Meet the Adams Family : The Short Story

 

Cystic Fibrosis.

13 years ago, those words meant nothing to me. Now they influence almost every decision I make.

 

Before We Knew

 

In 2002, I fell in love with Frank and we got married. Five years later we were preparing to bring our first baby – a beautiful girl – home from the hospital. Our doctor noticed an issue with her stomach and she stayed in the NICU for a week to clear it up. A blood test was taken to rule out a long list of potential causes for the stomach issue. Bottom of the list and least likely (according to our doctor) was Cystic Fibrosis. Neither Frank nor I had any relatives with Cystic Fibrosis, so we didn’t think much of it. However, when the results came back her diagnosis was indeed CF.

 

Unknowingly, both Frank and I were carriers of a genetic mutation called DF508. 1 in 30 Caucasians are carriers of this defect and if two carriers have children, there’s a 25% chance that their children will have two copies of this genetic defect and be diagnosed with Cystic Fibrosis.

 

Initially, we were told our daughter had a terrible disease and she would die young. However, after working with the angels at Phoenix Children’s Hospital Cystic Fibrosis Clinic, we learned what CF means and how to help our daughter not just survive, but grow, learn and thrive.

 

Learning About Cystic Fibrosis

 

In its simplest terms, the salt and water in a body with CF don’t work together like they should. The incompatibility of salt and water in the cells causes thick mucus to form and coat the organs of the body – typically the lungs and digestive system. Although a person with Cystic Fibrosis has a normal immune system, the bacteria that gets stuck in their lungs when they get sick has a very hard time leaving.

 

Additionally, people with Cystic Fibrosis are not able to utilize the natural release of their own enzymes from their pancreas because the pancreas is blocked by mucus. This requires that they take prescription enzymes with all food to try to mimic the natural enzymes needed for digestion and nutrient absorption. If these enzymes are not taken with all food the result is no digestion, no growth and no weight gain.

 

Continuing to Live

 

Despite the difficulties, our daughter’s medical treatments soon become routine and we were blessed to give her a baby brother two years later. He had only one copy of DF508 – so he is only a carrier like ourselves. Two years after his birth, we were expecting our third child, another boy. Prior to his birth he was diagnosed with CF. Now four years later, we are expecting our fourth child. A little girl due in June. Baby Ruby has also been diagnosed with CF.

 

Many people ask how we could continue having children when we knew there was a 25% chance they could have CF. Why would we not seek adoption or fostering a child in need. Didn’t we feel guilty?

 

Yes, feelings of guilt and confusion the past seven years have at times been overpowering. We have wrestled with what was the right decision. In the end, adoption was not the right avenue for our family at this time.

 

Through our darkest times, the overwhelming anxiety about the trials and struggles our children would face and the crushing guilt as we expanded our family, Frank and I have tried to focus on three things –  hope, our faith and the love we can share with our children to give them the courage to face every challenge that will come. We know that Cystic Fibrosis is not forever, but our family is!

 

 

The Cringe of Comparison

 

As ignorant as this sounds, Frank and my attitude about CF is to try not to learn too much out of what our own kids are dealing with currently. You know, keep the blinders on a little bit. Clearly it is silly to pretend that more couldn’t happen, but since our thoughts already wander to the extreme of the disease more often than we like to admit, it’s easier to control those thoughts when we don’t go hunting for extra amount of Cystic Fibrosis information that we currently don’t need to apply. The thought of CFRD (Cystic Fibrosis Related Diabetes), organ transplants (he have heard lots of lung transplant stories but also have heard liver and kidney transplant stories with CF as well), and then the dreaded Burkholderia cepacia (I know I diligently try to avoid any more than what I already know about it), have the same effect as the one scary movie I have ever sat through – literally haunting (12 years and I can’t get this awful movie out of my head seriously).

Knowing our own attitude about in essence keeping to ourselves a bit about our little regimen we duly note that potentially other CF families feel the same way. When you do get into who does what and how long they have been doing it with meds and such it creates new thoughts. Why is my kid on more/less meds than theirs, I wonder if we will have to add that med, are we doing something wrong, etc?

But it is also interesting to note that each step, each med is added gradually. I worry about how we will remember to add another in, stress out about trying to get it done, feel really bad for missing it some days, then finally one day it’s totally part of the routine and fits right in. All of that said here is Maelee and Orson’s morning routine. This all happens before 7:30 am on school days. Sprinkle in the kids eating a good amount of something healthy-ish for breakfast and I feel pretty accomplished when we leave for school.     

Walking Alone to the Nurses Office

First Day of Kindergarten

 

We experienced a common parenting panic attack when we sent our first daughter Maelee to school. We were concerned about all the regular things that parents worry about. Will she be safe? Will she behave for her teacher? Will she make friends? Will she learn all she needs to keep up with the kids in her grade?  

But we also worried about increased germ exposure. Exposure we couldn’t control really at all. Would she start school and then have her first throat culture at the CF clinic after and it contain a dozen new strains of things we needed to treat with new meds? Was she going to get really sick (something that every cough we heard her make while she played during the day or slept at night already brought into our heads anyway)? How would she do with taking her enzymes before she ate snacks and lunch? Would the teacher keep them in her desk and dispense them to her, or would she need to go to the nurse every time? Would she be able to use the restroom when she needed to, often frequently and urgently? Could she reasonably take care of herself without us?

Before she even had her first day of Kindergarten many of these questions were answered. One that as her parents we thought was a huge deal was her having to walk to the nurse every day for snack and for lunch time eating before she could participate in these things. It seemed like it would feel completely isolating. We worried it would be a sad time of day for her, a multiple time a day reminder of how she was different from all the other kids in her class.

We also understood that this was an unavoidable part of her (and any other kid with a daytime med requirement) public education. It would not be safe for her medication to be anywhere but the carefully charted and monitored nurses office. We did all we could to keep a positive attitude about it and check it off as something we needed to help her feel as normal about as we could.   

When school started we began to see Heavenly Father’s hand in this part of Maelee’s life. The women in the nurses office quickly proved to be one of the highlights of Maelee’s day. Cheerful. Kind. Positive women.  The kind of people who anyone would make an effort to stop by and see, only Maelee didn’t have to make an extra effort to see them, she got a specially scheduled time of day where she was guaranteed to. And if for some rare reason she didn’t get in there when she should have, they patiently search her out and help her with her meds. For 3 years there have been a few different faces in the nurses office, one constant, all incredibly awesome.

As her mother I remain to be so grateful to these women who do for her at school what I cannot be there to do, make her feel loved, important, and worth helping. Plus it’s not just my daughter that these women help. Of course every kid eventually ends up at the nurse for all kinds of random reasons, but there is a whole group of kids like my daughter who have daily med routines that cannot be done only at home and require the nurses specialized aid and ability. I have had the chance to see a handful of the other kids with these med routines in the nurses office as I have visited Maelee’s school. The nurses role in their day and the boost of love and care that these women give these kids who need to feel normal when doing things no other classmate is doing is constant.

 So the answer to one of our parenting worries about Maelee making friends at school is “yes” she did! We just didn’t realize that one of her best friends would be the school nurse.

 

Cheering for the Poop Brigade

 

My Mom is a huge parade fan. So was my grandma. When my grandpa retired from working for the Arizona Highway Department they paid for all their kids and grandkids to go to the Rose Bowl Parade and Disneyland. It was so fun and my first memory of cheering for the poop brigade.

You know the ones, the group of people who follow behind the horses with a trashcan on wheels and big snow shovels and brooms. It is an absolutely necessary job, but maybe not one you would pick if you were picking from all the parade jobs. Perhaps you would want to ride the horse and wave to the crowd. But someone has to pick up the poop so that no one else steps in, well you know.

My family grew up in Tempe, Arizona a city that hosts the Fiesta Bowl Parade. My family attended every year while we were growing up. My husband is from Show Low so we have had the chance to attend their magical 4^th of July parade, and even got to see parades in other beautiful Arizona towns such as Eager and St. Johns. Each parade has horses, each parade has a poop brigade.

Sometimes the poop brigade has hilarious outfits on. Tutu’s, clown noses, etc. Other times it is just a group of people who are wearing regular clothes doing their best to shovel the poop. It does seem though that the ones who dress up have a little more fun. They add to the parade, making their job as fun to watch as the people riding the horses and waiving.

Keeping a good attitude about the things in our life that can be compared to shoveling poop is a very hard thing to do. Choosing to wake up and put on your tutu and clown nose can sound harder and require more effort than just showing up for the parade and not making eye contact with anyone while you do that job that has to be done (good for you for showing up for the parade anyway though and not staying in bed).

Having a daily ongoing relationship with Heavenly Father through prayer and through the peace and comfort that can come through reading the scriptures and words of the Prophets has the power of tipping the scale to the tutu and clown nose outfit choice when we have to make it each morning.

We recently met with the surgeon to discuss Orson’s new button (gastrostomy tube) and what kind of care it requires. He is going to have to learn how to shovel some poop with this new part of his life. We are blessed though to know that he will have the help he needs because we know about prayer, and we know that the words of ancient prophets and the words of today’s prophet (General Conference is this weekend!) have the ability to lift our souls to the levels they need to be at to put on our tutu and clown nose and have a good attitude about the poop we shovel. Hooray for the poop brigade! Keep up the good work guys!

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Here is a link to a video about the 185th Annual General Conference of the Church of Jesus Christ of Latter Day Saints happening this weekend April 4 & 5 where you can hear the council of today's prophet Thomas S. Monson.

https://www.lds.org/church/events/april-2015-general-conference?lang=eng

 

 
 
 

"Cover Them Up" He Said?

This has been a very surprising and intense month. We never expected that our little idea for a bake sale fundraiser would turn into what it has. One of the things that really shook things up was when Vistalight Productions volunteered to make a video about our family to raise awareness about the fundraiser and why we were doing it.

 

Frank and I were both nervous. Him because he would rather be able to be completely off the grid in all aspects of his life (you know live at a high altitude making furniture from the cast off branches of mother nature never seeing anyone or anyone seeing him type thing), and me because I am easily flustered and stutter and don't feel like I can express how I feel when I speak as well as when I can write it down.

 

We know the company though and trusted them and were extremely grateful for the opportunity it was and their generosity. I did exactly what I thought I would do, rambled on, told side stories, etc. Somehow, someway they worked their magic and made a fantastic video. It was never my plan to mention not having a place to plug in the new equipment that Ruby would need. I had thought about it, but figured we would cross that bridge later on. But my comment about it made the edit. It was another way that Heavenly Father would bless our children and our lives.

 

Each CPT vest needs its own designated plug or it voids the warranty for the $15,000 machine, they use a lot of power. Every morning even now we have cords stretched all over creation to make treatments work.  Also needing an outlet are 2 (soon to be 3) SVN machines to be used simultaneously with the vest.

 

A man who doesn't know our family donated the work of an electrician to come into our home and add all the necessary wires, conduit, outlets, breakers, and other electrical words I don't know so that we can all be together during treatments. This effects our family now in providing ease and accessibility to the machines that we use everyday. It will also effect the future of the kids. We have attended a CF Family Education day at PCH when a nationally renowned CF doctor gave an hour long talk on studies that have been done on CF patients and how when they have parents who will sit near them, be in visual sight, make their presence known during the patients treatments that in turn these CF patients will see the value of doing their treatments and have a much higher chance of doing them regularly on their own as they leave the home as an adult. No one will have to go into a separate room to do their treatment, we will continue to be together. These new electrical outlets are HUGE for us!

 

The very nice electrician who came over to do it was talking with me about where would be best for the new outlets. In the room we have the plugs are your standard low to the ground outlets which is tricky because furniture blocks most of those and needs to be moved when we do use them. I asked if we could put the new ones up a bit higher above couch level. He said we could, and then suggested that I could find a way to cover them up if I wanted so they wouldn't stick out.

 

We have them now! He came yesterday and worked hard to get them beautifully installed. We are totally ready for Ruby's vest and can easily access the outlets. Cover them up? Absolutely not! They are a totally wonderful unexpected blessing and we are incredibly pumped to have them!

 

Again, this is something we could never have done on our own and could never have imagined that Heavenly Father would prompt a complete stranger to help our family in this way. Thank you, thank you, thank you!

 

“Peace Be Still” When I Needed It : The Male Nurse

 

One of our family’s favorite hymns is “Master The Tempest is Raging.” I am not a singer AT ALL, but this one is just fun to sing. It takes you from the terror and misery of being in a storm in the middle of the ocean to the instant calm and sweet “peace be still,” that the Savior brings.

Singing this hymn is a beautiful way to remember the end result of a common pattern in life, at least my own life. An unexpected (or even anticipated) trial comes and I totally freak out, then hopefully (sooner than later) I can remember to ask the Savior and Father in Heaven for help and somehow I get the “peace be still” that I need to shoulder the trial until again I break, freak out again, and the cycle starts over.  

It is impossible to tell how Heavenly Father will provide our “peace be still,” but I have seen in my life that when I sincerely ask for help from Father in Heaven I will get it, somehow, and usually in a way I could never have guessed.

My 7 month old baby was in the hospital and I was 100% certain that it was my fault. For a week I had desperately tried to get him to eat but he wasn’t interested. I even stopped nursing and started pumping so that I could measure exactly how much he was eating but to no avail. It quickly snowballed and in the time of 16 hours we had seen our PCP, our CF team, triaged at the ER, and now were being wheeled into our own room of the PICU at 9pm.

Frank had gone home to take care of the other kids and the unintentional hurtful question had just been asked to me by the girl running the babies IV, “how could you let this happen,” and there I was there sitting in a little row boat alone in the middle of the ocean with the “tempest raging” around me of guilt and despair.

I sat there thinking about how I couldn’t get my baby to eat and look at what I did, ended him up in this hospital bed, away from his soft crib and family to get stuck with needles and pumped with medicine. Orson was being still and the IV was in so I just sat there with the things I had thrown together in the hour before we left for the ER and stared into space. I was hungry, I hadn’t eaten dinner, I was worried about the next time I was going to pump because I knew that the nurse would be coming in and out frequently - and for the first time in my stay in any hospital it was a male nurse - and I am admittedly a prude.

Soon the male nurse, whose name I really wish I could remember, came into the room. He attended to the machines and the baby then looked over at me. He asked if I had eaten anything then offered to get me a sack lunch from the cafeteria because it was closed and I wouldn’t be able to get anything myself. Then the next thing he did was notice that I had brought my pump. He said, “I see that you brought your pump. I will bring in plenty of sanitized bottles and some labels for you and I can get them put in the freezer for you anytime you say. I will keep the curtain closed to my nurse station and leave you guys alone for the evening as much as possible.”

I didn’t even say a word about being hungry and stressing out about when I could pump next, but that incredibly kind male nurse somehow knew what I needed and didn’t hesitate to provide what he could. For the moment while I ate my sack lunch (most appreciated sack lunch I have ever eaten) and before the guilt and blame storm cycle would start again he had been how I was given my “peace be still.” My Savior knew how I was feeling and Heavenly Father heard our family prayer before we left for the hospital, and the plea for help we asked of Him on behalf of everyone in the family. It was very clear to me that I was being watched over and walked through my personal little storm that night.  

Mesa family expecting 3rd cystic fibrosis child - East Valley Tribune: East Valley Local News

Mesa family expecting 3rd cystic fibrosis child - East Valley Tribune: East Valley Local News