Booger Suckers and Damaged Goods

Today was the day of results. We headed down to the medical center, early this morning, in horrible weather. It was rainy, cold and a huge mess. Even the HOV lane was a mess. We made into our first appointment, immunology, at 8:55 am. We checked in for our 9:00 appointment and got Kate's vitals checked. She loves that she has graduated from the baby scale to the scale she can stand on. At her last GI appointment her stats showed she had lost about half a pound since September of last year. That was confirmed again today. She is definitely slacking in the weight department at 23 1/2 to 24 pounds. To not have gained any in 6 months, especially at this age, is less than ideal. I think she is in the 14th percentile for weight now. This is despite her finally taking to cow's milk AND having Pediasure in the mornings. Kate impressed the nurse, asking for "pressure" and "temperature" after getting her height and weight. She's becoming all to accustom to this stuff.

We made our way back to our room, where we waited for 45 minutes. Finally the fellow, that is currently training with Dr. Ha, came in. He went over all the lab results from the draw on her procedure day. Long story short, they were all normal. Her IGG was 518, IGA was 47.1 and IGM was 97.4. Those are all in normal range. He basically said she was normal from an immunological stance and that he would be back in with Dr. Ha soon. We waited another 30 minutes. At this point it was 10:30 and we had tentatively set an appointment with pulmonology at 10:00. Dr. Hi was seeing us today to save us an extra trip down, since he normally only sees patients on Mondays. We were supposed to page him when we were ready. I left the room to see if they could page him and ask if we could see him in the afternoon, as we had an 11 o'clock lab appointment for a sweat test to rule out Cystic Fibrosis. I knew this couldn't be bumped because 11 is their last appointment for the day and I really didn't want to return a different day for one little test.

As I was talking to the nurse, I saw Dr. Ha (pumonologist) walking towards our room. He came on in and talked with us. He started out by saying that there wasn't a GI issue, there wasn't an immunology issue and that he really felt like she was a child who "wheezes with viruses". I seriously had to restrain myself from A) laughing B) hitting him or C) walking out. It seriously felt like ground hog's day. He finished with the whole "wheezes with viruses" spiel and then referenced the biopsies or "brushing" they took of her lungs. He commented that he was VERY surprised that her cells were ALL damaged. He continued that this can fit in with "wheezes with viruses" or WWV (yes, I made that up.....or more appropriately IDHAFCWYCISATT....I don't have a freaking clue why your child is sick all the time) because some of those kids get so many viruses it can damage the cells of their lungs.

While we were at the appointment, I felt like I had a great grasp on all of the knowledge we did get. Trying to explain to Daddy-O though, I learned that I didn't have as many answers as I needed and Dr. Hi can be VERY confusing at times. I'll try my best. If anyone KNOWS what I'm trying to talk about, feel free to correct me in the comments. I asked for more information about the brushing and he explained that the cells that the cilia sit on can be damaged by lung infections, viral and bacterial, and the body normally repairs them. They can also be damaged by genetics (Primary Ciliary Dyskinesia or PCD). I asked what was so surprising about Kate's results and he explained that usually they see some damaged cells, but not ALL damaged cells. Now, I am not sure what that would indicate, but my gut says that means she has had a LOT of frequent chest infections, more than the normal WWV type of kid or there is something else going on. I didn't get a name for the actual damaged cells, a final lab report or any indication as to whether or not the cilia are truly damaged, and if so what part of them, or if they are missing all together. I will call tomorrow and try to get more info so that I can email our pediatrician. This will give him a chance to read up on it before we see him next week.

Basically since the cells are damaged and will continue to be until she can be well for a long period of time or find a new set of genes (no, that's not really an option), the treatment is the same. They drew labs for the gene test today, but it has a 30% accuracy. If it comes back saying it's genetic, then it is and we know we are in for the long haul. If it is negative, she still could have PCD, but we will have to wait until she is healthy for a while and redo the brushing. If we see that after being well they are still damaged, we know that it's permanent. So until she gets well for a while, we will be treating as PCD (unless her pediatrician tells us different). Confused yet? I am!

So what's the treatment..... pretty much nothing! There are a few things we can do and for those of you we know personally, YOU can help us with some of these!!! We need to do the following according to Dr. Hi's plan:

- Keep her away from anyone who is sick.

- Avoid Bible class, public playgrounds, childcare until AT LEAST May, if she is well some of the time before then.

- Absolutely no sharing drinks, food, utensils or anything else that enters her mouth.

- Save kisses for the top of her head only. No more chubby cheek kisses or open mouth, slobbery kisses. :(

- Wash hands before interacting with Kate.

- Remind her to keep things (ie. toys, colors, etc.) out of her mouth.

The fact that Leah is in school is actually beneficial. This gives Kate an opportunity to pick up some viruses, that Leah carries home, in a very light, watered down form. If Leah were sick with them, then Kate would get them full force, but even without catching these viruses, Leah is bringing them home. Her immune system is strong enough to fight them off, but it gives Kate's a chance to experience them. This is VERY different than exposing Kate to someone who is sick. Exposing her to a sick person directly, gives her the full blown virus. The little bit Leah drags in is just enough to give Kate's immune system a taste of it. Unfortunately that little bit is often enough to sink Kate. The good part is, it will save her from having NO exposure built up once we do expose her to the real world. It is helping her system learn how to take on the little things.

In addition to this, we are to continue treating symptoms, which includes oral steroids with flare ups. When I asked about PCD, in the genetic sense, Dr. Hi said, it was unlikely, that most kids have bad ear infections, have to have tubes put in and have sinus infections a lot. Three for three on that list. Then when I told him I had read on some PCD forums that some people recommend doing percussive therapy, similar to cystic fibrosis treatment, he said that most of the time PCD effects the sinuses and ears, but sometimes the lungs. Yep, got it. He continued that percussive therapy would only be useful if she was getting lung damage. I guess that all of her lung cells being damaged doesn't count? I'm not trying to be a sarcastic, I really just don't know. He said that we would do a CT scan of her lungs this spring to check the damage, but he really wanted her "well" for it. I did talk him into lowering her daily steroid since it doesn't seem to be helping at all anyway.

By the time we wrapped up with him, it was 11:15 and I was frightened that the lab wouldn't take us. I left my cell number at the nurse's station and told them to call me and let me know if Dr. Ha wanted to see us or not. We rushed down to the lab and got in pretty quickly. The sweat test basically involved them cleaning Kate's legs, applying some chemicals, I think, and then shocking her skin with electrodes for 5 minutes. She was less than thrilled and kept kicking the lab tech. Once the 5 minutes was up, they remove the electrodes and place a piece of gauze over the sight. They place plastic wrap over that to create a sterile pocket and then tape it all up. The poor girl had tape from her ankles to her knees on both legs. You then wait 30 minutes for the gauze to collect sweat. We ran to get a quick lunch while we waited. Just as we were wrapping up, they tracked us down and said our time was up. We went back and had it all removed. They drew the rest of the blood work while we were there too.

Then it was back upstairs, since I hadn't received a phone call from Dr. Ha's office yet. We told them who we were and headed back to the same room. Forty-five more minutes passed before I went out and asked if she REALLY needed to see us. After all, I had already received copies of all the labs we were there to get results for. Finally she came in and went through each and every lab Kate's had drawn, what they mean and what normal ranges are. I appreciate her thoroughness, but I was SO ready to be home and poor Kate was done! She didn't really have anything different to say from the fellow. We will go back in a year, unless the pedi thinks we need to be seen sooner. We will probably do allergy testing at 3 years old. Other than that, we are cleared from their office!

So now we've dropped 2 docs, 1 med and lowered one. That's some progress! I was hoping for giant steps and we are getting baby steps, but at least we are moving. While I don't know that all of our questions are answered, we have all the pieces to take to the pediatrician, Dr. McP, next Thursday. Hopefully he will be able to piece them together and get us on a good plan.

Some of the other things I've read about regarding PCD are that we need to treat aggressively at first potential of infection (cough, sniffle, etc). Many groups also recommend doing quarterly swabs to check for harboring bacteria. PCD patients are very prone to super bugs (big bad viruses) that can lie undetected in the lungs and be a real problem to treat. To be honest, I'm not even sure what type of doctor PCD care would fall under. I've also read that percussive therapy and suctioning can be helpful. It is interesting how much these little "hairs" effect too. Fifty percent of PCD patients have all of their organs reversed, like a mirror image. Apparently cilia control the movement and placement of the organs in the embryonic state. They also assist the egg in moving through the fallopian tubes. They are also the tail on sperm. Because of this, there is a high rate of infertility associated with PCD, especially with men.

After seeing Kate's nose today, the doctor did recommend that I get a good aspirator. We've used saline and a bulb syringe in the past, as well as Q-Tips. We refer to Kate's boogers as "bungee boogers". Those things are seriously strong. I can catch one on a Q-Tip, pull and have it way out, only for it to snap back in to the abyss. Too much info, I know. I went to find a better aspirator and spoiled myself. There was one that requires you to provide the suction via a straw. I went all out and got the battery operated one. I'm not so keen on sucking on a straw to remove the bungee boogers from my daughter's nose. Call me crazy! We used it tonight, as she is horribly gunky, and her boogies are almost to thick for it. If any of you have used one of these and have advice, I'd love to hear it. I do LOVE the concept, but am not 100% sure it's going to work.

At this point in time, a part of me hopes that the genetic panel comes back showing that it is PCD. By no mean do I want there to be something wrong, but this way we have a clear answer, diagnosis and know what to deal with. The other side of me hopes that it is negative so that there is a chance it will go away. More than anything, I hope and pray that God protects her, continues to keep her as healthy as possible and that she will thrive, inside and out.