So much has changed, but really nothing has...

At the request of our pediatrician, Jeff and I took Rylee to Chapel Hill on Friday, May 3rd for an appointment with the top pediatric pulmonary doctors in North Carolina. This winter has by far been the worst season for Rylee ever.  She constantly has what seems like a sinus infection and eventually drains into her lungs, causing pneumonia.   With the last bout of pneumonia wreaking so much havoc, taking a month to get rid of and conflicting reports on the structure of her cilia, her pediatrician thought it was best to take her to Chapel Hill.  After a 2.5 hour appointment, the head doctor thinks it's about a 5% chance that Rylee really does have PCD. The ultra-structure of her cilia are normal and her nitrous oxide result was within normal range. She said she can't say with 100% certainty that Rylee does not have PCD. Diagnosing is still a complicated task and it is possible that Rylee could have a variant that is not yet able to be diagnosed.
So while we all think this is great news, I'm having a bit of trouble. At least with a diagnosis, I knew what I was up against and Rylee was getting the best medical treatment she's ever received. It is a possibility that she still can develop bronchiectasis, a permanent widening of her airways. With each repeated lung infection, especially in the same area of the lung, bronchiectasis becomes a greater possibility. With this appointment, other than taking away the diagnosis, nothing changes for Rylee. The head doctor at Chapel Hill is in total agreement with all of Rylee's doctors that the Vest is necessary for her mucociliary clearance on a daily basis, and may be for the rest of her life. She still suffers severe infection and constantly seems to have a sinus infection and a cough. It is a high probability that she can still get atelectasis, that could possibly result in another bronchoscopy.
We're still in limbo with the infectious disease specialist. Rylee received the adult pneumonia vaccine, Pneumovax, in February 2012. Checking immune response to certain viruses is how they test for immunodeficiencies. Her titers responded wonderfully in March 2012. Her titers were checked again this past March and she responded to less than 50% of the strains they checked for. Not the results an infectious disease specialist would like to see, especially in a child who has received the polysaccharide vaccine, Pneumovax. The vaccine should last at least 5 years and should have made her respond to well over 50%. With that being said, he ID specialist was not ready to start her on immunoglobulin therapy. It could mean life-long shots, giving her immune system that push it needs to help fight infection. A few weeks ago, she received the Pneumovax vaccine again. Her levels were checked last week, but we won't receive results until next Friday. If she responds as she did the first time, she'll be checked again in 5 months. If she again responds to less than 50%, we will begin talking about treatment plans. The main goal will be to cut down on the infections and the severity of them.
We appreciate the support everyone has given us. These past 6 months have been quite crazy and stressful, to say the least. Diagnosis or no diagnosis, we will continue to fight for Rylee and the medical care that she needs and is entitled to. 
So, for now, we continue on, just as we have been the past 6 months or so, daily nebulizer and vest treatments and keeping her sinuses and lungs clear of gunk.  After just finishing 4 weeks of antibiotics spread over 6 weeks, Rylee went off to school today with a squealing nose and junk in her throat.  But just as she always does, she didn't complain at all and continues to smile and sing!

More antibiotics

I've slacked a bit in posting recently, but it certainly has been an eventful 3 weeks for the Vogels.  After being off of antibiotics for a little over a week, Rylee had a follow up with her pulmonologist.  Report was sinusitis and junky upper right lung.  Before anything further developed, he felt it was best to put Rylee on a four week dose of gentamicin, though the nebulizer.  She'd do the gentamicin twice a day for two weeks, take a break for two weeks and follow up with another two week dose.  Not only was it another 10 minute nebulizer treatment twice to add to the 60 minutes she already does, but gentamicin comes with serious side effects.  While taking gentamicin through the nebulizer rather than intravenously or intramuscular lessens the side effects, there are still effects we need to be concerned about.  Hearing damage and loss is one of them.  So, at the request of her doctor, I scheduled her for a hearing test, to get a baseline and have results to compare to later.  When she asked why she was getting her hearing tested, I told her everyone does.  I couldn't bring myself to tell her that an antibiotic, which was supposed to make her better, could damage her hearing.  I didn't want her thinking that every antibiotic she takes, will have some extreme effect on her.

Friday past was our last day of our first two weeks on the gentamicin.  While I'm thankful she is off of it, I am extremely worried that her sinuses will begin to get bad again with the tree pollen so heavy.  We'll monitor the next two weeks and hope she doesn't develop anything before our next round.

It has been a tough winter for Rylee.  I kept thinking, if I can just get her through winter, we'll be fine.  These days I'm not so sure.  Since spring has sprung, she's had her first sinus infection with a junky upper right lung and 4 weeks of antibiotics.  She's been getting bloody noses just about daily.  I'm not sure if it is the saline nebulizer treatments 5 times a day, the dry air and pollen or Nasonex nasal spry--maybe a combination.  I have gotten her a saline gel to put in her nose and while it seems to have reduced the amount of times she's saying her nose hurts, it hasn't cut down on the amount of bloody noses.

Tomorrow we return back to the infectious disease specialist.  For the first time in a while, I am having pre-appointment anxiety.  Tomorrow's appointment will determine a lot for Rylee.  We will get back the results of the most recent blood work and will discuss a treatment plan, if one is necessary. Just like vest therapy, this will be a lifetime of treatment.  I'm hoping that whatever the outcome, Jeff and I both have the strength to deal with the results and help to make Rylee understand.  She asked us this weekend when we'd be done doing vest, making a 4 year old understand forever is so hard.  Giving her an explanation she understands, is so much harder.

Finally, pneumonia is gone!

Well, what was supposed to be a straight-forward treatment for strep pneumonia turned into Amoxicillin for 20 straight day in which 10 of those were double strength and a second antibiotic for 5 days.  At this point, we’re hopeful the pneumonia, fluid and atelectasis are all gone.  We are hoping that we’re in the clear, at least for longer than our last stretch, but we are never really sure.

We visited with the new infectious disease specialist on Thursday March 13th and both Jeff and I felt confident when we left.  Rylee had some more blood work done, to check her other Ig levels and to check her IgG response to vaccinations.  She was tested last March, a month after she received the adult pneumococcal vaccine and we were told by the doctor in Winston Salem that her titers were fine.  We figured at that point, we were finished checking and discussing immunodeficiency problems.  However, after some additional blood work in February, Dr. Ashe, her pulmonologist, referred her to an Infectious Disease Specialist.  After spending over an hour with him and briefing him on Rylee’s history,  he discussed his plan of re-evaluating her blood work and coming up with a plan after the results have been reviewed.  We are scheduled to return on Monday April 15^th.  At this point, we are unsure what type of immune deficiency Rylee has but we are hoping to find out.  The ID specialist told us that he was sure he’d figure it out, but that it may take a few months.  It seems that some children lose IgG from gastrointestinal issues, but we’re certain Rylee doesn’t.  He also stated that sometimes children produce enough IgG, but with the high demand of it in their body from recurrent infections, they can’t keep up the supply.  Other children just don’t produce enough.  We are thinking Rylee falls into one of the two last categories.

The ultimate goal is to cut down on the number of infections and the severity of them.  There is a chance that Rylee may need IgG replacement therapy.  It can be done through IV (IVIG) or subcutaneously.  IVIG is usually given once a month, in a hospital.  About 50% of children who receive IVIG suffer with adverse reactions such as fever, headache, nausea, fatigue or chills.  Subcutaneously, shots need to be given once a week.  There is less adverse reactions and it can be done at home, rather than in a hospital.  We’ll discuss further if Rylee needs IgG replacement therapy after the results of her latest blood work have been reviewed.

Rylee continues to cough like an 80 year old with emphysema, but after speaking with her doctor, she expects the cough to linger a few weeks more.  This past week, we are completely off antibiotics, Rylee already sounds congested.  We are hoping she isn’t coming down with a cold or something else as we have a full few weeks ahead.  The only thing we can do at this point, is continue nebs and vest therapy and hope it keeps the lungs clear.

Welcome back pneumonia

This pneumonia wasn't like many of the other cases.  Most of the time, it seems pneumonia settles in after a long battle with a sinus infection or upper respiratory infection. This time it settled quickly. Monday morning started off like any other, a mad rush around the house to get out the door, making sure Vest therapy and nebulizer treatments are done.  Just as we were getting ready to walk out the door, we heard a cough from Rylee.  The "junky" kind. We assumed it'd be like all the times prior, a lingering cough that would go from a sinus infection possibly into bronchitis or pneumonia, unless we could catch it at just the right time. So we'd watch and wait. Unfortunately, it came quicker than we were prepared for.

We picked Rylee up from school Monday and she looked drawn out and sad. My first thought was someone hurt her feelings.  The more I asked, the more she withdrew. We went to Chick-Fil-A for dinner and her mood didn't seem to get much better.  Jeff and I joked that she must have a case of the Mondays. Things seemed to progress normally until about 6:30 pm when I saw Rylee shivering under a blanket. For a kid who is constantly hot, I knew this was out of the sorts. I asked her if she felt ok and she replied that she felt fine, but was so shivery cold.  Before I knew it, she had resorted upstairs to my bed, alone in the dark, under two blankets still shivering.  I again checked, assuming she'd have a fever by this point. I was wrong. She fell asleep by 7:15, quicker and earlier than most nights.  Within a few hours, her temperature started to rise.  At this point, I was extremely concerned she'd picked up the flu.

We made a trip to the pediatrician early afternoon on Tuesday. Beforehand, I knew something wasn't right by the way Rylee was acting. We did neb treatments and Vest and had some breakfast together. I must admit, the one plus to having sick days is getting to spend extra time with my munchkin. After breakfast we headed back upstairs to get dressed for her appointment. It seemed like Rylee could barely make it up the stairs, hunched over using her hands to crawl up.  Once she got upstairs, she laid on my bed, watching TV and didn't move until I said it was time to go. She looked so pitiful, dark circles under the eyes and such pale skin.

At the doctors office, Rylee sat on my lap and fell asleep. This is usually the kid who dances and makes faces at herself in the mirrors at the office. Once her doctor listened and heard the symptoms, she immediately diagnosed pneumonia, bi-lateral.  We opted to get the x-ray.  Although it wasn't going to change the course of treatment, but for a kid who just had a mucus plug fished out via a bronchoscopy, an image might be good for future use.  The x-ray confirmed exactly what the doctor diagnosed, lower lobes of both lungs were invaded by pneumonia.

Rylee and I returned home and immediately started antibiotics. Tuesday seemed to progress without further issues, although Rylee continued to look completely drained.  Just after 1am Tuesday morning, Rylee woke me up saying her tummy hurt. Knowing how often Rylee deals with constipation, my first question was asking if she needed to go potty. Before she could reply, she immediately started vomiting-all over my bed, me and herself. I tried to rush her to the bathroom, but was not successful in getting her to the potty before she vomited on the bathroom floor.  Finally she stopped and the clean-up effort began. Jeff (who is so wonderful to clean up vomit without batting an eye) worked on cleaning up Rylee and the bathroom as I cleaned myself up and changed the sheets on the bed.  I think it was almost an hour before we got back to bed.

Rylee stayed home on Wednesday with Jeff and vomited several more times throughout the day. Our main concern was if the antibiotics had time to be absorbed before throwing up.  Our first thought was that the vomiting was a reaction to the antibiotic.  We promptly called the pediatricians office and asked for a different medication. We started the new antibiotic Wednesday evening.  Within 45 minutes, Rylee has vomited again. At this point we assumed it was a stomach virus and not the medication.  About midnight, Rylee woke up again feeling nauseous. We once had been told by a pediatric ER doctor that Benadryl could be used for nausea, so we quickly gave her a dose.  She soon fell back to sleep.

We had already decided she'd stay home on Thursday.  So Jeff and I could return to work, My mother -in-law said she'd watch Rylee for the day. Things seemed to be on the up and up. Although she still looked tired and drawn out, she was holding down food and liquids, but more importantly medicine. Energy seemed to be slowly coming back, but we certainly didn't want to push her. Our initial thought was to have her return to school on Friday, until I had a quick conversation with the daycare director. She informed me there were a few viruses floating around.  It became a last minute decision to keep her home again Friday, to help avoid picking up any other crud.  

Sitting here now at 10pm, I am certain it was the right decision to keep her home. When diagnosed with pneumonia, for the 8th time in less than 4 years, we were told it was a straight forward treatment and Rylee should be on the mend quickly.  But of course it isn't, not much has come easy to Rylee the last few months.  In the last 10 months, Rylee has had two episodes of bronchitis, four episodes of pneumonia either bi-lateral or right lung and multiple sinus infections. In those past 10 months she has been hospitalized 3 times and this weekend we could be headed for her fourth.  I'd like to go to bed tonight knowing those little lungs are kicking pneumonias butt, but I am just not so confident.  I will continue to monitor her breathing closely, giving nebulizer treatments as often as I have to.  So, welcome back pneumonia, you really didn't have to return so soon.

Two Weeks Post Bronchoscopy

We headed into the bronchoscopy procedure knowing exactly what was going to be done. Not that procedures or your child having anesthesia ever gets easier, but I feel like this time I was more prepared. We knew that once Rylee was asleep, the lungs were going to be cleaned of mucus, especially the lower right lung where the atelectasis remained. All mucus that was suctioned out was being sent for culture-to check for bacteria, viruses, fungus and anything that may be antibiotic resistant.
We arrived at Levine on Friday morning and everyone was calm. I couldn’t believe how well Rylee was coping. We usually try to prepare her for procedures the best we can, giving her the vital information, but doing our best not to scare her. Jeff and I went with her into the pre-op room and she was holding together like a champ. She changed clothes and before long she was headed to the procedure room. At Levine, the nurse told us that one of could escort her to the procedure room and would leave once she was asleep. I looked at Jeff and said “I’m going with her,” as if he didn’t already know those words were going to come out of my mouth.
I could tell as we walked down the hallway, waving good-bye to daddy and Grandma, Rylee was beginning to get a bit nervous. She looked up at me and said “Mommy, hold my hand.” I grabbed her hand, gave it a squeeze and looked her in the eyes and told her “Dr. Ashe promised Daddy and me that he would take good care of you, and he will.” These days, it seems Rylee is becoming more trusting of her doctors, conversing with them more than she ever has. And so we continued down the hall and into the procedure room with our hands locked and an understanding between us that everything was going to turn out just fine. She trusted me, but more importantly, she trusted her doctor.
We were greeted by quite a staff when we got in the procedure room—Dr. Ashe, her pulmonologist, the anesthesiologist, the nurse aesthesis and a couple other nurses. Now, for anyone who knows my little girl, you know that she had every one on the team laughing within no time. Rylee brought Sidney with her, the Cabbage Patch that was once mine as a little girl. Sidney was adorned in a Kiss onsie that Rylee wore when she was a baby. It was within minutes that Rylee and the nurse aesthesis were putting coban on Sidney, so he’d look just like her post-op. Soon after, they were jamming out to Kiss videos on his iPhone. Not only did this help Rylee relax, but it certainly passed the time while she had to do a nebulizer treatment of albuterol from some wheezing. Once albuterol was done and wheezing had subsided, it was time to start the bronchoscopy. I stood next to Rylee, holding her hand as she inhaled the gas through a mask. I have never seen my child suck in as hard as she was. Later, I found out the gas smelled like bubble gum, so that could be the reason. Within minutes her eyes closed and I was out the door.
A nurse escorted me back to the waiting room, where Jeff, my parents and mother-in-law were waiting. On our walk, the nurse said “you’ve been through this a few times, it gets easier, right?” No, it doesn’t and I am quite certain it never will. I tried to be as relaxed as I could in the waiting room, but my nerves were out of control. 30 minutes felt like an eternity…I just wanted someone to tell me my baby was fine and the bronchoscopy was done.
They finally came and got us, we raced to the door as if we couldn’t get to her fast enough. We walked into the recovery room and Rylee was still sound asleep. Jeff and I immediately checked stats and asked how they have been handling. They seemed to stay steady between 96-97%. We sat and waited for Rylee to wake up, doing our best not to disturb her, but just staring at her as if we couldn’t get enough.
Dr. Ashe came in a few minutes later and told us she handled the bronchoscopy wonderfully. She had no problems with coughing or wheezing and didn’t require an albuterol nebulizer treatment post procedure. I remember him saying that she needed the bronchoscopy, a part of me thinks most of her doctors know how often I second guess myself when Rylee goes for procedures. He told us he cleaned all portions of her lungs, especially the right lower. He showed up pictures of all her airways, reporting no damage or widening to any. Great news and such a relief!
We had a follow up on Wednesday with Dr. Ashe. The report from the culture of the sputum and mucus they collected from her lungs and nasal cavities all came back within normal limits. Good news again! However, when listening to Rylee, he said her upper airways are raspy.
Today, we are two weeks post bronchoscopy and the congestion continues.  It started just as it always does—a runny and stuffy nose. Yesterday, she woke up with a wet sounding cough. While we try to remain positive, we know it is the same cycle every time. We’ll keep hitting her hard with nebulizer treatments and vest therapy and hope it doesn’t progress into something worse. The saga continues.

Preparing for the first bronchoscopy

Well, we knew the day was coming, but we weren't sure when they would schedule Rylee for her first bronchoscopy. This Friday, 11am, the date is set. Our goal, keeping Rylee healthy enough that she can have the procedure, but more importantly that she will recover with no breathing issues afterwards, causing her to have to be admitted.  The pulmonologist went over the procedure with us in the office today and while it all sounds fairly simple and quick, I feel terrible that my little girl has to go through this. 

I'm still riddled with guilt, even two months post diagnosis.  I know there wasn't anything I did to directly cause Rylee to have PCD and that is not where most of my guilt comes from.  Seems like these days my guilt is more from things like sending her to school when I know she is not at 100%, feeling that I'm just not doing enough for PCD research or not taking her to the doctor soon enough to avoid more severe infection.  I feel guilty for being a working mom, when I feel like the only place I should be is at home, taking care of my little girl.  The more I try to be Supermom, the more I realize I'm not.  I'm not sure that the guilt will ever subside permanently, but I'm hopeful that someday things will get eaiser for Rylee.  Sometimes when I feel guilty or sad about the whole situation, I remind myself that it isn't me who fights daily for breaths, it is Rylee.  And really, if she isn't complaining, who am I too?  So I continue on, doing the only things I know to do--therapies and treatments for her.

So on Friday, we'll take Rylee to CMC-Levine for her bronchoscopy.  The whole procedure should last about an hour.  They'll go in and suck out all the mucus they can find.  What they suck out will be caught in a trap and sent off for culture--checking to see if it is bacterial, viral, fungal or antibiotic resistant.  The positive side is that while she has the scope, there will be a camera to take pictures--pictures that can be used later for comparisons.  They will also be able to tell if the atelectasis has caused any permanent damage thus far.

My next goal, once we get Rylee on a healthy track--date night with my husband.  We have spent countless hours the past two months taking care of Rylee, doing all we can to get her better and keep her that way, that we haven't had a date night or quality adult time in what seems like forever.  At this point, I feel like it's too much work to have someone watch Rylee for extended periods of time, so I hardly ask.  Maybe I can even sneak a night away to watch my husband perform with his new band!

My two nieces have entered a contest on the Disney Channel, the Friends for Change grant.  It is a grant for organizations or individuals to help make lasting, positive changes in the world.  Their goal is to bring awareness for all those suffering PCD, especially their little cousin.  I can't express in words how I felt when they called to tell us, each with their own projects, to help make any bit of a difference they can.  Rylee doesn't understand the impact that this could have for the PCD community, but Jeff and I do.  So, Haley and Natalie, thank you!  The three of us will be here to help you in whatever way we can.  But no matter what the outcome, Uncle Jeff and I are so proud of you for trying to do such a great thing for your little cousin and we will always be thankful!

But for now, we take it one day at a time.  I am so happy to say that when necessary, this family pulls together for one special little girl!

Justin Bieber

Well, I was sure in my last post that something had gained a hold of Rylee's respiratory system, but I wasn't sure what.  As she laid on the couch watching TV, looking so worn out, I checked her levels again to find they had dropped to about 90%.  I knew it wouldn't be long before we'd be heading to the hospital, but hoped the Augmentin she'd just started and the prednisone that she'd been on for a 24 hours would quickly kick in. After Rylee went to bed, we again checked her oxygen level and it had dipped below 90%, hitting about 87% before stabling off.  We did back to back nebulizer treatments of Albuterol, as we have been told before.  Neither treatment helped.  We placed the call to our pediatrician and headed to Hemby Children's Hospital.  We spent Tuesday night in the hospital and the doctor was sure she'd be discharged that afternoon if her oxygen levels stayed steady and if a repeat chest X-ray looked the same or better than the one she had the Friday prior.  We were certain it did, how could it not with all the Vest Therapy, hypertonic saline treatments, antibiotic and oral prednisone.  About an hour after the X-ray the doctor informed us that the X-ray looked worse, they suspected pneumonia was beginning for form in the right lower lobe where the atelecstasis has remained for the past two months.  The pulmonologist had warned that the mucus plug (atelecstasis) was a breeding ground for bacteria, and that would be our major concern with it not dislodging soon.  We stayed Wednesday night, to make sure Rylee could hold her oxygen levels during sleep and to make sure the pneumonia was being contained.  Things seemed to go as planned on Wednesday night and Rylee was discharged Thursday late morning.  We kept her out of school Friday and somewhat hibernated throughout the weekend.  I had planned to be off Monday, Tuesday and Wednesday, since Tuesday was the Justin Bieber concert and Grandma and Pa were in town.

Rylee got to attend the concert and I made every effort I could to make sure that she could.  We had an appointment with the pediatrician on Monday, just to have a listen and to have an x-ray to see if the atelecstasis was still there and if the pneumonia was looking any better.   The X-ray technician was out, so we were told we could come back sometime this week to get it done.  A part of me was scared to take her Tuesday morning, fearing something might interfere with her going to the concert that she has been waiting for.  So I held off on taking her in Tuesday. 

The day was so great and I hope that Rylee remembers it that way as well.  I wanted it to be about her and special, she deserved this day more than anyone I know.  We made signs, took pictures, had a special outfit and got to attend a pre-concert party downtown.  Rylee has found a new heart throb, Cody Simpson who opened the show.  Another teenie-booper artist we'll have to listen too.  I don't mind though, I know Rylee really enjoys her music.  Carly Rae was great and Rylee really enjoyed her.  Waiting for Justin to come on and Rylee started chanting Justin Bieber with the rest of the crowd.  She was excited to see Justin but was a bit disappointed that she couldn't see his face.  She held out until just before 10pm when she asked to go home because she was tired.  We knew it was the end, so we beat the crowd out!  On the way to the car, she told me she was sad because she didn't get to give the shirt we brought to Justin.  We had brought an extra PCD shirt and she wanted Justin to have it.  I thought it would have been great, any more exposure PCD can get would be great.  It was hard for me to hear that because she's starting to ask questions and understand more of why she's constantly coughing or battling some issue with her lungs.  I think she is starting to understand how much her dad and I want to raise awareness for PCD and how important it is and so maybe that was her way of raising awareness.  I told her I would try to find a way to get it to him, so my new mission begins.  I'm not sure exactly how I'll get it to him, but I'll do my best to try.

It was on the way out of the concert, Rylee and I decided that Never Say Never should be her PCD theme song.  With her being such a Bieber fan, I thought the lyrics were perfect--"I will never say never, I will fight til forever"  "whenever you knock me down, I will not stay on the ground."  Rylee is a fighter and some how she always gets back up-from atelecstasis, pneumonia and sinus infections.  Justin's music always puts a smile on her face and seems to let her escape whatever is trying to bring her down.  Music, singing and dancing it what she enjoys, what makes her happy.  Her dad and I enjoy watching her, simply because we know how happy it makes her.

She had her repeat chest X-ray today and the initial report from the tech was that the right lung looked worse than it did on the 11th of this month.  We'll get a more detailed report from the pediatrician in the next day or so.  Rylee has an appointment with the pulmonologist on Monday and from there he'll decided whether she needs a bronchoscopy to remove the mucus plug, based on the last 3 or 4 X-rays.  As much as we don't want her to have the bronchoscopy, we'd like to have this plug removed before it does any further damage and causes any more infection.