I realize we are just getting started helping our children and family as a whole unit live and succeed with chronic disease. We have definitely had more experiences in the hospital and at the doctors than an average family, but know there are other families that absolutely have had more experience than us. Watching each of our infants experience at some point an extended hospitalization left us feeling confused, afraid, lost, guilty, and often very angry. In the beginning I remember a toe to toe nose to nose discussion with one pulmonologist on call one day in the hospital with my 7 month old son (who heart breakingingly screamed bloody murder through every IV). The pulmonologist told me to “get used to this mom, you have to get used to this, you have children with a chronic disease you need to expect weeks in the hospital.” I was furious. I told her I refused to accept the hospital as normal and I refused to quit asking to go home as soon as we could as often as I could. She never came back to our room, and I never saw her again. She asked another pulmonologist to handle us (lets be honest me). I think I would like to let her know now that I apologize and I understand. I’m not happy about it, and it still feel angry about it a lot of the time but I understand that being intermittently hospitalized is part of my children’s life. We also understand that our emotions are second to helping our kids do their best to prevail with positivity and hope. Our attitude will be mirrored and magnified in them especially if it’s a negative one.

Sunday, April 5, 2015

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!




 
 

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