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.