Tim’s story
If I were asked to sum up my experience with hairy cell leukemia in one word, it would be “disbelief.”
If the doctor had said a few months ago, when I was undergoing tests to see what was wrong with my body, that I had heart disease or brain cancer or an ulcer or an infection, I would have been scared and sad. But I wouldn’t feel disbelief.
When he did tell me over the phone that day in mid-November that it looked like I had something called “hairy cell leukemia,” a rare blood disorder, my response was a wave of disbelief. As I did some quick Web research to find out more, that feeling only intensified. I had never heard of it, nobody in my family had ever had it, and most of all, these things don’t happen to me. Only about 300 people a year are diagnosed; I figure more people win lotteries. Surely there must be a mixup.
A flurry of questions followed: What exactly is it? Is it fatal? What’s the treatment? What’s the long-term prognosis? And, why me?
I’m a male, 45 years old, athletically active, with a wife and three teenage sons. I’m about as statistically normal as can be. My worst vices are chocolate and a beer once or twice a month. Here’s my story, written to offer anyone else out there diagnosed with HCL any help my case might offer. For I’m sure that you, like me, are in disbelief.
Chapter 1: The start
I first went to my family doctor in mid-October to ask about a sore abdomen. About a week earlier, I had played tennis on a Wednesday night. We play doubles, and the Wednesday players are much better than I am. They let me in because they’re usually hard-up for a fourth. When I play with these guys, I always have to run and hit harder.
The next morning at work, I felt very, very tired, which isn’t unusual after Wednesday night tennis. I just felt like I’d been really beaten up … sore muscles, little energy. No big deal, I figured. I’m just not as young as I used to be. In times like these, I’d often sneak away at lunchtime to me car and grab a quick 20-minute nap. But that day, I went out to the car and had such strange pains in my shoulders that I couldn't even recline in the seat. I never felt anything like that before. I figured it was like bursitis or arthritis: something deep in the bone. I closed my eyes for a few minutes sitting up, and when I got home I took a couple of Excedrins, which completely eased the shoulder pain.
But my abdomen muscles kept aching. I figured it was time to see the doctor. He figured I had pulled muscles while playing tennis and prescribed 10 days of Naproxin, a super aspirin of sorts. After 10 days, the pain seemed to have localized to an area below my rib cage on the left side. I went back to the doctor, and he looked puzzled. He suggested a complete physical, including a blood test. He also referred me to a gastro-intestinal specialist, saying I probably needed a colonoscopy to find out what was wrong in there. It was the start of my confusion and of a string of visits to various doctors who poked and scanned my body to make a diagnosis.
My chapter 1 actually begins several months prior to October. As mentioned, I was athletically active. In the spring and summer, I train for mini-triathlons. During lunch, a group of us either run (4 to 6 miles, hills or flat), work on weights, run and swim (1/4 mile), bike and swim, or bike. I often add a bike ride home (15 to 28 miles, depending on route). I also coach soccer, so I might play in scrimmages with the kids (ages 15 and 16). Plus there was tennis one or two nights a week, mountain biking or hiking with the kids on the weekend, etc.
Last summer, I noticed that my training was going poorly. I just didn’t have the strong drive to go long distances. Even short runs seemed like more of a struggle. I found myself backing off from really strenuous outings, and taking days off for rest. I really figured it was just a sign of getting older.
One day I knew something was wrong. It was a beautiful summer morning and I had arranged to work a later shift. I got a full night’s sleep and set off on my bike for an extended ride to work. The conditions were perfect, and I set out full of optimism. But during the ride, I simply had no energy. I tried to drive hard up some hills, but it was like pushing the gas pedal and not having your car move. I ended up actually walking my bike up some hills and straggling into work, very disappointed and puzzled.
My chapter 1 probably begins before summer. I can’t put my finger on it, but I have had an intuition for a long time now that something is wrong. I had a fleeting thought or impression that I might not live long. I don’t know why. It was just something that came across my horizon, and I never voiced it to anyone.
Chapter 2: The diagnosis
My meeting with the gastro-intestinal specialist was on Wednesday, Nov. 21. He sat me on a bed and listened to my description of the pain, then probed gently along my abdomen. Nothing pointed to an obvious problem. He said that people get their insides probed in three basic ways: colonoscopy (a tube inserted through the colon), endoscopy (a tube down the throat), or CT scan, which is a high-tech imaging system. Much to my relief, he suggested that I start with a CT scan, the least invasive of the three.
“Meanwhile, we should do some more bloodwork on you,” he added, almost as an afterthought. I could go to another area of the hospital that day and have a blood sample drawn, so I did.
His phone call came on the following Monday. He said that my blood counts were irregular. The white, red and platelet counts were low, and the appearance of the white cells “raise a concern.” “It’s possibly a leukemia -- a very treatable form, fortunately,” he said. “It’s called hairy cell leukemia.”
I turned pale and tried to speak lowly to him to prevent my co-workers in the cubicle from hearing. I asked him to spell it out. He assured me that this was a very preliminary diagnosis and referred me to an oncologist at the same hospital who had dealt with this disease. In fact, he had already made an appointment for me the following afternoon. I shouldn’t wait any longer, he said.
Within just a few days of going to my family doctor for an abdominal ache, I found myself referred to an oncologist. Cancer. Leukemia. Hematology. These were all foreign concepts. This type of thing doesn’t happen to me.
I’ll call the oncologist Dr. K. Trying to find his office, I was directed to the cancer area of the hospital. In the waiting room, people in a variety of stages of cancer came and went, some using walkers to get around, others wearing hats to hide their bald heads. Dr. K. finally called me to a small room. “So what do you know about this whole situation?” he asked. And then he listened while I told him all about the Web research I had done on HCL. He listened to me until I had exhausted all my knowledge and mis-knowledge. He was the first doctor whom I had seen who listened before talking.
Dr. K told me that all signs pointed to HCL. The abdominal pain, he explained, was my spleen, which had become enlarged because it was filling up with clusters of the rogue white cells. They would take another blood sample for a flow cytometry analysis to confirm the finding. He recommended against getting a bone marrow sample, saying that the flow cytometry was proving to be accurate 99 percent of the time. “If you’re going to get leukemia,” he said. “This is the one to get.”
Dr. K called me at work a couple of days later. I transferred his call to a private office where I could ask questions freely. The flow cytometry confirmed the initial diagnosis. Again, I was stunned and shaken. And I didn’t know whether this was a big thing or not. There were the dangerous, loaded words involved: leukemia, oncology, chemotherapy. Yet this was definitely curable. It was all still sinking in.
He said I would still need to have the CT scan done to check out the condition of my spleen. I did so the next day. A CT scan is a very simple procedure. In the hours before the test, you have to drink several bottles of a vanilla-flavored liquid barium that coats your insides. You then lie on a table, and they put an IV into your arm that feeds even more of the stuff into your veins. Then, the table moves under a big tube. You take a deep breath and hold it for 24 seconds, and basically you’re done.
The young woman taking the pictures came running out of the room after my pictures were taken. “Have you been in a car accident or something like that lately where you had an impact?” she asked.
“Are you referring to my spleen?” I responded. “It’s enlarged from a leukemia. Just how big is it?”
“Well, we’re not supposed to tell you because we’re not radiologists, but it is pretty big,” she confided. “How big?” I pressed. “Well, BIG,” she said.
Dr. K said the CT scan showed the clusters typical of HCL, and it also showed some bleeding areas at the bottom of the spleen. That’s a concern. The spleen is a blood filter, and you don’t want its contents seeping into the rest of your body. But he said it wasn’t time to panic because my blood counts were still above the danger thresholds. On November 28, they were:
WBC: 5.2 K/ul
Lym: 3.1 R2 60.1%L WBC
Gran: 2.1 39.9%G
RBC: 3.85 M/uL
HGB: 11.9 g/dL
HCT: 35.0%
Plt: 90 K/uL
Dr. K suggested this course of action: Monitor my blood levels over the course of a month, and have another CT scan taken in about a month. That way, we could see whether the spleen was still “leaking,” or whether the body had absorbed the blood. The idea was SOS: save our spleen. He predicted that my blood counts would continue to regress over the course of a month, and that I should plan to have chemotherapy within a month or two. His recommendation was 2-cda (commonly known now as cladrabine), administered over a week-long period through a PICC line (more on that below). He said it could be administered in the hospital, or at home through a small pump mechanism that you carry around. My choice depended on my personal preference, and what my insurance covered. We discussed alternatives: interferon or a splenectomy. But he said his experience with cladrabine had been very positive. I couldn't find anything in my research that suggested otherwise.
My second CT scan, as well as another blood test, was Dec. 20. Good news: The spleen looked healthier and could stay put. And the blood? Dr. K looked at me directly and said, “It’s time to schedule chemotherapy.” My counts were:
WBC: 3.7 K/ul
Lym: 2.8 74.6%L WBC
Gran: 0.9 25.4%G
RBC: 3.65 M/uL
HGB: 11.1 g/dL
HCT: 31.8%
Plt: 83 K/uL
Chapter 3: Preparing for treatment
On Friday, Dec. 28, I went to the hospital to have a PICC line inserted in my arm. PICC stands for peripherally inserted central catheter. It’s a small, soft tube that is inserted into a vein in your arm pushed all the way up the arm and into the largest vein in the chest, the superior vena cava. They choose the length of the line based on your height; mine was 54 centimeters. I asked the technician if I could see the line. It was a small, white tube, about the size of your vein. The technician felt around on my arm, and found a good vein right in the inside crook of my arm. The location was good because I could still bend my arm with the line in it.
Preparation is simple. They prep the arm by washing it with disinfectant, give you a quick shot of novacaine or something to numb the arm, then feed the tube into the vein. Don’t look if you’re squeamish. I didn’t. The tube has a small metal feeder that helps push it through. The technician tapes a small holder onto your arm to keep the tube in place. It’s topped off by a small screw-in cap. At the end, they take a quick X-ray to see if the tube took a wrong turn somewhere and ended up toward your ear inside of your chest. My X-ray showed that the tube was in the right location, so the IV technician pulled out the metal line and wished me luck. The whole procedure lasted about an hour.
Dr. K and I had several conversations about the best way to undergo chemotherapy. He likes the idea of people being as “normal” as possible during the process, and having things to do to make the time pass quickly. He never came right out and said it, but I sensed he thought at-home treatment was best for cladrabine. But I had other questions: Should I do it with my family at home? Will I need help? What happens if something screws up in the middle of the night? One of my sisters works in the oncology unit at a local hospital and recommended that I do the treatment in the hospital, where they could respond quickly if there was a problem. He said that it was his experience that most people don’t get very ill while taking cladrabine. In fact, he said some people continue to work, or work part time.
I decided to choose the at-home option because it sounded most comfortable to me, I could have access to things like the computer, e-mail, TV, VCR and my own bed, and I was confident I wouldn’t get too sick. I liked the idea of thinking and acting as though I were more healthy than sick. Plus, my insurance covered this option.
Here’s how this option worked for me. The hospital installs the PICC line, but an infusion company, which is a home-health care service that specializes in at-home chemotherapy treatments, comes to the patient’s house and sets up the chemo. They came to my house the afternoon after the PICC line was installed.
The nurse unpacked a large box of materials, including pamphlets, the pump, and the small bag of cladrabine. The pump is called a CADD Prizm. It’s about the size of one of those old tape recorders, before microcassettes (9 inches by 4 inches by 2 inches thick). It goes in a small purse that you can hang on your shoulder. The nurse hooks up a plastic tube from the pump to the screw-in cap at your arm, and welcome to the world of chemotherapy. The pump makes a quiet whirring sound about every time minutes as it sends another minute dose of cladrabine into your bloodstream to do battle with the rogue hairy cells.
But before leaving, the nurse said there were a few things that we had to know. Looking directly at my wife, she said that if I broke out into hives, or stopped breathing in reaction to the chemotherapy, I would have to be given a shot of epinephrine -- immediately in the thigh. Then we would have to call 9-1-1. She also said to watch for any redness around the area where the tube went into my arm, and to be aware of spreading numbness or tingling sensations in my fingers and feet. She recommended that I take my temperature twice a day to watch for fevers. If I had a fever about 101 for a prolonged period, I should call my doctor. She also showed us how to replace the battery in the pump without erasing the program.
She said to keep my arm dry and told us how to use saran wrap at shower or bathing time. And finally, she went over a patient disclosure form that indicated the price for 2-cda was $1770 per mg and that I would receive 49 mg. That’s nearly $87,000 worth of medicine alone. I signed a bunch of forms and that was it.
One more step. The doctor and the infusion company recommends that the PICC line dressing be changed a day after installation. The next day, another nurse came out and peeled off all the old bandages, and put on fresh ones. From there on out, we would be on our own.
Chapter 4: The treatment
I’ll present this chapter in brief diary form to show what occurred during treatment. You can see my activity level, pains, temperatures, etc.:
Friday, Dec. 28: Chemo started at 3:30 p.m. Temp. 98.1. No immediate effects. To protect the line from getting pulled, the nurse gave me a netting/stocking that goes over the arm. It’s a nice way to feel that everything is snug.
Saturday: Slight headache during the day. Infusion nurse came out to clean the dressing. I wish they’d create some kind of tape that wouldn’t pull your arm hairs out. My blood pressure is slightly elevated (140/62), and my pulse is definitely faster (76, when it usually tends to be in the 50s). Either I’m nervous about the nurse ripping the tape off my arm, or the chemo is racing me. Took 2 Tylenol (500 mg each) in the afternoon for headache.
Sunday: Temp. 97.1 at 9 a.m., 98 at 7 p.m. My right shoulder aches from 4 p.m. to 7 p.m.: a deep, strange ache. Took 1.5 hour walk. In the afternoon, I had the “Is this pump working?” discussion/argument with myself and my wife. It didn’t appear there was any fluid in the line; also, the small bag didn’t look smaller after three days. Did the infusion company screw up? The pump sounded normal. I finally called the infusion company to ask them. The guy on the phone assured me that if no fluid was getting through, the pump would beep. Recommendation for peace of mind: After the infusion company installs the line from the pump, confirm that there’s fluid in the line, and have them explain you can’t see it because it’s in minute amounts and it’s clear.
Monday: I had a huge “night sweat” in the early morning that lasted about two hours. Temp. 97.6 at 8:30 a.m.; 98.1 at 6 p.m. I felt like I have a slight flu during the day: my right shoulder aches and I have a dull headache. Appetite level is slightly down. Took an hour-long walk. Took 3 Tylenol at 2:30 p.m. for worsening headache. The toughest time yet that night: had bad chills and fever of 101 at 11 p.m.; got into bed with extra clothes, socks, hat, heating pad and tried to stabilize. Took 2 Tylenol. Took temp in half-hour increments. Fever lessened to 100.5, then to 99.8. Then sweat fitfully.
Tuesday: Temp 98.6 at 9 a.m., 98.8 at 6 p.m. Very weak today in the morning. Went to bed from 11 to 1. Took 2 Tylenol at 3:45 p.m. for headache. Walked around the neighborhood for half hour. Tough times again at night, just as previous night: Fever at 11 p.m. of 101.9. Took 2 Tylenol, fever subsided over an hour and a half to 99.8. However, I couldn’t shake the headache, which was intense. Walked around the house and sat in a chair most of the night; slept only two hours. This sucks, but Friday is just around the corner.
Wednesday: Temp 98.3 at 9 a.m. I called Dr. K to ask about the fevers, a cough I had, the headache, and whether I could take more Tylenol or something stronger. He said the fevers were to be expected and weren’t at the worrisome levels, which would be 102.5 and higher. To my disappointment, said I should stay with Tylenol, and only 2 per six hours. Slight headache still there most of the day. Took 2 Tylenol at 3:15 p.m. A nice period of calm, comfort through dinner and the evening. I really needed that.
Thursday: Temp 98.7 at 9 a.m.; 100.7 at 10 p.m. Amazing day: relatively pain-free all day. What’s going on? I haven’t felt this “good” (or un-bad) in a long time. Just a cough that comes and goes, though not tons of energy.
Friday: Temp 98.7 at 8:30 a.m. Only hitch: The pump battery went low in the middle of the night (2 a.m.), and it started to beep. Had to get up out of a nice sleep, get the manual, and change the battery. Easy task. The infusion company was scheduled to come at 3 p.m. and unhook me, but the pump started beeping at 1:50: “Reservoir supply is empty.” There’s no drug left. I called the company immediately, and the guy on the phone said he was worried that the line might clot up, and that I might have to inject saline and heparine into it. “I’ll find out and call you right back,” he said. He never did. The nurse showed up at 3 p.m. and said there was no problem. Jeez. Anyway, I got unhooked and then drove to the hospital, where a technician just pulled out the line, put a piece of cotton over the hole, and told me to press it for three minutes. I’m free. It’s time to start healing.
The next several days: Felt great during the days. Huge sweats at night. My first doctor’s appointment was Monday, three days after going off chemo. Dr. K said the cladrabine definitely did its work: I have virtually no white blood cells now in my body. I have to go back each week for more blood tests, and most importantly avoid infection: Stay away from coughing, sick people, wash my hands frequently, have everybody else in the family do the same. I asked about working out again to regain some muscle use. Dr. K replied, “Use common sense, and don’t go in any sweaty gyms.”
The idea is that when the white cells replenish over the next four to eight weeks, they’ll come in healthy and strong. I’m going to meet with a naturopath to discuss how to do this. No more HCL. No more sideaches. I’ll have “me” back.
Chapter 5: Am I healed?
Sorry, readers. I won’t know if I’m free from hairy cell leukemia for several more weeks, or even months. As I post this story, it’s the day of the first post-chemo blood test. You’ll have to tune back to this Web site for the conclusion.
Chapter 6: The angels
Am I blessed, or am I cursed? Am I lucky, or unfortunate? Should I be thankful, or angry? Should I feel relieved, or guilty?
On one hand, I have this rare blood disease, and remember, things like this just don’t happen to me or to anyone I know or have ever known. On the other, it’s treatable. One one hand, I have leukemia. On the other, it’s “the good kind.” On one hand, I’m confident that the chemo worked; on the other, there are hundreds of others out there who are told every day that they have to undergo months of chemotherapy and radiation, with no guarantees of success.
So how should I be?
The angels help me answer this. They’re the people -- dozens, maybe a hundred -- who inundated me with cards, e-mails, phone calls and prayers, all offering to do whatever was needed to help me get better. Drive me places. Donate blood if I needed it. Take care of things around home. Sit with me. Go for walks. Anything. These were family members and close friends, but they were also people who I didn’t necessarily know well, or at all. For example, my youngest son had a sleepover at a school friend’s home on the last day of my chemo. When they brought him home, they arrived at our doorstep with a large bag of food: a ready-to-go dinner, with salad and dessert and other goodies thrown in. It was overwhelming that while I was being introduced to this mother, she was handing us food.
One of the first angels was Fr. Phil, who works in the parish where my wife teaches. He sent me a copy of “It’s Not About the Bike: My Journey Back to Life” by Lance Armstrong. Fr. Phil wrote, “Dear Tim, My prayers are with you for healing …” This is incredible because Fr. Phil is fighting multiple myolema, and has plenty on his own hands. Yet he took the time to reach out to me.
The angels took various shapes. Some of them sent me cards, saying special people were praying for me. Many sent dinners. A close group of friends postponed our annual ski vacation together until the spring, when I would be better. They broke our rule that has stood for a dozen years: The trip is bigger than any individual. Some of the angels just said, “I love you.” Others did research. Not a day has gone by without several phone calls, letters and e-mails to ask how I’m doing.
My favorite ones were the classmates of my youngest son. They gave me an “angel-gram” of sorts -- a sketch of me, signed by each of them, with a promise to say a prayer for me each morning. They also sent me a Christmas e-mail, with wishes for health and happiness. They had all forwarded to each other, with each one signing this electronic card. These kids are 13 years old. Why would they care anything about some adult? Because they’re angels.
So I am blessed, lucky, thankful, relieved. I accumulated a huge debt of hugs and learned how to accept love, and how to return it. From all this support -- which I didn’t deserve and happened spontaneously -- I gained needed strength. The angels have affected my life much, much more than those rogue white cells ever will.
Chapter 7: My advice
Here are some miscellaneous things that might help you during chemotherapy:
1) Find out specifically what your doctor thinks is a warning level of fever, and how long it should last before you call him or her in the middle of the night.
2) Find out what pain relievers you can take, and the dosage.
3) Drink, drink, drink. Teas, juices, water. Use a variety to avoid getting sick of one beverage. I drank some canned nectars at the beginning, but I’m sick of them now. Remember to be aware of electrolyte replacement. Sports drinks like Gatorade are the most popular, but they can taste too sweet. Try some Pedialyte (an electrolyte liquid for babies) mixed in a juice.
4) For my walks, I put the CADD pump in a fanny pack turned around to the front. That saved my shoulder from getting tired or tense.
5) I finally felt comfortable with the PICC line wrapped in the sock-net, and then covered lightly with an ace bandage. I had a subliminal fear of ripping it out in the night. But the net made me feel secure.
6) My feet got cold a lot for some reason. The heating pad felt very relaxing.
7) Bathing seemed a lot easier than a shower. I just kept me arm out of the water.
8) The various aches, pains and headaches seem to come and go. When one of these pains would come, I’d find myself naturally thinking, “Oh no, I don’t think I can handle this for seven days. This is going to be bad.” But for me, the pain would mysteriously disappear. Until, of course, something new rolled down the pike. But be patient: You can work through it.
If you have any questions for me, you can contact me at TimHCL@hotmail.com. I’ll try to answer whatever I can. Best of luck in your journey to wellness.