Winnipesaukee for a week and then back to the Treatments

We took advantage of the third week of the first cycle, the week when you have no treatments and the toxins have a chance to wash out of your system, to go to New Hampshire.  It was very nice and relaxing, but somewhat frustrating in that there were all sorts of tasks to be done that I normally would do, but which I cannot do in my condition.  Initially it was chilly, but then it got good and hot.  Having the lake right there was a blessing.  Not that I could dive in, but I could walk down the ladder and experience getting used to the bracing water slowly.  Swimming turns out to be good, but I am still reluctant to do any sudden motions, for fear of breaking yet another bone someplace.


Five guys from a men’s group I am part of came up for the weekend, and attacked Barbie’s list of man-chores.  They worked like demons, and got everything on the list plus more done.  A good time was had by all.  Barbie fled to Vermont to do Tyler babysitting.  Had she been here, she would have been compulsively cleaning after every move the men made.  We batched the job, and cleaned up at the end.  Here are a few pictures of the group, just to show this blog can feature pictures as well:

Then it was back to Stow to resume “normal” life and treatments.  But first there was some drama about the 3rd drug I am to receive in this new cycle of treatments.  The new drug is Revlimid, which is related to Thalidomide, the drug that led to deformed babies in the 60’s.  I had to sign a statement saying I would not engage in unprotected sex with a fertile female, not share my pills with anybody, not let a fertile female even touch the pills.  Then came the issue of Cost.  The retail price for 14 days of pills through a mail order pharmacy (RightSourceRX) is $5453.97.  Under my Humana Drug Insurance plan, the co-pay for this prescription is $2404.48.  Since this is a drug I will get from here on in, and maybe for maintenance as well, this was quite a shock.  So the Nurse Practitioner put me in charge with a lady at Celgene, the manufacturer of the drug.  Apparently there is a foundation that can help people who cannot afford to pay for the drug with their co-pay amounts.  If this were to depend upon last year’s tax return, we would never qualify.  But this year I have not been working and we just decided to take Social Security.  That plus the fact that Barbie does not teach during the summer enabled us to get below the magical level of $75K of income to qualify.  For this round, our co-pay is $30.  I suspect it will go up for subsequent rounds, but never be close to the $2404.48 mark.  Our health care payment systems make so much sense!

Upon return I had the following questions for Katie Conway & Dr Levine.  The questions in black are literally what I took with me to the hospital.  The answers are in red.
1. What are my blood readings?  Can I get them on a regular basis, so I have some knowledge of how I am reacting to the treatments?  We now know about the International Staging System, which is based on blood characteristics.  What are my serum beta-2 microglobulin and serum albumin levels?
KC: Normally they don’t share the blood results, as the raw numbers can alarm some people, and they want to deliver the information in person.  But if we are interested in the raw numbers, they would be happy to share them.  The batch of tests they requested today are ones that take a few days to process, so she could phone or email the results when they come in.  We traded email addresses.  The INR was one test that comes in quickly, and I am at 2.4, so Dr Levine is happy, and we can proceed with the Revlimid.
2. Where do we stand with appealing to the drug company for a reduction in the cost of Revlimid?  Do you have an English version of the Revlimid pamphlet?  You gave us a Spanish version last time.  Can we get a medical letter of necessity to use with our drug insurance company?
KC: See above with the Revlimid cost appeal.  I completely forgot to mention the Spanish pamphlet.  My GP later in the day volunteered any letter of necessity should the insurance company require one.
3. What will the dosage of revlimid be?  [I assume they will tell me that.  I suspect I don’t need to ask.]
KC: She had already phoned in the prescription to RightSourceRX.  25MG capsules for 14 days.  The shipment was coming by UPS.  I left home around 9:30 on Tuesday, and they had not delivered by that time.  They did arrive Wednesday afternoon, and I started the 14 days Wednesday night.  They suggest taking them at night, as they make you tired, and it may counteract the wakefulness that comes from the steroid.
4. When will I next get a dose of Reclast?
Dr Levine: Reclast they only give once a year.  There is a different form of Zoledronic acid that they give on a monthly basis to Multiple Myeloma patients, I think the name is Zometa.  They gave that to me that Tuesday.  [So my list of appointments shows who I am going to see, not what they are going to give me for treatment.]
5. I have continuing pain in my ribs.  I don’t feel as if I am breaking anything new, but there is ongoing discomfort.  Could that be because there was damage to the ribs and they are never allowed to be completely at rest?  Did the 5/20 MRI show any damage to the ribs?  If not, why should I be experiencing rib pain?
KC: The MRI analysis says nothing about ribs.  The MRI was focusing on the spine, and normally they don’t pay much attention to other things.  At any rate, if there is rib damage, they just have to get better on their own.  Which they normally do.
6. I would like to resume some physical activity.  I have done some swimming, but initially I felt that I had to be careful to avoid sudden moves, which might cause additional damage.  Would joining a local pool be a good idea?  Would doing sessions on a stationary bike be OK?  How soon should I try to increase physical activity?
Actually by this time I had twice done the stationary bike in the barn, armed with a cell phone at Barbie’s insistence, in case I did any harm to myself.  It felt good to actually get my heart beat up a bit and to breathe hard.  KC said be careful, but do whatever works for you.
7. How long should I expect the “wired” effect of the steroids to last?  Even during my week off I was experiencing wakefulness during the night that I do not normally have.  Yes, you gave me a prescription to deal with that, but I am loathe to take too many pills.
KC: She reviewed my list of prescriptions, as she had prescribed a couple of pills for nausea and one for nausea and insomnia.  She suggested that I take Omeprazole on a daily basis to reduce stomach acid.  She pointed out that Lorazepam should address the insomnia, and I should take that as needed.  This gets me up to 10 pills a day, at 3 different times per day.  I am like clockwork with the morning pills.  I am less reliable with the other pills at 2PM & 10PM.  I often get out of bed remembering that I forgot to take the bed-time pills, only to find the 2PM pill is still there.
8. Dr Levine was skeptical about my Coumadin schedule of 2.5 every day except 3.75 for Mon, Wed, & Fri because the dosage was not even.  If I should have a different dose that is even every day, then maybe I should have different size pills.  My pills are 2.5 and I take 1 ½ on the days I take 3.75.  The total for the week is 21.25.  Would 3 milligram pills be better?
KC: Manage it as best you can.  No apparent need for a prescription change.  I have my own testing device.  Use that to adjust the dosage appropriately, getting the INR into the 2-3 range.
9. Can we communicate with you via email?  Frequently questions occur to us when it would be inappropriate to call the phone #’s available.  We would prefer email.  Possible?
We exchanged emails.
10. Bridget said there is a process whereby I can access all my medical records at BIDMC.  How do we start up that process?
KC: BIDMC has that process, but the Oncology department does not participate in it.  There are some readings that can be quite alarming to patients, and they would rather deliver those messages personally.

Overall the treatment went well.  But as I was sitting there receiving my Zometa (or whatever it is called), I got a cell phone call from Susan Avery.  She was on a return flight from Tanzania with her whole family, and I had arranged to pick her up at the airport.  I had completely forgotten!!!  So it was a race to my car and off to Logan Airport as soon as the drip finished.  I got there and hit redial on my cell phone.  It shows you how much time has elapsed since the prior call: 56 minutes.  So I made them wait an extra hour at the airport, after they had been travelling for 14 hours already.  But we had a great talk about Tanzania and the safari they had gone on.  It was a family-oriented trip with 8 of the 17 people on the trip kids, and they had a great time.  But by the time we got to their house, my bladder was just about bursting, as the drip sure puts your kidneys into overdrive.  So I got to see the inside of their very nice Victorian home.

The extra trip to the airport, which hadn’t even made my cell phone calendar, put me behind for an appointment with my general practitioner, Dr Kafina.  He reviewed my records and expressed sympathy.  But he said I looked very good and appeared to be bouncing back nicely.  He searched in the medical records for various tests and procedures that were done to see if there were any signs that should have been interpreted differently.  I am convinced I had a bone marrow biopsy as a result of a test that revealed a Monoclonal Gammopathy of Unknown Significance (MGUS).  Dr Kafina could not find that in my records.  He asked the administrator/receptionist to follow up on that.  I asked if people with MGUS shouldn’t be monitored more closely.  I did have a session with a hematologist (Dr Humberto Rossi) in 2009, so it is unclear how much more should have been done.  He gets lots of patients with MGUS who live on into their 80’s before anything happens, and usually it is something unrelated that does them in.

That evening I had a very nice dinner with friends down the street.  I have been absolutely brazen about inviting myself out to dinner with friends during this time home.  The Fischers on Tuesday night, Firooz last night, and the Brownfield/Lents tonight.  In return the Brownfield/Lents are coming up to New Hampshire this weekend, but I am hitching a ride with them, so it is not totally without strings.

In general, I am feeling much better.  It feels as if the bones have stopped breaking and are on the mend.  The worst part about breaking new bones was the knowledge that the 6-8 week time clock for bone healing was being reset to 0.  Now maybe all the calcium, Reclast, and Zometa is having the desired effect of building bone strength back up.  Katie Conway did call with a report on my blood results.  The key measure they are using to gauge the progress against the disease is the serum free lambda (whatever that is).  The target is less than 26.  My initial reading was 1329.  My latest reading is 317, which means the treatment has cut the reading my more than a factor of 4.  She said this was a great first response, and she wanted me to know it as soon as it became available.  Hooray!

Multiple Myeloma – Links + References

When you first look up Multiple Myeloma (MM) on the web, you get a fairly pessimistic outlook.  Wikipedia (http://en.wikipedia.org/wiki/Multiple_myeloma) says median survival is 3-4 years with conventional treatment.  Advanced treatment may get you 5-7 years.  The Mayo Clinic gives all sorts of details, but no prognosis.  http://www.mayoclinic.com/health/multiple-myeloma/DS00415.  Similarly, the U.S. National Library of Medicine site, http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001609/ provides lots of information, but no hint of survival rates.  To quote, “Survival of people with multiple myeloma depends on the patient's age and the stage of disease. Some cases are very aggressive, while others take years to get worse.”  


The American Cancer Society page on MM (http://www.cancer.org/Cancer/MultipleMyeloma/index) has lots of information.  Their MM booklet is somewhat pessimistic: 
The American Cancer Society's most recent estimates for multiple myeloma in the United
States are for 2012:
•About 21,700 new cases will be diagnosed (12,190 in men and 9,510 in women).
•About 10,710 deaths are expected to occur (6,020 in men and 4,690 in women).
The 5-year relative survival rate for multiple myeloma is around 40%.
I believe my MM is classified as Stage I.  They say the median survival for stage I is 62 months.

Dr Levine gave us booklets from the Leukemia & Lymphoma Society on Myeloma and Blood and Marrow Stem Cell Transplantation.  You can get a .pdf of each of these from http://www.lls.org/resourcecenter/freeeducationmaterials/myeloma/myeloma and http://www.lls.org/#/resourcecenter/freeeducationmaterials/treatment/.  Barbie and I have found these booklets very helpful, especially the first one on Myeloma.  It does not get all that specific in terms of the prognosis, but it remains hopeful: “Myeloma is a cancer of the plasma cells in the bone marrow. It is estimated that 20,520 new cases of myeloma will be diagnosed in 2011. Although myeloma is incurable, this is a very hopeful time for patients. Dramatic advancements in new treatments over the past decade are resulting in better overall survival, allowing many patients to maintain a good quality of life for years. An estimated 74,800 people in the United States are living with, or are in remission from, myeloma.”

I find I only read the parts of the documents that apply directly to my case.  I had an MGUS (Monoclonal Gammopathy of Unknown Significance), a blood level that was detected several years ago, probably around my second pulmonary embolism in 2004.  I had a bone marrow biopsy then, which proved to be negative.  Then some time later another blood test raised the issue, and additional tests caused the hematologist to decide everything was still benign.  We are now wondering why someone didn’t call for ongoing monitoring tests.  I do not have kidney problems, high calcium readings in the blood, fatigue, or recurrent infections.  Primarily I have bones that have been weakened to the point where they break without any traumatic event.  The recommended treatment is for an Autologous Stem Cell Transplant.  Therefore I ignore everything they say about Allogenic Stem Cell Transplants.  Funny thing, how something that is happening to you yourself can concentrate the mind.

Second Opinion, Decision

Monday we met with Dr Jacob Laubach at The Dana Farber Cancer Institute (DFCI).  It was a big deal to get our records from Beth Israel Deaconess Medical Center (BIDMC) to DRCI, as the two hospitals have incompatible computer systems.  But we got all the records and even filled out an 11 page form listing all sorts of details in my medical background.  But the doctor reviewed what was available to him over the weekend, which did not include the CD of images I got on Friday from BIDMC, so we had the feeling that some of the recommendations were based on incomplete information.  Just the cost of spanning different medical institutions.

Dr Laubach was very hopeful.  Had I gotten MM 30 years ago, it would have been 2 years & out.  20 years ago it was up to 3 years.  Now it is expected that they can fully suppress MM, and that MM should not be the ultimate cause of my demise.  They cannot cure MM, but they have excellent tools for putting it into remission, checking that it is still there, and beating it back if it comes out of remission.  Dr Laubach examined me and declared that I was healthy other than the MM and the cracking bones.

Dr Laubach recommended a different course of treatment than the one I am on now.  He said the standard for MM care at DFCI is a 3-drug cocktail consisting of Velcade, Dexamethasone, and Revlimid.  I am currently on a 2-drug cocktail consisting of the first 2 drugs.  Actually, the second in the list is a steroid, not a drug per se.  Dr Laubach said he would contact Dr Levine and discuss why Revlimid is not included in my treatment.

Dr Laubach described two clinical trials I could participate in.  One (which is actually being run at BIDMC) is to create a vaccine from your stem cells and the MM cells themselves (somehow).  The second is a 3-part trial at DFCI where they are testing 3 different treatment protocols to see which has the most effective cure rate.  The first group would get the standard stem cell transplant and normal follow-up.  The second group would get the standard stem cell transplant followed by a maintenance dose of Revlimid.  The third group would get the standard stem cell transplant.  Then 3-4 months later they would get a second stem-cell transplant.  In other words, instead of practically killing you just once, they would practically kill you twice.  I cannot claim to be very interested in that option, given that they say the standard protocol has a very high success rate.  And what if MM does come back?  Can’t they do a second stem cell transplant again then?

Overall we were impressed by Dr Laubach and by DFCI.  Cancer is all they do, and they do it in bulk.  Dr Laubach clearly knew his stuff, and he explained the options and the reasons for them very well.  He opened up the possibility of me getting treatment at Emerson, supervised by him.  That would certainly be more convenient logistically, but we have already switched horses from Emerson to BIDMC, and I am somewhat reluctant to switch back.

We stayed Monday evening in Jamaica Plain with Brian and Bridget.  Bridget advised that the key decision is the 2- vs. 3-drug cocktail.  The clinical trials or the logistics are lesser concerns. It was great to talk things over with Bridget, as her medical background makes all this make sense to her, unlike us.  Even if she is not an oncologist, she has the intellectual context to fit the details in, so she can retain them.  We don’t, so things go in one ear and out the other regularly. 

Tuesday I had the 3^rd chemo treatment session.  I ate less for dinner Tuesday night.  I also took some antacids after dinner, as well as the anti-nausea medicine.  There was some digestive unease and a fair amount of burping, but no real nausea.  The real effect was being awake for a good part of Tuesday night.  Now sleeping is something I am pretty good at, so reading until 2AM and then not being able to get to sleep is very unlike me.  I spoke with my sister Teena (who is an ovarian cancer survivor) on Wednesday, and she said that is the effect of the steroid.  You are simply wired.  At least I have not had more severe effects from the steroid, which can include manic mood swings, even to violence.

Today we had the 4^th and final treatment in the first cycle.  We met with Katie Conway, a nurse practitioner who works with Dr Levine first.  She answered a few questions and deferred some to Dr Levine. Dr Levine then discussed his conference with Dr Laubach, and outlined our options.  Specifically, he discussed the 2-drug vs. 3-drug cocktail. 

The 2-drug cocktail was a very deliberate decision.  I have had 2 pulmonary emboli, and I was on Coumadin for 7 years.  I got off Coumadin recently because we found an internet article describing a study in 2007 that found a correlation between males on Coumadin more than a year and a 63% increase in bone fractures.  So, when I saw Dr Levine, I was not on any anti-coagulation regime.  Dr Levine was very worried about blood clotting, as I now have several factors that cause increased clotting.  First MM itself can cause additional clotting.  Second, bone fractures can increase clotting.  Finally, Revlimid results in a 20% increase in the likelihood of clotting.  Since we were eager to get started on treatment, Dr Levine chose to refrain from using Revlimid until I could get my INR reading up into the 2-3 range.  After all, even if the probability of a blood clot is low, the risk associated is high.  I have survived 2 pulmonary emboli, but the third could be fatal.  This restored our confidence in Dr Levine, and reminded us that this is all largely Greek to us.  We both remembered that Dr Levine had been concerned about another embolism, but we had not put that together with the choice of the 2-drug cocktail.

He also said that BIDMC and DFCI are actually both under the umbrella of Harvard Medical School, so they have the same protocols available to them.  Yes, DFCI handles more cases, but BIDMC certainly handles enough to have a very reliable practice.  The option of having the routine treatments administered at Emerson and direction of the treatment come from BIDMC is not available.  We have to choose one horse to cross this stream.  At the end of the day, Dr Levine answered all our questions satisfactorily, and we choose to continue with treatments at BIDMC.

Revlimid will be part of my treatment next cycle, assuming I am fully anti-coagulated.  To that end I am upping my dose of Coumadin to get my INR over 2.  Revlimid turns out to carry its own set of issues.  A monthly dose of 28 pills costs $12,000 at the pharmacy!  Buying directly from the insurance company the price declines to $10,000!  Since we never took drugs of any scale, we got the cheapest drug plan on the market.  The co-pay for revlimid is 35%.  Apparently there is an appeal process that can get the co-pay down to 20%.  Still pretty hefty.  Plus it is in the family of thalidomide drugs.  So it is a very tightly controlled substance.  I had to sign a pledge not to get anybody pregnant (easy, I’ve had a vasectomy), not to have unprotected sex with anyone who is fertile (who would have me?  I won’t be taking the drug for another 2 weeks, so ladies, there is an opportunity), not to share the pills with anybody, and not let a fertile female even touch the pills without rubber gloves on.  So I signed the form, which will let us open negotiations with the drug company to determine how much this is going to cost us.  Barbie has long been concerned about how much this illness was going to cost us, and here the beauty of our health care system has raised its ugly head.  Once again, when I told her ‘Don’t worry about it’, I was dead wrong.

Next week is the 3^rd week of the first cycle, so there are no treatments.  No overnight trips into Boston.  Nuts!  So we will be forced to go up to our place on Lake Winnipesaukee and rest and recover.

Multiple Myeloma - 6/1/12

We got a call from Dr. Levine Thursday (5/31) saying the results of the bone marrow biopsy were in, and we should keep the appointment on Friday.  So then we were on pins and needles wondering what the results were.  Well, they were positive.  Despite some conflicting symptoms, I have Multiple Myeloma (MM).  Dr. Levine was quick to point out that MM is currently quite a treatable disease.  When he started out in this area 20 years ago, the life expectancy of a MM patient was 3 years.  Things are moving so fast that he doesn’t really have a statistic of life expectancy now, as there are too many survivors who have not yet kicked the bucket, so they don’t know how to count them.  For example, on one extreme he has a woman he treated 12 years ago whose MM has been in remission ever since.  On the other extreme he had a patient who did not respond to one combination of drugs.  Nor did he respond to the second combination.  By the time they switched to a 3^rd combination, it was too late.

Barbie asked what stage MM do I have?  Dr. Levine said he only has a snapshot of my symptoms, so it is difficult to say what stage it is in.  Furthermore, unlike some other cancers, the stage does not really affect the treatment plan, so he did not spend much time on that issue.  For example, if you have early stage lung cancer, you can operate and remove the small tumor.  Whereas in advanced stage lung cancer the tumor would have grown beyond where it could be cut out, so you have to use a different treatment.  With MM, there is a tumor in the sense that there is uncontrolled growth.  But the result is dispersed throughout the bone marrow instead of being rooted to one spot.  As a result the treatment is the same, so Dr. Levine did not really answer the question.

Dr. Levine gave us some brochures that explain both the disease and the treatment(s).  We have not yet finished reading the brochures.  Dr. Levine spent quite a long time explaining both.  There are clinical trials of new treatment protocols being conducted at Dana Farber, which is adjacent to Beth Israel.  Dr. Levine had gotten the forms to enroll me in one of those trials, but it turns out you have to be 65 or younger to qualify.  I am TFO.  So I will receive the “current” standard protocol.  The general idea is that they use a cocktail of drugs to suppress the MM.  That takes about 3 months.  Then they harvest your plasma stem cells.  They put your blood through a process that extracts the stem cells and returns the blood minus the stem cells back into your veins.  They freeze the stem cells for use later.  Then they put you into the hospital and use very aggressive chemo-therapy to attack any remaining MM in your body.  This is done in the hospital because your immune system is compromised, and you need to be in isolation to avoid infection.  After all your plasma cells are the ones that create antibodies, and without antibodies, you are completely open to infection.  Then when you are on death’s door, they reintroduce the plasma stem cells, and they regenerate plasma cells without MM.

The first step is to perform 4-6 three-week MM suppression cycles.  Each cycle consists of 2 weeks of 2 days per week of drug treatments.  Then you have a week off to flush the toxins from your system.  They perform blood tests before each drug application, and adjust the dose depending upon the results.  Once they get the dosage correct, the treatment should not be debilitating.  Dr. Levine has treated a physician colleague who was able to maintain his practice while receiving the drugs.  You just have to be able to adjust your schedule to include 2 visits per week to the hospital of 2+ hours each.

So we have a diagnosis, at last.  It may not be what we would have chosen, but who gets to choose?  Barbie points out that it is a good thing that this is not a result of something stupid I did, as in that case I would be the object of the full brunt of her anger.  Thank heaven I am innocent, as I doubt I could withstand that!  This will certainly make for a ‘different’ summer.  There probably was additional information that Dr. Levine gave us, but given the weight of the initial message, we really weren’t taking everything in.  It is one think to know, really know you are mortal.  It is another thing to be told you have cancer.  Yes it may be treatable, but it still is cancer, and your life expectancy is suddenly different.  We will certainly pick things up and attack this latest challenge, but for right now we are still absorbing the message.

Progress on the Cracking Backbone Front - 5/25/12

Today we had 3 medical events.  They were all positive, but we are still in limbo since we don’t yet have a positive diagnosis.

The first event was an infusion of Reclast.  Reclast is the brand name for Zoledronic Acid.  It is given annually for people with osteoporosis.  People lucky enough to have multiple myeloma (MM) get it monthly.  So regardless of the diagnosis of what I have, initiating this treatment made sense.  It is a bisphosphonate, whatever that means.  Somehow it helps to increase bone density and strengthen the bones.  The infusion happened first thing this morning, and only took about 25 minutes.

We had driven into Brian + Bridget’s house in Jamaica Plain the night before to avoid the traffic in the morning.  While talking things over with Bridget, Brian and Elizabeth (a physician’s assistant living on the 3^rd floor of their house), I testified how much I hated my back brace.  It hurts in several different ways, adding to chest pains, as it restricts my breathing.  Elizabeth suggested taking the brace to the representative of the company that made it who is on site at the BI.  So that is the second thing we did.  The fellow looked at it and said there were several adjustments that could be made, but he didn’t have the equipment to perform them there at the BI.  A company office across the street did have the tools, so we went across the street.  The guy there ended up taking an inch away from the armpits, where the brace had been binding.  He also removed an inch or more at the bottom where it was binding whenever I sat down.  The result was it rode up, causing an indentation that should have been just above my hips to dig into my ribs.  With that gone, I am sitting here with it on after a full day, and there is no problem.  He also extended a piece of plastic on one side to make it easier to get into and out of.  He was going to do the same on the other side, but we ran out of time.  Thanks to Bridget and Elizabeth, I am now MUCH more comfortable.

The third thing was to meet with Dr. Levine, a hematologist.  He specializes in blood disorders and blood-related cancers.  He also took a detailed history, probing for family members who had cancer.  There are quite a few of them.  Elizabeth and Brian attended this session, Brian because he was at the hospital to set up an operation to fix his shoulder and Elizabeth because she suspected that with her medical background, she would recognize and remember many of the terms that might just go over our heads.  Dr. Levine was also qualified to take a bone marrow biopsy, which is the definitive test for MM.  That he did, after doing a general exam and responding to a number of questions we had.  The bone marrow biopsy involves numbing the lower back over the pelvic bone.  In my case it was the left one.  Then they put in a fairly hefty needle to draw out some fluid from within the bone.  Getting through the surface of the bone involved quite a lot of pushing.  Dr. Levine commented that my vertebrae may be cracking at an alarming rate, but my pelvic bone was not very mushy.  Finally, they put in a larger needle and take out an actual sample of bone marrow.  This too involved a good deal of force.  It didn’t hurt because of the local anesthetic.  But somehow my body decided it didn’t like all that pushing and shoving, and suddenly I felt faint.  I was lying down on my stomach, so I had nowhere to fall, but I was told I turned quite pale.  Remarkably gray or white.  Soon they were all finished, and tidying up.  The nurse stayed with me for a while, concerned about my reaction.  She brought a cup of water and took my blood pressure.  It was down, but within normal range.  After a while, I perked back up.

The bone marrow biopsy now goes off to the lab for analysis.  That should take 7-10 days, so we are still in the dark as to what is happening to me.  We have an appointment to go back into the BI to see Dr. Levine next Friday, but especially with Monday being a holiday, that might get pushed off. 

So the mystery goes on.  I am to be religious in taking my pills that provide Calcium and Vitamin D, as that is what the Reclast is working with.  It is supposed to help build up the bone, but we were warned not to expect anything over night.  Healing bones takes multiple months, even years, and we still don’t know the cause.  More to come, ideally soon.

Treatment! - 5/23/12

Yesterday (5/22) at 4:15 I called Beth Israel to get in touch with Dr. Rosen.  While I was still wending my way through the phone labyrinth I got a call from Dr. Rosen.  The additional tests have not revealed anything new to nail down the diagnosis.  The protein electrophoresis still shows a low level of the protein associated with monoclonal gamopathy.  If I have multiple myeloma (MM), I should have a high level of that.  I do have mild anemia, but so do lots of people.  The skeletal survey (the 12 x-rays I took on Friday) should have shown gaps or punched-out areas in various bones if I had MM.  It didn’t, except for one in the skull.  [So I have a hole in the head.  What else is new?]  A possible explanation is a venous lake, whatever that is.  None of the other blood or urine tests showed anything unusual.

The MRI that was taken on Sunday shows fractures of multiple thoracic vertebrae.  The x-ray on 5/14 showed a fracture on T9.  This latest MRI shows fractures of T6, T10, T11, & T12.  That is in addition to 4 fractures of lumbar vertebrae, for a total of 9.  Dr. Rosen has asked for how far up the MRI taken back early in April went, in order to determine if these fractures are recent.  He said something about swelling indicating they are recent, but I wasn’t really listening, as I know the pain has just been in the last couple of weeks.  If my rib cage felt as it does now, I don’t think even I would have gone on the trip to Nepal.

So Dr. Rosen (endocrinologist) spoke with Dr. Levine (hematologist).  Dr. Levine will look for an opportunity to see me before my 5/30 appointment.  Dr. Levine would be the one to do a bone marrow biopsy, the test/procedure that would nail down whether I have MM or just garden variety osteoporosis.  But they agreed that while the test would nail down the diagnosis, it is not an emergency situation to do the test because the initial treatment is the same in either case.  Just the frequency is different.  If you have MM you get the dose monthly.  If you have plain osteoporosis, you get the dose annually.  Dr. Rosen gave the name of the drug(s), and in fact he may have mentioned synonyms of the drug or he may have named several drugs to be taken together.  Neither Barbie nor I got them down, as they were not names we had ever heard before.  It is administered intravenously, and I would have to go into BI to get it, ideally late this week.  Dr. Rosen’s secretary is working on the administrative side of things, as the treatment has to be approved by my insurance companies.  If we are lucky, Dr. Levine could see me when I come in for the treatment.

We continue to be very impressed by Dr. Rosen.  He anticipated many of our questions, and he assured us that he would continue tracking things until the mysteries involved are resolved.  It is equally clear that the doctors at Beth Israel operate as a team.  So I will cancel my appointment with the hematologist at Emerson/Mass General and concentrate the treatment at BI.

It would be nice to get an answer - 5/20/12

Barbie & I met with Dr Harold Rosen at Beth Israel Friday, 5/18.  He is an endocrinologist specializing in osteoporosis highly recommended by Bridget.  He spent over an hour with us, and lived up to his billing.  Unfortunately, at this point he has not yet figured out what is going on.  He was very thorough, probing for things like childhood illnesses that would have led to loss of bone mass.  But he noted that everything points the other way until February of this year, when the back pains suddenly appeared.  He was impressed that whatever-it-is suddenly appeared, and is still occurring, as the latest batch of fractures occurred when I was fully aware of the danger and doing virtually nothing.

Dr Rosen ordered a series of blood and urine tests, and set up two MRI sessions for Sunday morning.  One of the possible diagnoses is multiple myeloma.  But Dr Rosen said he would expect that there would be a strong spike in unusual blood proteins if that were happening.  The unusual blood proteins are present, but only slightly elevated.  So myeloma needs to be investigated, but Dr Rosen does not expect it to be the culprit.  I had a series of x-rays over most of the bones in the body to see if there were tell-tale bone density gaps.  Bridget knows the pathologist who read the x-rays, and she says no density gaps were found.

Dr Rosen was dismissive of Coumadin as a possible cause.  Heparin, which is another blood thinner that they give you when in the hospital for pulmonary embolisms, could be a cause, if I had been on it for any length of time.  But I have only been on it while in the hospital, probably 3 times.  There were other suspect drugs, most of which I had never heard of.

The definitive test for multiple myeloma is a bone marrow biopsy.  Dr Rosen does not have authority to do biopsies.  That is the realm of hematologists, and I have an appointment to see Dr Levine at BI on 5/30.  Dr Rosen will talk to Dr Levine, and convey a sense of urgency to him (which may result in the appointment being moved up).

Overall, given that we were expecting Dr Rosen to definitively declare what the cause of the bone fractures is, we were disappointed.  But we now realize that our expectations were unrealistic.  Clearly we have a good team of experts working on the case, and my case is not the first to present odd and contradictory symptoms.  They will get to the bottom of this and come up with a course of treatment.  Meanwhile I just have to be patient and move around as if I have a backbone of eggshells.

Yet another medical bulletin - 5/16/12

Yesterday was a pretty bad day.  I stayed in bed most of the day, as my upper back was really hurting.  In fact, I didn’t get dressed until about 5PM, and I blew off a church committee meeting in the evening.  As long as I was lying down, the upper back pain was not so bad.  Of course, I’m not sure how bad the raw pain would have been, as I took vicodin to address the pain.

Report from Dr Goller

In the morning we received a call from Dr Anca Goller, the endocrinologist at Emerson who ordered blood + urine tests.  The results were:

1.       My calcium + vitamin D levels are OK.  The volume of my urine (collected over a 24-hour period) is quite low, as I must not be drinking enough fluids.  As a result the concentration of everything is high, but adjusting for the lack of volume, the levels are OK.  [This puts Barbie in a nagging I-told-you-so position, but she may even be right.]

2.       Testosterone is normal.

3.       The test for Celiac disease is negative, but it does not rule out Celiac disease.

4.       My thyroid tests are slightly under normal.  That is good in that a hyper-active thyroid is one possible cause of osteoporosis, so thyroid activity is not causing my current problem.  The thyroid is not so low that it needs to be treated, but it should be monitored.

5.       The one thing that stands out as a problem is protein abnormalities in the blood.  Barbie, taking notes as usual, took this down as PEPSAS, but I cannot find any acronym of that name on the internet.  This needs to be followed up with a hematologist who also does oncology.  She has talked to a Dr DuBois at the Mass General – Emerson Cancer Center.  I mentioned that in 2006 a Dr Humberto Rossi did some blood work following an unusual blood test in an annual physical.  He ordered additional tests and found I had a monoclonal gammopathy.  His conclusion was that this was not a problem in my instance as some % of the people with this condition lead normal lives.  We need to get the tests that led to those conclusions and see if anything has changed since then.

6.       Creatine in urine was also high.  Dr. Goller did not say that this indicates anything.  (Does it mean I am a cretin?)

She was both faxing and mailing the results to Dr Rosen for my visit on Friday.  The protein in the blood could indicate something as bad as multiple myeloma, so it is not to be ignored.  We should keep her informed of what happens with Dr Rosen.

I registered with Mass General for access to Drs DuBois + Rossi.  I told the scheduling nurse that Dr Rossi had done blood work back in 2006 (or so).  She ordered the records of those tests from their off-site record storage location.  I now have an appointment to see Dr Rossi on 5/25.  Naturally, Mass General and Beth Israel have different computer systems, so they cannot share test results.

No call from Dr Muzin

Dr Muzin had said he would call if there were new results from the x-rays taken late Monday.  I called his office at the end of the day, but did not reach anybody.  How could I feel this bad without something new showing up?

Dr Quinn

We called Bridget after dinner, as things felt worse, not better.  She had access to the x-ray results from Monday.  It turns out that I have a compression fracture of the 7^th thoracic vertebra, a new fracture of the 5^th lumbar vertebra, and an increase in the fracture of the L3 vertebra.  It is odd that the pain is so concentrated in the upper back.  Clearly my bones are continuing to crack.  Looking back at the last few days, I cannot identify anything that would have caused the fractures.  I’ve been wearing the (hated) brace, and I have not done anything at all stressful or vigorous.  Getting in and out of cars, dental chairs, or x-ray platforms have been the most difficult things I’ve done.

Bridget will email Dr Rosen to inform him of the additional fractures and to see if there is any blood work he wants done in advance of my appointment on Friday.  I can go into a BIDMC location in Lexington for the blood tests, which is far more convenient than either Needham or Boston.

We will get the records of the 2006 blood work to take into the Friday appointment with Dr Rosen.  If nothing else, they should provide a baseline to compare current tests against.

We still don’t know what is causing all these bone fractures.  Clearly something is radically wrong.  We are getting to the specialists now, and they should be able to find the cause.  More to come…

Dr Muzin Questions + Answers - 5/15/12

1.       Do I still need to wear the back brace?  I dread it.  Actually wearing it for most of the day is not so bad, but by the end of day it usually hurts quite a bit.

a.       Would a smaller, elastic “girdle” type of support be sufficient to prevent damage and promote healing?

b.      At this point, is it “dangerous” to go without the brace, especially when it is causing considerable discomfort?

c.       Trade off – no brace, no vicodin; brace, vicodin?

Yes, wear the brace.  The bones need stabilization, and a smaller brace won’t do it.  The 3 lumbar vertebrae that I’ve broken are 1-3, and the numbering starts from the top.  [I think as a result of a Google search] the lumbar vertebrae extend up to where your lowest ribs attach to thoracic vertebrae, so you can’t stabilize them with just a lower back brace.  Yeah, it is a major pain, but healing is happening, and it should just be another 3-5 weeks.

2.       It feels as if the problem is migrating up in my torso.  The primary pain is now in my rib cage.  Could it be that my ribs are turning to mush as well?  Or is that the reaction of my breathing muscles to being constrained by the back brace?  Now in the evening it really hurts to cough.
Dr Muzin ordered a set of x-rays.  He will call if the x-rays reveal anything that is unusual or dictates a change of treatment.

3.       I am getting no exercise.  Are there exercises I can do while waiting for my bones to heal? – Weights with arms? Or those stretch bands?  Recumbent bike? Stationary bike? Eliptical (no impact)?
Hold off until the bones are healed.  Then he will recommend a course of physical therapy.

4.       I have given blood + urine under a program dictated by an endocrinologist.  Presumably the tests will shed light on why I have osteoporosis.  Do you have any insight as to why I am in this condition?  Does the MRI give any clues?  I am due to see Dr Rosen in at BIDMC this Friday.  Should anything that you have gathered be fed onto him?
[I neglected to ask this question.]

5.       My wife found Coumadin study that shows men who have been on Coumadin for more than a year have a 63% increase in the incidence of bone fracture.  http://www.drugs.com/news/warfarin-raises-bone-fracture-risk-1705.html.  Have you seen other men who have developed this condition while using this drug?  [Shouldn’t a bone density scan be recommended with the use of this drug?]
He was unaware of this study.

6.       I don’t know if it is an after-effect of the hip operation, but lying on my right side while sleeping does not last long.  At times my right foot also feels tingly.
He tested my legs and back for strength and responses.  All appear OK.

7.       What is the prognosis for (1)healing of the lumbar vertebrae

a.       Upper back/posture reversal

b.      Regain full function.  Time line?

He did say that PT can address the old lady’s hunch I now have.

8.       Do you recommend PT?  

I held off on this, as PT will come after the bones can handle some strain.

9.       Who should manage this type of “disease/injury” – you or the endocrinologist?
He handles the pain and manages the broken bones.  The endocrinologist figures out what caused the osteoporosis and fixes that.

I then went to have x-rays.  It was a major issue to get down flat on my back, as my backbone was protruding from my back, and lying flat on my back hurt.  We started with x-rays while I was lying on my left side, and then I could roll over onto my back.  You’d think a slightly soft surface to lie upon while getting x-rays would be a no-brainer.  By the time I got home, the upper back was in full revolt, so I shed the back brace, took a vicodin, and went to bed.

Medical Report 5-8-12

Yesterday morning  (5/7) I saw Dr Anca Goller, an endocrinologist recommended by my GP.  The GP had put me on Calcium Citrate with Vitamin D and an extra dose of straight Vitamin D, but said a specialist should look into why I have such mushy bones.  Dr Goller went to medical school in Romania and did her residency at UMass Medical in Worcester.  One decision was whether to keep the appointment with Dr Goller, as Bridget (our doctor daughter-in-law) had set up an appointment with an endocrinologist at Beth Israel who is very respected.  I chose to keep the appointment so that we could start the tests that might reveal the underlying cause of my osteoporosis.  I told Dr Goller that I have an appointment in two weeks to see Dr Harold Rosen at Beth Israel.  Small world: she did a rotation while at UMass Medical at Beth Israel and had worked with Dr Rosen.

Dr Goller did a very thorough inventory of symptoms, conditions, history, etc.  She set out a series of blood tests that will indicate whether any of the most likely causes are in play.  She also prescribed a 24-hour urine collection regimen so that they can tell how much calcium my body is getting rid of.  There is another series of tests that they use to monitor how well any course of action is working.  She would normally have had me do that, but she knew that the tests available at Beth Israel are better than the tests available at Emerson, so she felt it was better to have Dr Rosen prescribe those.

Barbie had found a 2007 study on the internet that looked for a connection between Coumadin and osteoporosis.  It is logical that there might be a connection because vitamin K is crucial in blood clotting, and Coumadin works by interfering with vitamin K’s role in clot formation.  Similarly, vitamin K is key in bone building.  If vitamin K’s role is compromised, bone formation might also be affected.  The St Louis University study examined the medical records of 15,000 people who had been on Coumadin for more than a year.  The vast majority were women, and there was no correlation.  However, in the male subset, there was a 63% increase in bone fracture.  We asked Dr Goller about the study, and I can’t remember what she said.

Dr Goller set out a 5-point plan of attack:

1.       Blood diagnostic work.

2.       Urine collection + analysis

3.       Weight bearing exercise.  Walk.  No high impact or vibration, heavy lifting, ladders, etc.

4.       Calcium and Vitamin D.  Protein helps.

5.       Avoid falling.  Use a shower that has grab bars or a seat, so that you are not in danger of slipping on a wet surface.  Clean up things on the floor that can interfere with placement of the canes I walk with.  Turn on lights when getting up in the middle of the night for a bathroom run.

After seeing Dr Goller, I picked up the images from my bone density scan, as Dr Rosen had requested them.

That afternoon I saw Dr David Green, the pulmonologist who treated my 2 pulmonary emboli and who put me on Coumadin.  He reviewed the printout of the St Louis study, but I couldn’t tell if he had already seen it and whether it affected his opinion about prescribing it.  He had access to the medical records.  My first embolism was in 1998 and the second was in 2004.  Both occurred right after long plane flights during which I did not move about much.  Various blood tests were done in 2004 to determine if there were any cause other than inactivity during a long flight to suspect.  There were none.  So we concluded that medicating specifically for plane flights makes sense.  That can be done with an injection of Lovanox.  In fact, immediately after my hip surgery in December, 2010, I recall getting Lovanox in the stomach.  I believe I came home with a few syringes of Lovanox, and I self-administered some of the doses.  So in the future I am to inject Lovanox 1 hour before any long plane flight, wear compression socks, and move about on a regular basis.  Given all that, I can come off Coumadin!

There is an alternative to Coumadin that is available now.  I did not get the name.  It operates in a completely different way than Coumadin, and the dosage is very standard.  You just take a pill; there is no need for continual monitoring, as there is with Coumadin.  However, unlike Coumadin, there is nothing that can reverse the anti-clotting effects of the drug.  So, should you have an incident, such as a car accident, where you are bleeding profusely, there is nothing they can do to enhance your ability to clot.  They are working on drugs to reverse it, but none is available yet.  So Dr Green does not choose to prescribe it yet.  I had the feeling that if there were a way to reverse it, that Dr Green would have put me on that.  I am happier taking Lovanox only when flying, as presumably there are fewer side effects (assuming I am not gallivanting all over the globe).

Today I got all the way to Emerson Hospital to do the blood tests, when I realized I had left the list of tests Dr Goller gave me at home.  Mistakes still happen.

Catching Up

I have been distributing detailed documents to friends via email, as I have been going through the steps to discover what was going on with me.  Keeping the email list up to date proved to be impossible, and we have been impressed by other people's blogs, so I am switching technology.  I will publish the individual documents as individual blog posts.  Note the publish date is well after the original composition date of the document.  I will try to make the composition date clear.

Tmeline of Events through 6/14

12/14/10  Bicycle accident in Jersey City where I broke the head of my right femur.  I.e., broke my right hip.
12/16/10  Pin & Repair surgery on the hip at Beth Israel Deaconess Medical Center in Boston.
November, 2011  Resume running & biking.  Running quite slowly, but gradually making a comeback.
2/19/12    Brush burning resulted in a sore back.  Refrained from running for a week, then resumed.  Seemed OK.
3/10/12    Brush burning #2.  Developed very sore back during the day.  Unable to complete the work, as I just had to sit down to relieve the pain.  Never had a major trauma, just normal hauling, bending, and shoving.
3/12 - 3/23  Visits to Chiropractor Greg Symko.  Ultrasound, electrical stimulation treatments.  No effect.  Terrible pain getting down on or up from the treatment platform.  Ended up taking treatments virtually standing up.
3/19  Symko sent me to Emerson for an x-ray of the lower back.
3/21  Symko says x-rays reveal 2 compression fractures of vertebrae.  His treatments will not affect them.
3/27  Visit Back Doctor Stefan Muzin.  General diagnosis.  Orders MRI for further diagnosis.
3/29  MRI confirms 2 compression fractures, some rheumatic wear on vertebrae.  Muzin prescribes back brace to stabilize the bones + Vicodin to deal with pain.
4/2    Bone Density Scan.  Ordered by Dr. Kafina's office, but requested by Bridget (daughter-in-law).
4/4    Fitted for Back Brace
4/5    Pick up Back Brace
4/6 - 4/22    Nepal.  Quite painful, as roads are 60% bone-jarring potholes.  Ended up standing on steps of bus where the door opened in, with bent knees to absorb the shocks of the potholes.  Refrained from many activities, and used 2 walking sticks to move around.  Clearly sustained additional damage.
4/23   Dr Muzin, x-ray.  Quite hard to get into position for the x-ray.  X-ray reveals 1 new fracture, 1 of  existing fractures made worse.
4/26   Dr Kafina (primary care physician).  Bone density scan indicates osteoporosis.  The definition of osteoporosis is 2.5 standard deviations below normal density.  I am 2.7 standard deviations below.  Refers me to endocrinologist Dr. Anca Goller.  Prescribes Calcium Citrate + Vitamin D, plus an extra dose of Vitamin D.
5/7    Dr Anca Goller did a thorough inventory of symptoms, history.  I am now 2 1/2 inches shorter than originally.  She ordered a full array of blood tests to identify the cause of the bone problems.  Informed her of appointment to see Dr Rosen at BIDMC.  She knows Dr Rosen, as she did a residency round with him.
5/7   Dr David Green agreed to allow me to get off coumadin.  Barbie had found a 2007 study that shows males taking coumadin have a 63% increase in bone fractures.  Generally the doctors dismiss that study, as coumadin is very widely used and considered to be understood.  Dr Green prescribed lovanox to be used whenever I go on long plane flights, to be administered by self-injection.
5/9    Report from Dr Goller.  No smoking gun in the blood tests.  Yes, a high level of a protein associated with Multiple Myeloma, but not as high as one would expect for a disease that would cause my bones to break.  She sent the results to Dr. Rosen.
5/14   Dr Muzin again.  Pain has migrated to upper back.  Orders another x-ray.  Result of x-ray is a new compression fracture in the thoracic spine.
5/18   Dr Rosen.  Very thorough exam, ordered yet more blood tests.  Said he would speak with Dr Levine, and expedite the appointment with him.  Ordered MRI of both upper and lower back.
5/20    MRI reveals yet more fractures.  1 new one in the lumbar spine (total now 4) and 4 new ones in the thoracic spine (total now 5), for a total of 9 fractures.  No wonder I was hurting.  And I have been doing NOTHING.
5/24    Call from Dr Rosen.  Still no definitive diagnosis, but a treatment.  Reclast, which helps build bone.  Same treatment for normal osteoporosis and multiple myeloma (MM), just MM indicates a much more frequent dose.
5/25    Infusion #1 of Reclast.  Meet with Dr Levine, who also probed background, symptoms.  Dr Levine performed a bone marrow biopsy, the test to determine MM or not.  Went to NOPCO to get back brace adjusted, as it was really painful.
6/1    Dr Levine delivered the diagnosis: MM.  Normal levels of plasma cells in bone marrow is 2%.  Less than 10% is considered within range.  I have 50%, which is a positive indication of MM.  50% is bad, but he has seen as high as 95%, so my disease is in an early stage.
6/5   Start treatment.  Treatment plan is to receive 4-6 cycles of chemo-therapy to knock down the MM.  Then to harvest my plasma stem cells and freeze them.  Then they will admit me to the hospital, attack the bone marrow with aggressive chemicals, totally wiping out my immune system.  Then (when I am on death's door) they transplant my own stem cells, which should grow into a replacement bone marrow system without MM.  Finally, you are on a maintenance dose of the chemo-therapy to prevent any recurrence of MM.  The actual treatment went without any major effect until 11PM, when nausea kicked in.  Also not sleepy, so got up and wrote an email to the people on the Nepal trip.  Subsequent treatments produce less nausea, as I took antacids and ate less.  The wakefulness did persist, which is totally unlike me.
6/11  Second opinion from Dr Laubach at Dana Farber.  Generally upbeat, as MM is now a very treatable form of cancer.  He recommends a 3-drug cocktail to knock down the MM.  Dr Levine has prescribed a 2-drug cocktail.  Dr Laubach mentioned a couple of clynical trials I could participate in, if I so choose.  He will confer with Dr Levine about the 2-vs-3 drug prescription.
6/15  We are scheduled to meet with Dr Levine and get the last treatment of the first cycle.  Then we plan to retreat to NH.
Summary: Bones broke unexpectedly early in March.  Took until the start of June to diagnose MM.  Now on a treatment plan that has good prospects of achieving complete remission.  It won't be easy, and there is disagreement on the specifics of the initial treatment, but the prognosis is good.