Kartagner Syndrome

Lily has always been known to throw curve balls but she threw not only one today but multiples. We went to see the Pulmonary Specialist due to her bouts of sleep apnea. We got there and went through the norm of height, weight, bp, o2 Sat's, then off to the room we go. The only difference was I didn't see a single face in the office that I recognized or that knew Lily. Which is different seeing our PCP knows us so well along with the staff there, and we all know the H&LI know Lily. Lily took the appointment in stride, she didn't want to give her BP or O2 Sat's like normal, cried when they listened to her chest, refused to open her mouth for the tongue depressor...for those of you who have followed Lily for some time now, you know this is nothing of surprise, just something to chuckle about as she breaks in a new doctor to her stubbornness.

At the clinic today I sat and talked with the doctor as Lee-lee's walked around the room doing her usual "what's this" routine. Thankfully she hasn't masted saying it, she is still in the process of opening everything and holding it up at me, screaming at me if she wants my attention. The greatest part about it is, once you tell her what it is, you say, "Ok Lily put it back." and she will. She is a great listener.

Ok..getting off topic, Focus Jenna, Focus like a laser beam.

I answered sleep questions, about how much she sleeps, how many naps, along with how long have I noticed this breathing problem. Lily sleeps 18 hours a day, that is with a 12 hour stretch at night, and two 3 hour naps during the afternoon. The amount of time she sleeps has always disturbed me but has never seemed to bug anyone else. Over paranoid most call me, but I have always had this nagging feeling she is sleeping to much. The pulmonary specialist said now that Lily is 15 months, she is bordering into too much sleep. But for right now that isn't going to be our main concern until we start with some other things.

1st: We will be doing a sleep study. She will go in over night, get all the do-dads and doma-hickies all hooked up and call it a night party at the sleep clinic..we will get the results shortly after that.

2nd: She is still aspirating, Surprise surprise, I hope you all caught my sarcasm there. I have been saying for months now that she is still doing this. When ever I brought it up I was told a GI specialist was needed and they would refer us to one. Never got the name and number, etc, one thing would lead to another, such as another cold and we would have to put it on the back burner and ride the cold out. By the time she would get better, we would bring it up again, only for her to be sick yet again. Round and round we went...

Speaking of sick...lets talk about this sickening news that weighs heavy on me tonight

Lily will have to undergo some testing in the future, not near future, but sometime within the year because of her colds. With Lily being Dextrocardia, it has always been a bit of a problem but has never caused to much of a ruckus since now, or at least I thought it hadn't. But speaking with the Pulmonary specialist she told me if Lily would have just had Dextrocardia we would have been fine, but with the Situs Inverses she falls under the category of getting sick more often with upper respiratory problems. She said it is very rare disorder called Kartagner Syndrome only 1 out of every 32,000 live births does it occur, and usually only in patients who have Situs Inverses. What does all this mean you may ask. Well, we will have to see what comes of all of this, but what has me down about all of it, is that is causes infertility, the one thing that has had me scared since I was first told about Lily's heart. Besides losing her, this has always been my other fear is that my little girl will never be able to have children, let alone because of her heart, but now if this is confirmed then this will cause more problems. BUT we will not worry about it now, we will cross that bridge when we get there. I will think about it for today, and worry about it when the time comes. No point in worrying over something that hasn't come to pass and may never come to pass.

Back to the Kartagner Syndrome, in order to test for it, she will have to be sedated in order to pull tissue samples, which will then be biopsied. What gets me is that it is an inherited disorder, which means someone in the family has to have it. Which raises the question of who has had it or who has it?

For some strange reason I think it is me. I have always have had chronic sinus infections, headaches, upper respiratory infections, ear infections (never had tubes in the ears) etc. We have always put it down to bad allergies but wouldn't shock me if I was to find out other wise. Defiantly something for me to look into, would explain why I was always sick in my childhood and why I am continuously sick now.

Off topic once again...kind of...

Lily was put back on Prevacid, she has been throwing up a lot lately, and seeing she is aspirating, it is not a good combination. So, back to 2 meds a day instead of the one that we got use to. She might have to have an upper GI done while getting her swallow study, the doctor hasn't fully decided on that yet.

So we have a full schedule as of now. Cardiac, Swallow study, Sleep Study, a possible Upper GI, and sometime in the future more research about Kartagner Syndrome...Like I have always said, when you think you have figured Lily out, and are in a routine, she ALWAYS, throws a curve ball. You are not always on your feet, but in the crouching position ready for it all...I just wasn't ready this time around.

That wraps up this blog, I am going to go take something for this migraine yet again, and head to Starbucks to unwind and process some more!

Thank you all for your continuous prays and constant support.

Love Hugs and Blessings
Jenna