went off without a hitch and Thursday night, after I was done working, we headed to Salt Lake for Sam's cardiac testing.

The drive down was tiring as it was kind of late and sitting at a hospital all day is the most fatiguing thing you can possibly do.  I don't know what it is about hospitals but they make me tired.   The kids slept off and on and in the darkness of the night sky, it was hard at times to stay in the moment.  Anxiety continued in waves and it was hard.   I wondered if I would ever know how it feels to live carefree and without my companion of anxiety and worry.  No matter how hard I try, it always comes back to this sooner or later.

At 10:00, we decided to stop for dinner.  The kids wanted me to try In n Out. I've never been there so we decided to be adventurous.  I couldn't believe the line up for hamburg…