post surgery report : from UWMC

Hello All ,this is posted by Keith,  Emily's dad. Well, it has been well over seven years since I posted on the blog. That's been a good thing as there have a lot of healthy days but with the serious decline in lung function these past weeks  Emily decided to reactivate the blog to keep folks up to date.

Today, Tuesday the 30th of august was sinus surgery day. Yesterday actually as it is now 2:30 am Wednesday. The goal was to clean out the sinus cavities and open up larger passages so the too thick CF mucous can drain away better. A major procedure but meant to be an out patient stay. The surgery itself went very well and a lot of nasty stuff was cleaned out and airways were opened. This is a very common surgery for CFers. But out jumps the devil and Emily has had a tough recovery and  has been admitted to UWMC for the night. She experienced very bad pain that was tough to control and lots of puking caused by blood draining into her gut from the surgery. This went on for quite a few hours and an awful lot of blood  came up. After many tries of different meds the pain and nausea were pretty well controlled about 10 last night.....the surgery had ended about 3 so that was a long haul. It is pretty complicated for her as there are many of the good pain meds that she can not tolerate. Or they control pain but cause nausea, that would be morphine tonight but not in the past. Some good work by the medical team has gotten things under control and she is getting some  hard earned and well deserved rest. Before sleeping she did an excellent walk around the floor and was able to eat a little jello. Big deal since last intake was 24+ hours ago. The really good news is that she can breathe through her nose for the first time in forever. That was one of the goals and is wonderful.

So I think the day is ending very well. The hope is that the surgery will help with the lung function issues. Hopefully discharge will be tomorrow and perhaps home to Eugene on Thursday. That would be sweet after being up here since last Tuesday and having some medical activity every day except Sunday. Emily was in good form as she went to sleep saying that at moments like this she still thinks that  as crappy as it is to have a disease like CF she is fortunate to have the extra ordinary care she gets, have her family for support and her community around her.

Thanks for following on the blog or Facebook. Please comment and stay in touch.  Being back up here reminds me of how lonely it can be when dealing with chronic health problems. It takes you away from home,  your regular life and is very isolating. Any hello, no matter how brief is  welcome and appreciated.

It has been a long week. We are finally starting to get some answers and paint a fuller picture as test results keep trickling in and watching how I respond to various treatments.

I had an Esophogram yesterday to look for evidence of acid reflux; the study was conclusive and distinctly showed active reflux. It feels good to finally pinpoint a definitive symptom because that means we can make a plan! (If you know me; I am a planner. I absolutely despise ambiguity). I have begun a PPI (a proton pump inhibitor, a.k.a an anti-acid). Normally one begins a PPI right after Tx as a prophylactic measure; reflux can cause serious damage to transplanted lungs; the wear and tear on the esophagus causes an immune response in the lungs that can unleash a domino affect of complications that lead to rejection).

I could not begin a PPI seven years ago because I was still battling my chronic fungal infection and the antifungal medication severely interacts with PPI's in the liver causing major damage. CF life is full of Catch-22's; I would have become extremely ill and compromised from the untreated fungus so I had to stay on the antifungal thus putting me at risk for reflux without being on a PPI…… at the time the fungus was more of an immediate threat than reflux.

I do not have really severe reflux so we are starting with treating it with meds not surgical intervention. (for REALLY acute acid reflux they do what is called a Fundoplication; that is a fancy way of saying we are going to tie your stomach in a knot. This is really major surgery, one I am not eager to have because you can’t puke after. What is one to do if one has the stomach flu?! Or food poisoning?! Or has one too many shots of tequila and needs to vomit and pass out in a gutter…..These are important life scenarios to evaluate). I am also just really fucking over having surgery.

The evidence is mounting up for the other major culprit that we have suspected from the beginning; my chronic sinus disease. I have the sinus surgery on Tuesday so that component will be addressed.

The next step is to let me heal and watch and wait. With this second round of IV prednisone, treating the reflux, and having the sinus surgery we will be treating the two primary sources that are causing the massive inflammation in my lungs and causing lung function to drop.

My doctors are reasonably optimistic while be honest and realist. A combination I really appreciate. I am a no bullshit kind of woman; tell it to me straight no sugar coating ambiguousness. I hate that shit.

If the sinus surgery, reflux meds,  and steroids work I should see a jump up in my lung function. ( most likely not back to my previous baseline, but higher that 37%, re-stabalization, hopefully going off O2, and some room to work with rehabbing up to better lung function). Then it is a matter of assessing if any permanent damage has been done to the lungs. The odds are in my favor right now for that piece because I am not suffering from a massive lung infection like pneumonia, the flu, bronchitis, pseudomonas, etc. So much of this information is new for me because I have been so lucky with my Tx; I have not dealt with major rejection and infection issues.

The next phase post-surgery will be to try and gain some ground with reconditioning by doing Pulmonary Rehab; conditioning and strengthening the lungs after all this stress and trauma. (Kind of like rehabbing a knee after you’ve blown out your ACL, but different because knee injuries typically don’t kill you). I will start that back home in Eugene after I have had a few days to rest from the sinus surgery.

This is not the summer I planned for after graduating from college, but life never goes as planned. One of my favorite quotes that I come back to often is from Eleanor Roosevelt: “Sometimes you have to give up the life you planned in order to live the life you were meant to lead.”

This is never been more true in navigating life with a chronic illness. Trying to find my place and purpose in the world after my Tx has been the biggest struggle; I don’t fit anywhere. I have always been an outlier because of the regimented and restrictive nature of my life in how I have to manage this disease. Understanding the purpose of it all is an infuriating philosophical journey.

Quick Update (CMV, Prednisone infusions)

A quick update. I will be doing another round of pulse dose steroids (the IV methylprednisolone) to try and get the inflammation under control and my lung function back up. I did another set of PFTs on Tuesday and I am hovering around 31% TLC. I am needing 4L of oxygen all the time now to stay comfortable and 5L when walking.

We also did another blood test on Tuesday to check for a viral load from CMV.
(CMV = cytomegalovirus; a very common GI virus).

Okay pay attention now: my donor was CMV positive; I have never had active CMV, but upon transplantation CMV was introduced into my body. In normal people CMV is not a big deal; in immunosuppressed Tx patients it can cause lots of serious problems.  I was on IV valgancyclovir for about six months post Tx to keep the CMV from flaring. So far I have not had any issues with an active CMV infection but they monitor this closely. (Once a virus is introduced to your body it is always there; it becomes dormant after the initial infection. But with being immunosuppressed my ability to make antibodies for new infections is all kinds of messed up so there is always a chance the CMV could flare up).

So, the take away from that is that my CMV is not active and that is good.

I've got a doctors appointment and several more tests today and tomorrow which hopefully will continue to yield more useful information.

In the meantime it's back to being a cracked out jackrabbit.

Chapter 7.5 Summer 2016

Alright Folks,

Here is the much anticipated Medical Update. (when I type that I hear the intro for “Weekend Update” from Saturday Night Live. If only I had Seth Myers and Kristin Wiig to read this to you.) It has taken so long to write this because we have been waiting for all for the test results to trickle in.

Since last Fall I have been experiencing a slow overall decline of my health. This was previously attributed to the fact that I had surgery three times last year, a Staph infection, a Norcardia infection, and a Pseudomonas infection in my sinuses all while desperately trying to finish up at the UO and working part time. I was tired and stressed out.

It turns out perhaps something more insidious was happening. Three weeks ago my FEV1 dropped dramatically and suddenly. My base line Total Lung Capacity (TLC) oscillates between 75% and 80%; almost overnight my TLC dropped to 38%. Not good.

(For the uninitiated: FEV1 = Forced Vital Capacity. This is one of the many numbers that is measured when you do Pulmonary Function Tests (PFTs), and is one of the key markers for monitoring signs of rejection in transplanted lungs. FVC = Forced Vital Capacity. Another number looked at on PFTs. FEV1/FVC = TLC).

My folks and I headed up to Seattle to my Transplant Center (from here on out Tx = Transplant. I get really tired of typing that over and over). I did three days worth of pulse dose steroids. This is where they blast you with 1000mg of IV methylprednisolone to beat your immune system into submission.

Let me put this is perspective; if you went to the ER with, say, a moderate to severe allergic reaction to poison oak they would give you about 40mg of oral prednisone. I was having 1grams worth injected directly into my veins. One of the many side affects of prednisone is insomnia; really what that means is being a cracked out jackrabbit. It’s like doing some meth and then adding a couple lines of cocaine just for fun. It also makes everything hurt. Fun times.

The IV pulse dose steroids are standard for when there is suspicion of rejection or inflammation in a Tx organ. After those three days you then continuous on an oral prednisone taper; decreasing from 1-5mg per day for a couple of weeks. (you have come off of prednisone slow from that high of a dose or else you will blow up your adrenal glands; mine are already fucked (I have Chronic Adrenal Insufficiency) so we don’t wont to destroy them twice over).

There are four main categories that doctors look at when dealing with an issue like this in a set of Tx lungs:

- Infection

- Rejection

- Gastrointestinal-Esophageal Reflux Disease (GERD) (a.k.a. Reflux)

- Post-nasal drip

What we have to do is go through these categories and try and pinpoint what is triggering this inflammation in my lungs.

I had a bronchoscopy up in Seattle and they took a bunch of samples and biopsies and tested me for everything under the sun. All viral, bacterial, and fungal panels, Tuberculosis, pneumococcal hemorrhagic fever something-or-other, aliens……

All viral, fungal, and bacterial panels were negative; so that means we can cross off infection.

INFECTION

The biopsies that took to test for rejection were also negative; so we can check of rejection.

REJECTION

That leaves us with SINUSES and REFLUX.

(Or some other mystery problem. I have habit of manifesting strange, unusual complications; I cannot tell you how many times I have had a doctor say “Well, I’ve never seen that before….”

Note: It is not a good sign when a doctor says this to you. That is the wrong kind of special).

We know I have sinus issues; I am having sinus surgery on August 30.

(All CF patients have sinus issues; just part of the package of CF). We also know that I have history of sever GERD. (Almost every Cystic fibrosis patient has GERD; again just part of package from the digestive disease of CF).

I initially responded to the IV prednisone great; after a few days my lung function began to go back up. I got all the way back up to TLC of 60% in four days. A significant improvement from 38%.

Besides from allowing me to feel better and not require as much oxygen use this response tells us a lot of important things:

1)   That type of response to prednisone is further evidence that I do not have an acute infection. (if you give someone with an infection that many steroids it will make them very ill).

2)   That quickness of a response indicates that inflammation is the primary issue in my lungs and this is easier to deal with than an infection or rejection.

The bad/interesting news is that as my prednisone dose got lower my lung function began to decrease. I had tapered all the way down to 20mg of prednisone and my TLC was at 40%. (down from the regained 60%). So we increased my prednisone back up to 40mg and I am beginning to show some improvement, although I am needing 3L of oxygen almost all the time still.

We are holding this dose of 40mg until tomorrow (Tuesday 8/23) when I will be repeating a full set PFTs and getting a chest x-ray. Depending on what those yield we will tweak the prednisone accordingly.

-----------------------------------------

The next step is pinpointing the source of the inflammation; which in all likelihood is sinuses or reflux or a combination thereof.

Tomorrow my dad and I will be heading up to Seattle for a week where I will have a weeks worth of testing before my surgery on 8/30. My mom will be joining us later in the week. We have a strange concept of “family time.”

The other half of Cystic fibrosis is digestive disease. Folks generally think of CF as a respiratory illness because that is most often the most severe set of symptoms and the part of the disease that kills you the fastest.

What happens in CF is that the pancreas is unable to make digestive enzymes which leads to our own special version of malnutrition due to the inability to digest our food properly and mal-absorption issues in the lower intestine. (meaning we cannot absorb the nutrients properly out of partially digested food).

This means I poop a lot. I don’t digest half the food I eat so it has to go somewhere! If you are ever hanging out with me and I disappear to bathroom for twenty minutes it’s because I am taking a colossal, and often very painful, CF bowel movement. CF poop smells particularly awful because of the lack of enzymes. I always apologize silently to the poor unfortunate soul who has to follow me in a public bathroom.

 I am also diabetic (which is a secondary complication from the pancreatitis from CF. I have Cystic Fibrosis Related Diabetes, it’s our own special version). The combination of CF gut disease and diabetes creates another problem called gastroparesis. This loosely translates from Latin to “slow stomach emptying.” This leads to a whole host of problems and causes a lot of dietary restrictions. (This is already a long ass email so I will forego the in depth gastroparesis explanation. If you are just dying for more medical mumbo jumbo let me know and I’ll give you the long version).

Since all of this began three weeks ago my gastroparesis has been really kicking up, and that is probably tied into the GERD and lack of oxygen. Shit just doesn’t work right when it doesn’t have enough air.

What I will be doing up UWMC this week is getting a complete GI work up. We are going back to the drawing board, if you will. to try and sort out what is causing what and how it is all tied together. We are dealing with a complicated chicken or the egg scenario.

I will be having:

- a 24h Ph study

-Mamometry

-Esophogram

- Bariun swallow

- Complete work up with the specialized GI team

- possibly and endoscopy

I am going to ask for a colonoscopy. (Because they are SO much fun!!! No, they are not. Actually colon and bowel cancer risks go way up in post Tx CF patients so I want to establish a baseline. The last thing I need is some version of ass cancer; I mean seriously I have enough problems).

It is going to be an exciting week.

Monday 8/29 I will have my pre-op with the ENT surgeon who will be doing my sinus surgery. This is planned as an outpatient procedure, but you never know. Since things have become so complicated they may want to admit me post-op but we don’t know yet.

I will keep y'all updated as we learn more. Thank you for all the love and support!

         

Year 3.

So I didn't post on my three year anniversary. I was busy celebrating by skiing with my boyfriend and my Dad, and then all of a sudden it was April. That was bad of me because everyone has been so supportive and you deserve an update. I apologize for neglecting my blogging duties.

April is National Donate Life month, so this is an appropriate time for a blog post. If you are not already an Organ Donor, please become one. The impact you will have on an individuals life, their family, friends, and their community is really beyond words. Presently, several people close to my heart need organ transplants. My Aunt needs a new liver, my friend Talana needs new lungs, and my friend Kari also needs a new liver. (Both Talana and Kari have cystic fibrosis like me.) Follow this link to become a donor today, and please spread the word to your friends, families, and your community. http://www.lcnw.org/

As the third year of my transplant life rolls along I find myself still in a continual state of transition. Most other transplant patients I know say it took them about five years before they felt like they returned to a state of "normal." That time line seems to be true for me so far as well. Being able to breath is the dominant feeling, but year to year my body is still making a lot of changes and I still don't feel like I have reached my new equilibrium. It is an interesting journey to say the least.

I tried working two different jobs in 2011, being back working felt good because I had not been able to do that in such a long time, but it was also very difficult. Even though I can breath now, I still do not have normal energy levels. I cannot work a full eight hour shift and do all the other things that normal healthy people do in a work day. Like go to the grocery store, do laundry, run a few errands, or go out for a beer with co-workers. I basically have to go home immediately and go to sleep, so that I can have enough energy to get through the next work day. The most difficult part of this is explaining my restrictions to bosses/managers. The fact that I look so normal is a disadvantage in this situation, it is VERY difficult to paint the picture of life with CF and transplant life and the challenges they present when you don't look sick at all. When I was hauling around an oxygen tank all hollowed eyed and emaciated and weighing maybe 118lbs it was fairly easy to convince people I was ill.

I struggle everyday thinking about what the hell I am going to do for income and a job. I do not qualify for state disability aid, and I am way too young for my social security to kick in. I am presently back in school (yay!) slowly working towards finishing my bachelors degree someday. But even if I am able to finish that, I don't know what kind of work I can do. I will need an INCREDIBLY understanding boss and a job with lots of flexibility, (which I am so far convinced doesn't exist.) I don't think I will ever be able to work a 40 hour week either.

I have been talking a lot with the social worker at my transplant center, asking what other patients have done with this problem. She is an awesome lady and very honest, and is very open about the fact that they do a really good job of getting people new organs but a horrible job of preparing people for life after transplant. I have always had to think outside of the box in order to participate fully in life, so I will just continue to do that and hopefully figure something out someday.

In the meantime I am enjoying being back in school, and it has been really sunny in Seattle and sunshine always makes everything better. (Especially in April in the Pacific North West.) It is beautiful in the city, there is still snow in the mountains, and climbing season is right around corner. All in all life is good.

(Oh, AND I can eat spinach again! Why is that exciting you ask? Well, I have this long list of can't eat food because of transplant and spinach was on that list because there were so many instances of E. coli and other gross bacteria that can make you sick, growing on spinach so it was blacklisted for people with compromised immune systems. But it is safe again, so I can eat it now! It never fails that when someone says you can't do something, or can't have something, you want it really bad. Believe it or not, but I have been craving spinach for the last three years, and now I can finally have it! Spinach salads, here I come!!)

I hope life is treating all of you well, I apologize again for the delayed blogging. Please help spread the word about Organ Donation.

Love and Light,

Emily

Birthday Post

Hello family, friends, and others!
So today is my birthday, and it is a particularly special birthday for me and my parents. This is the third birthday I have had since my transplant, and each birthday I have post-transplant where I wake up happy and healthy is quite the milestone.
Another thing that is special, significant, and unique about this birthday is that I tuned 28. When I was first diagnosed with cystic fibrosis the average life expectancy at that time was to the age of 18. Today I have beaten that diagnosis by ten years. That feels fucking awesome.
I have my donor to thank for being alive today, without her I would not be here. I also have my parents, my friends, everyone who supported us through the transplant and ensuing months, my doctors, every health professional who has ever treated me, and my transplant team to thank for me being alive today. A special thank you goes to Nicky Connors, who so selflessly came with us to all the surgeries, spent countless nights with me in the hospital, spent months of her time not working and staying with us in Seattle, and always being there, even at 4 in the morning. Most importantly she kept me and my parents sane. Thank you Nicky, it is hard for me to tell you how important you are in my life.
This has been a bumpy year, but where I find myself today is happy and at peace with a lot of things that in the past have caused me turmoil and stress. I look forward to this new year and what it will it bring; good, bad, exciting, stressful, frustrating, and happy. Whatever it is I am excited to live it, after all that is why I have fought so fucking hard to stay alive and why I got this transplant, so that I could live.
So much love to all of you. I hope you find many blessings and love in your life.

Hello friends and family. Today is my two year anniversary for transplant!!!! The time has gone by so fast, and yet so slow at the same time. This year has been filled with many a deep breath as I find my way through the transition of healing and recovering, to figuring out how to live a normal life. (normal being a relative term of course.)

Today I am reflecting on many things, and continue to be in awe of this whole transplant process. This day of February 16th will always be a duality for me. While I and all of you celebrate the fact that I am alive and continuing on with these new lungs, there is a family somewhere who is grieving. This day is a reminder to them of the loss of their loved one.

That duality of celebration and grief is something that I will always be turning over in my head, trying to understand the philosophy and meaning of it all.

I do not dwell on it and I do not feel guilty. However, the fact that today marks the anniversary of one person's death and another's life is a duality I cannot quite wrap my mind around.

What I can do is breath deeply and live fully for my donor and her family, and for me and my family.

To the right you see me and an extremely phallic looking Eiffel tower. I am holding a sign that says "Thank You UWMC." (as in University of Washington Medical Center.) I spent about 8 weeks traveling in Europe this past September and October. That trip was a mixture of experiencing the joy of traveling by myself, a victory lap for getting my transplant, and a big Fuck You to cystic fibrosis. It was amazing!

I hope this finds everyone well. I continue to prove that I suck at blogging, the last post was in May 2010, so most likely see you next you year, same time, same place. Except that it will be a thursday and not a wednesday. Much love to you all.

-emily