Tuesday, March 3, 2015

The Original LONG Story

   
     

                    I met a man in 2002 that really impressed me. He was to me very different from the other guys I had dated in all the best ways. I knew he was a hard worker and full of goodness. He treated me sweet and he loved that I laughed at all of his jokes. 5 years later we had been married, both graduated college and had our first baby. We were about to take our baby girl home from the hospital when a problem with her stomach was noticed and we stayed a week in the NICU clearing the problem up. The doctors gave us a long list of reasons why the problem could have happened. At the bottom of the list, least likely we were told, was that she could have Cystic Fibrosis. A blood test was given to our baby before we left the NICU to rule out this possibility. A few weeks later I hadn’t heard anything back about the test but the thought kept coming to my mind that I should find out the results.

                     The result of the test that had been lost in the shuffle at our previous pediatrician’s office revealed that our baby absolutely did have Cystic Fibrosis, in her case 2 copies of the DF508 mutation. We had never heard of CF and had no family history on record of anyone having it, but non-the-less this genetic mutation had been passed both in my husbands family to him and in my family to me - through a long line of ancestors. The difference between our mutation and our daughters was simply that we had one copy of the mutation, making both my husband and myself only carriers of the disease.

                     This genetic mutation is found in 1 in 30 Caucasians. When Frank and I had our first child we had no idea that we both were carriers of one heterozygous copy of DF508. Since we are both carriers of DF508 it means that there is a 25% chance each time we have a baby that the baby will receive 2 copies of our mutation which would mean that they have a Cystic Fibrosis.

                      We had some scary experiences in the first few weeks and months of discovering the disease, including having our first and short lived pediatrician tell us that our daughter had “a terrible disease and would die young.” It seemed like all we could find our see online was memorial websites and horrific stories of children and teenagers who were suffering and dying from this disease that was foreign to us. We tried to learn all we could about Cystic Fibrosis and began to then mistakenly view our daughter as a time bomb. We weren’t all together sure how it would happen but we had been told she was going to die soon. We are so thankful that we have more knowledge and understanding of the disease now.

                      Help came to us when we met our team of angels at Phoenix Children’s Hospital Cystic Fibrosis Clinic. They taught us more about the disease and helped us to get a grip on the situation (also they gave us a referral to a more CF knowledgeable pediatrician). What it means for a body that has Cystic Fibrosis very simply is that the salt and water in that body don’t work together like they should. This seemingly simple function of the salt and water not working properly together in the cells of a person’s body causes thick mucus to form and coat the organs of the body. The organs that this super thick mucus effects the most in CF bodies are the lungs and digestive system. Although a person with Cystic Fibrosis has a normal immune system the bacteria that get stuck in their lungs to make them sick have a very hard time leaving. It was explained to us that each cold and respiratory problem our daughter would get could be compared to nickel and dime damage to her lungs. Getting into the digestive system, people with Cystic Fibrosis are not able to utilize the natural release of their own enzymes from their pancreas because their pancreas is blocked by the 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 resulting in constant diarrhea. I have given a very basic description of CF and the workings of meds and the CF body is much more complex than I can write myself.
           
                        Our second child, a boy was born two years later with only one copy of the DF508 mutation making him a carrier of the disease like my husband and myself but he did not have Cystic Fibrosis like his sister. It was very strange to raise a baby without CF and not have to mix applesauce and enzymes with all of his food and do breathing treatments and lung therapy every day. He was however born with a very sensitive and caring heart and sits with his sister during all her treatments and has always been a big support and cheerleader for her.
           
                         Our third child, another son, was born two years later. We found out that he had Cystic Fibrosis before he was born through amniocentesis. This threw a huge wrench into our family as the time and effort put into daily medical treatments doubled, doctors’ appointments doubled, the time on the phone ordering meds doubled, and the cost of Cystic Fibrosis doubled.

Orson

           
                       My husband and I are both active practicing members of the Church of Jesus Christ of Latter Day Saints. This faith brought us knowledge of the plan that our Heavenly Father has for all of the children on the earth. We both have sure testimonies that we lived with our Father in Heaven before we came to earth and that we are here on the earth now to be a part of a family and have joy and trials and persevere and work towards returning back to our Heavenly Father again after our time on earth ends. This knowledge brings us peace because we know that Earth is not the end for us, our children, or our family unit.
           
                       Despite this knowledge of eternal life and “the big picture” that we are here to work as families to return home to a loving Father in Heaven my husband and I began to go through a vicious and dark struggle. We went in blind to the knowledge and had one baby with Cystic Fibrosis not knowing it was a possibility, then we made the choice to have more children. We saw the work and the life long trial our children would have to face and were currently facing to take care of their bodies and we felt it was all our fault. How could we do this? How could we choose to bring people to earth who we knew had a chance of walking with this disease their whole life? The guilt was then and can still be now extremely powerful and the blame and fault seemed obviously ours as parents. We did this to our children. We gave them this disease.
           
                         Then light would come into our struggle. Heavenly Father would bless us with the beautiful experiences that can happen in families where you see through the grind of the day and into the beauty of watching our children grow and learn and do amazing things. Those were the times when we saw that Cystic Fibrosis, just like any other physical or mental challenge doesn’t matter when you know and can feel the love that God has for us as His children. We would see the big picture again. God knows why we have our specific trials in life, we do not. We do not need to know why we have struggles, but we do need to try to be willing to show our loving God that no matter what happens we trust in and have faith in His love for us.

                       I recently re-discovered a talk by Elder Lynn G. Robbins. His talk puts into words better our conflict. My husband and I struggled when we saw ourselves and our decision to have more children through the “eyes of men.” When we did that we felt bad and dark and miserably bitter, but when we had the “courage to do an about-face,” and remember that as individuals and as a married couple we stand accountable to God only, our choice was then seen as it should be – a joyful and totally awesome blessing from Heavenly Father to get to have these children be in our family. There was not “fault” then in the CF, but it became just another part of helping our kids grow up knowing that even when things are very hard we are loved, guided, and blessed by our Father in Heaven AND most importantly that we can totally do hard things.

                      Throughout this struggle we were having after our third son was born, my husband and I would have stretches of time where we would talk about more children. It sounded crazy to us to have any more. Then one day one of us asked one of those silly “what if” questions. “What if Cystic Fibrosis wasn’t on the menu for us, would we have more children then?” The quick and easy answer for both of us was YES, of course we would. With the answer to that question ringing in both of our ears for the next year or so, and through other sacred and special experiences to us we are expecting our 4th baby four years after our last was born. Her name will be Ruby when she is born in the summer of 2015 and we found out this month that she also has Cystic Fibrosis.

                       As we continue to live our struggle and are trying to let the light of God’s love into it as much as we can, we are choosing to instead of being bitter – to be better with God. We are so excited to be “even steven” and have two beautiful girls and two handsome boys in our family. Our kids are going coo-coo waiting for this baby who is half way here to be born. They dreamily talk about her every day and kiss my belly and cheer for the day they can hold her in their arms and love her stinking guts!
           
                         As a mother and woman of action I have hatched a plan to help prepare. I want to have a fundraiser before Ruby is born for the Cystic Fibrosis medical expenses our family pays and will need to pay. There are several pieces of medical equipment that we need at our house. The grade of nebulizers we own for our two CF kids are not meant to be used as much and as often as we use them and we have replaced them several times because of this. There exists a more “industrial” grade model. Insurance does not cover this equipment and I would like to raise the money needed to buy one each for the three children who will be needing them. Another tricky part of CF is that it is detrimental for people with CF to be in close proximity with each other due to cross contamination - and by no means ever share equipment. Clearly our three CF kids share a home, but I bring this up to note that sharing medical equipment is out of the question so three of everything will be needed. Our home currently has two CF “vests” for our son and daughter to use daily to shake the snot out of their lungs. Ruby will need one too. Retail these machine’s cost upwards of $15,000. Insurance covers a percentage of the vest but we will need several thousand dollars to be ready to buy Ruby one so she can start benefiting from its use as soon as her body is big enough (our son was able to start using a vest at 8 months old). Our son will need a stomach port placed at some point this year because his growth is not what it should be so we will need to work on saving the money for the surgery and equipment necessary to use the port once it is put in.

                      These things I mentioned above are needed on top of the monthly basic CF prescriptions to buy (when no extra antibiotics are added in basic CF meds for each kid after insurance pays their part for our family come out to about $150 a month per kid – we will have three kids this year needing those meds every month), when special antibiotics and medications are needed such as TOBI that adds another layer of cost (depending on the insurance we have had TOBI has cost us $1,500 for a one month supply out of pocket), specialty doctor appointments (the kids do PFT Pulminary Function Tests quarterly throughout the year, we are charged $500 for this 10 minute test to be done on top of the cost of the specialty doctor appointment. This is just the cost for one kid to do the PFT one of the four times a year they need it, our second CF child has started to do PFT’s this year), lab work, chest x-rays, equipment for the nebulized medicine, and nutritional supplements needed for our CF kids adds up to a small fortune. We have been trying hard to keep up with it for the past 7 years but it has been difficult.
           
                      The fundraiser or bazaar will be held on April 11. Each family member will all have a table where they will be selling things unique to them. The youngest will sell his homemade “light sabers.” Our middle son will be selling model robots. Our oldest daughter will be selling hand drawn cards. I will be selling handmade dolls in honor of Ruby our soon to be born baby girl. My husband will be manning up the limited edition t-shirts and wrist bands. Our slogan (if you will) is "CF Is Not Forever" because WHO doesn’t like a good novelty t-shirt! We plan on making it a fair/carnival type experience and a fun family activity. Please join us on April 11th - start time 4:00pm. Smoked BBQ dinner, delicious baked items, salsa, silent auction, raffle, boutique items, bounce houses, and unlimited amounts of family fun. Location Cardon Park 2540 E. Fairview Mesa,AZ 85204.

1 comment:

  1. Hi! I'm Jennifer...a friend of Jami's. :) I'd love to help with your fundraiser! I can help set up, clean up, whatever you need!
    My cell # is 480-435-2260

    ReplyDelete