Last year I left my own six children in capable hands to travel up to Virginia. My baby sister, Ann, had her third baby and I wanted to be there, to help and play with her other kids, and make dinners, and clean, the things women do for other women when we bring new life into the world. So, I overlapped my time there with my mom's (because we like to play, too).Ann's firstborn is Mary Deane, the namesake of our maternal grandmother, lover of high heels and all things blue. Mary Deane was born with Cystic Fibrosis, a genetic condition that wasn't routinely tested for at the time, but now is mandatory in 37 states. A condition that shortens the life span of it's victim.
But, let us wind the clock back even further. Let's say 20 some odd years when Ann was a child. Ann was the meticulous type of child that liked to know all the facts. Not just the pertinent ones, the superfluous ones as well. When a medication was administered for whatever ailment, even a relatively benign one, Ann was the type to not only read every word on the back of the bottle, she would pull out the elephantine pamphlet with it's abundant mapfolds, smooth it out over the counter, hunch over, and read every warning and caution, quite possibly also in all 10 languages.It drove my parents nuts, that she just wouldn't accept their word that what they were giving her to swallow was okay(!). She needed to find out for herself and sometimes emphatically inform them that she could have a seizure, or paralysis, or even die! Little did any of us know that she was given the tenacity and the persistence that she would need for her own children.
When Mary Deane was born, Ann knew, as a mother usually does, even when "they" discount your feelings, that something was not right. She went to pediatrician after pediatrician until finally, one actually listened. One who asked questions that might not come up in a normal exam such as skin saltiness. This doctor listened and suggested they test for Cystic Fibrosis.
Ann and Brendon are both carriers of the gene, unknown at the time, and have a one in four chance having a child with CF, a 50% chance of having one as a carrier, and a one in four chance with no CF gene at all. That there is my 9th grade biology manifest, thank you very much. ;)
"1 in 3,700 children are born with CF. Cystic Fibrosis (CF) is a lifelong, hereditary disease that causes thick, sticky mucus to form in the lungs, pancreas, and other organs. In the lungs, this mucus blocks the airways, causing lung damage, making it hard to breathe, and leading to serious lung infections . In the pancreas, it clogs the pathways leading to the digestive system, interfering with proper digestion. In 90 percent of cystic fibrosis cases, the airways are affected. In the 1950s, few people with CF lived to go to elementary school. In 1985, the median survival age was about 25 years. In 2007, the predicted survival age was 37.4 years. Thanks to dedicated researchers and earlier diagnosis, the age of survival for patients with CF continues to increase" sourceWhen I stayed with Ann, she taught me what needs to be done for Mary Deane when she is feeling well, including medications, nebulizer treatments, the vibrating vest (I'm sure there's a more technical lingo) that loosens the mucus in her lungs, and then the enzymes that are essential for her to have before food to help her absorb fat, both administered through her G-tube. It's such a feeling of awe to feed someone and watch the food go directly into their stomach all the while watching cartoons on the computer without so much as a glance in my direction.
Ann said to me one day, "you and mom are the only ones who really understand what we go through each day." Because it has to be learned, and you can't just run out the door for a fun outing because everything is revolved around getting each medication and procedure done at the right time. It is about milking every day of her life, as much as possible.
Ann and Brendon went on to have sweet Clark, a nod to my mother's maiden name, who is blissfully CF-free. The "easy" one. And I have never seen siblings who play together more peacefully and kindly as these two do, because they are best friends.Then came Hyrum who was thought to have CF because a mass on his lungs was found inutero. But, it turned out to be unrelated to Cystic Fibrosis, though worrisome nonetheless.
And now we are close to present time. Mary Deane had a surgery scheduled to enlarge her sinuses and remove her adnoids, and was supposed to be quick, easy, and simple. That was going to be the smooth surgery. Hyrum had a surgery scheduled for days later to remove a portion of his lung that was several hours long.
My mom flew in, once again, to be there to help. Mary Deane had her surgery and all was thought to be fine. She returned home for recovery. Days later Ann and Brendon took Hyrum in for his surgery. Meanwhile, Mary Deane began throwing up blood profusely and was rushed to the hospital by ambulance followed by an emergency surgery to stop the bleeding.
Over the course of the next week, Ann and Brendon were torn over which hospital room to be in, which child to hold, and wondering what was happening.
There was another emergency surgery for Mary Deane, more ambulance rides, and at one point, she had only 50% of her blood keeping her alive. All week long texts were dinging between family members, and asking for prayers and offering prayers. So many prayers, and so many questions, and faith being tested to what felt like the very capacity.
At one point Ann asked my mom in the hospital room if she thought Mary Deane was going to live. My mom responded that she had, up to this point, tried to hard to be positive, but just wasn't sure anymore.Ann said that she was willing to give Mary Deane back to God if it meant that her suffering would end.
But she never let her faith waver that all was in His hands. We all watched her with her tenacity and her persistence in her belief that there were things to learn in this trial and that she did not doubt God's path and knowledge of Mary Deane's sweet life.
Hyrum's surgery went so smoothly. His body adjusted and his lungs did just what they were supposed to do.Mary Deane, being the feisty soul she is, finally got her smile back, which meant her body was not giving in.
Her one request was to see grandma in her bathing suit. Haha!Clark had been scooped up by grandma and cared for in the midst of all the chaos.
While I was there, Ann told me of an experimental medication that was just getting ready to be put on the market, it doesn't cure, but greatly helps those with CF. There are several types of CF and it happened to be for the type that Mary Deane has.
But it isn't released until September. And you have to be 6 to take it.
Guess who turns 6 in September?
I have followed your blog for a while now and as best as I recall, have never commented. I totally understand what your sister, bil and sweet Mary Dean go through in a day. My youngest daughter, now 13, was diagnosed at 5 with cystic fibrosis. It is a horrible disease. We no longer use the Vest for mucous but instead use a percussor...much like a mini jackhammer that I can use to concentrate on problem area's on the lungs. I am glad to hear Mary Dean will be able to use the new meds when they become available in September...unfortunately, my daughter is not a candidate this go around. I will pray for your family.ReplyDelete
Loved the pictures and sharing their life and love with us. They are in my prayers. Such wonderful faith your sister has and beautiful news about the medication trial!ReplyDelete
My kids call the vest "bumpy hugs"! My sister's husband has CF so we've watched first hand the treatments, coughing fits, hospitalization, etc.. But he is still alive and kicking at 39 years old thanks to tenacity, lots of luck, and a rigid treatment schedule. So glad that MD was able to recover! MD and her family are, as always, in our prayers. Here's hoping for a cure! #cfsurvivorReplyDelete
Thank you for sharing your family with us! Please know they are in my thoughts and prayers. I know how having a special child can be difficult! The feeling if helplessness when you watch them in their hospital bed. To know their little bodies fight...always!! Mine is 14 and she shines!! On good days and bad! A true blessing in our lives!ReplyDelete
wow. just...tears. as any mother would. empathy is described as feeling your pain in my heart. and right now, that's exactly how I feel for you sister and family. <3ReplyDelete
HAce tiempo que sigo tu blog, pero no suelo comentar. Creo que en un momento así hay que dejar unas palabras de aliento. Espero que todo vaya bien y tu sobrina pueda llevar una vida lo más normal posible. Le deseo lo mejor a tu familia. Un abrazo enorme.ReplyDelete
So glad to hear that MD is doing better and qualifies for the new med. One of my best friends has CF. Reading Mary Deane's story reminds me a lot of hers. She writes a great blog about life with CF. If you want to check it out you can read about her here: http://www.cheriz.org/.ReplyDelete
I am in tears reading this post. I lost my sister in law and unborn nephew to CF almost three years ago. My sister in law, Nicole was 6 months pregnant when a lung bleed brought her into the hospital. During surgery to stop the bleed, my nephew was deprived of oxygen for too long and died. Nicole followed him to heaven 10 days later. This is such a horrible disease that desperately needs a cure. My prayers are with your niece and family. Keep fighting Mary Dean!!! I prayer that one day CF stands for Cure Found xoReplyDelete
I never could imagine the heartache that comes at the thought of losing a child until I had one of my own and now I have tears in my eyes for your sister and little Mary Dean - What a woman and what a family you have?! She's a lucky little girl to have so many that love her so much!ReplyDelete
I didn't realize it, but I held my breath until the end of this post, and tears formed when I read that they both are OK. I cannot imagine what this whole experience was like for all of you, but I am so glad there seems to be light in the near future. You are all in my thoughts.ReplyDelete
I am so glad that you have such a wonderful supportive and close family.
Much love to you all.
prayers for your family. what beautiful photos for a treasured family. xReplyDelete
I was reading this and I thought what a wonderful, wonderful, loving family. Mary Dean, you fight on, keep fighting that fight! You are a tiny super hero to me. Many prayers to you and yours.ReplyDelete
Thank you for sharing! This is such a powerfully written and beautiful post. You and your family are so inspiring. I wish the very best for you all.ReplyDelete
Bless you, dear Katy.ReplyDelete