Breathing treatments begin!

We just started Kelsie's first breathing treatment last night, hypertonic saline.  Her doctor has talked about doing it for a while as a preventative measure but since she never had any respiratory symptoms, there was never any real indication to start it. Then she got that illness with the cough that never wanted to go away, so she felt it was a good time to start.

The first one was kind of rough. For both of us. She didn't like us putting that mask up to her face and I didn't like the fact that I had to. I felt full of tears inside thinking "this is permanent." I give breathing treatments to my patients and their parents have to give them at home too, but for them, its temporary or just when needed. For Kelsie, we have to do this EVERY DAY. SHE is going to have to do this every day! Its both a blessing and a curse. I'm so thankful this exists and that it will hopefully prevent her from getting as many lung infections and untimately prolong her life. But it still makes me sad that I have to make her do this every day. I will not waiver though. It too, like everything else will become part of our daily routine. We will figure out how to add this piece into our lives, embrace it, and thank God we have it available to keep our little girl healthy!

Kelsie got a baby doll after her first treatment. We told her she would get a prize if she did it so I had to follow through. I had a baby doll I was going to give her for Christmas but decided to return...needless to say, I decided to go ahead and give it to her as her reward for doing the breathing treatment. I guess we will need to scale down the prize value if this is going to be a daily routine!! :)

Weight check

I had to go to the hospital yesterday to pick up Kelsie's nebulizer for her new breathing treatments so I just took her with me and put her on the scale to see if she had gained any weight. And to my surprise, she has gained almost a full pound in just 1 week! (And she has been pooping like crazy so she had to have lost a lot of that extra weight during the past week as well.) She weighed 27lbs 6oz!

It was really cold and windy too so I had her all bundled up and had to snap a few pictures of her in her wool coat! (Which I got at a consignment sale for $3!)

Learning new words

Kelsie makes me laugh everyday with the funny things she tries to say. Her language really started coming out over the summer and she has been gaining new words every day. She will also repeat just about anything.

Lately we hear alot of:
Mine!
stop ("dop", "bahp" or other variations)
arn-doof (orange juice) - We hear arn-doof or just "doof" every night after she goes to bed and every morning when she is ready to get up! And she yells it.
Yay! (when she is excited or proud of something)
Woowww!
NO-NO!
toys
walk (she will come up and grab your hand and say "walk", this means she wants you to come play with her)
baby (or just "bay")
Elmo
"Beht-Beat" (Fresh Beat) - her favorite TV show. This is our go to for distractions during chest PT or mealtime or to calm her down if we are out and she is fussy.
"Moan" for phone
Mommy and Daddy of course!

Other words: Mommy, Daddy,  "Keh-sie" (Kelsie), apa (grandpa), mehma (grandma), "Valulwee", "Dew", Nana, papaw, wahwah (Mamaw Roberts), Will, Ava, Keh-kie (Kelly), eat, doggie,  apple, pear, hairbow, ball, book, baby, Alright (or just right), now, tree, star, eyes, nose, arm, hand, feet, toes, beh-ba(belly button), "bupp" (her bottom), hair, ears, eyebrow, throat, teeth, neck, earrings, what?, fit (when she is playing with blocks or shapes), "Kah-ket" (chocolate), bread, bird, "fovers" (covers), mess, wash, bath, "fues" (shoes), boots, shirt, pants, socks, chair, light, poop, peepee, hat, cold, hot, seat, car, off, on, stairs, march, "te-te" (TV), Tarheels, Ramses, ah-pack (wolfpack), bed, night-night, pizza, pickle, cheese, milk, fall, fell, dizzy, help, toys, Ah-Clause (Santa Clause), Reindeer, stocking, tirsty (thirsty), oh-k!, Jesus, angel, shy, house, flowers, more, brush (toothbrush), oopsie, wiggle, blocks, wet, food, pictures, watch, cook, duwl-dee (dirty), moon, macaroni, Max and Ruby (mat and ru-ree), spoon, fork, too, heavy, bye, c'mon, ummmm (when she is thinking about something), num-num, (yum-yum), nummy (yummy), vest, broke, laugh, trash...and I'm sure there are more.

She is starting to try to put together words but doesnt have many 2 word phrases yet: help me and all gone are the only 2 I can think of off the top of my head.

She tries to count but its always the same: "two...nine..." :) (no idea why she picked that up)

When she falls or hits something and gets hurt, she will go back to it and yell "NO NO" like she is getting on to it. I started that...I did the same thing when trying to console her one time after she fell.

Christmas card

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Follow-up

We went back today for Kelsie's follow up appointment. She has been doing well and her cough has essentially gone away. The cultures did not grow any bacteria or viruses so NO INFECTION in the lungs. Very happy about that! So we are now able to stop the antibiotic.

Her weight was up slightly from last week - like 2 oz - but it is still down from where she was 2 months ago so we are going to have to work hard to get that back up. She weighed 26 lbs 9oz today and was 26lbs 13oz on Oct 13th. She's still at or slightly above the 50th% weight for height and they like to keep their CF kids from falling below the 50th%. Kelsie is still eating really poorly but we think that might be coming from constipation so we are going to ramp up the Miralax a little more and make sure we are getting everything cleaned out. We also had long discussions with the nutritionists about how to manage her meals/snacks, new ideas for getting her to eat, and implementing supplements such as Pediasure. She has hit a picky phase of eating which is typical for all toddlers, but with her, we have to more aggressive about it. The Pediasure will help fill in the gaps and give her 100% of what she needs in a meal. So if she goes a whole meal and refues to eat, we can rest a little easier knowing that she is getting the fat and protein she needs from the Pediasure. We are also going to keep trying things like distraction to get her to eat, adding more fat to her diet by adding butter and/or heavy cream where we can, and giving less juice (low calorie beverages) and more milk based drinks (even if that means giving an extra enzyme when her meal or snack dose has ran out).

We are also starting a new therapy called hypertonic saline. This is like really salty water that she will breathe in through a mask for 15 minutes twice a day during PT. they did a "challenge" to make sure she could tolerate it. She did ok in the beginning but only made it a couple minutes before she started pulling it off and fighting it. It causes the airways to spasm causing her to cough. So she has to take albuterol first to keep her airways open so her breathing won't be compromised by the spasms. The salty solution helps balance out the sodium chloride and water transfer which is the foundational problem of CF. This has been shown to be a beneficial preventative measure in people with CF by helping to clear secretions from the lungs. I don't think it took Kelsie long to realize that she doesn't like this new therapy. It causes the nose and throat to burn and makes you cough. Doesn't sound too comfortable to me! They say it will take some time for her to get used to it and we will have to slowly work up to our goal of 15 minutes twice a day.

HOME!

From yesterday:

What a whirlwind of a day! We went in this morning with so many questions and uncertainties. But are thankfully back home now.

Her PFTs (pulmonary function test) looked great, as good or better than the last! Which was great news. There did not appear to be any obstruction in her air movement. They also give Albuterol during that procedure to see if there is any improvement by opening up airways. There wasn't which is a good thing suggesting that things are working like they should at baseline. There was one number that was slightly worse, suggesting some air trapping, but was still within normal limits.

The bronchoscopy showed no secretions in her left lung but did find some white secretions in the right lung - upper lobe and lower lobe. There was not a significant amount and the secretions were not yellow or green, both good signs. They also found her airways to be very irritated and inflamed. There a few things that could be causing this. It could be reflux, could be viral, or could be something like asthma. They also looked in her nose and saw that her adenoids were really large.

The doctor said based on what they saw, she did not see any strong indication to proceed with the IV antibiotics today. But still gave us the option. She said the most aggressive approach would be to go ahead and do the PICC line, start the IV antibiotics and make sure we were getting a head start on it if it was a bacterial infection causing the secretions in her lungs - all with a hope to be done with it by Christmas. But the other option was to wait and see what the cultures show, knowing that if they do grow bacteria, we would have to go back in for the IV antibiotics - potentially putting us in the hospital over Christmas. We took the chance and decided not to put in the PICC line and admit her, thinking this is unlikely to be something that needs IV antibiotics. And if by chance it does grow something that requires strong IV antibiotics, then we will deal with whatever we have to do when the time comes, even if it is over Christmas. I just didn't want to put her through everything that goes along with a hospital admission without knowing it was absolutely necessary.

We are also adding some new treatments to help with the information they found during the bronch. So this is the plan from here:

Restart Zantac (medication for reflux) - we started this medication when Kelsie was only a month old but have tried stopping it several times. She also takes another medication for reflux but the Zantac was added as an adjunct. In the past, each time we have stopped the medication, I have noticed her to start coughing. We last stopped it in September, she then got sick with the cough the first of October. So we never had a chance to see how she would respond to being off the Zantac. The doctor also said they tend to see secretions in the right lung when a person has chronic reflux. So I really feel like this is the issue. Reflux alsos causes chronic inflammation in the airways, so thats where that could be coming from as well.

Restart Flonase (nasal steroid spray) and increase to twice a day - this helps with the large adenoids, inflammation in the nose and nasal congestion.

Start hypertonic saline  - we go back next week to do this "challenge". This is a breathing treatment and it helps her clear out secretions in her lungs. Some times kids do not tolerate well so they like to do a trial in the hospital setting in case she has problems with it. If she does fine, then we will start doing those treatments at home on a daily basis indefinitely.

Continue Bactrim - She still has a little over a week left on this antibiotic so we are going to continue doing that to clear up any infection that is left.

Start nutritional supplements - She had lost weight since her last appointment (about 1/2lb), which was concerning to all of us. Kids with CF are at an increased risk of nutritional deficiencies since they do not absorb fat/protein on their own. She has not been eating much over the past couple of months and has been really picky. Basically, she is eating like a 2 year old, but we can't let her do that. I refuse to get into food battles making her eat because I know that only makes them want to eat less. When you do that, mealtime becomes more of a battleground and less of an enjoyable experience. It is really important that she eats, and that she eats high fat/high protein meals and snacks. But at this stage, its hard to find many things that she will eat alot of. She loves cheese and would eat that at every meal if you let her. I don't like doing that because she still needs a variety of other foods, plus cheese is very constipating and that is another problem for her. Yes, we can treat it with Miralax, but she still has to eat something besides cheese! So for now, we are starting nutritional supplements like Pediasure and Boost. I am also going to look into some new ways to increase the fat content in her food, like using whole milk Greek yogurt for example. Its definitely time consuming not only to find some recipes, shop for the food, make it, and then try one thing after the next during mealtime before you find something she will eat. I think every mom with a toddler has frustrations trying to get their kid to eat, but mine are taken to a whole new level because her nutritional needs are so much higher than the average kid. So I HAVE to figure out how to get her to eat again!

Reassess next week -  We go back next Thursday and the doctor will go over her culture results, check up on the progress of her cough and we will go from there. Hopefully, things will be at least somewhat improved. We do not expect the cultures to grow anything significant but if they do, she will have to get the PICC line put in and be admitted to the hospital after all. But we will continue to pray that doesn't happen!

After coming home - Saturday 12/8
Kelsie is doing really well today. She was really tired after getting home yesterday and unsteady on her feet but otherwise seemed to be back to her usual self. She wanted to lie down a lot, and would snuggle with me on the couch which was unusual for her but expected given what she went through yesterday. She drank a lot of fluids after coming and ate a fairly good dinner last night. She had a fever but they told us to expect that. They wash out the airways with sterile salt water but some of it gets left behind so the body recognizes that as foreign and raises the temperature to try to take care of it. That extra fluid left behind in her lungs also causes a lot of coughing so we've definitely been hearing quite a bit of that. And she is a little extra tired today. Still happy, just sleeping a little more but that will be good for her.

This is in the PFT lab right before falling asleep for the test. They give her oral sedation medicine for this procedure. Its a lot of volume and yucky but I got it all in her. She then sat in my arms watching Fresh Beat Band on the iPad until she fell asleep. 

 

 This was after the bronchoscopy. It took her a long time to wake up from the anesthesia. She first came out of it coughing like crazy, gagging, and very agitated. I would kiss her and rock her and she would calm down, go back to sleep, and then that mucous would come up and gag her and she would get agitated again. She finally went back to a deep sleep and we had to get out a cold wet washcloth to wake her up. I was happy to have it all over with and enjoying the cuddle time! <3

 

Here we are heading home! Getting some juice and puffs after being NPO all day!



 

Finally back home! Some strawberry milk and peanut butter crackers. Mmmm.

Good to be home! 

 

 

Taking it easy with mommy! So glad we got to spend our evening like that and not in a hospital room! Thank you God!

What is necessary

When Kelsie was diagnosed with Cystic Fibrosis, I knew there would come a day where she would be sick. I knew there would likely be frequent respiratory infections and hospitalizations. But of course, I didn't know when to expect them. At first, we expected the worse. But little by little, those anxieties were put aside as we just enjoyed each day with our perfectly normal and healthy little girl. She has been exceptionally healthy and has only had 3 colds her whole life - which in my line of work in pediatric primary care, I know how rare that is. So I think we even started to think at some point, "maybe she is just going to be one of those CF kids who have little respiratory involvement." She literally NEVER coughed until this infection started 8 weeks ago. But now, here we are, her first cough and she is getting a bronch and facing possible admission. And she isn't even 2 yet. I still can't believe it. I know some kids with CF don't experience their first admission until they are teenagers. Others are infants. I always thought, and truly believed, that we would at least make it until school age before Kelsie got admitted. And we still don't know what is going to happen tomorrow, so I'm still going to hope and pray that happens!

I just hate putting my child through these scary procedures. She is too young to understand why we have to do this. I don't want her to hurt and I don't want her to feel scared. I feel so sad that she has to go through this and that I can't stop it. I have done everything I can to avoid this and I still don't know that I have made the right decision. I didn't want to overreact and make her go through all of this without knowing it was absolutely necessary. But I didn't want to blow it off either and risk a subtle infection causing damage to her lungs. I felt completely torn. But then when she woke up this morning coughing much worse than she has been, I knew it was time. I realized it was now necessary and I couldn't prolong it any longer. The realization hit me hard. That no matter how well I take care of her and do exactly like I am supposed to, and no matter how closely I monitor her every symptom, I just cannot prevent her getting sick. Its simply the nature of the disease. I have to accept it. I know its not that bad right now and in the grand scheme of things, things could be a lot worse. But its the first time we are having to go through this and its our first real confrontation with her disease. So its hard. Hard to accept and hard to deal with. But I will muster up the strength to make it through it and will do all I can to make sure it is the best experience for her it can possibly be. I will be right there with her through it all and will make it as fun as possible. We will make it through it regardless of what happens. God will give me the strength to be my best for her and He will protect her. When I can't be there during the procedure, He will be the One holding her hand. And I have to trust that He has a purpose in all of this.

First bronchoscopy

CF seems to be rearing its ugly head. Kelsie has now had a cough for 8 weeks now. It is also the first cough she has ever had, but she just can't seem to get over it. It started out with a fever and a couple of coughs here and there. That went on for about a week and then her doctor decided to start her on and antibiotic (Augmentin) for 20 days. She continued to cough the entire time and the cough even worsened during that time. We saw the doctor again about halfway through that course of antibiotics and she thought she looked great and just suspected that she was hit with 2 respiratory viruses back to back. She advised we continue with the antibiotic regimen and also started a nasal spray to help with any nasal congestion/allergic etiology. We did that for a couple of weeks, but the cough persisted. The amount has varied but there definitely hasn't been a day without cough since this all started.

So about a week and a half ago, I talked to her doctor about her peristant cough and she wanted to start a different antibiotic and talked about the possibility of doing a bronchoscopy and admitting her if the cough did not improve while taking the antibiotic. The cough has since been waxing and waning. Some days the cough is very minimal (maybe 1-2x), but other days it has been much more. The cough seemed to be better overall, especially in the quality (not as wet and junky). It was better over the weekend and I thought it was going away, but 2 days ago she coughed 12 times. Then only 7x yesterday. We talked to her doctor last night and she said she was still on the fence about whether or not to proceed with the bronch. After much discussion, we decided to hold off on the bronch, and do hyptertonic saline (an inhaled therapy) instead. This is like really salty water that she would breathe in through a breathing machine and it would help her cough out secretions. Her doctor and I both thought this was a good middle ground and a good option to avoid the more invasive procedures. We also thought it would give this cough more time to settle out if it were viral or the result of sinus drainage. However, this morning she woke up with a lot of cough again. She has probably coughed 25x today and thats the most I've heard in several weeks. :/ It also sounds junky again too and has almost gagged her a few times. I broke down a couple of times realizing that this bronchoscopy procedure is now inevitable and necessary to make sure infection is not growing in her lungs.

Prior to the bronch, she will have PFTs (pulmonary function test) which measures how well her lungs are working. She has had two of these before and she will get these routinely for the rest of her life. Since she is still too young to breathe on demand and show how hard she can breathe, they have to sedate her for this test, put her in a special box, force air in, and measure how well her lungs are doing. This isn't a bad test, its just the sedation that is the hardest part. So she will have this in the morning around 9am, then while she is still sedated, they will move her to the bronch lab where they will put her into an even deeper sleep, but she will still be breathing on her own.  During the bronchoscopy, they will go down and look into her airways for secretions. We know that kids with CF have more secretions and have a harder time getting rid of them. So this poses this risk of bacteria growing, causing pneumonia and inflammation of the airways.  So in kids with CF, it is sometimes necessary for them to go in and look closely at all the small spaces in the lower airways to make sure there isn't any infection.

If they do find secretions in her lower airways tomorrow, they will take samples and culture it to see exactly what type of bacteria it is growing. They will then go ahead and put in a PICC line(peripherally inserted central catheter), which is long catheter that is inserted in a vein in her arm and ends in a large vein near her heart. This will be there to allow her to get long term IV antibiotics if needed without having to worry about getting multiple IVs and doing damage to her veins. It ensures good IV access so that she can get the stronger IV antibiotics if the cultures show they are needed.

So at this point things are still up in the air. She will definitely get the PFTs and bronch in the morning. If both look good, they will send her home. If they find secretions they will take cultures, put in the PICC, then admit her, and start IV antibiotics until the cultures come back. And the course that follows will just depend on what they find. So please keep her in your prayers and lets all hope that her lungs will look clear and that this cough is just coming from some post-nasal drainage from a sinus infection or virus. Which is also a very real possibility since people with CF are also at a greater risk of sinus infections. They will also look at her sinuses while doing the bronch as well so we will know if thats the source of the inefction as well.