Ruby had her first day of kindergarten yesterday. She was so excited until we got to the door, then I suddenly had a growth on my leg. We did finally get her detached but I left with her crying. When I picked her up from school I asked how it went and she said, "I was a little sad when you left me there, and I had water coming out of my eyes." So sad, but she did great. Today was not much better, her teacher had to take her from me again. I think I won't be walking her in anymore. We are super excited about her teacher this year, first of all Lucy (Ruby's big sister) had Mrs. Reiber in kindergarten and we loved her, but second of all her son has Cystic Fibrosis. It's so nice to have someone that is comfortable with Ruby's disease and will be aware of her needs. (and YEAH to almost 3 hours a day without children)

Ruby had a follow-up visit at Primary Children's on Monday and it went great. She was the best patient. She also has grown 2 inches and 4 lbs since her last visit. INCREDIBLE! We think it probably has something to do with her finally being off all steroids. Pretty amazing. The greatest part of the appointment was, since Ruby does all her treatments via mouth piece now, Dr Chatfield said we could try to use the faster nebulizer, it will do her medication over 2 minutes instead of 30 minutes. IT WILL CHANGE OUR LIVES! We have to wait for the pharmacy to get it to us and then go up to Primary's to be educated about how to use it, but I'm super excited. The funniest part of the appointment was when Lab came in to draw blood. She normally needs to held down, but when the Lab tech asked if she needed another person, Ruby informed her that she is really brave. While the lab tech was looking for a spot to draw from, the nurse came in to give me some prescriptions and as I looked over, Ruby is holding her breath and closing her eyes while the Lab tech drew blood. Ruby didn't move or cry or even make a sound. Have I mentioned that she is my hero? I just love her. She is such a great kid.

DADDDYYYY!

We had a great Memorial day weekend. Ruby's daddy was able to come home for a couple days, but the kids didn't know he was coming home. Her dad had told her he was coming, but not when, and he told her that it was a secret. Ruby didn't tell anyone her was coming home. I'm not sure if that is good or not that she is so good at keeping secrets. It was so great to have him home for a couple days.

Nevermind

Ruby had her second CF clinic visit this week. It was not as good as her last visit, although she was a great patient. She lost weight, almost 2 lbs, she had a couple new medications added to her regimen (because really the 9 she was on before were getting way too easy), so now we are up to 11 and a vest treatment. For my nurse friends, I really need to have a MAR printed up daily and signed off. They were concerned about a possible diabetes diagnosis in addition to CF, however after checking her blood sugars for a couple days, they seem to be normal. PHEW! That might have thrown me over the edge. Her pulmonary function tests (PFT's) were not as good, but she has a cold so they didn't seem too concerned. She has to have sputum culture done each time we go and last time she couldn't cough anything up so they just did a swab and the culture came back negative for psuedomonas. So over the last month we have been practicing "expectorating." We are really lady like at our house. So they were super impressed when my cute little 4 year old could "hock a loogie." Unfortunately, the culture of her "loogie" has come back positive for psuedomonas again, which is what I was expecting. Oh well, this is where my attitude of "plan for the worst and be happy if that doesn't happen," works out for me.
Her pulmonologist talked to me about getting her to swallow her pills whole, she wasn't liking the way I was having her take her enzymes(oops). So she gave me idea to have her practice just swallowing 1/2 the capsule without the medicine first, then the empty whole capsule, and then the capsule with the medicine. She said just have it be her idea and work on it slowly over the next month. So when we got home I thought I would have her try. She had freaked out so much at the beginning that I didn't think she would even try, but she did and was able to swallow the empty capsule the first try and then immediately did the entire pill. LOOK WHO CAN SWALLOW PILLS! WooHOO! She can even swallow her GIANT vitamins. She's pretty amazing. This picture is of Ruby and her new addiction, LaLaLoopsy's- Blossom Flower Pot, Patch Treasure Chest, and Mittens Fluff and Stuff. (Oh, did I say her addiction, it might be mine)

Sayonara Pseudomonas... SWEET!

We had our first visit to the Cystic Fibrosis Clinic at Primary Children's last week. It went really well. Ruby is growing so fast! It's truly unbelievable. I can't believe she is the same kid. She weighed 33 lbs when she got admitted to the hospital and weighed 40 lbs at her visit this week. She is in the 73% for her BMI, they want her between 50-75%, so pretty much perfect. She has no cough, and for those that know her, again unbelievable. I don't remember a time since she was born that she has coughed. She feels so much better, has SOOOO much energy. She still is not in love with her new life, she doesn't like doing the vest treatment or the countless nebulizers, and she especially doesn't like flonase spray up her little nose, but she does it everyday. She does think I'm the meanest mom and really wants her dad. (Little does she know, I'm the nice patient one -Love you Honey!) For the most part, things are going so smoothly, I feel so blessed to have caught this when we did and for having such great Dr.s.

She had her sputum cultured and had been growing pseudomonas in the hospital, (NASTY bacteria, common in CF patients) this meant possibly a lifetime of inhaled antibiotic nebulizers (TOBI), every other month. It adds an extra 60-80 minutes of nebulizers a day when she is on it. We were on it last month and are currently off for a month (SO NICE!). So, her culture from our clinic visit came back negative this time, she will be cultured again next month at her clinic visit and will for sure do one more round of TOBI in May, but they are optimistic that they got rid of the bacteria. So we are praying for clear cultures.

We came home from the hospital on Feb 23, we were in the hospital a total of 21 days. Ruby was most excited about getting her PICC out, until she realized that the tape would have to come off to get the PICC out. Luckily the nurse let Ruby take the tape off this time. She was SO SLOW, but he was very patient and let her take her time. She did a great job! The last day was a little frustrating. A lot of confusion concerning her medications, 3 hours of waiting due the mix ups. I finally just said I'm leaving, we were still missing two of her medications and didn't have the right amount of another one. I knew I could get a dr to call them into a pharmacy for me. They weren't too happy about me just leaving, but I had home health meeting me to teach me how to use the home equipment and finally left around 2:00 pm. It took me three wagon trips to get all our stuff out. It was so nice to leave!

So we have been home almost two weeks and things are going well. As I have mentioned Ruby is not in love with her treatments. The morning treatment takes about 1-1.5 hours and the night treatment takes about an hour. Sometimes it's a battle the entire time, other times she will just make comments like "I really wish I didn't have to do the shaky treatment." ME TOO!

I can't believe how much better she is doing though. We went for a follow up visit at her pediatrician's and she had put on 6 lbs since her last visit. When she only weighed 30 something lbs, that is impressive weight gain. She is finally putting on some weight. I sent her daddy a picture the other day by text and he had to ask if it was Ruby, he couldn't believe how much she had changed since he left. Every night she comes in my room after I've put her to bed and asks, "Can I have some nachos?" That might be contributing to her weight gain, but it is perfect for her high calorie, high fat, high salt diet. She used to cough to the point of throwing up almost every morning and evening. Now I hear her cough during her treatment sometimes. AMAZING!

She is happy to be back at school and playing with friends. Really I just want to keep her home in a little bubble so that we don't have to go back to the hospital, but obviously that isn't very realistic. We went back to church last week and she was not in love with staying the whole time. I've been taking her home when we separate for classes since before Christmas because her cough had been so bad and she'd been so sick. So when she had to go to her class she was not happy, she asked if I would take her home and when I said no, she said "how bout Annie?" She keeps joining me in my class. I'm not used to having a mama's girl. She's never been like that before.

Thanks again to everyone for their help and prayers. We have truly felt blessed through this whole experience.

Ruby: If I don't have my cough anymore, do I not have to do my treatments? Me: No, kids with cystic fibrosis have to do treatments every morning and every night even when you don't have a cough. Ruby: Oh well, I don't have cystic fibrosis, I think I have asthma again.

Still not loving doing treatments. She is doing it though. I have only had to physically fight her a couple times since we have gotten home.

GOING HOME.....soon

So they are saying we get to go home tomorrow. Although they just said this morning that they want to check glucoses for 48 hours, do they know that would end Thursday? I'm not telling them. Ruby's vest system is at the house, we are just waiting for her pari compressor to do her nebulizer treatments and we are set to do this at home. We are so excited to get out of the hospital. I asked Ruby what she will miss at the hospital and she said, "Steak," then licked her lips. Ruby has had over 40 steaks since we have been at the hospital. She has had it for breakfast, lunch and dinner every single day since we got to primary's. The only changes she makes is what fruit or vegetable she has with it, although if it were her choice it would be broccoli every time. But we are in isolation and I couldn't take it, broccoli three times a day, everyday, not good! She has put on about 2 lbs since we've been here though and that is good.

We have been treated so well here, we have had great nurses, doctors and respiratory therapists. Our poor respiratory therapists have had their work cut out for them, especially Shontae, Ruby bit her. Our favorite therapist was Jaynie, she let Ruby's pillow pet try out the vest too. It made such a difference to have therapists that work with her and give her as many choices as we can vs the therapist that fight with her to have it their way.

Ruby has loved all the gifts she has received and has played non stop with squinkies, zoobies, pollys, pet shops, stuffed animals, pillow pet, stickers, ponys, playdough, bubbles, puzzles and has drawn, cut, glued, crafted, and colored enough for a life time. Thanks to everyone that gave her stuff to play with, not sure how we would have made it 21 days without them. She hasn't been bored at all the entire time. She even made herself a little fort to hide in under a counter. Really, I think I have been so blessed, she has been so great.

Something Ruby will not miss is pokes. My little girl that used to not even cry for shots and her first couple IV's, is a bit over the top now, but I can't really blame her, she has been poked a lot in the last 3 weeks. What she hates the most though are her PICC dressing changes. She will have that pulled before we go home on Wednesday, only one more crack down smack down. So happy I won't have to do those at home.

So we are excited to get home and Ruby can't wait to see her friends and get back to school. We are both excited to see Annie and Lucy and sleep in our own beds. Now to get used to our new normal life. We are going to try to have the attitude of "Come what may and love it." Better to laugh than cry, right?

Today respiratory therapy came to teach me about what I will be doing with Ruby once we go home. YOWZERS, is all I have to say. She told me she would be here for 1.5 hours and our teaching lasted 3 hours. I was a little frazzled by the time she left and all I can say is I felt overwhelmed. First she taught about the inhaler (luckily she's been on that forever), she will be on flovent and albuterol, 2 puffs twice a day. She taught about using the spacer, cleaning the equipment, teaching Ruby how to use the mouthpiece vs the mask. Next she taught about all of her medications she will be receiving through a nebulizer. She'll be on hypertonic saline 2x day, pulmozyme 1x day, tobi (inhaled antibiotic) 2x day. Each of these take 10-25 minutes to do. They have to be done in a specific order. None of the medicine can be given together or even poured in the same device, so I have to have three different nebulizer devices for each treatment. All the nebulizers need to be sterilized between treatments.

They discussed the vest systems and made me decide on which one we want at home. We tried all three systems. I feel like I'm buying a car. It's a huge decision, they are expensive and the one I choose will be the one she has to use everyday. What if she hates the one I choose? I hated making that decision on my own. There should be a panel of other CF families telling why they like and don't like certain systems. Then they also taught me how to do CPT (chest percussive/physical therapy). That was super fun. I'm feeling very lucky to have a child born with this disease in this era where we don't have to do that very often. They just wanted me to know just in case the power goes out or our machine breaks. She also taught me the breathing techniques Ruby needs to do 5x during each vest treatment, these are done to move mucus from smaller airways to larger airways and then out.

Anyway, not very interesting, but I'm feeling overwhelmed as I said. Ruby keeps saying I want to go home and I remind her we still have to do all this there and then she cries saying she doesn't want to do this everyday.

Child life (a great program at the hospital) comes in almost everyday to help her deal with things or just to hang out and do projects. On Monday they came in because she hates having dressing changes on her PICC. They had her put a PICC in her baby and then do a dressing change. She did an excellent job btw, I was impressed with the how intently she had watched her own. She new the right order, how to tape it, she flushed it. It was adorable and had to admit I was a little proud. They came yesterday to help me figure out a way to help treatments go more smoothly (it's WWIII sometimes). She brought in a Little Pet Shop house for her to take home with her and we decided she can only play with that during treatments. We've tried to let her do fun things during and after treatments to make them more fun. Today was a lot better, we still struggled by the fourth treatment, but progress was made. Her favorite thing to do is blow gloves up with the oxygen. Not just make a balloon, but actually blow them up. She loves it! We also made a little fort under a shelf where she can hang out during the treatment, she loves that none of us can fit down there. She's doing much better, she sounds much better, still coughing LOTS, and it still looks like we have another week here.

Well today was a good day, Annie and Lucy's test came back in the normal range. Ruby has figured out how to take her enzymes. We had a great nurse Corey that gave us the idea to pour the "sprinkles" into apple juice and then Ruby gets to vacuum them up with a straw. She loved it and no more fights (today) on taking her enzymes. We have new battle. She is so smart and strong willed, eventually those will become very positive qualities, but today they are not my favorite. She has vest treatments every 4 hours and she has to practice a breathing technique which we call "HUFFS." Well these "huffs" are the only thing we can't actually force her to do. She has realized this and sometimes she's not in the mood and then a 30 min treatment becomes a 90 min treatment. Between her attitude and my lack of sleep sometimes we clash. This morning after battling for 30 minutes for her to take 6 breaths, I finally said to the therapist, I think I'm making this worse, I'm going to leave. I took a little break and came back 15 minutes later and the nurse told me it still wasn't going well and I should take a longer break. The whole treatment took 90 minutes. It has go to be so frustrating for her to have to do so many things she doesn't want to do and at four she really doesn't understand why it is important. I need to pick my battles, I just wish I didn't have so many battles to choose from.

Relieved

I've been asked a couple times by nurses, doctors, and friends, if I'm just relieved to finally know. At first I thought that was an odd question, relief was not how I was feeling. But now that shock is wearing off and reality is setting in, I think I am. I have felt like we were missing something on her for as long as I could remember. So although I would have never wished for this solution, I am amazed at the progress she has made in 2 days of correct medicine and correct treatments. She slept through the night last night without oxygen. I actually slept 4 hours in a row last night. Unbelievable.

Yesterday we battled a lot over take her enzymes, she's calling them sprinkles. She hates them and she can't eat anything without taking them first. We tried them in applesauce, gagged them back up, we tried to learn how to swallow a pill (in pudding, in a straw, by itself, not quite there yet), we finally put the sprinkles in pudding (this seems like the easiest option, although she still hates it). Yesterday was a battle of wills. She choose to kick her tray (glass cup shattering on the ground, I doubt they'll send up glass for her again), she chose to not eat lunch, she chose not to have a snack before bed. I chose not to beat her up. Hehehe. As I was eating a cookie late last night with my dinner at 10:00 pm (Thanks Steph for the salad, delicious), she said, "It's not fair. You don't have to take those sprinkle things before you eat. Daddy doesn't, Annie doesn't, Lucy doesn't, only I do, it's not fair." Boy is she right.

Feeling Better

Well on the bright side Ruby Duby is starting to feel better. On the not so bright side, she is fighting me on EVERYTHING! She doesn't want to do her treatments or take her medicine, or a bathe, or eat. It's been a very tiring day. She's been playing with all her new toys and art supplies. She's being spoiled rotten here, but that is the only thing keeping her sane right now since she isn't allowed to leave her room. We can do it. Only two weeks right? I sure hope so!

Ketamine, ahhhhh

Today Ruby had a PICC placed. They did such a great job sedating her and slammed the PICC in with one poke. THEY ARE AWESOME HERE! She was so adorable coming out of sedation. She looked at me and slowly said, "Mama, my eyes aren't working." I asked what was wrong and she said, "There are two mommies." I told her to go to sleep and one would go a way. So when she woke up 30 minutes later she said, "Phew, only one mommy." LOVE that girl! It's been a good day. Lots of information and education about her treatment. She's taking enzymes any time she is going to eat something. She's taking lots of inhaled meds (pulmazyme, flovent, albuterol) and a nasal spray (which she hates!) A multivitamin for her deficiencies. 2 strong antibiotics, to fight her pseudomonas infection in her lungs, and Lactobacillus because she is on some killer antibiotics. They do her vest treatments 4 times a day, which involve a vest shaking her chest for 30 minutes with breathing exercises in between. She's going to have ab's of steal. She will not be getting the squishy and juicy tummy she has always dreamed of. Yet through all of this she smiles and says things like, "Mama, I forgot to tell you, I just really love you. You are the best mama I ever met."
We met the Pulmonologist over the Cystic Fibrosis clinic. She was wonderful and super informative. She had been consulting on her when Ruby was still at the hospital in AF and was incredibly surprised when her results came back positive. She was impressed she had never been hospitalized before this and how great her growth has been. She helped me feel more comfortable about the long road ahead.
We had lots of visitors today and Ruby feels like it's Christmas all over again. Thanks to everyone that has sent things over for her to do. She loves it and it's making entertaining her much easier. The shock is settling in. Someone asked me to rate today on a scale of 1-10, yesterday was a 1, today is maybe a 3.

Diagnosis

Ruby Jane has been in the hospital for 7 days now. She was originally admitted for asthma because she was requiring oxygen to keep her oxygen saturation above 90%. (She was diagnosed with asthma as a baby) We figured she would be in the hospital a day or two, wean off the oxygen and go home, possibly even go home with oxygen. While there everyone was confused by her diagnosis of asthma because she coughs and doesn't wheeze. On day 5 when she still required 2 liter of oxygen to maintain her sats, her pediatrician decided maybe we should rule out some other options. They did another x-ray to make sure it wasn't pneumonia- it wasn't that. They did a heart echo to make sure there was no problem with the heart- it wasn't that. Then the doctor suggested a sweat chloride test to rule out cystic fibrosis. We would need to go to Primary Children's to run the test and my pediatrician wanted me to get in with a pulmonologist anyway and suggested we get transported up to Salt Lake. So on Tuesday, Ruby was transported by ambulance up to Primary Children's Hospital. We didn't get here until late in the afternoon, so we couldn't do the test that day. So early Wednesday morning they ran the sweat chloride test. The test was simple, they put gauze on the inside of the elbow then wrap it in saran wrap, then we warmed Ruby up with blankets and warmers for 30 minutes, then they remove the gauze and test it for how much chloride was in her sweat. Unfortunately, although it is an easy test we waited for 8 hours to get the results. When the doctor walked in with the results, suddenly 8 hours was way too fast. Now if I'm recalling correctly (I've received a lot of information today) a normal range is 40-60, so above 60 raises concerns. Ruby's results were 109 and 112 (each arm). So, Ruby's diagnosis has changed from asthma to cystic fibrosis. Not our best day. They are going to do the test again on Friday to double check. He told us to expect to be inpatient for at least 2 more weeks, they will do what is called a "Clean Out." It sounds less than fun, but Ruby has thus far been such a great little patient and constantly amazes me with her patience and bravery. I am feeling so blessed to have such great friends and family that have been helping my little family the last week and especially feel blessed to get to spend so much time with Ruby Duby.