Our E.N.T. just published Vivs case in the American Journal of Otolaryngology. We have a more extensive version of the article, but you can see part of it here: http://www.amjoto.com/article/S0196-0709(14)00103-3/abstract
This last month has been the roughest in awhile as Vivs hasn't been the same since surgery part 2 to debulk tumors in her upper airway. We went ahead with this 2nd part, basing it on the success of the 1st part, done in March, which Vivs recovered quickly from and seemed to have a little more speaking pressure and volume after. We also wanted to see if it would help her tolerate wearing her speaking cap, which makes her voice stronger and smoother. We are starting to regret doing part 2. She's had increased coughing, especially at night. However, her coughs are now more unproductive, taking several tries to clear, and then sometimes, suddenly, out of nowhere, w/o any warning, a huge cough that fills her whole HME so full that she yanks it off because she can't breathe.
Her energy is lower, laboring to breath and seems to work up a sweat easier now, from just moving around a bit. Her breathing is also very turbulent and forced on exhalation at times and even what sounds like "snoring" from her trach, but that would not be a good sign as our ENT thinks it could be turbulence from her narrowed bronchus. If I had any doubt before that she wasn't the same, it was confirmed this week at our Little Gym class, after taking a summer break, where she could barely do anything w/o tuckering out and laboring. It seemed like she'd lost all interest in anything active and that it was even making her mad and discouraged. It actually really scared me. Little Gym is an inner struggle for me because I get to see a good friend who takes her daughter as well, but if weeks like these continue, I'm going to have to give it up. It's not worth it if it's exposing her to germs that could make her sick and cripple her airway. Maybe I'm just pressing her too hard physically when I just need to realize it's amazing that she's even alive...who cares if she STILL can't run or jump yet.
So, with all of the weird things that we are noticing about her, we are "THINKING" (we are wrong lots of times!) that before surgery, she had more upper airway room than we thought and now there's none from swelling, so all air is forced through trach, where as before, some made it up through her nose and mouth, relieving a little pressure. We are hoping that this is just a much slower recovery from surgery, but we also fear a bigger problem below the trach, with the tumors in her lung area. For the 1st time ever, she's telling and signing to us that her chest hurts, pointing to where her lungs are, also hitting her head in frustration at night when she can't seem to cough it up. Seth and I have almost daily conversations about Vivs "airway" in our attempt to make sense of what's going on. It's often the first thing we talk about when we get up or he gets home as we report to each other how the night or day has been with her. I am often so caught up with our daily life of managing her secretions, suctioning, treatment schedule...that I feel like I stop seeing the forest for the trees...forgetting about the seriousness of her situation and how complex it is. Even though we are used to this type of life and responsibility, it continues to be a huge strain in our marriage and we (ok, mainly I!) still struggle with my attitude. Seth is always more gracious than I deserve.
A week after surgery, she seemed to get sick, although her oxygen and heart rate were normal. We weren't sure if she was still swollen from surgery and slowly pooping out from laboring or getting her #20th illness since trached. We knew it wasn't an infection because there were no colored secretions or high fever, but she was starting to retract and pull in her chest area, having faster, shallow breaths. We waited to see what would happen over the next few days as we increased treatments and then took her to her post op where we were prepared to be admitted. The scope of her upper airway showed a lot of swelling. She was having the hardest time breathing yet, so we went to ER to have an xray, blood work and culture done, then spent 2 days in the hospital, but nothing significant came out of any tests. She didn't seem to respond to the 1st does of steroids and spent the whole 1st night retracting heavily, almost needing to be put on the vent. I could even feel the vibration of the bed when her chest popped. With frequent albuterol treatments and more sleep, she slowly improved after the 2nd steroid dose. Steroids always make her hungry and irritable...a humorous combination of traits at times!
Even though, I thought we might be admitted, it was the first time EVER that I didn't pack ANYTHING. I guess it's taken me 2 dozen trips over the years to finally relax and not worry if I have this or that. I've finally grown tired of packing up the entire house for a possible long stay, then it taking a week to get it all put away when we get back. The only thing I grabbed on the way out the door this time was some underwear and several of her "sleeved suction catheters" that we can't get in the hospital and make suctioning so much easier for me, since I do most of it there. I am so thankful to God for a family type atmosphere on the peds floor at Kaiser, where we know so many of the Drs., R.T.'s and nurses well and feel so taken care of.
Even though it's tough if Vivs is very sick and needs lots of attention, I always look forward to seeing everyone and visiting with them! I also love that I can snuggle on the bed with Vivs because all of the "hook-ups" are on the wall, right behind us...a luxury we don't have at home. Grandma Viv is invaluable during these times, as Seth goes to work if things aren't too serious and she is able to do all of the things that I can't do if I'm in the bed with her, like keeping the room tidy, refilling things, cleaning this or that, getting food...). Grandma never seems bored, but keeps herself busy visiting or doing this, that or the other. It's also nice to have the company and she's great at entertaining Vivs when I'm exhausted. She still loves to come for a few days every week or two and never tires of playing made up games like "Guess the Animal" in the car and sings old song to Vivs like "Ceci, old playmate", "If the Lord Makes You a Plumber", or "He's Still Workin' on Me". Anyone remember any of those?
We came home with an added treatment of pulmicort 2x/day (an inhaled steroid). As much as we question the value of the many treatments we do every day, we feel that we have to continue them to mobilize her secretions in fear of her narrowed bronchus making it more difficult to clear secretions and develop pneumonias. I always wonder if we are just wasting valuable play time, since it's very difficult to see a clear improvement after them, but I'm so regimented and scheduled about things that I do, only missing one scheduled treatment in months. Part of my strict regimen is my "follow the rules" nature, but another part is fear of something even worse happening to Vivs if I don't do them., like the one time I stopped giving the vest treatment for a day because I became fed up with our lack of play time and didn't feel like it was helping...she got sick the VERY NEXT DAY...perhaps not because of my decision, but I can never prove it! Sometimes, I feel like all the treatments are what drives me crazy. I'm shaking from holding her while she shakes, while also holding the nebulizer, then I'm sometimes suctioning every few minutes. Then the TV is blaring so she can hear it over the 3 machines that are going and if she's sick and fussy or really uncomfortable, I have to keep coaxing her to stay there. Sometimes I even leave the pump on accidentally, out of brain-dead-ness and then I think "Why is it so loud? Oh, I left the pump on!"...or better yet, go to suction her, but don't even turn the pump on because it already sounds like it's on! Then I think "Why isn't the suction working? Oh, it's not even on!"...or this morning, when I realized I'd been running the nebulizer for 20 minutes before putting anything in it! So most of the time I just want peace and quiet the rest of the day. I thought I had the answer to all of this when I let her do some treatments by herself for awhile, which she sometimes does successfully, sometimes not...then I'm freed up to clean the kitchen or whatever else needs to be done...but a lot of times, she ends up needing so much suctioning, that I'm running back and forth...so I mostly prefer to just sit and hold her. I'm hoping this changes as she grows! She's almost too big for me to hold her on my lap anymore. Sometimes, she'll say and sign "all by myself today" and I'll let her do it, in hopes of building future autonomy!
We were starting to fear permanent vocal damage as she had no voice for 3 weeks after surgery and had to almost fully depend on her sign language. I have never been so proud of her and all of her hard work! She tries very hard to learn as much as she can, always copying any new signs I do. She's just getting her voice back and now frequently says "Mama, how to sign ___?" and fills in the blank with any new words that she doesn't know in sign yet. I hope to continue more tutoring sessions this fall to continue our fluency. Her E.N.T. agrees that we probably shouldn't do anymore of these type of surgeries, as her response is so unpredictable and they don't seem to help. She still hates wearing her speaking cap and it doesn't seem like it's going be her "thing" and I've almost given up trying it anymore. Sadly, I think she was better off before these 2 surgeries and we should just leave the tumors alone, but I fear we've messed it all up and have to adjust to this new normal. I never thought her secretions could get any harder to manage, but I was wrong!
During this last hospital visit, our beloved E.N.T. told us that she was leaving Kaiser. My eyes were welling up with tears and I'm pretty sure Seth's were too. She's known Vivs for 3 years, since her very first surgery at 7 months old. Although we have other doctors, she's been the one that we feel has really taken a lead in Vivs care, spending a lot of time with us and talking us through decisions and options and sharing any new research with us. She had a very serious talk with us about Vivs future care due to her rare medical issues and location of her tumors. If anything needs to be done to decrease tumors below VIvs trach, Kaiser will not be able to help us and we will need to go to Stanford or UCSF. She mentioned that, at first, rare cases get a lot of attention because everyone seems to want to learn about them and see if they can help (like the calls we received from all over the nation after our ENT sent Vivs 1st MRI scans around to NF and tumor specialists)...but after awhile, you get LESS attention, because no one really knows what to do with you! I'm sure it's very difficult for her too, as she won't be allowed to view any of her "cases" after she leaves and we won't see her again unless we need to go to Lucille Packard. We will have to explore the options of an HMO or PPO. We also heard that if we can get her diagnosis fully covered by C.C.S. (CA Children's Services), that we could take her anywhere for care. We pray the tumors around her lungs never cause more serious problems (we aren't convinced that they aren't right now), but we know that everything about Vivs is unpredictable so we have to ready for anything.
Because we fear problems in Vivs lungs and have never seen what it looks like, (besides MRI images), we did press our pulmonologist to schedule a lung bronchoscopy in a few weeks, Sept 15. It is a visual exam of airways of the lungs. She will take pictures and a tissue and secretion sample to see any infection causes. This will hopefully tell us if we need to do the extra hour a day seasonal inhaled antibiotic TOBI treatments from October-March, like we did last year. If no pseudomonas is found, then to my understanding we don't have to do it. That would give us much more play time! We hope this will be an out-patient procedure, going home after Vivs wakes up from sedation, but because of Vivs tracheomalyasia (floppy trachea), everything collapses when messed with, attacked or inflamed, especially when lying down.
The airway is suppose to be rigid and hold up under pressure or stress, but hers does not, so even a small procedure could be a big deal. Her G.I. Dr. will also take samples to test stomach acid PH to tell us if we need to continue acid reflux meds, something she was diagnosed with before she was trached. However, I'm sick of giving the meds and wonder if she even needs them, but I've been in fear of weaning, since many trached people are on reflux meds simply to protect their more sensitive airway and her GI dr. always says that Vivs airway is special because of the extensive work done on it and wants to do all we can to minimize any acid injury to her throat. I weaned it once last year on my own, and sure enough, the next time we looked down her airway, we saw a lot of acid coming up and irritating her airway. I felt horrible.
Nights continue to be our biggest challenge, even more since surgery. Every night is different. Some nights, she coughs the whole 1st half, then settles, other nights it's the opposite. Some nights, the coughing is irregular, other nights it's regular intervals apart...and once in a while, a night with only a few coughs here and there. It's crazy. It's not unusual for her to cough every few minutes, all night long, w/ only a few minutes of reprieve here and there. Before surgery, this was something she did ONLY when she was sick. The poor girl is exhausted by nap time and goes to sleep rather quickly (a blessing for me as I am too). She now de-sats more frequently (low oxygen alarm goes off), sometimes taking an hour of unproductive coughing for her to finally cough up a huge blob for her oxygen to return to normal, only for it all to happen again and again. She's always had a harder time managing secretions when lying down (diaper changes always require 1-3 suctions), but after this last surgery, it seems that even suctioning doesn't seem to help. Before, we were able to easily suction anything that she might cough up into her trach, but now it seems more difficult to get, so we've started night treatments, which is also something that she only needed when sick before. She's also needed more saline in her trach at night, because her secretions are so thick and mucousy that it hardens in the trach, and if given a chance to dry, narrows her airway and causes even more straining. Thankfully, our night nurse had an extra nebulizer machine that we can keep in her room instead of dragging it back and forth. We are grateful to have had the SAME night nurse for almost a year! She comes about half of the week.
It's not only the lack of sleep that we fight, but the physical strain of getting up and down dozens of times a night, bending far over the crib to suction (harder for Seth as he's taller). We just recently turned her crib into a toddler bed, hoping it would ease the strain a bit. We always lie down after each suction because we don't know when it will be her last for awhile and we can get a few minutes sleep. This used to drive me insane, almost making me to scream, but some time recently, after over two years of this exciting night life, I just stopped caring. I can tell my body is adjusting to it all because it's been awhile since I've had a breakdown. The most recent one came a few weeks ago, after our 2nd hospital visit in 2 weeks and me going on 5 nights of little sleep. It happened when one of our Drs. came in to check on us and I was in the middle of answering one of her questions (which wasn't sad at all!), but the lack of sleep just hit me like a ton of bricks. She was gracious and spent a few minutes just chatting with me...but I'm sure that went into my "social notes"! I guess that's how long it took me to accept it. Seth says at this point in my life (well, since I had a baby, really), I REALLY should consider taking up two things that I've never drank: caffeine and liquor...caffeine to pep me up and liquor to relax me! But I don't like either one, and I've never wanted to depend on a substance for a feeling. Maybe I'll regret this one day...who knows? I have found an unexpected ironic benefit to our active night life. On a recent girls night out, where I didn't get home until 2am, I didn't even feel phased (far from my pre-baby life, where I rarely stayed up past 9:30!). If I'm on night duty, I might haven't even gotten to sleep yet from all of the suctioning by 2am! Let's just say that the term "pulling an all-nighter" was something that I would never have been physically able to do in college and couldn't even understand how people did it. Now I do it at least 2 nights a week.
For the last year, I've let myself get extremely frustrated with not being able to stay in a wellness groove and have a consistent exercise routine, due to our situation and everything having to stop when Vivs is sick. But recently, I guess I finally accepted it and now just try to jump back in to the swing of things when she's well or try my best to take advantage of the time when Seth or my mom are here. My back, neck and shoulders are in horrible shape and regular exercise is the only thing that helps so if I can wake up early enough and VIvs isn't up yet, I try to do 30-60 minutes of yoga (w/ my favorite 2 PBS shows!) in the morning, and a fast walk/run in the evening when Seth gets home. If I have any other "me-time", I try to hack away at one of my many projects. Honestly, it drives me insane to only have a few minutes here and there to tackle them, but it's the only way I can get something done over time. One of them I'm trying to finish by November (3 year anniversary of her 1st surgery) is compiling all of my FB posts and many of the "comments" that have helped me feel less alone over the last 3 years, into one book...not a published book, but just a book for us. So many of you have taken this journey with us and mostly because of social media called FB. You have taken the time to check in and then to type things that still resonate in my heart that always make me feel less alone in the world and that I like to re-read from time to time. While cutting and pasting comments from 2 years ago, I came across a very short, but meaningful one from a college friend..."God is there with you...and that changes everything"...always a good reminder for us all that God will never leave us or forsake us!
Now, more than ever before, I have to be very careful about what I let through my eyes into my soul...like watching the news. At night when thoughts have more time to roll around in my head, a lot of fear can grow out of thinking about things that could happen. I think of Seth dying and leaving us alone or a natural disaster that separates us while he's at work, or something happening to me or my health that would hinder my care of Vivs. Without watching the news, I have plenty of things that bring fear. But if I let myself watch it, even a little bit, I grow terrified of living in and raising a child in the current conditions. So I rarely watch it anymore. The other day, when listening to a friend talk about going through all of his Grandma's material belongings after her death...the question came up "It's not going to be like that with our generation. Who's going to go through all of our DIGITAL stuff to find treasures like old letters and pictures after we die?" For some reason, this made me incredibly sad and I still can't stop thinking about it. One morning, I felt God gently reminding me that "His Eye is on the Sparrow, and I know He Watches Me", when I heard the song on the car radio (which I NEVER even listen to anymore) and then again that morning in church. I needed that right at that moment as my night fears were escalating.
Even though I do my fair share (and more) of complaining, I am trying to be consistently thankful to God for my health so that I can take care of Vivs. OK...maybe the gratefulness is just a selfish attempt at illness & disease prevention! I have a wonderful husband who does more than his share. I hardly ever have to buy Vivs clothes or shoes, because Grandma finds everything at the thrift store. I am also specifically grateful for 2 neighbors, that have become precious friends, who KEEP (even though I say I'm ok now!) bringing food almost every week. One will always share her own meals with us which are healthy and delicious and the other loves to buy me a bunch of stuff during her Costco trips! It's irrelevant that it takes us over a year to finish that 200 pack of corn dogs...I won't complain because it means less work on my part! They also come and visit often in the late afternoons, which any mom knows can sometimes seem endless. We are truly blessed by so many other wonderful and caring neighbors that we get to visit with often.
In June & July, Vivs had her longest wellness streak EVER (2 months) and we tried to enjoy every minute! During this time, we braved a trip to Missouri to visit family and farm. My parents and I drove back most summers of my life and I try to go back at least every 2 years. I would rather go there than any beach, resort or cruise and want Vivs to know her family & have all of the same experiences I did. Yes, I regretted it after the minute we purchases plane tickets, thinking "VIVS IS NOT THE TYPE OF GIRL THAT YOU TRAVEL WITH! SHE HAS 6 MACHINES AND ENDS UP IN THE HOSPITAL ALL OF THE TIME! WHAT ARE WE THINKING?", considering the 2 months before this trip, she couldn't stay well for more than 10 days. I guess I was most afraid of being somewhere ELSE and her getting sick. Our life is so inconvenient when she's sick and I didn't want our family to see that side of our lives, where Vivs coughs all day and night and we do treatments all day and night. I spent almost a full month organizing, researching, calling airlines, getting dr notes and any meds we might need, and prepping how to get some of her equipment delivered, some sent with my parents (who drove) and making sure we kept everything we needed with us in case she got sick and we were separated from the stuff being delivered and with my parents. I kept Vivs sequestered for weeks before our trip. I was so nervous about going through security and spent way to much time prepping when it ended up being no problem at all.
Because I'm still so absorbed in what everyone else around is thinking when she coughs and we suction and I know what I would be thinking if I didn't know what I was hearing, I had plans to stand up and tell everyone on the plane "FOR THE NEXT 4 HOURS, YOU WILL HEAR MY DAUGHTER COUGHING HER BRAINS OUT AND THEN WHAT SOUNDS LIKE A GENERATOR AS WE SUCTION, BUT SHE IS NOOOOOT SICK, SHE JUST HAS TUMORS IN HER AIRWAY, SO REST ASSURED!", but after we got settled in our seats and we heard the lady behind us start a jag of loud, hacking smokers cough (isn't that ironic!) I thought "Who cares? I'll never see these people again" and never carried out my well practiced announcement. Even though I was able to relax and have a great time with family, I studied and watched Vivs carefully, night and day, knowing that at any second, she could start coughing and not stop for a week. If I noticed any changes in Vivs, I would start trying to gear myself up for what our plan of attack would be. There were two times, both in the evening, when I just KNEW that it was coming. I could see it in her eyes. She would not be interested in a meal (very rare!), start coughing more and become very low-energy and clingy to me. We immediately gave extra treatments and prayed all through the night and both times, she would wake up just fine! WHEW! I was most afraid of her getting sick just before we had to fly home...then we would have to stay (for who knows how long) until she got better and I would keep thinking to myself "Why didn't I just make the reservations for ONE DAY EARLIER?" Of course, our family probably wouldn't have minded this a bit, but I just didn't' want them to see this side our life.
It ended up being the miracle trip, all I had prayed for, for weeks. I don't even think I've prayed harder for Vivs to be healed, even during the times before she was trached, dying in front of our eyes. In those two weeks, Vivs received about 6 months of physical and social therapy with all of the cousin play and things to do, stairs to climb, and autonomy like she's never experienced before as we allowed her to gallivant anywhere she wanted. She had her 1st marshmallow pepsi at the creamy, learned how to carry the kitties w/o strangling them and caught fireflies. Hearing my aunt Violet's infectious giggle when she watched Vivs eat, play or sign made it all worth it! It was a huge deal in our little world. Never mind that it took me over a month to get all of the medical equipment that I packed PUT AWAY!
