Friday, May 20, 2016

Hospital Admit for CF Exacerbation Review

Thursday 5/19/2016 was the day . . .  that I finally felt like I'd found my feet again and things were back to our "normal". . .
it's been a long time coming.

Between the increased cough that began after Easter that just wouldn't go away, to the hospital admit for a week, to the 8 days immediately following of iv antibiotics administered at home, to the follow up visit at CF clinic to re-evaluate pft's and lung function, to the 7 hour visit Wednesday for the research study Olivia is in - it's been a whole heck of a lot in a very short amount of time. 

But, now it's the calm after the storm. Olivia got to go back to school Thursday for a full day for the first time in I can't even recall how long. The house is quiet, I can finally catch up on cleaning and laundry and just sitting down for a minute to just . . . sit. Although I must say, the quiet isn't all it's cracked up to be, I miss my kiddos.

On Tuesday 5/17/2016, I took Olivia down for her hospital admit follow up with our CF team. Her pft's are going up, the FEV1 values were at 110% and 111%. Thus, she is inching back up to the goal of 114% and we could't be more ecstatic, knowing that sometimes lung function isn't fully restored after these hits and she's pretty darn close and to be over 100%, is great! Her weight was up, and for the first time in a long time her BMI was over the 50th percentile at 60th something and most importantly the cough is gone. Since it was a good report, she was able to have her PICC line removed on Tuesday. The minute she was able to, she was doing cartwheels and handstands and riding dirt bike; all things she had missed doing since she was sick and had the PICC line in place. Then on Wednesday, we were back down in the city for the research study she's enrolled in from 9 am to 4 pm; it's been busy, but I couldn't be more happy that we are getting back to normal around here with school, living life and just 2 treatments for Olivia per day. 

So on that note, I wanted to review our hospital admit and document the week.

On Monday, May 2nd when we were admitted, it was about one of the only few appointments Jeff hasn't been with us to and it was one of the few where I had to bring Landen along with us because of the time. That was tough; to not have Jeff with me and to try to be composed as to not upset either of the kids, which was a fail. We were thrown such a curve ball beings this appointment was supposed to be a pre-op appointment that I was scared and sad and all kinds of emotions so that made it much worse for Olivia and Landen playing off the emotions I was displaying.

When we walked over to the hospital not even 2 hours after Olivia did her pft's in clinic, (our CF team doesn't mess around) and settled into our hospital room, the fear was apparent on both of my kid's faces. I sat on the bed with Olivia and she matter of factly asked me if she was going to die. By this time I was composed enough and the reality of it all had sunk in so I was able to answer her honestly as I always do, telling her that no, she was not going to die from this nor while we were in the hospital.

It was a hustle and bustle as the team wanted to get an iv in place as quickly as possible to begin the iv antibiotics. Through the entire admit process of tell and retell your situation to about 10 different people, I was able to get my sister on the phone, get her over to my house to help Jeff pack up, feed Kitty and make arrangements for my dog. I can't express enough how fortunate we are to live close to Darci who can help out in times of crisis like this that are unexpected. By dinner time Jeff had arrived with all of our stuff and bags in tow. Olivia managed to get through the iv placement, realizing it wasn't so bad and the 4 of us, Landen included, hunkered down in the hospital room for the night.

On Tuesday, May 3rd, it was much of the same, rest, eat, watch movies, play on tablets, 4 aggressive 1/2 hour each nebulizer and vest treatments, iv antibiotics at 6 pm and we did venture outside to the park and sports court on the hospital grounds. I must say, having these outdoor facilities is amazing. Even though I still felt like a prisoner while being in the hospital, having the ability to get outside, get fresh air, let the kids play and be kids, was terrific. Olivia also received gifts from the gift shop from Grandma and Grandpa and Aunts Em and Jennie and family's which definitely brightened her mood.

Tuesday afternoon when the nurse went to flush Olivia's iv, it was leaking and needed to be removed and a new one placed before we could do her iv antibiotics. Since she was scheduled for PICC placement the next morning, I asked if they could do it sooner as to not have to undergo another poke, but unfortunately, they were not able to get Olivia's PICC placement moved up so she had to endure the whole iv placement process again, which is a huge anxiety inducer for her, but the 2nd go around at it was better. Tuesday evening Olivia was NPO so no g-tube was run that night.

On Wednesday, May 4th, Olivia had her PICC line placed in the morning, which ended up being quite a long process because of delays in the OR, but we were back up to our room around 1:00 for some lunch, and by this time, Olivia was hungry! Much of the same with treatments, eating, resting and playing outside. Wednesday evening was tough for all, Jeff and Landen headed back home, so that Landen could return to school on Thursday. Once the boys left, it was quiet and we surely did miss them. It was a sleepless night with Olivia's g-tube pump alarming nonstop due to clogging of the viokase enzymes through the feeding bag. At home, I crush the enzymes into a fine powder with a mortar and pestle set and we use a different bag and pump than the hospital uses. Our bags at home have a larger diameter tube and thus they do not clog. It was a long night and our night nurse was in and out of our room so often dealing with that fiasco,we definitely kept her on her toes.

On Thursday, May 5th, Jeff dropped Landen off at school and then headed back down to the hospital in time for Olivia's 2nd go at pft's. Things were better when he got back. Olivia had pft's and her FEV1 was up to 84% so heading in the right direction but not quite high enough for discharge. Olivia and I did arts and crafts and coloring and decorating of her hospital room windows, knowing it'd be home for a while longer. Keeping busy while trying to stay sane. More outside time and then since we knew we weren't going to get to go home, I left for home on Thursday evening to get Landen from my sister Darci, unpack, repack, do laundry, etc. It was hard to leave and I was exhausted, yet it was still after 1:00 am before my head finally hit the pillow but things were better having my little bud Landen with me.

Friday morning, May 6th, I took Landen to school, finished up some things at home and then I picked him up early, we grabbed Culver's for lunch and then headed back down to the hospital for the weekend. It was so much better for everyone when we were all four back together. Makes for cramped quarters in a hospital room with a chair and couch and that's it to sleep on, but so worth it. Landen missed and worried about Olivia and when he wasn't there she sure was missing on him. Friday afternoon we played outside, checked out some movies and video games from the Patient Center and escaped the hospital grounds for just a bit for a car ride and some new scenery. Jeff thought it was okay to leave until one of the RT's was telling us, "maybe you could get a pass, you know a pass to leave". To which after she left the room, Jeff looked at me and said, I didn't know we weren't supposed to leave, I told our nurse we went out and ran some errands! However, we didn't miss anything, we left in the afternoon when there was nothing but sitting around to do anyway, and it was a nice mental break for all of us. When we returned it was iv antibiotic time, supper and then we had a late night with movie and a popcorn before calling it a night.

Saturday, May 7th, was a pretty good day for Olivia. We lounged around in the morning and of course ventured outside for our daily park and sports court time. Olivia's teacher Mrs. Heath and her family came to visit Olivia in the early afternoon. Olivia was so excited with anticipation of Mrs. Heath's visit, she even had to wear her very special and brand new kitty shirt for Mrs. Heath. Mrs. Heath brought gifts and goodies, gifts from Landen's teacher Mrs. Reed and cards from Olivia's classmates, which were all very sweet and one of the highlights of the day. Let me tell you, the cards from Olivia's kindergarten friends were so, so sweet. When her and I read through them, the amount of time and coloring some of those sweet kiddos put into those pictures . . . priceless and definitely something for the keepsake box. In most all of them, the kids had each drawn a cat, knowing how much Olivia loves kitties!

The other highlight of the day was a visit from Grandma Pam. She got to come out to the park and sports court with us, she brought a video game for the kids which is now a favorite of the kids' and mine, and what we three play nightly during Olivia's treatment and we got to have a dinner of pizza with her before calling it a night. It was a good day.

Sunday was Mother's Day. Definitely a Mother's Day I'll never forget. Even though the perfect gift would have been to be at home, it was pretty special all the same. Both Landen and Olivia gave me homemade gifts they made at school, which are always, always my favorite and most cherished.
Landen made me a very neat magnet gift and Olivia made sugar scrub.

The kids and Jeff bought me flowers from the hospital gift shop and gave me a keurig coffee machine which I have been getting lots of use out of. Our attending cf doctor for the week brought me in cookies from a cookie shop which were very yummy and very sweet of her and the hospital brought in a Mary Kay gift basket that had donations in it from different MK reps throughout the metro region and a Starbucks gift card, all in all, very, very sweet. The best part was that we were all together, even if it was in the hospital. We were still able to play outside and go for walks and know that the end of our stay was near, unlike many of the other family's and mother's that were spending their Mother's Day there.

Landen didn't want to have to leave on Sunday night to go home and get to school on Monday. I tried kicking Jeff out and sending them home as he was definitely in need of a break, mentally and emotionally but in the end we settled on all hunkering down for another night in the hospital. Landen and I instead got up around 5:30 am Monday morning and headed for North Branch. I dropped him off at school and turned right around and headed straight back to the hospital. I made decent time and I was back before pft's.

PFT's were supposed to be at 9:00 am, the team came in for rounds and said it would be closer to 11:00, I think it was closer to noon. We were supposed to be first in line but RT ended up helping our neighbor with CF, first, which didn't sit well with us and by Monday after a week in the hospital with very little sleep and the stress and mental games that a hospital does to you, Jeff and I were both about ready to throw a chair, or two through the window. Finally, pft's were done, her highest number was 99%, we started packing, were discharged a few hours later to do iv antibiotics at home for 8 days and were home before 4:00 pm. Leaving that last time was bliss and being home and sleeping in our own beds was the best medicine ever.

It was a long week with ups and downs, but the hospital stay and iv antibiotics did what they needed to do and having gone through it once and for the very first time and the emotional roller coaster that comes with it, well now we know and when it comes down to it, to finally have Olivia be cough free and spunky again and back to the Olivia we know, well there is nothing better than that.

Monday, May 16, 2016

Home IV Antibiotics using the Eclipse Device

While Olivia was in the hospital for her first cf pulmonary exacerbation, upon admittance and within a few hours, she began her first round of iv antibiotics. An iv was placed in her right hand to administer this first dose. I was told the iv was placed in her hand rather than in her arm just in case the iv would be pulled out or need to be replaced, they would gradually move up the arm for subsequent placement location. It was a good thing, because the iv was not working on day 2 and a new one was placed a bit further up her arm.

The antibiotic Olivia was prescribed was a broad spectrum antibiotic that would cover that which was cultured in her latest sputum culture. I was told this would also work on pseudomonas if it were lurking deep in her lungs. Olivia has never cultured pseudomonas but what is cultured in a throat sputum culture does not necessarily match that which can be lurking in the lower lungs.

Once Olivia's PICC line was placed, the other iv's were removed from her hands and all antibiotic infusions from this point forward would be administered through the PICC. She is to run iv antibiotics for a little over 2 weeks. Olivia's PICC has a needleless connector on it that has a check valve in it and the connector on the iv bag simply threads/screws onto this needleless connector attached to Olivia's PICC line. In the hospital, the antibiotic and sodium chloride were infused at the same time using an iv pump.

When I attended the required PICC training session at the hospital for at home infusion I was pleasantly surprised that the iv's we would infuse at home would either come in an eclipse device/medicine ball or in a pop can looking device. Olivia's came in the eclipse device and the benefit is that you do not need a iv pump to infuse it. It's this cool little medicine ball, containing everything you need all in one. Once you've cleaned, sterilized, flushed the line and primed the eclipse, you simply unclamp it and it does it's thing. Genius! Since there's no machine needed, nothing alarms you when the infusion is complete. However, we know that Olivia's med runs for an hour so after that time I check on the eclipse and if the ball now looks like a flat butterfly or moth with a hard cylindrical core, it's done. I just set an alarm on my phone for about an hour and check it after that. We infuse Olivia's antibiotic at 8:00 pm and it's once daily. Therefore, she's sleeping or at least in bed, but if one needed to do the iv antibiotic two or more times a day, the child can simply place the medicine ball in their pocket or clamp it to their shirt and away they go, no pushing around an iv pump and pole or having to miss out on things while infusing. I love that.

So, here is how we infuse Olivia's once/daily antibiotic at home using the eclipse device. This is not intended as medical advice, but rather a write up of how we do things so that the next time I have to do it, I can refresh my mind with this checklist.

1. Clean your surface; we use a clean cookie sheet with a sterile drape to place all of Olivia's supplies on.

2. Wash hands: wash, wash and wash again and then dry thoroughly with paper towels.

3. Gather your supplies. Make sure your iv device is actually yours, not expired, the correct antibiotic etc.

4. Place all your supplies on the sterile surface (in our case, the cookie sheet)

5. Put on rubber gloves

6. Prime the sodium chloride pre-filled syringe: twist off the cap, "flick" to remove the air bubbles and push up the syringe until a few droplets of solution come out. Recap and set down on your sterile surface.

7. Prime the Eclipse Device (unclamp the clamp and twist cap slightly, do not remove it, once you see a drop of the antibiotic bubble come out of the connector, re-tighten cap and clamp the clamp connector and set down on the sterile surface).

8. Tear open 2 antiseptic wipes.

9. Scrub the end of the needleless connector that is attached to your child's PICC line for 15-30 seconds

10. Let air dry! This is crucial, it needs to be dry to be sterile - to kill germs.

11. Uncap the sodium chloride syringe, be careful not to touch the sterile parts, apply light pressure and screw on to the connector. Do not over tighten, The needleless connector can crack/break.

12. With the syringe of sodium chloride attached, flush PICC line using a push-pause, push-pause method.

13. Unscrew empty sodium chloride syringe from your child's connector and discard.

14. Scrub the end of the needleless connector again for 15-30 seconds.

15. Let air dry! This is crucial, it needs to be dry to be sterile - to kill germs.

16. Unclamp the clamp on the Eclipse device, remove the cap from Eclipse connector, and with slight pressure screw onto needleless connector of child's iv catheter, do not over tighten.

17. Clamp Medicine ball onto clothing or put in a pocket, or in our case, we clamp it to Olivia's pillow.

18. Let the eclipse device do it's thing for an hour and that's almost it.

*You could choose to get all your supplies at the beginning but I choose to get my "setting up" supplies at the beginning and then I get my "completion" supplies at the end. I don't want the 2nd sodium chloride syringe needed for flushing after infusion to fall or get bumped or what have you.*

19. Gather your supplies. You need to clean and flush after the iv infusion is complete.

20. With clean and gloved hands, tear open 1 antiseptic wipe, prime 1 sodium chloride syringe, disconnect the eclipse iv device from the child's iv catheter and discard. Scrub the end of the needleless connector on the PICC line for 15-30 seconds, let air dry, flush the PICC line with the pre-filled syringe of sodium chloride using the push-pause, push-pause method. Unscrew and discard. Cover up the PICC line and connector with whatever you are using for a sleeve to protect it (sock, tights, long-sleeve shirt etc.) and you are done.

Again, so very crucial to be sterile and clean but once you get all the steps down it's very easy.

If I had a choice, I'd of course never choose to have to administer iv antibiotics to my daughter at all. However, not having that choice, on any given day, I'd every single time, choose to infuse antibiotics at home rather than have it be an inpatient hospital stay.

Olivia is on iv antibiotics for 15 days minimum right now. We had no choice to be in the hospital for a week, the doctors would not release us until Monday 5/9/16 after the increase in pft's. At that time our team was confident we could do iv infusion at home which I'll take that every time over sitting in the hospital for the full 15 days of iv antibiotic treatment.

We are glad to be home and as with all things cf, it's never a matter of IF, but WHEN, so when she needs iv antibiotics again I'll be ready for the next round of iv infusion.

I take Olivia in for a CF follow-up visit and to do pft's Tuesday, 5/17/16. If all things look good, her PICC will be pulled and the iv antibiotic will be finished.

Thursday, May 12, 2016

Olivia's PICC Line for IV antibiotic infusion

On Wednesday May 4th, Olivia had a PICC line placed. A PICC line as defined by the CFF is:

 "a peripherally inserted central catheter, often called a "PICC line," is a long, very thin, flexible tube that is usually placed into one of the large veins in the arm, often just above or just below the elbow. This tube is threaded into a large vein above the right side of the heart."

Olivia had to go through surgery and sedation to have her PICC line placed. For this surgery, since she had an iv placed in her hand, she was able to have her sleeping medicine administered through the iv. The mask that she's had for sedation for other surgeries scares her so this was a much better route. The time it took for the team to place the PICC and have her back into our room for recovery was less than an hour so it was a quick procedure. She woke up within 10 minutes after she was wheeled back to us and she was loopy and quite hilarious but so much better waking up from this anesthesia than the heavily sedated ones where she's so irritable and mad and sad . . .

The iv's she had placed in her hands were then removed and she was given the clear to head back to our room on the 6th floor. Once we were there she was pretty much good to go and acting like herself again. She got out of bed, got dressed back in her own clothes, ditching the hospital garb and was ready to have some lunch. By the afternoon we were taking walks around the hospital grounds and playing in the hospital park and sports court.

Of course in true Olivia fashion she wanted to do handstands and cartwheels and hang from bars, all things we were told need to be limited while she has the PICC line. It's been challenging trying to keep her from doing these things that she loves. It was also a pretty big bummer realizing that her gymnastics show that she's been working up to all year long happens to be on Sunday. Since she has her PICC line she will not be able to participate in it and had to withdraw from gymnastics for the remainder of this year. For as much of a bummer as it is, I've told Olivia this PICC line isn't forever and to remember things could always be worse. She will still receive her trophy for all her hard work this year in gymnastics so that makes up for some of it.

She didn't seem to have any pain from the PICC line placement. She was cautious of using her arm and moving it as I'm sure it feels weird and different but other than that, she's done just fine with it. The first night after placement, when we got back to the room after our outdoor adventures, we noticed that the disk covering her PICC incision site was bloody and that the blood had extended beyond the disks edges. Olivia of course began to freak out, (as anyone would and I too in fact had a moment of panic set in), thinking that she had pulled it lose or who knows what. I called the nurse and to everyone's relief, she told us that the bleeding like that was completely normal the first day. Since the blood had spread however, her bandage would need to be replaced. The nurse did this later that evening and once it was done Olivia was much more calm.

One note worth noting that is pretty common sense is that the PICC line has to be kept sterile and clean. The entire site is covered with a watertight bandage and it must be kept dry. The connector that extends outside of the bandage that hooks up to the iv connector is a check valve type of connector. Impeccable cleaning and sterile procedures must be used each and every time a syringe or iv is hooked up to the PICC line. If germs or bacteria get into the PICC line that goes into your blood stream . . . well you get the picture, not good. Everyday we need to monitor the PICC line to make sure that more of the line isn't sticking out, that more of the line isn't pushed in, that signs of infection are not present. If any of these things are to happen, it's a call to the hospital and go from there.

In order to protect her PICC line from getting caught on something, getting pulled out, cut etc. we keep it covered. At the hospital they had a fishnet type of wrap . . .

. . . this was okay but the connector would always push it's way through the netting, making for a tangled mess. We were also offered a soft stint to keep it covered; this was kind of bulky. Our CF social worker yesterday recommended socks, cutting the toes off and using that. I thought this was genius. Of course we could just wear long sleeves too but some of Olivia's shirts can be tight so I found an old pair of tights, that no longer fit. This was perfect, I was able to cut off the tights at the heel and it made a nice tube covering for keeping her PICC line covered and secure and it's a triple bonus that what we had, happened to be Kitty tights! We have a kitten and kittens like strings and dangly things so keeping Olivia's line covered completely so that the cat doesn't try to attack it, will be very important!

Olivia's PICC line is held into place by two stitches on either side of it. When she is finished with her iv antibiotics, in clinic, they will simply snip away the two stitches and pull the line out. I was told that, "we want to remove the PICC line as soon as possible". The plan right now is to run Olivia's antibiotics through Monday, 5/16/16. We will then return to CF Clinic for pft's followed by a check up with our CF team. If her pft numbers are back near the FEV1 value of 114% baseline, her check up goes well and the antibiotics can be stopped, the PICC line will be removed.

Thus . . . Olivia's story with her very first PICC line.

Next up I'll post about the at home iv antibiotics and how pleasantly surprised we were that we wouldn't have to use an iv pump for this!

Wednesday, May 11, 2016

CF Pulmonary Exacerbation - what is it?

Olivia was admitted to the hospital on 5/2/16 for her first CF Pulmonary Exacerbation. What exactly is a CF Pulmonary Exacerbation? I could not find a clear and precise definition but this link from the National Institute of Health provides the following information on CF Pulmonary Exacerbation:

"The chronic infection and inflammation of cystic fibrosis (CF) lung disease causes a progressive decline of lung function resulting in daily symptoms such as cough and sputum production. There are intermittent episodes of acute worsening of symptoms, more commonly referred to as pulmonary exacerbations. Despite this being a common event, there is still no standardized definition of an exacerbation. 

"The treatment of an exacerbation is intended to resolve the worsened symptoms and to restore the lung function that is commonly lost in the acute presentation. A most striking finding is the observation that for many patients there is no restoration of lung function, suggesting we either need better therapies to prevent exacerbations or better treatment of exacerbations."

I myself had to look up exactly what a CF pulmonary exacerbation was because this was our first go around with one. I've been told of symptoms to look out for, I've read about them, I've recited them to the home nurse that calls to followup every 12 weeks, but having never dealt with one and having few of the common symptoms, it was all new and foreign to me. After all, I had been in contact with the CF Clinic on and off through emails and phone calls and clinic visits regarding Olivia's health and her cough for the past 2 months. Medications continued to be thrown her way consults and followups with ENT were done and we were kind of told in the end to "hang in there" and wait it out. Never once did CF pulmonary exacerbation present itself in any of the conversations. Olivia didn't have a fever, she was eating better than usual for the most part, her weight was up dramatically and her pft's were the best they had ever been. She was more tired than normal but with school and gymnastics and everything else, being tired wasn't that abnormal. It was pretty much the cough that would not go away.

It was a clear case of the silent destruction that is CF. Olivia looked great, she was able to keep up with all her activities and yet she was one very sick little girl, her lungs were taking a huge hit and were very, very ill.

Well . . . now we know.

Now we know what to look for with Olivia and to trust our guts. I kept preaching to the choir, that something had to give, this cough was not normal, something was wrong. I should have preached longer and louder and have been more persistent. Live and learn right?

For as crummy and scary and all the other things that this CF pulmonary exacerbation and hospital stay was, we learned a thing or two as well. We now know what to look for in the onset of an exacerbation for Olivia.  We know what a PICC line is and how it's used and how to administer iv antibiotics through it. We learned somethings about her medications and nebulized meds. We know that every time a cf patient takes a hit in their lung function, that the lung function may never be restored. That's quite a bite to chew right there. On one level, in the back of my mind, I knew that and understood it, but it's like so many things, until it happens to you, you don't really put it on the front burner. We now know what to do when the next CF exacerbation hits, and I hope and pray that it is not for a very, very long time.

It's tricky because this school year Olivia has had "chest colds" where she's had a cough with increased sputum and chest congestion, clear signs and symptoms of CF pulmonary exacerbation, but those cleared up without any intervention aside from increased respiratory therapy. So for us, what I will look for going forward are those same "chest cold" symptoms that last longer and don't go away. In Olivia's case of cf pulmonary exacerbation the symptoms signalling another one are:
*cough with sputum (at her baseline she never has a cough)
*lethargic and tired
*chest congestion

At our last CF clinic visit, I was told by our team that there are 4 signs that they look for when determining a CF pulmonary exacerbation, they include: weight loss, increased cough, new crackles and/or wheezing and increased sputum. Olivia didn't have weight loss, she had a huge weight gain, and she had no crackles or wheezing. The cough was there with increased sputum but because she didn't have all the symptoms it wasn't clear if she was experiencing an exacerbation or if she had pneumonia. She also does have ENT issues so it was a grayish area for us. The chest x-ray showing a mucous plug or infection in her lower right lobe along with the decrease in her pft's were the deciding factors in admitting Olivia to the hospital for cf pulmonary exacerbation treatment with iv antibiotics.

So . . .

I've learned that a CF pulmonary exacerbation is not always clear and concise to determine. I also know to trust my gut and instead of "waiting it out" I'll push harder to have all the tests and procedures done earlier so that her pft's maybe won't drop as drastically before she's admitted next time. I know that while Olivia's lung function is inching back up near the 100's and will hopefully get back to that 114%, going forward and even now, it may never be restored to what it was. I know now, more than ever, that a cure is so very much needed because in just a matter of days, or weeks, your lungs can have a FEV1 of 114% and drop to 77% just like that.

Tuesday, May 10, 2016

Discharged . . . Home!

After a very stressful and long morning, the tech finally showed up to do Olivia's PFT's yesterday afternoon. We were originally told 9:30 am, then that it'd be closer to 11:30, then that we were first inpatients to do the PFT's . . . but it was closer to 12:30 and we were told, "it'll be a little longer while they help out your neighbor first", . . By this point, Jeff and I were both about ready to throw a chair through the window. As is the title of one of the Piggie and Gerald books that Olivia reads . . . "Waiting is not easy".

Definitely not easy, especially when we had waited and waited and waited to get the heck out of there. All we wanted to do since we got there was go home. After the pft's Olivia had done last Thursday with some increase in her FEV1 and no change in the plan of care. we knew we could do the increased RT therapies at home as well as finish out the iv antibiotics there. Home is so much better than the hospital. 

So finally when it was Olivia's turn to do the PFT's. we were all ready. Her first few attempts weren't that great, she was getting FEV1 values of 85%, just 1% better than she had blew last Thursday. Jeff and I both looked at each other in a bit of panic. But if you know my engineer programmed husband, you know he is brilliant and is always working things out in his head, usually at least 5 steps ahead of me in his thought process at any given time. At this point he politely looked at the data as the tech explained to him that which he already knew and I chimed in as we all agreed that this test isn't error proof and that it is highly affected by user compliance and that Olivia needed to be given a bit more instruction and a few more tries. After about 7 attempts, the numbers were increasing, she was blowing values in the 90's and when her FEV1 hit 99% and the data looked accurate, we called it good. The tech was on her way out and Jeff and I rejoiced and immediately began packing up. 

(*Side note, the fact that the pft's can go from 85% to 99% in a single session and if the child is only give 3 attempts, we'd still be hospitalized, doesn't sit well with me. This clearly proves that this test isn't 100% accurate and while I agree that it is very useful and it's what we've got, I do not agree that this is the best measure of lung health. I have a whole soap box issue on this, I'll share that in another post )

We were finally driving out of the parking ramp for the last time this hospital stay yesterday afternoon at 2:30. As it goes, the weather was gorgeous while we were in the hospital and crummy and rainy when we left but regardless, it was the most wonderful feeling to be leaving yesterday with Olivia in tow. 

We got home a little after 4:00. Olivia was so excited to be home and see her Kitty. Jeff and I were in a bustle to unpack, put away and clean up as the home nurse was coming with the supplies and training to do the iv antibiotics at home. She showed up at 4:30, we were busy with her until 6:00. After that it was supper, showers, iv antibiotic, g-tube feed, sleep for an hour, then up again for the last RT treatment of the day and then off to bed for all. Sleeping in my own bed last night, was pure bliss. 

There is so much more to share about our hospital stay, a memorable Mother's Day in the hospital, pictures and process for the PICC line, etc. etc. . . I'll be posting lots of updates in the coming days, for one so that I don't forget all that I want to know and remember and also to share with other cf mom's out there what I've learned, what our process is and all that. 

But for today, I'm going to relish in the bliss of being home, drinking a "fancy" coffee from my new keurig that the kids got me for Mother's day, all while hanging out with my sweet girl. I also need to put on my "teaching" hat today to catch her up on some of the school work she's missed.

More to come, but that's all for now. Thanks again to all for your love, support and prayers as we trudged through this uncharted territory that is cf exacerbation. 

Thursday, May 5, 2016

PFT Results

Olivia had her pft's this morning around 10:30. While her FEV1 value is going in the right direction, it only increased from 77 on Monday to 84 today. She gave it her all and we know this is as good as her lungs can do right now but it was still heart breaking. She wants to go home and we want to go home and despite our best efforts to advocate that we should be able to go home and continue her iv antibiotics through her picc line in the comforts of home, the team of doctors would not go for it. Jeff and I both understand their points and reasoning for wanting us to stay but we finally had to agree to disagree that this was not the best course, especially given that nothing is changing. Increased vest/neb therapy and the once daily iv antibiotic dose through her picc line and that's it, all which we can do at home since I took the required class this morning to learn how to administer her antibiotics through her picc line.

If you know us, you know we are very diligent in Olivia's care. There is not one thing we wouldn't do for Olivia and her health is always our number one priority. If needed, we get up in the middle of the night to do her treatments in times of illness. We've successfully kept her as healthy as possible for 6 years and out of the hospital for a cf exacerbation in that amount of time. We have to be one of the most germ conscious family's around and the thought of remaining any longer in a hospital, the ideal place to pick up other bacteria(s) and/or viruses, just doesn't sit well with us. Not to mention, that even if the hospital overlooked the most beautiful scenery in the world, it still plays heavily on one's psychological state. The U of MN Children's hospital is great and they've made it as awesome as they can but it's still a hospital, you still feel trapped, isolated, alone and just being there and being in a place where people go when they are really, really sick, plays on ones mind and makes you feel off and not good. As I told the team of docs today, I don't think you'll get an "I'm good" out of Olivia until she's not here any longer.

So, with that, not what I had hoped, but we will stay in the hospital through Monday.

Definitely not what we had hoped for at all and once again I feel it's the whole robbing Peter to pay Paul situation. I'm a mom . . . to 2 children. My little girl is in the hospital, my son was back at home with my sister. We have to keep some sort of normalcy for Landen and school is still in session. For as much as we'd like to just pull him out of school and keep him with us, that's not feasible for a week to 2 weeks at a time. So last night Jeff and Landen left the hospital to head back home, get more things we needed for the stay and get Landen to school. It was very quiet and lonely once Olivia fell asleep and my boys were gone. Tonight it absolutely broke my heart and I couldn't hold back the tears when it was my turn to leave for the night to go be with Landen, do laundry etc., and Jeff stayed the night with Olivia.

My intent was to not leave her side until we walked out of that hospital hand in hand together, which I had unrealistically hoped would be today. Since that was not the case, it's not fair for Jeff to have to do the leaving all the time and for as much as I love that she usually asks for mommy for most things, I want her to also know that daddy is just as good, if not better at all that stuff too. Daddy can walk her back to surgery until she falls asleep, Daddy can be with her overnight while Mommy takes care of Landen . . . neither Jeff nor I can be in two places at once, neither one of us wants to have to choose to be with one child and not the other, but just as with most things cf related, you aren't given that choice. CF definitely is a disease that affects the entire family unit, that is for certain.

All in all, Landen is handling it well. He was very sad to leave Olivia and I on Wednesday night but he was much happier tonight when I got home and I'm sure it helps that tomorrow is Friday, the weekend is coming and that he and I will head down to the hospital to cram into one hospital room for the weekend so that we can all be together again. Olivia can't wait to have Landen back and he's just as excited to see her too and for as walking on top of each other as it kind of is in that hospital room, I'd have it no other way.

Our weekend will be much of the same with no changes in Olivia's plan of care anticipated until after Olivia completes her pft's on Monday. I'll of course post more updates when or if I have them.

Thank you all for your thoughts and prayers and a very Happy Mother's Day to my mom and to all the wonderful mom's I know.

Wednesday, May 4, 2016

PICC line placed

Olivia slept pretty well last night. Jeff and Landen decided to stay one more night so the 4 of us were all together again, makes for cramped sleeping quarters but it's always better when we can all be together as a family.

Both kids slept until about 7:00. After vitals and wrapping to waterproof Olivia's iv, she had a quick shower and then was wheeled off to sedation area. The picc line placement was scheduled for 9:30. Olivia was pretty worked up as we anticipated but we had a wonderful child life specialist come in. She came with a horse for Olivia and a colorful teddy bear for Landen and they both got to practice placing their own picc lines on their new buddy's and that seemed to help quite a bit. The operating room was held up because of an icu case that was taking longer than expected so Olivia wasn't wheeled back to have the line placed until almost 11:00. The waiting around beforehand was the hardest. She was given some versed in her iv line before she was wheeled away to help ease her fears. This time she said Daddy could go with her back to the operating room until she fell asleep right around 11:00 they were off.

The procedure was pretty quick and she was back to the room in under 45 minutes. She woke up pretty quickly and because this wasn't heavy sedation she was pretty happy when she woke up, hungry and quite funny, which was a super nice change given how she usually comes out of sedation.

She's super hungry, which is good so we are just waiting for the NPO order to be lifted so we can get our girl some lunch and get on with our day. We missed rounds this morning but I'm not assuming there to be much change in way of the plan to continue on course, iv antibiotics again tonight, lots of chest/neb therapy and then pft's tomorrow. We should get a better feel tomorrow after pft's on when we may get to bust out of here.

Tuesday, May 3, 2016

Hospital Update Day 2

Is there such a thing as actually being able to sleep in the hospital? I don't think Jeff slept a wink, literally. Landen slept on the "bed", I attempted to sleep in the chair and did get a bit of rest and Olivia slept okay. I don't think she slept very soundly as she'd wake up and mumble or what not but all in all, it could have been worse, at least we were all together for the first night.

Olivia and Landen were up this morning a bit after 6:00. Olivia was again nervous and worked up but was able to not vomit this morning, however she wouldn't eat anything either. Thankfully, we were able to run her g-tube feed last night so she has something in her. Her first respiratory vest and nebulizer therapy was this morning around 8:30. During this therapy she was able to run at her full program that she does at home so that was a good start. The team came in around 10:00 for rounds and went over the plan.

We are here because Olivia's pft's dropped from her baseline of 114 down to 77. She was started on iv antibiotics last night and these will continue for 2 weeks. She will have a picc line placed tomorrow morning to receive the iv antibiotics for the full 2 weeks. She will repeat her pft's on Thursday and that will help us determine the length of this stay in the hospital.

If her numbers are back up toward that 114 mark, we could potentially head home toward the end of the week or weekend and finish out the duration of the iv antibiotics at home. If her numbers are not were they need to be or are not showing much sign of improvement and she needs to continue the 4 times/day vest and neb treatments we will remain inpatient. So we won't know a whole lot else until after pft's on Thursday.

I think the mindset of being in the hospital has played heavily on Olivia. She was so worked up yesterday and it was hard to tell if she didn't really feel that good, if she was just nervous or if she felt like she was very "sick" being confined to the hospital room. Today midmorning we took her outside to the park they have on site and that helped tremendously. She was able to play and run and realize that even though she is a very sick little girl right now, she isn't confined to the hospital bed. Since then her attitude has improved and she was able to eat some lunch.

We are continuing the added treatments, her iv line had to be taken out because it was leaking so that was a bit anxiety inducing which we will have to have replaced tonight as we couldn't move up the picc line placement. So tonight we will have an iv placed to administer her iv antibiotics and then tomorrow morning her picc line will be placed and Thursday will be pft's.

That's the plan for now, I'll update as I know more.

Thank you to all for the prayers and to Grandma & Grandpa Oltmans, Aunt Jennie, Aunt Em and families, Grandma Dianne and Aunt Amber for the get well wishes and gifts. Thank you to Mrs. Heath for the phone call and sweet, sweet, message, you are adored. A huge thanks to Darci and Braelyn for picking up the pieces and taking care of Shelby and the house and getting everything we needed . . . we so appreciate all of you and the love and support you provide.

Admitted to Hospital

Sunday night Olivia was riding dirt bike. Monday she went to school. Monday afternoon at 3 I brought her down to CF clinic for a pre op visit for her ENT surgery. Monday at 4:30 we were admitted to the hospital. 3 weeks ago when I brought Olivia in for a check up because of the cough that wouldn't go away she had the best pft numbers she's ever blown, her FEV1 was 116%. Today her FEV1 was 77%.

This is a very significant decrease and definite cause for alarm.  A chest X ray was ordered.  It showed a clear picture on her lower right lung where there is a mucus plug or pneumonia or something. It could also mean that she has some nasty bacteria, like pseudo growing that just isn't showing up on her sputum cultures.

So here we are on the CF floor over at the U of MN. This is the first time Olivia has been admitted for an exacerbation. While we were applauded for that it still breaks my heart. Today we were definitely thrown a curve ball. On one hand I'm not too surprised. The cough wouldn't go away, I sensed something was up and Olivia just hasn't been herself. But the fact that she gained 3 lbs., and her sinuses have cleared up and while the cough is still lingering it has lessened, I didn't see this coming.

That's cf isn't it?  The silent killer. Wreaking havoc inside while looking healthy on the outside. I also know that Olivia knows more about cf than we often think. I also think that she on some level feels her cf can be a burden and I honestly believe that if something did hurt she wouldn't necessarily tell me. She doesn't want to be in the hospital,  she doesn't want to miss school or gymnastics. Today when we got here she looked at me with a face ridden in fear and asked me if she was going to die . . .
Some days cf stinks. I know enough about cf to know this is reality.

Tonight she was started on iv antibiotics and increased respiratory therapy. On Wednesday she will have a picc line placed under sedation so that we can continue iv antibiotics at home. On Wednesday or Thursday she will redo pft tests to see if her lung function is increasing so we won't know more until then. She was also started on zonfran to help lessen the anxiety induced vomiting so we do not lose ground in the weight category.

I'll post more as updates become available.  In the meantime prayers for recovery are welcome.