Living Pandemic Life for 13 Years

Technically we have been living "pandemic life" for 13 years and counting. In 2007 with a tiny new baby in our arms we met and listened to the first pulmonologist we had ever met. This pulmonologist explained what life would be like for our cystic fibrosis baby. He asked us about our habits as a family and then very honestly gave us his advice on how that will change in order to most protect our baby. In addition to the vital daily lung treatments we would give our daughter to keep her body in as healthy condition as possible he also gave us guidance on our time spent outside the home. He recommended we get rid of our dog, stop attending church, avoid crowds at all costs, and stay away from symptomatic people in general. He meant very well, he wanted to start us on the right foot. He wanted us to understand the importance of keeping her environment as healthy as possible.  

It was very shocking to us. We did not understand cystic fibrosis then and the very dangerous effects of nickel and dime damage caused by repeated respiratory illness. We had to accept that the lives we had lived as children would be different in many ways than the life we needed to give our child. It was hard to understand what to do. The burden particularly on me as the mother was very heavy. I lived in a great deal of fear and trepidation as I tried to do my best to protect my daughter. My hands would shake in public places, I was terrified to touch anything and I kept hand sanitizer and wipes everywhere. I was always thinking about when I could wash mine or her hands next. My stomach lurched when other people would touch her or ask to hold her, even family. I felt that I was always putting her in danger or potentially exposing her to something that would send her into a tailspin of cystic fibrosis exacerbation.  

Soon that pulmonologist left to work at a hospital on the east coast. A new pulmonologist became our daughters caretaker and helped us have a more balanced approach to life. He advised not to keep our daughter in a bubble but to live life as best we could with appropriate caution. He helped us to slowly feel more comfortable in managing cystic fibrosis in our family. 

But my hands still shook in public places and I was always worried. Everyday it felt like rolling the dice on her health. I very quickly realized that naturally no one else, outside of my husband, would understand the degree of burden I placed on myself to keep her healthy and how seriously I took it. I felt insane with anxiety many times and shock at other peoples lack of general healthy behaviors in regards to coughing, touching, and washing hands. 

Twelve years later I still am always looking sideways and backwards for some exposure I may be missing oftentimes to the point of insanity. Also in the past 12 years I’ve seen my kids spend a few weeks in the hospital, have surgeries, struggle with their health and help them repeatedly overcome low lung function, related weight loss and a myriad of associated physical and mental  distress of chronic illness. We’ve also watched as seemingly healthy cystic fibrosis adults and kids around us “catch” just the “wrong bug” and die. Quickly. 

Sitting here now with a virus impactful enough to literally break and shut down the entire globe it feels like I’m holding not one but 3 completely vulnerable infants in my arms watching for danger. There are 6 of us in this family. Three with cystic fibrosis and three without. One of us gets sick and the dominos fall for the rest. We are grateful for the people around the world who see this pandemic for what it is and what it means for people who are already struggling with health. What an amazing opportunity to teach people about acting in ways that benefit the whole community and not just heightening the importance of personal convenience.  

Letter to a newly diagnosed family

Dear Newly Diagnosed family of a precious cystic fibrosis human,


When my daughter was diagnosed with Cystic Fibrosis at 6 weeks old I felt buried under the ruble of what I thought her life would have been. "Fortunately the sun has a wonderfully glorious habit of rising every morning," and slowly but surely this initial cloud of misconception cleared (quote by JC George). We managed together as her support system (including family, friends, CF Clinic team, and fellow CF families) to climb each challenge of meds, routine treatments, and nutrition. Now I see my teenage daughter soaring through life experiencing all things with arms wide open and it is in great part her Cystic Fibrosis that has molded her into the confident, brave, smart, fun woman she is! Since her diagnosis we have had three more children. Three of our four children have cystic fibrosis so we have a unique take on life and on cystic fibrosis.

Here are 3 beginner cystic fibrosis nuggets of life that we love and live by.

1.       Be an exemplary hand washer. Nothing prevents getting sick better than clean hands. Simple, true, and easy to do!

2.       

Keep enzyme supplies close always. For non pill swallowers that means squeezable applesauce packs for the road, and bottles of applesauce in your pantry always. However in a pinch we have used jam or ketchup (the kid loved it). Pill swallowers should never leave home without enzymes. Pass out extra bottles of enyzmes to family and friends whose homes you frequent.

3.       Keep the end in mind from the beginning. When physically and mentally possible you want a strong self capable human ready to live with Cystic Fibrosis on their own to me this means they need to learn three things from you as their parent.

a.       Routine, routine, routine. Find what works for your kid and family and stick with it. Our families experience on treatment routine and treatment responsibility is its own separate essay. Routine changes many times as kid grow from infants to toddlers but never give up, keep trying, and appreciate your efforts! It’s a big deal that you are dealing with!   

b.      Positive attitude. The more accepting and matter of fact you are as the parent of treatments being an essential part of a regular day then the more your CF child we adopt that attitude themselves.

c.       Celebration. Taking care of your body is worth it! Accomplishing that everyday for anyone is a celebration but particularly those who have extra medical requirements tacked onto their regimen. Celebrate your successes and talk positively about them. Cheer your heart out for your baby and for yourself for helping them do challenging things! Let them hear you speak positively and see you celebrate with them. Their attitude is most often a reflection of yours.

It has also helped us to find positive cystic fibrosis role models. Through a fantastic CF podcast called Breath In we have met an inspirational Harvard gradudate, a Canadian farm boy who grew up to invent a life changing inovative vest (out of a sewing machine motor) for himself and his fellow CF peers, and many other mind blowing awesome CF success stories. The cystic fibrosis community is full of hope, kindness, and indiviudals and families who appreciate every breath of life. You got this! You can do CF!


Keep on the sunny side,


Kamarah Adams  

7,000 Hours Later

Gut reflections, 4 years into g-tube life.


Orson has had his g-tube for four years now. It is still strange, incredibly cool, and can be emotionally draining for him all at once. He loves having a trap door for liquid meds. He loves watching youtube (studio C, and kid snipets) videos while his morning bolus feed is accomplished. He typically does not like talking about his g-tube with people. Sometimes however he will randomly offer up the information depending on the social environment. He hates that it is always there. He hates the scared feeling he gets when someone on the playground accidentally kicks/bumps him in the stomach or it gets snagged on something unexpectedly. He has adapted exceptionally well to having a piece of plastic hooked into him every second of the day for the past 4 years. He still plays rough with siblings and dad, swims, does full participation in PE at school, and runs around like a maniac on the playground at recess. He has one skip night a week so if you add up the skip nights (52 and round up to include when his stomach is too upset to hook up at nights, or he skips for other reasons to 65 total skip nights a year) and get him doing his button 300 days a year every night for 4 years, he has run 1,200 nights of 6 hour continuous feeds. Approximate total hours tethered to his formula pump for the past 4 years is 7,200. That also means Frank or I have gotten up to unhook our buddy and get him to the bathroom 1,200 times.


It has blessedly never had to have been surgically placed again since its original placement in 2015. We have become experts in removing the old button and replacing it quickly for him. We have learned the best kind of tape to use and how to prevent it from tugging on him while he sleeps like a wild wiggly child each night. His teachers or myself have taken a “foley” and/or tape with us on each field trip he has ever gone on, or anytime we leave the house at all for just in case emergencies of it coming out and needing to save the sight from closing up.


He continues to not have the desire to eat much orally. I continue to wonder if I’m doing the right thing using the canned formula or if I should use a fancy blender and do custom formulations for him like other g-tube moms I know who do that very successfully. I tried it for a while back in December of 2017 when he had tonsil/adenoid/sinus/bronch done on the same day and afterwards couldn’t eat orally for 9 days. It was really neat to do the blends but for several reasons we went back to the canned formula.

He periodically asks when he can get rid of the g-tube. We explain that it would be a slow process over a year or more where he would taper the use of it while continuing to maintain or gain weight and then finally end use of it and then the decision could be made at that point to remove it or not.  


I went back and read the post I wrote after his surgery and it really was a terrible time. We had no idea how awful the entire anesthesia experience would be for him. Four years and a handful of other anesthesia’s later we see that its just extremely taxing on him mentally and physically every time he goes under. Torturous on the kid. It was also our first experience in a negative hospital environment. Insurance had us use a different facility than we were used to and it was not great. A huge miscommunication between the surgeon and the hospitalists happened and no one wanted to contact the surgeon to clarify so Orson ended up fasting unnecessarily for 39 hours. My hindsight and gained experience over the past 4 years of life with hospitalized chronically ill children has me seeing the situation he was in with anger. I would do it so differently with my now knowledge. I’m sick to my stomach now thinking about how we let him suffer because we were afraid to defy the “hospital” when we knew better from the surgeon. But then I try to remember that anger solves nothing and that we have learned a truckload since then and most importantly I remember the sweet sweet King Of Brave who fed his bear when he could not eat himself. What a sweet daddy he will be.


The g-tube has been a mighty lesson for Orson in strength, endurance, compassion, and courage. We’ve definitely learned about all those things from watching him.

King Of Brave 2019

2019 April vs. 2015 April family photo

A Cf Is Not Forever Christmas Story In February

In our designated corner of the procedure room, masked and ready to cheer for the PICC'ed

I was surprised that Maelee ended up in the hospital this past December. I can never guess right when it comes down to it if she needs IV antibiotics or not. The previous year, December of 2017 we had Orson’s surgery and the timing of it had him and us as a fully functioning Christmas machine down and out until literally Christmas Eve, so I thought for sure (not that this makes any rational sense) it was looking like we wouldn’t have any issues for Christmas 2018. But I was wrong. Fortunately even in my October minds blissful ignorance of December’s hospital stay Heavenly Father knew I would need to prepare early. It was brought to my mind, “get ready now for Christmas,” all the way back in October. My mom happened to come over on the day after I had bought the Christmas presents (on a smoking hot sale of course) and she helped me wrap them. She kept saying, “whoa Kamarah I am so proud of you for being ready for Christmas this early.” It was not my idea though I was just listening to the whispers of my Father who loves me and who knew I would not have the chance to procrastinate for December of 2018.

With this being her second hospital stay for the year it felt to her like she was just there. They keep putting her in the same approximate floor and room area and it’s a repetitious routine of being trapped and waiting and waiting and waiting until we are allowed to go and finish at home. It was difficult for her to miss all of the school and friend Christmas activities. She felt left out of things and lonely. But she also had some very supportive friends, school, and church teachers who came and did everything they could to help her feel remembered and included.

As I watch her mind and body take these steps up in an unceasing uphill battle with the repercussions of her disease I’m continuously amazed by her strength. Although she has no option of going through the challenges of her body she does have the choice on how to face them. Which attitude pants is she going to put on? Well bless her heart when it comes right down to the tension filled hospital decisions and then even more emotionally filled long stretched out days and nights of her hospital stay she chooses to put on some really beautiful attitude pants. My favorite for instance of this particular December 2018 hospital stay was her courage with her PICC placement. November of 2016 she got her first PICC and it was placed under anesthesia. Then July of 2018 they gave her a dose of Versed to get her through placing it in a procedure room and not put her under. Finally the big drum roll of December of 2018 she was able to muster up the courage (and this is them threading a line from her arm to her heart while she is awake and alert) to get her line placed with no Versed at all. She was of a fully sound mind and body for this PICC placement which just to me shows her absolute fight and courage for success. I continue to be so proud of her.

It was rough to have her be trapped and struggling in the hospital during the busy and exciting Christmas season and then came the familiar strain of helping the home kids feel happy and well in tandem with the hospital kid feeling happy and well. But again we continue to be blessed with family angels and friend angels who help us get through it all.

When she was able to go home to finish her IV antibiotics I was feeling pretty sorry for myself. I was upset that I wasn’t able to get out Christmas cards as early as I wanted and frustrated I wasn’t able to do the seasonal making and baking that I usually did. I was focused a great deal on things that didn’t matter most. It seemed important to me though and I was sad. Then I got a text from an unknown number in my phone. Someone was asking me how Maelee was.

I was grateful for the kindness of the check-in and didn’t want to be rude so I gave them an answer then asked how my mysterious texting friend’s family was as well, figuring this would clue me in on who they were without me being a jerk and not knowing who the number was. The answer took my breath away as I realized who had taken then time out of their day to text me.

It was a beloved friend and example in my life who I usually talk with face to face or through Facebook messenger. This friend has a tremendous load to carry in her life and does it with such grace, confidence, acceptance of God’s will, and kindness and concern for others that I couldn’t believe that with what all she does everyday for her family that she would take the time to check on me and Maelee. It was a very powerful moment for me of feeing the love of God in my life during the Christmas season. I also quickly added this sweet friend to the contact list in my phone and expressed my gratitude for the love she allowed me to feel on that December evening. What a tremendous lifting hope it is to be surrounded by people trying so hard to follow the example of Jesus Christ.

That is the end of my Christmas story in February. It was a rough month and different than the other two CF exacerbation stays she’s had. This time she sounded really terrible and everyone told her that constantly. It was like taking a beating mentally considering the work shed been doing for her lungs already. It seemed so insulting and defeating to hear that they weren’t reacting positively to her work and effort. The bronch shows her lungs were/potentially still are recovering from having very thick (cheese curd consistency) mucus plugs throughout the bronchable areas. Maelee will have her second sinus procedure (procedure, surgery, whatever you want to call it but her ENT will be scraping all of the compacted bacterial tar out of her) in March to hopefully alleviate the constant head pain, nausea, ear aches, and overall head throbbing she deals with always. The current idea is that the bacteria that is continuing to give her lungs such a work out is also in her sinuses and they in turn are re-infecting each other. She continues to work hard and we continue to encourage her to work hard to stay on top of her treatments. Beautiful discoveries are happening in both her life and ours as parents  as we work through the regular and universal growing PAINS of life and the strange world of CF mixed in.  

Days are easy in the hospital, nights feel sad and

all emotions are coming out.

Mornings in the hospital are wiped of the negative

night emotions because your just so happy the night is over.

Getting to take a walk out of the room after 72 hours inside is

really very exciting.

Maelee's dear friend and child life specialist who is allowed to

be by her during PICC placement. She is an angel on earth. 

The procedure room and Maelee being filled with courage

getting her PICC like a champion of life.

Spark the Fight & Weight Loss Update

Sparking the Fight of Compliance

This is a year of blossoming and growth for all the children. Particularly it seems for our oldest child as she reaches the end of elementary school. It is on my mind always that this is the last year that they will all be together at the same school. I guess I didn't realize I would only have two years of that and once I did the last year seems very precious. Also thinking of the schedule change and location change and routine change that comes with next school year, this same daughter starting junior high and leaving the home 50 minutes earlier than she does now really really really seems impossible. We need a solid two hours to successfully complete our morning routine (sinus issues, bathroom time, vests, g-tube work, inhalers, nebs, antibiotics, pills, scripture study, breakfast, pack up) so that pushes us all up to waking up at 5 and getting started. 

I have been aware of the school day morning miracle happening each day this year again since school started. All summer long we drag through treatments and breakfast each morning and I think "how in the world do we ever get this all done and packing lunches by our 8:00 departure time on school days?" We really have angels helping us each morning. Perhaps they hold down the hands of the clock just a few seconds each minute so somehow it works. Even more miraculous are the school day mornings when I see each Saturday and Sunday all year round being the ultimate struggle to get through treatments.

She received one of the biggest surprises of her life when she tried out for the school play. She tried out for a supporting character and landed a lead instead. That boost of confidence gave her the courage to run for student council and although she didn’t win she chose to still be a class representative. Then at church she joined a group of other musicians with her viola and they have been practicing for a “I Am A Child of God,” primary program.

With this grown up schedule added on to the regular family seasonal schedule of soccer, fall parties, and her activity days for girls meetings it’s been exhausting for her. She loves all of it and doesn’t want to miss anything. She is trying very hard to keep up with it all. Then as predictable as every start of school year she begins to loose the desire to eat to make more time for friends and activities. Not taking the time to eat begins to snowball the health of anyone but particularly someone whose body already demands extra calories and oxygen.

At clinic a week and a half ago it all showed up clear as day on the vitals and pulmonary function tests. Her tank is running on empty. Her PFT’s were down 15 points below what they were BEFORE she was hospitalized over the summer. Her weight gain has slowed down enough to crash her off of her trend line. What was most disheartening and discouraging was how she took the news at the clinic. Resigned. Passive. Acquiescent.

So I come home and as I tell my husband everything I saw and heard at clinic which usually takes a few days for me to remember all the details we slowly together come up with our parent plan. We have to spark the fight of compliance and extra compliance and assertive compliance to her treatments. We keep asking what more can we do to help and what more can we do to encourage her in this life altering skill of compliance to her detailed and arduous never ending medical regimen. She has the pain, discomfort, and burden of the disease and we do what we can to help her figure out what works for her to relieve that as much as possible. I don’t like to ever  use the H word but I HATE when she’s suffering digestive wise of sinus wise or airway clearance wise and I have no more tools to hand her.

Things are slowly dropping off of her busy schedule and by the end of this week things will have gone back to our baseline of family business but then she’s left to dig herself out of the health hole she has gotten into by participating in these amazing social and mentally growth promoting activities.

The pulmonologist was so kind and understanding at clinic with Maelee’s drop in statistics. She was encouraging, overlooked the angsty elleventeen jazz and said come back again soon so we can check all your numbers.

We know she is full of strength. We watch her use it everyday. Now we need to be the best compliance coaches we can be as parents because it is not only her that is benefitting but all her siblings watching and coming up behind her. The clinic in November will bring more news to see what is next for Maelee in the last few weeks of 2018. And by that I mean another possible hospitalization or the best side of the coin soaring numbers of plenty and sleeping in her own bed.

Kamarah’s weight loss update. 

The stress of the last four years showed itself in the choices I made with food. Last time I wrote about it I was at 38 pounds lost. I am so grateful to say that right now I am holding steady at 67 pounds lost. I was selfishly hoping to get to a full 70 pounds before I blogged about it again but I’m very thankful for the success of 67 and need to remember that. I have lost more than I ever thought I could. I met two of the three goals I had and have accepted that the third goal will remain as a war wound of motherhood.

The three goals being. One: fit into my awesome red pants. I fit into them and now they are much to big for me so I am on the hunt for just as rad of a pair or red pants. Two: my wedding ring fits on my finger again, and also loose at that. The third goal which is actually a great way to keep me humble and kind will not as it seems ever be fixed by weight loss. I have a long spray of bulging spider veins on my left leg that wrap around the back all the way to the front and down my leg. I thought loosing weight would diminish those but it turns out you can’t un-stretch blood vessels and that’s OK because I’m full of joy everyday by the babies I carried that brought those veins.

My weight loss strategy has remained the same. 1,000 – 1,200 calories (tracked by me on My Fitness Pal App by UnderArmor the free one not the premium version), and to get 10,000 steps and day and or 30 “active minutes.” I would say 98% of my active minutes have come from walking either in place while I watch or listen to something or by walking in my neighborhood. I do not have a trainer, a gym membership, use pills, surgery, or anything else besides those things. My favorite way to think about my calories is waking up everyday with $1,000 to spend. I have become really good at finding the things I like and feel the best spending that $1,000 on everyday, and do still sometimes fall for traps of delicious sugar cookies, donuts and pizza. But I own up to those traps and pay out the money and don’t overspend.

My personal goal is to make it to my year mark of when I started loosing weight which was January 29^th. At that point I will evaluate my weight and see what I need to do to maintain that weight. I sit comfortably smack dab in the middle of the healthy BMI range for my height of 5’10. Look up my weight I dare you. 

Double Bunk Us I Dare You

They did it together. Being hospitalized as brother and sister who share the same disease. Orson and Maelee. Maelee the big sister who had done this type of hospitalization before began from the start to be protective and ever the teacher to her younger brother. He needed her support and she gave it. As a mother who sees their relationship I was grateful for this turn in the tide as the two usually have the hardest time getting along together. But her mother heart wrapped around him and she did everything she could to comfort and help him while at the same time getting through the hospital stay herself. Maybe it was a good distraction for her.

When Orson was told that he would need to fast to be prepared for his PICC placement and bronchoscopy Maelee quietly told me that she would fast as well. She didn’t want Orson to be sad seeing her eat. So we all fasted and waited. I was there alone with the kids so my husband could get at least a half day of work in. My younger brother came for a visit that morning. He was also a welcome and lovely distraction from the waiting, because that is what the hospital is, waiting. That day we were waiting for Orson to have his PICC placed and bronchoscopy under anesthesia scheduled for noon, and also waiting for Maelee to be called in by the PICC team to place her line in the procedure room on the same floor we were staying in.

The kids received lots of instruction and explanation on what a PICC was and how they would have them put in. This was done by a cheerful and kind Child Life Specialist named Kylee who was absolutely incredible at her job. I was worried that the procedures for both kids would collide and I would not be able to be with one of them. They pulled Maelee back into the procedure room, just a few doors down from where they were staying. Orson was so scared for his anesthesia, he hates everything about anesthesia and he knew it was getting close to when he would have to do it so he was hesitant to watch me walk down the hall with Maelee but he told me to go with her.

She was really being taken such good care of by a room full of capable and kind women. They were all so upbeat and caring. Kylee was right at Maelee’s head all masked and gowned so she could be so close to her. She told me she would stay with her throughout the whole procedure so that I could go with Orson if need be. Maelee was extremely brave and was doing so well but I could hear the fear still right on the edge of her voice. Orson’s nurse came in at that time to let me know it was getting close to Orson’s time for preparation downstairs. I told Maelee I was leaving for a minute and ran as fast as I could down the hallway because I knew he’d be upset waiting for me.

He was balled up in the corner of the room crying. He had just tried to escape the room running down the hall looking for me as soon as the nurse told him it was time to change into a gown for the procedure. He got scolded for leaving the room and was sitting holding his procedure gown crying as far away from the nurse as he could get. I calmed him down and helped him prepare and then ran back down the hall to let Maelee know I was leaving with Orson and thanked Kylee for being so generous with her time and staying right by Maelee.

Orson was absolutely inconsolable. Any time that he could remember anesthesia had either lead to him waking up with a plastic straw sticking out of his stomach that still hadn’t been removed three years later or vomiting blood for hours after waking up. On top of that the idea of being forced to sleep makes him angry and feeling powerless to stop it. So when transport arrived to wheel his bed to the OR he wailed the whole way there. I don’t blame him. He sobbed and cried out in frustration literally every second until the mysterious vile chemicals of anesthesia commanded his body to stop. It was torture for him and torture for me. The child life specialist on the floor there convinced the anesthesiologist to let me go back into the OR with him while they put him under so he wouldn’t have to be alone. It was the first time I had been allowed to go beyond the usual “goodbye doors.” I tried to comfort him throughout this process. He asked to pray together so we did that a couple of times. I tried to absorb all of his emotional pain but there was so much I wasn’t enough of a sponge. I watched them plunge (the nurse didn’t go slow he was trying to get this kid to stop screaming) the milky concoction through Orson’s IV while Orson screamed that it was burning and I wanted to punch all of the men in the room (there wasn’t any women in there which for some reason also made me furious that it was all men). As I watched him instantly go limp after screaming the previous hour all I could do was give his hand a kiss and be lead away to wait. It wasn’t supposed to take as long as it did but between waiting on the pulmonologist to arrive, finish, and complete the bronchoscopy and then trying to find a vein large enough to PICC Orson it took twice as long as they said it would. One of Orson’s issues with anesthesia is the moments he’s alone in recovery and waking up without me. Because of this I was pacing getting more worked up and more worked up with each minute. Oh and we had a fun surprise right before we got hospitalized, our insurance deductible that had been met days after January 1st of this year got mistakenly reset unbeknownst to us July 1st (still trying to clean that mess up) so I was fielding calls about medicine during this anesthesia prep time.

In the mean time Frank came to the hospital from work and was with Maelee so at least she wasn’t alone anymore. Finally when the nurse came back to get me I just lost it. I wasn’t crying yet but I was so upset I couldn’t complete a sentence just spewing angry half sentences like the wind was knocked out of me. Then when I got to him he was already awake which then made me start to cry with him because his fear came true and he had woken up alone. I couldn’t describe to the nurse how deeply upset I was. I was so disappointed that I had let him down and felt so helpless as I watched him again get so sad and disoriented watching him live his dread. It was a small procedure. I understand that. But every time he goes under it feels like reliving all the trauma. The devastation and pain he felt as he sobbed in my arms so confused and hurting after g-tube placement surgery, and the feeling of catching a bowling ball in his nose and throat and then repeatedly vomiting blood after his surgery in December of 2017 all those memories just hang in the air around us and its awful. Its difficult not to feel like its your fault as a parent when your child is in pain. Surely there was something you could have done to stop this or fix it but you failed and now your child is in their pit of despair and you have to slowly and carefully do anything you can to coax them out of it.

Then PICC day was over and we had rainbows of visitors everyday to go with the waiting. Waiting for each doctor to come in and give us a two to five hundred dollar handshake everyday, and IV’s to run, four vest treatments a day, and trying to convince the kids to eat.

Maelee has never struggled eating well, but Orson has struggled with eating enough food to sustain life since birth. This bothered many of his nurses who would pepper him and then Frank or I whoever was in the room with questions of why he didn’t eat more. This would make him cry. He did find a way to successfully get his blood glucose measured. When the nurse would come to check his sugar he would call for the parent in the room to come and hold his ears to block the sound of the little machine. He didn’t mind the poke so much or seeing the blood even but the sound of the machine was very grating to him. Good news though his numbers looked swell so they stopped checking after a few days.  

The picture we took before I drove Maelee and Orson to be admitted to the hospital.

The picture we took when I came to switch places with Frank the night of the day my friend had brought Charles to spend the day visiting his siblings.

We switched parent schedules on the weekend when Frank wasn’t having to go into work. He slept at the hospital with the kids on Friday and Saturday night. Which gave me the chance to be the one at home waiting. What was special about it was that because of how the visit played out Frank and I both had alone time with each of the kids at one point or another to express our love, spend time with, and listen to them. Charles and I stayed up later than usual working on a puzzle and with Charles legos are always involved so that was fun too. Ruby and I randomly got cauliflower soup from Zupa’s on her request. I have to admit I was dreading church on Sunday. I felt so fragile and tired and I didn’t want to get into in depth conversations about the hospital because I was worried it would make me cry. But we went to church anyhow. Myself, Charles, and Ruby and once I got through sacrament meeting and into my primary class and sat with my CTR 7 class the most comforting feeling of love stayed with me the whole time. I forget how it came up but each of the 7 year olds in the room started asking me questions about the kids in the hospital. But it was so sweet, kind, and innocent that it was easy to talk about with them. Then without being asked during the opening and closing prayer my primary kids prayed by name for each of my children. I felt so loved.

After church waiting for news from Frank was killing me. The kids had a lung function test to do scheduled for noon, then they would hear from our pulmonologist and she would let us know if we could come home that day or Monday or Tuesday. Then I started getting calls from the home health care company who would be supplying us with our home IV supplies and nurse visits. I started to panic thinking about being responsible for running two IV schedules. So I did what seemed the most natural and beautiful thing a mother could do on a Sunday afternoon. I baked cookies. These are spectacular cookies by the way (Neiman Marcus cookies from that ancient e-mail forward years ago – a family favorite!) that I knew I would have to resist eating two dozen of in thirty seconds because that’s what Kamarah last year would have done, but Kamarah this year has lost 55 pounds and is going for 15 more. But I baked anyway because the process itself was what I needed that day not the eating. It worked. Baking made me feel better and more like a regular mother and not a mother waiting for IV meds to be delivered.   

The IV and vest schedule I wrote out to keep things straight.

The most exciting grocery store trip ever.

 When they came home we ran their IV’s through their PICC’s and it was fine. It was hectic and touch and go for sleep but we got through each day hunkered down happy to be home and not stuck in the hospital room anymore with insanely expensive handshakes from doctors and nurses breathing down our necks. Maelee was so concerned with us having to get to her arm in the middle of the night for IV changes that she would sleep in a very specific thoughtful way with her PICC arm laid nicely on her pillow. The day after we came home we needed to go to the grocery store to replenish the necessities. Both Maelee and Orson practically raced us to the car when we asked if they wanted to go and walked around in Fry’s like it was the bright lights of Paris so happy to be somewhere besides room 320. It was comical and fun to watch them be appreciative of home life.

Now our goal is to ring out the towel of summer for the next few days. It’s that special nostalgic time of year for me when I get sad thinking about having the summer be over and the kids gone but I also want to do a cartwheel because school starting means the expectations of Mom performing entertaining magical experiences stops as well. Cheers to a long and lovely break before our next hospitalization.

Neiman Marcus Cookies

1 cup butter

1 cup butter flavored shortening

2 teaspoons soda

2 cups white sugar

5 cups oatmeal

24 ounces chocolate chips

2 cups brown sugar

1 teaspoon salt

1 - 8 ounce Hershey bar cut into pieces

4 eggs

2 teaspoons baking powder

2 teaspoons vanilla

(9 minutes in 360 degree oven or when slightly browned on top)

PS - Having your insurance deductible reset and then trying to convince people it was a mistake and your deductible has indeed been met is what I can imagine would be similar to sitting in a maximum security prison and trying to convince everyone your innocent. No no no I realize the prison scenario is much worse but its pretty ridiculous none the less. 

Doing Drugs Again and How to Fail A Glucose Test

A month ago today we had clinic. Each of the kids Cystic Fibrosis story remains to be curiously different to us as parents. We knew the disease would not affect them all in the same way of course, but it is interesting to us to see how it plays out symptoms wise and milestone wise simultaneously as a family experience.

Here we are seconds before vomit ignition on lab day

We see one child has taught us what asthma is in tandem with Cystic Fibrosis. I didn’t understand and couldn’t tell the difference between an asthma exacerbation and a CF exacerbation until this past year and a half. Now I know the difference and I also understand that they are exceptionally frustrating when they happen at the same time.

I never realized as a parent of a chronically ill child how thrilling and crushing new drugs can be. Relizorb for instance has been nothing but a massive blessing to our g-tube child who does a continuous feed 6 hours each night. Relizorb I’ve mentioned before, but to recap its this genius cartridge of enzymes that gets hooked up to his feed tubing that continuously releases pancreatic enzymes so he is literally receiving a continuous dose of enzymes. Life changing drug. Now cramps, malabsorption, diarrhea, middle of the night pain, less pill swallowing (at 2 in the morning no less), and overall miserableness is greatly reduced. We are so grateful for Relizorb. It has made life so much for pleasant for our son which makes life so much more pleasant for us.

Unfortunately due to the nature of tube feeding and a medicine prescribed to be used in tandem with tube feeding its coded under medical and not prescription codes as far as insurance goes. Because of this it has been explained to me over the past couple of months not something that any pharmacy wants to fill. Our insurance has said that they would cover it. Great! But then there are not pharmacies that will fill it that are “in network” with our insurance. So now there are I don’t know how many other CF patients who are also being told along with me as a mother of a CF son who is benefiting significantly from the drug that the “bridge program” provided as a very generous blessing by the pharmaceutical company will be running out for us in about a month and a half unless a miracle can happen and they can get a pharmacy set up to handle this hard to handle code. In our prayers we pray for Relizorb to be made available to all of the CF patients who are benefiting from it.

Then we have Orkambi. A new with in the past couple of years CF drug. Big big big news in the CF community. One of those new high faluting fandangled gene altering meds. It is supposed to help correct the core problem with CF bodies, which is how the water and salt function together on a (get ready for a big science word) “molecular level.” We jumped on that wagon as soon as we could for our only child who was old enough about a year and a half ago. The side effects were awful. She was miserable. Our parental decision then was do we encourage her to suffer through these side effects in hopes they will go away as some patients have found. Do we hope that pushing her through the literal pain and nausea and tearful stomachaches will be worth it if her lung function improves? It was hard to know what to do. We decided to pull her off of the medication. We still wonder if it was the right decision.

Now we are having our son start Orkambi. It took some doing and lots of phone calls on my part and work from a dozen people besides myself (you get very personalized service when our dealing with a $200,000 + a year drug) but he will start the med before the month of June is over. Are we nervous? Yes. He already has so many layers of mental and physical issues regarding his stomach, digestion, and overall well being. But then we remember how different he and she are in their CF story and we have hope that it will work for him. We have hope that he’ll be blessed by the drug like so many others with the DF508 mutation. That specifically will be in our prayers as we start him this month during the summer so if he does have unfortunate symptoms he will be at home and not at school to deal with them.

Now for the rest of the story on Cystic Fibrosis Related Diabetes. We went a few days after school got out to do the highly anticipated two hour glucose test to get a reading on blood sugar levels. I got to the lab with both daughter and son in hand. When we got called back and saw that the drink was 10 ounces I gulped. Orson is absolutely definitely not physically capable of doing a 10 ounce bolus feed. His typical tolerance on a fantastic morning of health is a 6 ounce bolus feed and that is with me or his brother providing a cheerful convincing mode of assorted distractions during the bolus feed (not the typical morning when it’s a rushed and grumpy experience before school when we are lucky to get in 4 ounces). But we pressed on because that’s what parents do, convince their kids to do things that they don’t want to do. I had brought the syringes I needed and a handy dandy 24 inch extension set and I got to work cheerfully bolus feeding the 10 ounces into Orson’s g-tube while the very kind lab techs wearily watched from a two foot distance (it’s a very small room those lab rooms, the walls close in pretty fast). The whole time attempting to distract him from the experience with jokes, stories, encouraging words, assuring words. But the pressure on the 8^th ounce was too much not only for his mental state but for his physical state. He begged me to stop. He pleaded with me to stop. He began to cry. He asked to go to the bathroom (classic). The lab tech’s eyes got big. They expressed their concern the he was going to the bathroom to throw up. I vehemently guaranteed he wasn’t throwing up. I’m simultaneously saying mental prayers with me teeth clenched into my medical mother smile chatting with the lab techs and getting Maelee ready to drink her drink as well (orally thank heavens and much much much easier of a job than getting Orson to do it) while Orson was in the bathroom.

He returns from the bathroom. I slowly and very carefully finish bolusing the full 10 ounces of sugar water into Orson’s gut. He cries a little more but relents to the feed and gets through it. We move to the waiting room. Daughter is grateful she got through the drink. Son is hurting. Oh he’s hurting. He’s trying to put on a brave face. He’s trying to push through it by being excited to get “free” tablet time during his projected two hour wait for the second blood draw. His mental game was strong. He really tried hard to get through it. But his stomach, the same stomach he struggles with every minute of the day was not having 10 ounces of nasty sugar water sitting in it empty of anything else. He makes it about 27 minutes before his very sensitive stomach violently takes over and he vomits everywhere. I was in the splash zone. The tablet was in the splash zone. The carpet and upholstered chair in the semi-private waiting area were in the splash zone.

He was mortified. The semi private waiting area cleared quickly of any other waiters. I walked him dripping and in tears to the bathroom. I told him to wash his hands and wipe up the best he could after I used my purse wet wipes to get him started in the sad public vomiting experience. I also snagged some paper towels and began to clean up the mess and then peaked my head into the lab tech room to let her know of the room clearing vomit. The janitor came quickly. I wanted her to know I had been working on cleaning it up and that I was sorry for her trouble. She kindly assured me it was OK.

I walked Orson back to our lab camp site and sat him down (a couple of chairs over from the one with the caution cone on it in the drying process from disinfectant) in the still empty semi private waiting room. Maelee still had an hour and a half to wait before she could finish her testing with another blood draw. I let the lab tech know that I was going to take my son home (who wants to sit in their own vomit for an hour and a half in a public place or ANYWHERE?) and come back to sit with my daughter. The lab tech with sympathy told me that typically she would not allow me to leave but since my daughter was older and could safely wait alone she would let me. Thanks friend. Thanks.

So my buddy went home and got fixed up while I ran back to the lab to sit again. I felt so guilty and bad for literally forcing him to do the bolus. Long story shorter we are staring another mode of testing for CFRD which is good old fashioned glucose monitoring in July. I say in July because that’s when they’ll go back for their hospitalization determination day. Both of the older CF kids are on watch for a potential summer hospital stay with all-inclusive hospital monitoring and IV antibiotics. Weight and lung function have stayed low enough for enough clinic visits in a row to create concern medically and get them on “hospital watch,” to see if a round of IV antibiotics are necessary during the summer before school starts up again.

It is what it is. They are doing their best. We are really trying hard to be extremely diligent with treatments and to get them moving physically (basketball camp, lots of swimming, family running in place timed sets), to be able to say we’ve done everything we can to avoid the hospital. We hope it works and they stay out but they may still need to go in. Which would be OK and we would all survive it. It would be unfortunate to have PICC lines because it rules out the pool in the sweltering AZ summer but we’d deal. Time will tell. July will tell. So for now we will take June and hold her in our arms and thank her for lots of pool time and family memories made. I'm just now realizing though that if we did come home with IV's that would mean running to IV med's potentially simultaneously  which does make me sweaty.