We spent Friday afternoon at the clinic to meet with the cardiology team and the pulmonary team. Iris has been doing really well and I was feeling upbeat about the visit. As I've mentioned in the past, I realize I have not been particularly explicit about all of her diagnoses. I have tried to right that on this page. We had been under the impression that she was perfectly healthy--save for a case of reflux--so this was all a huge surprise to us.
As far as prognoses go, the doctors had prepared us for the worst. The worst being: shortened life span, neurological and cognitive development problems. After being shocked with the worst possible outcomes we have continued to ask what they foresee for Iris.
Their responses are quite positive: from a cardiac perspective, they will monitor her closely for years to come but hope to have her off the medications by age 1. She still benefits from the oxygen's vasodilation [widening of the blood vessels] effect but they do not expect that she will need extra oxygen for the rest of her life. On the pulmonary side, she is breathing much better and her oxygen saturation levels are up. There is added risk during flu season so we are to be extra careful not to let her get sick and if she does to respond immediately. The pulmonologists think that she will grow out of all of her lung problems so we have to just wait and monitor her ease of breathing and oxygen saturation. Yesterday we tried her off the oxygen for a short time and her saturation levels quickly dropped to the low 80s. When she is on a 1/4L of oxygen the saturation levels are in the mid-90s, right where we want them. Finally, we have to wait until October to see the ophthalmologist again. Previously we had been told that the eye structure looks good and that she probably has pretty good vision. They will test her vision when we visit again. She does not have total aniridia so she is able to handle being exposed to some light. She does show sensitivity to indoor light so we try to keep the lights low when it seems to bother her. Outdoors she always has sunglasses on, unless it is dusk.
She has been tested for genetic disorders associated with aniridia and cardiac problems - all of the tests came back negative. This is a good thing, however, it is confusing because aniridia results from a deletion of the PAX6 gene on chromosome 11. The geneticist said that this deletion probably didn't show up on the test because it is a smaller deletion and suggested [at my request] that we get further testing to determine how large the deletion is.
We won't know about cognitive problems for quite some time. The surgery could have caused some brain damage and lack of oxygen could have also caused problems. But she is hitting all her developmental milestones perfectly so we're hopeful. She is one of the most social babies I've ever seen [not that I have much experience] and Evan is very proud of how happy and interactive his extroverted daughter is. "It's so amazing to see her growing and developing," he remarked the other day. "I'm sure all parents say that." Yes, an awful lot do, but we still count ourselves very fortunate to have such a sweet girl.
Because she is looking so well we don't go back to the clinic until November. Now we just focus on growing -- she is now 12 lbs and 25 inches long -- and playing.
As far as prognoses go, the doctors had prepared us for the worst. The worst being: shortened life span, neurological and cognitive development problems. After being shocked with the worst possible outcomes we have continued to ask what they foresee for Iris.
Their responses are quite positive: from a cardiac perspective, they will monitor her closely for years to come but hope to have her off the medications by age 1. She still benefits from the oxygen's vasodilation [widening of the blood vessels] effect but they do not expect that she will need extra oxygen for the rest of her life. On the pulmonary side, she is breathing much better and her oxygen saturation levels are up. There is added risk during flu season so we are to be extra careful not to let her get sick and if she does to respond immediately. The pulmonologists think that she will grow out of all of her lung problems so we have to just wait and monitor her ease of breathing and oxygen saturation. Yesterday we tried her off the oxygen for a short time and her saturation levels quickly dropped to the low 80s. When she is on a 1/4L of oxygen the saturation levels are in the mid-90s, right where we want them. Finally, we have to wait until October to see the ophthalmologist again. Previously we had been told that the eye structure looks good and that she probably has pretty good vision. They will test her vision when we visit again. She does not have total aniridia so she is able to handle being exposed to some light. She does show sensitivity to indoor light so we try to keep the lights low when it seems to bother her. Outdoors she always has sunglasses on, unless it is dusk.
She has been tested for genetic disorders associated with aniridia and cardiac problems - all of the tests came back negative. This is a good thing, however, it is confusing because aniridia results from a deletion of the PAX6 gene on chromosome 11. The geneticist said that this deletion probably didn't show up on the test because it is a smaller deletion and suggested [at my request] that we get further testing to determine how large the deletion is.
We won't know about cognitive problems for quite some time. The surgery could have caused some brain damage and lack of oxygen could have also caused problems. But she is hitting all her developmental milestones perfectly so we're hopeful. She is one of the most social babies I've ever seen [not that I have much experience] and Evan is very proud of how happy and interactive his extroverted daughter is. "It's so amazing to see her growing and developing," he remarked the other day. "I'm sure all parents say that." Yes, an awful lot do, but we still count ourselves very fortunate to have such a sweet girl.
Because she is looking so well we don't go back to the clinic until November. Now we just focus on growing -- she is now 12 lbs and 25 inches long -- and playing.
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