Iron Jak: Bad Blood x 2

Miracles Take Time

2011-01-24T22:42:00.000-07:00

Blood counts were not changed from last time - possibly the level of Rat Poison that I'm taking only reduces the load to a certain point. The counts were flat since the last month. The Huntsman people are writing a change proposal to double the dose again - possibly in 2-3 weeks.

Christmas Miracle at the Huntsman?

2010-12-27T22:00:00.004-07:00

Hurrah! 53 days after doubling the dose of the Phase I test chemo, my blood counts have come down! Because the chemo doesn't "kill" blood cells, it takes at least the "life-span" of a stem cell to see a decrease in the resulting blood counts.

Although the decreases were not large (8% on platelets, 6% HCT), it was the first platelet decrease below 600 since starting the new chemo! Although there can be other explanations for the decrease other than the chemo, I'll choose the optimistic approach: IT'S WORKING! Recent bone marrow biopsies should indicate any change in reduction in my JAK2 mutant allele burden.

I'm so grateful to the corporate sponsor, the many researchers, doctors, nurses, phlebotomy professionals (and amateurs!) and friendly Huntsman Cancer Institute staff for all of their assistance. From the front door to the second floor and into Clinic "A", I find friends of 5 years, helpful professionals and intelligent scientists who help me understand the nature of the mutant enemy! Thank you to all.

Good times at the Huntsman: 11/29/2010

2010-12-01T14:47:00.002-07:00

Today was a super day at the Huntsman. At the end of a major cycle, the sponsor, needs to know what effect the drug is having on my body. So, when I say, it was A super day, I really mean that they did lots of tests-for over four hours.

Bone Marrow Biopsy That which I feel may not be exactly what happens, but after numbing my hip in the middle of my back with a great deal of novocaine, the doctor inserts a large needle into the bone and breaks through the bone to get inside the hipbone. She then pulls out this including the bone marrow from inside the hip. Today, she did that twice and then using something like a hollow drill, she corkscrewed into the hip and extracted a bone sample. I confess, I yelled pretty loud, and it hurt like - - !

The biopsy site will be painful for 24 to 48 hours and makes sleeping with my ankle in a cast more difficult. The biopsy will be used to determine the proportion of JAK2 to regular stem cells and will indicate whether the rat poison is functioning as intended.

Blood Tests Normally, they do a single needle into the major vein in your elbow, and from that needle they extract 5 to 11 vials of blood. Today, they only extracted five vials of blood. I told them that I was not loved very much if there were only five vials. Later, after the bone marrow biopsy, they came in for 11 more files. So, I must still be loved by the testers a great deal.

As expected, there were not many changes in my blood counts: my platelets were about the same and my red and white blood counts were normal. I expect to see lower blood counts at my next appointment, December 29, 2010, because of the increased dosage of rat poison.

EKG and Vital Signs I'm pretty normal: 97.6 degrees, 116/62 (a little low), and down about 10 pounds after my surgery.

There is always great food at the Huntsman. The Point restaurant and deli are subsidized by the Huntsman with food provided by Gastronomy restaurants. Today, however, I have been nauseated and was restricted to Sprite, soda crackers and a roll with butter.

A FRIEND AT THE HUNTSMAN “Pat” is a 72-year-old woman on the same study protocol as myself. She is slight of build and very friendly. One day In Clinic A, I noticed that she carried the same red protocol book as me and I introduced myself to her. She has a similar condition to mine (myleoproliferative, related to Leukemia) but it is not polycythemia vera. Her disease seems to be more advanced than mine, and she requires 2 to 3 units of blood every two weeks. She is also hoping to increase her dosage of rat poison. We talked today, and occasionally on the telephone. She has good friends and family who drive her to the Huntsman. Like anyone on a study drug, I worry for her.

Up and DOWN and UP...

2010-11-04T10:21:00.004-06:00

Although I have been on new medication since June, the doctor says that I am under-medicated which explains the constantly increasing blood counts. They have been going UP approaching levels which could be dangerous for stroke or heart attack. I've been a little DOWN Today I got an e-mail from the study coordinator, which said:

"your case was discussed during our 11/3 teleconference with the Sponsor and they would like to have you initiate a dose increase to 60mg once a day to see if we can't better control your counts. Please start tomorrow morning with taking 6 pills in the morning. I will get you additional pills to hold you over until we see you again."

So, now my dosage is UP and we'll expect to see the blood counts come DOWN in about a month.

11/7/2010 The increased dosage has caused a few side effects: nausea, dizziness and fatigue. Nothing new! It will get better.

11/16/2010 The fatigue lasts 4 to 5 hoursand takes a large chunk out of my productive day several weeks ago, I had requested permission to take the pills at night. I believe that by taking the medication at night that he will happen during my sleep and pass unnoticed leaving me in better physical condition during the day. Today, I got an exciting e-mail:

"I apologize for the delay in getting back with you on this. I have spoken to the Sponsor and they will allow you to take the pills at night, just as long as you have food approximately 20 minutes prior to taking the medication. Can you tell me how many pills you have left? I need to get you additional pills as we have increased the dose."

11/20/2010 I'm sleeping well and rising with more energy, no nausea, and not as much dizziness.

NEW HOPE!

2010-06-15T16:26:00.002-06:00

During this clinic appointment, I was very excited about being in a Phase 2 PV study for an Interferon 2 Alpha (IF2A) study that had shown great promise. I had studied all of the preceding publications with the IF2A and had convinced myself that this was the "promised land" and that I finally had the hope of being cured from PV.

During the appointment, Dr. Prchal told me that because of my other genetic issues, I would not be accepted as a candidate for this study. I was devastated! However, Dr. Prchal told me about a Phase 1 study (first human consumption) with Eli Lily. The drug has shown promise with mice and dogs - and now needs human rats (or guinea pigs). I will go through the screening in late June and start the study in July.

Same old stuff...

2010-03-01T18:20:00.002-07:00

During the past 4 years, I have been taking Hydroxyurea as a chemo for my PV. There are few side effects known and it manages to keep my blood counts quite normal - platelets between 200 and 300 and RBC and WBC in the low-to-normal range. Monthly, I go to Clinic A and have my blood drawn and note any changes. There have been no other alternatives to this point, but the literature indicates growing interest in Interferon and other approaches to controlling PV.

Discouraged, encouraged...the HHC swings.

2009-03-22T21:46:00.003-06:00

Iron Jak finally updates the blog. After a rocky emotional start on this journey, the results have been felt and the mood is decidedly UP!

Ankle: after being diagnosed with bone spurs and serious arthritis damage to my right ankle, it was surgically repaired on October 16, 2008. Now, it's March 22, 2009 and I'm limping but still much better than before. I fit in my ski boot without pain, I have a normal walking schedule and if I over-do the exercise, I limp for a day. Gradually, it has improved - and faster than many ankle surgeries.

Hemochromatosis: after removing more than 5 gallons of my blood, my ferritin level has come down from almost 3,000 to 208 (in mid-February) New orders were written last week and I'm now on a once/month schedule of phlebotomies for 6 months. According to the general medical consensus, I'm "out of the woods" for HHC. BUT - just because the blood level is down, at 208, it's not normal (25-35) quite yet. The concern I have is for the organs that were damaged prior to any therapy. Only time will tell how the liver and kidneys function. The knuckle damage will never be undone.

So - dear HHC friends, the HHC front continues to improve and twice weekly phlebotomies weren't all that bad. They were helping keep my weight in check - and since turning off the blood spigot, I've gained some weight.

Take heart - that dark cloud has turned into rain and the skies in the HHC direction are turning brighter shades of blue.

A good question from David...

2008-11-27T18:37:00.002-07:00

I have a question for you: if you are worried about driving your HCT too low, why are you on hydroxyurea?

Because I have two abnormalities - both related to blood counts and chemistry, the two principal researchers on each condition (PV and Hemochromatosis) have me on 5 separate studies - and trying to save my health in related ways.

My PV doc and his study protocols want to retain the Hydroxyurea to target the Jak-2 effects, and the twice weekly phlebs are really designed to eliminate the iron overload in blood and organs and reduce the amount of damage being done to joints (iron-induced arthritis) and organs - we want to keep the HCT as high as possible so that the iron reduction is maximized with each phleb. With that thought, we then reduce (but don't eliminate) the amount of HU so that the HCT doesn't go below a threshold and cause a temporary hiatus on iron reduction.

Good news all over - my iron (ferritin levels) have gone from ~3000 in July '08 to only 830 last week! So - another 2 months (8-10 phlebs) and we will then reduce to a maintenance level. At that time, we'll probably increase the HU to manage the WBC and platelets to acceptable levels.

Other good news - the Huntsman Cancer Institute (University of Utah affiliate where I am treated) appears to be the first U.S. location for a "new" Interferon study, similar to one just successfully completed in France. If all goes well, I will be on IFN in February - possible breakthrough protocol for PV patients.

Long-time no write...

2008-11-23T09:19:00.002-07:00

Many things have happened since last posting to my blog. Let's play catch-up. First the current news and you can skip the rest of the travelogue if you wish.

Hemochromatosis: After 3 more months of phlebotomies, my ferritin level is down to an astounding 990. Orders have been written for 1X week until 2/18/09. I'm tolerating the phlebotomies quite well - tired for 2-3 days afterward following a normal day of work, but not out of service. Everything else is normal. The graph is quite impressive.

Polycythemia Vera: No changes for now.

What a great weekend!

2008-08-24T16:16:00.003-06:00

Since going for a week without a phlebotomy, I've had a surge in energy. Yesterday, I installed a back porch door - latch, strike, closing mechanism, glass, etc. Then, I totally re-plumbed the upstairs bathroom shower with a line straight to the basement source. Now, it has a great stream of water (up from the piddle) and is not affected by flushing, running taps, etc. Fixed the motorcycle and went for an evening ride.

WOWSERS! It felt so good to be more "normal". Tomorrow is another phlebotomy and a trip to the Cancer Institute for my regular monitoring of the Polycythemia Vera. The PV has been lurking in the background, with hydroxyurea (oral chemotherapy) everyday. Tomorrow's tests will determine the effects of the combination of chemical and phlebotomy treatments. the platelets, RBC and WBC counts should all be normal or below, and the HCT count should be headed back to normal.

I'm appreciating all of the new friends and medical associates who I've met because of this bump-in-the-road. I'm also gaining a sense of oneness with others who are afflicted with more visible and/or more short-term mortality factors. I have great feelings of caring and compassion wash over me at times - seeing a child with cancer or an elderly person struggling to "check out". I have dear friends with grave afflictions who serve as positive role models, never complaining but facing the day's challenges with optimism. To all of them, I say, "thank you for being a dear friend".

Another day - another change.

2008-08-19T22:45:00.002-06:00

As of now, my ferritin (iron,easurement) is down to 1832 from 2942 on the ferritin. With that decline, Doc K. has taken me back to a phlebotomy once a week. I don't know whether to cheer or not - I'd rather get the iron out, but could see the toll it was starting to take on m'body.... So, now, every Friday is now a holiday!

This week: Billings, MT

2008-08-11T23:15:00.002-06:00

A great ending to the week - coming from meetings in Billings, MT back for a Friday phlebotomy. At this time, I'm used to the tiredness that is always present. Keeping the longer goal in mind - of reduced iron and organ damage, makes it easy to cope with the physical tiredness.

Another contributor to the fatigue may be lower sugar levels. I've earnestly started losing 20-30 pounds and I'm making a little headway - down from 209 to 203 in 8 days. The lower weight is important to lower the risk of diabetes from an iron-damaged pancreas. Good attitude here.

Equilibrium - and - Synchronicity?

2008-08-11T21:07:00.006-06:00

Great news: Celebrating life's little Gifts!

My computed model of HCT (hematocrit) suggested that I could be well below 32 today - the temporary cutoff for phlebotomy. This would NOT be good news, because continuous IRON removal is key to eventual reduction of ongoing organ damage. But the 5th floor Angels delivered the good news: HCT was 37.2 (!) Not just a short delay, but a substantial comeback from the declining trend. Soooo... this old body continues to have surprises - and gives me some feeling that just perhaps, maybe, we may be able to make progress against the "iron devils". What is, is. But, sometimes, "what is" isn't what it seems.

Just a reminder to myself: there is no change in the Polycythemia Vera component of the blog. The PV moves ahead, continuously, with the work of unconstrained cloning of stem cells within the bone marrow, and the chemotherapy and phlebotomies simply keep the blood counts within normal ranges.

If you have questions or comments that you would not like posted, my email is iron_jak@rocketmail.com. I'd love to communicate with you - sometimes we can learn the reasons that paths cross.

A very long trip - and success in W. Yellowstone

2008-08-09T23:26:00.009-06:00

It's a pleasure to announce that thanks to Darla (FNP) and Ashley (MA), there was success in the Yellowstone UrgentCare facility! Just arrived back from West Yellowstone (it's after midnight) and tired from driving. It's pretty amazing that my recovery times seem to be improving. Today is the 2nd day after phlebotomy - and although there was some dizziness and weakness in the morning, I was then able to drive 13 hours (including stops), concluding a 1400 mile trip since Tuesday morning. So much for being tired?

Great People in the Big Sky

2008-08-07T22:54:00.004-06:00

I'm in Big Sky country - West Yellowstone, Montana for a conference. Meeting so many charming and well accomplished people from many walks of life - corporate presidents, trucking company presidents and owners, an accomplished eye surgeon and his lovely wife, and a world-famous writer of American novels, Stanley Gordon West (who gave me a signed copy of one of his books, Blind Your Ponies).

Tomorrow is V-day (vampire day?) with my phlebotomy kit - in search of an accomplished nurse practitioner to remove the Friday pint without leaving tracks.

I want to continue to document parts of a life that say to others with PV or HHC, "It's OK - have a great life - and keep taking chemo and having phlebotomies and continue to enjoy all of the beauties of the world.". Yellowstone is beautiful - and the pizza was great.

There is an afterlife...

2008-08-05T07:08:00.005-06:00

After yesterday's phlebotomy, I worked a normal but sedate work day. Today, I'm driving to West Yellowstone for a conference, but with a phlebotomy kit in the car. A nurse practitioner in Montana is willing to drain this vein. (Not funny, I know!)

I'm getting familiar with feelings of tiredness, and they are not so debilitating now. I'm having a hard time with weight gain - eating balanced but smaller meals and still fighting for every pound. The reduction in physical activity probably accounts for lower metabolism and the "proud porker" feeling.

I look forward to going to the CRC (in the hospital) because of the nursing staff. They are so friendly and each one so good to work around - it's a pleasure to get stuck in the arm. I can't think of a group that is nicer - on a par with their fellow Huntsman clinicians and doctors. I think that their attractiveness as human beings might disguise their excellent medical skills.

I've added some charting (I must be a little OCD) to help me anticipate interruptions to my iron-shedding activities. The CHART (on the side panel) allows me to keep up with the response to cycles of chemo, phlebotomy and regeneration.

Another week, another pint.

2008-07-29T22:17:00.006-06:00

Better days. Not quite so tired - but getting a good night's sleep each night and eating well - trying to lose 20-30 pounds suggested by the doctor to reduce the likelihood of diabetes. (Currently at 204 pounds!) This week, it was a good poke in the arm - hit the vein the very first time. There is already scar tissue forming inside the elbow of my "big vein" arm. It will get worse, I'm told.

The secret... In personal discussions, I'm starting to describe the symptoms rather than the complaints or feelings. With this approach, I can still be my happy, upbeat self - rather than whining, complaining or shaking my fist at the heavens.

What is, IS. This seems to embody my approach lately. No changing reality, but my hope for the future is in my ability to accept "what is" for what it is, namely, another phase of life to be experienced and celebrated. And, with that attitude, I can move on to helping others, providing for temporal needs and working on talents and introspective development. I'm loving life more, but needing less to cling to the idea of longevity than the need to improve the value of each day. Blah, Blah, Blah...

Getting on - with a surprise or two.

2008-07-20T21:40:00.004-06:00

The arms are getting scarred a bit, the staff is as friendly as ever. But since the testing, liver biopsy, and almost 4 units of blood, my ferritin level JUMPED up to 2990. Waiting to hear a theory on why that may have happened. Traveling to Alabama this week and looking for anyone with a needle and a tin can. There is a center for iron studies - HHC - 12 miles from my hotel. We'll beg 'em to take the blood.

TIRED - so tired on the 2nd or 3rd days that getting off the floor is difficult at times. I left off the chemo treatment the night before the last phlebotomy and felt better this time than the previous ones.

PAIN - Friday, 7-18-08, my son and his wife had a new girl - my 4th grandchild, Anna, a beautiful child. My joints were so sore, I could barely walk the hospital halls. No complaints - just slow - and the granddaughter made it ALL WORTH WHILE!

The aftermath... 3 days later.

2008-07-12T23:48:00.002-06:00

Extreme tiredness, internal feelings of "almost pain", Restless Leg Syndrome; After Thursday, Friday and half of Saturday, the fatigue which had kept me from any activity lessoned. Now, Saturday night, the RLS is keeping me from sleeping. Luckily, less than 18 months left of this excitement!

And the results: TAH DAH!

2008-07-10T14:18:00.008-06:00

GREAT NEWS!!!
The good doctor said that he fully expected to find cirrhosis of the Liver or at lease fibrosis and scarring. BUT! He explained that for some reason, some mice NEVER develop the ugly things, no matter how much iron is put into their system. As of today, I have NONE of the uglies! Conclusion: I must be a mouse (or a Caucasian RAT!)

Any damage to my pancreas is partially reversible and with weight reduction and iron elimination, it should last me through my lifetime. The damage to the adrenal system isn't covered by his grant monies, but he will set me up with a Doctor to address those issues.

Best news of the last several weeks. We're now cleared for phlebotomy twice per week and reduction of iron for a long time. The ongoing challenge will now be to balance the chemotherapy and the phlebotomy therapy so I don't run out of blood cells. Both sets of doctors will monitor my blood cell levels and make adjustments as needed.

Punched in the ribs... cont.

2008-07-09T19:34:00.002-06:00

Great day - all the tests were concluded and I got out of jail early! Nothing 100% conclusive but all prelims suggest that I have averted diabetes by starting now. Dr. M. said that I would likely have developed it in "a year or two" and that I was now Insulin Hypersensitive. Meaning: that the pancreas has been damaged and that less insulin is being produced, but that my body has had to become so sensitive to the smaller amount of insulin that it can still cope with changes in sugar level. Rx: get rid of 10 lbs of iron and 20-30 pounds of fat cells, eat reasonably and come back in 1-2 years when my iron is under control!

Tomorrow (Thursday) we'll get all of the results. Fridays and Mondays I'll give up a pint of blood as long as 1-2 years or as long as my veins can take a needle. (I bought long sleeved shirts to wear!)

Thanks for all of the calls and texts and emails. I love ya! Really.

Testing: First day feels optimistic

2008-07-08T07:18:00.006-06:00

6:00 Left for the hospital. My body tells me why I'm going to get cut, poked, prodded, x-rayed and all of the other things: I'm tired all of the time; exertion drains me of energy; my joints keep me awake at night with pain; and, there is a big question of how much damage there is. I'm hoping for the best and expecting the worst.

7:22 Sitting on the bed, waiting for transport to the first set of tests. Life is good - I have a lot of love supporting me and have a brain and an iPod full of picture memories and music. The ultrasound tech has drawn pictures where my gall bladder and liver are located with magic marker on my chest. The "X" spot is between two ribs under my right pectoralis minor.

9:30 Oral Glucose Tolerance Test "OGTT" - both arms are plumbed for saline drip, 7 blood vials taken, blood drawn every 30 minutes. Headache from the Glucola - solid sugar drink - tastes like sugar and orange juice.

11:00 "I've been stabbed with an ice pick." The liver biopsy procedure was done in about 30 minutes. After an incision between two ribs, a 3-4 inch needle was inserted through the ribs, into the liver and a pencil-lead-size tube of liver was extracted. It was a visceral shock - more than pain, but left some lingering pain for an hour. On a pain scale of 1-10, the pain was less than 4.

The liver sample was dark brown (instead of light tan) indicating a high concentration of iron. It mostly stayed together when pushed from from the needle - indicating that the damage is not to the cirrhosis stage. <> The results from the pathologist will be available on Thursday morning.

The Doctor and HOSPITAL STAFF are fantastic - knowledgeable, friendly and efficient. By nature I am a happy optimist - and they feed me with timely information and positive attitudes and feedback. There are always exceptions.

The OGTT indicates that sugar levels are close to normal and lessens the likelihood that I have progressed to diabetes. Tomorrow, the big test will find the degree of insulin sensitivity I've developed because of the iron. During the night, I'll have blood drawn every two hours and around 4:30 will have a calorimetry test to determine my resting metabolism.

End of day: My apprehension level has dropped a great deal today. Today's initial tests suggest that although there is organ damage, it is not as severe as it might have been and may be partially reversible. The symptoms, however, will continue for a long time - some indefinitely. Overall, it was a good day.

Finding the extent of the HHC problem...

2008-07-05T23:17:00.002-06:00

7/9/08 - 7/11/08 Scheduled into the University Hospital for testing: liver biopsy, glucose tolerance and other tests to determine any organ damage. Iron deposits from HHC most often affect the liver, adrenal glands, the heart, and the pancreas. Arthritis in my hands and one ankle are also typical and present.

Bad news. It's really not arthritis...

2008-06-26T16:45:00.005-06:00

The "arthritis" in my hands and ankle had been growing progressively more painful - to the extent of 6 extra-strength Tylenol a day. Finally, a doctor at the university hospital diagnosed calcium deposits and bone damage - caused by an excess of iron in my body. Later, a DNA test confirmed that I have two copies of the mutated C282Y gene - one from each parent, and the most common genetic mutation for Caucasians - about 10% of the population are "carriers" and 1 in 300 are affected in some way.

BOMBSHELL! Hemochromatosis or "Iron Overload" has deposited a large amount of iron in many of my body organs - and extensive testing will be required to determine the extent of organ damage. The treatment for reducing iron consists of phlebotomy: removing blood from my system - 2 pints a week for up to a year. Phlebotomy or "blood-letting" is an ancient practice - going back over 2,500 years.

I'm unhappy that this was not discovered years earlier - and I'm troubled by the negative possibilities which testing might reveal. "Let not your heart be troubled" has become my mantra, but mortality is a sobering thought. Life is good - and life's experiences are good. And "What is, is".

Polycythemia Vera (PV) Wake up!

2008-06-15T21:00:00.002-06:00

In the fall of 2006, I applied for life insurance and had a blood test. The policy was denied because my blood cell counts were abnormally high. The platelet count had risen from normal 300,000 ("300") to over 600 and by year end - over 1,300. Platelets cause clotting in the blood - and strokes and heart attacks.

Luckily, Dr. Joseph Prchal at the Huntsman Cancer Institute is world-renown for his understanding of the JAK-2 stem cell mutation which causes unchecked production of stem cells and blood cell production in the bone marrow. DNA testing showed that I have a rare cancer-like mutation which, untreated, often leads to strokes and heart attacks. It is incurable, but can be treated with daily doses of a chemotherapy to kill excess stem cells and the associated blood cells. I am part of a life-long study of the Myleoproliferative Disorder called "Polycythemia Vera" or "PV".

So - after a huge wake-up call, I take a daily dosage of two drugs to reduce PV to a background nuisance. The typical prognosis is for a normal life for some number of years. With enough time and a good attitude for now, the scary nature of PV has been reduced to a schedule of medication and regular blood sampling.

I have great respect and appreciation for the ongoing efforts of Dr. Prchal and his associates at the Huntsman Center. It has become a friendly place to visit.