Nashville

Well we did it. We drove 16 hours in 2 days and survived. Not something I want to do every weekend but it wasn't horrible. We got our answers, finally. Amadeus has NEHI. NEHI is the only ILD that is not fatal and does not require a lung transplant. He is on the mild side, since he doesn't require a lot of oxygen. The doctor was pleased to see how we were dealing with the disease. She would like to have a couple of more tests done, but they are not major tests. She would like for us to do the sweat test again for Cystic Fibrosis. If the test comes back elevated like it did before she wants him genetically tested for all the different types of CF. He has already been tested for the 60 most common types, but there is 1600 known types of CF. She would also like a chest x-ray done when he is well. All of his x-rays have been when he is sick, and she wants to know how much damage is really there. According to his CT scans he has a lot more diseased area in his right lung than what is typical for NEHI. She isn't sure why. We talked about research. I told her that I would be happy to submit any of Ami's info for research. She said he is an unusual case but not uncommon. About 1 in 4 NEHI cases have results like his. She told us to continue using the inhaled steroids. She told us to treat his symptoms even if it doesn't go along with typical NEHI treatments. She did not know how much longer he would need the use of oxygen. She said that everyone is different, and it is based off of how well the child is or isn't doing. We also talked about things like daycare/preschool and future children. She said that by 3 or 4 she would be okay with introducing him to a school setting, but be prepared for him to become very ill. She told us to look into the school carefully. To make sure they understood his disease and how to properly manage it. She also said to look into their cleaning practices. Which are all things I had planned on doing. She said that because he has NEHI not to let it keep us from having more children. As many families that have multiple NEHI children also have just one affected child. She said typically it is easier the second time around because you know what to look for and how to deal with it. With all that being said that is still not a risk Josh and I are willing to take. I can't put another child through all of this again. Even though Ami has NEHI he will NOT out grow this disease. The damage to his lungs will always be there. He will always breathe differently. He will get winded quicker. He may always need oxygen. He may develop asthma. This is something that he will have for the rest of his life, and will be a constant struggle for him. Because so little is known about NEHI no one knows for sure how this will affect him later in life. He goes to see his pulminologist and pediatrician next Tuesday the 12th. That is when we will know when they will be doing the other tests. Until then more waiting.