Stories 2009

2009 starts here

Judith’s Story

Hi, my name is Judith.

I recently contacted Betty about starting a message board where those with CAD can communicate in a group forum. So, The CAD Chatter group was born.

I am a 45 year old mother of two. I have been married 23 years to a great man, Brian. We have two grown children, and live outside the Fort Worth, Tx area.

I was officially diagnosed with CAD in 1998. After reviewing my health records, symptoms go back to 1983. However, it was in the fall of 1998, when I got strep throat, that things really kicked into gear. The prior year I was told that my thyroid was shot and that I had mono. I had already received a fibromyalgia diagnosis five years before that. Okay, so it would seem that my immune system ain’t so great!

My official diagnosis was followed by two weeks in two different hospitals and a stroke, which messed up my vision for several months, and my entire left side is numb to this day. I have had 3 or 4 other “small” stroke events but their effects were minimal. Although my blood level was down to a 4.7 at the time of my stroke, I was not transfused as my bone marrow started to do its job.

I used to pay closer attention to the numbers, but really don’t anymore. I pretty much go by how I feel. I do have a daily medicine regime which addresses thyroid, fibromyalgia, peripheral neuropathy, Raynaud’s and opthalmic migraines.

Other than the CAD, I try to enjoy life despite the limitations. I live in A/C country, and it is hard to go anywhere without getting
cold. I generally try to escape every year to somewhere tropical.

I was so happy to have finally see a website devoted to CAD. When I was first diagnosed there was nothing. I look forward to getting to know you all and hearing how you cope with this stuff as it seems to be a real roller-coaster ride for me.

Judith

Valérie’s story

I am from France and have been diagnosed with Waldenstrom’s macroglobulinemia in 2005.
The diagnostic has been confirmed by bone marrow biopsy. My bone marrow produces IgM Kappa light chains.
I have a secondary CAD (Cold Agglutinin Disease) linked to IgM Kappa as well as a Coombs Positive Hemolytic Anemia.
My oncologist (Mr Decaudin, Curie institute in Paris) and his colleagues do not have much experience on that particular rare form of WM.

I am interested in hearing from other patients or experts, who may have experience on this specific disease, and in appropriate treatment possibilities and results.

In addition my mother has very similar WM cancer. She was diagnosed in 1999.
She presents a renal insufficiency : IgM Kappa light chains precipitate on the glomerular basement membrane forming deposits that occlude the capillaries.
She does not have Coombs Positive Hemolytic Anemia.
In her case the only treatement that have given good result so far (remission during 4 years) is chlorambucil associated with prednisone.

Here are the details of the particular symptoms I have:

  • Tiredness
  • Severe anaemia (hgb between 8 and 9g/dl blood cells)
  • Night sweats
  • Pain and damaged in the joints

But I do not show the following symptoms:

  • monoclonal IgM peak in electrophoresis analysis —> my IgM are 500mg/dl with no visible peak
  • Increased size of the spleen, the liver, and some lymph nodes –> not visible by Scanner
  • A tendency to bleed easily and to bruise easily (too few platelets)
  • Headaches and dizziness
  • Various visual problems
  • Pain or numbness in the extremities

As I am positive at direct coombs test results. That imply Fludarabine treatment is not recommended for me because of hemolytic risks.

Treatment history and results:
No symptom 2005 – 8/2007 – anemia started in Aug 2007 ( Hb 9 to 10g/100ml)

  • 11/2007  ( Hb felt < 9g/100ml) –> 3 injection Rituxan :  with no effect
  • 3/2008 prednisone 1mg/kg with no effect
  • 6/2008 to 11/2008 RCVP (Rituxan, Cytoxan, Vincristine and Prednisone) with very some improvement of Hgb (                  around 10g/100ml ). My mother has also been treated with this protocol with no result.
  • 12/2008 : wait and watch my Hgb are stable around 10g/100ml

The next step for me should be chlorambucil.

Thank you for your help.

Best regards
Valerie P Debaix
(France – Paris)
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Lael’s story

I am Lael, 67, living on a farm in Montrose, Colorado.  This high-country desert is less than two hours drive from three ski areas. Our valley has an arid climate, temperatures occasionally reaching highs above 100 or lows of below 0.

For me CAD is as if I were putting together a jigsaw puzzle that has no picture to guide me.  I don’t know how many pieces are in the puzzle, and someone gives me the pieces a few at a time over the years, occasionally throwing in pieces that belong to some other puzzle.

The first piece of the puzzle I received over 50 years ago.  My pediatrician told my mother that I should be protected from the cold.  I had a marker in my blood that indicated I would be susceptible to cold and to arthritis.

I have had restless leg syndrome since childhood. The NIH web site says, “People with low iron levels or anemia may be prone to developing RLS. Once iron levels or anemia is corrected, patients may see a reduction in symptoms”.  When I took the blood test to get married, I found that I test a false positive on the syphilis test. In my late 20’s I tried to become a blood donor.  I was refused because of the false positive that I test and because as my blood cooled the red blood cells “clumped”.

In the early 90’s I began to spike high fevers, have hallucinations, then a day free of symptoms and back again.  I grew more and more debilitated.  I tested a high titer for rheumatoid arthritis and was treated for RA until the day I was put into intensive care.  There, a bone marrow test revealed that my bone marrow had ceased producing red blood cells.  A second doctor diagnosed the illness as Borellia, relapsing tick fever.  (There is another strain of Borellia that causes Lyme Disease.)  A round of tertracycline cured the disease, and my bone marrow resumed production.

Sometime after that I was told that I had cold agglutinins that were causing my red blood cells to agglutinate when cooled.  Since they were giving me few problems, I forgot about them.

In 2005 I began to be fatigued, have plantar fasciitis (an inflammatory disease of an area in the foot that has a limited blood supply), eventually had heat exhaustion.  When I finally saw my own physician, she took one look at me, declared I was severely anemic, and put me in the hospital.  There, another bone marrow test revealed that I was not producing sufficient red or white blood cells.  I was given four units of packed red blood cells.  A distant lab looked at my blood and said I, almost certainly, had leukemia.  My spleen was swollen and what red blood cells my bone marrow was producing were large and misshapen.  By the time I got an appointment with an oncologist/hemotologist, my blood counts were rising.  Two months later, well on my way to good health, no leukemia, the CDC finally let me know that I had West Nile Virus.  I began at that time to have escalating problems with hands and feet during the winter, but I managed the problem, didn’t mention it to my doctor, and went on with normal life.

From the summer of 2007 until December 2008 my doctors and I dealt with pain and spinal issues until lumbar fusion was scheduled. Meantime, I was having growing problems with CAD.  Even in our home, heated to 70, my toes, fingers, nose would turn white then purple.  Any exposure outdoors would add in ears, cheeks, mottled legs, and purple knees.  I tried heated gloves and socks, wool socks, chemical heat packs, only receiving partial relief.

In December as I lay waiting to be taken to the operating suite for the lumbar surgery, in rapid succession a nurse, the anesthesiologist, and the woman running the blood cleansing machine came in, read my chart, and said, “Cold agglutinins, I don’t know anything about them.  I’d better go study up.”  At that point my husband was ready to kidnap me, hospital gown and all, to get me away from these uninformed people.  My surgeon reassured him that the operating suite would be heated higher than the normal cold temperature, that I would have warmed blankets on me at all times, that two units of blood were typed and cross matched and would be given warmed, and that the surgical staff was prepared for my case.

The blood cleansing machine would not be used because there was no guarantee that the blood could be kept warm.  Of the blood that runs through the machine, only red blood cells are returned to the body, the rest are eliminated.   If the red blood cells agglutinated, that would mean that there would be nothing to return to the body.

We made it as far as the operating theatre when the pathologist called down.  My blood cells from the previous days test were hemolysing, and he did not feel that we had sufficient information on CAD to a risk a six-hour surgery.  Surgery was cancelled until I saw a hematologist.  The hematologist okayed the surgery without the blood cleansing machine and with a sufficient supply of typed and cross matched blood for transfusions and suggested that, long range, we consider moving to a warmer climate.

The surgery in January was successful, used only five units of donor blood.  Four days later I was released from the hospital having had countless blood tests, about a third of which had to be repeated because even with heat packs and heated blankets they did not make it to the lab before they agglutinated.

Ten days later I had a second operation because I was not healing well and had a staph infection.  This entailed four more units of blood, a wound vac pump attached to my spinal wound for five weeks, intravenous antibiotics 3 x daily for 7 weeks, and weekly blood tests at the hospital lab. The hematologist kept track of my blood tests, declared I was improving, and once again suggested that we consider moving to a warmer climate.

To speed healing of my back I was supposed to be walking 1½ miles per day, impossible in Colorado winter weather with CAD.  When I was in town I walked up and down the aisles of the supermarket, wound vac in the basket, PICC line in the arm, turning purple even in the heated store.

Spring began to flirt with winter.  The wound vac and PICC line were finally removed.  I walked until…the pain in shoulders, arms, wrists and hands, once attributed to spinal problems, returned, accompanied by a full-body assault of pain in joints and muscles everywhere except the lower back.

My GP sent me for—you guessed it—more blood tests.  I thought the diagnosis would be rheumatoid arthritis, but I tested off the charts for lupus.  I have now added a rheumatologist to my medical team.

I still don’t know what the total picture of my puzzle will look like.  The rheumatologist questioned the long-ago diagnosis of Borellia, asked me questions about conditions I have never heard of and some I had (pernicious anemia).  There will be a change from Prednisone for pain to something with fewer side effects.  Eventually, I may be sent to Mayo Clinic for further testing.

I suspect my puzzle will never be finished, but some of the pieces are coming together.  Both CAD and lupus are autoimmune diseases.  Some people with lupus test a false positive on the syphilis test.  Rituximab is a drug used for the treatment of both lupus and CAD, and, yes, we are trying to figure out if we can afford to be snowbirds, flying to a warmer climate in the winter and continuing to farm in gorgeous western Colorado in the summer.  Lots of pieces, large puzzle, no big picture….

Peace and grace,
Lael

Ruth Ann’s story

My name is Ruth and I live in Smithfield, Utah (I am 62 years old). Sue told me about the Cold Agglutinin Disease (CAD) Website and it is so nice to know that I’m not the only person in the world with CAD. Thanks again for responding. Sorry I’m so slow in getting back with you. (Life has been a little crazy.)

I also have Waldenstrom Macroglobulinemia without the syndrome. I have known that I had CAD since Oct 2006. (I know now that I have had it much longer. In the past, when I would cross-county ski or would get cold, my hands and feet would get so cold I could hardly function and then they would start turning purple.)

Back to 2006 – The Doctor did some tests and my “M spike” was very high so he sent me to a Cancer Doctor in our area. The Doctor did a bone marrow biopsy and it showed that I had something wrong, but the tests were inconclusive. The biopsy also said that I needed additional tests, but they were not done. Both of my Doctor’s said to get back with them if I started feeling ill, whatever that was. So I went along knowing that I had cold agglutinin disease and not knowing what the underlying cause was.

Last year was full of all kinds of surprises. I am not sure if that had an effect on my disease acting up. In September I started feeling very tired and had a hard time functioning. On Oct 13, 2008, I received two units of blood. (Prior to the transfusion, when they drew blood they did not keep it warm so I had to go back again.  It was also difficult to find a blood match, so I had to wait four days before the first transfusion.) They did heat the blood when I received the blood transfusion, thank goodness.  My Doctor told me it may not last and that I may need an additional transfusion, but to always keep warm.

I asked for additional information about CAD and he did not get back with me, so I contacted the Huntsman Cancer Center in Salt Lake City, Utah and made my own appointment. (I think they were interested in me because I had CAD and my condition was rare)  My first appointment was Thursday Oct 23, 2008 and they did numerous blood tests. On Monday, October 26, I had to have a blood test at a Hospital close to where I live and my blood levels were lower so they had me come to Salt Lake. I had to go back again on Tuesday Oct 27, because they were concerned about me and they wanted a CT Scan as soon as possible. They had also forgot to draw enough blood on Monday, so they could find a match for a blood transfusion.

In the afternoon after the CT-Scan I was having a hard time functioning and they were very concerned about my levels as they were still going down. Hematocrit – 20.8 and my Hemoglobin – 7.4. They admitted me in the Hospital and from there I got a final diagnosis and started treatments. Two units of blood, Rituxan and Cladribine.

I received four treatments of Rituxan in November 2008. I am currently doing very well! I went to Doctor Gilbert, in Salt Lake on Thursday, July 16, and my levels are – Hemoglobin 12.9; Hematocrit – 35.0;  Platelets 163. I have to go back again in three months, unless I start feeling ill.

My main concern is that I don’t want this to happen again and I want to do whatever it takes to make sure it doesn’t. So I’m interested in what I need to do to stay healthy, and if there are any precursors to the disease. “Please let me know your secrets.”

I lead a semi-active life style and I’m always doing something – from gardening, crafts, sewing and traveling to our cabin in WY, keeping up two houses and two yards and working full time. I’m not biking because I think I’ve gotten lazy. Please let me know what I need to do to stay healthy. Do I still have the disease? Is it always lurking in the back waiting to flair up again?

Thanks,
Ruth

Barbara’s story

I’ve been visiting your website for over a year now.  My anemia was discovered on a routine CBC in June 2005.  That winter I began exhibiting Raynaud’s symptoms.  It took the lab staff 3 hrs to type and crossmatch my blood prior to my first, and hopefully last, blood transfusion in Feb. 2006.  The nurse informed me they’d encountered a “cold agglutinin”, and she provided me with a printout explaining what it is.  Back home, I searched the web to learn more about cold agglutins and came to believe I might have CAD.

I was referred to a hematologist in April 2006 because my PCP believed I had a hemolytic anemia.  The hematologist ran more bloodwork, but said it was not hemolytic.  He started me on routine Procrit injections for 2 yrs.  All that time he knew I had a “hard” agglutinin and sent me to the hospital lab for my CBCs since my blood plugged up the clinic’s automated machine.  More than once I mentioned CAD to him, but he insisted I didn’t have it.

In July 2008, I turned 65 and immediately changed health care plans and doctors.  By August I’d been set up with a new hematologist who said I certainly do have CAD.  My cold agglutinin titer was 1:20480.  Monoclonal B-cells showed up in my blood and bone marrow.  I was diagnosed with low-grade B-cell lymphoma and received the 4-wk round of Rituxan in Oct 2008.  I had a good response to it and haven’t needed Procrit injections since then.

However, I and my hematologist were disappointed this week to see that my red cell count has dropped to the low end of normal, and my LDH is above normal.  He showed me how my counts had slowly dropped since summer.  I’ll be going in for labwork monthly now and more often if I have symptoms.  He promises not to let me get too anemic before ordering another round of Rituxan.

My doctor also strongly encouraged me to start spending the winter months in Arizona.  He emphasized that my efforts to stay warm aren’t helping that much, and winter isn’t even here yet.  I’ve discussed this with my husband, and it would be hard to swing financially.  Still, we might be able to find a way to spend at least a month there each year.

I’m wondering if it’s even worth looking into.  Have your other readers (CADys) any specific ideas on good, affordable winter destinations?

Thanks so much for this great website.  I enjoy checking back now and again.  My heart goes out to all the others who share this disease.

2012 UPDATE

I first contacted Betty in December 2009.  I was concerned that my blood counts were beginning to fall again that winter.  My hematologist decided to watch and wait to see if they’d rise with the coming spring.  By April 2010 my hemoglobin had dropped to 9.6, and hematocrit to 25.8 when the doctor decided it was time for another round of Rituxan (4 wks).  I again had an excellent response to the treatment which lasted 19 months.

My hematologist ordered another round of Rituxan in December 2011 when my hemoglobin suddenly dropped to 9.4 and my hematocrit to 29.0.  He thought it was better to start treatment then since winter was just starting.  I reluctantly agreed, but two months after treatment, I’m feeling great, and my blood counts are well into normal range.  My LDH which had reached a high of 540 is now 181.  My hemoglobin is 14.1, and hematocrit is 43.6.

When I had my last checkup in January, I asked my doctor how much longer he thinks the Rituxan treatments will work.  I was concerned because an acquaintance with follicular lymphoma said Rituxan stopped working for him after 5 years.  My doctor assured me that because my underlying lymphoma is very slow-growing, Rituxan should work much longer for me.  Since this disease is here to stay, I certainly hope so!

My very best to all the CADdys out there!

Sincerely
Barbara

Don’s Story

I am 70 years old, retired and living in a golfing community near Providence Forge, Virginia.  My CAD saga began in 1999 when my primary care physician noted that my hemoglobin count was low.  He put me through a series on tests to determine the cause but nothing surfaced.  At the same time I noted some physical symptoms that I now know is associated with my disease.  However, at the time I did not realize the connection.  These were nose and ears turning blue when exposed to cold weather and difficulty in getting back to my jogging regimen that after a hiatus of ten years or so I had determined to start again.  I felt weak and unable to do more than a fast walk for a mile.  I quit after about two months effort.

Finally, my primary care physician referred me to an oncologist who almost immediately recognized the symptoms.  A bone marrow biopsy confirmed that I had a low grade lymphoma accompanied by cold agglutinins.  He had me go through a couple of treatment of oral chemotherapy.  (I’m sorry that I can’t remember the name of the medicine used.)  In any event, the treatments appeared to have no effect.  A second bone marrow biopsy after the treatments showed that the lymphoma had increased slightly and that the cold agglutinins were still present.

At the time my hematocrit readings were in the low to mid-30’s – Low but my life was not substantially affected.  I learned to stay out of the cold and that I would be unable to participate in any significant physical activity.  So it was determined to check my red blood cell levels every three months and monitor the situation…  I was living in Northern Virginia at the time, working 8-10 hours a day, playing golf every weekend, and generally enjoying life.

The only thing worth noting was that sometime in 2004-05 (I can’t remember exactly) the machines used to measure hematocrit were unable to get a viable reading as the blood was clumping too quickly – even keeping it warm up to the time it was processed was inadequate.  We adapted and began to measure red blood cell levels by the hemoglobin readings.

In 2004 my wife and I moved to our present home near Providence Forge and began looking for a good oncologist or hematologist in this area.  We found a superb doctor at the Peninsula Cancer Institute in Williamsburg, Virginia who was well versed in the eclectic nature of my disease.  After a few sessions we determined to continue the quarterly monitoring.  This went well for a couple of years.  But then increasing attacks of chronic sinusitis begin causing problems.  Each time I suffered a sinus infection or other stress related condition my Hemoglobin count would crash to 6-7 levels.    After each attack my blood levels came back up on their own once I had treatment (usually antibiotics) that alleviated the sinus attack or stress related issue – A radical prostectomy was one such incident and my blood levels were low until the catheter was removed.  After a year of so of this my doctor, concerned about a massive drop, decided that I should undergo two years of Rituxan (Rituximab) treatments.

Things went along as usual – And the quarterly blood tests showed their usual ups and downs.  About this same time my frequent sinus attacks were controlled by a daily dose of Allegra-D 24H and I felt good up until this last summer (2009).

This summer in late August after return from a trip abroad my hemoglobin count went down to 6 and didn’t come back up on its own.  I was about to begin my 4th and supposedly last series of Rituxan treatments in early September.  In the interim, my doctor prescribed a massive dose of prednisone and wanted to see how the Rituxan and prednisone would work before considering other options.  Unfortunately, this was the last time I would see this excellent doctor.  He suffered a back incident in early Oct and has been out of action since.

His absence from the practice caused a bit of an upheaval to say the least.  I was assigned to a new doctor who ordered a bone marrow biopsy and continued the prednisone – 100 mg per day.  And I started the Rituxan treatments on 8 September.  The bone marrow biopsy showed that my lymphoma was in remission but there was no doubt that my cold agglutinins disease remained in full force as my hemoglobin count hovered in the mid-6’s.  My doctor ordered a blood transfusion and in the period 22 Sept to 11 Nov I had five blood transfusions.

My doctor told me that while the Rituxan worked on the low-grade lymphoma it sometimes took a bit longer to work on the cold agglutinins anti-bodies.  Essentially we were in a wait and see mode.  This was extremely frustrating for me, particularly as it looked like I was in for a regimen of transfusions every couple of weeks…  Under doctor’s orders I was still taking prednisone although it appeared that I had “chronic CAD” and everything that I had read indicated that prednisone was not recommended.  I had backed down to 60 mg per day.  I was also taking folic acid, iron tabs, and B-12 shots (once a week).  And had an iron transfusion as the doctor thought my iron reserves were low and wanted to bring them up.

I decided I needed a second opinion and arranged a visit to the Massey Cancer Center at VICE, Richmond, Virginia.  My first appointment was on 18 November.  The doctor that I saw there was extremely knowledgeable about CAD and associated diseases. And as it turned out the doctor that I had been assigned to at the Peninsula Cancer Institute had studied under him at VCU.  Not only that but PCI is collaborating with Massey in a number of cases.  I was added to that list.

More interestingly, a couple of days after the 11 Nov transfusion my hemoglobin count was 8.4.  In the next two weeks it worked its way down to 7.7 then stabilized for about a week.  On my last test on 16 Nov it had moved back up to 8.6.  We’re hoping this trend will continue.  If it doesn’t the doctor appears inclined to have me take another series of Rituxan treatments.  No matter what happens he plans to have me take these in February but will move the series up if my blood levels don’t continue to rise or begin to fall again.  And with Massey doctor’s concurrence I’ve backed totally off the prednisone.

I might also add that after spending more time exploring and reading the CAD website and about the same time I saw the doctor at Massey I decided to swear off cold drinks and ice cream.  Prior to this I liked my drinks with lots of ice, liked to chew ice, loved ice cream, and dearly loved a nice, cold beer.  Now I fairly rigidly follow a regimen of room temperature drinks and except for an occasional yogurt have no ice cream whatsoever.  I have no idea whether this has triggered the upward movement of my red blood cell count but I’m not a great believer in coincidence.   I asked both the doctor at Massey and at PCI whether they knew anything about cold drinks or ice affecting RBC levels for CAD sufferers.  Neither did but both said it couldn’t hurt.

As you can see the story is not complete.  I’m hopeful that the upward trend will continue but there are still unanswered questions.  Why did it fall so drastically in the first place?  There was no sinus attack or other physical disorder that I could discern.   Will Rituxan be a regular every six months?

In any event, like all of you I’m sure, I found the CAD website extremely helpful.  If any of you have any comments, ideas, suggestions I’m all ears.

UPDATE – 27mar2013

Since the events described above my hemoglobin levels have been fairly normal, at least for me, ranging from about 9.0 to 11.0.   I can’t remember exactly but I believe my medical records will show that I underwent a Rituxan treatment in the spring of 2010 but none since.

I continue taking Allegra D 24 hour and have not experienced any sinus attack during this period.  I had one bout with food poisoning while visiting my mother in San Diego.  My blood crashed severely.  It had been close to 11.0 prior to my trip and when I returned it was on the low side of 9.  And I was by then feeling much better.  I don’t know how low my hemoglobin levels went but it most have been somewhere in the 7 range or lower considering how I felt.

As a follow on to the notes I made in late 2009 regarding my long period of complications with CAD where my hemoglobin was 7.0 or below, I now believe this was caused by a low grade chest infection that was eventually cured by my primary care doctor who prescribed some antibiotics after hearing pneumonia type sounds in my chest at the same time that I was undergoing transfusions.

I also note from my earlier notes that I had been fairly consistent about avoiding cold food and drinks during that same 4 plus months of complications.   I have to admit regressing a bit on this.  I generally have ice in sodas or drinks that call for ice, and will enjoy ice cream regularly.  I haven’t noticed that doing so has had any adverse impact on my CAD.

As I write this my doctor at the Peninsula Cancer Institute in Williamsburg, Doctor Kerbin, is relocating, and I have scheduled an appointment with Dr. Ginder, Director of the Massey Cancer Center at VCU…   Of some concern is a gradual lowering of my hemoglobin levels over the past year.  Where I used to be regularly in the 10 plus area, recently this has been more at 9 plus level.  My life style is still active but I’ve noticed a slight physical impairment with these lower levels.  Dr. Kerbin thinks maybe another series of Rituxun might be advised and I will discuss this with Dr. Ginder…

Regards,

Don

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