General update 5 weeks post-op

I had an appointment with my local pulmonologist today; overall it was a good visit. I have been running a low grade fever for the past few days and developed a dry cough and I have not been sleeping well. I had a chest x-ray today that showed an infarction in the upper lobe of my right lung; all of that added up points to a low-grade bacterial infection. I will start with a weeks worth of an oral antibiotic and then recheck with the doc next week. The good news is that my lungs sound really clear! No crackles, wheezing, or constrictions; and overall the chest x-ray looks good except for that one spot. It sucks to have one more thing to deal with, but honestly I am surprised I haven’t picked up a bug yet through all of this.

Even with this new infection I am actually feeling a bit better overall. I am recovered from both surgeries; everything has healed up nicely. I’ve got some nice new scars; five to be exact, all a half inch or less scattered around my abdomen. I still have the random giant bloody booger make it’s way out of my nose from the sinus surgery, but after the initial hell my sinuses have recovered and that operation has been very helpful in regards to breathing and sinus headaches/migraines. We are still trying to sort out the violent dry-heaving. Knock on wood, the heaving episodes have lessened, I have even had a few days without any heaving.

Today we also got the most definitive diagnosis anyone has been able to give us: Viral Induced Rejection; meaning I had some sort of low grade long term viral infection that triggered my immune system into overdrive causing a sort of attack on my transplanted lungs resulting in the reduced lung function. This fits with the overall trajectory of my health the past year; I had a lot of idiopathic symptoms that never fully presented themselves or lead to a real illness or problem. Sinusitis, allergies, stress…many things were tossed around but that is the trouble with rejection; it is very sneaky.

The official term for what is happening in my lungs right now is bronchiolitis, which is a fancy way of saying inflammation in the small airways. “Rejection” is used lightly here; there are multiple types of rejection and the positive side to this scenario is that recovery and regained lung function are very possible. There are no guarantees, but all the pulmonologists we have spoken with are optimistic that we can hope for recovery. That is a very long road; I don’t know how long I will have to be on oxygen or when I will be strong enough to begin pulmonary rehab. My personal goal is to be off oxygen by spring; I don’t know yet if that is realistic or not.

Right now it’s just rest, rest, rest, and eat. I have lost about 20lbs and all my strength; picking up a full pint glass of water is taxing. Walking down stairs makes my quads shake because they are so weak. All the medications have made my hair really weak and brittle and I have a weird rash on my chin. It is difficult to shower or get dressed by myself, and I am heavily dependent on my wheel chair because I fatigue so quickly trying to walk around. Having your physical independence taken away so drastically is incredibly frustrating. Thankfully I have awesome parents who have fully devoted all their time and energy to caring for me. My mom has taken Family Leave from work for three months and I will be reapplying for Disability so that I can hopefully have a little bit of financial independence while I am unable to work.

This past year a new legislation was passed that changed the rules (in a good way) for folks with respiratory illnesses to qualify for disability. Post-Transplant it is even more difficult to qualify because the laws are archaic and they view you as being “cured” since you got a new organ; but organ transplant is not that cut and dry and life as a Tx patient is very expensive. I would like to be able to work, but I just don’t know right now if that will possible. Even if I recover well from this set back working full time with all of my health issues has never been a realistic thing; I am just too damn tired all of the time even when I am “healthy” and stable.

Oct 19 2016 : Two weeks post Surgery and back home for a week!

Sorry not have posted sooner but it has just gotten away in the blur of the last few weeks. We have been back and forth to Seattle many times beginning with the first week in August. This was all started by the serious decline in Emily's lung function. The basic goal has been to stop any more decline, stabilize and then hope for improvement. No guarantees that any of those things were possible. There have been many procedures for diagnostic reasons. results from the testing indicated that two surgeries would hopefully be helpful. On August 30th was the first a sinus surgery. This had an unexpected long and painful recovery but seems to have helped with the post nasal drip and Emily can breathe through her nose for the first time in years. Tougher than expected but good result.
We were back in Eugene for a week or so then back to Seattle for visits at the Post Transplant Clinic and a post surgery visit. These trips have become epic . There is in the background the profound fatigue and nausea/pain combined with totally unpredictable traffic. We have become adept at keeping the O2 going  as Peg switches the valve  in the back seat where she has a tiny space among O2 tanks and other medical supplies. Our regular stops are a couple of Burgervilles where they have clean bathrooms that are wheelchair accessible. There are also really excellent fresh berry milkshakes but that is not why we stop there.

After a week back in Eugene from the post sinus surgery check up we planned to head back to Seattle for a quick three day trip to meet the surgeon for the next surgery, the Nissen Fundoplication. This is the stomach surgery to stop or limit acid reflux. The reflux can cause major lung problems. Well  best made plans. The three day trip turned into three weeks with fifteen days of that in the hospital at UWMC. (We have looked at the calendar and realized that we have not spent more than ten days in a row in Eugene since early August) This time in the hospital involved more testing to evaluate for the stomach surgery and an attempt to stabilize and gain some strength in anticipation of the surgery. It was notable the number of medical folks of the highest level that were watching over Emily as this surgery approached. Not until the day before was final go ahead made. Her surgeon is head of of general surgery at UWMC and was very attentive before and after. His team was constantly present in the hours and days post surgery. The post surgery stay was three full days rather than the normal one. We remained in Seattle an additional three days to be close to UWMC and rest before driving back home a week ago this past Monday.


We can not over state how good to is to be back home under our own roof and in our own bed with dear old Gus, our 12 year old golden staying as close to Emily as he can get. Medically, Emily still is on O2 all the time. She has been able to walk on her own to the living room. Small but big steps. Because of the surgery diet is limited to really soft foods for a month. That southern favorite Grits and Eggs all smashed together, and know as Goop, is most popular. Pain is much better but nausea still rears up pretty often. The recovery from this surgery is pretty long and the true evaluation of just how well it works is probably six months out. There has been a pretty serious rearranging of the anatomy. At this point though it seems to have helped a lot with the acid reflux. Lung health : it will also be a long time in  evaluating just what the new normal is. But a very good first step is the in house walks and being able to converse without going into distress.

We all want to make a few special thanks. As always our dear friend Nicky has been there for us. Taking care of Gus for a few days,  picking up Rx,  helping get Emily into the house and just being part of the foundation of our lives. Our good neighbor Joanie has watched over the house and is a good friend to Gus. When three days turned into to three weeks she not only watched over things but had the backyard looking perfect which was a sweet greeting home. Bobby picked up very important O2 supplies and mowed the grass at a rental. Our friend Bruce in Seattle appears magically to help out as needed. As does Mike to raise spirits and carry Emily down the stairs, princess style, so we could head home. I know I missed folks but thanks to all. This recovery is going to be a long journey. Please keep in touch via comments here and on FB with Emily.

Emily has been receiving a lot of cards, letters and packages via mail. UPS, etc. They have come to our house in Eugene, The Collegiana in Seattle (this is the excellent place we stay that belongs to UWMC and is for folks with family being treated) and a few things arrived at the hospital. These are great. I can not over state how much they mean. They often bring a bit of light and laughter to some long days. We are back home but the day to day health issues remain at a most serious level. These personal contacts mean so very much. Thanks and keep em coming. Take care. KM

Wednesday: One day post surgery .

     Here is a bit of an update of the last day or so. Emily's stomach surgery was yesterday  morning. She was the first of the day. Peg spent the night with her and I went over at 5:30 am. Emily was moved by gurney to the pre surgery area about an hour later. She left for the surgery area about 8:00 and was finished by 10:00, when we saw the surgeon. He told let us know surgery had gone well.
   
She then went to the recovery area which is a pretty amazing and kind of unnerving place when you first see it. Unfortuantely it is all too familiar after all the procedures of the past years. It's all business back there. We finally got to go back at 1:00. Those three hours had been tough for Emily with the dreaded nausea and some pain. The entire team had  been ready for this as it has been such a problem in the  past. One of the docs said they basically used everything available before getting things truly under control. This was extra important as heaving after stomach surgery is pretty high on the list of bad things to have to do. Especially since this surgery makes puking almost impossible.
The pain however was  pretty well controlled early on. She remained in recovery until about 5:00pm. They were being very cautious of her lungs and wanted to monitor things extra long and careful. the recovery area which has about two dozen curtained areas had become very empty and quiet. When busy there is a boggling amount of activity with all of the ace medical folks going about their tasks. By the time we left it was like being at the pub long after last call. Most of the energy has gone out of the room as folks cleaned up and send drifted out the door.
   
    Last night was about as good as a first night after gets. Emily has a magic button that she gets to push for pain meds and this lets her stay ahead of the pain curve. Anti nausea meds are at the ready and the night nurse staff was excellent at staying on top of things. There were a coup;e of adventures with controlling blood sugars (Emily is diabetic). This is tough post surgical. Sleep was in short stretches but everyone worked very well to manage this. It's important because getting too far out of whack can bring on the nasty fiends: Nausea and Heaving. The evil twins.
    So as of mid day Wednesday things are going well. The goal of the next few days is to continue to manage the pain and nausea and evaluate when discharge will be. Not today for sure. As much as she want to get out every one is being very careful. Then we will stay in Seattle for at least a day before taking on the trip back to Eugene.
     Thanks for the good wishes that folks have been posting here and on FB. Emily reads them all and appreciates the contact.

October 3rd 2016 .....UWMC........brief update.....from keith

    Its Monday here is a quick update. We have been scrambling since the last post. The last post had some info about the 48H PH study with the capsule in Emily's esophagus. The study had to be stopped at about 30H because of severe reflux which caused terrible burning pain.  Emily was discharged on Thursday evening and the pain continued throughout the next day. By early evening it was just not ok and with a lot of calls it was arranged for Emily to be readmitted directly to the hospital. Not through ER. Admission was at 10:00 at night on Friday just as the UW game was over. (The UWMC is next to the stadium) We were like a spawning salmon going against the flow. First the traffic cops would flag us down and then clear the way. We got her checked in and by about 3:00  in the morning she had a reasonable level of pain control. There was a drug reaction which caused uncontrolled muscle spasms mixed in just to keep things exciting. Since then the reflux is reasonably controlled and yesterday was able to eat for the first time since Wednesday.

    This is all in preparation for the potential surgery on Tuesday morning. Tomorrow. This is the Nissen Fundoplication  surgery. Rearranging the top of the stomach to prevent reflux. This is a major surgery with a long recovery time. The major concern is that with reduced lung function Emily is considered at high risk for surgery. However it is also the considered opinion that to protect the lungs the reflux must be controlled. Rock and a hard place. A lot of very good and skilled folks have been giving her there attention. As of late Monday afternoon the surgery is a go.

    The plan is for surgery early tomorrow and then one or more days post op at UWMC. Then we will wait a few days to rest and be near the medical team here. Once Emily feels strong enough we will drive back home to Eugene for prolonged recovery.

    Send good thoughts  Emily's way tomorrow we all think that they help.

Today is Emily's Birthday !

    This is the birthday time of year at our house Peg's on Tuesday and Emily's today. Since we had not planned on being in Seattle we are a bit short on birthday cakes and cards but  are still having fun talking about birthdays past and plans for future ones. There is one big present today: Emily is being discharged from UWMC this afternoon. This is a good thing. On Wednesday Emily had a small capsule placed in hers esophagus to measure the acid levels from reflux. The data is sent to a small recording device worn around the neck. This goes on for 48 hours. The data will then help make the decision about a major surgery  possibly next Tuesday. This would basically redo the opening into the stomach to limit reflux. It's a pretty big deal. This is to hopefully reduce damage by reflux to the lungs. A major consideration is that she is considered a high risk candidate for surgery because of decreased lung function.  Each time under is a worry. Yesterday's procedure also required full anesthesia but was for a very short time. It took her most of the day to fully wake as some of the post surgery anti nausea meds really made her sleepy.

    We will remain in Seattle til the test is finished on Friday. We will stay here in anticipation of the surgery on Tuesday.  Hopefully the hospital stay has calmed things down and Emily can rest and gain strength for the next step.

Hello to all from us three.

Today is Emily's Birthday !

   



This is the birthday time of year at our house Peg's on Tuesday and Emily's today. Since we had not planned on being in Seattle we are a bit short on birthday cakes and cards but  are still having fun talking about birthdays past and plans for future ones. There is one big present today: Emily is being discharged from UWMC this afternoon. This is a good thing. On Wednesday Emily had a small capsule placed in hers esophagus to measure the acid levels from reflux. The data is sent to a small recording device worn around the neck. This goes on for 48 hours. The data will then help make the decision about a major surgery  possibly next Tuesday. This would basically redo the opening into the stomach to limit reflux. It's a pretty big deal. This is to hopefully reduce damage by reflux to the lungs. A major consideration is that she is considered a high risk candidate for surgery because of decreased lung function.  Each time under is a worry. Yesterday's procedure also required full anesthesia but was for a very short time. It took her most of the day to fully wake as some of the post surgery anti nausea meds really made her sleepy.

    We will remain in Seattle until the test is finished on Friday. We will stay here in anticipation of the surgery on Tuesday.  Hopefully the hospital stay has calmed things down and Emily can rest and gain strength for the next step.
Hello to all from us three.

Update from UWMC our Home away from Home

    Hello All. This is from Keith. I think many are keeping up to date via Face Book but Emily wants to keep this Blog a bit more current. My last post was at the end of August. It is now September 26th.(now the 27th ran out of steam late last night) We are back in Seattle at UWMC and have been since last Wednesday. There was also another three day trip to UWMC in mid month. We may be here through the end of the first week of October depending on tests this W-F that will help determine if another surgery is indicated on the 4th.

    The last month has been very tough for Emily. We returned to Eugene after the sinus surgery and she had to endure a truly horrible multi day migraine. This set back her recovery dramatically.  The last few weeks she has experienced continued reduction in lung function and profound nausea, weakness and fatigue. She requires oxygen 24 hours per day and needs to use a wheelchair to get around, even at home inside the house. This trip was to be Wednesday the 21st through Friday. This was a post op check for the sinus surgery and a meet and greet with a new GI surgeon. Unfortunately on Friday Emily was too ill and weak to travel back home. (These trips back and forth are a pain. The traffic is a nightmare and managing O2 and the chair make us a bit of a circus act.) Things didn't get better over the week end and on Sunday Emily had to go to the ER. She was dehydrated and exhausted. She was admitted that evening and remains at UWMC. She has received a lot of fluids via IVs. She is definitely feeling better but there is no discharge date yet as she needs to get stronger. All of this is compounded by the reduced lung function which makes just plain old breathing serious work. If strong enough she will have a 48 hour PH study to determine the extent of acid reflux, this will be on Weds the 28th. This is a procedure that places a device in the esophagus to measure what is going on. While the procedure is pretty straight forward it does require anesthesia which is a concern with her current lung function. Depending on what is observed Emily may have surgery on the 4th  on her stomach to hopefully stop the reflux from occurring. This is a major surgery and again full anesthesia. There is quite a bit of caution at the moment. The most basic first step is for Emily to recover enough strength to be a reasonable candidate for surgery. Then we go from there weighing the risk vs benefit. Difficult days. Hopefully, tomorrow Emily can begin some very basic PT. Simple walking with assistance. Was to start today but just needed to rest. The reason for the sinus and stomach surgeries is to stop post nasal drip and acid reflux. That doesn't seem like the biggest deal but in the world of lung transplant it is. These things can spark forms of rejection. That is the working theory on the decrease of lung function.

   Even though this is not a vacation Seattle is a beautiful place. Emily recalls fondly the several years she lived here. From the wing where here room is the views are special.  One way is Huskie Stadium with the lake just behind. The other way we look down on the Montlake Cut and can see the racing shells training.

    It is now mid morning of the 27th. I am  at UWMC with Emily and Peg is back at our room. She stayed  at the hospital last night. We take turns.  It was a rugged early morning with nausea and dry heaves. There is an unexplained pattern to the days. The very early hours tend to be just plain bad with the nausea then an exhausted sleep of many hours followed by several hours of feeling good into the late evening. In the early part of the day no appetite and then a good one. Got the medical folks stumped on why this occurs.

   So these early fall days are special around our house. We usually go out for a family dinner to a favorite place. Most often Chefs Kitchen.  Today is Peg's birthday and Thursday is Emily's. I think we will do something extra  to celebrate this year once we are back in Eugene.  I will post this now and hopefully make the link to FB. Please let other friends know so they can be up to date. As always it is great for to hear from folks on the comment section here or Emily is on Face Book a lot. The days and nights get long and any distraction is welcome.

 

Well my worst fear about sinus surgery has been realized: I have the migraine from hell. Good news is my sats are back up to 97% on room air!!! (I was hovering at 92%). I am hardly needing the oxygen and I can breath SO well through my nose. It's crazy. I don't remember ever being able to breath like this through my nose. (I probably could as a small child, but that was a long time ago and I lacked the foresight to appreciate it in the moment).
On the less glamorous side of this diseases I am fighting off a bowel blockage. Chronic constipation is just part of CF but then you add and  narcotics for post surgical pain and you have a recipe for disaster. (If you e never taken narcotics before constipation is one of the main side affects). I am trying to keep popping with max doses of miralax (that is the over the counter white powder you see a lot of commercials for in relation to IBS), magnesium citrate, and prune juice. (Prunes are magic for constipation). I have  a round of Go-Lightly waiting for me at the pharmacy (that is the bowel prep for colonoscopies) if shit goes really sideways (heheheh) I am tolerating the norco (pain med) okay (everyone was worried if I would be able to keep pain meds down after my hellacious time in post op), but even though it not making me sick and doing its job for pain it shuts down your guts. One of the many Catch-22's of chronic illness life. All and all its just a wild party of constipation, migraine management, and bloody nasal lavages. Exciting times at Casa de Monfort.

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.

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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!