Seattle--again...

After all the ups and downs there is still things that have to be done and that includes the next big test she has to have. Every 6 months to a year Alana has to have an ECHO and EKG done to look at her heart. This is for a couple of reasons but the most important one is to look for pulmonary hypertension. When children, or anyone for that matter, is on O2 it is something that you need to keep an eye on and she in no different. I really like her heart doctor. He’s told me on more than one occasion I am Alana’s advocate, that if we are not making headway with someone to move on or make more noise until we do make some headway.

It’s nice when I can report great news here and so after having her ECHO and EKG the doctor told me the results right there, her heart looks great! Whooo HOOO! There’s no evidence of PH and everything else looks good. She’s still has her murmur and we are told to keep a close eye on the episodes of tachy and bradycardia that she’s having and if things get any worse or we notice fainting spells or other to bring her back right away. Otherwise we are good for 6-12 months. It’s so nice to have an appointment go smoothly and to leave with no thoughts of bad news to share. This was just last month so guess what?! We are here now, the present; crazy huh?

I’ve now been kicked back in my chair for awhile trying to think of where to start…she’s still having bad days, a lot of them in fact. To summarize this month in regards to her and her illness it’s been slow going. She’s been having fevers closer together and had a good set this weekend. As well as that she’s been really struggling with PT to the point that she was over 2 ltrs of O2 and still struggling to breath. I sit at poolside and watch her try with all her might to swim with help from a float or other and she cannot do it. Her feet and her hands no longer work in coordination with her mind. It makes me cry at night.

She went into McDr (yes, we are now calling her McDr, Dr. McArthur, according to Alana) today to see if she has developed pneumonia again because she’s coughing way more then she was even 5 days ago, she’s running fevers, she’s pale, and she’s needing O2 at higher flow rates. The good news is, that just from looking at the x-ray and not having the radiologist report yet, it looks OK. There are still infiltrates in her left lobe but they are always shifting and there. Tomorrow we should have the full report and will make the decision to start antibiotics.

We did talk about her increased lack of coordination, the fact that it’s been months now since the cough started again and then the biggie that she’s not gaining weight again. And in again I mean in the past 2 years. She still has not gained back the weight that she lost in October and December and in the words of the dietitian, her growth is “stagnant” all the way around. We’ve heard mention, from some of her other doctors that a NG or G-tube might be in her future but I’ve been resistant to think about it. I don’t know how long that can keep happening though. I was looking through photos of her way back in Craig when she was 3 and she has on this jacket that my mom made, well this weekend she was wearing that same jacket and it was still a bit big on her. *sigh* McDr said today that we might have to talk about this soon, like in a few weeks. If we do an NG tube and she gains weight then she will have to have a permanent g-tube placed to help her in the long run. Not something I want to think about right now.

McDr also mentions “progressive” again. I did it, I couldn’t help it; I started to cry. Please someone explain to me why I can’t seem to hear that word and not get upset?! I get it though, her increased loss of coordination, her increased O2, her increase in autonomic issues, her increased motility issues, the fatigue, the fevers being closer together, they all scream progression. Don’t cry just breath, right?

Another thing that I haven’t mentioned is that next week we leave for Seattle Childrens again. If you recall the last update I mentioned this and it’s now that time. Her appointments start on the 10th and we have no idea how long they will last. We are meeting with this guru on mitochondrial and other metabolic diseases and she will be having another surgery, this time a thigh muscle biopsy. No word on what day that is going to happen as we are meeting with the surgeon and the GA clinic on Thursday to see what they think about her going through another surgery. It will be while we are down there (don’t get me started on Seattle Childrens again!) is all we’ve been told.

So as usual we have a lot going on around here. Its summer break and the other 2 chicky-poos are home and I love having them around to myself during the days and taking them places with me. They are all giggling upstairs right now and its music to my ears! They are supposed to be folding their laundry but I think I will let it slide that they are not and in its place they are playing with each other.

Now that we are in the now, the present, the moment I will be doing the updates as they happen. The laptop is coming with me to Seattle so that will make things easy to keep current, also, here’s another link that I will be using while we are having “issues” or in-patient stays:

http://www.caringbridge.org/visit/alanastephens

On that page you can sign up for automatic updates whenever I post something. I will still always be on here, but it’s nice to have automatic updates when we are going through something big with her.

Our Christmas angel...

So yep, she had pneumonia again for sure. Her CRP was elevated, she had an infiltrate in her right lung and she was miserable to boot. Needless to say we did not get to do the EGE testing that time; instead we went on home with ‘scrips for steroids, antibiotics, and orders to check back in with Dr. McArthur the next day or sooner if she got worse overnight. We did end up taking her in the next day to see the doctor and as expected there was the “do we admit her or not” issue to be dealt with.

I know I said that I’d just say go ahead and let’s admit but it just isn’t that easy. There is a fine line that I feel I can walk with Alana in regards to when to admit. At that point I felt that if she could just get some fluids in that she’d feel better, ergo, be able to stay home longer. For the time being the doc agreed and what we settled on was doing an IV in the office. It wasn’t going to be a magic fix by any means but it might allow her the strength that she needs to make it through this. While they are getting things ready Dr. Tapple, whom she’s seen many times when Dr. McArthur isn’t around and admitted Alana for her October stint, sees that we are there again. He inquires as to why, is told, asks if she’s going to be admitted again and shakes his head. I think that the Alana frustration is rubbing off on him as well…

Once the line is placed she promptly falls sound asleep and we just wait for the bag to run through her. It’s amazing how much it can hurt to watch her have a line placed even though you know that she desperately needs it there. I’ve seen her have so many placed I couldn’t tell you a number, I can tell you it still hurts me; not as much as it hurts her, but in a very different way. Over time the line seems to be working and she’s sleeping a bit better and has some color to her cheeks. This is all great news at this point as it means that there is hope that we can go home!! It’s right around Christmas time and she really wants to be the angel in the play at school and I really want her to be that angel.

It does end up working out and she is allowed to go home on tight orders. If she gets worse, doesn’t eat or drink in ‘x’ amount of time, or has an even harder time breathing then we have to return, not only return but return with the knowledge that she will be admitted straight away. I can handle that and I do think that she is OK enough to be at home for the moment.

The play rolls around and she’s WONDERFUL! Thank goodness for such an amazing teacher (Mrs. C) who just takes Alana in stride; she knows when to push her, how much to push her, and when to just offer her a hug without any pushing involved! I am so very blessed with some of the people that are in Alana’s life. She does her lines wonderfully and is in great spirits for the duration of the play. Lucky for us it’s not very long and she’s able to make it through without coughing to bad.
We go back the next day for another check-in and there’s been no real change in Alana. We are given the go ahead to head down to Cooper Landing to spend Christmas with everyone again, conditional; she gets no worse, the hospital in Soldotna is within a short driving distance, and nothing new pops up. This is all doable in my book so off we go to grandma’s house.

Christmas was as well as could be expected. She did have problems with eating and drinking like I’d anticipated but not to the point of dehydration. She did lose more weight, a total of 12% of her body when it was all said and done, but she enjoyed Christmas at home, with her family…not in the hospital like the previous year. It took almost 3 and a half weeks for the pneumonia to finally clear up and things never went back to the ‘old’ way again. Her baseline had officially changed, not for the better.

There are things that I don’t remember to mention in here until I am at a point like I am now. These are the little things that no one can put their finger on but are like pink elephants starting to fill up a room. Alana has had new things going on that I’ve been, for lack of a better word, ignoring. I know that sounds ignorant, foolhardy, neglectful, you name it I’ve thought it. I wasn’t ignoring them because I thought that they were not worthy to be followed, I was ignoring them because I was worried that they needed to be followed. Of course this didn’t last long at all. As Goldfinger so put it, “the first time is happenstance, the second time is coincidence, and the third time is enemy action”. None of this was really going un-noticed by me; I just didn’t know what to make of it was the problem. Here’s a partial list of what I was noticing:

Extreme redness on her face
Hot ears
Hot and red hands and soles of her feet
Raw, painful patches on her tongue
Increase work in breathing
Decrease in her SATs even with the increase in work
A lot of rashes
Sleeping 2-3 hours a day
Increase in her heart rate as well as dips in her heart rate that last longer than they should

I didn’t know what to make of those things or even what to do about them so how the heck was I going to take care of them!? I did take heart that I was not the only one noticing them, Dr. McArthur noticed them as well and agreed that this indeed seem like a new baseline even though it was lower than her crappy baseline to begin with!

Things pretty much continue in that same manner for the next few months. Alana sleeps even more than usual, she is coughing more, she’s running fevers tighter together, not gaining back the weight that she lost in October and December, having frequent rashes and tongue issues, etc., etc. Amongst all those concerns I notice that she’s just really struggling to do daily activities that she used to be able to do, swimming for instance. Granted she still had some issues with them but she still was able to pull them off. Now she can’t even do that and it frightens me to see her literally and figuratively, sink. We are now in February of 2009 and there are changes on the horizon that shake up a lot of things.

The new way of things...

By now I feel like I should be used to this; the unknown of all of it and what it will bring this round. I am not though, nothing seems to make it easier or better or even okay. The same labs are run, the same doctors are contacted, and the same things happen. She holds onto the fever for 11 days and as soon as the fever starts getting better the cough picks up and holds on tight.

There are a few new things that have been creeping up on Alana lately though and here’s something that I feel holds true about her when it comes to new “things”: 1st time a fluke, 2nd time happenstance, 3rd time our enemy. She is now doing something that fits into that last category…she’s having periods of bradycardia (abnormally slow heart rate). Her sleeping heart rate is around 80-120 bpm and now she’s alarming because she’s getting down to 48-50 bpm and not bouncing back up unless she’s jostled. It’s enough of an issue that when it happened in the past the nurses came in and just hooked her up to the heart monitor on their own accord and watched her. When they do it again this time at 2 a.m. because she’s been alarming so much I realize that it’s time to ask the cardiologist what he thinks about it.

When the next morning rolls around and Dr. McArthur is making her rounds she mentions the heart issue and says that she is going to ask Dr. B (cardiologist) about it today and she will call and let us know what he says. Something isn’t right in the state of Denmark and it just cannot be ignored. Calls get made and guess what! Not a big deal at this time. As long as she DOES bounce back then it’s nothing to be worried about. This doesn’t sit well with me on so many levels but again, I am not a doctor right.

As Alana starts to heal from the inflammations attack on her but we are still having problems with eating and drinking. Dr. McArthur at this point is thinking that she might have to start TPN (nutrition through an IV which can cause its own mess of problems) in order to stop her from losing any more weight and not getting any nutrients and vitamins. She’s got 1 more day to stop losing weight before it has to be done. Wouldn’t you know it the next day rolls around and she’s not lost any more weight!! She is holding steady and there is hope that she is going to pull through it without needing any more support than shes already been given! Whew!

I’d like to say that we left the hospital and she was much, much better. If you’ve been reading then you may have picked up on the fact that this year has been like no other with Alana, she’s sicker more often, she’s having issues with gaining and holding onto weight, she’s getting dehydrated within a day of major illnesses, and she’s just miserable more often than not. When we leave she is better, just not better than she was a year ago. She is still so tired and fighting us on eating and drinking. She will eat 1 small meal and not want anything else for the rest of the day. She’s now starting to flux between diarrhea and constipation and that is bringing on stomach pains. There seems to be no end in sight as to what is going to happen with her the next day or even the next hour. Her O2 needs are creeping up and she is struggling with things that she could do almost with ease before. Things like swimming; she used to love to swim and was very good at it for her age. She could do stroke, stroke, breathe and she could dive off the edge of the pool and swim up to the surface by herself no problem. Now she can’t do either of those things even with help. I cannot figure out what is going on and why she’s having such a hard time with everything, is it her lungs? Is it inflammation we cannot see or find? Is she having another issue that we are not aware of? All these things are not things that I want to add to her unknown "in basket"!

Things go by really fast because it’s the holiday season. After Thanksgiving I start to gear up for Christmas and Alana is very excited about something, she’s the Christmas angel in the school play-again! Last year she was slated for the role but where was she last Christmas, oh that’s right…she was in the hospital again. :’( This year will be different though because according to the doctors this too will pass and she will be okay by the time the play comes around. I won’t mention that I’ve taken her into Dr. McArthur almost every week ‘cause her cough is so bad that she cannot make a single sentence without pausing for air and having a coughing fit. I won’t mention that Alana herself has asked her doctor how she’s suppose to talk for her part in the play without coughing. Nothing is making her cough better besides increasing her O2 and that’s a hindrance in itself.

A week or so before the play things start to change for Alana again. We have a big appointment coming up with Dr. Demain to redo her EGE testing and we are all looking forward/terrified of it! Last time we went from 3 foods up to 9 so it’s anyone’s guess how this is going to go. Her appointment is on a Monday morning but by Saturday night it’s not looking good-Alana has a fever of 102.8 and it’s climbing. By Sunday we are hanging steady at 103.2 and pushing Tylenol and Motrin to keep it in check before making her an appointment to go see Dr. McArthur on Monday. By Monday morning though things start to improve and she’s down to 100.4 and looking a little better. Not much mind you but enough that I take her to see Dr. Demain first (it’s a 6 month wait for these appointments and yes, been there done that with her fevers) and figure I will take her into the ped. after her visit with Dr. Demain. Well that goes over like a charm!

She’s lost more weight and after he listens to her lungs during the first part of the exam it all goes south. She has pneumonia. Go get a chest x-ray right away and walk the films back up with you to the office. Stop and get labs on your way as well so we can check her CRP, SED rate, WBC, and start cultures. What’s the motto? Don’t cry, just breathe.

Here comes round 2...

So where’d we leave off? Alana just did her first hospital stay and had her first surgery. We are at almost at 2 years of life for her. She’s such a trooper though. Through all this she’s still smiling, playing, and bringing joy to our family. She’s keeping not only Larry and I busy but her sister’s as well. They think she’s the doll-child and always want to hold her and play with her. Noelani and Shaylin are sometimes left out in this equations that is Alana. They are so good and understanding that it’s easy to forget that they are gong through this too. That they are seeing what she’s having done. Seeing the missed trips to the park because Alana isn’t well enough or the dinner out because she’s coughing so badly. The girls have so much compassion and strength in them that they make Larry and I look weak.

After Alana has healed from her surgery we hope and pray that this is going to be the thing that makes it alright. Having the tubes in will help with the ear infections and having her adenoids out will help with the coughing and swelling she’s having around her nose. It’s been almost 2 years now and it’s about time that she had a break from feeling cruddy you’d think. We did dream, and we did hope, and we did pray. The ear infections were cut in half! She was still having them and she still does but they are nothing like how they were. It was so nice to not have her on antibiotics for ear infections finally. And yes, on that note, her cough did not go away. We were still so very happy that at least one thing seemed to be moved outta the picture that maybe with time her cough would go away. It just needed more time to adjust to the new structure of her face right? So yea, the cough goes on and we go on and the world keeps moving around. She’s still in the doctors a ridiculous amount of time and we are still debating the use of antibiotics to try and clear what is a sinus infection? Cold? Flu ? Bad luck?

By July of 2006 she’s been sick again for months. Coughing, fevers, dehydration. Her CRP level keeps going up and they are not sure what to do about her. She’s now about 30 months old and has spent so much time sick. It isn’t long and she’s admitted yet again. Same diagnosis. Pneumonia. I remember being so upset that I could barley talk one night. Larry and I are fighting because things are not going well. Who do you take the most out of when things are not going well? Yea, your spouse or other significant person in your life. I was so very angry with the doctor at this point that I couldn’t see straight. This would be the last time this pediatrician was involved in Alana’s care. We were told that okay, now we can see that maybe this might not be normal even for a sicker kid who might just be unlucky and “catch” everything. We now say no, we want to see Dr. Demain now. This is it there is something wrong here and it’s not at all normal.

Dr. Demain comes in and sits beside her bed and tells us that now we are moving into a new area. It’s not just little tests anymore and the things they are looking at are dig deal immune problems. Okay we tell him. Better to know and deal with it than to not know what to expect at all. He then gets the ball rolling by giving her an adult dose of the Pneumococcal vaccination in her thigh. He tells us that the reasoning for this is that if her body does what it’s supposed to do then we will see redness and inflammation around the injection site. That means that her body is reacting to the shot/dose and there’s a sign that her body is indeed fighting foreign things.

So what has proven to be a consistent in Alanas life happened. It came back as a normal reaction. She did have a boat load of labs done at the same time and those came back normal for the most part with only a few things slightly eyebrow raising but nothing worrisome. T&B cell panel all within normal range, neutrophil oxidative burst: normal, 23 serotype S. Pneumoniae IgG: detectable but low, etc. etc.

This time they feel it would be beneficial for her to see the Infectious Disease doctor as well. He was a very nice man and I’ll give it to him that he tried. He ran a few tests and asked us a lot of questions about her and her past. He was very kind and told us that he would be grasping at straws to fit her into anything that he’d be able to help with. So again, we do our time and she improves. We try to remember that it’s not over this time and we are not taking any more of this “She’s just sicker than other kids” crap. We are now on the search for a new pediatrician on top of all the other things that we are trying to sort through.

Time goes on and she’s having good days and bad days. We are still seeing Dr. P. the ENT, and he’s trying to help us with her and come up with things that may help. She’s still not sleeping through the night and she snores on top of that. Given the size that her adenoids were we start talking about maybe taking her tonsils out. There is a relation to the size of one’s adenoids and their tonsils it seems so hey, maybe this route will at least work to help with the sleeping issue. If she could sleep through the night then maybe she will feel better and be able to stay a bit healthier in the long run.

October of 2006 she has just turned 3 years old! She’s a bright little bugger & is so used to seeing different doctors that at this point she’s calling them her “friends”. There were so many little things in the in-between that I’ve failed to mention. At 9 months of age she stopped growing. Just stopped growing and we were not sure why. She's not added failure to thrive to her list of "things" going on with her. Her bowel problems had remained consistent so there wasn’t any flair up in that area. She was just being how she’d been in the past. This went on until she was 18 months old (right before she went into the hospital). During that time we saw an Endocrinologist to see if there was anything in that realm that would account for what was going on with her. She, the Endo., ran tests for a few things like Turners syndrome & other genetic disorders, bone scan to check just that, her bone growth, and then a few other random things. Through all this Alana is just hanging tough. She’s sick for months at a time then better for a few weeks or even a month. I think she went 3 months without a cough or fever one time!! Through all this I think there was two different trains of though not only with the doctors but with our family.
They included:

1. She will outgrow this. You hear about kids all the time who have strange things going on with them and then one day *poof* they are better. No reason for them to be sick is ever found but keep the faith and in time she will show you that it’s going to go away.

2. That this isn’t a fluke thing. That this is the way that she is and wishing and hoping it away isn’t going to make it go away. Sitting back and just waiting for her to get sick again and then dealing with it isn’t an option.

3. And my personal favorite that we still hear off and on to this day "Well, she doesn't look sick to me."

As you can guess there were a lot of emotions running around by now. Every one of them was spoken with love and caring with no harm intended. It’s hard though when you are the one running to and fro for appointments. You are the one who’s always hearing that not only can they not fix it but they can’t even tell you what the bloody hell “it” is. No matter how strong your spirit and resolve are those things get hard and you can feel so lonely. Not lonely like alone in the wilderness but alone like you are screaming and people are just walking by, they can see you but it’s as if you are mute. That what you are saying isn’t important enough for them to take notice until it’s too late.

Somewhere along the way...

Dr. Demain felt that Alana was having sinus issues. Yes, she was a baby and sinus cavities are not formed and yes it’s unlikely that a sinus infection started in her when she was 3 months old but she had signs that lead him to think that. She had developed saddle bags across the bridge of her nose. She had dark circles under her eyes. Labs were done and she was having elevated white counts and CRP levels (C-reactive protein is a measure of inflammation on the body). His theory was that the cough was being caused by drainage dripping down the back of her throat and causing irritation. If we could clear the infection then she should be fine. This was going to be done by starting her on antibiotics above the normal amoxicillin (which we learn she’s allergic to) or z-packs. She had a series of x-rays done to see if the bitty sinuses she did have were compacted. I need to back up though; one of the things that I’ve failed to mention is that by 6 months of age Alana has had so many ear infections that I’ve truly lost count. That tied into Dr. Demains thoughts as well. If she was so blocked up then the ear infections could be a direct relation to her whole head issue. So onward and upward again. He starts her on stronger antibiotics, a 6 week course of Clindamycin. After that we will redo her sinus x-ray and hope that the coughing will stop.

Okay, alright, no worries…the coughing hasn’t stopped. I think she’s even managed to get another ear infection on top of all this. She’s continuing to have multiple things going on here. She’s falling further and further off the growth charts and she’s having rashes on her truck and legs. Her feet have started to peel so bad that she cannot walk; the skin is cracked and bloody. Her teeth have started to come in and they don’t look right. They are tan looking and pitted. We take her to the dentist and they ask if I used a lot of antibiotics while pregnant (no) or if she was a preemie (no, remember the “overcooked” comment). Again, now what the duck chuck is going on. We were seeing either Dr. Demain or her primary pediatrician every week. From her primary we were getting “Kids are sick all the time” or “We see children like this all the time in the office and they outgrow it” finally what became one of my favorites, “If you hear hoofs in the distance you think horse’s not zebra’s”.

All the while we are trying different antibiotics and different approaches. We’ve moved onto drugs like Ceftin, Biaxin, Sulfatrim, Predinsolone, Prevacid, Nasonex, and Albuterol. In a time period from January 2005-January 2006 she was on 17 different or repeat courses. Now tell me, where in the bloody hell was I?? Why didn’t I take more notice that my baby, my 16 monthish daughter, was on more drugs than most geriatric patients?? Yes I understand that again, nothing I can do, can’t go back, shouldn’t beat myself up over things that I didn’t know. The fact still remains that I trusted the doctors and they didn’t come through like they should have. My bad.

Now after 6 months of straight antibiotics things actually get worse. Alana was running a fever for a few days, nothing that unusual, but this time she was different. May of 2005 now and she seemed to be having a hard time breathing. We took her back to the primary and for the first time there is something very clear going on. She has gotten her first pneumonia. We were admitted straight from the primary’s office and she was given more antibiotics, more steroids, and albuterol. She tested negative for RSV, and other bacterial infections. She did however test positive that she had already HAD Epstein-Barr virus. Not that she had it then, but that she’d already had it and gotten rid of it. Now at the time the doctors chalked it up to a mistake with the lab. There was no way that an 18 month old had already contracted and gotten over EBV. Another thing that they take note of with Alana is that she’s again got an elevated CRP level. Normal range is below 12 (normally) and her’s is 30.

We do our time in the hospital and now both Larry and I are asking more questions. More detailed ones. How in the world could she have gotten pneumonia when she’s been on antibiotics for 6 months? How and why is this going on? We leave the hospital and are home for 2 days. She’s running a really high fever again and we rush her back to the doctors. She’s now got “otitis media with perforation”. I don’t know who cried more that day, me or her.

We spend the next months battling infections. Her skin, her ears, her body. We try more drugs. More time. More everything that we are told. The infection in her lungs doesn’t really go away now. She’s always coughing and she’s breathing faster with each little ear infection. She’s constantly miserable and we can seem to do nothing that will help her.

By January of 2006 we go and see an ENT on our own. He looks at her history, she’s at 30 or so ear infections and there’s been some mention that maybe her adenoids are large and causing blockage. That if they are removed, then possibly her sinus cavities can drain and stop the backing up of her face. The ENT we see is great. He feels that PE tubes would have benefited her awhile ago. He also believes that he can look at her adenoids while he’s in doing her ears and if they seem enlarged then he will remove them. We agree and in the middle of January she undergoes her first surgery.

I cried. I admit it. I was a wreck when they took her. She didn’t care. She was in la-la land with the help of some versed. I think when you are waiting for them to come and let you know that everything is A-OK time stands still. It’s so strange. That surgery was the first procedure that she truly had and it was the one I cried the most at. It had been such a long road and when they even hinted that she might be better after this. No more cough, no more ear infections. No more antibiotics. There was hope that this would end.

She pulled through like a champ. They adenoids did need to come out and they were large. Dr. P put the tubes in place and came out and told us how wonderful she had done. We waited to see her. And we waited. We saw kids who went in after her come out and go home and yet we waited. Finally after an hour or more they came and got us. It seemed that Alana didn’t want to wake up. She was quite fine in la-la land and was not to be disturbed. Well this wasn’t good enough for a nurse there. She got the bright idea to wake her up. Mentioning something like, “Oh sometimes they just need a little help waking up…”. Famous last words! Needless to say after Alana screaming her little head off for 45 minutes they decided that she was well enough to go home. Yea thanks lady. We had to listen to that screaming for another 4 hours. Again, looking back this showed us something with Alana that would come in handy in the future. If only I hadn’t been such a slow learner on that one though.