September 18

I wrote this for a CF fundraiser that a friend of mine in Australia was doing. Of course, we cut most of this out, as it is very long. Please do not feel that you have to read it. I figured now would be a good time to post it. Of course this is just a brief glimpse into Amber's life as a CF patient - it is hard to put all of the day to day into words. Even harder to put that last month into words. There isn't a day I don't think of Amber. She was the best daughter a mom could ever ask for.

Amber was just like any other high school girl. She loved shopping, boys, clothes, makeup, hanging out with her friends. Her concerns were mainly grades, getting a special boyfriend, preparing for college, and her job. She also was concerned about making sure no one looked at her differently, that no one pitied her, or worse, avoided her. You see, Amber had Cystic Fibrosis. That made her different from all of the other girls in her high school.

Amber had to have breathing treatments several times a day. She used a PEP valve once she got old enough. It vibrated the inside of her lungs to help her get the mucus out, because her body didn=t thin it enough to get it out on its own. The mucus threatened to scar and clog all of her bronchiole tubes, so it was a constant effort to keep it cleared out. When she wasn't strong enough to use a PEP, she had to have someone, usually mom, pound on her lungs to get the mucus out. She coughed a lot at school, but just told everyone she had allergies, which was true. The medicine for allergies is counterproductive to the medicine for CF, so it was a balancing act.
At home, taking medication was no big deal, but at school it was a completely different story. First, students aren't allowed to carry medication with them so she had to go to the office prior to lunch to take her meds. (That lasted about nine weeks before she just started carrying them and mom dealt with the principle) Second, it is hard to explain to everyone why you are carrying medication and taking it every day when you eat. But, she was able to explain it away. It was extremely important to her that no one knew.

Because Amber was a strong girl, and loved school, she went to school even when she was on I.V. antibiotics. Her veins had been used up by the time she entered high school and so she had a port-a-catheter in her chest. When she was sick enough for I.V. meds, her port was accessed and she got them that way. The bad thing about the port was it's placement. The doctor had placed it slightly above her right breast. This was at a time when she was at her heaviest weight, 105 lbs (47.7 kg). So the placement appeared to be good and well concealed. But then she lost weight and the port stuck out pretty far. She had to work hard sometimes at keeping it hidden, especially when it was accessed since then it had a needle, tubing, and bandages. But she did it...and she went to school as long as she was well enough.

During 10th grade, she had a sinus surgery, her gallbladder removed, a fundalplication (a portion of the stomach is wrapped around the esophagus to help control acid reflux). Her port-a-cath became infected with yeast and had to be removed. She was put on a medication that the doctors and nurses refer to as the Shake and Bake drug because it causes fevers and shaking. She was in the hospital for several weeks over her 16th birthday and once she was released, had to finish the school year at home because of the yeast infection.

Even with everything she went through, she was a fierce little fighter. She never gave up hope that she would be cured. She was planning on a career in nursing and wanted to adopt a baby with Downs Syndrome (having her own baby would have been incredibly risky to her health). Amber had five college credit hours when she completed the 11th grade. She had taken a Certified Nursing Assistant class so she could begin working in her chosen field. She could have graduated as a junior, but for the two required senior year classes. She was planning on taking all college classes her senior year, except for those two classes, and graduating midterm, with more credits than required.

Two days before Amber started her senior year of high school, she got very ill. Prior to that she was working at a video store, practicing cheerleading, and hanging out with her friends. That illness was her last. She had contracted C-Diff. C-Diff is basically harmless to those who don't have compromised immune systems, but for someone weakened by illness, or on long-term antibiotics it is quite serious. Very rarely fatal, unless you are like Amber and it causes your stomach to swell so much that your lungs are squashed. Amber went from having a lungs that worked in the 90% range, to lungs that functioned at 28%. Her infectious disease doctor was able to, finally, get the C-Diff under control, but her lung function never increased.
Unfortunately the damage had been done at that point. She was very weak and her lung capacity was down to 28% still. Her nurse practioner (Ericca - a most wonderful person), started pushing the CF doctor to get Amber started on the lung transplant process, but the CF doc stalled and wouldn't make the decision. Her regular pediatrician released her on September 6 against my better judgment. On September 8th, on our way to the pediatrician (Dee Spade, another wonderful person), because she wasn't doing well, her oxygen saturation rate dropped to 20%. She was rushed to the hospital in an ambulance and we were told she wouldn't make it through the night. She did, and started improving over the next two weeks. Her CF doctor finally started planning for the living donor lung transplant and I thought everything was going to be okay.

On September 17, she got ICU psychosis, and a couple of psychiatrists came in to talk with us, but we didn't realize that it meant anything. They never really expressed to us that ICU psychosis was a sign of impending death. They made it sound like it was just something people got sometimes in the ICU. She seemed fine, just occasionally didn't make sense. For a couple of days prior to that, her infection kept mutating. The infectious disease doctor kept changing her antibiotics and her care, but eventually he said there was nothing left to give her because it was mutating into strands that he didn't have antibiotics for. In the evening of the 17th she really started going downhill. She had a C-Pap (Continuous Positive Air Pressure - it forces oxygen into the lungs) on throughout her ICU stay, but she could take it off prior to that night. She had even gotten to where she only had to have it on when she was sleeping. But that night she got to where it was doing all of her breathing. Her kidneys started to shut down. At one point in the middle of the night she pulled her mask off and said, "mom". I leaned over and told her that it was okay she had fought hard and she didn't have to fight any more. I think that was what she needed to hear because after that she went into a sort of coma. The doctors said that her blood gases wouldn't allow her to wake up and that at that point she didn't know what was going on. (She had way more carbon dioxide in her blood than oxygen). Her pediatrician came in and told me that she wouldn't make it much longer. We could have intubated her, but apparently CF kids who are intubated never come off of it. I made the decision to pull the plug. I had the nurse drug her up with morphine. Even though the doctors said that she didn't know what was going on, I wanted her pain free. After that I took all of the monitors off of her because I didn't want to see or hear that flat line signal. Then I turned off the oxygen. She took a couple of breaths and then she was gone. It was the hardest thing to do, but there was no way I was going to have her stay alive for my own selfish reasons, it wasn't fair to her.

Amber had wondered what her funeral would be like. Her uncle had died a few years before and had a huge attendance. I would say that hers surpassed his. Her high school closed for the afternoon so that the teachers and students could attend. Her brother's teachers attended (he was in first grade). Kids from her previous school attended. All of her doctors attended, which is unheard of, as well as all of the nurses who took care of her in the hospital. I don't know how many people were at the church, but the people who ran the funeral home said it was one of the largest funerals they had ever witnessed. There were more than 200 at the grave site. Amber left an impression on everyone she touched. She was a sweet child, an angel on earth, and everyone loved her.

Cystic Fibrosis is a terrible disease and no child should have to suffer what Amber suffered. Although CF affects thousands of children yearly, very little is known about it. One doesn't hear the term unless they know someone affected, and then they have to search to find out about it. For a disease that affects so many, it is not popular. There are no televised fund raisers; no celebrities are out fighting for the cause; a portion of the proceeds of products doesn't go for research. It is an ignored disease and awareness needs to be raised. These kids need our help.