Final Post

One month ago, on November 30th, 2011 my beloved wife Jackie sucummbed to her illness. She was approximately 5.5 years post transplant. She was 53 years old at the time of her death.

From the time of the previous post until her death she faced a huge number of challenges due to advancing graft-versus-host (GvHD) disease. At the time of her death she was receiving weekly photopheresis treatments at the Mayo Clinic. She had become highly susceptible to pseudomonas infections due to the open wounds from the GvHD. She was hospitalized a number of times for sepsis requiring IV antibiotics.

In the end she died of probable sepsis from an infection, dehydration due to leakage of fluids from her GvHD wounds, and mostly from a lack of energy and will to continue a valiant fight. Not one person in one thousand could have fought the fight she fought. She leaves behind myself, and three children. She will be missed by all those who ever knew her.

It is my great hope that medical advances will soon make this terrible disease a thing of the past. To this end, I wish to thank Dr. Clive Zent of the Mayo Clinic for his faithful service over the past 7 years.

Rest in Peace Jackie Sue Taylor

A Kinder, Gentler Soul Never Walked This Earth.

Signed,

Your loving husband of 32 years

Bruce M. Taylor

Three and one half year update (Day +1281)

Someone asked why I always list the number of days before and after transplant on the blog. It comes from the Vietnam era when men fighting in battle counted down the days until they could go home. At least that is the way this started. We counted days until the battle began prior to transplant. Now we count up the days from victory.

Jackie is doing reasonably well. I'm happy to report she has conquered the Shingles. Of all the things that took place in this long process Shingles was the most painful. It is not something you would wish on anyone! She still has some minor twinges but it seems to be mostly behind us.

We were at the Mayo a week ago for her monthly visit. While there we met with the usual battery of doctors. She also had her monthly photopheresis treatments (2 days, 2 treatments). These have become almost routine. They seem to be helping the GvHD but it is hard to know for certain.

It seems we have finally settled on a dosage for prednisone. She takes 50 mg on one day and 10 mg on the following day. Anything less than this causes her GvHD to flare. The docs would like to get her to a lower dose but it doesn't seem possible. The high steroid intake is causing her to suffer from osteoporosis. The steroids cause a drop in calcium and a decrease in bone density and an increase in her alkaline phosphatase levels. To counter this she takes vitamin D along with big doses of calcium. That still isn't doing the trick. They believe the GvHD in her gut doesn't allow for effective absorption so they will begin giving her IV calcium ever 3-6 months.

The biggest problems come from the skin cancers. Each month it seems that a new one pops up. All have been on her arms and hands. Each month she requires a biopsy or excision. This month she had two skin punch biopsies on her left arm and an excision of a previous biopsy on her right arm. The excision was about the size of a silver dollar coin. Because the layers of skin on her arms are so damaged from the GvHD it was not possible to close the wound. It simply would not pull together. Therefore, the docs were forced to leave it as an open wound to heal. This lengthens the healing time from days and weeks to months and leaves open the possibility of infection at the wound site.

The good news is that a lot of the sclerodermoid GvHD issues seem to be improving. The skin tightness continues but seems improved. The "plastic" patches have also improved. Perhaps this is due to the photopheresis.

The docs continue to tell us they expect the GvHD to eventually "burn out". There are very few patients in the world who are 5+ years out of mini allo transplant. The best data seems to come from Germany and suggests that after about 5 years the body learns to cope with the new stem cells and GvHD improves.

So, with this in mind....I'm wondering if it is time to begin counting DOWN again!

Until Later....

Bruce & Jackie from Pryor

Three Years and About 3 Months (+1207)

It has been about 3 months since the last update. I don't do these very often because I doubt anyone reads them any more. But just in case there is someone out there still following I'll continue to update from time-to-time.

Over the past three months Jackie has continued to have a lot of ups and downs. Most of the problems have come from her GvHD. It continues to attack her skin and gut. To combat the problem she takes two-sessions-once-per-month of photopheresis at the Mayo Clinic. This seems to have helped her scleroderma symptoms with minor benefit to her GvHD skin issues. Her skin has become thinner and some of the splotchy patches have improved. It remains to be seen how long she will need to continue these treatments. She continues to have problems with small skin cancers and warts due to her weakened immune system. It seems that each month she needs some type of minor surgery to deal with these problems. It is amazing how fast something as simple as a wart will grow without a healthy immune system to keep it in check. Minor problems can become major problems in as little as one month.

Since the last update she was hospitalized for about one week due to a leakage from her stomach/gut into her bloodstream. This allowed e coli to pass into her bloodstream making her quite ill. Fortunately IV antibiotics put her on the road to recovery within about 24 hours. We were lucky we were able to get her to the doctor at the Mayo in short order. Within minutes of seeing her he diagnosed the problem and had her the antibiotics within the hour. I really think had we waited another 6-8 hours and the outcome might not have been good.

The latest hammer to drop has been a recent problem with shingles (Herpes zoster). About 6 weeks ago she was having an outbreak of GvHD. Without warning she began experiencing excruciating and debilitating pain. Of all the issues she has dealt with the shingles episode has been the most painful! The problem is slowly improving but there is still a lot of pain. It is not something to be taken lightly. The standard pain meds of Oxycontin and Oxycodone have little or no effect on the pain.

There continues to be no sign of the original disease; CLL. Her blood counts continue to be completely normal. Hopefully she will be able to decrease the steroid and anti rejection meds and still keep her GvHD under control. The big goal now is to get her on the smallest possible doses of immunosuppressive agents. The doctors say that over a period of five years GvHD usually improves naturally. At 3+ years post transplant we certainly this to be true. It continues to be a long road and a difficult journey.

Until Later,

Bruce and Jackie

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!)