A Fighting Chance

 By fall of 2019, I had been with my specialist team for several years and was being seen on a monthly basis. My MELD score had crept up to over 15 (which is qualifying to be listed for transplant).  When I broached the subject of getting listed, I was told to wait until it hit 18 - then we would talk again.  I was getting edgy, but I thought the doctors were doing what was best for me.  So when I heard in no uncertain terms that there were no plans to put me on the transplant list, I had a choice:  be crushed or fight.  Once again, I became my own advocate for transplant.

MELD SCORE:  is calculated from lab tests for creatinine,  bilirubin and INR and ranges from 6 to 40 (6 is normal, 40 is.. well, dead for all intents and purposes).  It is use to predict your mortality rate for the next 3 months.  It is also used to calculate how often you need to see your doctor and have labs drawn.  

In October of 2019, I asked a coworker whose daughter was a doctor at the Cleveland Clinic liver transplant center to refer me to a good doctor.  I set up my appointment to be evaluated.  It's amazing that two doctors can have the same degree and background, yet one sees your situation as a possibility while the other an impossibility. This doctor believed I had a chance and scheduled me for the transplant evaluation.

While I was waiting to be listed for transplant, I can't count the number of times people asked when I would be listed.. as though it can just be a scheduled surgery.  There is so much involved in a transplant.  Before I saw the evaluation team, I had to have all of my dental work complete, have a dermatologist go over every bit of skin on my body to check for cancer, see my gynecologist... just to name a few.

In January 2020, I finally got to the evaluation... which is a full 5 days of back-to-back appointments!  I had to have a caregiver with me, meet with every kind of specialist you can imagine, had MRI's, CT scans, ultrasounds, x-rays, more bloodwork than I ever dreamed a person could donate (something like 30 vials).  In addition to physical criteria, you have to go through financial evaluation, behavioral health evaluation, nutritional evaluation, then a social worker helps you make a will and sign legal guardianship over to your caregiver.  It's a lot!  By the time it was over I was totally drained - physically and emotionally.

I was assigned a pre-transplant coordinator nurse, then had to wait.  If everyone agreed that I was a good candidate, then it would go to UNOS for a board approval.  After that....

APRIL 14, 2020 WAS A DAY FOR CELEBRATION!  

I WAS FINALLY ON THE TRANSPLANT LIST!

April 9, 2020

Tired and waiting

The Long, Slow Fade

I've always burned the candle at both ends, but the slow descent of my health from 2018 to 2019 left me with only one very small flickering flame.  Every few months I'd be in the hospital or emergency room.  I made constant trips to Pittsburgh specialists and had regular endoscopies and colonoscopies.  My body began to manifest the failure of my liver to do its job.  I developed:

• Portal Hypertension (pressure in the vein the goes from the gastric region into the liver)
• Splenomegaly (enlarged spleen)
• Esophogeal varices (enlarged veins from the back up of pressure)
• Fluid retention
• Malnutrition
• Bouts of supraventricular tachycardia requiring cardioversion (heart rate above 180 that doesn't resolve without the heart being stopped and restarted via either medication or electric paddles)

With all of things happening to me, the hardest thing for me to deal with was the tiredness.  There is a tiredness that has nothing to do with the lack of sleep.  It drains the life from you.  I still wanted to be in the thick of family and church gatherings, but I sat quietly and watched rather than participating.  At home I began breaking common tasks into small increments.  I learned to cope. Through it all, I continued to stay in good spirits, to hope and believe everything would be okay; but that dark cloud was getting closer and I couldn't just ignore it anymore.

In the fall of 2019, my brother-in-law passed away and was an organ donor.  My sister-in-law offered his liver to me if it was a match, so I called my doctors to find out the answer.  What I found out shocked me!  My doctor had no intention of putting me on a transplant list.  She thought my lungs would not hold up to a transplant.  I was devastated!

Me having a particularly hard day in 2018:

I kept my smile on and wore a lot of

really bright colors to stay positive

My First Crash

 My health held steady for most of 2016.  I had a few hospitalizations and bouts of pleurisy - very painful, but treatable with antibiotics and steroids.  Things changed early in 2017.  My liver function crashed in April.  I still don't totally understand why liver failure causes pleural effusion for me.  Most people struggle with ascites (fluid in the abdomen) instead.

Pleural Effusion is the buildup of fluid between the layer of pleura and the outside of the lungs. It causes the lungs to become compressed and breathing becomes difficult.  

When the layer of pleura becomes inflamed it is called Pleurisy.  Many describe the feeling of pleurisy as sandpaper rubbing again the outside of the lungs with each breath. 

Because I had reached crisis stage and was really struggling for breath, the doctor attempted to draw fluid from my side with a needle and then inserted a chest tube.  It was my husband's job to hold me still.  To say it was painful is an understatement.  My tough husband had to let go and sit down to keep from passing out.  It was rough!  Finally, the chest tube was inserted and I was transferred to Allegheny General Hospital in Pittsburgh.   

As the fluid drained from my chest, it continually refilled.  It wasn't working and I was in trouble! My right lung had collapsed as high as my shoulder blade and the left lung was on its heels.  At some point I had also developed a pneumothorax (air in the pleural cavity) in addition to everything else I was struggling with.  It did mend without surgery, thankfully.  With everything happening to me, I thought I would receive a transplant and get back on my feet.  My doctors said no.  They wanted to do a procedure call TIPS.

TIPS(Transjugular Intrahepatic Portosystemic Shunt):  the doctor enters the jugular vein in the right side of the neck, inserts a needle to tunnel through liver tissue then places a shunt in a hepatic vein.

I waited for 3 days for a specialist doctor to fly in to do the procedure.  It worked.  The fluid began to slow and the buildup finally drained.  But the crisis wasn't over.  I began to run and unexplained fever.  They discovered that I'd developed a blood clot in the medical port I'd had put in for my weekly Prolastin C infusions.  The port had to be removed before I could go home.  

For 7 months, I felt great!  The terrible pressure in my chest and abdomen had lessened, I had energy and hope that this could last a long time.  The doctor assured me that she had patients for whom it had worked for 10 years.  It made it to seven months for me. 

2017:  The hole from TIPS and a scar

from the Mediport removal (which was 

later replaced on the other side).

Faith - from my Experience.

 In my last blog, I mentioned the stages of grief one might experience when faced with their own sickness or impending death (denial, bargaining, anger, depression and acceptance).  The one experience I don't feel like I dealt with was bargaining (with God).  I really didn't.  I've had people tell me they admired my amount of faith, others encouraged me to have faith, while others called on me to reach deep into myself and stir up an enormous amount of faith to get God's attention. I'm not a minister and don't have any great theology to share; I'm sure there is validity in all of that.  However, I can only tell you what I experienced from my own perspective. 

I was five years old when I fell in love with Jesus.  One evening at church, as the music played, I lifted my hands and began to sing from the bottom of my heart to Jesus.  In that moment it was as if the heavens opened and His Spirit descended all around me and over me, and I knew for myself that he was real. Not only was He real, but He took a little five year old girl seriously and responded to her. It was the bud of lasting relationship that blossomed and has never faded.  As a young girl/woman I can remember often praying this prayer:  "God I want you to be pleased with me.  When your eyes travel through the earth, I want you to smile when you see me.  Help me live a life that brings joy to you." I haven't lived perfectly, but I have lived loving Jesus.

Faith to me has been an extension of that kind of loving relationship between two souls that have shared a lifetime of love and experiences together.  It wasn't something I had to dig deep to conjure up.  It just was.  I believed I was in God's hand.  I talked to God about the things I was going through and was confident He cared.  I asked Him to heal me and help me.  I remembered all of the times He had in the past, and believed that whatever came, He would be on my side.

So did I have a lot of faith?  I honestly don't know.  I just quietly and simply hoped for the best outcome and believed Jesus would do what's best for me. I really don't see myself as having a lot of faith or lacking in faith.  

Me at 5 years old

Challenges of a Rare Disease

 Living with a rare disease can be unsettling.   In my case, I diagnosed my disease and took the results to my doctor, who then confirmed my diagnosis.  The problem comes when you have done a ton of research and your doctor studied Alpha 1 Antitrypsin Deficiency one semester in med school a long time ago, but has never dealt with it personally.  On many occasions when I was being treated in a doctor's office, emergency room or in the hospital, I had to explain the disease to the medical staff.  When one of them gives you off-the-cuff information and you've read the medical studies that refute the information, you feel pretty vulnerable and frustrated. I learned I had to be my own medical advocate early.  

My first experience with liver failure and doctor error occurred in the summer of 2015, I just didn't know that's what it was.  I was hospitalized for 3 days locally and asked the attending physician to make sure my liver was okay, because I knew Alpha 1 is a liver disease.  He didn't take it seriously; said I was overweight and had some fatty liver; then diagnosed me with gastroenteritis.

  Early in 2016, I was again severely ill with abdominal pain, right shoulder pain, vomiting, and shortness of breath, but this time I didn't waste energy on the local hospital.  I went to a UPMC facility, and the doctors there heard me.  Once again, no one really knew what Alpha 1 was, but they figured it out and sent me to a specialist.  After a biopsy and a month of waiting for the results to come back from analysis at the Mayo Clinic, my suspicions were confirmed.  I had Cirrhosis, Stage 4, caused by Alpha 1 Antitrypsin Deficiency.   The only cure would be a liver transplant... if my lungs could stay healthy enough and hold up to a transplant.  (I've since learned you can get both a liver and lungs, but that's the information I was given at the time.)

Did you know that when you are faced with terminal illness that you go through the same stages of grief (denial, anger, bargaining, depression and acceptance) that you do when you lose a loved one?  I did know that in the back of my mind, but I didn't apply it to myself for quite a long while.  Perhaps I was in denial.  Perhaps I had faith.  Perhaps it was a little of both.  I saw Cirrhosis as a black cloud on the horizon threatening to bring in a bad storm.  I kept my chin up, my back to the cloud and just kept right on with life... until I'd deal with a bad flare up and end up in the hospital.  Deep inside, I believed I would make it to transplant then everything would be fine.

2016:  I stayed pretty active, but developed a relationship with my hammock on sick days. 

Left:  Me in my hammock on Mother's Day

Right:  Climbing the rocks at Freedom Falls with my hubby on July 4th.

I Thought I'd Won

I like to win... games, tests, arguments, life... whatever, whenever and however.  I like to win.  By Spring of 2016 I thought I was the winner in my battle with Alpha 1 Antitrypsin.   At diagnosis, my first of many Pulmonary Function Tests showed my FEV1 to be 52%, but a year later it had risen to 68%.  Everyone was surprised!

A PFT involves sitting inside a glass booth and breathing into a tube.  The test measures how well your lungs exchange oxygen and carbon dioxide as well as how much oxygen you can inhale and exhale (the short version).  FEV1 is the reading that tells how your lungs compare to the expected function of someone your height, weight and age.

Prolastin C only makes the claim to stop the progression of the disease.  It makes no claims to improve lung function, so when my lung function improved that much, I was ecstatic. 

I knew that viruses were lethal to my health and took steps to avoid sick people, but I had one more hurdle to get over... pollutants.  One early summer night I left the bedroom window open.  The air smelled great, and I enjoy the sound of the creek flowing near my house.  I did not know that the neighbors would start a bonfire after I went to bed, or that the smoke would enter my window.  Breathing the trace smoke from that bonfire caused me to have a respiratory illness that took 6 weeks to recover from. I learned that my doctors' warning to stay away from any kind of smoke or pollutant was vital to my wellbeing.  

I had a three-pronged plan to win my fight with Alpha 1:  stop progression of the disease with Prolastin C Infusions, stay away from people who were ill with a virus, and stay away from air pollutants.  It worked!  My respiratory infections decreased drastically.  I really thought I had won... I felt like a champion!
 

Me teaching at LTI

Feeling like a winner

The Way Maker Steps In

I can't count the number of times God stepped in and made a way when there seemed to be no way over the last twelve years.  My husband tells me every time I worry about something that I know God will take care of it for me, not to worry.  He's right, of course. I remember a pivotal point in my life when it seemed that God started showing me that He had my back.  It was September and my first year as a single Mom.  I was barely able to put food on the table and keep the utilities on, but I needed tires and had no idea how I was going to pay my propane heat bill or afford Christmas that year.  One day, I believe it was the kids' bus driver who handed me a $20 bill and said, "I feel led to give this to you.  It isn't much, but I asked God to multiply it for you."  It started multiplying that day.  Someone else handed me $50, I went home and opened the mailbox to find a card from a friend in Indiana with $100 in it.  Later that week someone asked to borrow my car and returned it with all new snow tires.  By the first of October, all of my bills were paid through the end of the year, and I had Christmas money.  After many instances of God's providence like this, you'd think I'd give up on worrying. lol

I had a new problem to needlessly worry about.  It was May of 2015, I was recently diagnosed with Alpha 1 Antitrypsin Deficiency and I needed weekly infusions of Prolastin C.  

 

Prolastin C is an Alpha 1 Proteinase Inhibitor made from human plasma.  It takes one year to make and requires dozens of human plasma donations for each dose.  Its purpose is to coat the lungs of the Alpha patient to protect them from sustaining more damage.  Without the coating, little holes develop in the lungs' alveoli and emphysema symptoms appear.  Prolastin stays in the patient's system for 7 days, so weekly infusions are needed.  The cost for this medication is $13,000 per month.

I was teaching at a business college and had insurance, but it only paid 80 percent of my medical costs.  I did the math and knew I couldn't afford $2,600 a month for the medication.  I prayed and worried, of course.  But I didn't have to worry long.  My company changed insurance every year in July, so in June we got an email inviting us to the annual insurance meeting.  That year, for the first time ever, the company was able to get insurance with a $5000 deductible and 100 percent coverage.  So instead of $31,200 per year, I could get the medication for only $5,000 per year.  I was amazed and grateful!

And so I began my weekly infusions.  Medication was shipped to me in small bottles (6 for each dose).  I'd receive a month's supply, which had to be refrigerated.  I'd take them out the morning of my infusion (cold liquid into the blood stream is pretty uncomfortable and not a good idea), a nurse would come, set up an IV, and infuse the medication.  It took about an hour for the infusion.  The stick of the IV needle and a bit of shivery coldness were the only discomforts of the procedure, so I adapted quickly and didn't mind it.

The Beginning of a Long Journey

Today, I saw a picture of me .. a picture of me a year ago.  Feelings of gratitude and thankfulness swept over me as I pulled a grandbaby close for a snuggle.  That woman had faith.  That woman had hope.  Today, this woman has the joy of living in the warmth of what was once just sparks of faith and hope.

If you ask me to define my miracle, I will say that I'm alive - but that doesn't begin to tell of the many miracles that kept me alive.  I tend to live in the moment, enjoy the gift of each day to its fullest, and try not to dwell on the hardships of getting here.  Lately, flashes have been popping into my mind.  Maybe it's time to talk about some of these experiences.

Sometimes when I try to remember, I just can't seem to breathe.  The memories merge into a feeling of panic and I pull away.  But today, I will find one memory to face.  Perhaps I should start at the beginning.

Pre-Diagnosis

It was January 2009.  I shivered with fever and cold as I lay underneath my mobile home with a hairdryer attempting to thaw frozen pipes.  It was my first winter as a single mom.  I knew to plug the heat tape in when winter came, but I didn't know there was a light that indicated whether or not the heat tape was working.  When my pipes froze, my 102 + fever didn't have a say in what needed to be done.  I couldn't afford to replace cracked pipes, so I had to fix the problem immediately.  So there I was... racked with coughing, burning up with fever... laying under my mobile home in snow at below 0 temperatures.

That winter seemed to be horrific for viruses at Sunset Heights Christian Academy.  The students continuously brought in viruses and I had pneumonia five times that school year.  I fractured ribs and separated my sternum coughing.  I even managed to get whooping cough in addition.  I didn't know what was happening to my body, but I knew something was wrong, seriously wrong.

I struggled with bouts of pneumonia and sickness for five more years.  My doctor thought maybe it was lupus, but it didn't fully add up.  My inflammation markers were always extremely high, other labs were inconsistent as well.  Finally, in 2015 one of my mother's doctors who was treating her asthma-like symptoms suggest an Alpha 1 Antitrypsin Deficiency test (though he thought it was highly unlikely).  

Alpha 1 Antitrypsin Deficiency is a hereditary disorder.  It lives in the liver and mutates the protein enzymes that produce Antitrypsin, which protects the bodies organs.  The organ most impacted by low levels of Antitrypsin is the lungs.  Alpha's often suffer from COPD and/or Emphysema without having smoked.  It usually presents in the patient's 50's.  Additionally, the liver can become damaged by the mutated proteins and Cirrhosis develops.  While this occurs later in life in some patients, most patients experience liver symptoms from Alpha 1 in the first two years of life.

Mom tested positive as a carrier.  She had 1 mutated gene and 1 normal gene.  I immediately went to AlphaNet.com and ordered my own free test.  It made sense!  All the years of suffering suddenly might have a name.  While I waited for results, I researched.  What I found was not good news, but there was a treatment.  That meant hope.

It was May 2015 (I don't remember the exact date)  I graduated with my Masters Degree, celebrated, then came home.  I remember my heart beat seemed to pause for a second when I saw the letter in the mailbox.  My diagnosis was Alpha1 Antitrypsin Deficiency ZZ (which meant my father had to be a carrier too).  My Antitrypsin level was 4  - normal is around 100.  I finally knew why I had been struggling, but I had no idea the journey I was setting out on.  

Welcome

 Hi, I'm Angela. Welcome to my blog.

  Have you ever been to a place in life where you are starting over - starting from scratch - and are both excited and terrified of the possibilities? My friend, I am there.  Welcome to my journey of self-discovery.

God has been good to me.  I survived a severe health crisis.  I'm on the other side and ready to get back to life.  There's only one problem.  Life moved on in the year and a half I was focused on my survival.  I fought so hard to get back to life, only to realize there's no getting back to what was.  Fortunately, it means a whole new world of opportunities has opened up.  

So now, I'm asking myself...

• Who am I?
• What's important to me?
• Where do I want to be in a year, 5 years, 10 years and at retirement?