Lucas has heterotaxy syndrome.
Heterotaxy syndrome is an extremely rare birth
defect that involves the heart and other organs. The beginning of the
word (hetero-) means “different” and the end (–taxy) means
“arrangement.” There are different forms of heterotaxy. All
usually involve heart defects, of varying type and severity. In
heterotaxy syndrome, paired organs, such as the lungs or kidneys, are
often mirror images of one another instead of having the unique
characteristics of right and left that are normally present.
More easily stated, heterotaxy is
comprised of 4 defining characteristics:
- Deranged abdominal organ asymmetry: stomach, spleen and liver are commonly misplaced.
- Major heart defects: almost always including the absence or poor formation of major vessels.
- Rotation errors: such as the heart rotated so the side normally facing the chest is facing the back.
- Organ malformations: commonly the spleen is absent or deformed.
Sadly, most heterotaxy babies don't live to see their first birthday. There are several reasons why:
- Many parents elect to abort the pregnancy.
- They require several open heart surgeries. With a heart the size of a walnut, the risk of death during open heart surgeries is very real.
- Infections from surgery are extremely serious because at least half of babies with heterotaxy don't have a spleen, and an infant without a spleen has an extremely weak immune system- unable to fight off most infections. Lucas doesn't have a spleen.
- Sometimes babies with heterotaxy simply die in their sleep.
- Of the few babies that live to be year old, even fewer survive to be adults.
Here is a story of a baby with
heterotaxy who survived for 22 hours after birth:
Here is a story of a baby with
heterotaxy who is defying the odds, and had made it to 3 years old:
I know this all sounds extremely grim. But,
I'm confident that Lucas will beat the odds. He has every possible
advantage a baby with heterotaxy can have:
- We chose NOT to terminate him, even though this option is available even into the third trimester. A lot of heterotaxy babies never get a chance to survive.
- Heterotaxy was detected very early, giving us a giant head start of planning care and interventions.
- We chose USC Medical School as the basis for the prenatal care of Lucas. USC is the epicenter of heterotaxy care west of the rockies.
- We have chosen Los Angeles Children's Hospital as the site for Lucas's surgeries, care, and recovery. Los Angeles Children's Hospital provides outstanding care, and leads the country in many statistical categories for infant crital care.
- He will never be alone. Either Melissa or I will be with him 24 hours a day. I will be in his room for at least 12 hours a day, and sleep in the ICU on a chair next to him for 6-8 weeks. I will watch every blood test, monitor every intubation, and count his heartbeats.
- Lucas will have his own highly skilled ICU RN 24 hours a day.
- He is a Paton. All Paton. There is no such thing as a half Paton. Patons are beyond stubborn, to the point of being obstinate. We never ever give up. Lucas will hold up his end of the fight.
- The muscle in Lucas's heart mucle is healthy and well equipped to function.
- The Smiths are a tightly knit family and are known to take care of each other. Lucas's Smith grandma, grandpa, uncles, aunts, great aunts and uncles are all geared up and ready to be at LA Children's Hospital any day for as many days necessary.
I can't imagine anything else that
could shift the odds into our favor any more. We are very confident Lucas will get
through this.
We are ready for the fight.
