Archive for the ‘Cyanosis’ Category

Joshua

October 20, 2010

When Joshua Haskins passed away earlier this month there was a great deal of controversy. This post is not about Circumcision safety issues, but about a related and equally important topic.

Joshua’s mother is heartbroken – she’s supposed to be her child’s advocate, his defender, but now he’s gone and she blames herself. But I don’t – she had been a Heart Mom for seven weeks. There was a lot that Jill Haskins didn’t know, including one critical fact that I didn’t mention.

As regular readers of this blog know, I have a hernia. A hernia can be fixed with a simple operation;  when my dad had his hernia repaired it required an overnight hospital stay, but even that may not be required anymore. The average hernia can be repaired pretty easily, but mine can’t.

My Cardiologist has vetoed a hernia repair more than once, because my blood oxygenation level (the familiar PulseOx reading) is low. A Heart Healthy person will register a PulseOx of nearly 100%; but mine is normally 80 to 82%. “I don’t have any doubt we can put you to sleep,” my Cardiologist says. “The real challenge is waking you back up!”

Not only is anesthesia an issue, there’s more. “I could spend all day describing your cardiac anatomy to the operating team,” my heart doctor continues. “And I don’t know if they would understand it any better when I finished.” That’s quite a statement, considering my doctor has won several teaching awards in his career. I’m 44, and one of the open heart repairs I have had has changed greatly over the years. Another one just isn’t done anymore – there are only three places that I know you can find it: 1) in a musty old surgical textbook; 2) in the brain of a very old cardiac surgeon; and 3) in my chest.

If my hernia were to cause enough trouble that it had to be repaired, I couldn’t just have it done anywhere. I can’t run the risk of having a surgeon who has done this operation 12,759 times before and considers me just another patient, because I’m not. Because of my heart, the plan is to go to Emory University Hospital and have my hernia repaired with a Congenital Heart Surgeon in the room, just in case of trouble. Afterwards they would keep me in the Intensive Care Unit for at least 24 hours – again just in case.

I know this… and I never mentioned it to Joshua’s parents. I don’t recall them mentioning the circumcision to me before it occurred, but that doesn’t matter. I should have said “Look, with Joshua’s heart, there is never going to be a ‘simple’ medical procedure. More than likely he’ll even need antibiotics before and after he sees the dentist, so you really need to think about any kind of operation or procedure that can be avoided.”

But I didn’t say that.

We are each put on this Earth for a reason, to accomplish something. Perhaps Joshua’s task was to remind us all that when you have a Congenital Heart Defect, no medical procedure can be considered “routine.”

Scream

October 17, 2010

Funky Heart! readers know I am an optimist; hopefully you also think of me as honest. And while I try to live life to the fullest despite having a Congenital Heart Defect (“I view my CHD as a challenge, not a curse,” a friend often says) sometimes it just gets to you.

This week has been one of those times.

My hand seems to be no better. It could be just a tiny bit better; but I want to improve by leaps and bounds. It seems that I am moving forward by millimeters, if that much. I’ve had a couple of gout flare-ups – easily controlled with the medication, but until the meds kick in I hurt. Both of my ankles and my right wrist and hand.

Another complaint, my hernia is acting up. Not the one that flares up every now and again, I know what to do to get that one under control. When I feel the first twinge of pain, I know to stop as soon as possible and lie down. I know how to maneuver it back into place, and I know where to put pressure to keep it down. If it were to hit me right now, I could likely have it under control in half an hour, perhaps a little more. This is the hernia on the other side, which rarely (if ever) bothers me.

Adding to my misery, this morning it was pretty cool here. The low was in the 40’s, which wouldn’t bother most people. But I’ve always been intolerant of the cold, and I felt like I was freezing. Observe me in church – why do I sit in one particular spot every Sunday morning? That floor to ceiling window that allows sunlight to fall on my pew has a lot to do with it! (I swear, I think Fall and Winter used to be warmer. In the early 1970’s the church would host a yearly Halloween Carnival on October 31; I used to be outside for practically the entire festival. Nowadays by mid-October I am freezing.Weren’t we supposed to be going through Global Warming?)

It’s nothing big; no Earth shattering health problems are going on. But a lot of little things have happened to get me down and throw me into my own little pity party. And I hate pity parties.

But days like today make me want to SCREAM!

The PulseOx test – not perfect, but good

September 21, 2010

If you have been reading Facebook or Twitter this past weekend, you’ve probably seen the news: The Health and Human Services’  Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) voted to recommend pulse oximetry screening for critical congenital heart disease be added to the newborn screening uniform panel.

According to what I have read, the Health and Human Services Secretary now has 180 days in which to consider the recommendation and decide to either accept it or reject it. Historically the Secretary acts fairly quickly and usually accepts the recommendations given by her advisors, so the major hurdle could well have been cleared. It could only be a matter of time before the Pulse Oximetry becomes standard practice.

No PulseOx testing protocol exists yet. The official recommendation by the Health Resources and Services Administration addresses this issue, by stating “The Health Resources and Services Administration shall guide the development of screening standards…

This will delay the process – with no testing protocol, the recommendation can’t be accepted on a Tuesday and the program begin at 12:01 AM Wednesday. For the results to mean anything, a medical test must be administered in the exact same manner each and every time it is given. For example, I need to have some blood drawn this week. The nurse will apply a tourniquet to my arm, inset a needle into a vein in the crook of my elbow, and draw three vials of blood. Everyone else, no matter where they are, who has those tests done will go through the same process. Protocols are an essential part of medical testing. As this 2008 report notes, “Nursing staff received in-service training in the screening protocol and performed the pulse-oximetry procedure.”

In this study the type of  Oximeter is specified (under the heading “Methods”). This is also an important part of the protocol – perhaps not the exact same type of testing equipment, but a standard that must be met, and probably an agreed on calibration procedure. If you step on and off a manual scale enough times, the pointer will no longer return to zero… and your weight will be inaccurate until the scale is “zeroed out.” A pound or two won’t make much difference, but a point or two difference on a PulseOx reading could mean alarmed parents and unneccessary testing. A proper testing protocol would even specify a testing location, as studies have shown slightly lower PulseOx readings when the test is performed on the foot. Fussy or crying babies also produced lower saturation numbers.

Despite what you may have heard, the PulseOx test isn’t that accurate when performed on heart defects that do not cause Cyanosis. Cyanosis (medical term: Hypoxemia) comes from the root word Cyan, meaning “blue,” and is caused by low levels of oxygen in the blood. Cyanosis can be hard to detect, as the oxygen level has to drop below 90% before it begins to appear. Cyanosis will cause the lips, fingers, and toes to have a blueish tinge. (SEE THIS ILLUSTRATION) Non-Cyanotic (also known as “acyanotic”) Heart Defects account for 70% of all defects. When these defects are present, blood oxygenation (and therefore the PulseOx reading) is usually normal. In fact, the recommendation is only meant for CCCHDs, or Critical (sometimes the word Complex is used) Cyanotic Congenital Heart Defects.

While the Pulse Oximetry screening test is not the complete answer for detecting Congenital Heart Defects; it is certainly part of the answer – another piece of the puzzle; another arrow in our quiver. My idea – and I don’t know if it is just a pipe dream, or a discovery we haven’t made yet – is to find a “bulletproof” test that indicates the presence of a heart defect. Does a defective heart release a gene, an enzyme, or any kind of biomarker into the bloodstream?   If we could find that marker and learn how to detect it… Gotcha!

But until that day comes – if it ever comes – you throw everything but the kitchen sink into the fight.

One Year

September 9, 2010

…who knoweth whether thou art come to the kingdom for such a time as this? -Esther 4:14

One year ago Paul Cardall got the news:  The heart he desperately needed for transplant was available! Since then he’s resumed his career as an award-winning musician, started a Foundation that grants scholarships to CHDers to attend college, written a book, and become one of the most effective Congenital Heart Defect Advocates that I know of.

Congratulations, Paul! Even though we have never met in person, I am proud to call you my friend and fellow Heart Warrior. Thank you for all you do to fight heart defects, and may your new heart be continually filled with the love of your friends and family.

Yours;

The Ol’ Man

July 12, 2010

When I head to Houston, I’m going to be carrying all new gear!

Sorry, no Greater Regional Memorial Hospital has decided to sponsor the Funky Heart. (Well, no hospital or other organization has offered yet… maybe someday!) The reason is a little more mundane than that:  I’m getting older, and my carry-on is getting heavier.

As far as I know, I am the second oldest living person with Tricuspid Atresia. My Cardiologist told me that I was his oldest TA patient a couple of years ago, then I met a group of other Tricuspids at the Adult Congenital Heart Association’s national convention in Philadelphia. Most of those people were in their 30’s – except for Pam, who is *AHEM* years older than me. (I’m not stupid, I know you don’t reveal a woman’s age without her permission!) So at 43 years old, I think I’m the second oldest person there is with this “fatal” disease.

But my laptop seems to be getting heavier and heavier with every step. It’s a good machine, it just has a few years on it and it weighs in at about five to six pounds. And my computer case is just that, a case, and there isn’t much room for my pills. My ticket and flight schedule – all the paperwork associated with a trip – fit in a folder that slides into the case easily, but my pill containers barely fit, especilly if it is a longer trip. And the Eleventh Commandment states, Thou shalt not pack thy medicine in thy checked luggage; lest thy bag go to Detroit while thy go to Denver. So I wouldn’t mind a new laptop case, either.

The carrying case came first; I got a Swissgear ScanSmart bag. Instead of a case, the ScanSmart is a backpack – it’s bigger and deeper and has more storage space. And it is light, too! I would highly recommend this bag – you don’t even have to take the laptop out of the ScanSmart when you go through airport security. The Transportation Security Authority (TSA) says that you don’t have to remove your laptop IF the laptop is isolated so that the x-ray unit can “see” above and below the laptop. Open a zipper on the ScanSmart and it lies flat like a clamshell; one side contains all your gear and the other side contains  the computer – and nothing else. No problem at all for the TSA. (Of course, they do reserve the right to search any package or bag. Hopefully mine will make it through without any questions!)

And next came the computer – I had to look a little bit before I found the one I wanted. I finally purchased a Toshiba Netbook that weighs two and a half pounds. It works great, but I don’t know all the little tricks and shortcuts that make it work better just yet. That has me a little worried – I hope to be blogging from the conference in Houston, not reading the instruction manual!

Of course, getting older is a good problem to have and something I am looking forward to. If events had occurred as some top doctors had predicted, I would be a 12 inch long grave in the cemetery behind my church, long forgotten by just about everyone.

I’m really looking forward to my impending “Ol’ Man!” status!

Ask Questions!

April 6, 2010

Here’s a scary report, courtesy of Kevin, MD: Patients don’t ask questions of their doctors.

While there are a precious few patients who are totally involved in their health care, the vast majority just take their doctor’s advice at face value. A 2008 study found that when 181 people were prescribed a new medication, they asked a total of 199 questions (or made a comment) about the new drug. That’s an average of 1.09 questions/comments per patient!

What’s worse, the same study showed that the doctors didn’t talk, either. The average office visit was 15.9 minutes, and the patient and doctor spent an average of 49 seconds discussing the medication. The length of discussion ranged from a high of 351 seconds (5.85 minutes) to an amazing 1.9 seconds! (What can you say in 1.9 seconds?!?!)

As noted before, patients who are more involved in their own health care ask more questions. That’s you. Having a Congenital Heart Defect means that you are, for all intents and purposes, a patient pool of ONE. Others may have the same defect that you do, but no CHD ever treats its owner like everyone else.

As I’ve written before, I have a hernia. It’s usually well-behaved, but occasionally it will get pretty angry with me. A hernia repair is a fairly simple operation these days, and usually doesn’t even require an overnight stay… except for me. My Cardiologist does not want to authorize the operation, instead asking me to just fight through the bad times by prescribing couch time and TV. “I could spend a day explaining your anatomy to the surgical team,” he has said. “And they still wouldn’t understand it.” It’s not that he can’t, my doc has a couple of teaching awards to his credit. I’m complicated.

So if you don’t know what’s going on with your body, it is time to learn. And ask questions – what is this medicine supposed to do? What are some of the side effects? What do you think would happen if I decided not to take this drug? Are there any other options available? All of these are legitimate questions – and if your doctor gives you an answer in 1.9 seconds, ask another question. You can control how long he talks to you. Conversely, you can find a doctor who will spend the time needed to help you make a good decision.

It’s your body, and the medical decisions you make affect you, and rarely anyone else.

Late Isaac Update!

April 3, 2010

If all continues to go well, Isaac very well could come home tomorrow!

Hmm… since he’s a patient at Duke, I wonder if Coach K would let him play in the National Championship Game Monday night? (Assuming Duke wins tonight, of course!)

Probably a little soon for that!

Bing

April 2, 2010

He was born in Germany and composed many works of classical music. He graduated from medical school twice. He worked with Charles Lindburgh. And he started a Cardiac Catheterization program at the hospital where heart surgery was born. Not only did Dr. Richard Bing see history being made, he helped make it.

Hired in 1942 as an instructor in the Johns Hopkins University Department of Medicine, Bing traveled from New York to his new job in Baltimore. Crossing the Chesapeake Bay on a ferry, Bing noted no less than six people with Cyanosis. Their destination, he surmised, was probably the hospital. It was two years before Blalock and Taussig would attempt the first Congenital Heart Surgery, but Johns Hopkins already had a reputation. If your child has a bad heart, it was said, get them to Hopkins.

There wasn’t much at all that could be done, but Dr. Helen Taussig knew almost everything there was to know about the human heart. Taussig was a huge believer in research, and would spend hours examining defective hearts. Despite her best efforts – she often asked the parents of her deceased patients for permission to dissect the heart, and spent hours studying them in her lab – there was very little progress being made. Even after the first heart operation, very little was known. There was ONE operation, and it was designed to help relive only ONE defect. Everyone else was still out in the cold.

By 1945  Dr. Alfred Blalock asked Bing to set up a Cardiac Catheterization unit at the hospital. Dr. Helen Taussig, who had already been eyeing him suspiciously, hit the roof. Dr. Bing explained their constant disagreements:

She was very jealous and guarded her territory; she considered the sick children as her own, having no family herself. She was also deaf, which increased her suspicion of the world beyond.

(That link contains not only a good description of Taussig-Bing Anomaly, but an in-depth look at Johns Hopkins Hospital during the Blalock-Bing-Taussig years. I highly recommend reading it!)

Dr. Bing eventually left Johns Hopkins, continuing to do major research in the Cath Lab. While at Wayne State University he did some of the early work involving PET scans, using computers belonging to Ford Motor Company to run the scanner.

Dr. Bing retired and now lives comfortably in California, and last year celebrated his 100th birthday!

Pulse Oximetry explained

March 30, 2010

A Pulse Oximeter (PulseOx) is a useful tool, but exactly how does one work? Obviously, it’s not magic, though it might as well be. Very few people can tell you what happens in the couple of seconds that occur after you place it on your finger.

Pulse Oximeters were invented in the 1940’s by scientist Glen Milliken, who published his ideas in a research paper titled The oximeter: an instrument for measuring continuously oxygen-saturation of arterial blood in man. But it looked nothing like the Oximeters we have today: those wouldn’t appear until the Japanese refined the technology in 1972.

But the average person still wouldn’t have been able to purchase a Pulse Oximeter. Biox made it into a commercial product in 1981, and it was the late 1980’s before they really began to catch on in the United States.

So how do they work? A Pulse Oximeter emits light from two different sources: a red light that you can see, and an infrared light that you can’t see. As the PulseOx is placed on your finger or ear the lights activate automatically.

The red light (which has a wavelength of 660 nm) shines through the finger and is absorbed by hemoglobin. The problem is, everything else in the body absorbs light, also. So the infrared light is used too – it has a higher wavelength and is absorbed at a different rate than the red light. A receiver picks up the light waves from both sources as they leave the body, and the Oximeter’s computer chip compares the two. The comparison values are entered into a mathematical formula (CLICK HERE and page down to see the formula that makes Pulse Oximetery possible) and the answer is displayed on the screen – all in a matter of moments!

If I had to do the math to figure out the Oxygenation level, It would take all day to get one reading!

Oximetery does have its limitations: while most people think it is a beat to beat accurate measurement, it is not. It is an average over time. Another big drawback is that it can be fooled by Carbon Monoxide poisoning. hemoglobin mated with Carbon Monoxide responds like oxygen mated Hemoglobin, so a patient dying of Carbon Monoxide poisoning will give a PulseOx reading of 90% or higher.  Also, dark skin could cause inaccurate readings. This 2007 report contends that it does; later reports either report no effect or draw inconclusive results.

Change of Heart, Part II

March 29, 2010

Sharon concludes the story of her husband’s heart transplant. I’ve also included a link to Part One just in case you missed it!

It’s been a year since these events took place, and Anthony continues to do well with his new heart! And be sure to leave a comment (on Sharon’s blog, not here!) wishing her and Anthony the best!

If you haven’t signed an Organ Donor card yet, why not?

Heart-a-versary, Part I

Heart-a-versary, Part II