Three Year Anniversary (Easter Sunday +1095)

Today is Easter Sunday and it has been exactly three years since Jackie Sue's transplant.  Three short years ago the doctors at the Mayo Clinic hung a small bag of red cells on a stainless steel pole and our transplant journey began.  Jackie's blood counts were at ZERO and her life hung in the balance! 

I haven't updated the blog in a long time as I didn't know how many (if any) were still reading about her progress.  Somehow I couldn't not update at such an important time and date.

There have been a lot of ups and downs in the last few years.  The first year after transplant was probably the best.  She was weak but continued to see steady progress.  She didn't have any significant GvHD issues and life seemed to be good.  She lost 100 pounds through the transplant process and looked and felt like a different person. 

Shortly after the first year anniversary she began to have issues with graft-versus-host disease.  At first the GvHD was an annoyance.  Over time it has become as big a problem as the original disease.  As you may have read in previous posts she has experienced severe scleroderma-type effects.  Over the last six months these have subsided but she has some damage and scaring from the process.  The progression was stopped by high dose steroids (prednisone) and ECP (extra corporal photopheresis) treatments.  She is on a 50-10 schedule of prednisone and and a two-treatments-once-per-month ECP schedule.  

As part of the GvHD she has experienced skin lesions that are matched by lesions in her stomach and gut.  As a result of the GI involvement she has experienced leakage of bacteria into her bloodstream.  In the first case it was identified as e coli.  In the current instance (just last week) the cause was not identified.  Both required hospital stays and IV antibiotics followed by oral antibiotics.  Recently we have spent a great deal of time at the Mayo Clinic dealing with these issues.  The good news is the ECP treatments seem to be helping.  Her skin and stomach lesions have improved and have allowed her to decrease steroid dosages from 50 mg/day to the current 50-10 dosage level.   As a result of the steroids she has experienced a significant level of steroid side effects.   She has puffiness of face, the classic "buffalo hump" at the top of her spine, and a redistribution of fat from hips and thighs along with weight gain.

In addition she has faced a number of small skin cancer problems.  In one case it required a skin graft and Moh's surgery to eliminate a cancer that grew on the top of her hand.  She has had a number of small cancers burned off.  Each month there is always new biopsy or cancer to be dealt with by her docs at the Mayo.  Naturally these open wounds are sources of infection and a constant source of worry for an immunosuppressed patient.

The good news is that she is completely free of the original CLL.  There are no hints that it will ever return.  In almost all respects her blood counts are entirely normal.  A standard CBC never shows anything "out of range".  This, of course, is a relief.  The docs also tell us that given her level of GvHD it would be highly unlikely to see a relapse.

Finally the docs tell us that GvHD tends to get better with time.  The "magic" time period seems to be 5 years.  For whatever reason most people tend to improve with time.  Their bodies learn to deal with the new, foreign stem cell invaders.   In many cases the immunosuppression can be reduced to much lower levels and life becomes more normal.  So...I hope to update this blog on the 5 year anniversary and report that things are dramatically improved and the GvHD we deal with is under control.

So, that's it for now.  Have a Happy Easter!

Bruce & Jackie

From Pryor

Jackie's Surgery (Day +850)

We arrived yesterday about noon.  We transported the father of the lady who is the marketing manager for the building we stay in to Rochester as well.  He lives in Wagoner and came along for the ride so he could visit his daughter.

We went to Jackie's appointment at about noon today.  She was scheduled for 12:45 PM and they told us to arrive at least 30 minutes early.  In the end it didn't matter as they were an hour late getting her in for surgery.  She went in just before 2:00 PM.  They finished at about 5:30.

It took two attempts to get the entire skin cancer.  This is normal.  Many of the people in the waiting room required at least three attempts.  The surgeon takes as much tissue as he believes necessary to get the cancer.  They then stain slides and have the lab examine them to see if they have a "clean edge".  If not, they then take more tissue and re-examine to see the result.  In Jackie's case they had to go really deep to get the entire growth.  It exposed the tendons but it was not necessary to remove them.  Naturally this is very good news.  She should not lose any use of her hand.  If we had waited another month for the surgery I think the result would have been very different.  

Unfortunately the wound was large enough that a skin graft was required.  They took the donor skin from her left upper chest.  We return in the morning for an exam.  We return again on August 25th for further examination and removal of the stitches and a consultation with the surgeon.  All-in-all the result was about as good as could be expected.  

She is currently asleep.  The surgery was conducted under a local anesthetic only but they gave her some type of sedative that made her sleepy.

Sitting in the waiting room (which was VERY busy) it make you a firm believer in suntan lotion!  Most (all?) of the people in the room were there as a result of exposure to the sun.  I'll certainly use more lotion in the future.

Until Later....

Bruce and Jackie

From Rochester

Rochester in the Summertime (Day +844)

We arrived back in Rochester this morning.  We were supposed to arrive last night, but mechanical difficulties delayed our departure until very early this morning.  The weather was great and we overflew Canadian airspace and the Great Lakes on the way over.  The new Mayo Clinic Plaza has been finished and they have a lot of street vendors selling and displaying everything from jewelry to homemade pies.  It lends a carnival atmosphere to downtown.  Summer  in Rochester certainly beats winter in Rochester!

This morning Jackie met with Dr. Zent.  He is very pleased with her progress.  I also learned a very important fact this morning.  Jackie does not have Scleroderma!  She has chronic graft-versus-host-disease (GvHD).  Of course we already knew this, so why the confusion?  It seems Scleroderma is the closest disease they can use to describe her situation.  They don't really have another name to describe GvHD/Scleroderma.  I'm thinking of coming up with with a fancy-sounding Latin name and giving Jackie a spot in history.  How about.....Jackiecilius Epidermatosis.  Maybe we can abbreviate it to JED ?  Latin scholars are probably having heart attacks as they read my new name :) Why is a separate name necessary?

A separate name is needed because there are some serious differences between Jakiecilius and Scleroderma.  Scleroderma is a disease that responds poorly to most treatments.  As it progresses it often invades heart, lung, esophageal, gastrointestinal,  and kidney tissues.  While, this is certainly possible with JED, it is far less likely.  It is also possible to reverse Jackie's disease by increasing her immunosuppression.  So, why don't they do that?  Because it is a balancing act.  The immunosuppression can also lead to problems.  The good news is that chronic GvHD tends to improve over the years.  The body learns to live with the new immune system and immunosuppression can be lessened.  The primary problem with JED is the potential for skin scarring.  Like Sclerodera, it can cause changes to the skin that are irreversible.  This is primarily limited to skin thickening and contractures which are limits on joint flexion and tension.  

Jackie also had completely normal blood tests.  Her hemoglobin was in the mid-12s.  Her platelets were 260 (remember when they were 2!).  Her lymphocytes were slightly elevated at 3.6 but remain stable from last September.  All-in-all the counts are completely unremarkable. 

The primary concern at this point is the skin cancer on her hand.  She is currently scheduled for surgery in early September.  Tomorrow we see the dermatologist.  Dr. Zent believes they may want to schedule surgery sooner.  The growth has been quite rapid.  We will know more tomorrow.  The surgery will be a Mohs surgery which is a highly precise surgery conducted using a microscope.  The surgery will likely last all day due to the fact they will excise small samples of tissue, stain, and examine under a microscope before making another incision.  They will also need to do some reconstructive surgery due to the size.  The actual lesion is approximately the size of a quarter and protrudes approximately .25" above the surface.  Skin cancers are a common problem after transplants due to the amount of immunosuppression.  

That's all for now.

Until later

Bruce and Jackie

From Rochester

Visit to Mayo and Chemo Results (Day +823)

We returned to the Mayo Clinic about a week ago.  While there Jackie had her last infusion of Rituxan.  They also performed a biopsy on a spot on her hand.   In addition they increased her Cyclosporine and CellCept in order to better get the scleroderma under control.    There were discussion as to whether to begin UVA-1 light treatments immediately.  A decision was made to wait one month to see if she improves.

I'm happy to report that she has seen some improvement in the scleroderma.  Her skin is less "tight" and the opens sores and lesions have subsided.  She is still a long way from normal but there are signs of improvement.  We have no way of knowing if it is due to the chemo or the increase in immunosuppression.  In either case we will gladly take the improvement!

The docs did note an increase in her lymphocyte counts.  They felt this was due to reactive T-cells and planned to perform a flow cytometry test to make certain this is the case.  Her other blood counts continue to be very normal and still improving.

The results from her hand biopsy confirmed that she has a squamous cell carcinoma.  This will be removed during our September Mayo visit.

Jackie is feeling well enough to return to Nantucket.  We plan to leave on Thursday of this week and hope to stay until her next visit in early August to the Mayo Clinic.

So, all-in-all things have improved somewhat since the last report.  She still requires several pain pills each day to get through the day.  The pain seems to be subsiding, however and we hope the pills won't be needed much longer.

Until next time.....

Bruce and Jackie

from Pryor (but not for long!)

More Chemo (Day +791)

Jackie's scleroderma continues to progress.  It is still very painful.  Fortunately her local doc is very understanding with regard to her pain meds and she is now getting enough to help make her comfortable.

After consulting with the Mayo docs it was decided to try a course of Rituxamab (Rituxan) chemotherapy.  Some of you may recall Jackie received about 10-12 doses of Rituxan to help get her platelets back under control prior to transplant and to help condition her for the transplant.  Now we will try to reverse the process; but only partially.

She took her first course of treatment on Thursday of this week.  As some of you may recall Rituxan is created using cells from mice.  It is often referred to as "mouse juice" around the hospital.  As a result of the way it is created it often causes a severe antibody reaction when first administered.  It is a  peanut allergy type of reaction.  This was the case when Jackie received her first treatment.  Her blood pressure dropped, heart rate went through the roof, etc.  In some cases the people die from shock.  It is a very dangerous thing to do the first time.  One the body begins to recognize the mouse cells it learns to get along with them and subsequent transfusions are usually a non-event.  That was the case with Jackie.

In this case nobody could tell me if the antibodies still existed in her body.  Did the stem cell transplant kill them?  It should have!  She has a completely new immune system.  Well....it didn't!  The transfusion was a non-event.  Not even a blip on the blood pressure gauge.  So for anybody who would ever happen to follow us on this crazy journey you now have the answer.  Rituxan antibodies survive the transplant.

Jackie will get four transfusions over four weeks.  As best I can tell there is one clinical study in existence for this situation.  It consisted of three people.  All three saw improvement.  One person saw minor improvement, one fair improvement, and one dramatic improvement.  This was over the course of one year.  

The disease has progressed very rapidly.  In the course of a few months it has affected most of the skin on her body.  Her arms, legs, thighs, back, are all involved.  It is beginning to show on her face and scalp.  She may also have some organ involvement as she seems to have some nausea.  She will have to be "scoped" to see if there is any GI involvement.  She is beginning to have problems with stiffening joints and walking.   We can only hope the Rituxan begins to reverse the process soon.

Should the Rituxan not work she could try long-wave ECP (extracorporeal photopheresis).  ECP would require a 6 week (minimum) stay at the Mayo Clinic as they are only one of 5 sites in the world that can conduct the therapy.   We will know much more when we return to the Mayo on July 7th.  For more info on regular ECP click HERE

I should take the time to once again remind EVERYONE that Jackie is highly immunosuppressed.  The Rituxan will make things much worse in this regard.  She is highly susceptible to disease and infection and will have no way to fight this off when the Rituxan kicks in.  DO NOT come around if you are sick or if you've been around anyone that has been sick!  Do not be offended if I ask to leave our presence, refuse to shake your hand, or give you a hug while this takes place.  We simply cannot take any chances while she is so suppressed!

Hopefully the next post will be more positive.  

Bruce and Jackie

From Nantucket

 

Back in ACK (Day +779)

We are back in Nantucket (ACK, is the airport designator; like DFW or LAX).  We flew up yesterday.

We were back at the Mayo about a week ago for Jackie's monthly checkup.  Her blood counts are all within normal ranges.  There is no sign whatsoever of CLL.  That is the good news.  As I have done all along I've shared the good news with the bad.

The bad news is that her GvHD continues to progress.  It has turned into scleroderma.  You can learn more about the disease here.  In a nutshell it is a disease with no cure.  The disease causes problems with the connective tissues within the skin due to excess collagen production.  The skin thickens and feels like plastic.  The sweat glands and hair follicles are choked off.

In Jackie's case the change has occurred very rapidly.  The process began some months ago as the GvHD attacked her arms first, then her legs.  The scleroderma is rapidly advancing and is beginning to attack her upper legs and thighs as well as her back.  It is extremely painful.  It is limiting her mobility.  She has difficulty tying her shoes, walking, and climbing stairs.  Wearing clothes is painful.  She says it "feels like she is wearing a suit of armor and if she bends her arms or legs they will burst open".  There is also a great deal of itching.  She slathers herself in various creams and ointments including hydrocortisone cream; all with little or no benefit.  She soldiers on but it is not a pleasant time for her.  Due to the over prescribing of pain medications the doctors are hesitant to supply here with something to relieve her pain.  I'm certain if they had to live one hour in her condition their views would be quite different!  On Monday we will contact her doctor and beg for pain meds for relief. Tonight she took another one of the precious few tablets she has left.  

The doctors have no idea of what will happen with regard to this problem.  There is just too little data.  They are not very optimistic about an improvement in the condition.  It is possible that Jackie's hybrid immune system will eventually adjust and things will improve or return to a more normal state.  It is also possible the scleroderma may progress further; only time will tell.  I have to admit this is one possibility that I (we) never considered when we began the transplant.  I suppose it beats the alternative of dealing with CLL.  Almost certainly Jackie would not be alive today had the stem cell transplant not been performed.  Hopefully this is just another bump in a very long road.  The doctors tell us that over time GvHD has a tendency to subside and improve.  Hopefully this is true of side effects such as scleroderma as well.

I assume that anyone still following this blog will be familiar with PC and Chaya Venkrat's CLLtopics site.  Chaya's husband PC has recently undergone a double cord blood stem cell transplant.  PC's progress is being blogged as "Harvey's Journal".  It will provide some insight into the similarities and differences between a mini-allo transplant (like Jackie's procedure) and PC's double cord blood transplant.

Until Later...

Bruce and Jackie

From ACK

Clink Update (Day +747)

Jackie was discharged on Monday.  We are headed for home today (Tuesday).  I can tell she will be "down" on her non-Prednisone days.  They have changed her Prednisone dosage to a total of 30 mg every other day.  On her "off" days her body will attempt to make natural steroids.  At least this is the hope.  On the off days we will probably see her GvHD flare as well.  They tell us it will be at least a couple of weeks before the CelCept will kick in and begin to make a difference.  Until then she will continue with the moist (wet!) pajama wraps.  The warmer climate of OK will probably make this a little easier than MN.  

Until Later...

Bruce and Jackie

From Rochester (but for only about another 30 minutes!)

Clink Update (Day +745)

The full-body moisture wraps have dramatically improved the GvHD rash on Jackie's arms and legs.  They are still red, itchy, and somewhat inflamed, but they don't burn and cause constant pain.  She has gone from a 24 hour per day full wrap schedule to a 3-a-day schedule.  The newest routine consists of completely covering herself in hydrocortisone (Triam) cream and then getting into wet cotton pajamas.  She then puts on a sweatshirt and sweatpants.  She keeps this on for 90 minutes.  This routine will continue on a taper-off schedule over the next several weeks while the CelCept begins to kick in.

On our first day here at the Mayo they performed a skin biopsy  on her arm.  This biopsy turned out to be a small pre-cancerous spot.  We are still waiting for the full pathology report to see if it will be left alone or if a small area will need to be burned off.  The docs don't seem terribly concerned.

We hope to be sprung from the hospital tomorrow.  Since we don't know the exact time we plan to stay overnight on Monday for a departure home on Tuesday.  We'll both be glad to a warmer climate.  It snowed here on Friday night and during the day on Saturday.  While we got only a dusting in Rochester, over a foot of snow fell in northern MN.  And these people think it is Spring???

Until Later....

Bruce and Jackie

From Rochester

Back in the Clink

Jackie is back in the hospital.  Nothing serious!  Her graft versus host disease (GvHD) gradually became worse.   It finally got to the point it was very painful.  We flew up for our usual 3 month checkup and the docs felt the best course of action was to get her into a hospital environment to treat the painful skin rash.  The rash had gotten to the point of causing blistering.  Due to the inflammation Jackie was also running a low-grade fever much of the time.  

The good news is that her blood counts are normal in all respects and there is no sign of CLL.  This is likely a thing of the past.  After two years with no relapse she has a very good statistical chance of being cured of her original disease.  Of course the GvHD is no fun and needs to be taken seriously.  The docs tell us they see the average patient needing three years of immunosuppression after transplant.  They tell us that most patients with chronic GvHD tend to see gradual improvement.  We certainly hope this is the case with Jackie.  At this point there seems to be no involvement of GvHD with other organs.  They performed a pulmonary function test this morning.  We don't have results, but I don't think anything of concern will be found.  

They are changing Jackie's meds.  She will continue with Prednisone at a slightly reduced level.  She will alternate days on and days off.  One day she will take 30 mg the next day she will take nothing.  They hope to get her body to produce more natural steroids.  In addition they will be decreasing her Cyclosporine level to 50 mg per day and replacing this immunosuppressant with CellCept.  CellCept is newer and far more expensive.  They believe it will work better.  If this doesn't work there are several other treatments that are possible.  It is possible they can treat the GvHD with some of the same chemo agent they used prior to transplant (Rituxan and Pentostatin).  They also have a UV light treatment that is only available at the Mayo Clinic (UVA 1B).  Finally they have something called Extracorporeal Photochemotherapy (ECP).  This involves removing your blood and passing it through an apheresis machine and shinning a UV light on the cells as they pass by.  It somehow improves GvHD response.  I'll have to research the issue further if this becomes an option to better understand how it works.

In any case, Jackie is doing well.  Hot oil baths with full body wrap moisturizing treatments sounds like a spa treatment to me!  I asked the nurse when they had scheduled the Asian hot stone message:)

Until Later...

Bruce and Jackie

From The Mayo Clinic

Two Years And A Day (+731)

Well, it has been two years since the transplant.  It seems like it was a thousand years ago.  We have had a lot of ups and downs along the way.  Initially we dealt with the fear of the disease and then the fear of the treatment and the cure.  In the beginning it seems we dealt with nothing but fear.  From the beginning I've covered the good as well as the bad parts of the ordeal.  That is still the case today.

We still deal with some fear.  Jackie is still having problems with GvHD.  The fear is that it will become worse.  She has progressed from a simple irritating skin rash to Stage II GvHD with painful lesions on her arms and legs.  The increase in GvHD has taken place over the preceding six months; most in the past three months.  We thought we had escaped any major transplant problems but it seems there are still some challenges before us.  

To keep the disease at bay she is on a 20 - 25 mg of Prednisone regimen.   One day she takes 25 mg and the next day she takes 20 mg.  This is supplemented by a 100 (temporarily 200) mg per day intake of Cyclosporine.  Over the last few days we have increased this to 200 mg to try and get the disease further under control.  It is too early to say how effective the doubling of the Cyclosporine will be.  One year ago she was taking only 5 mg of Prednisone every other day and 50 mg of Cyclosporine.  As her new immune system kicks into high gear problems with GvHD pop up.  The good news is that even with the compromised immune system she has been able to fight off (rather quickly) several bouts of common colds and a minor case of flu.  I'll bet if she gets back to a minimal amount of suppression her new immune system will stomp out any germ or bug that comes along in record time.

Finally she is taking PUVA light treatments.  This consists of taking Psoralen which makes the body more receptive to UV long-wave ultraviolet radiation.  A pill is taken and then you spend a few minutes in a special tanning booth.  The treatment is often used to treat psoriasis.  It has been used with some effectiveness against GvHD.  It is difficult to tell if the PUVA treatment is helping.  Her skin is very raw and seems to be sunburned.  All-in-all it is a fairly painful experience.  The Cyclosporine causes her feet to be numb and she experiences muscle spasms.  The Prednisone has caused weight gain and she is experiencing the "moon" face that is often seen with steroid usage.  Through the initial transplant she lost 100 pounds.  She has regained about half that weight.  All this in spite of the fact she doesn't eat a great deal and gets a lot of exercise.  Again, the Prednisone is the culprit.  

The doctors tell us the GvHD should improve.  They warn it will take a lot of time.  They talk about years.   We return to the Mayo in about a week.  We will see what, if any, changes will be made in the treatment plan.

In spite of the somewhat negative report Jackie has no signs of CLL.  I believe it is a thing of the past.  In spite of the pain she is living a more or less normal life.  She isn't able to do as many things as she would like and she does have a lot of frequent pain that is controlled by use of standard pain medications.   She is able to work part time.  I know this is a contradiction; living in pain - but living a fairly normal life; but this is the best way I can describe her situation.  Post transplant life certainly beats pre transplant life.  I remind her the GvHD is also providing GvL effect.

So how normal is life?  What can you expect if you decide on the mini BMT?  Tonight she is in the kitchen making Cakelettes for an upcoming food show next weekend.  That is NOT something she would have been doing 3 months prior to transplant!  So...as bad as things sometimes seem it beats the alternative.  This is just another bump in a very long road.

Until Later...

Bruce and Jackie

From Pryor