Good News!

On June 24th we scheduled a doctors consultation to plan Daniel's open heart surgery. This was to check the status of Daniel's condition and plan for his surgery. We both knew it would take place in SF, but weren't sure where we would all stay and what to expect. The past six months leading up to this have been filled with much uncertainty and dread with the idea of Daniel on an operating table with doctors cutting into his small lifeless body. So with heavy hearts we brought him to the appointment expecting to hear the worse.

Skipping through the process of arriving, sitting in the waiting room, and eventually waiting for the nurses to check Daniel's vitals. I noticed something was a little out of the ordinary.
The nurse took his blood pressure two times. The first time it read 126, I can never remember the second number. She said that the reading was not right and checked it again and it was something like 107 over something. I thought to myself, "for a boy who's condition gives him high blood pressure, that sure isn't very high". After all of the tests were completed, we were lead into the room where the echo cardiogram was going to be performed.

Daniel got hooked up to a bunch of electrodes all the while watching, "Bob the Builder". The cardiologist slathered the gel on Daniel's chest and started moving the sensor thing all around trying to get different pictures of the heart. I was amazed to see his heart in such clarity as it pulsated. The whole time I was expecting the woman doing the echo test to say, " oh, here it is!", but nothing. As time passed, I could sense some frustration from her. She eventually started complaining that there might be something wrong with the electrodes connected to Daniel, and they must be "dry". Whatever that means.

After a long time the woman finally informed us that the wall thickness to Daniel's heart was normal. Usually someone with this condition would have the left side of the heart thicker or bigger than the right side. This would be due to creating higher pressure to push the blood past the narrowing of the aorta. She showed us the Aortic valve, it had three separate "leaf like" flaps that were opening and closing with each beat. In the last check up two of the flaps were partially fused together, another sign of his condition. We were asked if the initial doctor said anything about Daniel's condition getting better. We said no he didn't.

She then left us to get the doctor who came in to take a look at the readings. He and the assistant (I think that was what she was) conversed a little and then he turned to us and told us that Daniel's condition had almost completely disappeared! After checking Daniel's vitals, he told us that the blood pressures in both lower and upper were normal. He did say that he (Daniel) still had a murmur, but that the coarctation was small enough to not effect his blood flow. He told us that we should still come back every year to monitor, but, with enthusiasm, said he will not need surgery!
Something important the doctor told me was this:

"...the reason why children have surgery is because the coarctation impedes the blood flow causing high blood pressure. After the surgery, scar tissue will form and create its own narrowing, but we rely on the surgery to lessen the disruptive flow enough to bring the blood pressure down to safer levels. In Daniel's case, surgery would in fact worsen his condition..."


The funny thing about this whole experience was the comparison between the woman who took the echocardiogram on Daniel and the doctor. One seemed disappointed about not finding problems and the other was very happy that Daniel would not need surgery.









The good news took a while before it finally set in and we celebrated over a Chicken Cesar pitta wrap at La Bou!

Well, I guess all we need to worry about now is Daniel's normal life of rascalism, rambunctiousness, and tormenting his older sister!

This ordeal has proven that miracles really do happen! I would like to thank everyone for their prayers and to especially to thank God for healing my son!

Statistics and Symptoms You Might Want to Know About

I would like to apologize to everyone for the great delay in posting any updates on Daniel's condition and further information about Coarctation of the aorta. In the past two months, work has heaped itself upon me and I could do nothing but welcome it with open arms!

The Symptoms:
Sometimes it can be difficult to tell if a child has Coarctation of the aorta. These are some signs a radiologist looks for in a ecocardiogram:

• Widening of the left subclavian artery with or without an apparent double aortic knuckle and with modification of the oesophageal impression. I have no clue what that means.
• Discrepancy of pulsation between the two components of the double aortic knuckle.
• Demonstration of the coarctation by angiocardiography.
• Heart murmur

I decided to add the heart murmur, because we knew Daniel had one since he got his first immunizations. The doctor's didn't seem alarmed about Daniel's heart murmur, so neither were we. We were always told that the murmur would eventually go away.

I found the outward symptoms to be interesting and helpful. They are:

• pale skin
• fainting
• heavy sweating
• high blood pressure
• frequent nose bleeds
• frequent headaches
  
• leg cramps
• visible pulsation of the neck

The greatest symptom is a significant difference in blood pressure between the upper and lower extremities of the body. Now your you or your child may not have all of these symptoms, but if you notice more than one, then I would consult with a doctor. However, the sad story about doctors is that they usually will shoot down any upfront diagnosis you give to them unless it was by another physician. Liesl had to be pushy with her doctor before he suggested sending Daniel to a specialist. Perhaps doctors view suggestions by anyone other than another physician to be an insult to their position and education. On a side note. The best way to approach a doctor is to make it seem like they are coming up with the solution by you asking simple questions to confirm your initial opinion of what's going on. But that's just my opinion.

How common is Coarctation of the Aorta?
The probability of a child born with Coarctation of the aorta (COA) has been found to be 1 in 80,000. Of that number male babies have a 3 to 1 higher chance of having it over girls. About 50% of children/adults of those who have COA also had a heart murmur as one of their symptoms. COA makes up anywhere from 5% to 10% of all congenital heart conditions.

What happens if nothing is done about the condition?
The severity of each condition varies among children and adult, but the typical lifespan is between 34 and 40 years of age. I read one journal that said," infantile cases die in the first decade. The ones with symptoms before 15 die before the age of 30." The message I get from all of the journals I have read is simple. The earlier COA is detected, the better.

What Coarctation of the Aorta is and how it happens?

What it is:

Coarctation of the Aorta is the congenital heart condition where there is an abnormal narrowing of the aorta. In my previous post I called it Aortic Coarctation, but according to the ICD-10 (International Statistical Classification of Diseases and Related Health Problems 10th Revision) the official name is Coarctation of the Aorta. Coarctation is located in the aorta right after the arteries leave the heart. There are three different types Preductal coarctation, Ductal coarctation, and Postductal coarctation. The different types are classified on where the narrowing is located in relation to the ductus arteriosus (ligamentum arteriosum). I haven't asked the doctor which one it is, but I'm guessing it is postductal.

Coarctation of the aorta has been linked to other heart problems such as: Bicuspid aortic valve, Ventricular septal defect, Patent ductus arteriosus, Aortic valve stenosis, Mitral valve stenosis, and Turner syndrome.

When and how it happens:

While the fetus is developing in the womb, the Ductus arteriosus (DA) appears as a shunt connecting the Pulmonary artery to the aorta. This is to help the lungs and strengthens the right ventricle. After birth the shunt normally closes with in a week. The shunt, or blood vessel, has tissue in it that allows itself to close when the baby takes it's first breath. This tissue could be the very thing extending into the aorta and causing it to narrow when it contracts.

Aortic coarctation claims my son!

It seems like once you get a certain grip on life, life fires back a curveball when you least expect it! Living in California has been a blessing and a hassel. My wife and I have both enjoyed the weather and people here. The cost of living is really difficult to keep up with, and with loosing my job last September didn't make things any better. Things started to look up for us when I landed a contract position for the company who laid me off. Then about a month ago we found out that my youngest son, Daniel, had Aortic coarctation, or Coarctation of the aorta.

People have told us how lucky it was that we caught it so soon, and by accident. I would have to agree with them. One night the family was finishing their dinner and decided to take pictures of everyone up close. When I got to Daniel I noticed his uvula was split! I had never heard of this before and thought it was the coolest thing ever! After much research Liesl and I found out that split uvulas had been linked to Loeys-Dietz-syndrome, which can lead to death if not caught in time.

Well, we took him to a cardiologist, and he was not diagnosed with Loeys-Dietz-syndrome but with Aortic coarctation. This means that Daniel will need to have open heart surgery. Although the actual manner in which the surgeon will fix his condition is uncertain at this point. The biggest question is when the time will be right to do the surgery. Daniel is only two years old! I think the doctor wants to wait a little longer, so in about six months we will be taking him back for a checkup. I figure anyone with small children would be interested to follow this blog, because I will be posting updates in Daniel's condition, and in the research performed.