As our first born child, Jason and I were so excited to learn that we were having a baby in 2005. In December of that year, we went for our 20 week ultrasound thinking we would learn if we were having a boy or girl. We did learn that we were having a little girl, but we also learned that she had a condition called echogenic bowel which meant her bowel appeared too bright, or dense, on the ultrasound. This sounded relatively harmless, but it was explained to us that in 2% of cases it was indicative of Down's Syndrome, 2% was other birth defects and 2% was indicative of Cystic Fibrosis. In the remaining 94% of cases, an echogenic bowel didn't mean anything. Numerous measurements were taken that reduced the likelihood of birth defects or Down's which largely left Cystic Fibrosis as a concern. While certainly frightened by the possibility, we took comfort in the knowledge that no one in either of our families had ever had CF. Within a few days, I had bloodwork drawn to determine if I was a carrier. The first of a number of periods of waiting then began. It was nearly two weeks before the results came back that I was a silent carrier for Cystic Fibrosis. Now panic began to set in. It didn't matter that I was a carrier so long as my husband wasn't, so his bloodwork and another two week wait commenced. Again, we learned the shocking news that he was also a carrier. With this knowledge we knew that our child had a 25% chance of having CF. Now the real waiting began. We still had three months before our daughter would be born and could be tested for CF. We gained what knowledge we could and focused on the excitement of becoming parents.
On April 6, 2006 our miracle arrived. At 7 lbs 8 oz., Jayla was beautiful and by all appearances healthy at birth. Blood was drawn and sent off to determine if that was reality or an illusion. The pediatrician told us that a short wait for results would likely be bad news and a longer wait likely meant good news. In my mind, I hoped not to hear from him before Tuesday and focused on not hearing anything before Thursday. On Wednesday morning, the call came. I didn't know what Wednesday meant! The news was simple and stark... and completely devastating. Jayla was positive for Cystic Fibrosis. I always thought it was a figure of speech, but at that moment it truly felt like the world stopped and everything shattered. I held my tiny 6 day old baby and rocked her as I sobbed. At that time, all I could see or imagine was losing her, burying her. I absolutely cannot describe how devastated we were.
We moved in a daze of desperation and sorrow that lingered for some time, but we also began down a path that no one would choose to travel. Within a few weeks, we attempted a sweat test to confirm the diagnosis. Jayla's tiny body was too small to produce the sweat necessary for valid results but the process caused her leg to twitch violently and evoked a profound reaction from this new mom! We met with the CF team and began a treatment regimen that initially included pancreatic enzymes and albuterol.
Those early days were so overwhelming as we struggled to grasp what Jayla's diagnosis meant. Just as we grasped a treatment regimen, additions or changes would be made that would again throw us in a tailspin. We struggled to keep Jayla's weight up and made deliveries to the hospital of bowel movement samples to establish pancreatic insufficiency.
There are still days when Jayla's diagnosis is completely overwhelming, but it's no longer every day. We understand what CF means for Jayla, and we've learned how to help her fit it into her life without allowing it to rule or define her. Today, Jayla takes a host of medications in the course of the day including inhaled mediations like albuterol and Pulmozyme. She spends a total of an hour and a half a day hooked up to her compression vest that shakes her to loosen mucus (I'll try to figure out how to post a video of her doing her therapy on here later). Other medications include pancreatic enzymes anytime she eats, specially formulated vitamins, flonase nose spray, allergy medications, appetite stimulants and antacids.
We have been very fortunate that Jayla has maintained relatively good health but having CF means there will be hiccups along the way. Jayla was first hospitalized when she was two after being diagnosed with pseudomonas, a particularly dangerous lung bacteria. She was inpatient for a week, and then we were sent home to complete her IV antibiotic regimen. This was something I was completely unprepared for. I was terrified to inject medications into my small child, but I was also desperate to go home. It's funny to me how much stronger we are then we give ourselves credit for. I learned that I was capable of administering these medications and have on several occassions since then.
As Jayla began preschool, she struggled with more frequent illnesses but was able to recover well. Kindergarten this year has posed a greater challenge. She has been diagnosed with pneumonia twice and a parainfluenza infection. In the last year, she has twice been hospitalized. The first hospitalization was for eight days with home IVs following discharge. During this hospitalization Jayla also developed a bowel obstruction that required aggressive treatment. Remarkably, she was able to drink magnesium citrate for 9 hours to avoid having a nose tube put in! This past September, Jayla was again hospitalized, this time for the full two weeks as she struggled again with bowel issues and a reaction to her IV medication. Since her discharge several months ago, Jayla has been doing great. We continue to struggle with weight gain, but Jayla is otherwise back up and running!
Besides hospitalizations, there are lots of hiccups along the way. Weight gain is always a challenge for us, largely because Jayla is so tall it's difficult to keep up! Jayla has learned this is a point of power for her, so we unfortunately have frequent power struggles to keep the calories up. I've begged, pleaded, threatened and demanded. On one occassion, I ultimately threw my arms up and, in tears, told Jayla how sad I was that she would choose not to keep herself healthy when that was the absolute most important thing to me. My amazing then-three-year-old wiped my tears and put her hands on each of my cheeks. She looked me in the eyes and said, "Don't cry, mommy. I'll eat" and began eating that same night. We're learning not to make it about the power!
Jayla is a wonderful advocate for both the hospital and for Cystic Fibrosis awareness. She does an excellent job explaining CF to anyone who will listen - which has, on occassion, included stopping strangers at Walmart! She also loves MU Children's Hospital and all the staff. She frequently asks to visit "her friends" at the hospital and is generally sad to go home after a hospitalization!
We are so fortunate to have this amazing little girl in our life. She is intelligent, articulate and compassionate. We have eagerly shared our story in an effort to support the hospital and CMN Hospitals, and Jayla has participated in a variety of media campaigns.
While we are thrilled by Jayla's designation as the Champion child, she has recently assumed an even more exciting role, that of big sister. Our family welcomed our son into the world in February. Initially, Jayla was devastated to learn that her brother would also have CF. It took me awhile to figure out why she was so upset. She thought it meant that we would not be able to bring the baby home or, perhaps worse, that she would not be allowed to return because she knows that kids with CF are not allowed to play together due to germ control challenges. Once she found out that this rule wouldn't apply to siblings, Jayla was so excited. The arrival of her little brother means that Jayla will have someone to share her CF experience with who will be able to understand in a very different way. Jayla is also very excited to be able to teach her brother all about CF and the treatments that he will also complete.
I will work hard in the coming weeks to share more on this blog about our lives with CF and preparations for our role as the MO Champion's family. In the meantime, enjoy Jayla's story in the form of a photo montage set to her Radiothon piece
(Radiothon montage) and the video that was created for the Champion program
(Miracle Video).
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