I was officially diagnosed with Stage IV lung cancer on February 14th, 2017, Valentine's Day ... not the most romantic way to spend Valentine's Day with your husband! That was the official start of my lung cancer journey.
But that's not the real start of the story. The real story begins before diagnosis, when subtle symptoms began and were ignored, first by me, then by my care providers. Over time, things gradually worsened, and then suddenly went downhill quickly. By diagnosis, I had nearly all of the symptoms of lung cancer, including the classic ones that use the mnemonic BREATHE:
- Blood when coughing
- Recurring respiratory infections
- Enduring cough
- Aches in the shoulder, back, or chest
- Trouble breathing
- Hoarseness or wheezing
- Exhaustion
In addition I had significant unintentional weight loss and the unusual symptom of skin metastases.
Even with ALL these symptoms staring them in the face, my care providers still did not recognize lung cancer in me. That's probably because they didn't expect to see it in someone my age who had never smoked. In addition, because I am plus-sized, weight bias blinded them, giving them something to blame my symptoms on so they didn't seriously consider lung cancer.
By the time I was diagnosed I was nearly dead. Even with treatment, a month later I was in the hospital in the Intensive Care Unit at death's door. But let's start at the very beginning to better understand the journey.
This is the long version of my story. For the shorter version, see My Treatment Story.
This is the long version of my story. For the shorter version, see My Treatment Story.
Symptoms Appear
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| Subcutaneous skin lump (not mine), bottom right hand side |
The really noticeable symptoms didn't start until October 2016, but then they accelerated quickly. I developed weird lumps under my skin; more and more began appearing in the next couple of months. I thought maybe it was Dercum's Disease (painful fatty lumps all over the body), but my lumps weren't painful. It wasn't Dercum's; the bumps were subcutaneous metastases of my cancer but I didn't know it yet.
I made an appointment to see my primary care provider (PCP) at Thanksgiving. She canceled the appointment for a family trip. Rather than see someone else, I rescheduled for Christmas break. Just before Thanksgiving I developed a nasty respiratory bug that just wouldn't get better. I worked all through December but I was miserable. I had a tremendous wheeze and rattle when prone and I had trouble catching my breath when trying to do anything. I had a fever. I was sure I had Walking Pneumonia.
I had an ultrasound of one of my bumps, but it was inconclusive. No one seemed very worried about it. I went to my doctor appointment in December feeling very ill and hoping for antibiotics, which is rare for me. The traffic on the way to the appointment was extra horrible and I arrived 15 minutes late. My PCP felt she couldn't fit me in. Because I was feeling so sick, I did get an appointment with a clinic doctor who had a free spot. Unfortunately, he was a lousy doctor.
Bad Doctor
I did have a fever so he concluded I had a sinus infection. I was insistent that something was going on with my lungs. He gave me some antibiotics that he said would take care of the sinus infection and the pneumonia if there was any. I went on my way, expecting to feel better soon, but I didn't. The antibiotics helped my sinuses a little but did nothing for my breathing.
I went back or called in January several times. My breathing was getting worse and worse, but he dismissed my symptoms. He didn't believe how sick I was. He would not consider a different type of antibiotics, he would not order a chest x-ray or CT scan, he did not do percussive testing, he didn't seem concerned when I told him how much trouble I was having breathing. I was so frustrated.
At one point he told me it was probably asthma (the implication being I must have asthma because I am fat) and gave me an inhaler. It didn't help.
By the end of January I couldn't even walk down the hall without taking breaks because I was so short of breath. This was not at all normal for me and a big change from just a few months before. I knew something was really wrong and I was getting scared, but I couldn't get him to take it seriously. When I began coughing up blood, I knew I had to go back again and get some answers.
Finally, Some Results
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| Nearly 2 L of fluid drained from around my L lung; the color should have been yellow but was red instead |
She was very concerned about all the lumps, the trouble breathing, and my 40 lb. unintentional weight loss since November. She too was wondering about asthma (?!!?) but needed to rule out other things. A chest x-ray was finally ordered.
I had the chest x-ray on Friday February 3rd. The radiologist was alarmed and wouldn't let me leave until I saw the image and promised to get follow-up ASAP. One of my lungs was surrounded by fluid (pleural effusion) and was basically two-thirds collapsed. No wonder I couldn't breathe!
It took a week to arrange with insurance, but we finally got a thoracentesis. They put in a big needle and drained 1.8 L of fluid off my lung, nearly the equivalent of a big bottle of soda. There was more, but we stopped because doing too much at once can be dangerous and I couldn't tolerate any more. My lung had been compressed so long that scar tissue had formed and it had trouble reinflating. I kept coughing but it was very painful, truly miserable. They sent my pleural fluid off to a lab to be tested for cancer and type of mutation. Afterwards, I had a bunch of CT scans.
I asked what color the drained fluid was. My husband showed me the picture he took, the one seen above. It was bloody. My heart sank. I had done some reading on pleural effusions and knew that if a pleural effusion was bloody, chances were quite high that it was lung cancer. That's the moment I knew I had lung cancer, although no one had said it yet.
I was absolutely floored. How could I have lung cancer? I had never smoked, not once. Nor did I live with smokers. I didn't know at that point that non-smokers can get lung cancer too. Lungs are all you need.
We went back to my primary care provider for preliminary follow-up on Friday, Febrary 10th. I was pretty sure I knew what was coming. I went in by myself; I didn't want to have to manage my emotions for my husband. My PCP, bless her heart, was very empathetic but didn't beat around the bush, for which I was grateful. She told me it was cancer and it looked like it had spread all over, but we had to wait on the final results to know for sure. They brought my husband in. We were all stunned. Lung cancer in a never smoker? In someone middle-aged?With no exposure to radon or asbestos? In someone who did all the cancer tests and had good results - colonoscopy, mammograms, pap smears, etc.?
I went into information-gathering mode and was mostly matter-of-fact in that appointment. I knew that I had more medical understanding than my husband and really needed to pay attention. I was plenty emotional but needed to keep it together for my husband. I also didn't want to walk out of the office in front of others as an emotional mess. When we got home, my husband took the dog for a walk so we each had private processing time. We each had our own small meltdown.
After that, we were calm enough to talk about things and most importantly, how we were going to break it to the kids. We concluded that they were old enough that we should just give it to them straight. We told them one at a time and they took it as well as could be expected.
The first available oncologist appointment was the following week on Valentine's Day. I hated that. I didn't want a cancer diagnosis screwing up what should have been a joyous and sentimental day. It was a very, very long weekend waiting for the appointment. At that point I was so physically sick I could hardly eat anything. The most I could keep down was a spoonful of honey.
My Terrible, No Good Valentine's Day
Silence.
I will always appreciate that he gave us time to absorb the news before going on; I needed that time to process it all. I sat in stunned silence for a moment and then said "F&*!#%?!!" I don't usually swear much but the F bomb was all that was in my brain right then; I had no other words. He wasn't offended and waited patiently. That was one of his best gifts to us in this process. Most doctors start spouting information about treatments, further tests, survival, etc., immediately, but we needed the time to absorb the actual news. Anything too soon just wouldn't have been heard.
When I was ready, he went over my scans with me, image by image. The cancer was everywhere. That took care of any denial that was left. I was Stage IVb, the worst you could be. The primary tumor was in my left lung, but it was also in my lymph nodes, my liver, my bones, and of course, my skin. We found out the following week it was also in my brain (including Leptomeningeal Disease, LMD) and in my breasts (despite a clean mammogram 9 months previously). I was well and truly screwed. I was in despair.
I shared that my (adoptive) father had died of lung cancer and I had helped with his care. I knew what I was in for and that terrified me. He stopped me right there and said that lung cancer care was very different now and there was much more we could do. He began telling me about newly identified mutations that actually had a much different prognosis. We wouldn't know for a couple of weeks whether I had those mutations but if I did, there was a lot that could be done to prolong my life and improve its quality. Even if I didn't have one of those mutations, there were still treatments available. It wasn't hopeless.
Apparently, the field of lung cancer had undergone revolutionary changes in the last 5 years or so. A straight-shooting nurse friend had told me that the weekend before; I wondered if she was just trying to cheer me up but apparently it was true. A co-worker shared with me that his aunt was still alive quite a few years after a lung cancer diagnosis. That gave me some hope that I might be around a bit longer for my kids. But so much depended on the type of lung cancer I had, and we wouldn't know that for another week or two. So we got more tests, arranged for time off work, and waited. It was a long couple of weeks, but the results finally came in.
The "Lucky Unlucky"
Turns out my cancer is Non Small Cell Lung Cancer (NSCLC), with the subtype of Adenocarcinoma. Within that subtype there are a number of different genetic mutations, like EGFR, ALK, ROS1, KRAS, BRAF, RET, and others. Some have targeted therapy treatments; some do not.
I am a Lucky Unlucky because my mutation, ALK Positive, is one of the mutations with the most treatments. Only around 4% or so of NSCLC is ALK+ but they already have 5 different targeted therapies for it and hopefully more in the pipeline.
People who get ALK+ lung cancer are usually never-smokers and younger than average. Average age for LC on the whole is about 70; for ALK it's 55. ALK tends to be a cancer of young and middle-aged adults. I was 55 when I was diagnosed -- right about average -- but there are people as young as their early 20s who are getting diagnosed with ALK. I guess I should be thankful that I didn't get it until my 50s. At least my children were older when I got it, and at least I had the opportunity to have children! Not everyone does. It's heartbreaking.
No one knows why some non-smokers get ALK. The biggest culprits are probably radon exposure, asbestos, or pollution. To the best of my knowledge I didn't have any of those exposures. The only factor that I can confirm was hereditary. My birth father, a doctor, died of lung cancer near my same age; he died at age 59.
Lung cancer is not directly inherited but there does seem to be a modest increase in risk if you have a first-degree relative with lung cancer. This is usually blamed on shared environmental exposures, but since I was adopted and never shared a home with my birth father, that couldn't be it. Likely there was something genetic about it, considering we were both diagnosed in our 50s, or it was one heck of a coincidence. I suspect my birth father was ALK+ and that was the source of his lung cancer, but no one knew how to test for it back then. We'll never know for sure.
Early Treatment
Shortly afterwards, I started Whole Brain Radiation. The radiation oncologists did not advise me to stop the crizotinib during radiation. That was probably a bad move and may have caused complications later.
We debated whether to take steroids concurrently with the radiation. I had seen how destructive steroids had been to my (adoptive) dad in his lung cancer, so I chose not to start them and hoped I wouldn't need them. Alas, I did.
Radiation was easier than I supposed at first. Hardest part was having the mask over your face so closely. About as claustrophobic as a brain MRI, I guess...both are pretty hard when you are claustrophobic like me. But you just tough it out and deal with it. After a week or so, I started getting headaches. We realized I was having brain swelling so they started me on the steroid dexamethasone. The first couple of days were a hoot, because it makes you really hyper and busy. My house got really clean!
I finished up my brain radiation without too many problems, but then the steroids made my normally good blood sugar go through the roof. We weren't warned that this might happen and they didn't have us taking any medication to counter it. I began to get sicker and sicker. We didn't recognize that I had all the classic symptoms of diabetes like thirst and a constant need to pee. I got weaker and weaker. I had a hard time eating much. I thought I was just tired from the radiation but it was actually more ominous.
I was volunteering at school a couple of days a week; I went in a wheelchair. Pretty soon I couldn't even do that. One evening I came back from an appointment and collapsed on the stairs into my house. That's all I remember; after that it's a blank. My family tell me they helped me into the house, where I collapsed onto a recliner in the living room. I spent the night there. In the morning, my husband could not rouse me at all, so he called 911. I was in some sort of semi-coma.
Emergency and Hospital Stay
On Tuesday, March 22, 2017, an ambulance came and took me to the hospital. At the E.R. they placed a central line and did a bunch of tests. I was either unconscious or altered during the whole thing. According to my husband, they found:
- A Collapsed Lung (Pneumothroax)
- Atrial Fibrillation (heart rhythm problems)
- Dehydration
- Hyperglycemia (one of the highest blood sugars they had seen) from steroid-induced diabetes
- HONK syndrome (Hyperosmolar Non-Ketotic coma)
- Hyperkalemia (too much potassium) and Hyponatremia (low sodium)
- Severe Sepsis and Septic Shock (blood-borne infection all over my body)
- Protein-Calorie Malnutrition
Basically, this episode was caused by a combination of complications: high blood sugar from the steroids I was taking because of the brain radiation; severe dehydration and malnutrition; a very bad systemic infection of some kind; and the TKI working so well that it actually collapsed my lung as it shrank my tumors.
I was extremely ill. Honestly I think I was close to dying. I didn't have the classic out-of-body near-death experience, but my consciousness was definitely altered. I kind of felt like a balloon that was tenuously tethered in the darkness. I was floating around, not sure where I was or where I was supposed to be, but not quite floating completely away either. My husband, God bless him, played my favorite classical music on my phone for me continuously. I remember feeling like that music was what was keeping me tethered to this world. It got through to me on a level of consciousness that nothing else did. It made me want to stay and listen when I felt like I could easily have floated away.
A day later I woke up. I stayed in the Intensive Care Unit for several days, then transitioned into a regular hospital floor after the chest tube was out. We slowly began weaning from the I.V. and getting me up and moving, but I was incredibly weak. During Spring Break my children were able to come spend time with me in the hospital. They tried to arrange it so I was never alone. After about a week I was discharged to a rehab center and they continued to visit me there. I was so thankful they were old enough to understand everything and to visit.
Rehab Center
I had lots of Physical Therapy (PT) and Occupational Therapy (OT) while I was there. My task was to regain my strength, start eating again, and get functional enough to go home. I started in a wheelchair and graduated to a walker, eventually taking longer and longer walks with it.
One of the things that was irritating about rehab was how they assumed that because I was fat, I had type 2 diabetes already and must know all about insulin. I kept telling them this high blood sugar was because of the steroids I was on; I wasn't actually diabetic normally. But they always assumed I knew how to manage insulin dosages. I kept telling them I had never used insulin before so I had no idea what dose I should be taking. No one had ever given me any training in managing the insulin or proper dosing of the various types of insulin. They kept asking me what dose they should give me! I had no idea.
At one point they were planning to discharge me home without ever having taught me how to manage the insulin or be able to get up the steps to my house. My husband and I wouldn't agree to a discharge until they had a plan in place for the insulin and I was able to navigate steps again. Fortunately, as my dose of dexamethasone tapered off, I no longer needed insulin. My oncologist, bless him, discontinued the insulin, despite the protests from my nurses. They would have kept me on it because they only ever saw me as diabetic.
My blood sugar drifted back to normal when I finally got rid of the dexamethasone. I was able to eat more food, and I began feeling a lot better. My PT concentrated on helping me build endurance and learn to climb stairs again so I could go home.
Pleural Effusion
The oncologist suggested I consider a PleurX Catheter, a small plastic tube that would stay in the space around my lung and which we would drain daily. We had a thoracic surgeon place that in mid-June 2017. He had me stay overnight in the hospital just in case I had another lung collapse, but all went well. The nurses taught my husband how to drain it and keep it as sterile as possible. Nursing is not in his comfort zone but he did great. We even had some of my teens learn it for when he had to be out of town.
I didn't love having the drain in because sometimes the bandaging irritated my skin or got itchy and sore. However, it was a HECK of a lot better than going in for a thoracentesis once a month and worrying about if I was numbed enough. The hope was that the plastic tubing in there would cause inflammation and scar tissue in the area, and the regular drainage would make it easier for that space to close up and scar over. Often, with enough time and the right meds, a pleural effusion will slowly go away.
My pleural effusion never went away while on crizotinib, but once I was on alectinib, it finally began drying up. It was a long, slow process, but little by little each month we drained less. Finally, about 15 months after placement, we were able to have the PleurX Catheter removed. Huzzah! That was a wonderful day.
In January of 2018 I had a spell where I either passed out or had a seizure. We decided that probably represented progression in the brain, which crizotinib is especially bad at preventing. So we switched TKIs.
Now I continue on alectinib. Part of me is waiting for the other shoe to drop, but the other part of me remains grateful for the extra time that being ALK+ has given me. Thank you to all the researchers and doctors who have worked so hard to make this a possibility. I hope for the day when lung cancer truly is a chronic disease to be managed, rather than a death sentence.
In the meantime, I am trying to stay in the moment so I don't miss out on the joys of everyday life and being with my family. Carpe Diem.
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