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.