The Short Version:
Wednesday, March 28th, I was told I had Hairy Cell Leukemia. Cancer. I am meeting with a hematology oncologist in Lee’s Summit, Missouri next Tuesday and should undergo chemo within the next month.
The Longer, more involved, version with headings so you can skip around:
Hairy Cell Leukemia. Cancer. Simple. Concise. But I’m only 41. Forty-one. You’re not supposed to hear that “Cancer” word at this time in your life. Personally, I was hoping to never hear that word in relation to me, or my family. But he said it. HCL. Consequently, here we are. To better understand the whole scheme of things, we have to start a few years back.
In April of 2000, my left lung collapsed for the 3rd time. They called it a Spontaneous Pneumothorax…again. That’s a fancy name for “Your lung collapsed, and we don’t know why.” In September 2010, our youngest was born with a rare congenital heart defect called Hypoplastic Left Heart Syndrome, or HLHS for short. Basically, the left ventricle of her heart never developed so she has had to undergo 3 open heart surgeries, multiple heart caths, lymphangiography, lymphatic duct embolization, etc. etc. etc. (read her story here) And no one knows why. In January 2013 my brain bled. I had a subarachnoid hemorrhage. No one knew why. Do you see a trend here?
But, as I sat in the ER at KU med, due to other complications after being released from my hospital vacation, I had doctor after doctor coming in to see me. Finally, on the 10th or 11th doctor, I asked “Who are you and why do so many new doctors keep coming in?!” He kinda chuckled and said “Your chart is out there and we’re all reading it. Collapsed lungs, HLHS, subarachnoid hemorrhage. When we see things in multiples like that, there’s usually something else going on. An underlying cause. We’re just all trying to figure it out.” Well that’s comforting – dripping sarcasm
The next time I went in for a checkup with my primary care physician, she talked about how “sick” I had been and how “close I had come to death.” After hearing that I mentioned what the KU Med ER doctor had said. She replied, “Yeah, we’re just waiting to see what happens to you next.” Wait, what?! SERIOUSLY? You just told me I nearly died from a brain bleed and now you’re just waiting to see what happens next? WHAT’S NEXT?
It was time to go to Mayo. If you haven’t ever tried to get in at Mayo, it can be an interesting process. Fortunately for me, “When we see things in multiples…,” (maybe I should be saying unfortunately for me…) after sending my information in, they called me for an appointment within two days. It wasn’t a big deal.
At Mayo, I went through all types of testing, from genetic blood work to ultrasounds, and saw any number of doctors, all attempting to tie my collapsed lungs, brain bleed, and HLHS daughter together – I have been told multiple times that although they don’t know what causes HLHS, it’s not genetic. Yet, within the past two years or so, Brandy and I received a package from Texas Children’s wanting a saliva sample for some genetic research they were doing in relation to HLHS – and I digress. Back to the topic at hand. At one point I was asked by a doctor if I was super flexible, thinking maybe Marfan’s syndrome. My wife started laughing and said, “He’s the most inflexible person I know.” – Was she talking about my body stretching or my attitude? Did she just call me stubborn?
HHT and AVM’s:
Regardless, nothing was coming together, until I saw Dr. McPhail. Yes, that is his name. Dr. McPhail. He is a vascular doctor that asked for a CT scan with contrast, but to only put the contrast in while I was getting the scan. Kind of a simultaneous deal. In doing so, they came up with this computer-generated picture of my heart.
All the spaghetti like structures are not supposed to be there. They are called Arterial Venus Malformations, or AVM’s. Apparently, I grow extra veins and arteries, which is not a good thing. The veins and arteries in your are separated by capillaries. The blood from your heart flows through the high pressure arties to the capillaries, where the pressure is diffused, and then into your low-pressure veins. When these AVM’s are formed, there are no capillaries. Consequently, the junctions between the arteries and veins are weak and have a tendency to break. Knowing that I have had crazy nose bleeds since I was a kid, and now that I have AVM’s, Dr. McPhail started looking at Hereditary Hemorrhagic Telangiectasia aka HHT. HHT is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes – my nose bleeds, and often in organs such as the lungs, liver, and brain – hence my brain bleeding. This could also be a possible explanation for my lungs. But says nothing about a connection to my HLHS daughter.
In order to be diagnosed with HHT, you have to have 3 of 4 factors. I was already at 2, so I either had to have a family history where others were diagnosed with it, or there is a genetic test that isn’t 100%. Neither of which panned out. But at least now I have a possible explanation for my brain, lungs, and my random nose bleeds.
The majority of the time, when an AVM is found, they go in and remove it. Because of where this particular one is, they’re not going to mess with it. Consequently, every two years I get to go back to Mayo, have an ultrasound, have blood drawn, and see Dr. McPhail. Every 4 years, I get to have the CT scan with contrast that created the above picture. 2017 was a CT scan year.
As for my AVM’s, heart, and blood work, on Dr. McPhail’s account, I was a healthy 40-year-old that should easily live another 40. But, for some reason, my spleen was MUCH larger on the CT scan that it had been previously in 2013. There was something else going on, but Dr. McPhail had a theory that he was going to check out and there was nothing to be worried about. So we went home.
The next day, Dr. McPhail called. His theory was not correct, and he wanted me to go see a hematologist. We returned to Mayo in Minnesota the next week. Met with 3 different doctors, got asked 900 questions I answered “no” to the majority of the time, had some blood tests done, and an ultrasound of my spleen. Nothing was conclusive. They told us to go home, have the same blood test and ultrasound done again in 3 months “just to be sure,” but likely I had had some infection and my spleen was enlarged because it was filtering out bad cells. We were told, “No need to return, just have the tests done in Lawrence and the results sent here.”
We decided we would rather go back to Mayo. When my wife called for an appointment, the doctor didn’t have any for a few months because he was on hospital service. He told the nurse that I could meet with his PA. He was certain there wasn’t going to be any problems and that my spleen would have returned to normal.
We went back to Minnesota. I had an ultrasound and bloodwork in the morning, then went to meet with the PA in the afternoon. As we were sitting in the room, the PA walked in and then the Dr. walked in right behind her. He said “I’m so glad you came back! I was just sure this was nothing.” My mind whirred. I said, “I thought you were on hospital service?” He replied, “I am, but I read your reports and called her (the PA) to tell her I would be here.” Wow, um, this is not good. He asked me 900 questions again about pain and an asundry of other things I had no answer to. He said he already requested that they do a different test on my blood and pending the results of that test he wanted me to have a bone marrow biopsy. Did I mention I hate needles?
The receptionist asked the PA if she was ordering the sedated bone marrow biopsy or non-sedated. I interjected and said, “If it’s not the sedated biopsy, I will have my wife punch me until she knocks me out.” She ordered the sedated biopsy. They scheduled the bone marrow biopsy the next day for 12:15, pending the outcome of the blood test.
We hadn’t heard anything from the Dr. by 11:30 so we went up to his receptionist desk on the 10th floor. They called the PA who looked up the test on the computer. It was still pending, so she was going to call the doctor at the hospital. He called the lab and then called Brandy. He told us the test was still pending, but he really thought we were delaying the inevitable. If it were him, he would go ahead and do the biopsy. I cringed but agreed.
HAIRY CELL LEUKEMIA:
After the biopsy, we were packing up to head home because the results of that were not going to available for a few days. The doctor called. He told Brandy that my blood test had come back positive for Hairy Cell Leukemia. The bone marrow test would confirm or deny it as a diagnosis. He didn’t really tell us anything about it. I was in shock. Being a pessimist, my mind went directly to death. Do I need to make videos for my kids for the future? Do I need to put together my funeral? Etc., etc.
We drove home, and I read about HCL online. After reading about it, it didn’t really sound like it was that big of a deal. I told Brandy that and she glared at me saying, “You can’t say cancer and not a big deal in the same sentence.” Ok. That’s fair. Point well made. The doctor called the next day to say that the bone marrow biopsy confirmed Hairy Cell Leukemia.
I was standing in the office of my pastor when Brandy called to tell me. After I got off the phone, he asked how I was. I had to say I wasn’t really sure. It was surreal. I didn’t know how I was supposed to feel. I don’t know that I know now what I’m supposed to feel or think. I went to the bathroom, looked in the mirror, and said, “I have cancer.” Nothing changed. I didn’t feel any different. Life was the same. It’s strange that way…
We returned to Mayo to meet with a specialist, Dr. Call, who only treats Hairy Cell Leukemia. To this point, all the information we had came from the internet, and none of it was about me specifically. We were eager to hear what he had to say. And he had a lot to say. He probably spent an hour to an hour and a half talking with us.
In summation, the Leukemia I have is rare and makes up only 2% of all leukemia’s. It used to be very difficult to treat. But because of a drug approved by the FDA in 1992, it is very simple. Chemo is 5 treatments. 1 per day for 2 hours, 5 days straight. Everyone who has this type of cancer gets the same treatment. It’s an “easy” treatment, but it’s a matter of how your body responds. 2/3’s of people go into remission for 15-20 years after going through chemo once. That sounds awesome! Yes? He said we would talk about the other 1/3 if it came to that. In other words, it ain’t good. I assume it goes back to the “it’s very difficult to treat” statement. But, it’s also not really the cancer that they’re concerned about with me. In fact, the doctor said it should be a “fairly boring process” for me. It’s all the other complications, especially with my other health issues – i.e. AVM’s, sickness, and the higher incidence of other cancers. Now I get to see a dermatologist at least once a year because of a higher incidence of skin cancer. Yipee!
For me specifically, he explained that 70-80% of my white blood cells are cancerous, meaning I have a greatly weakened immune system. With HCL comes a lower platelet count as well. A normal platelet count is between 150K – 400K. In 2013 mine was 230K. In normal people they would do major surgery with a platelet count of 80K. They would do minor surgery at 50K, and give a transfusion at 10K. But, because of my AVM’s, they don’t want my platelet count dropping below 50K. Right now, it is between 70 and 90K. They don’t want my brain bleeding or something and having my platelet count that low. This explains why my nose bled like a faucet over a trashcan for 40 minutes the other day.
Dr. Call said it wasn’t an emergency but advised me to be treated within the next month. He recommended that I go see a Doctor closer to home for treatment. That way, if something did go wrong, especially during the 6-week follow-up when they check your blood counts regularly, I would have a doctor that knew what was going on close to home. Only problem is, do they really know what’s going on? See, when we asked about the chemo going through a filter in the IV because of my AVM’s, Dr. Call responded, “I’ve never heard of that. You’re teaching me something here.” Awesome, if you don’t know, why would someone with less training than you? Needless to say, I have reached out to Dr. McPhail, the vascular doctor discussed previously, to get his thoughts.
In the end, I am headed to see a Dr. Smeltzer in Lee’s Summit, Missouri, tomorrow. He comes with high recommendations as Dr. Call said he would send his own family to him – Which is great, but would he send his own family to the hospital there if something went wrong? Ahhhh, the pessimist in me took over – We will see what Dr. Smeltzer has to say and what kind of plan he comes up with for the next month on my life.