Founder Joyce Niblack

Joyce’s Story

My name is Joyce Niblack. In 1988, at age 49, I was diagnosed with essential thrombocythemia after suffering a mild stroke that left me with difficulty speaking for about a year, followed by a thrombophlebitis in one calf. My platelet count at time of diagnosis was 980,000 (WBC was also elevated at 27,000) and both spleen and liver were significantly enlarged.

The period leading up to diagnosis was frustrating. For about a year, I had suffered from repeated bronchial infections. My white count kept climbing higher and higher despite repeated rounds of antibiotics. I was tired and had a host of little niggling complaints (tingling, transient numbness here and there but mostly in toes and fingertips, burning, etc.) I also was experiencing what my doctor referred to as “silent migraines” because I never experienced the severe headaches that go along with classic migraines. But I did have visual disturbances-light auras with temporary loss of vision for about 20 minutes as the auras filled up my sight and these were usually accompanied by some nausea. I also was experiencing intense pain if I twisted the wrong way. Just simply putting on a seat belt brought me to tears. I later learned my spleen and liver were enlarged from my condition. Throughout this period, my internist kept brushing off my complaints saying “it doesn’t mean a thing, you’re just heading into menopause”. I can’t believe now I was so stupid as to believe that. Rather I think I was suffering from “I really don’t want to know” syndrome.

Once I snapped out of my “ostrich with head in the sand” period and got insistent about getting to the bottom of this, I was first sent to an infectious disease specialist. He looked at my blood counts, poked around on my tummy, told me my spleen and liver were enlarged, called a hematologist and when he got off the phone pronounced, you have polycythemia vera, you have around 5 years to live, try to enjoy them. (If you have PV, relax, those statistics are now known to be untrue-you can live out a normal life span if you are properly monitored and treated). I was in shock! He then sent me off for a red cell mass test to confirm his diagnosis. It was normal so I was back to square one. However, looking back, there was a period in my late thirties and into my forties where my allergist would remark that my hematocrit and hemoglobin were high but he didn’t think it meant anything. More about that later.

I dragged along for a few more months until my doctor decided that I should go on estrogen replacement therapy and maybe that would take care of all of these crazy symptoms and my fatigue. Within 6 weeks, the frequency of the “silent migraines” had increased, then I had a blinding headache that lasted several hours and left me with mild speech aphasia, and a few days later I had a badly swollen leg from a blood clot. I changed doctors and was immediately referred to a hematologist at what was one of the strangest periods of my life.

The day I received the phone call from my new internist that my platelet count was very high and I needed to be worked up by a hematologist, I also got a call that my mother had a stroke and was in intensive care, please come home. My father was 82 at the time and was recovering from major surgery. So we traveled to Ohio and my parent’s internist put me in touch with a local hem-onc who performed a bone marrow biopsy at the hospital where Mom was admitted and then sent me off for another red cell mass study and liver and spleen scans. I got the news of my diagnosis and was started on Hydrea on the same day we also got the news that my mother had not had a stroke after all, she had lung cancer that had spread to the brain and adrenals before diagnosis. I still had the bag from the drugstore with my first supply of Hydrea and my parents’ internist was harping on me to sign a do not resuscitate order for my mother. I remember feeling like my head was numb and it took a very long walk with my very supportive husband for it to thaw out.

There followed a difficult three month period where my husband and I (we are attorneys) took the files we thought we’d need, loaded a computer and printer and drove back to Ohio where we stayed with my parents through my mother’s final months. We set up a home hospice program which was of enormous help and I did a lot of reading on relaxation, meditation, mind-body connection to getting well again. I also took many long walks and did a lot of talking to myself. By the time my mother died, I was so grateful my diagnosis was “just essential thrombocythemia” and not cancer that I was able to adjust fairly rapidly. I really hadn’t had time to focus on my condition for the first three months. I remember my principal concern was whether I would be around long enough to take care of my parents.

After Mom died, we brought my father back with us to Illinois, I started researching my condition and the Myeloproliferative Neoplasm Disorders in general. I read everything I could lay my hands on and gave myself a few good cases of the quivering quakes because the literature at that time was still talking about shortened life expectancies. One of my uncles is a psychiatrist in New York City and he kept insisting that I come to New York for a consultation with Dr. Harriet Gilbert. His wife’s brother’s wife was/is a patient of Dr. Gilbert and was being treated with interferon for polycythemia vera. I discussed this with my hem-onc, he felt it was a good idea. There were clinical trials in progress testing interferon for Myeloproliferative Neoplasm Disorders and I was enrolled in the ECOG (Eastern Cooperative Oncology Group) trial which called for 3MU daily.

Gilbert felt this was an inadequate dose but she will not interfere with a patient’s physician’s advice. I should mention that the Hydrea had been discontinued after 6 months because my marrow became severely depressed and took 6 months to recover). I was started on interferon the first time in mid-June 1989. After I got past the initial flu like symptoms, I was still feeling lousy. It turned out that for me, combining maximum doses of Tylenol with interferon and a few drinks thrown in wasn’t a good idea. My liver enzymes were elevated substantially. I was taken off the interferon, stopped Tylenol and liver enzymes were normal within a week. I was restarted on interferon with the admonition no drinking, no Tylenol. That solved the problem. About 4 months into interferon therapy, I traveled to NYC for my first consult with Dr. Gilbert. I shouldn’t embarrass her by sharing this with you but this was a wild and crazy day. She had fallen and had both arms in a cast from her knuckles to her elbows. The electricity went out in her building about 30 minutes after we arrived and we kept seeing a flashlight bobbing around the office. The wait was long but this was my first experience with the sharing that went on in her waiting room and it was marvelous. We all started talking and my husband and I learned more about MPN’s, treatment options, etc. than we had since I was diagnosed.

I also must say that talking with her and getting my questions answered (first one naturally was can I live out a normal life span-her answer yes you can) did more for helping me to restore my mental balance about living with this chronic condition than anything else I had done. She again expressed the view that 3MU daily was an inadequate dose and for me she was absolutely right. About 9 months into the clinical trial, a required bone marrow biopsy revealed development of early stage myelofibrosis. I dropped out of the clinical trial immediately after a phone consultation with Dr. Gilbert, my doctor increased my dosage to her recommended 5MU daily, I started feeling very well, my hair, which had thinned substantially on the lower dose grew back in thicker and curlier than it had been (I looked like little orphan Annie for a while), the myelofibrosis reversed and my marrow came darn close to returning to normal, my liver and spleen shrank and I was free from pain when I twisted or turned. I started out with an M/E ratio of 7:1. After 4 months on 5MU daily, it was normal. I started out with cellularity to fat ratio of 95/5. After 4 months on 5MU daily it was 60/40. I remained on interferon for a total of 2-1/2 years. Neuropathy was a limiting factor in continuing treatment with that drug.

My counts remained normal for 3 years without any further treatment. Then platelets started to rise and I suffered a transient vision loss in one eye following a week of silent migraines. We had moved to Scottsdale, Arizona the previous year and I was referred to a marvelous hem-onc at Mayo Clinic (John Camoriano). Even after 3 years, my spleen and liver were still normal, my bone marrow was not completely normal but my new hem-onc said if he hadn’t reviewed my medical history, he wouldn’t necessarily have diagnosed a myeloproliferative disorder. Since my only problem at the time was a platelet count of 540,000, I was placed on anagrelide therapy. A dose of 0.5 mg three times a day held my platelets in check at 350,000 for around 14 months. Then all counts started edging up a bit and I started feeling pressure in the area of the spleen. Still, counts were within normal range. A few months later, both hematocrit and hemoglobin were at the upper end of normal and Dr. Camoriano started talking about a possible conversion to polycythemia vera. I was having headaches, didn’t feel well and the pressure in the spleen area kept increasing. So in May of 1996, I had another bone marrow biopsy and spleen scan and learned that I once again had developed early stage myelofibrosis, my spleen was indeed enlarged, and I probably now have polycythemia vera rather than essential thrombocytosis (this based on borderline high Hct and Hg, absence of iron in the marrow and erythropoietin level below 4).

I was started back on interferon on May 22, 1996. Initial dose was 4MU daily which was increased to 6MU daily after 3 weeks. My liver functions remained normal this time and as with the first time I was on interferon, my blood counts were well controlled in the lower range of normal values. A bone marrow biopsy and spleen tomogram taken in November, 1996 showed no change. This was not surprising. My experience has been interferon works fairly quickly in normalizing peripheral blood counts but takes a longer time, at least for me, to affect marrow status and spleen size. My doctor decided to increase me to 8MU daily. I tolerated that dose for several months and then begain getting weird periodic episodes which I’m told are similar to panic attacks. When one of these occured while I was waiting to have blood drawn and a spleen tomogram before my next bone marrow biopsy, I reported this to a nurse, she called my doctor and I was whisked off for an electrocardiogram, arterial blood gases, chest x-ray and when those and blood worked proved normal, on to the spleen tomogram and BMB. While uncomfortable, the higher IFN dose did start shrinking spleen size and improving marrow status. However, my doctor lowered the dose to 6MU again remarking that I would probably have to remain on interferon longer at the lower dose.

My next full exam was scheduled for early summer of 1998. My blood counts and blood chemistry throughout this period remained rock steady. Due to my schedule, the next bone marrow biopsy was postponed until July, 1998. You cannot imagine my joy when my doctor informed me that the fibrosis had reversed. He took me down to the pathology department and reviewed my slides with me under a double-headed microscope with a pointer so he could pinpoint what he was explaining. He then showed me my pre-interferon slides for comparison. I was thrilled at the difference. Not only had the fibrosis vanished, there were fewer platelet precursors (megakaryocytes) and those present were not in large clumps as they had been in 1996. Cellularity was more normal. Fat cells were more abundant. I was a very happy camper as was my doctor. I was a bit disappointed however in spleen response. My spleen was 26 cm in length when I started back on interferon. It is presently around 18 cm. While this is a very decent decrease, I want normal! (10-12 cm.) My doctor pointed out however that I was looking at this as a one-dimensional organ and and in fact, in volume it had decreased by at least 30%. He told me to picture a watermelon and then picture it shrinking in overall volume by 1/3. Now my spleen was not watermelon size but that’s a good analogy.

When we discussed further treatment, my doctor said wanted to back me off interferon slowly over a period of about 1 year, watching carefully to make sure I remained stable. He likened maintenance to lion taming. He said once you think you have the lion tamed, if you turn your back and start running out of the cage, the lion will attack. But if you continue to face the lion and back away slowly, its more likely the lion will stay put.

In late July, he had me cut back from 5MU daily to 5MU 5 days a week with weekends off. Blood counts and spleen size remained stable. In October, he instructed me to take 7MU 3 days a week. That was a problem. I developed increasing signs of neuropathy. We went back to the 5MU dose but the neuropathy worsed and at the point where the bottoms of my feet felt like they had a thick layer of cotton on them which reduced any sense of where I was putting my feet, I noticed clumsiness (mild “stone fingers”) when I tried to type and other signs that could not be ignored, a decision was made to halt the interferon until these symptoms resolved. That was on December 14, 1998.

My platelet counts had been holding steady at around 220,000 throughout my time on interferon. They rose to 322,000 about 3 weeks after stopping and I was a bit upset when I had an episode of tingling that left me with left-sided weakness for a few days. I went though an MRI, echocardiogram, ekg, platelet aggregation tests and then was referred to a neurologist. All test were normal and the neurologist felt I was experiencing “migraine equivalents”. Aspirin was increased from a baby aspirin to a 325 mg aspirin daily and so far no further problems. My spleen has not changed so that was good news.

Counts on January 21, 1999 were more encouraging with platelets at 266,000 and all else also within normal ranges. However, I got a bit of unwelcome news on Febrary 12. Platelets weren’t the problem-my hematocrit had shot up to nearly 48 and I needed my first phlebotomy. This was scheduled the following morning in the chemo unit of the new Mayo Phoenix hospital. The nurse gave my husband and me a tour of the transplant rooms while we were waiting for a CBC following the phlebotomy. Impressive. There are two recliner chairs in each room that fold out into single beds for family members, a column with all manner of equipment which makes each room a self-contained ICU unit if needed, and a treadmill. The nurse said they find that patients who use the treadmill recover strength and suffer less fatigue both short and long term. I had to return the next day for a second phlebotomy-great way to spend my valentine husband’s birthday but we got that out of the way early am, it brought my counts down to safe levels and we were on our way to celebrate with a lovely dinner and then a week in Palm Springs.

I started treatment with Hydrea when platelets hit 400,000, scheduled phlebotomies as needed and restarted interferon after a six month break from interferon. In the summer of 1999, samples of my blood were sent to Dr. Joe Prchal at University of Alabama at Birmingham. Dr. Prchal is doing clonality studies and is hot on the trail of identifying the PV gene. He is studying families where multiple members have PV. It turns out that interferon therapy has allowed the return of normal stem cells in my marrow. This was quite an exciting find and is reported in the American Society of Hematology (ASH) 1999 Abstract # 4514. I still have a small percentage of PV clones but in view of a finding of polyclonality, my Dr. has recommended that we collect and store my stem cells as insurance for the day when I may no longer respond to or tolerate intereferon. My insurance company has refused coverage and I have just filed an appeal. I remain on interferon at 5MU 5xweek. One final note. My father also had essential thrombocytosis. He died on August 9, 1999 at the age of 92.

MPD Voice Newsletter

Dr. Harriet Gilbert, MPN Research Center New York, first published the MPD Voice newsletter with Joyce as the editor, and Joyce continued the publications for many years after Dr. Gilbert died.

After Joyce died and the publications ceased, Dr. Claire Harrison an MPN expert in the UK, approached the MPN Education Foundation and asked if she could use the name for her publication. It was agreed that keeping the name of the newsletter alive was a tribute to both Dr. Gilbert and Joyce.