Day's Three & Four on Inrebic

Side effects? What side effects?

I haven't been able to answer the question, "Are you experiencing side effects?" because I haven't been sure what all the side effects might be. The paper that came with the meds was very generic - not specifically about Inrebic at all. Dr. Gerds told me that diarrhea was common - but yesterday I wasn't having diarrhea.

I did have a cough and chest congestion. But my son's family and other families in the church had the same thing last week. Seems likely this is a gift from them.

I also had a lot of arthritis pain. But I've been working on getting a bathroom exhaust fan installed in our master bath. Some people probably handle this better than I - but for me it involved multiple (5? 10?) trips into the attic crawl space and some very cramped working conditions. No wonder I'm sore.

But today - day four - I believe I have begun experiencing a true side effect of Inrebic. Sure enough, the diarrhea has arrived! (Got that bathroom exhaust fan installed just in time!)

It could be worse - the cramping is not too terrible. I have made frequent trips to the bathroom, but I haven't exactly spent all day in there. So far I have not even taken Immodium. (Maybe I will do that before bedtime tonight?)

While I could stand to not have diarrhea and I hope it goes away fairly soon, having the one true side effect that I know about is kind of reassuring. The medicine is working in my body like it has in most people for whom it also worked to decrease the symptoms from myelofibrosis.

Second Day on Inrebic

As the Jakafi was losing its effectiveness I was having more constitutional symptoms. The worst of it was the pruritus - intense, painful itching - as if you were being eaten by fire ants.

When I stopped taking Jakafi so that I could go on Inrebic, I found out that Jakafi had been working better than I had thought. I suddenly had some terrible attacks that lasted for hours. It was terrifying.
The worst of it was that I didn't know how long Inrebic would take to start working. I figured I might have weeks of these attacks and it was NOT a happy thought.

Well, I do not know for sure that the Inrebic is the cause, but I did not have any serious attacks of pruritus today.  I spent much of the day doing some really dirty and sweaty work that would normally be an invitation for pruritus, but I only had a slight case that lasted about 15 minutes.

I even took a shower after I got all that work done. That is a recipe for a puritus disaster. After you get out of the shower and start to dry - that is when it frequently hits. But not this time. No pruritus!

I say praise the Lord! Evidently the Inrebic is working right off the bat - and that is a very hopeful sign. May it work on into the distant future and get my numbers into even better shape.

First Day on Inrebic

Since my spleen was very large and I was having some constitutional symptoms like pruritus after showers and some night sweats, I thought Jakafi was not doing much for me. So I didn't expect much of a change when I went off the Jakafi.

Well, I'm here to tell you that my pruritus went wild after only twelve hours without the Jakafi. When I got up from bed and got dressed it started and it was intense. Now we are a few hours down the road and it is not so intense, but it is still stinging me - particularly on my legs.

I took my first dose of 400 mg. of Inrebic at about 6:00 am. This medication has just been approved about a month. The capsules are orange/brown with no markings. They don't have 400 mg. capsules, so you have to take four 100 mg. capsules.

Someone told me not to expect much beneficial action from the med for a few weeks, but I'm sincerely (very sincerely) hoping that it has an early effect on my pruritus. Yowza!

I followed the advice of the pharmacist to take the med with fatty foods. (A benefit already!) So I had bacon and eggs and a buttered English muffin.

A minute after I took the capsules I started to have a bitter taste at the back of my mouth. Eating helped, but now - more than an hour later - I am still having a diminished but noticeable bitter taste.

We will see what the side effects are. They said diarrhea - but my bowels are already messed up because of the package of fig newtons I ate yesterday!  We will see.

Time For Something New

I haven't written for a long time now. That is partly because my myelofibrosis has been chronic and not really in the forefront of my mind. About once a year I would be concerned about my blood counts.

Last January Dr. Gerds was concerned as well because my WBC was up in the high sixties and my spleen was growing again. He had a bone marrow biopsy done along with extra blood work. But it came back negative. Pretty much the same old thing. No sign of AML starting to develop! (Praise the Lord!) We talked about trying one of several trials that he was doing, but it seemed to me that I was borrowing trouble - more blood draws, daily record keeping, more BMBs, and side effects from the drugs - without any idea if it would help my situation. I decided to wait.

Since then my wife and I have been buying a new house in a new town, preparing our old house for sale and our new house to live in. I have been working on plumbing, electric, and carpentry. And I'm not done yet. It has been a tremendously busy time. It has been an extreme challenge. And in the midst of moving my spleen has been marching on.

Dr. Trehan told me it was very big again. Dr. Gerds measured it at 18 cm and noted that it was past the midline of my body. (It hasn't affected my eating... I eat too much no matter what.) My WBC has been up to 75 and over twice, but mostly ranges from the upper 50's into the upper 60's. I was concerned two months ago when my HGB was down to 10.8 and my platelets were at 102. But they sprang back the following month.

Constitutional symptoms have increased too. I have occasional night sweats,  and frequent pruritus. My fatigue is worse again too.

So, Dr. Gerds and I have decided on a change. Inrebic (Fedratinib) was approved by the FDA in mid-August. I will start taking it this coming Tuesday, Sept. 24.

Tune in here to read about my experiences with this new medication!

Not Everything Is Myelofibrosis

I remember when I was first diagnosed with MF - suspicious that every pain, burp or pimple was part of the disease.

Eight months ago I stepped on something that pierced the sole right on the ball of my foot. It bled, but neither I, nor my PC doctor could find it. Since then I have been unable to exercise as I have for the past 30 years. Anytime I walked very far or was on my feet very long - particularly in dress shoes - I would be lame for a day or two.

To make things worse, I had a pain develop in my neck. My neck has crunched and made gravel noises for years and years, but now it was hurting. The doctor had me get X-rays and then I went to PT. The PT exercises made the situation much worse. My doctor sent me to have an MRI and then I went to a neurosurgeon. He sent me to pain management. I had three courses of epidural shots into my spinal column. They helped for a week or so, but then we were back to serious paralyzing pain.

Well, between my lame foot and my very painful neck I was not a very happy camper. I was very limited and was constantly grunting or exclaiming in pain.

I would have liked to have blamed MF. At one point I thought, "This pain in my foot can't be some small thing I stepped on. It must be part of my gouty-arthritis." I also thought, "This pain in my neck isn't from having a bundle of shingles dropped on my head from a story up - it must be because MF is breaking down my neck bones.

Glad to say, both of these have been resolve for me in the space of about three weeks. The neurosurgeon offered me a three vertebrae fusion or Gabapentin. I took the Gabapentin and the worst of my pain was gone almost immediately. 

The shingles dropped on my head didn't do me any good - and maybe MF is making my "Facets Disease" worse - who knows? But the problem had a solution and I am blessed.

Then, a spot on my foot swelled up and finally burst with pus. That didn't fix my problem, but I kept drawing salve on the spot for about two weeks, then one day the spot felt rough when I passed my finger over it. When I looked, the plastic shard glinted at me and I was able to pick it out with my fingers.

My foot is restored! I'm back to exercising and full steam ahead activities. It wasn't gout, it was a small foreign object in the ball of my foot. Now it is gone and I am good to go!

Gradually I have learned that it is not always the MF - and that is a good thing!

Certainly you should get everything checked out. But don't give in to "This is part of MF and I am going to have it for the rest of my life." No! Hang in there. Keep looking for solutions. Many of the things that trouble you have solutions.

Doctor Shopping

When I joined the MPN community I became aware of something I call "Doctor Shopping."

In the simplest example people are "firing" their doctors because they don't like the way the doctor is treating them. Fair enough. Just because someone is a doctor doesn't mean they have a license to be a bully or a jerk. Sometimes patients feel like they have to twist the doctor's arm just to get test results. Sometimes it is apparent that the doctor thinks his patients are idiots. A friend of mine who asked intelligent questions had her doctor toss her paperwork at her and sneer, "Where did you get YOUR medical license?"

But there is another scenario that is not so reasonable. In this scenario a patient decides that he knows how to treat his rare disease, but his doctor disagrees.  So the patient goes on a quest to find the doctor that agrees with him and will give him the treatment that he has chosen for himself.  The patient may have good information or bad information. He may be taking cues from fellow patients on the internet who have had good success with one or another treatments.

I'm not sure that we would not be better off just randomly picking out a medication from the pharmacy shelves because we like the shape and color of the pills. They are taking the advice of "friends" they don't really know who are generalizing from their own medical condition and response to treatment to a person across the globe who they have not even met. We are supposed to take the advice of non-doctors who have not examined us over the advice of real doctors who have examined us.

I do know a lot more about myelofibrosis than most people in the country and I know more about myelofibrosis than many doctors. But that doesn't make me a doctor. I know some facts about myelofibrosis, but I do not understand the underlying chemistry, biology and genetics that are at work or how various treatments might effect a person. I do not dare pretend to be a doctor.

If you think you are getting inadequate medical care, by all means get a second or third opinion and switch to another doctor. Find a doctor you trust and trust that doctor to give you good advice. In my opinion a good doctor should be willing and able to explain what they are doing and why they are doing it. But don't decide beforehand what treatment you must have and find some doctor who will give it to you no matter what.

I have one caveat in this.  Sometimes there are doctors who are mavericks who are swimming against the tide.  One who comes to mind is Dr. Silver from Weill-Cornell. He has long been a proponent of using interferon in treatment of PV and ET (and maybe in very early MF.)  Some people who have heard of the success of others have sought out Dr. Silver because they want interferon treatment. Well and good - because Dr. Silver is not just giving people whatever they want. He has done years of research and he carefully examines new patients and does all the necessary tests to see if he thinks interferon is appropriate for them.

Be careful. Find a good qualified doctor with experience in your particular disease. Make sure he or she is a good communicator. Ask good questions. Consider them your partner in pursuing better health.

Lease or Buy? Myelofibrosis makes me morbid.

Last week I traded in my 2015 KIA for a 2019 KIA  and I decided to go from a car purchase over five years to a lease over three years.

My wife and I thought the lease made sense because it was cheaper than the purchase on a month by month basis. (You don't build up equity, but if you are going to buy another new car when the first is paid off, the equity disappears into the next purchase anyway.)

I like my new car, but within a day or two I had this morbid thought... "A three year lease is perfect because the future of my life is so uncertain.  I might not need a car three years from now."

While there is some truth involved in this thought, overall it is completely false and filled with maudlin self pity! Shame on me!

It is true that I do not know what will happen over the next three years or what condition I will be in by the time these years have passed. Proverbs 27:1 says, "Do not boast about tomorrow, for you do not know what a day may bring." The truth is that the car itself and the activity of driving it on the same roads as distracted drivers might be a bigger hazard to me than myelofibrosis.

Note to self: Stop the self pit and morbid thoughts! Enjoy your new car and be grateful! You don't want to waste a single precious moment of this life and its blessings.