The ' T ' word

I’m finally wrapping up my 2 week hospital stay. Between my PH diagnosis and testing, Emily’s death and now lastly, transplant, my mind feels like blubber and I’m still exhausted.

Transplant Evaluation.
Did I just say that?
Or did my head shake and wake me from a dream.
Maybe some sort of denial is preventing me from actually believing that my doc is suggesting it’s “T” time. Time to think about being listed for a double lung transplant.

Now I have always known that the transplant decision was in my future. But it always seemed so far off in the distance. A place I saw myself physically and slowly moving towards, knowing that the time would be crystal clear in my thinking. So why do I feel like the timing is not right? I don’t feel THAT sick yet. Usually, lung transplantation is recommended when a patient’s lung disease is so severe that their chance of surviving with their original lungs is estimated to be two years or less. Am I actually there? The lung transplant itself carries it’s own risks and set of survival rates. About 1/2 of recipients will last 5 years or more post transplant. So of course, it would only make sense that I would want to get the most use of my own God given lungs, for as long as they are still able to function. After all, they have given me 54 years, and counting, of life.

Yes, I have been pretty sick this year, with 4 hospitalizations in 8 months, which is too much. My left lung is at end stage lung disease, there’s just not much left to function. My right lung is still functional, still feels clear. But I, of all people, should know not to get to comfortable in that thinking. To think my right lung will carry me, for a while longer. De ja vu.

I was at that place 11 years ago, when MAC was driving, 100 MPH and taking me to the grave. I was dying and I knew it, I could feel it. I had all the symptoms of end stage lung disease . . . constant, unrelenting infection and coughing, fevers, chest pain, high heart rate, losing weight, couldn’t eat, no energy, couldn’t do anything! My left lung was consumed with MAC in places while my right lung was fine. Wedge resection surgery was my last ditch effort to remove all the life sapping sections of my left lung  . . .  hell bent on killing me. Lung transplant surgery was still a fairly new procedure at the time, so wedge resection was decided instead. My surgery worked, it restored my health and life, it was my saviour. It gave me 10+ years of quality life until now, some of the best years of my life. I never once regretted having that surgery, I’d do it again in a heartbeat. It was high risk at the time, but a risk I was willing to take. Either that or die.

The decision for my last surgery are the same reasons as for transplant now. My left lung will eventually spread the frequent infections to my right lung. My right lung is keeping me going at this point. It too is progressing downhill, just not as fast as my left. As Dr. R tells me, transplant (Tx) will be my safety net when my right lung begins to fail. It is a given guarantee that it will happen, just don’t know when. For now, Dr. R wants to play it safe, get all the Tx testing out of the way, while I’m still healthy enough and put me active on the list me when the timing is right. *shakes my head again*

The Tx coordinator and the Tx social worker both visited my room for introductions and some lengthy talks. They basically described the process, how it works, answered any questions I had, evaluated my mental stability and home life, wanted a complete ‘life’ history both medically and just in general how I’ve lived my life. This is so important because a Tx is such a life changing experience they want to make darn sure that I can mentally adjust to the changes and responsibilities of upkeep post Tx. I have many more meetings to be scheduled with other members of the Tx team, including the surgeons. All this will happen when my evaluation testing is complete. Then a determination will be made if the timing is right for me to be actively listed or wait a while longer. I just have to put trust in the docs that they know the right time based on my test results. Ugh, it is all overwhelming for me.

While I was in house, I got most of the physical testing I needed done.
Here are the tests I completed:
1. Complete PFT aka Spirometry. Pulmonary function testing measures lung volume, small airways, % of lung function, and liters of O2 I can take in and out in one big breath. A complete explanation is here

2. The 6 minute walk test. Simply, see how far and fast I can walk in six minutes, up and back in the hospital hallway. They also measured my O2 sats to see if I need supplemental oxygen. The lowest my sats went were 91, I passed. :) Anything under about 90, indicates supplemental oxygen may be necessary.

3. The blood sucker test. I call it this because the Phlebotomist from lab came into my room and stole about 22 vials of blood. A multitude of tests are determined by my blood. Blood typing for donor matching, kidney function, liver function, vitamin levels, drug and tobacco screens, and to rule out other maladies such as active chickenpox, TB, Epstein-Barr virus, hepatitis, herpes, AIDS, & HIV.

4. Arterial blood gas aka ABG. A blood test taken from an artery instead of a vein - ouch! ABG is mainly used in pulmonology, to determine oxygen and CO2 exchange levels in the blood related to lung function.

5. 24 hour urine collection. Tests kidney output and function.

6. Chest Xray


These are not my xrays. 
The left xray is of normal lungs, the right are CF lungs, they turn white when damaged.

7. Chest CT scan

8. Electrocardiography aka EKG. Used to measure and diagnose abnormal rhythms of the heart.

9. Echocardiogram with bubbles. Ultrasound of the heart while injecting saline microbubbles into the venous system. This shows any abnomalities in the cardiovascular sytem such as diseases of the heart valves, any damage to heart tissue, and severity of any present coronary artery disease.

10. Right heart catheterization - to check for artery lung pressure. I described this procedure in my prior post.

Tests I still have to do:
1. Left heart catheterization with angiogram, scheduled for 6-14. Assesses any cardio damage or coronary blockages. I only need to have this done because I’m over 50. If the test shows any abnormalities in my heart, such as a small leak or hole, it will be fixed during transplant surgery. Barring any complications, this will be outpatient, but will require me to spend a full day at USC.

2. Complete and current gynecological exam - I was due for this anyway and have an appointment scheduled in July.

3. Mammogram - ditto for this, appointment scheduled in July.

4. Complete oral exam - I’ve been waiting for authorization for this appt to be checked at the USC Dentistry school.

Between all the physical tests and mental ping pong I played, this hospitization was exhausting. I’ve been out now 2 weeks, and I finally feel somewhat normal again. I’ve been reading transplant info whenever I can and making lists of ?? I have for the Tx team. I’m also going to attend a Tx support group at USC the first Tuesday of every month. I know several online groups too, which I plan to start participating in. And lastlly, I have other CF friends who have had a Tx or are on the waiting list. I will defintiely pick their brains too.

One more step of my journey, that I must embrace. I can only think positive thoughts that the Tx will work out great for me. I’m hoping for many more years of good life left, to spend with my family and friends.

Now if only I can finish wrapping my head around the whole idea and accept it wholeheartedly. But I know, I will, it takes time. :)

In Honor of Emily

I don't even know where to begin. 

My dear friend Emily Haager lost her battle with CF 

on Saturday May 1.

When I heard the news, I was stunned and overwhelmed to the point of curling up and hiding in my hospital room. This can't be true, no not Emily, I still can't believe it. It took me days to process what had happened. It was even more surreal because I was in the hospital, just 2 floors below her. I finally feel that I'm in a place to write about her and my feelings of her untimely passing.

Emily and I go back probably 8-9 years. We were both patients at CHOC and I met her when she transitioned over from peds @ 18. She began attending the Family Education Days, picnics, and the other events our clinic held. She was at first shy, but she soon opened up and we exchanged our CF stories, our common bond. We often saw each other at clinic and caught up with our lives. 

We were both invited to speak on an adult panel at CHOC clinic Education Day. We told our CF stories and answered questions from parents of CF kids. These events really gave us time to socialize and get to know each other better. After years of attending our Ed days, our clinic discontinued seeing the adults as patients and we were told to transfer to an official adult clinic. We both ended up at USC, and again, saw each other often. We laughed that we were on the same 'sick' schedule being admitted at the same time and having our follow up clinic check ups the same day.

 

Emily's story is one that not only is heartbreaking but also a wake up call to me how quickly CF can take an unexpected, ugly turn. She was extremely active, compliant, and always on the go between being a college student, working, involved with church, CF and just living and loving life. Emily was also the CF ambassador for Pipeline to a Cure, surfing, fundraising and raising CF awareness. Last summer her FEV1 was in the 70s and she was well. 

 

She contracted H1N1 in October and admitted to the ICU at the same time I was in for a tune up. We emailed back and forth, she was very sick, but eventually got better. By Christmas, she was very sick again and shortly after diagnosed with MAC, Absessus. She came to me, knowing I had MAC 10 years ago and beat it. She wanted to hear my experience and treatment for MAC. 

 

In late January we were both at clinic and she told me that she was being admitted for another exacerbation and to begin MAC IV treatment. Within a couple days, she had a severe reaction to Colymycin IV that shut her kidneys down. Dialysis began as her kidneys slowly healed, but the many weeks of hospitalization took it's toll and she ended up on a vent as her lungs began to fail. She had been making some baby step positive progress, but one thing after another happened and her body finally gave up after 3 months in the ICU.

 

Emily had an incredibly supportive and loving family who was by her side 24/7. Their unending faith gave them the strength and tenacity to be there and help her in any way they could.

I take deep comfort in knowing that Emily is in a much better place, breathing easy, free of suffering, and in the arms of the loving Lord. A wonderful place that someday I hope and pray, I meet her again. Rest well, sweet Emily.

The Great, The Ugly and The Overwhelming in between . . . post 1 of 3

 Home sweet home!! :)

I was released from the hospital late Wednesday 5-12 and got home around 8:15pm. After 2 weeks, I was so ready to be home, in my own, bed, smells, comfort and familiarity. It felt so good to walk through the front door, having the grandkiddos screaming, "Grandee!!!!" fighting for my hugs . . . I so missed the little boogers. :)

To say this hospitalization was different from any other admit, would be an understatement. It started with me being very sick and being told you "will stay in the entire 2 weeks until the IV course is finished." I settled in and adjusted my thinking to that fact from the get go. I usually stay in about a week or so until I turn the corner, and then come home to finish my IVs. But with all the circumstances leading to this admit, Dr. R knew I needed the bed rest this time. I didn't argue or ask to go home early, I knew it too. Little did I know what this admit had in store for me. It encompasses 3 story lines, so I will post my stories in 3 separate posts.

On Friday April 30th, Dr. R felt it was a good time to address the lung transplant issue again as he had in clinic weeks earlier. With the continuing progression in my left lung causing my infections, he is worried that it will in turn start affecting my right lung. If that happens, my only reserve to breathe would be compromised, with no other options, transplant or die. He wants me to start the process, having the physical evaluation testing done while I'm in house to make it easier for everyone, especially me. Ok fine, let's do it.

Also, I came into this hospitalization with a new diagnosis of Pulmonary Hypertension. (PH) The echocardiagram ultrasound done the week before indicated problems, but I would need to have a right heart catheterization to confirm the PH diagnosis by measuring the artery pressure in my lungs. The Transplant evaluation also requires this test to be done. Dr. R thought it best to kill 2 birds with one stone, and move forward. The cath was scheduled for Friday the 7th @ 8am. The plan was to have the cath, determine the extent of the PH, and them meet with the PH Dr. who is also a pulmonologist on the transplant team. How convenient, one stop shopping. :) And, oh goody, a whole week to stress about the right heart cath test I've heard so many CFers talk about.

The cath procedure itself actually wasn't as bad as I had thought thanks to Versed. The stressful part was lying on the table, watching the staff set everything up in the OR, me naked from the waist down as several people buzzed around me. Uh yeah, check your modesty at the door, please. The Dr. walked in and asked how I was feeling. "Nervous," I said. "Not for long," he answered, "I need you to be fully awake to follow instructions yet comfortable, give her 1 Versed."

The nurse came over and pushed Versed into my port line. Hello, woozy head rush! Two minutes later, Dr. asked again how I felt. I said, "just a little woozy." I looked at the clock, it was 8:35. He told the nurse, "give her another." Shoot in my port, la la land, yet still hearing the music and conversations. I felt the sting and burning of the 4 or so lidocaine shots in my right groin, ouch. The Dr. said, you are going to feel pressure." Yeah, what ever, I don't care, just do it. Good drug, that Versed, I wasn't nervous anymore. :)

He jammed a guide line and tubing into my main groin vein and threaded it all the way up into my right heart followed by the contrast dye. I had an xray looking device hovering over my chest taking pictures on 3 screens beside me. The Dr. maneuvered, recited numbers to the nurse, had the tech take pictures from different angles, then told me to hold my breath, push down, like having a baby or bowel movement. They put O2 on me, and did the whole drill again. I heard the tech ask if it was time to go to the second level, and the Dr. said, "nope, I got it, we're done." (I gleaned from the OR convos that there were 3 levels to measure the artery pressures) The Dr. Snapped his gloves off, leaned over my face, and said, "everything looked great."

Really?, as I thought in my foggy brain trying to process what he had just said to me. Was I dreaming this or was this for real?? I laid there while the techs cleaned me up and put a nice, heated blanket on me. Ahhhhh, felt great, I was done and warm. I glanced up at the clock, 9am on the dot . . . that was quick!! As the techs wheeled me towards the door, the Dr. said again, "your pressures are in normal range and looked great for your age and considering you have CF." I didn't dream it, I'm more awake now, and I HEARD it, woo hoo, no PH!!!!!!! As we exited the room, Terry was at the door waiting for me to follow me to the recovery room. Two hours of laying flat on my back, which is really hard for me to do. My lungs got really junky and I coughed up crap the whole time. Finally, they wheeled me back to my room.

My hospital pulmonologists were waiting for me with smiles on their faces, and reiterated again, normal pressures and no PH!!!! YES!!!!! But, now you need to lay flat for 4 hours, no exceptions. Ugh, here I was thinking the 2 hours were hell, now they tell me 4 hours. Dr. R came into my room shortly after, and he said, "nope, I want you to lay flat for 6 hours." He said, "we really don't want you to get a blood clot in your lung after an invasive procedure." Hrrrrrrummmmfff. I couldn't wait to get out of the bed, coughing like crazy and feeling like I was laying in a puddle of OR goo and blood.

3pm rolled around and Terry helped me to my feet. I was unsteady and a little sore but determined to clean up and get dressed into my comfy garb. I felt a thousand times better, crawled back into bed and finally got to eat my lunch. Terry went home once he realized I was fine. I stayed in bed the rest of the day except to go to the bathroom, but overall felt OK. I was thrilled beyond belief that the echocardiagram was wrong and that I didn't have PH. I kept thinking that over and over, thanking God for taking that horrible problem away. Apparently the echocardiagram, is not a fool proof test, but rather an indication only that the cath needs to be done. I wish someone had told me that before I had already stressed so much about thinking I had PH. Oh well, it's all ancient history now. :)

I slept good for the first time that night. Relief that PH was not, the worst test was done and I could concentrate on getting better. Thank you God.

This was my GREAT news, which I wanted to write about first. I'll back up in my next post to Saturday May 1, to the horrible news, the shitty reality that is CF. It deserves a post by itself.

To be continued, in a couple days . . .