I check my phone and see I missed a call from my daughter, Danielle. My heart sinks a bit since I know she had a doctor appointment this morning and I have felt for a few weeks now that she’s not doing so well. She’s been tired and I’ve noticed she’s lost weight and sounds congested. The doctors had already started her on an iv antibiotic treatment and took sputum samples to culture. We’ve been waiting 2 weeks to see what organisms she’s growing. So far the tests have been negative, and to a normal person that would be good news. But to a patient with a lung disease such as Cystic Fibrosis it can indicate an insidious, slow growing bacteria such as Cepacia or Mycobacterium Abscessus, also known as MAC.

Today she went to the doctor to see if there is any improvement in her lung function and to decide what to do. You see, she has grown both Cepacia and MAC but if they aren’t causing issues, we don’t treat since the side effects are harmful and the more we treat, the more resistant the organisms get and eventually there won’t be any drugs left to beat down the infections. I’m so afraid that today, the news won’t be good.

I look at my phone as a text comes through from Danielle before I can call her. “I’m now at work but we are starting treatment in a week once results are back. It’ll be two iv antibiotics three times a day and two orals for about 4-6 months. Today I will be sad but I have you all in my corner fighting with me and God! I just want to feel better and beat this thing for good and we will!!!!!!!!!!! Call when you can.”

My stomach clenches, my breath catches in my chest, and my heart races. I call her and she answers. Her attitude is good but tears spring to my eyes. My brain flashes memories of the last time we treated this. The nausea, the weight loss, the severe diarrhea, the exhaustion and her having to hook herself up to med balls every 6 and 8 hours respectively to drip the antibiotics into her veins, and those are just the initial side effects, as she continues she has ringing in her ears, her kidneys and liver start to be affected and then they have to stop her IV treatment and continue with oral drugs that usually are less effective.

I know she hears the tears in my voice and I try to stop them. I smile through the tears but I can’t stop my shaking and the pictures in my mind of what the treatment will do to her, but the alternative is worse, much worse…..

I reassure her that we will help her fight. She has an amazing support system in our family and friends. We will start the prayer chain. She tells me Nick, her brother, talked her off the cliff when I didn’t answer. I’m so grateful they have such a great relationship. Even though he’s married with a newborn baby girl, he answers his sisters phone call and they support each other and are as close as a brother and sister can be.

It makes me think about our family and what a disease like CF can do. It hurts us all, leaves us breathless with worry and pain for Danielle and frankly, for each other. But…. It has brought us closer. We know how to fight for Danielle’s life, we know how to sink to our knees in prayer and how to find joy in the midst of despair. We’ve been told we’re too lenient with our kids, but I disagree. We know the fear we could lose them, it never goes away. So we love them and we love them unconditionally, without holding back because we know how fragile our lives really are. We know how quickly a few words uttered by a doctor can change the way we look at life.

I remember the day they told me Danielle had CF. She was in the hospital because she was considered failure to thrive. She never got above her birthweight and she was 3 months old. The nurses took her from me and brought me into a conference room, sat me down at a table and uttered 10 words that changed our lives forever

“Our worst worst fears are founded, your daughter has Cystic Fibrosis.”

It’s amazing how quickly your life changes with a diagnosis. Things you thought would be horrendous become your new normal. You learn new words, new treatments, how the body systems work, how to hold your child down while they do painful procedures. You learn to fear words such as blown veins, exacerbations, bronchial drainage, sputum, sensitivity reports, resistant strains, picc lines, pulmonary function, liver enzymes, ports, bio hazards, bio security. You become a walking encyclopedia on CF. You absorb everything you can and spit out statements like, “every 20,000 live births result in a CF baby, leading genetic killer of Caucasian children of European descent, 30,000 patients in the US, life expectancy of 18, then life expectancy of 30, then 40 now with Tricafta a full lifespan if there is no damage.”

Tricafta, the miracle drug. It is a game changer. It changes her DNA. Two tiny little pills allows for the chloride channel to work which causes her body to function more normally. We were ecstatic, but our normalcy ended abruptly. She has damaged lungs. She’s still susceptible to dangerous organisms, and now one has her lungs on fire.

“It’s good she was diagnosed young”, they said, “she won’t know any better.” My heart screamed at that statement. I wanted to hit someone,something, or at the very least scream and never stop! It’s not fair. I want her to know how to live! I wish she knew what is was like to not do 2-3 bronchial drainage treatments a day, I would love for her to eat without taking digestive enzymes, to not see a doctor every three months, to not have to haul bags of meds and machines that clear the mucus from her lungs on every single trip she takes. I would love for her to just live!

They say when CF is in your family you go through the stages of grief over and over again. I have those stages memorized. I’m in the anger stage again. I’m angry. I’m very angry. You see Danielle is so happy. she moved away from home. She has a great job she loves and she’s a very hard worker. She often goes in on her day off, even while fighting this disease. She is one they can count on and often shows up when she’s struggling to breathe. She’s tired and that scares me which in turn makes me angry. My anger will dissipate and I don’t find I want to bargain anymore, that stage of grief never made sense to me, but the depression sometimes sneaks in next and usually I will enter the acceptance stage pretty quickly. I’m just a bit uneasy about her treating this bacteria without me. How is she going to do this? How will she hook up to her IV’s around the clock without my help? Who will cook for her when she’s just too tired? Who will search for food she can eat while so nauseous? How will she continue to work?

Danielle and I talked the other day. She’s ready to tell the world about what it’s like to be her. We’ve both have showed mostly the positive side of handling this disease, but she wants people to know both sides. I told her I would help her by writing about her journey in my blog as well. It’s time we bring awareness to this disease. We still have to fight, we need to let you all know what it’s like and to join us in the fight. Pray with us for healing. Pay attention when CF is in the news, and if you’re so inclined, you can donate to the CF foundation to help find a cure. We’re close, and the babies that are born today may never have to fight like Danielle. So far she has beaten down the disease but over the years we have seen so many kids and young adults lose their fight. It’s a long fight, and painful fight and it would be amazing to know that the kids today won’t have to fight so hard just to keep breathing.