Do you remember the parable about the blind men and the elephant? Each of a group of blind men feel a part of an elephant, and give a description of what the elephant is – all of them disagreeing – because they’ve only experienced a tiny part of the whole. That is how I feel about CLL. It is a huge entity, with so many parts I have no chance of conceiving of the whole thing. This story is my tiny part of the whole.
Frequently people are proud of traits that run through generations in their families. Beauty, brains, grit, compassion; the list goes on and on. I was recently surprised to find that what runs through our family was not a common thing, or even a thing of which to proud. Instead, it’s a dark thing that has been stealthy through the years, right there all the time, but still managing, somehow, to hide from view.
My dad’s mom, my Grandma Louise (or Grandma Punkin as we called her) died in 1974, when she was 72 and I was 11. Typical of a kid, I don’t remember much about her. We didn’t visit often, and when we did, our visits were short. Mom said we didn’t want to be in the way. Grandma was always so busy. She ran a boarding house in a small town. She had lots to do every day, with many people to care for. She was an excellent cook and baker, and worked endlessly every day in the kitchen. She was a wonderful, giving and loving woman. Sometime in the mid-1960s, Grandma started becoming ill. Eventually she was diagnosed with leukemia, and after a while, more specifically CLL. CLL is what finally killed her after an approximately 7 year battle.
When my dad was diagnosed with CLL, I was a senior in high school. I remember thinking what a lousy piece of luck for him to have contracted such a nasty disease. At the time, I didn’t think much of it. Dad was a big strong man, and surely wouldn’t be sick. We had a large family with seven children. We needed him. After his time in the military, Dad spent his entire adult life working for the railroad – 36 years. It was a good job, and he felt lucky to have it. It kept all of us fed and housed, a better life for his family than he had had as a child. Life was relatively good until 1980, when he started showing signs of illness. He was 57. Dad had these funny red spots all over him. Now I know those were petechiae, a sure sign of trouble. He also was bruising easily and just generally felt unwell. Tests confirmed a diagnosis of CLL. He was sick enough that he was hospitalized in a local facility. The petechiae were everywhere, inside and outside his body. The internal ones, especially the ones in his mouth and throat, kept rupturing and blood would flow from them. It was hard to watch, especially as a teenager. As a veteran, Dad was able to seek treatment from experts at the VA Hospital. It was there that he met Hematologist Dr. Neil Kay. Dad’s disease progressed. He was treated with chemotherapy and steroids on a regular basis. He was hospitalized at the VA hospital often, frequently with fevers and critically low blood numbers. We got used to the repetitiveness of it – they’d always fix him and send him home. It seemed like it worked well, and that it would go on forever. In late 1989 they removed Dad’s spleen. It was dangerously enlarged, and his doctors felt that its removal could make him healthier. It was hard on Dad, but he endured.
In the spring of 1990, Dad was especially ill. Dr. Kay explained that an experimental medicine had become available at MD Anderson Cancer Center in Houston, Texas. Dad would have to go there if he wanted to participate. He did. He wanted to fight as hard as he could, so Dr. Kay made arrangements to send him there. Neither of my parents were big travelers. I couldn’t let them go alone, so I made arrangements to go with them to Houston. It was a good thing I did. After the first night, Dad was admitted to the hospital, where he stayed throughout our visit to Houston. I was glad I was there for my mom, who I later learned was just starting to suffer from Alzheimer’s. By the time we got to go home about a week later, Dad was very sick and very weak. We journeyed home with wheelchairs and taxis back to the VA Hospital. Dad spent the better part of the first six months of 1990 in the hospital. He was very sick. And very thin. Dad died from his CLL in July of 1990, one day before his 67th birthday. He fought CLL for 10 years.
All through his fight Dad wanted to help with study against this disease. He donated his blood and tissue for study whenever he could. He even made sure that after his death his body was donated for medical science. His family was given a mere shell of him to bury. I’ve always been proud of his fight and his contributions to research. He loved people, and always wanted to help. I convinced myself that medical science would surely make great strides to stop this disease, and I didn’t give it another thought.
An annual visit to my GPs office this spring started my CLL ball rolling. Blood tests proved abnormal, so more blood tests were ordered. In early April I, too, was diagnosed with CLL. The funny thing is that CLL wasn’t even on my radar. Even though my dad and grandmother had had the disease, I never worried that I would have it. We had always been told that CLL doesn’t run in families. It was just very bad luck on my family’s part that two of them (that we know) had died from the disease. It was very uncommon and unexpected. Alzheimer’s was always on my radar. It’s what I feared. But surprise, surprise – at 53 years old I have CLL.
After diagnosis I read anything and everything I could find online. Reading it and understanding it are two completely different things, but I wanted to learn as much as I could possibly absorb about CLL to be prepared for my fight. The CLL Society website was a godsend. I was so happy to find so much accurate information available in one spot. My gratitude turned to amazement when I saw Dr. Neil Kay on the site doing interviews related to CLL. Wow! Twenty-seven years later he was still fighting CLL! I decided I’d have to get in touch. Because of the affiliation with my Dad, I’m pleased to say that Dr. Kay is now my CLL doctor too. I am beyond thrilled and grateful to have him in my corner. I’m especially grateful after having a short experience with a local hematologist/oncologist. During my short time with the local doctor, I was told that my cancer was puny, and that I should go home and enjoy the summer. Even though I understand he was taking care of people significantly sicker than I, it was not particularly confidence building, especially considering that I’d already lost two people I loved to this disease.
Another surprise came when making calls to my siblings to tell them of my diagnosis. My 57 year old brother was slow to admit it, but he too had been diagnosed with CLL the previous month. He had planned to keep it a secret with his wife until some later date. Once he heard my diagnosis, he told me about his. We are both classified as Stage 0 on the Rai scale. Even though I have enlarged lymph nodes obvious on CT scan, I’m told only palpable nodes count. My analysis shows two 13q14 abnormalities, one at 22%, and one at 11% on FISH panel study. Cytogenetic chromosome analysis showed 4 of 20 metaphases had 13q deletion, and an unbalanced 13q arrangement. 2010 findings in an article I’ve recently read imply that familial CLL may have an earlier age at diagnosis than other CLLs. It also may affect a higher number of females compared to other varieties. Both of these seem to be the case for our family. The good news, if you can look at it that way, is that familial CLL seems to consistently take an indolent course. Hopefully that will give researchers time to fix this before it’s too late for my brother and me. I can’t be sure of how many other relatives of ours have had CLL in the past. We were never close to Dad’s family, so I don’t know much about them or their health history. It makes one wonder how many others have had this familial form of CLL through the ages.
On the surface it seems unrelated, but my sister Sally died in January of 2016. Sally was 68 years old. She was never diagnosed with CLL, or any kind of cancer. It’s only in retrospect that things seem a little clearer. Sally was frequently sick with sinus- or lung-related ailments. She coughed and sneezed her way through many years of her life. In early January 2016, Sally developed a boil on the upper part of her leg. She went to see her GP for treatment, and was given antibiotics. A couple of days later, her pain was significantly worse, so she visited the local ER. More antibiotics were prescribed, and she was sent home. Two days later, Sally was found essentially unconscious in her bedroom, her body septic from infection. Major emergency surgery was performed to stop the “flesh eating staph” bacteria that was moving rapidly down her leg. All of the skin and fat tissue were removed from her hip to her ankle. During her crisis, Sally appeared to have suffered significant brain injury caused by what was believed to be strokes. Sally died two weeks later from the damage her body had sustained. I’ve since learned that CLL can be present in people, and remain hidden and dormant. I will always wonder if Sally’s immune system was compromised by CLL, making her more susceptible to infection. We will never know. No autopsy was performed, and she was cremated. It just seems curiously coincidental that she had a compromised immune system, and two siblings with soon to be diagnosed CLL.
Like my dad, through my journey with CLL, I will do what I can to assist medical research. I sincerely hope these efforts will prove fruitful in the hunt for a cure. I remember announcing in grade school that I wouldn’t have children of my own. For me, this turned out to be a fortuitous decision and a wise choice. I won’t be responsible for passing CLL to another generation. That makes me happy.
A lifelong lover of nature and animals, Janet lives in very rural northwest Wisconsin with her husband, three dogs and three horses.
Originally published in The CLL Tribune Q4 2017.