Family: forever, for always, and no matter what

Sunday, February 27, 2011

Again, Really??

It was just two weeks ago today that Kaylee started with an ear infection in her right ear. We spent 10 days on an antibiotic and thought we had it licked! Unfortunately, today, Kaylee now has drainage coming from her left ear! She doesn't seem to be bothered by it too terribly. I've only seen her tug on her ear twice, and she isn't running a fever yet. I'm hoping that when I call Dr. Liedman tomorrow, he'll just call in another antibiotic, rather than making us drive all the way to Des Moines, again! I'm comepletely frustrated that we keep getting these ear infections when she has tubes in her ears. My other frustration comes with the effects of the antibiotic. She has had the worst diapers EVER. I can't begin to count the number of clothes changes we have to make each day. Not to mention, her poor bottom is bright red and irritated. Since finishing the antibiotic earlier in the week, her bottom is healing really nicely. I hate to think that we'll have to put her through it again.

Other than that, Kaylee is her usual self, STUBBORN! We've been trying to get her to work on walking on her own, but she is so stuborn, she'll just sit herself down. She'd rather scoot to wherever she wants to go. She has even managed to wear a hole in the "butt" of her pants! Only Kaylee! We are also still working on trying to get this girl to swallow! She fights us every step of the way. Last night I tried to get her to eat some applesauce. She was so mad, she was purposely gagging herself. I tried to get her to just play with it, and she did do some. She tried to feed me, which was interesting to say the least. She eventually got to where she'd put the spoon in her mouth with maybe just the smallest bit of applesauce on it. I wish there was a magic swallowing would solve so many of Kaylee's struggles. She has also been working really hard on annoying her brother. Poor Dylan, he'll get all of his trucks or legos set up just how he wants them, and she'll scoot right in and start destroying all his work. Dylan is only patient for so long before things begin to get ugly. Dylan had a friend stay the other night, and Kaylee was just as excited as he was. Anywhere those boys went, she was right behind them. They were both really patient with her, and put up with more than they had to.

Well, here's hoping for a Monday that doesn't include a trip to Des Moines. I think I'll keep my fingers crossed!!!

Tuesday, February 15, 2011

Keeping our fingers crossed!

Well, we were feeling like we had done pretty good so far this year, up until Sunday night. Miss Kaylee had spent a few restless nights, but otherwise seemed to be doing just fine. However, Sunday evening she started running a fever and pulling/tugging/hitting her right ear. We started with the Tylenol and were lucky enough to still have some ear drops from her surgery to put tubes in, in November. She gave her night nurse, Cami, a definite run for her money Sunday night/Monday morning. Kaylee was so uncomfortable her heart rate even got up to 203bpm. We haven't seen her heart rate there since last March when she went into Mercy with a "bug" in her trach. Any time Kaylee gets sick, it is time for us to get serious. We made sure we had the oxygen ready to go, if in fact, she'd need it. After a couple of intense trips to the ER last year, we make sure everything is ready to go and we have our ducks in a row.

Thankfully, we made it through Sunday night without any major issues....well for Mom and Dad anyway. Poor Cami, got here at Midnight to start her shift, and found that Kaylee's med-port and come unplugged and her feeding was pumping out all over her crib. So, not only was Kaylee feeling miserable, but now Cami had to try to change her and her bedding without really bothering her. If that wasn't bad enough, Kaylee threw up a couple of times in the night, so Cami had to change her then too. Cami does an AMAZING job with Kaylee, but I'm sure she was ready for that shift to be over!

I called up to Kaylee's pediatrician Monday morning, hoping to just get a prescription called in, but no such luck. We had to make a trip to Des Monies to have them look her over. Usually I wouldn't mind, but I could clearly tell it was just her ear bothering her. I didn't really want to have to put her in the car for two hours when she was so miserable. Naturally, we get there and he checks her over and says, "oh, that ear is the problem." DUH!!! I tried to tell him that earlier! He prescribed Kaylee an antibiotic and we were on our way home again. She got her first dose when we got home, and had a much better night than the previous ones. She did need a little O2 to help her out at night, but I think it was because she was so completely exhausted. Today, she is like a whole new little girl, she is playful and getting into everything! I'm so grateful that she is able to handle these things without having any respiratory distress. We are keeping our fingers crossed that this will be our only health issue, and we'll make it into summer where we can shake off all of these germs!

Monday, February 7, 2011

Two years ago today....

What were you doing two years ago today? Don't remember? Me either! However, because the staff at St. Mary's Hospital were so great, they recorded Kaylee's day, everyday, for her entire stay! It is so nice to be able to go back and see where she was then, and where she is now.

So, two years ago today Kaylee was still on a ventilator. She wasn't actually getting any oxygen through it, but is was giving her pressure to take those deep breaths. She was also only getting 45ml of formula every three hours. For those of you who don't know, 45 ml is equal to 1 1/2 ounces. Can you imagine eating only 1 1/2 ounces every 3 hours? They also started her on the Robinul this day. This is the medication used to help control her secretions. It was also two years ago yesterday, that Kaylee underwent an EMG.  This was the Electromyography (EMG)  which is a technique for evaluating and recording the electrical activity produced by skeletal muscles.  The EMG is performed using an instrument called an electromyograph, to produce a record called an electromyogram. An electromyograph detects the electrical potential generated by muscle cells when these cells are electrically or neurologically activated. The signals can be analyzed to detect medical abnormalities, activation level, recruitment order or to analyze the biomechanics of human or animal movement.
For as inactive as Kaylee was at the time, she was too active for them to get an accurate read on the test. It was decided that they'd have to try it again with her sedated.

Funny thing, after reading through these cards telling of Kaylee's day, I realized that I haven't forgotten any part of those days. I can even remember what I was wearing on certain days. When I read about the first EMG, I remembered that they had started it before Troy and I had gotten to the hospital that day. When we walked in the room, there was this massive machine in Kaylee's room with about 3 or 4 people standing around her. They were holding onto her and using this machine to "jab" her with needles in an attempt to measure her muscle response. It was a little scary to walk in and see.

As I think about her stay at St. Mary's, I also think about the other families who had children there. I wonder how their story turned out and where they are now?!?! I am hopeful that their story has turned out like ours....a little miracle that has changed their lives for the better.

I'll finish this post with just one of the funny stories generated by our little miracle:
A while back, I had ran downstairs to switch the laundry around. When I came back up, Troy had just gotten home from work and had walked in the door. He gave me a puzzled look and then looked down at the floor. At this point, I followed his gaze and saw that my purse had been emptied out on the floor. My wallet was wide open, pictures and dollar bills scattered around the floor, and no Kaylee or Dylan anywhere. Troy and I exchange glances again, and realize we should probably locate our children quickly. I hear Dylan in the bathroom so I head that way to find both of my children looking in the toilet!  Yep, you're right...inside my toilet was a dollar bill and a coupon to The Children's Place. Dylan is laughing like crazy, and Kaylee is trying to fish the items back out of the toilet!  I couldn't be mad...we've waited for two years for her to be able to do something ornery like this. However, it does make you wonder....what kind of trouble would she get herself into if she didn't have the CMS to slow her down????

Saturday, February 5, 2011

The Rest of the Story!

Chapter 2: Kaylee is home! 

We were so grateful to finally be bringing Kaylee home were she belonged! We really had started a new chapter in her life. Upon arriving at home we were greeted with a "Welcome Home Kaylee" sign, balloons, a driveway covered in messages made with sidewalk chalk, and Grandma Joyce and Dylan waiting at the door for us. Dylan was SO excited to get to see his sister again. He had only seen her once while at St. Mary's in Rochester. He was too little to be allowed into the NICU at Mercy.

Also waiting at home for us was Kaylee's first in-home nurse. It took us a while to get Kaylee all settled in that day. Bringing Kaylee home also meant bringing home LOTS of equipment. We had oxygen, a pulse ox monitor, an apnea monitor, and a suction machine. Kaylee had to be assessed by the nurse before their services could "officially" begin. Finally, we got Kaylee in her room and in her new crib. It had been a crazy day for her and she was exhausted. One thing we weren't used to having to do was train a nurse on Kaylee's cares. While in the NICU, they were the ones telling us how to care for her. Now the shoe was on the other foot. It was up to us to train them on how to care for Kaylee. Even though we knew her better than anyone, and we were very confident in our ability to care for her, it was still a very overwhelming feeling. Also overwhelming, was the idea of having a "stranger" in your house for most of the day. Our privacy was gone. As thrilled as we were to have her home, there was now, never a "break" from it.

The first couple months seem like a blur now! It took us a while just to get used to everything. Dylan did exceptionally well. For as shy as he was, he warmed up to the nurses very quickly. He adored his sister, and she was quickly working very hard to gain the attention of her brother. We had several different nurses working with Kaylee and after a while it became clear to us that a couple of them just didn't fit for us. They were great people, but we just didn't feel like Kaylee was getting what we wanted for her. Most of the time she spent her days in her crib. It was a lot of work just to get Kaylee out of her room and into the living room. However, we began to get more comfortable with all of the equipment, and we'd spend our evenings all together in the living room. At that time, it seemed like a huge accomplishment. Troy began to really work with Kaylee. Jane Brown, our PT, would come every other week and show us exercises to work on with Kaylee. She started making great progress, and we were thrilled with the small milestones she was making.  In August of 2009, Laura became Kaylee's primary day nurse. She was energetic and amazing with her nursing skills. Kaylee began to do more and more everyday. Things were going really well. Well almost.. during that time we were having terrible issues with Kaylee's G-Tube. The balloon that get inflated to hold the tube into her stomach kept "popping" and her G-tube would fall out. These things are supposed to last for 3-4 months. Kaylee's were lasting for maybe 3-4 days, sometimes even only hours. We were constantly having to call the manufacturer and order replacements. It seemed like just "one more thing" on top of everything else Kaylee was already having to deal with. In September, after a follow up visit to Dr. Selcen at the Mayo Clinic, we saw the doctor who originally placed Kaylee's G-tube. They suggested another brand, and maybe not filling the balloon quite so full. That seemed to do the trick! Thankfully, we were done messing with those pesky things.
While meeting with Dr. Selcen at that September appointment, she was so thrilled with Kaylee's improvement that she called Dr. Engel over to see Kaylee. He rarely sees patients anymore, as he spends most of his time in the lab doing research. He too, was very pleased with Kaylee's progress. They increased her medications, and told us to follow up in 6 months. At that time Kaylee was on Mestinon and Robinul. The Mestinon worked to allow her muscles to pick up more of the chemical needed to make them work. The Robinul was to help control her secretions. Because she wasn't swallowing, she was a constant drooling mess.

Things were going great. We finally felt like we could relax a little. We should have known better! In October of 2009 Kaylee came down with H1N1. After a very scary trip up to Blank Children's Hospital, Kaylee was admitted and placed on a vent to help with her breathing. She would spend 3 weeks there, working to recover. We were incredibly disappointed in the quality of care she received while she was there. Often times we'd go to visit her and we'd walk into the room and find her an absolute mess, with secretions coming out of her trach and mouth, and no one was around to help her. We decided then, that if Kaylee were to ever have to go back to the hospital, we'd return to Mercy. Like it wasn't bad enough to have Kaylee back in the hospital, we hit a deer coming home from seeing her one night! UGGHHH! At that point we were sure we didn't have any luck at all, except bad luck.

In November, Kaylee came home, and by the grace of God, was able to go off the O2 for a while during the day. We were beyond thrilled. This was just one more step in making her a little more "normal", not to mention, easier to travel with! She still required the O2 at night, but it was a minimal amount. She also had tubes placed in her ears that month. December was uneventful, if you can believe that! January rolled around, and with that came Kaylee's 1st birthday. Beyond celebrating her first year, we felt like we were also celebrating the fact that we had all survived that last year! Because of Kaylee's fragile health, we passed on having a birthday party for her. We just didn't feel like we could risk exposing her to something that could put her back in the hospital.

In March we returned to Mayo for another visit. By this time, Troy and I had lots of things to discuss with Dr. Selcen.
#1: Kaylee has ptosis (drooping eyelids) and we were concerned that it could be affecting her eyesight.
#2: Kaylee still wasn't swallowing and we anted to know what more we could do to help with it.
#3: After having all of those spells in the NICU when she'd stop breathing, we were worried about her cognitive development
#4: Now that Kaylee could sit up, she seemed like her head was always tilted to one side
Being as amazing as she is, Dr. Selcen set up several appointments for when we were to return in June.  We would see a pediatric eye doctor, a physical medicine doctor, and an orthopedic surgeon. Other than that, I don't think much else happened at that appointment. We returned home, and the next week Kaylee went to see her Pediatrician. She got her usual 18 month shots and came home. As usual, things didn't slow down. The next week Kaylee got sick again and landed in the PICU at Mercy Hospital in Des Moines. This time, it didn't seem as bad, or so we thought. She had caught some sort of bug in her trach, and it later turned into pneumonia. She was one very sick little girl!  It took some time before the medicine began to work. Again, she spent 3 weeks in the hospital before we finally got to bring her home again.

While all of this was going on, Kaylee's day nurse, Laura, had taken another job. We were beyond sad to see her go, but understood that she needed to do what was best for her. Luckily for us, Crystal, who started as Kaylee's first night nurse, agreed to come on as her new day nurse. It was such a great feeling to know that Kaylee would still be in great hands. Crystal had been with her since the beginning, and we knew she'd have high expectations of Kaylee.

Troy and I began to grow suspicious of whether or not Kaylee's medication was really working for her or not. For most people using Mestinon, you'll see them gain a lot of energy about 10-15 minutes after receiving a dose, or you'll see them "wilt" right before their next dose. With Kaylee, we never saw this. We visited through email, with Dr. Selcen and she gave us the go ahead to take her off of it for a week to see what happened. At first, we didn't notice a change at all, but after about 3 days, we started to see that Kaylee couldn't do the things she could normally do. She would try, but she just couldn't get it to work. We put her back on her meds and things returned to normal.

 In June we went back to Rochester for our follow up visits at the Mayo Clinic. We met with a physical therapist and an occupational therapist. They showed us several activities to work on with Kaylee, and we felt like we could go home and get to work on them. We also met with the orthopedic surgeon, as it was discovered that Kaylee now had Scoliosis. It is very common for kids with muscular issues to develop this condition. Her spine had a curve of 37 degrees. We would later take her to a prosthetics place to have a brace "molded" for her back. We also saw an eye doctor, who gave us great news. Although Kaylee did have ptosis, her eyesight was just fine! Thank God, we couldn't imagine having to try to keep glasses on an 18 month old. Overall, it was a great trip. We would return in July to pick up Kaylee's back brace. She would have to wear it for 12-14 hours a day. She tolerated it really well, and continues to get along fine with it.

Summer and fall were good to Kaylee. She has continued to make amazing progress. In July she started scooting around on her bottom. Most kids wear holes in the knees of their pants, not Kaylee, she wears out the butt of her pants. Once she realized she could get where she wanted by scooting, she was off, and into everything. We still continued to work on teaching Kaylee to crawl. Troy worked every night with her. Between our PT and Troy, she soon figured out how to crawl too. She still prefers to scoot, but does a lot of crawling as well.

In November Kaylee had to have a 2nd set of tubes put in her ears, as the first set had fallen out. She handled it like a champ. I was grateful that such a simple surgery, could really be simple for her too.

In December we made another trip the the MAYO clinic for more follow up appointments. There was A LOT to be done this time. Now that Kaylee was almost 2, there was a new medication Dr. Selcen wanted to try her on. Before doing so, we had to have several tests run to make sure Kaylee would be a candidate for it. This meant that we'd have a couple days of appointments. Dylan is always very curious about Kaylee's doctors, so we took him along. Our morning started early with a blood draw. Kaylee is terribly hard to stick, and I had my doubts about how this was going to go. Much to my surprise, it didn't take nearly as many tries as I had anticipated. Next was the EEG. Kaylee had to have several electrodes "glued" to her head so they could monitor her brain activity.
One of the side effects of this new medication is seizures, so we had to rule that out. This test took about an hour, and yet again, Kaylee surprised me. She was calm through the whole thing, and even fell asleep for a while. I was almost starting to wonder what was going on, because things never run this smoothly for us. Following the EEG was an EKG. We had to be sure her heart was functioning properly as well. This was a quick test, and with the help of her brother, she stayed entertained enough to let them get the information they needed. Next up, an x-ray of her spine and a meeting with Dr. Stans. Kaylee's brace is working! With it on, her curvature was reduced to only 17 degrees. Dr. Stans was very happy with this. He was also impressed that she was doing so well walking while holding onto something. He thought we should be very pleased with where she was. He did suggest maybe wearing the brace a little more throughout the day just to see if it would help her more. We'll follow up with him again this summer.  Later that afternoon we met up with Dr. Selcen. Kaylee was exhausted at this point and was not up for showing off her new skills. Dr. Selcen wasn't able to really get a look at where Miss Kaylee was. She did have all of our test results though. Everything showed that Kaylee could start taking this new med, called 3,4 DAP. (One of the tests did show a possible enlarged ventricle in her heart, so we would have to set up an appointment for an Echocardiogram the next day.) This new drug is so minimally used, that it is not FDA approved, so we'll have to go through MAYO to get it. Without the FDA approval, insurance doesn't cover the cost, but thankfully, it is really cheap! The purpose of this drug is to increase the amount of the chemical needed to stimulate the muscle. The idea is that between the Mestinon and the 3,4 DAP, there will be more of the chemical for longer periods of time, allowing Kaylee's muscles more time to pick up the chemical in order to function properly without fatiguing. We were very excited to get her started on this, and were able to give her the fist dose that evening. It was hard to tell if it was the new med or that she was just happy to be out of the stroller and free to roam around the hotel room, but Kaylee seemed to have a lot of energy that night. We were very hopeful! We would go back to see Dr. Selcen the next day, to see if she thought the 3,4 DAP was making a difference.  Our appointment the next day was scheduled for an hour after Kaylee was to get a dose of the 3,4 DAP. Dr. Selcen thought she noticed Kaylee's mouth being more closed, among other things. Troy and I also felt like maybe she was moving her eyes more and that maybe her tongue was moving more. We were very excited about what we were seeing. Later that day Kaylee had the Echocardiogram done, which revealed a very small hole in her heart. The cardiologist who read the report said this is the case in about 5% of people, and that it was nothing to be concerned about. WHEW!! I'd be lying if I said I hadn't been worried about it.

Two weeks later we celebrated Kaylee's 2nd birthday!! She was spoiled rotten, but deservingly so! We had a Princess Party. Dylan and Nana made her a pretty pink princess cake. Although she wouldn't eat any of it, she did throw it on the floor!

Maybe one of the most exciting things in our lives just happened this week....Kaylee started walking on her OWN!!! It was the most AMAZING thing I've ever seen. Making it even better, was that Troy and I were both home to see it happen. Kaylee was so proud of herself, and you could see her smile when she realized she could do it.

While I feel like I've written a book, I know I have left out TONs of details of Kaylee's journey. Hopefully throughout this blog, I'll be able to go back and touch on those missing pieces.

Until next time....

Here We Are!

Welcome to our blog!! I'm super excited to get this up and running! It is my goal to try to update weekly, but lets be honest...we'll be doing good if we update once a month! :-)                                                       
For those of you who know Kaylee's story, we are hoping this will be a way for us to better keep you up-to-date with Kaylee's progress and her everyday happenings. Not to mention, add a little humor to your life with all of the chaos that exists in the Hoepker household. 

For those of you who don't know the journey Kaylee has been on, here you go:

                                           Chapter 1:
Kaylee's story begins before she ever entered this world. In December of 2008 I found out that Kaylee was not swallowing any of the amniotic fluid. There were also a few other concerns, her little hands were in fists and her skin appeared to be really thick, she also had a pinky toe that slightly hung off to the side of her foot. Chromosome tests were done, but everything came back normal. We would just have to wait for Kaylee's arrival to know for sure what was going on. After many ultrasounds, and amnio reductions, and 4 weeks on bed-rest, in and out of the hospital, Kaylee entered this world on January 14, 2009, three weeks early.

Not breathing at birth, Kaylee was intubated and put on a ventilator to help her breath. The first few days of her life were pretty touch and go. We didn't see any movements from Kaylee and she was still not swallowing. The doctors started ordering all sorts of tests to try to narrow down what could be going on. The MRI came back normal, the EEG came back normal, the EKG came back normal, and all of the chromosome tests came back normal. As great as it was, we still didn't have answers. On February 4, 2009, Kaylee was flown to the St. Mary's Hospital (MAYO) in Rochester MN. There she was seen by Dr. Andrew Engel, and Dr. Duygu Selcen. She was diagnosed with Congenital Myasthenia. We spent about a month and a half in Rochester learning about Congenital Myasthenia, having a G-Tube put in place, and continuing with the intense physical and occupational therapy originally initiated at Mercy Hospital in Des Moines. There were many ups and downs throughout that time, but eventually, we got to a point where we thought we could bring her home. Kaylee had evntually been weened from the ventilator and was breathing with a little O2 help. However, before we could do this, we had to get in-home nursing set up. Thankfully, we had wonderful insurance who would cover this need. Being almost 5 hours away from home, we were struggling with staying up in Rochester with Kaylee, and coming home to Dylan (our then 2 year old). Working together, the St. Mary's in Rochester, and Mercy in Des Moines arranged what they called a "charity run". Mercy would come to Rochester to get Kaylee and bring her back to Iowa at no cost to us. We were more than thrilled to have Kaylee only an hour away from home. We were able to be with both kids in the same day!! Once Kaylee was back and settled at Mercy, she started having "spells" where she would stop breathing. One of Kaylee's biggest struggles with her CMS was that she couldn't swallow. It was determined that because she wasn't swallowing, those secretions were building up in her throat, blocking her airway. As a result, she couldn't breath past them. As our ability to get Kaylee home became more of a reality, we had a new struggle: What happened if Kaylee was home and had a spell? Would we be able to get her breathing again? Our local hospital doesn't offer care to babies other than delivering them. We were confronted with the hardest decision either of us had ever had to make: Do we, or do we not preform a tracheostomy? As the spells continued, we realized the only way we'd ever be able to get Kaylee home and provide her with the best care, was to do it.

On May 7th, 2009, Kaylee underwent surgery to have a tracheostomy tube inserted into her airway. That decision was the best decision we ever made. Days after, we saw more energy in Kaylee than ever before. Taking away some of the work of breathing for her, she had more energy to put into other playing! Finally, on June 4th, 2009, after 142 days in the NICU, we brought our daughter home!

Chapter 2 to is almost midnight, and I'm tired! More to come!