I met my husband, Paul, in March of 2006. We talked on the phone a week or so before meeting, but he told me right away he had CF. I knew a little bit about it since I had become interested in knowing what it was all about. I didn't know at the time why I had an interest in it, but when I met Paul, I realized it had to be more than a coincidence. I already knew how very serious it was. However, when I mentioned this to Paul after he told me he had it, he pretty much dismissed by concern.
At the time we met he was doing fairly well. He was working on graduating with his second degree, and soon to be going on job interviews. He had been on disability for the past 18 years, but after the passing of his dad five years earlier, he decided he wanted to see if he could go back into the workplace with a different degree. He wanted it to be something that he knew he could do at home if the time ever came where he wouldn't be able to work out in the public.
At this time his FEV1 was in the 30s and 40s. He got the first job he interviewed for and absolutely loved his job. The only problem was I didn't know enough about CF to know the schedule he was keeping was not conducive to having the most optimal health for a CFer. He was spending a lot of time with me and skipping many treatments. I didn't know at the time how crucial these were to his maintaining the best possible health.
He was almost 49 and I was 47 when we met. His gene mutation was DDF508, the most common as well as very severe mutation for somebody with CF. He was hospitalized a month after we married, which was in November of 2006. We spent our first Christmas together with him being very sick and being hospitalized for two weeks. There were many more hospitalizations, tune-ups, and home IVs while we were married. We did things as best we could like other couples. In reality, though, everything we did revolved around his breathing treatments. We both became diligent in him maintaining a proper schedule after that first hospitalization.
We loved each other very much; however, his illness, his family background, and what I believe were his genetics, made him very angry a lot of the time. It took its toll on me, and there were times of great struggle in our marriage. Paul was very intelligent, graduating with a 4.0 GPA, with him having hopes of becoming a doctor. He shared with me that because of CF, he didn't feel he could pursue that dream.
As time went on, his health began to decline. After 6 1/2 years of marriage, his CF doctor mentioned the word transplant for the first time. It was another year before he was placed on the transplant list. During that time we started thinking about where we needed to live. The area we lived in, in our opinion, did not offer the kind of care needed for adults with CF. It seemed logical to try to relocate. In 2014 we were able to move to Dallas, where his CF doctor practiced. Paul immediately got sick after the move and was hospitalized. He was put on the transplant list, but only for one day. They found he had a mycobacterium infection, and had to go on long-term antibiotics before they could relist him. Later that year he had pre-transplant testing. Since he wanted to continue working, it took much longer to complete, and he did it on an out-patient basis. He finished the testing in January of 2015, at the same time purchasing our first home. He was getting sicker and had to go on oxygen full-time, quit working, and go on disability to concentrate on being as strong as he could when transplant time arrived. It took until May of 2015 to finally get relisted. We were so excited and hopeful.
We got a call in mid-August that lungs were available. However, after being prepped for surgery and spending many hours in the hospital waiting, the donor's family changed their minds. It was quite a blow, but we had to go home and wait until lungs became available again. Meanwhile, our relationship became very difficult. Paul got very withdrawn, moody, and we started counseling. He would rage and it was frightening. I know he was scared, so I tried to internalize my pain and fears.
In March of 2016 we got another call that lungs were available. This time it was a go and Paul was wheeled into surgery. It was a very long surgery, but I was updated periodically by the OR nurse. She informed me everything was going well. When the surgery was complete, the surgeons came out to talk to me. They told me Paul was in very critical condition and had to have numerous blood transfusions. I was later told he had lost 13 liters of blood. He was on ECMO, on three different vasopressors, and every conceivable machine and IV medication. Days went on and his condition continued to get worse and worse. All of his organs were failing, and he would have had to have dialysis at least three times a week or be placed in a facility with full-time dialysis, if he survived coming off of life support.
After 5 1/2 weeks of being on life support, they basically told me nothing more could be done. I knew what they were trying to say, so between sobs and telling myself that he could hear me, I asked him what HE wanted. I got my answer quickly, and had to do the hardest thing of my life. Paul passed away on May 6, 2016, and my life will never be the same. I know he is pain-free and breathing perfectly, but it has been excruciating for me. My wish and hope is that a cure for CF will be found soon, and be eradicated from the face of the earth. Nobody should have to go through the agony the disease brings to the people who have CF and those who love them.