Saturday, January 6, 2007

As I sit and reflect......

on life, I think about all of the trials Rhett has been through. He came into the world so small, and had alot of problems. When he was first born, he had a blood disorder, where basically his blood had a consistency of oil. This comes from his body making too many red blood cells. While in the NICU, they performed an infusion to correct it, where they pull blood out of his body, and mix it with basically a saline solution, to thin it out, and then put it back in his body. Luckily the first time they did it worked, and they didn't have to do it again.

The NICU, was a great place for him to be, they took really good care of him. Sometimes too much. The nurses were so over protective of him, sometimes they wouldn't let me take care of him. I finally quit asking if I could do stuff, and would just go over and pick him up, wires and all. After all he was my fourth child, I think I know how to hold and feed a baby.

I think the hardest thing resulting from his birth, was the open heart surgery. I never had any problem whatsoever with the fact that he had Down Syndrome. I felt very excited and blessed that our Heavenly Father had chosen us to take care of one of his perfect children. When we were sittin by his warmer and Dr. Judd was performing his Echocardiogram, I was very uneasy. Through all of the ultrasounds, nobody had ever given us any indication that there was any problems, and I had ALOT of ultrasounds. There was never any indication that he had Down Syndrome either, but I'm cool with that.

When Dr. Judd started explaining everything that she was seeing, I just got sick. I knew that heart problems were very common in children with Ds, but every indication had told us that he had escaped it. Dr. Judd was so wonderful and so kind at answering all of our questions, and explained that he had an AV canal, and a VSD heart defect...yea two for the price of one, was all I could think. I was so shocked I didn't know what to do. I spent the night at the hospital that night, and just cried my eyes out. How was I going to be able to handle it. I was so afraid that he would die from this. THAT is something that I cannot handle. I had to go up and nurse him everytime he woke up, and the first time I went up after hearing the news, I disscussed it with his nurse.

That same day was the day that we got the chromosome test back, and I was telling her that felt so lucky to have him, but that it just broke my heart knowing what he was going to have to go through, with the open heart surgery. She looked at me with tears in her eyes, and was all choked up, and said, " I see alot of babies come in and out of here, some with Down Syndrome, and some without, and you and your husband have got to be one of the most loving, and sincere parents I have ever met. The love that you are showing this little boy now, will get him through anything he will have to endeavor." I knew then that I could do it. I will never forget her.

As the time grew closer for his surgery I was still so worried, and things were so crazy. He was so sick, his SATs kept dropping, he was on huge amounts of lasix, and you could see his sternum every time he breathed. He was almost 5 months old and was only 8 lbs. He was sooo tiny. The night before his surgery I was very uneasy, so we called over a neighboor and a member of our bishopric, to come and give him a blessing. I will never forget the spirit that was in the room that night. There was just this huge amount of peace that I cannot explain. After the blessing, the member of our bishopric looked at me and said, "He is going to be just fine, there is no doubt in my mind".

The surgery was not without complications, and Rhett was very unstable the first four days, he had so much swelling around his heart, that they had to leave his chest open for 24 hours, and he had a really hard time coming off of the heart lung bypass machine. His body was not reacting to the stress he was under, so his cortisol levels were really low, which would cause huge dips in his blood pressure. It is not a good thing when your child has a doctor sitting by their bedside 24/7. They had a really hard time keeping him sedated, and after 3 days of so much medicine, he was so sleepy he couldn't come off of the vent untill it wore off. After 4 days he decided that he was no longer going to be a living pin cushion, and he started progressing.

It was a long recovery, and he still needs O2 at night, because of pulomnary hypertension, but all in all he is doing really well. We just broke the 20 lb mark this week, and he is going to be 15 months old tomorrow. If it's one thing I have learned though this it's been that God never gives you anything you cannot handle, and also that everything he does is for a reason. Andy and I have become so close throughout all of this, and I have learned that we can make it though anything as long as we support each other. I love him, and all of my kids, I would not trade my life with anyone.

Here is some excerpts of the heart condtion, that explain exactly what it was, and also a descripton of pulomonary hypertension:

Congenital heart disease associated with Down syndrome
The overall incidence of congenital heart disease in the general population is 0.8 percent. The incidence of congenital heart disease in children with Down syndrome is up to 50 percent.

The types of heart defects in children with Down syndrome can be broken down into three broad categories:

Atrioventricular septal defects

Ventricular septal defect (VSD), atrial septal defect, or patent ductus arteriosus

Other complex heart disease
Atrioventricular septal defects (AV Canal) is greatly over-represented in these children, making up approximately 60 percent of the congenital heart disease found in Trisomy 21. For comparison, AV canals account for only 2.9 percent of the congenital heart defects in the general population.

Ventricular septal defects, atrial septal defects, and patent ductus arteriosus comprise another 20 percent of the congenital heart disease associated with Down syndrome, although these defects are much more common in the general population compared to AV canals.

Other complex congenital heart diseases including Tetralogy of Fallot and hypoplastic left heart syndrome comprise the rest of the heart disease seen in Trisomy 21.

In addition to the heart defects associated with Down Syndrome, isolated elevated blood pressure in the lungs (pulmonary hypertension) is seen with higher frequency in patients with Down syndrome. This high pressure may be related to malformation of the lung tissue, although the exact cause is not known.

The higher pressures may limit the amount of blood flow to the lungs and therefore decrease the likelihood of symptoms of congestive heart failure seen in babies with complete AV canals or large ventricular septal defects.

However, since the pulmonary hypertension can become irreversible, particularly if these large holes are not surgically corrected by a year of age, children with Down syndrome and AV canals or large ventricular septal defects are often referred for surgery earlier than a non-Down syndrome child with the same heart defect.

If the baby does have evidence of high pressures in the lungs, it may complicate postoperative management and hence prolong the recovery time.

The most common in babies with Down syndrome is called an atrioventricular canal defect or AV Canal, as it is commonly called. An AV canal is a large hole in the center of the heart. This means that the walls between the two upper chambers (the atria) and the two lower chambers (the ventricles), as well as the valves between them, may be deformed. This large opening in the center of the heart allows the red oxygenated blood to mix with low-oxygen blood and return to the lungs. This extra effort in turn causes the heart to enlarge. In addition, the body receives less oxygen since it receives red blood that is mixed with low-oxygen blood. Most infants with AV canal grow very slowly and remain small. Because of the high volume of blood that is pumped into the lungs, high blood pressure may occur there, resulting in damage to the lungs and blood vessels. Surgical repair of an AV canal usually restores blood circulation to normal.

Another common heart defect in babies with Down syndrome is the ventricular septal defect (VSD) A VSD is a large opening between the ventricles in which, as with AV canals, oxygenated and deoxygenated blood mix, resulting in similar problems to AV canals, including low blood oxygen levels, an enlarged heart, and hight blood pressure.

What is primary pulmonary hypertension?
Primary pulmonary hypertension is a clinical syndrome where the blood flow that leaves the right side of the heart faces an increased resistance (pressure).

Normally blood flows from the right ventricle into the pulmonary arteries and then flows into smaller and smaller blood vessels until it reaches the capillaries (thin walled blood vessels near the small air chambers of the lungs or alveoli). There gas exchange occurs where oxygen is picked up and carbon dioxide is released. The blood vessels have muscles in their walls that can regulate their diameter and thereby allow more or less blood flow to enter the capillaries.

In primary pulmonary hypertension, the blood vessels of the lungs have an increased amount of muscle in the walls. This causes a higher resistance in the lungs. The right ventricle then has to work harder to pump blood out to the lungs. The right ventricle will enlarge and thicken in response to this extra work. With time, the extra work placed on the right side of the heart can cause it to fail

Primary pulmonary hypertension causes
No one knows what causes primary pulmonary hypertension. It may also be referred to as idiopathic pulmonary hypertension. There are causes of elevated pressures in the lungs that are secondary or a result of some other process.

Certain types of congenital heart disease can, if left unrepaired, lead to high pressure in the lungs. Primary lung problems such as cystic fibrosis or bronchopulmonary dysplasia (BPD) can lead to elevated resistance in the lungs.

Infants are normally born with high pressures in the lungs which over the course of the first few hours to few days of life resolve.

However, some babies continue to have high pressures in the lungs even after this time and are referred to as having primary or persistent pulmonary hypertension of the newborn. Unfortunately there is no cure for primary pulmonary hypertension. There are some treatments that have been found to relieve some of the symptoms and slow the progression of the disease

Oxygen is a drug that can be used at home and can often help relieve some of the symptoms of difficult breathing. It is also known to relax the muscles in the arteries of the lungs, which will lower the pressures in the pulmonary arteries.

As far as I am aware, Rhett's may resolve itself in time, and hopefully he will be off of oxygen this summer. We will take him anyway he comes, and love him forever.


Tammy and Parker said...

I have to tell you that the mere words primary pulmonary hypertension bring great fear into my heart. Luckily it looks as though Parker's PH has been due to an extremly underdeveloped respiratory system, a 90% occulsion from his adenoids alone, and chronic lung disease. As we have been addressing these issues his PH numbers have been slowly....ever, ever, ever, ever so slowly coming down. His tricuspid valve no longer leaks and you can no longer hear the clacking noise it once made. Yup. Clacking.

Being chronically ill hasn't helped issues much either.

We just keep hoping and praying.

But it sounds as though Rhett is doing beautifully!

Anonymous said...

My Miss E doesn't have heart problems, but she is still medically fragile as a result of her respiratory issues. She also has an abnormal vascular system, always having a high potassium and iron level, her blood hemolyzes so quickly. It's hard to get quality blood draws.

I know firsthand how difficult it can be when all we want to do is take their health concerns away, to allow them to have less in terms of medical treatment and care.

I too have had that experience with the nurses trying to do everything, allowing us to do not much of anything. While it is an annoyance, at least we can rest easy knowing they have the best interest of our child in mind.

It sounds like Rhett is a real fighter, and such a handsome boy. I am glad to hear that he is doing better, you have come a long way.

jennifer said...


I started a new blog called Pinwheels, and I'd like to link to your site if it's okay? I think there are a lot of other moms with kids that have heart issues and your posts and experiences will be of great value to them.

You can check it out at:

Thanks for taking the time to think about it, and thanks for your post!

Miranda said...

Rhett is such a little trooper. Thanks for sharing his story. I have a 3 year old daughter who has T-21. We have been lucky with the health issues.

Would it be O.k if i linked to your site?

Jodie said...

Rhett is a beautiful boy! My name is Jodie. We have a sweet 2 1/2 year old boy, Tyler, with T-21. I'd love to link to your site if it's okay. Please feel free to link to mine as well. :)

Jen said...

Hi...I found your blog via Rebecca at The Quiet Storm. My 6-month-old son Evan also has DS and an A/V Canal, which is scheduled to be repaired this coming Friday 1/12. I am a bag of nerves just waiting for it to all be over with. It looks like Rhett is doing great!