A Difficult Subject to Write About...

So...  After all the testing we have a diagnosis.  I am not sure it reflects what was originally wrong as that was a lingering problem that went on for months and even a year or two, however we now have something to call it..

LYMPHANGITIC CARCINOMATOSIS

On the Wednesday before Thanksgiving I got another bronchoscopy and biopsy.  Because of issues with putting me under anesthesia, they were only able to test one mediastinal lymph node in my lung, but it tested positive for metastatic breast cancer.  

Lymphangitic Carcinomatosis has a very poor prognosis.  Typically the life expectancy is between 3-6 months which is why I  don't believe it is what was wrong initially, because I have been dealing with the symptoms for longer than that.  However it is the official diagnosis now.  

I received the news on the phone from the pulmonologist at the UofU.  He called, told me the results and then told me he knew nothing about the treatment, just that the prognosis is not good.  He then told me to follow up with my Oncologist.  So in the mean time I did a lot of Googling.  

I met with my Oncologist on Monday (Dec 6). I have begun a new form of Chemotherapy pills that hopefully will extend my life a bit longer...the doctor says maybe a year to 18 months.  To tell you the truth, I was relieved to hear it, because before I met with him, I kept thinking I only had a couple of months and I just couldn't imagine "wrapping up" my life in a month or two.  I mean, I guess the world will keep turning whether or not I get it "all done once and for all". But I kind of want to leave "my house" tidy when I go.  So at least now, I hopefully have a little bit more time.

I don't know how long I have, it may be a month or two, or it may be a year or two, or maybe some new treatment will come out and I will live a long time. My health is not good.  I require an oxygen compressor pretty much all the time.  It is still hard for me to sleep, because I cannot lie down and must sleep sitting up.  Some days I feel relatively good and think this is not so bad, and others I am so miserable I don't want to keep living.  The evenings are often the worst especially if I have overdone for the day...overdoing being something like going out to dinner.

I think of so many people I would like to see and spend time with before I die, but unfortunately do not have the health or energy to do so.  I have many good friends, that I love with all my heart that I am sad to leave behind.  Thank you my friends for being my friends. Whether you know it or not, I have learned so much from ALL of you.  

I am sad to leave my family.  They are among my best friends, and I love them very much.  We have so many fun and funny memories together.  I wish I was going to be around to make more of them with them. 

I am sad I won't get to watch my grandchildren grow up.  Some of whom I am only beginning to know and others that have not been born yet.  

I am terribly sad to leave my husband.  How I have enjoyed our 9+ years together.  What a blessing he has been to me.  I wish were going to have more time.  But I am grateful to know we will have eternity to spend together. How I love him.

I am not afraid of dying, that is something I came to terms with when I was first diagnosed with Breast Cancer, and then again in 2019 when I was diagnosed with Metastatic Breast Cancer.  This kind of blind sided me, because for some reason, I strangely never even considered cancer.  What makes me sad is leaving the people I love.  I wanted to be a part of their lives.  Once I die, life will go on as it should, only I just won't be there. 

.....the words are inadequate.  I rejoice in returning to live with my Heavenly Father and I know this time on earth is such a short time in comparison to eternity. I am kind of glad I will get to miss the upcoming APOCALYPSE 😅  But I did want to enjoy more time here with the people I love.  

To quote Forrest Gump...  "Mama always said dying was a part of life. I sure wish it wasn't"

                                            "....And that's all I have to say about that."

Happy Halloween! How IBRANCE is Like Mushrooms - Update on Me

These mushrooms are called Xylaria Polymorpha - Or commonly known as "Dead Man's Fingers".   I have never run across them personally, but I hope to some day. They are not an edible type of mushroom, but I think they are cool and kind of freaky. 

There are so many types of mushrooms.  Many people have been taught to avoid them, because they are poisonous, but those who adhere to that train of thought are missing out on a whole world of delicious wild mushrooms. It is true though, you have to be careful and be sure you are only eating the right kind...like any other thing.  You don't want to go around consuming any food (or drug) you aren't sure is safe...for you.  Some mushrooms are perfectly yummy and safe for most people and then there are those who react to a certain kind and it makes them sick.  So it is always recommended that you test a small amount first and if after a few hours you are fine, you can proceed with larger amounts. 

There are some mushrooms that are so toxic you learn to just avoid them.  The saying "When in doubt, throw it out" warns if you are not 100% sure what you are eating, DON'T!  There is one mushroom called Chlorophyllum Molybdites that is nicknamed the "vomiter".  If you are unfortunate enough to eat it, in a short time you will learn why it got the nickname...as I believe you will be "vomiting" from both ends for a long miserable time.   

There is another mushroom called "The Destroying Angel"  

If you mistakenly eat these little lovelies, from what I understand they will taste good and you will not know you have made a BIG mistake for 6-8 hours. By then it is too late. After a few hours you will have similar symptoms as other food poisonings and will begin to wonder if it was the mushrooms, but in this case, in that 6-8 hours, the toxins in these mushrooms will have been doing damage that many will not survive. After a bout of terrible gastrointestinal distress, you will feel relief, but the toxins have already done damage to your kidneys and liver and it is too late for doctors to get the toxic materials out of you.  You are left to the ravages of the toxins with little they can do but treat palliatively and hope somehow your body is strong enough to survive and heal.

Because I am totally fascinated with mushrooms right now, I thought I would share that analogy with you in hopes you would think it is interesting.  It is also what I realized was possibly true about the damage done to my body by the Ibrance medication I was taking.  

As I talk to numerous specialists about my condition, I am exasperated at how little they know.  Does it seem arrogant for me to suggest that the nation's top specialists are WRONG?!?  Maybe...but I think they are.  

I went to the University of Utah Pulmonology on October 19th.  The Doctor came in and told me he didn't think my respiratory issues were caused by Ibrance.  Now I am so sure that these issues ARE caused by Ibrance, that when any doctor at this point starts going down some other rabbit hole on this, I get like panicky, anxious, frustrated, desperate...like I just feel like I am in a horror movie screaming  and nobody can hear me.  Like the scene in Knight and Day, when June is trying to explain to her Fireman friend all the crazy stuff that has happened to her and he starts patting her hand, and she is like..."why are you touching my hand" and it is clear he has no clue.   You can see it here...

https://www.youtube.com/watch?v=3JO7SeXB2nI 

 

 Yeah. This is how I have felt for the past 4+ months. 

Back to the U of U Doctor.  He said he didn't think it was caused by Ibrance and that it didn't look like other cases of pneumonitis he had seen before.  In my frustration I began to cry and try to convince him that he is wrong, to no avail.  I asked him what he thought it was and he said he thought it was Non-tuberculosis mycobacteria and that they needed to do another bronchoscopy and a biopsy to be sure.  I mentioned that a bronchoscopy had already been done that ruled out any pathogens in my lungs.  (I mean they tested for dozens of viruses, bacteria, and fungi and there were NONE!)  He felt that maybe they might have missed something and was adamant that was the direction he was going to go.  He told me to be sure he would meet with a panel of highly experienced Interstitial Lung Disease specialists the following week and would get back to me on their opinions.  I asked him if he had ever seen a case of "Ibrance induced pneumonitis".  He indicated that he had seen one.  (That little bit on his resume didn't impress me...just sayin)

A few hours later I visited with an Oncologist at the Huntsman Center.  She emphasized that this Pulmonologist was probably right and that I should follow his directions about getting another bronchoscopy and biopsy.  She told me that my symptoms should have cleared up when I stopped taking the Ibrance, which is why she didn't believe this was caused by Ibrance.  I asked her if she had ever seen a case of "Ibrance induced pneumonitis".  She said "I think, I might have seen one."  Needless to say, that amount of experience did not impress me either". 

I left the U of U and the Huntsman Cancer Center discouraged and second guessing myself...but not much.  I mean, I am pretty certain what caused this, but again, who am I to second guess the nation's top doctors?  

I went back to St George to talk to my Oncologist and ask his opinion about the new suggestion of Non-Tuburculosis Micobacteria and he was on board with it.  Because, although he has never seen a case of "Ibrance induced Pneumonitis" he is also thinking that is not what I have. (Because the symptoms should have gone away when I stopped taking the Ibrance back in July) 

Moving ahead to last Wednesday. The Panel of ILD doctors have all convened and they have discussed my case.  U of U pulmonologist calls and tells me he no longer thinks my symptoms are due to non-tuburculosis micobacteria, but they all agree it doesn't look like pneumonitis either.  They now want to do a lung biopsy and bronchoscopy because they think it might be cancer.  I told him that the last bronchoscopy ruled out any malignant cells in my lungs, and he told me he wants to do it again, because he thinks they might have missed something.  I asked him if my symptoms match the symptoms he would expect of someone with the type of cancer he was talking about. He said no, not really, but they still want to do the procedure to find out.  I think he is wrong, I think THEY are wrong, but who am I to argue with, now a panel of, the nation's top doctors? So I am scheduled to have a bronchoscopy and biopsy of my lungs on Wednesday at the U of U.

One other little thing that popped up last week.  I noticed my right foot started to hurt a lot last week.  It hurt for a couple of days to where I could barely walk on it, then one evening, after 2 days of pain, half of my foot went numb...like from the two small toes down to my heel.  I almost laughed hysterically at the ridiculousness of having one more thing.  I was actually hesitant to get it checked out, because I must seem like a doctor hopping drug seeker to everybody by now, but the next day I went to my podiatrist who was baffled by why my foot would be numb and hurting.  After a little discussion he suggested I get an ultrasound to be sure there was no blood clot.  

Long story short...there was indeed a blood clot.  Vein doctor was concerned enough to put me on blood thinners right away and get me a High Resolution CT scan to be sure I didn't have anything life threatening..considering the troubles I am having with my lungs and heart.  Fortunately nothing serious was found and I am left to work it out with a blood clot in my foot for now.  

One thing they did find is that I now have 3 healed broken ribs.  I wasn't surprised by that, because my chest hurt so much and I could feel my ribs moving at one time.  What surprised me is that with all the x-rays and CT scans, that last one was the first to mention broken ribs.  How have they missed 3 broken ribs all this time?????  I was in so much pain, and yet they never even saw it.  Is it any wonder I don't trust doctors?

I am now on a blood thinner with orders not to hurt myself, or bleed.  For some of you that might not be a problem, but as my husband knows, not bleeding, for me, is a challenge.  Haha!  It also complicates having a lung biopsy a little bit.  But I have been reconsidering having that procedure done and I think I will cancel it and just wait a bit.  

Because I have to wrap up my thesis...Back to mushrooms.  I am like 95% sure my condition is "Ibrance induced pneumonitis".  I have done many hours of research on the subject and have interacted with real people experiencing the same symptoms as I am as well as read numerous case studies.  I think my symptoms don't look anything like the cancer I know he is thinking I might have...as I have also researched that for hours. (lymphangitic carcinomatosis) I am extremely frustrated, and have about lost all hope that they will ever diagnose, much less treat it correctly. 

For the most part, I see improvements over the past few months.  Some days feel better, some days are miserable.  But the good days are better and the bad days ...well maybe they are not as bad sometimes.  Yesterday was bad and I was feeling very dark and unhappy.  Sometimes I feel so hopeless that this is going to get better that I just cry.  Then I will take something to knock myself out, get a good night sleep (upright as always) and wake up feeling hopeful again. Mornings are usually the best and things get hard as the day progresses.

This is truly miserable, but I realized something yesterday.  Something I have realized before to some degree, but needed reminding of...something the doctors don't understand.  They don't have to get the diagnosis right for me to heal.  I believe right now I am kind of like someone who has been poisoned by a Destroying Angel Mushroom.  Just because I stop eating the mushroom, doesn't mean I will just get better.  The damage is done, we cannot change that.  I need to rest...a lot, and I need doctors who will help me heal.  I need to strengthen my body with whatever it takes for it to heal. It seems that my body is strong, my heart which is in a constant state of tachycardia seems to be strong enough to handle that, my liver and kidneys and other vital organs seems strong enough to handle the onslaught of meds and toxins they have been given, my mind  might be able to last little longer (questionable).  

That is where my focus is  going to be right now.  I do have appointments next month with the Mayo Clinic in Phoenix.  I am not holding my breath... **Chortle**  

Oyxgen!!

 

This song has been in my head for months now.  Maybe the universe has been trying to tell me something.  I've been listening, but my doctor hasn't.  I finally got some supplemental oxygen and what a difference!  The lung spasms have been reduced by 80% or better.  They only spasm when I get too excited, angry, emotional, etc.  And don't even think of making me laugh...it really scares me when I try to laugh.  

One of the things that makes me angry, is how much suffering could have been alleviated if my doctor had cared enough just to get me some oxygen.  It would have been great if he had cared enough months even a year ago to check out why my pet scans were lighting up with with hilar lymph nodes and correlated that with the cough I was telling him about. It would have been nice if he had been interested enough just to run a simple CT scan, just to check it out.  I bet he would have seen the ground glass opacities then.  And then we could have stopped my meds and let it heal without the damage that has been done by letting it run rampant for over a year.  

Woulda, shoulda, coulda...WHATEVER!  Right now, although relieved to have found some relief and I try to focus on that, the anger of how simple it would have been for him to give me oxygen, if he had cared; how much suffering over the past 4 months could have been avoided.  Not to mention the damage done by coughing and spasming for 4 unnecessary months.  It is hard not to HATE...but anger makes me cough.  I am working on it.   

The improvements give me hope.  I have often said to the doctors that "No one thing works for 5 days".  When I got the oxygen, I felt the difference within 10 minutes.  It was amazing that I could hold a conversation without coughing and spasming.  Friday was a good day.  I felt so good, Ron and I even went out for dinner and did a little shopping.  Then the batteries started to die on the O2 compressor and we had to hurry home.  I guess I have about 2 hours on that, but I do have a car charger, so I just have to plan better. 

 But for every day I feel good and do things while I feel good, I pay for it in hours and even a day of suffering.  Even with the oxygen, Saturday was a hard day.

 

Even with fewer spasms, I struggled to get a comfortable breath of air all day.  I think I would best describe it as having itchy lungs with an itch that just never goes away.  Suppressing a cough that is non productive and only hurts and injures.  The high dose of prednisone makes me swell up like a bloated pig.  On Friday that didn't happen and I credited the oxygen for that, but Saturday I thought my head and chest would burst.  I was so miserable yesterday.

Finally by about 6 pm last night I began to find some relief and things started to relax again.  I lowered my oxygen a little bit, maybe that helped... "Love is like oxygen, you get too much, you get too high, not enough and you're gonna die..."  Balance!   I took about 900 mg of Gabapentin, knocked myself out and got a pretty good, vertical, night's sleep.  This morning I feel better.  Days like yesterday make me think I am sinking, mornings like this morning make me think I can tread water a little longer.  I thank my Heavenly Father for every day I 

Rare Reactions to Meds

So, many of my friends don't know this, but my cancer came back.  In about August of 2019 we found a 2 cm tumor on my left hip.  So that made me Stage IV, metastatic breast cancer.  Bummer!  Next Wednesday, October 13th, is Metastatic Breast Cancer Day...By the way.  So metastatic breast cancer is treated differently than Stages 0-3.  When you are 4 they consider it incurable and all efforts are put into extending life with a variety of chemotherapy, immunotherapy, hormone therapy, etc drugs.  

When I was diagnosed in 2019, I received 5 radiation treatments to the tumor in Gilbert with my old Radiation Doctor.  (If anyone is ever looking for a good Radiation Oncologist in the Phoenix area, I totally recommend Dr. Tannehill)  Anyway, the 5 treatments seemed to take care of things pretty well.  After that they gave me a new, recently approved drug called Ibrance as well as an estrogen suppresant called Letrozole.

About the time they prescribed Ibrance to me, the FDA came out with a Black Box Warning about Ibrance about a rare, but potentially deadly side effect:

So I began taking Ibrance in November of 2019.  It has its side effects, but I didn't take much notice.  Dry eyes, mucus membranes, cough.  Seemed tolerable.  Surely much better than the Chemotherapy I had to do 4 years earlier.  I mentioned the cough to my Oncologist in April of 2020 after a few months of taking it, and he brushed it off as tolerable, so if he wasn't worried, why should I be?

In April of 2020 I also stopped taking the Ibrance because I had a Rare reaction to the Zometta they gave me to strengthen my bones.  This caused me to have what is called Osteonecrosis of the Jaw.  What happens with this is pieces of my bones in my mouth would die and then slough off...kind of like a splinter.  Very painful mouth sores that would eventually leave exposed bone in my mouth until that little piece of bone would work its way out and fall out like a loose tooth.  (That is an entirely different story of where the doctors would not properly diagnose or treat it. I consulted with 12 dentists, oral surgeons, endodontists, ENT, ets.  Told me it was rare...and that I was wrong about what it was.  I was always right and because I knew it, I avoided them pulling or root canaling a perfectly good tooth.  One Dr even wanted to grind down the bone and leave me with a permanent opening that would never heal and I would be on life long antibiotics.)

I digress... So in April 2020 I stopped taking Ibrance and the cough and other symptoms went away.  In August, the mouth sores had healed, so I began taking the Ibrance again.  Not surprisingly, symptoms came back.  As time went on, I found the cough increasing and I found myself increasingly out of breath.  I live near the North Rim of the Grand Canyon and as many people know I have recently found a fascination with foraging for mushrooms...and because of all the rain it was a wonderful year for mushrooms.  I didn't think much of being so breathless up at those high altitudes that I was out of breath, but sometimes I was shocked at how hard it was becoming for me to wander in the mountains.  I kind of just chocked it up to needing to lose weight and getting old and out of shape.  

I always mentioned the cough to my Oncologist on our visits, and he seemed unconcerned, so why should I be?  Ron and I joined the Gym in May and started playing racquetball and working out.  In June I got a job that I loved delivering Meals on Wheels and working a the Senior Center in Kanab. 

 I had begun to notice other problems when sleeping.  I would wake up at about 3 AM and my lungs would be crackling and wheezing and I would wake up feeling like a was choking.  The cough was getting worse and I was beginning to worry.  By then I had read about the Black Box warning and began thinking that was what I had.  I mentioned it to my Oncologist at my June appointment, and he seemed unconcerned..as usual.  In July I stopped taking the Ibrance on my own.  Then in early July I felt so bad I went to the ER in Kanab because I was struggling to breathe.  After testing negative for COVID and the flu, they sent me home with instructions of how to treat the common cold.  

The next day I called my Oncologist and told him he needed to take this seriously and that I believed I had Pneumonitis caused by Ibrance.  He told me it was rare and referred me to a pulmonologist.  Well, I don't know if you all are aware of how much medical care has changed, but getting into doctors is a matter of many weeks and months...even if you can't breathe.  I never heard anything from the pulmonologist for 3 weeks.  

Long story short, after months of  treating GERD and Asthma and me trying to impress on them that they are wrong and that it is Pneumonitis caused by Ibrance, they just would not listen.  I felt they were/are treating me like Hypochondriac.  I have had a Bronchoscopy, endoscopy (to finally rule out GERD), echochardiogram, EKG, Overnight oximetry, Breating tests, etc.  They still were treating GERD and Unexplained Adult Onset Asthma.  I have been to the ER 5 times because sometimes I feel like I am dying.  They assure me my O2 is fine, and I am not dying and send me home. ...and I guess they are right, but I have never been so miserable in my life.  I am shocked at how much unmitigated misery they believe is OK to allow a person to try to manage with little oversight or direction.  

I am completely unable to lie down for more than about 5 minutes.  I have slept for the last 3-4 months completely upright...think airplane with pillows and blankets propping me all around.  I am on super high doses of Prednisone.  They started me low, and I on my own have upped them much to their dismay, because they were not giving me enough and  from what I was reading they needed to be giving much more.  Once I upped the dose they went along with me, even one insisting it was his idea.  Eventually I was up to even 200 mg of prednisone..which largely unheard of outside of a hospital. 

My lungs show tree-in-bud and ground glass opacities, they spasm all day long which is exhausting.  I can scarcely hold a conversation because  they spasm.  Kind of like dry heaving, but with my lungs.  If I do have a few moments that are good, and I exert myself, I will pay for it with hours even a full day of spasms.  Not much seems to help, except for me to choose from an array of sedatives that I take with the hope of just knocking me unconscious.  Gabapentin, Promethezine with Codiene, Tesalon Pereles, Advil PM, Ativan.  I take what ever it takes sometimes to just be unconscious.  I don't have much direction from my doctors, as I seem to be a hot potato they don't know what to do with.

I did finally find a pulmonologist for a 2nd opinion after the first one dropped me.  He was I guess put out because I doubled my prednisone dose to 200 mg .  He told me that would be appropriate if I had Grade 2 Pneumonitis, but that is not what I have (remember he was treating Asthma).  So he told me to find another opinion.  So I found one, (and got in with him after a couple weeks), and he immediately called my Oncologist and asked him to get onboard with this being Pneumonitis caused by Ibrance.  At which point my Oncologist finally solidified his diagnosis of Pneumonitis caused by Ibrance.  

He also told me at that appointment that it is RARE, they don't know how to treat it.   He acknowledged that I was scared and he promised me he would research it and talk to other specialists and find out what they should do.  It was nice to finally get some validation and some compassion and I felt hopeful.

Our next visit, he kind of reiterated the same, except with no new information or treatment except to take my prednisone down a bit (I am now at 100 mg) and take an anti organ transplant drug called CellCept to hopefully stop perhaps an autoimmune response to my own lungs?  Then he said we don't know what to do for you. 

 I am the only case of Ibrance induced Pneumonitis any of them have ever seen, and even Pfizer doesn't know how to treat it. However I see warnings that say "Delays in diagnosis and treatment may be fatal"  I have researched for hours of sleepless nights and have read about treatments that work and the patient recovers and those that don't.   (I am realizing now why I spent years studying to go into PA school, only to not.  At least I know how to research. By the way, that is the kind of thing that totally pegs you as a hypochondriac...Doctors seem to be really bothered by people who know medical terms and do research) I have begged them to hospitalize me and stabilize me, but for some reason they refuse to see this as serious enough to put me in the hospital, because my O2 levels are in the  90s.  However the ignore that the heart rate is much higher to keep it there.  

Ron and I walked around the block slowly the other day.  I did an O2 test. My heart rate was 136 with an O2 of 93, Ron's heart rate was 69 with an O2 of 93.  My heart works 2x as hard on a slow walk.  Did I mention I sleep vertical? ...and the spasms...oh the spasms.  

This General Conference talk actually woke me from a drug induced "coma" and spoke to me. It was exactly what I needed to hear 

I have sought other specialists from Scottsdale and the Mayo Clinic, but everything takes months. I have had an appointment since August for November 15.  I have an appointment with a pulmonologist who is supposed to specialize in critical care in California next Friday.  Today I miraculously was able to get an appointment with a top pulmonologist at the University of Utah, Huntsman Cancer Center.  At first they told me they couldn't get me in until Dec. 22, but I told him I would drop everything to come...with 5 hours notice to get there.  He called me back and got me an October 19th appointment with one of their top doctors.  

The length of time it takes to get treated worries me.  I asked my Oncologist to get me some oxygen to help take the burden off my heart something he assured me I don't need, but ordered anyway.  3 weeks later I still had no oxygen.  I finally called and demanded they FRICKING GET ME SOME OXYGEN!!  So tomorrow, after numerous follow ups from there, I will get my oxygen. I really think it will help...while I wait for my next appointment.  

 I believe I am saving my life.  I believe my research and dose changes and demands from my doctor are what have led my treatment.  My oncologist would disagree, because he needs cover his rear, but at least he cooperates with me most of the time.  I mean I doubled my dose of prednisone to 200 mg for 4 days before telling them...what could they do...you can't just drop prednisone after that.  I have forced their hand many times to treat me better and they really act like they are treating an ingrown toenail.  It is crazy making, but maybe I have a few specialists who can really help me now.  I sure hope so.

So that is it in a nutshell...a big nutshell.  It is therapeutic to blog..gets it out of my head and written, so I can stop incessant thinking...or at least slow it.