What is it that you think about?

(This is me in 1987 recovering from post fontan open heart surgery - Surgeons opened my chest 3 separate times during this stay because of additional problems)

A close friend of mine asked me, “with your situation what is it that you think about?” That is a good question. When you’re waiting for an event to happen that could determine your fate what do you think about?

For me, it’s a chance to take a look back at life and reflect on events and who you’ve become and where you’re headed.

What consume my mind are what matters most to me, my playful little 3 year old and my beautiful angel of a wife.

It’s so unfortunate that millions of men don’t know what I am talking about. Comedian Jerry Seinfeld, who was popular for his long bachelor life, said, when asked about his new-found family life and fatherhood, “What the hell was I thinking? My child and wife are everything to me.”

False alarm. Staying home!

(This is what I sometimes do at the Children's hospital while I wait to have my labs drawn. Of course, the kids boot me off.)

False alarm. And I apologize for crying wolf again but now I am not moving into the hospital on Monday. We met with doctors today an apparently my body is no longer anemic and my protein levels have improved. We did not think that would happen. However, two weeks ago I was infused over a 5 day period with some iron to help improve my blood and give me strength. So this wonderful miracle is great news!

But, it is temporary good news. And again I may find myself up in the hospital with more challenges. You mentally prepare yourself for something and then things change. Life is unpredictable but it keeps moving along for all of us. I love it! And although I have anxieties with waiting for a heart I am truly blessed and grateful for the gift of life.

I am feeling good. So I'll just enjoy this time.

But what a roller coaster!

Moving into the Hospital on Monday? Maybe.

I am moving permanently into the hospital on Monday until doctors can get me a heart and I'm recovered.

WILD!

They'll also up my status putting me at the top of the waiting list.

More coming tomorrow. . .

Do I miss my day job?

People keep asking me if I miss my day job? No, I don’t. At least, I haven’t until today.

I play the piano and run a small independent record label called Stone Angel Music. Our artists mostly record peaceful instrumentals. The music we produce has been a major part of my life.

It was last fall when my health began failing and doctors listed me for the transplant that I canceled a little over 20 gigs and stopped spending time promoting the music.

But, that’s the difficult part. I miss playing for people. And more particular, I miss sharing my life’s philosophy in a concert or fireside setting.

I think I’ve been blessed not to really think about it. I guess it’s the drama of the unknown and the fact that all I can think about is my wife and little girl. I want more time with them and I don’t want anything to get in the way.

I look forward to recovering from the challenging surgery. It will be a difficult road for many months. But, I wouldn’t trade this experience for anything. I am learning a great deal about life and it’s purpose. You come to understand a great deal about the character of your family, friends, and other associates.

I’m getting anxious. I want to do so much in this life and give back to those who’ve selflessly served and sacrificed time for my family and me.

And, yes, I have music in me. It’s building up. I can feel it. Hopefully, with faith, prayers, and the incredible medical team, I’ll get chance to let it out.

Oh yea, listen to some of our music.
www.stoneangelmusic.com

(This is what I do during the day now - but, I don't dress up for the gig. And, that's my wife.)

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.