Things Are Falling Into Place

This is a time of intense insecurity with a backdrop of tremendous insecurity. 

COVID-19

COVID-19 is part of that background and at this very moment in my county we are at the highest level of public health emergency. Yesterday the deacons and I decided to once again cancel our in person services and ministries. Now we are going back to ministry by YouTube, email and phone calls. But how long will this last? When will things get back to "normal?"

POLITICAL DIVIDE

The recent presidential election is another part of the background. The country is very evenly split. One side things everything will return to "normal" now that their candidate has (barely) won the election. The other side thinks that we are descending into the abyss of socialism that will be working to decrease their personal freedoms and redistribute their wealth. Some think we are on the verge of a second civil war. What will really happen?

Against that background I am moving inexorably toward my SCT.

UNCERTAINTY

It is impossible to say what will actually happen. There are so many variables - known and unknown. Will I live or die? Will I suffer serious organ damage or have chronic GVHD? How long will it take for me to regain my strength - and how much of my strength will I recover? Will I be well - or will I be unwell? How long will it be before I can once again have an immune system that allows me to be in groups of people?

But as the title suggests, things are falling into place. 

MY HEALTH

I am in decent physical condition. I have not had even one transfusion. I do not have any blasts in my peripheral blood. I feel well enough to work. My main complaint is daily fatigue and itching.

THE MATCHES

Cleveland Clinic sent me three profiles of 10/10 matches and I understand that these have all been contacted to give approval for their information to be sent to me. I was also informed that the transplant team has chosen one of those matches as the one they would like to use as my donor.

THE SUPPORT

The matter of support during my hospitalization and subsequent convalescence looks encouraging. My wife can be my 24/7 care provider for the first 100 days as long as I can also have supporters who will commit to driving me to Cleveland for appointments and/or emergencies. We will also need help with cooking and cleaning during that three months.

THE CHURCH

Meanwhile, my church is willing to give me a year of leave to try to recover and return to ministry. Hopefully that will work out and we will also be able to provide a substitute pastor who will take care of the church throughout my absence. Those arrangements are still being made, but things look very promising.

GOD'S SOVEREIGNTY

Many years ago I surrendered myself to God for his glory. At the time I did not realize where exactly that would lead - but my commitment was to go anywhere and to endure anything to serve him. That is my prayer about this SCT.

I would rather work as a pastor without going through the near death experience of an SCT. But if this is what God has for me - then I know he will bring good out of it one way or another.

God's sovereignty is not "falling into place." (Smile) But my coming to a place of greater peace because of God's sovereignty is part of my sense that everything is falling into place.

Onward! Forward! Let's get this done!

Progression Catches Up With Me

 I'm glad to say that I had a rather uneventful year on Inrebic, in spite of the COVID-19 pandemic that started in the Spring of 2020. Life got incredibly busy with the pandemic and with some physical problems my wife began having just before the pandemic broke. The long and the short of it was that from March of 2019 the demands on my time became intense. On the one hand my wife needed quite a bit of support and on the other, my pastoral ministry demanded more time in unexpected ways.

You might have thought that a pastor would have time to burn during a pandemic when the church was closed down for three or four months. You would be wrong. First there was the learning curve in figuring out how to continue weekly Bible lessons for the church and then learning to make it work. I settled on making YouTube Videos and ultimately found that in addition to my normal sermon preparations, YouTube took me an extra day each week. I have a new appreciation of the many YouTube personalities that make it look so good. My efforts are pretty rough - but they accomplish their purpose.

Dealing with my wife's health issues and my own issues was also a source of stress. Everyone in the medical profession seemed more concerned about COVID than about any other physical issue. The virtual doctor visits cost the patients just as much as an actual visit - but in my opinion they are almost worthless. You get to tell the doctor how you feel - but they cannot take your vitals or listen to your heart and breathing or anything else. They are just guessing about how you are based on what you have told them. I ended up canceling a couple of virtual appointments that I had and I very much felt like my doctors did not much care one way or the other - if they knew at all.

I also went for several months without getting a blood draw because of the pandemic. I knew that the lab would be backed up and that there would be an increased risk for catching COVID at the lab. So I waited until June to get back into monthly blood draws. At that point I found that my platelets were continuing a downward trend. I wasn't happy about it, but I figured (or hoped) that it was because of the Inrebic. By August my platelets were down to the low 80's. I had a virtual appointment scheduled with Dr. Gerds for September, so I started getting ready by checking on my previous appointments where I read his notes. Realized that he was saying I was not interested in a transplant.

When I was intermediate-1 risk I was not interested in a transplant. An SCT is no bargain and I have had a dozen friends die during or after transplants due to the complications. It is not something that you just do for the heck of it. I had hoped that my myelofibrosis would progress slowly enough that I could make it to 75 years of age without a transplant. I figured at that point I would not seek to have a transplant. But I have always said I would definitely try a transplant if I became high risk before I was 70.

So, when I had the appointment with Dr. G. I pressed him about the platelets and he said it was because of my disease - not because of the Inrebic. (RATS). Then I asked him to tell me where I stood and he walked me through the MIPSS70 assessment to show me that I was at High Risk with only a 34% chance of living five years. (I suspect that I am very close to the cut-off between intermediate-2 and High Risk - but I'm close enough that it doesn't really matter.)

To make a long story shorter - Lola and I decided to move ahead with getting me a transplant while the getting was good. It is upsetting in many ways and daunting. But it seems like the only reasonable thing to do, given the progression of the myelofibrosis and my poor prognosis.

Now I just wish we could get it over with.

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.