No More Handshaking | Chocolate Medicine or Venofer®? | More on P.L.E.

Doctors suggest I wash my hands all of the time and try to avoid shaking hands with people. They also suggest I try to avoid large groups of people because they tell me I am extremely immunosuppressed and anything could get me sick. If this happens it stalls the heart transplant. I need to stay free of all bugs. This is tough because I enjoy being with my large family and attending my Church which is my other family.

Chocolate Medicine or Venofer®?
Because of my heart failure I am anemic. So for the past 5 days I have received an iron transfusion through an IV each morning for an hour in addition to the 3,000 other medications I am taking.

Venofer® is used to replenish body iron stores in patients with iron deficiency. It is also a brown, sterile, aqueous complex of polynuclear iron (III)-hydroxide. But, I told my daughter it was chocolate medicine.

Iron deficiency is the most common deficiency disease worldwide. More than 1 billion people have iron deficiency, and about 700 million people have iron deficiency anemia. Iron is an essential nutrient that is needed by every human cell. It plays a valuable role in the transport and storage of oxygen and oxidative metabolism, and in cell growth and proliferation.

PROTEIN-LOSING ENTEROPATHY
SO - I have P.L.E. which is the main reason we're in a rush to get a heart. This disease could eventually kill me. I know it might be boring but I add these blogs to my journal and so I need to document this information:

Background

Protein-losing enteropathy is characterized by the severe loss of serum proteins into the intestine. Normal protein loss in the gastrointestinal tract mainly consists of sloughed enterocytes and pancreatic and biliary secretions. Albumin loss through the gastrointestinal tract normally accounts for 2-15% of the total body degradation of albumin, but, in patients with severe protein-losing gastrointestinal disorders, the enteric protein loss may reach up to 60% of the total albumin pool.

The serum protein level reflects the balance between protein synthesis, metabolism, and protein loss. Protein-losing enteropathy is characterized by more loss of proteins via the gastrointestinal tract than synthesis leading to hypoalbuminemia. It is not a single disease, but an atypical manifestation of other diseases.

Pathophysiology

The pathophysiology of this disorder is directly related to the excessive leakage of plasma proteins into the lumen of the gastrointestinal tract. Mechanisms for gastrointestinal protein loss include lymphatic obstruction, mucosal disease with erosions, ulcerations, or increased mucosal permeability to proteins as a result of cell damage or death. Proteins entering the gastrointestinal tract are metabolized into constituent amino acids by gastric, pancreatic, and small intestinal enzymes and are reabsorbed. When the rate of gastrointestinal protein loss exceeds the body's capacity to synthesize new proteins, hypoproteinemia develops.

Medical Care

Focus treatment on correcting the underlying process causing the protein-losing gastroenteropathy. For example, the patient with congestive heart failure may respond to digitalis and diuretics, whereas the patient with intestinal parasites should be treated with the appropriate medication for the infestation.

Diet

A low-fat diet with supplementation with medium-chain triglycerides is theoretically of benefit in patients with lymphangiectasias. However, in practice, ingesting a diet containing medium-chain triglycerides results in increased blood flow with no reduction in fecal protein loss.

Primary Children's Medical Center

I am fortunate to receive all of my care from the good folks at Primary Children's Medical Center in Salt Lake City, Utah. I'm 35 but the oldest baby on the planet so they oblige. Actually, all of the experts and surgeons who specialize in my heart's anatomy and birth defect are there. I grew up going to this hospital when it was high up in the avenues up above the state capital and Mormon temple. Now, it is located on the University of Utah campus and next to the University Hospital where a lot of advances in medicine were developed.

Every time I go there I am deeply affected by the amount of ill children who I see. They are so helpless and hurting. Many are scared and some alone. I remember being there myself as a child and having the same emotions. But, there could not be a better place and staff to accommodate them and their families. These children bring a special feeling to the center. I believe angels walk the halls and provide a comforting spirit.


I'm also amazed at the advances in medicine since I was a child. When I went last week to have my picc replaced in radiology I saw a hallway with a sign that read "MRI". This was incredible because when I was 13 and dying with a staff infection in my heart we had to travel to LDS hospital in an ambulance to have an MRI. In fact, the MRI had only been there in Utah for 6 months. The full body scan would help doctors locate my staff infection and prepare surgeons which prolonged my life. Also, when I was there 2 months ago in the intensive care a nurse who I had not seen since I was 14 came to my bedside and we had a great discussion. There are many great folks who are still there and they remember the kids.

I'm very honored and feel greatly blessed to receive my care from the people at Primary Children's Hospital. As one of their patients I feel like a black sheep among some of God's purest sons and daughters. When you pray this week keep in mind the kids up there in that hospital even though we don't know their names, faces, or stories.

PICC Lines | Pick your PICC Lines.

This was a week of picc lines. (Incidentally, I should be grateful. Having a picc line with a good source of milrinone does make me feel better. So, being connected to a tube full time is not that bad.) I went in to the hospital on Monday because the area where my original picc in my right arm that I received 6 weeks ago was becoming irritated and red. We don’t want an infection so we decided to pull the picc and put a new one in my left arm. Only this time, I was not sedated like last time so I do remember the experience. Most adults aren’t sedated. But, I’m sort of a baby when it comes to needles and wires being thread through my veins so I usually beg for it. But, I manned up and went for it without sedation. We did the procedure in the cath lab. Needless, to say they gave me several shots that numb the skin before inserting the wiring and eventual picc. Like at the dentist when he numbs your gums before he drills. It wasn’t that bad. I went home 30 minutes later. Of course, my wife heard me say the whole ride home, “That wasn’t fun.” Oh well, I was happy to be home. After a few hours we noticed the leaking, slow leaking watery substance coming from where the picc was inserted. My wife, who is my home-health care nurse (lucky me), ended up changing the dressing over the next few days 6 times because of the leaking until yesterday I went in and had that picc removed and a new one placed in my right arm above my elbow where the original one was. Needless to say, the big needle wasn’t that bad and I made it home to watch the University of Utah destroy Alabama in a much-deserved BCS bowl. Let’s just hope this new picc doesn’t leak either. It’s only been a little more than 12 hours.

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A PICC is a long, thin, flexible tube known as a catheter. It is inserted into one of the large veins of the arm near the bend of the elbow. It is then slid into the vein until the tip sits in a large vein just above the heart.

The space in the middle of the tube is called the lumen. Sometimes the tube has two or three lumens (known as double or triple lumen). This allows different treatments to be given at the same time. At the end of the tube outside the body, each lumen has a special cap, to which a drip line or syringe can be attached. Sometimes there is a clamp to keep the tube closed when it is not in use.

A Heart for Christmas | Or Not

The call came at 12:45am. “We have a heart for you,” the voice on the other line said. I was in shock. I had prepared for the call but because it came early Christmas morning and the fact that it was a real call left me speechless and emotional. We had until 2am to get to the hospital. The operation would take place at either 6 or 7am.

I called my parents to meet us at the hospital. They called my siblings to let them know what was going on. Early that night, we had all gathered with the husbands, wives, and kids for our annual Christmas Eve dinner at my parents. It was a great night. We had a special family prayer and the kids acted out the nativity. My wife and daughter left to go home at 11pm. Before putting my 3-year-old daughter to bed we each opened a present. We put her in bed and then Santa came. Then the phone rang.

We arrived at the hospital feeling a great sense of peace and comfort. The time had come and I was ready. Nurses began the preparations. Everyone was extremely positive and saying things like, “Merry Christmas!” and “What a great Christmas gift.”

I received a rather larger IV and a lot of blood was drawn from my already existing picc line for lab work. They had me wash my chest and groin area (where the major arteries are for the heart lung machine) with special medical soap. It was a little cold. Then surgeons came into my room a little before 5am and told my wife and my parents what was expected. He mentioned the “difficulty” of my particular case because of my heart’s anatomy. It would be challenging to get the old heart out. There would be a lot of bleeding. He said, "There is an 80% chance we'll have to go back in later to help stop bleeding." He said chances are I might not make it. Although he had to talk about the downside or the surgery he also mentioned they have an 80-90% success rate with these surgeries on post fontan patients. But, the harsh realities left me a little depressed but I still felt great peace. I had understood the depth of this procedure previously but when you are minutes away from it actually happening life becomes quite sobering.

They came to get me at 5:20am and before the anesthesiologist took me into the operating room I talked briefly with my wife and parents. I kissed Lynnette, told her how much I love her, and then headed down the hallway.

In the cold, brightly lit, O.R., nurses lifted me from my bed onto that firm, narrow, operating table I have become familiar with over the years. I received a dose of versed (Midazolam) for anxiety and preparation. Before I went completely under we were waiting to hear from one of the surgeons, who had gone to harvest the donor heart, if everything was absolutely perfect and good to go. About 30 minutes later the news came. “The new heart had a problem undetected until they looked at it face to face and would not work,” they said. It had an aneurysm which would require some additional surgery on top of everything else we're dealing with.

I didn’t know what to feel. Shock? Relief? Disappointment? I have felt all of those things. Nonetheless, we called it off.

Lynnette and I came home 5 hours later to Christmas morning. My daughter, who had been taken good care of by my sister n’ law, asked if I brought her a sucker from the hospital and things seemed back to normal. I was anxious to experience Santa with my child. One of the gifts from Santa for my daughter was a Fisher Price Medical Kit. She practiced giving shots to me and listened to my heart beat.

I am thankful for the love and support of my family and friends, particularly my sweet wife Lynnette.

This is a roller coaster of emotions. In a way, this experience seems like a brief 2nd opportunity of time before we find out the real outcome of my life’s journey. I am ready for another call but for now I’ll enjoy this weekend with my wife and daughter as we humbly celebrate the birth of the babe in Bethlehem some 2000 years ago.
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Time, precious time
How quickly the leaves fall from the tree.
Time, oh precious time
In a blink of an eye a child grows old.
And in the pursuit of joy and happiness
Lies time
But quickly it fades
And all we have are memories
Precious moments
Remembered in time.
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The Blessings of Christmas

Two months ago all I could do is lay around because doctors were trying to figure out how to get my heart failure under control. I was in need of a serious tune up.

Whether it's a consequence or not, I was humbled to learn that my family, friends, fans of my music, and those I don't even know began praying for me.

I want everyone to know that God is good to me and loves you because for the past two weeks, as we get closer to Christmas I have felt great. Not 100%, but well enough to get out a few ours each day. Well enough, to make gingerbread houses with my kid. Well enough to laugh and enjoy this time of year. And it's because of prayer and the wisdom of my doctors that I am tuned up for a time. And what better time than Christmas!

Prayer is gift. Life is a gift. Family is a gift. And I am more than happy this particular time of year to celebrate the Birth of our Lord.

Ridin' The Hoverround in Costco!

Man, I can't believe how much fun it is to drive a Hoverround scooter all over Costco! I had seen how much fun the folks were having in those commercials during my daily watching of "The Price is Right" but I never new it was true. The other thing that amazes me is that you can scoot around Costco in less than 20 minutes and still spend almost $500.

On a serious note; and in regards to my PLE (Protein Losing Enteropathy), I finally learned the importance of avoiding fat food. The more fat I eat the more protein I lose from my system. I am also losing a lot of blood in my intestinal tract too. This is also another contributor to my anemia. I am greatly motivated to eat less fat and continue eating a ton of protein.

At Costco I bought protein bars and shakes for my diet. I am also eating a lot of meat, eggs, and anything else with protein. Of course, it is still difficult to avoid some fat.

Overall, it's been a good week. I'm stable and have been fortunate to ride a scooter in Costco for a few hours and go to Church for an hour. Life is good!!

106 days on the list. 15 weeks. All is Well.

106 days on the list. 15 weeks. Any predictions?

Despite the minor physical anxieties of waiting for a heart, always wearing oxygen, taking a bazillion pills, giving myself shots twice a day, and carrying around a bag of medication going directly into a permanent IV in my right arm, I have the larger anxieties experienced by those who are sick for a long time and happily married. Keep in mind that I am too tired to stay busy so all you have to do all day is think and reflect. Above all, you hope your wife, the love of your life, is happy, fulfilled, and appreciated because of her sacrifice, service, and love.

My dad is a journalist and once interviewed one of my hero's Howard Hunter, a former spiritual leader of the LDS church and community giant. The man was soft spoken and humble despite his enormous list of credentials and accomplishments. In the interview my dad asked about all the years that Pres. Hunter tenderly cared for his sick wife who later died in a rest home. "How where you able to do that all those years?" my dad asked. President Hunter with some emotion and pause in his voice said, "She would of done the same thing for me."

I'm also reminded of another time that I was at Bryner Clinic in Salt Lake to see my family doctor. While waiting I observed a feeble old woman waiting for the pharmacist to complete her prescription. She was with her husband. They were both very old, petite and weak. She sat in a wheel chair and was so tired that her head kept falling backward. And I watched this husband, who stood by her side, hold her head up, even though his hands shook and they had to wait for some time. I think he held her head for 20 minutes.

I also remember while serving a mission for my LDS church I was responsible for several missionaries of which were an old couple serving in a small mining town in California. Like all of our missionaries they were responsible to knock on doors and share a message of love about Jesus Christ. The wife had really bad arthritis and knees. At many times she couldn't use her legs because of the pain. But, she didn't want to give up. Her husband drove them in the car from house to house. He would get out and knock on the door while his wife waited. If people said yes to letting them he'd go to the car, open the door, pick up his wife and carry her into the home so they could do what they loved to do most which was bring hope to people through their message.

These several experiences that I observed are always on my mind. And I hope I get the chance to show the same expression of love and service for my wife who has so kindly sacrificed, served, and loved me through this experience. It is much easier to serve than to be served.