Help Iya overcome Tricuspid Atresia, Pulmonary Atresia, and Atrial Septal Deffect

Last October 2006, I made a blog post encouraging people to give prayers for my niece - Iya. It was an emotional moment for our family that got me crying for a week whenever I think of my sister and her daughter's condition. If not for a miracle that happened then, we realized that she might leave us anytime.

Jhermie, the original life blogger in our family, gave birth to Iya last July 6, 2006. At that time, Iya was not feeding very well thus they have to monitor her and do some tests. She was confined for 3 more days at the hospital. When they brought her home, the couple still continued her medication for a week.

Everything went okay afterwards. Iya’s very happy and healthy until when she was 3 months old. They noticed she’s more irritable, crying a lot, and having difficulty breathing.

It was October 2006 when they decided to go to a Pulmonary Specialist. They thought at first she's just asthmatic. The doctor said she doesn’t hear any wheezing sound that would suggest she has asthma. So the doctor suggested for Iya to have an x-ray and consult it to a Pediatric-Cardiologist.

After the x-ray, the Pediatric-Cardiologist noticed that Iya's left ventricle is larger than its normal size and suggested Iya to have a 2D Echo. The following day, in the 2D Echo, the doctor said casually that Iya has a Congenital Heart Disease called Tricuspid Atresia (TA), Pulmonary Atresia (PA), and Atrial Septal Deffect (ASD).

Jhermie thought it can be treated until the doctor said that they need to brace themselves because Iya could live for only 5 years even if they do all the operations needed! The whole family was shocked! Jhermie just hold her baby tight and was so dishearten to think that Iya could be gone soon! I remember going to the Philippine Children's Medical Center and thought of cheering my sister up, give her hope. But one look at her, I broke into tears as well.

Tricuspid Atresia is a congenital heart defect that occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy. The tricuspid valve, normally located between the right atrium and the right ventricle, does not develop properly during pregnancy.

Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into left ventricle and then is pumped through the aorta out to the body.

In Iya’s case (tricuspid atresia) the following occured:

• The Tricuspid Valve did not develop so the blood cannot get to the Right Ventricle and that ventricle in turn also is severely underdeveloped.
  
  
• Usually only a small part of the Right Ventricle is developed and is supplied with blood through a hole from the Left Ventricle called a Ventricular Septal Defect (VSD).
  
  
• A hole between the two atriums called a Atrial Septal Defect (ASD) is essential for the blue blood coming back from the body to get into the heart to circulate.
  
  
• The blood which is coming back to the Right Atrium has nowhere to go other than to go through the Atrial Septal Defect. When it gets through the ASD it mixes with the pink blood coming back from the lungs into the Left Atrium.
  
  
• The Pulmonary Artery and Valve are also severely underdeveloped. Since the Right Ventricle did not develop normally the artery coming out of it and its branches do not develop normally.
  

In order for Iya to survive, these are the treatments that she will need:

• Blalock-Taussig shunt
  The first operation creates a pathway for blood to reach the lungs. A connection is made between the first artery that branches off the aorta (called the right subclavian artery) and the right pulmonary artery. Some of the blood traveling through the aorta towards the body will "shunt" through this connection and flow into the pulmonary artery to receive oxygen. However, the child will still have some degree of cyanosis since oxygen-poor (blue) blood from the right atrium and oxygen-rich (red) blood from the left side of the heart mix and flow through the aorta to the body.
  
  
• Glenn shunt
  After 6 months is the second operation the doctor will replace the Blalock-Taussig shunt with another connection to the pulmonary artery. In this operation, the Blalock-Taussig shunt is removed, and the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery. Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen. However, oxygen-poor (blue) blood returning to the heart from the lower body through the inferior vena cava will still mix with oxygen-rich (red) blood in the left heart and travel to the body, so the child will remain mildly cyanotic. This operation helps create some of the connections necessary for the final operation, the Fontan procedure.
  
  
• Fontan procedure
  This operation will be performed when she’s about 6 to 8 years old, and allows all the oxygen-poor (blue) blood returning to the heart to flow into the pulmonary artery, greatly improving the oxygenation of the blood. The Glenn shunt, connecting the superior vena cava to the right atrium, is left in place. A second connection is made directing blood from the inferior vena cava to the right pulmonary artery. This connection can be created in slightly different variations.
  

Jhermie sought a second opinion and got referred to an Interventional-Cardiologist. Last October 15, 2006, Iya was scheduled for an Angiogram at PGH and found out that the diagnosis were really true. Fortunately in the Angiogram, they visualized a large collateral artery which supplies blood to the pulmonary artery. This collateral really helped Iya live up to now.

This February 2, 2008 Iya will have her first operation called Blalock-Taussig (BT) Shunt at Philippine Heart Center. The surgeon who will do it is Dr. Gisel Catalan. This operation could be done earlier but her pulmonary artery is too small then that’s why the Pediatric-Cardiologist suggested we wait and let nature take its course.

The operation itself can be fine but it is still quite high risk because the success of which would depend on how Iya will accept/respond to the Shunt or the tube that will be connected to her Pulmonary Artery.

There are two goals for the BT Shunt Procedure – first is to improve her Oxygen saturation and second is to enlarge her Pulmonary Artery. Right now the Oxygen saturation of Iya is 60-70 percent when the normal oxygen saturation should be 100%! This is the reason why she’s cyanotic or “blue”. The Pulmonary artery should also enlarge coz the next 2 procedures success would depend on her pulmonary artery’s size (This is where the tubes will be connected).

The Shunt is also high pressure and that’s why it should only be there for 6 months.

After 6 months, Iya will need to undergo another procedure called Glenn Procedure. The doctor will replace the Blalock-Taussig shunt with another connection to the pulmonary artery. In this operation, the Blalock-Taussig shunt is removed, and the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery. Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen.

This procedure is quite complex and hospital facilities are of great importance. The family at this point are working together to financially support Iya's procedure. Estimated cost for this operation is about PHP2,000,000.00 to PHP3,000.000.00 (to be confirmed with doctor). Donors are being tapped at this point to find ways to get part of the cost covered.

If the Glenn Procedure shall succeed, we will wait for 4 to 6 years and Iya will go through the Fontan Procedure. The doctors said we will wait until we have the biggest size of veins/artery so the Fontan will not have to be done again as there are previous case that the vein/artery of a patient expands.

For this procedure (Fontan), the cost is also around PHP2,000,000.00 to PHP3,000.000.00 (to be confirmed with our Doctor) .

Jhermie at this point still really doesn’t know how to have all the money that is necessary. Aside from the hope that they’ll get a support from family and if successful, from organizations or corporation who provides assistance in this area, they are also considering selling their house when the time comes.

Jhermie's husband, Dave, is also waiting for his working visa in Australia. My husband currently works in Adelaide and referred Dave to work there. If he can start work this March or April, they’ll be able to also save more for Iya's Glenn procedure this August 2008. She also has several projects outside work and hope it would help increase their savings.

Looking back, Jhermie realized that Iya's feeding problem during her first day of life is the reason for this.

TA is very rare (1 in 100,000 birth) and it happens sporadically (by chance) but there’s also information on the Internet about excessive alcohol consumption in pregnancy which could be one of the risk factors.

When Jhermie read about this, she realized that the condition of her baby now could be her fault as she found out about her pregnancy only after 2 months and according to research, the baby’s heart is developing in the first 6-8 weeks of pregnancy and this is really a critical period (first trimester). During that time, Jhermie have been going out a lot to parties and had a week long celebration of her birthday, and was drinking hard.

Although we kept telling Jhermie that she should not blame herself, she could not help but do. Her heart breaks every time Iya would cry and her lips, hands, and feet would really turn “blue”….

But all Jhermie can do now is to ask for God’s forgiveness and His help for Iya’s condition. She prays hard for a second chance to raise Iya, to give her full life and promise to love and take care of her.

I'm sharing this story because my sister (and her family) matters a lot to me. I'm reaching out so that Iya will get the resources she needs to make her operation a success.