Stories 2007

Starting 2007

Betty’s story

I am a 68 years young (in 2006) Caucasian female born in New York.

Ever since my 30’s, I had been considered to have low-normal hemoglobin trending between 11.6 and the low 12s. bettyIn early 2000, I wasn’t at all concerned when my gynecologist suggested I should consult my internist because my 11.3 number was too low. I was in for a rude awakening.

After several rechecks over the next 3 months, my internist referred me to a hematologist/oncologist for more in-depth testing In the late fall of 2000, I was very upset when I learned of my diagnosis of Cold Agglutinin Disease Auto-immune Hemolytic Anemia. I knew of no one with that diagnosis and felt very frightened and isolated.

To arrive at that diagnosis I had been given many sophisticated blood tests, GI testing–colonoscopy & gastroscopy, a CT scan of my internal organs, and a gallium x-ray with radloisotopes of my lymph nodes. Ultimately, I had the bone marrow test also. Fortunately, all tests were negative

Since no underlying disease was noted, the diagnosis was Primary Chronic CAD (Cold Agglutinin Disease). It is considered idiopathic (no known cause) Cold Agglutinin Disease. Secondary CAD is caused by an underlying lymphoproliferative disorder such as lymphoma or an infection such as mycoplasma pneumonia or another autoimmune condition like lupus etc.

I had monthly CBC (complete blood count) checkups when my hemoglobin was fairly steady in the 11 range and as it went lower, the visits became more frequent

I could almost predict what my hemoglobin number would be by the way I felt. When it was high, I’d be full of energy and could “chop wood”. When low, I’d be very tired and wake up feeling as tired as when I went to sleep. Also, I tended to feel more out of breath on exertion, was very pale, my eyes looked yellow, gums and inside eyelids were a very light pink color and workout seemed impossible because my muscles in my legs felt strained as if I couldn’t take another step and had to sit down. On two occasions I experienced brown urine–both times I was sick with the flu and a virus infection.

In 2002, I was started on Procrit, a bone marrow stimulator for red cell production and continued with weekly self-injections to help raise my hemoglobin.

ln the winter of 2006, even the Procrit wasn’t working and my hemoglobin reached a low of 7 necessitating 2 units of transfused blood. Additionally, I was treated with 4 weekly infusions of Rituxan that raised my numbers only slightly and for a short duration.

I should note that I had been ill several times in the fall and winter with various colds, a herpes outbreak, and other viral infections. There seemed to be a direct correlation between these illnesses and my lowered hemoglobin.

By spring 2006, my doctor decided to re-treat me with another round of 4 weekly infusions of rituximab (Rituxan) and this time I had a more positive response to the drug. By summer, my hemoglobin rose to 14.2 and now 7 months later, remains mostly at 11+ with bi-weekly Procrit usage. I am still hemolyzing, but apparently compensating for the loss with sufficient red cell production. I continue to take approximately 2mgs of folic acid daily, exercise, keep warm, and try to eat healthily.

Rituxan is a wonderful drug and can sometimes be more effective after the second or third round of treatments as it was in my case. Heretofore, corticosteroids and chemotherapies and immune suppressing drugs have been our only treatment options and all have side effects with the results being mostly temporary in nature.

There are other single agent monoclonal antibodies in clinical trials. These are designed to target a specific site in the body with the goal of eliminating damage to the surrounding healthy tissue. This is good news for all of us.

I have consulted with four hematologist/oncologists in the Miami area, and 3 in New York and all agree on the CAD diagnosis.

2010 UPDATE

For 3 years following the Rituxan treatments. I continued to fare well using only 2 mg. of folic acid daily and weekly 10,000 unit, low-dose Procit.  During the early Fall of 2009, my hemoglobin headed south due to a respiratory/sinus infection and the stoppage of the Procrit at the urging of my new New York Dr. Richard R. Furman who advised me of the FDA warnings of possible thrombosis. He also expressed concern that I wasn’t making enough reticulocytes-immature red cells.

I had a CT scan of the pelvis, abdomen, chest, Bone Marrow Biopsy, Metabolic Profile, Coombs Direct and Indirect Tests and more.  All ruled out Lymphoma.  My HGB continued to drop until it reached 7.2.  It was decided to treat again with Rituxan X 4 and also to do a Plasmapheresis to give me a better chance to respond to the Rituxan.  Also, I received 2 units of blood to raise the HGB so I’d have more energy and be out of the danger zone.

My numbers improved in the 9’s but after several weeks of watching and waiting, it was decided to retreat with Rituxan again between both my doctors, Dr. Michael A. Schwartz, Mt. Sinai Comprehensive Cancer Center, Miami Beach, Florida and Dr. Richard R. Furman, New York Presbyterian Hospital, Weill Cornell Medical Center, Hematology/Oncology, New York City. I have had to again use Procrit to avoid transfusion but stop when I reach a hemoglobin of 10 or higher. Presently, I am at 10 and on W & W (Watch and Wait) again.

We are discussing what, if anything, should be done next, to try to put me in remission. There are new single agent monoclonal antibodies like Rituxan which are being considered at this time. Some have already been approved for use for Chronic Lymphocytic Leukemia, Waldenstrom Macroglobulinemia (an indolent form of Non-Hodgkins Lymphoma) and Lymphoma and Multiple Myeloma and are sometimes used in combination with other treatments. These could possibly be useful for CAD patients.

To be continued….

Betty Usdan
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Graeme’s story

I first noticed my ears turning dark blue (I thought it was oil on graemethem) about mid 2003 when I was 62.  I was going to the gym at the time and I noticed my stamina and fitness were getting worse, not better!  I never realized there was anything wrong. Then my fingers started going numb if I touched anything cold and my Doctor diagnosed Raynaud’s syndrome. So I started wearing gloves. I noticed this particularly in the winter mornings when getting things from the fridge for breakfast. There was concern that my heart pills (I had two heart attacks in 2000) could have been contributing to this.

I am a keen yachtie and we had our Opening Day in September.  I got a shock when I went to the toilet after sailing and saw that my urine looked like blood. My Doctor arranged for me to be seen at our local Hospital and they started doing tests to try to find out what was wrong. There were confusing symptoms as I had a very sore prostate at this time and I know I felt sure it was prostate cancer. I had difficulty believing that they couldn’t find it. I also developed a very sore upper arm which stayed around for the next six months.

I started keeping a Diary on how I felt and an extract from a day about this time is:
“A cold wind is blowing. My fingers got very cold and my ears went black after I had been outside for about 15 minutes. My fingers were also very cold and after I had warmed them up under hot water, they again went cold if I touched anything cold. I am wearing gloves and a hat inside and out. I am wearing thermals to keep warm. I have put on two pairs of socks as my toes were tingling, and very cold. I passed blood again in my urine this afternoon. I have bottled some to show my Doctor. ”

A month later I wrote:
“While I don’t want to be pessimistic, I believe that I am getting worse. My fingers and ears are going blue/black more often even though the weather is getting warmer. I wear a hat and gloves a lot, particularly whenever there is a cool breeze, and also when I get up in the morning. Even then my ears can still go blue. My legs often feel quite weak, and I occasionally feel slightly dizzy. I didn’t have all these problems at the beginning of October.”

During this time I had many tests done at the Hospital……………….and towards the end of November 2003, they told me I had Cold Agglutinin Disease.

Over the next few months I was given some treatments to try to alleviate my problem. However none were effective and my condition deteriorated. My blood count fell to below 0.7 and I started needing blood transfusions. Initially these were at six week intervals, but by August 2004 I was down to two week intervals. It was then decided to give me Rituximab, and I am fine nearly three years later. My latest blood count is about 14.0, I can do most things, I still have to keep very warm or blood shows in my urine, but life is great!bootie

The cat’s name is Bootie.

 

Update February 2010

Bootie is now a little larger, but lovely. I have just seen my Haematologist and he is extremely pleased with me. It is 5 1/2 years since he treated me with rituximab, and I have had no other treatment since, apart from one folic acid pill daily.  At the time I was in extremely poor condition, and receiving blood transfusions every two weeks. My CAD is now in remission and I don’t have any visible symptoms. Last weekend I capsized my small yacht and was in cold water for about 10 minutes. I had no after effects, and my HGB was 13.6 three days later.

So am I cured? My haematologist doesn’t know the answer to this, and wants me to visit him annually so that he can monitor my progress. I can’t imagine that it will come back….but I don’t know why it started in the first place so maybe it will……?

On reading your stories I am very aware that I am very lucky, and that most of you struggle to cope with your CAD over many years. You are in my thoughts.

Update 2015

I am now about 15.5 after being at about 6/7 in 2004. I just had Rutiximab on its own as it was before the Rutiximab/Fludarabine combination was known, but it worked for me.

I sometimes wonder if it will ever come back and I still keep myself warmer than others. I feel very lucky to have escaped its clutches.

Graeme Hore
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Bea’s Story

This is a photo of myself and my husband, my grandchild and our new puppy!

beaIn the winter of 1982, our family, consisting of my husband, my 13-year-old son, my 7-year-old son, and myself were tobogganing in Palos Hills, a suburb of Chicago.  At the bottom of the run my son looked at me and said, “Mom, you’ve got dirt all over your face.”  I knew that couldn’t be true – we were in the snow!  So, off we went to the car and, sure enough, looking in the rearview mirror I could see what appeared to be purple vertical lines on my face.  For whatever reason, I then looked at my feet and legs and they, too, had dark lines on them.

My family physician sent me to a hematology specialist.  Tests were done and I was sent home with the admonition of: “Don’t go out in the cold until we get the results of your tests.”  I was found to have a positive “direct Coomb’s test and a positive antibody detection test.”  My “cells were coated with complement components and the serum reacted with all red cells tested.”

At that time I did not have a clue as to what that meant, but the diagnosis of cold agglutinin disease with autoimmune hemolytic anemia had been confirmed.  I was fortunate enough to be diagnosed correctly immediately and did not have to endure other tests or a bone marrow biopsy.

Being 37 years old at the time and never having heard the words “cold agglutinin” or knowing what that diagnosis would mean to me or my family, I felt very frightened.  Additionally, finding out how rare this diagnosis was left me feeling extremely isolated.  I had not heard of, nor did I know a single other person who had this rare diagnosis.  I know (now) that I am one in a million (literally), but finding that out didn’t make me feel any better either!

When I was diagnosed, my titer was quite high at 1:2048.  Living in Chicago in the winter is quite a challenge when you have CAD.  There were times when my blood counts dipped quite low and the fatigue became all encompassing.  When outdoors, every exposed piece of skin had to be covered for it would become mottled.  I found I had to use gloves to go into the freezer.  I could not longer put my hands under cold water or even hold the garden hose.  Down coats, down mittens, and boots, even when there was no snow, became the dress code.  Remote starter for the car so I could go from a warm house to a warm car.  Obtaining a handicapped parking placard so I could park close to entrances.  All this and so much more became the “norm” in my life.

Through  all this I had a compassionate and caring doctor at Loyola Medical Center (Dr. Messmore) who watched over my counts (and me) and taught me how to set limitations for my physical well being.  Fortunately, I have never had my counts dip to a place where I had to have any treatments.  However, I was told about plasmapheresis and that a “chemo drug” could be used if that happened.  I was also told that this could progress to a lymphoma or a myeloproliferative disease.  Again, there was no one to talk with about these possible “scary” prognoses.  My much-revered Dr. Messmore has since gone into retirement.  He was replaced by Dr. Godwin who has since gone on to become the Director of the Cancer Center in Southern Illinois.  And now, Dr. Barton has, very recently, replaced him.

It has been 24 years since my diagnosis of CAD.  I am now 61 years old (2006).  I have “coped” with my diagnosis.  It has profoundly changed my life.  It has changed the way I live my life.  It’s been an unseen presence every day in my life for all these 24 years.  It is a “hidden” handicap.  Because of my CAD I have had to enforce the Americans with Disabilities Act (ADA) at my place of employment because I could not work in the temperatures maintained in my workplace.  Because of CAD I have had to endure the taunts of uninformed and ignorant people when I stepped from my car in winter after parking in the handicapped space and all they saw was someone who was not using a cane or a walker or was limping and they assumed I was not “handicapped.” Because of people’s ignorance about CAD, I have been asked, “Is this related to AIDS?”

But, because of CAD I have learned:

Tolerance and Understanding that things are not always what you see on the surface.

Forgiveness of people’s ignorance.

Patience and Perseverance in waiting for this Web Site and the support of people with a shared experience.

That there is always Hope.

Bea

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Laurie’s Story

My name is Laurie and I am 51 years old.  I live in Washington State laurie riffleand would enjoy all of our 4 seasons if I could just go outside more than 4 months out of the year.  I used to love to ski and that is how I found out that I have CAD.  I had been skiing and was really without energy to walk up small inclines carrying my skiis.  I came in on my last run and took off my boots. My legs and feet were so ghostly white.  (2001)

I had been to the doctor a month earlier and when I had my CBC run, I was a little low.  The doc told me to take iron.  I took the iron and continued to ski.  I called them after seeing my white legs and told them that I could hear my heartbeat in my ears.  She said that they couldn’t get me in right away and I should go to minor emergency.  I went and they took one look at me and told me that they would need to draw blood.  The doc called me that night and told me to get to the store and take 1 mg of folic acid and stay warm and to see a hemotologist.  Well, the only hemotologist here are in the Cancer clinics.  I didn’t know that until I walked in and it was such a shock.  The doc tried cyclosporin on me…a cancer drug, and it didn’t work.  Then she sent me to a teaching hospital in Seattle.  They took more blood.  I was already at 7 on my hemoglobin and still they took more blood.  They also did the CAT scan and bone marrow biopsy.  All normal except the CAD.  Their recommendation:  Cytoxan….another chemo drug.  Of course, it didn’t work!  It just made me loose my hair and feel awful.  Then my doc decided to try the Rituxan.  It brought my titer down to 1:256.  It stayed that way for about a year.  Then it popped back up to 1:1024 and has been that way since.

The end of January I got the flu.  It went to battle with the CAD and I lost.  I ended up in the hospital for my first transfusion.  My Hemoglobin had been around 10 and within 2 days, I was at 6.  They gave me 4 units of blood and the doc also gave me  2 shots of Prednisone.  I left the hospital with a hemoglobin of 12.1 and felt great.  While I was on the Prednisone, I didn’t loose any blood.  Within a week, my hemoglobin was at 13.2.  I felt wonderful!  Then I was weened off of the Prednisone and I started to loose blood again.  Prednisone is only suppose to maybe work for CAD with low titers and high amptitude.  Well…I have high titers….I don’t understand why it worked…I only know it did, but it’s not a drug to stay on for very long.

March 2007…just finished 4 rounds of Rituxan.  Found out today snowdogthat my titers haven’t changed.  I’m still at 1:1024.  I also lost a little vision after the 2nd round of Rituxan and went in for a check. They said that I have Narrow Angles which can lead to a glacoma attach from pressure on the optic nerve????  Anyway, they have to do a laser hole in each eye for drainage.  The reason that I have to have it done now is because I am not suppose to have any antihistimines that can dilate your eyes because it can cause an attack.  They give Prednisone and Benedrayl (an antihistamine) with each Rituxan treatment and the doc wants to do another 4 treatments in a month or so.

June 2007….Life is good since the Rituxan treatments.  Last week I had a blood draw and my hemoglobin was 13.6 and I wanted to dance.  That’s the highest it has been in 6 years.  I feel great, and have lots of energy.  The only thing that I have changed is that I quit drinking cold beverages….it was hard to give up, and I started taking 500mg of Vitamin B5.

I think that I am going to try and ski this season.  I’m only going to make a few runs and hopefully on a sunny day.”

Life goes on……

Update July 2010

I went to the doc about a month ago for my 6 month blood test.  My CBC six months ago was normal and my titer was 1:128.  So, I wasn’t expecting much change.  I had walked outside all winter without covering my head and didn’t have any symptoms.  I very rarely have any symptoms with the titer that low…it’s only when it gets to the 1:1024 that the purple nose and blood loss come into play.  So, I went and had my CBC drawn and it came back at 14.2.  Highest it has ever been and very normal.  Two days later, they give me a call to tell me my titer is 1:8, which means that there was no cold agglutinins in my system.  I about fell over.  The doctor called me and said that my last round of Rituxin  (March 2009) must have killed the clone.  He thinks it is gone and won’t be back.  I will still go for a blood test in 6 months, but I will go on as if it is gone. Yeah!!!
It came out of the blue, and it left the same way…

Update December 2014

Since my last Rituximab treatments nearly 5 years ago, I have had no symptoms…I could eat and drink anything I wanted and get cold. No blotchiness, not blood in the urine, nothing.   So, when it came back slowly over the last 6-8 months and my titer began to raise, the only reason I knew it was there was because of the blood in my urine.   No Raynauds like before, no fingers or toes turning white and then blue…no blue nose.  So when the titer got to 1:1024, the doc said it was time to do Rituxan again.   So we did. I had the first infusion done on October 31. My Hemoglobin was 10.9.   It was stopped when I was only 1/3 through the Rituxan drug because of a reaction.  So I had to come back with a new protocol for giving it to me.   But, because I was super busy at work, I put it off for 3 weeks.

However, I began to notice that I was not seeing blood in my urine.  I started my first infusion again on November 25th.  I was surprised to see that my hemoglobin was 12.5.   We did the 4 infusions without a problem once they gave me a side by side infusion of Rituxan and saline at the same time.   No reactions.  My hemoglobon rose to 13.2 the 2nd week and third and then on my last one on Tuesday, it was 13.4.   My Cold Agglutin Titer went from 1:1024 to 1:128.   So when I saw my doc on Tuesday, I asked him why I got such a fast response to such a small amount of the Rituxan on Oct. 31st.   He proceeded to draw me a pictures of the lymphocytes and the CD something or other.   And said that when the Cold agglutinins came back this time, they came back in a weakened state and the Rituxan went right to work destroying the lymphocytes.  And because they weren’t as strong as the first time, I didn’t have the same symptoms as I did when I was first diagnosed.

I have been feeling great.  Very little symptoms right now.  Will see what my CBC is in February. If it’s good.. I’m buying ice cream!

Laurie

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Marilyn’s Story

marilynfishmanI am presently (2007) a 48 year old female and I was officially diagnosed with Cold Agglutinins 3 years ago, but the doctors think I had it longer than that.  I tested positive for Mycoplasma Pneumonia at the time also.  I have done just fine for 3 years.  My bone marrow has fully compensated me for the low grade hemolysis that my body goes thru.  I have my blood counts done on a regular basis.  At the time I was diagnosed my Hematologist wanted to be aggressive and try to reverse the Cold Agglutinins.  I checked with several other Hematologists in California and decided not to do anything while my bone marrow was fully compensating me.  I did not want to take steroids or immuran back in 2003.

Then on March 1st, 2007  I came down with what my allergy doctor thought was a virus.  I was home, sick for 5 days.  The first 4 days I had a fever of 101.6 that would go down to 100.5 and then back up to 101.6..  continually.. on the fourth day on March 4th I noticed I was looking pretty pale….  and I couldn’t keep my fever down so I contacted the doctor who told me to take Motrin ( do not take Motrin)  which I did, quite a bit .. by day 5 march 5th, 2007  my palms were white and my heart was beating really fast and pretty weak.  I ended up in the Emergency Room at 12:30am on March 6th, 2007.

My hemoglobin was 4.8 and I was told I needed a transfusion……  I refused..  I thought they didn’t keep my blood warm and they did the test wrong..  my RBC count was 1.7 and hematocrit was 13..  They called in a Hematologist that I had seen back in 2003…..  She advised me that I needed a blood transfusion, I refused.  She started me on Prednisone 50 mg a day…and my retic count was 0.06, that meant my bone marrow was not producing blood cells.  They called in a Infectious Disease specialist…  The both of them thought maybe I was exposed to something, or possible the heavy doses of Motrin  which can cause aplastic anemia… (don’t take Motrin)  only Tylenol…

Then they decided maybe I had (Parvo-virus b19) also called the (fifth disease).  Usually kids get it.  It gives them red cheeks and a rash on the trunk of their bodies..  It a simple virus for them..  but, People with auto-immune blood disorders… have major problems with this virus..  It tells the bone marrow to stop making red blood cells.  They did a bone marrow test on me that showed I wasn’t making any red blood cells.

On the 2nd day when my doctor warned me that I could die and or have a heart attack and stroke.  I agreed to the blood transfusion.  I ended up having 2 of them, which they gave me with a blood warmer..  I then went on 5 days on IVIG.. which is  Immuniglobins given by infusion for treating Parvo-virus b19..  They decided to treat me for the Parvo even thou the test results were not back yet.  On the 4 th day of treatment my test results showed I had Parvo-virus b19.   I was also given 4 Procit shots, one every 48 hours.  I was in Intensive care for 9 days and on the chemo-ward for 3 days for a total of 12 days in the hospital.

I was also given 1 dose of Rituxan on the chemo ward..  By the time I was discharged my hemoglobin had gone up to a 9.2 from a 4.9  and my hematocrit had gone to a 29 from a 13… and RBC was 3.2 from a 1.7….. my retic went up to 10.3 from 0.06 which showed my bone marrow was again making blood cells.

It was the worst experience in my life…  I had no idea that I could get this parvo-virus b19 that could kill me. I can never get Parvo again as my body will have anti-bodies for it. I am presently weaning myself off the steroids and am continuing my treatment of a total of (4 ) Rituxan treatments to try to reverse the cold agglutinin.  My cold agglutinin counts are pretty high right now.   My doctor here wanted me to stay on the steroids but the calif doctor advised me to wean off them.  I have high cold agglutinin count and low warm agglutinin count… with a positive Coombs test…

I have undergone 2 treatments on Rituxan  with no side affects… I have 2 more to go….  The doctor has discussed with me the possibilities of doing up to 8 treatments…..  I agreed to do 4 treatments and then we shall see what the results are.

I had great doctors here in Vegas, who came up with the right diagnosis and saved my life.  I wasn’t the most co-operative patient.  I questioned everything.  The lab at the hospital continually made mistakes in not keeping my blood warm.  They kept my room at 80 degrees to keep me warm….

I wanted to share this story.   This is something that could affect other Cold Agglutinin patients…..  The CDC -center of disease control has information on this virus… pretty informative..  90 % of the senior population has already been exposed to it and have anti-bodies to it..  It can only create a crisis situation with someone who has an auto immune blood issue..  like Cold Agglutinin patients like us…

I am on the upswing and feel pretty good..  It was a sur-real situation for me…  I had to take a step back and let the doctors do all these things to me.  My personality is such that I question everything and want to be fully informed.  Due to my low blood count, my head was pretty foggy at the time of admission…….  I had great support people ( my friends) who helped me thru my 12 days in the hospital.

You have a great informative web site.  I dont remember seeing this 3 years ago when I started doing research on my Cold Agglutinin disease.  I will be glad to receive email with any questions,

Marilyn Fishman Beach
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John’s story

johnharmsMy father, John, is 88 years old.  He also has lymphoma and has received a series of chemotherapy treatments (Rituxan) about once a year for the past 6 years or so.  His lymphoma did not respond to the chemo this fall so he received a stronger chemo after Christmas.  The treatment consisted of Rituxan, Fludara, and Cytoxan and really lowered his hemoglobin and threw off his white cell count.  He received weekly Procrit shots, received 2 units of blood, and stopped the chemo.  Finally, he had bone marrow extracted late in Feb. indicating his lymphoma seems to be back in remission.  As of 4/5/07, Dad’s hemo has come back to 10.9 following 2 iron I.V. infusions and a blood transfusion.  Currently, he is continuing with Procrit injections and feels better.

His Cold Agglutinin Disease was first diagnosed as Raynaud’s Syndrome in 1994. He tried to avoid the cold but he was still helping on his farm in Illinois so he was definitely out in some chilly weather.  I’m not sure of what particular treatment he has had specifically for CAD other than the Rituxan, etc. for lymphoma.

My mother has taken excellent care of Dad since his major heart attack in 1992.  Now when he needs her the most, she has been stricken with ovarian cancer.  I live in Georgia, they live in Illinois.

My parents do not have email so I’m your email link! …… Both my dad and mom are mentally sharp and had been physically active until these past months.

It was helpful to read of the CADdys’ treatments.

Thank you for your interest in my dad’s case—-Diane Harms Mulkey

Diane has sent us this addition to John’s story.
I’m sad to tell you that John passed away on May 13th.  Dad was doing well in the fall (08) but had to have an appendectomy on Oct. 8.  He seemed to be doing well but on Oct. 21 my mother died of her ovarian cancer.  Dad had a heart attack that same day!  He barely survived that but recovered and even lived alone at his home again.  He had a couple episodes with fluid in his lungs in Feb. and April and shortly after his 90th birthday, he was diagnosed with lung cancer.  Always a courageous fighter, Dad took a chemo treatment but it took his blood counts too low.  I don’t believe that his CAD was a factor in his final illness but his low hemoglobin affected his energy during his last years.  It was our family’s honor and privilege to take care of our awesome dad and we miss him greatly.  — Diane

We were very sorry to hear that John died on 13th May 2009.  Our deepest sympathy goes to Diane and her family. Personally, I was quite taken with the photo shown above which Diane sent us. For me it typifies the hospital or outpatient visits many of us have made. We sit in a comfortable chair, are connected to a blood warmer and get a transfusion. And the nurses always look after us…(Graeme)

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Katherine’s Story

I’m 34 years old and live on the northeast coast of England, one of the coldest places in the country. I was diagnosed with cold agglutinin disease 4 years ago, 2003, although I had been feeling poorly for the prior 12 years. I had had periods of anaemia and no one seemed to be able to tell me what was wrong with me. I experienced fatigue, muscle weakness, sore throats, and Raynauds and was told I had M.E/Chronic Fatigue Syndrome.

It was only when I had a viral infection in 2003 and became jaundiced that the Cold Agglutinins was diagnosed.  Doctors found an IgM paraprotein. My haemoglobin level had been fairly stable at around 9.  Following the few occasional drops, I was treated with steroids which raised my blood back up again. My haematologist believes that I, in fact, have had CAD all the time.

About a year ago, I started getting swelling and pain in my finger joints that were initially treated with cortisone injections. However, the swelling is still there and one of my fingers has become quite deformed. I’ve seen a rheumatologist who says its some form of inflammatory arthritis, which may even be connected to the cold agglutinin disease. I have recently had a bone marrow biopsy where no serious abnormalities were found but my bone marrow is apparently working very hard.

I have been treated with methotrexate and azathioprine (separately) which have both caused great drops in my haemoglobin levels. I’ve been on prednisone again which is presently maintaining my blood. Just started my first course of Rituximab yesterday (May 2007). Had no reactions so far but very tired as my haemoglobin is still at only 7. Hoping the Rituximab works!

Has anyone else who has CAD also developed arthritis? Also, has anyone had any similar experiences with the methotrexate and azathioprine?

Katherine

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Ken’s story

Hi there, my name is Ken and I live in Sydney, Australia.
My wife came across the CADdy web sight while searching for information on Cold Agglutinin Disease after I was diagnosed with the disease some 12 months ago.

I had been showing some signs of aneamia with my doctor suspecting some internal bleeding.
An investigation into the possibility of internal bleeding and the repair of a fistula at the same time resulted in heart arhythmia occurring in the cold operating theatre. A visit to a Heart Specialist gave no indication of a serious heart problem but a resultant blood test had me sent to a Specialist Haematologist who immediately identified the problem as Cold Agglutinin Disease.
A survey of previous blood tests indicated that I had had the disease for some years but due to our relatively warm climate it was not obvious till the stay in the cold operating theatre and the resultant anaemia. I have had various tests including bone marrow but no obvious cause could be found for the disease.
I have had two blood tests since diagnosis which indicate that I am producing sufficient red blood cells to replace those that are destroyed as a result of CAD. Currently I am to have blood tests at six monthly intervals to check progress. There is no suggestion that I undergo any special treatment other than to keep warm and take 5mgm of Follic acid daily.

It is a pleasure to be able to look at one web site for the information on the disease and I will continue to monitor and will contribute to the site when appropriate.

Ken

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Ken’s story

I am Ken 73 years young, living near Worcester England.

I was first told I had cold agglutinins in my blood about seven year ago, I hadn’t been feeling well, no energy also lost some weight and my urine was very dark. So went to the Doc’s and had a blood test the results showed cold agglutinins in the blood, I can’t remember whether the Doc told me my haemoglobin count was down, all I can remember was he told me to wear a hat and gloves and try to keep warm. He said nothing at all about what could happen if I didn’t keep warm.

Having always been quite fit playing golf three or four times a week and table tennis in the local leagues, I didn’t take too much notice of the Doc’s advice and carried on as normal. I first knew something was wrong when playing table tennis I couldn’t get my breath and my legs didn’t want to move, I had never experienced feeling that tired before, but never associated it with the cold agglutinins. I played golf at the weekend and found it very difficult, just walking was a great effort my legs felt like lead and I was struggling to get my breath. Being a complete golf nut I carried on playing for a while, each time I played I had quite severe pains just above my eyes, thankfully they didn’t last too long, about forty minutes. I went see the Doc; he told me they were cluster headaches. I am sure they are connected with the cold agglutinins. Every time I go out in the cold even with a woollen hat and gloves I can guarantee I will get the pains above the eyes, it doesn’t happen when the temperatures are in the sixties.

In March 2003 my wife had quite a bad bout of flu, which being generous she passed onto me, I can remember my eyes being very painful and feeling very low and weak. We went to the Doc’s together and explained the symptoms; he said there was no point prescribing anti biotic as we were probably over the worst. The next day I was much worse, struggling to breathe and just about able to walk, my wife managed to get me back to my regular Doctor and he arranged for me to have a blood test, my haemoglobin count was 6 so he arranged for me to go into hospital that day.

I had lots of tests; the blood test showed my haemoglobin was still 6. I also had an endoscopy, a body scan and bone marrow test. At first they couldn’t find anything wrong, after a couple of days they told me it was cold agglutinin disease. I was told I needed to have a transfusion, the nurses giving the transfusion had never had to heat the blood before, and they eventually managed to get the heater and the blood to work together. I had two units of blood but that wasn’t enough to get to get my count high enough to come home. Because of the shortage of beds I was moved to another ward to have more blood, the nurse giving the transfusion didn’t know the blood had to be heated, I said to her I think the blood has to be heated she replied we never heat the blood. I asked if I could see a Doctor.
And explained the situation and after some while they managed to find a heater. I had two more units of blood, which brought the count up to 9.8
I was told they would like to have got the count to ten plus but that I would be able to go home next day.

I have been seeing a Haematologist on a three monthly basis where my blood count is generally between 9.3 and 10.1 it has never got above 10.1. I ask lots of questions, but always come away feeling frustrated that there is nothing I can do to improve my blood count. I take 5mg of folic daily. Having accidentally found your website whilst I was looking for rituximab as a treatment, I was thrilled to read your stories, and find that you and some other sufferers had tried rituximab, with it seems some very favourable results.

I first read about rituximab about 10 months ago but the findings were a bit scary, in the article I read it had been tried I think it was twelve cases, but there were two fatalities that happened within twenty four hours of treatment. I didn’t like the odds and generally with a blood count around 10 seem to be coping fairly well, so didn’t follow it up.

But reading your stories has been a real boost to morale. I have to see my Haematologist in July I shall take your stories with me and hopefully he will look in depth at the possibility of me trying rituximab.

I am very pleased to have found your website and would appreciate any feedback that you feel would be of interest.

Ken
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Marylin’s story

My name is Marylin.  I am a 58 year old married mother of seven grown children ranging from 40 to 18.  Until this past January I considered myself a very active healthy person who walked 3 miles a day five days a week, weather permitting.  I ate healthy and worked in a school setting as a paraprofessional assisting teachers in a classroom.  I have 14 grandchildren who all live within a 2-mile radius of our home.

I started getting chest pains and had difficulty breathing when I exerted myself in January of 2007.  I saw my family doctor who immediately had me see a cardiologist.  I, of course, couldn’t pass the stress test and the blood work showed I was severely anemic.  I was hospitalized for my first blood transfusion on Feb. 8.  My  HGB was 9.6 and Hct. 27.  A hematologist was called in and they took many tests.  The transfusion brought my levels up to 12.0/33.5.

Six days later my counts had dropped back down (9.2/25.8).  I was hospitalized for five days for observation while they treated me with salumedrol injections, took a battery of tests including CT scans and a bone marrow test.  I was sent home on the 21st with my levels at (9.7/27.4) During this time I was very weak, jaundice, and was not very clear headed.  I was on prednisone and not sleeping very good at night.

Two days later I was back in the hospital for my second transfusion, (2 units) My count had dropped to  (7.6 /20.9) Exactly one week later my counts had dropped back down to (6.8/18.8).  At this point they gave me 3 units of blood which brought my counts to (11.6/30.8).  I was beginning to think that I could only live with other people’s blood.   By this time we had received a diagnosis of cold agglutinin/hemolytic anemia.  They were warming my blood before transfusions and at my children’s request I was receiving directed donor blood.  They were also premedicating me with Tylenol and Benadryl before all infusions because of the itching reactions I had gotten from previous infusions.

Ten days later I was receiving my 4th transfusion.    During this time I obtained a second opinion from a hematologist in New Orleans.  He agreed that we should proceed with Rituxan treatments.   We had been waiting for over a month for insurance approval.  During the month of April I received four weekly Rituxan treatments with no reaction.  My levels since the treatments have remained around (9.5/27). They fluctuate a little but I have not needed any further transfusions.  It seems as though my body has adjusted and I can function at these levels.

I have not returned to work yet, but I plan to in early August when school starts again.  I have started walking again, though only 1-½ miles a day.  I can maintain my housework though at a slower pace.  At the beginning of June we repeated the CT scans and bone marrow.  The scans showed no change and the preliminary results of the bone marrow were good.

I am very thankful for this web site. After reading the stories of others, it reinforced what I had already begun to believe; that with a positive attitude I could live with this condition and still function in the world.  I have faith that all will be well.

Marylin

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Heather’s story

May I introduce myself as Heather. I am an Australian aged 30 with a father (72 years) that was diagnosed “officially” with Cold Agglutinins Disease some 5 years ago, but it was only at Christmas 2006 that we really understood how serious it can be if not monitored!

My father visited several doctors and specialists over the past 5 years at several major hospitals, and had a battery of tests undertaken and the results sent on to his (then) General Practitioner.  The GP relayed to my father only the details “you need to take iron supplements, and keep warm in the cold, and come back for blood tests every few months”. So for five years, my mother (a retired trained nurse) and myself assumed only that he was iron-deficient and needed to have three-monthly blood checks with his GP. We didn’t know the true diagnoses by name at that time, or its implications.

We kept asking that GP when he would do the blood tests, but he kept putting them off and off because of the time involved to do them in a general surgery. My father has a host of other medical problems as well as, not least of all several skin melanomas that had to be removed in day surgeries.

In December 2006, I had booked a flight to New York to visit my boyfriend and his family for a few weeks. Those plans changed a few days before I was due to leave, when my father stumbled in the early hours of the morning from his bed, yellow as a banana skin from jaundice and barely conscious.  My mother called an ambulance and they rushed him to hospital. He was soon admitted through the emergency ward and observed by a registrar, who diagnosed a Urinary Tract Infection. Because my father had an elevated temperature (39C), and was feverish, they removed his blankets and gave him cold water to keep his temperature down, as well as IV fluids and antibiotics to treat the urinary infection. They stuck him in a bed underneath a rotating fan.  All bad moves. Unfortunately, we had no idea what my father’s true diagnosis was.

When things didn’t improve and he started to go downhill even more, they transferred him to that same hospital’s aged care ward, where they undertook blood tests. They started to request more information from my mother and I, but we had so little to offer. We had never truly been properly informed. They kept him in the aged care ward that night, but by the time I went to leave at 8pm, he was shivering madly and complaining to me of being cold. When I asked the staff for more blankets, they told us it was the fever, and to not put too many blankets on him. I left him to go home to sleep, and went back first thing the next day. What hit me that day will stay with me for the rest of my life!

I arrived with my mother to  find his ward full of staff from the Medical Emergency Response Team (the team that gets called when the ‘red’ cardiac arrest button above a patient’s bed is triggered by ward staff). They had even assembled a photographic monitor above my father’s bed in the ward to take photos of his heart. I couldn’t see him from all the staff. When I covered my face in shock, I was sympathetically ushered out of the room. Tears are welling in my eyes even now as I recall that day 8 months ago.

They still couldn’t tell us what was wrong, other than to relay a host of figures about my father’s extremely abnormal haemoglobin count, and to ask if that had been the case before. We knew he had had an abnormal blood count, but that was all. My mother gave the staff the contact details for the other hospital where my father had had the battery of tests years before, and they got in touch.  It was that night, while my father was now declining in intensive care, that we were told the true diagnosis of Cold Agglutinins Disease. He required several blood transfusions that night, and had to be transferred to yet  another hospital’s intensive care unit, as the hospital he was in didn’t have the critical care capabilities to attend to him (such as a specialist Haematology Unit).  My mother drove in the back of the ambulance with him, and I took my car. I remember nothing of how I got there. It was after midnight only 24 hours after my father first stumbled up out of his bed at home, and we had been told to expect the worst. We were even asked whether my father and our family had ever considered life  support. We were told that if the blood transfusions and other measures  didn’t help, they may have to induce him into a coma to allow the body to heal, which is a type of life support.

At the second hospital, they had my father hooked to IVs going into arteries in his leg because the regular way (through the arm) was not fast enough. They had him wrapped in bandages to keep him warm, and placed him in a special bed that had heating pads lining it. He then had foil insulation wrapped around  his entire body (the type they usually reserve for mountaineers who get hypothermia). Being an Australian summer, the outside temperature was 39C. It was quite a juxtaposition.  I couldn’t see my father without wearing a face mask or a special apron because his body just wouldn’t cope with any more infection. I felt so removed from him, and from my earlier plans. I had been through a whirlwind of emotions, from the excitement only three days before, looking forward to seeing my boyfriend, who I was in a long-distance relationship with and hadn’t seen in six months, and now to shock and anger because I knew so little of my father’s illness before now.

The Haematologist my father went under at the second hospital was excellent. Although she had never come across a single case of CAD in her career, she knew what needed to be done. Thankfully, my father came out of intensive care within 3 days, but he went into a cardiology ward from there and spent his birthday, Christmas Day and New Year’s Eve in it. The episode of illness had left a strain on his heart and his pulse was hovering at times around 50b.p.m.  Consequently, once he was over the infection, he was transferred to yet another hospital to have a pacemaker put in his heart.

8 months later, we are much more aware of his needs. The first thing we did was change GPs. Perhaps there are only a handful of medical professionals who come across this illness, but I can’t help thinking what might have been spared had we been able to tell the staff on arrival at the hospital that my father has CAD.  Families and patients should and must be properly informed about the illness once a diagnosis is made. Waiting for a situation like ours to arise is too late.  My father now never leaves the house without winter warmers (scarf, beanie and hat).  People do occasionally laugh and it makes him self-conscious, and I do get angry because we went through so much and the have no idea why he is dressed in that way. But then I think life is so precious and I still have my father here eight months after  I thought I may not.

There are some good sides to every story, and mine is not without them.  My boyfriend proposed to me last month, personally travelling here from the US to do so, and to ask my father’s ‘permission’ in person.  All I want now is for my father to be well enough so that he just might be able to travel to the USA (NJ) next year in order to walk me down the aisle. Of course, it will have to be a summer wedding!!!!!!!!! I’ve given my father an incentive to keep wearing his warmers and staying well.

I am glad there is now a support group like yours out there where there is information and understanding.  We are also blessed from being in a warm climate like Australia, although my father is originally from Scotland, so moving here was the best thing he ever did! My father’s haematologist is keeping her eye on him and we have regular contact with a major hospital. Things are good for now, although I currently have the flu, so I can’t give my dad even a hug in fear of infecting him. Small things I used to take for granted I just can’t do anymore.

I have printed some information off on Rituximab, which my family haven’t heard of. My father was treated with Prednisone for a short while, but he is off that now. I don’t know if being allergic to Penicillin (as he is), would rule him out, but I will take the literature to my father’s Dr incase it can be of interest. Not sure if the drug is available in Australia.

Thank you for your time taking to read our story.

Kind regards

Heather 

Update from Heather 28 Feb08:

I thought I should pass onto you now that my father is back in hospital tonight after catching a chest infection from me. I feel terribly guilty that I passed it onto him. I picked it up from work (I teach) and it hit me hard enough, so I wasn’t surprised when dad started coughing and spluttering this morning.

We took him straight to the hospital and he has had an ECG, blood tests, chest x-ray, cold agglutinins antibody test, enzyme test, you name it. We took him to Haematology (our local hospital has a specialty haematology unit……how lucky! That’s where he goes every month for routine tests). Anyway, his haematologist wasn’t there today and they directed us to emergency to have him seen by a doctor. They told me in haematology to make sure that the emergency nurse pages his haematologist, so I passed on their request, but the very new doctor in emergency didn’t call the haematologist and dad was in a bed under an air conditioner for four hours with only a sheet over him. When I asked for a couple of blankets to warm him and explained why (not that they didn’t already know that he had cold agglutinins!), the surly faced nurse clicked her teeth and complained.   I blew my top eventually and left the emergency dept. and walked up to the haematology unit myself and demanded to speak to a Dr there. Better than that, they actually called dad’s haematologist who was driving her car and let me speak to her myself. Oh boy, did she see red!! She told me that she was so glad I had called her and said “good on you for defying everyone and having the courage to stand up to the nonsense”.  Within the three minutes it took me to walk from the haematology unit back to emergency, she had already phoned through and ordered that my father be given warmed blankets and put out of the blow of the air conditioner. We got no apologies, but at least  I put the surly nurse in her place (believe me I have nothing against nurses. My mother is one, and many of them today were excellent).  But there’s one thing I’ve realised is how little medical staff are actually aware of this illness. I don’t mind ignorance, but I can’t stand indifference! Last time my father was sick, my father ended up in the ICU and  I am determined that it won’t happen again if I can help it. I do feel especially bad because I put him there with my flu. I’m getting married in October and how I would love to have my father well enough to attend the wedding.

I hope that other people who find themselves in my situation will do the same and hold their ground. A couple of warmed blankets might not seem much to a surly nurse, but it means so much to my father

………My father was released yesterday from the hospital. All my hard work paid off, because the Haematologist ordered my father two blood transfusions over the phone the first night he was in (his count had dropped to 80 from 92 the previous week). You know, had I not insisted they call her, she would not have known my father was there nor heard his blood test results and then been able to do what she did. My dad was admitted under a cardiologist who he didn’t see until the morning after he was admitted (i.e. he didn’t see a specialist at all until after he had had the blood transfusions), and even after 24 hours in the hospital, his past admission file (with all the details of his ICU experience last year) hadn’t been sent to his ward. He was feeling much better the following day, and the Haematologist came down to see my day in the ward. She shook my hand and told me how impressed she was with what I did.

Email Betty if you want to contact Heather, and she will forward your letter

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Nina’s story

Hi. I am Nina, a retired high school counselor, from Fairbanks, Alaska.

I have had CAD since about 1998. It took me a number of years before I was properly diagnosed. It was actually by accident in 2000 that I finally found the proper diagnosis. I had been to my GP and a dermatologist and no blood tests showed anything abnormal and everyone was guessing why I was turning a mottled purple all over when I went out skiing and for winter walks. I have always been a very out-doorsy person throughout the seasons, kayaking, hiking, skiing and biking. Fairbanks is not exactly a great place for someone with CAD!! I hadn’t been to the local health fair in a few years but was having other blood work done yearly with my physicals. I thought maybe something would show up that we were missing and went to the Health Fair. Well, sure enough, the lab spotted the problem. I researched the note on the bottom of the lab results by the technician and immediately called my doctor with my diagnosis. She called a hematologist in Seattle to see what tests to run to confirm my diagnosis. Sure enough my titer was and still is 1:4096.

ninaI went through the routine most did of tests, including bone marrow biopsy, to rule out lymphoproliferative diseases. Nobody could find a cause, so here I was turning purple in Alaska and nobody having a clue what to do with me. There was no web site like this and as you all know, not much research and not many doctors with experience with CAD patients. You have to be your own advocate and study as much info as possible. My holistic doc and GP, working together to try everything to boost my immune system and minimize the effects of CAD finally decided it was time to see a specialist in Seattle. While consulting with the specialist, it seemed Rituxan was the only treatment that seemed to have a chance at impacting the disease. So in March 2004, I did my first treatment in Seattle to make sure I didn’t have any reactions, and then they let me continue my other 7 treatments in Fairbanks. I did 2 rounds of 4. It did bring my titer to 1:64. I was really excited because I had no side effects with the Rituxan.

About a year or so later my titer was back to where it started. Bummer! We decided to try another round of Rituxan and did another 8 sessions. This time with hardly any change in titer. With the price of Rituxan and unknown long term side effects, since this is a fairly new drug, I am leery to try again. Except for the titer being so high and turning purple, even at my computer when my hands get chilly, my other tests are good and I am able to keep my energy levels and red blood cell counts up most of the time. My hemoglobin is usually in the 14.2 range. Winter is definitely a struggle (which is most of the year in Fairbanks) but with nutritional supplements, good diet and warm weather vacations, I am able to make it through. The disease has been a tough mental battle for me. Bundling up and staying covered enough to not be affected by the cold is nearly impossible. I retired in May of 2006 and am hoping the retirement will reduce my stress load and help the medical condition, along with longer winter vacations in warmer climates. Alaska is such a special place and I have been here since 1976 but is definitely not a good climate for me so I know I will have to face the reality of my new life and move at some point. Huge lifestyle changes.

One CADdy asked about arthritis. I am starting to get arthritic changes in my hands and believe the CAD has contributed to that. Hard to know. I’m 56 and have led a very active lifestyle. All my joints have taken quite a beating!  But I do think that all the cellular changes and destruction that takes place in my hands and throughout my body when my blood hemolyzes, can’t be a good thing. I work closely with a Naturopathic doctor who looks at all the systems impacted by CAD and we work on my circulation, immune system, energy levels, liver and kidney function and blood changes. Since my case seems to be basically untreatable, I feel good about trying a holistic approach to managing the disease!

Thank you again for giving people a chance to see they are not alone, to compare treatments, to gain more information and make more informed decisions. I try to look to the bright side and realize I could have something much worse or to have a much more complicated case of CAD, as some have portrayed on the web site.

Update September 2017

It is now 10 years since my first CAD story.  I am 66.  I have been spending winters in Panama.  We just sold our house this summer and next Spring will be loading everything that fits in our new, small RV and travelling in the US, Canada and to other countries.  We will be in search of warm places to enjoy hiking and biking and the outdoor sports we love. Alaska is a huge part of who I am but is just not the right climate for me to stay active and healthy.  It is scary, sad and exciting leaving a huge part of my life behind and moving on to new adventures.  I know many CADdy’s are tormented with the thought of having to move and to change lifestyles to accommodate our disease.

My naturopathic doctor has been an amazing part of keeping me healthy.  After my last Rituxan in 2005 I have only used natural remedies and healthy diet, and lots of layers of clothing, to remain healthy.  My titre is still very high, I still turn purple very fast in the cold, and my blood clumps so fast that I drive the lab techs crazy when they have to draw my CBC’s! 

The arthritis in my hands is much worse and I believe this is from the CAD.  The hands are the most exposed.  Every time my hands turn purple My finger joints get worse.  I believe CAD causes much chronic, systemic inflammation in our bodies that we have to be careful of.

All in all I have been fortunate and been able to keep my major blood counts in the normal range. 

I expect my move to a warmer climate will let my body systems relax and heal a bit. I hope to have tons more energy!! I’ll let you know.

Good luck to the ever growing group of CADdy’s we have on this site. What a wonderful resource this is.

Nina

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Ann’s story

I am a retired school teacher, wife of Bill, mother of Will (37) and Janet (33), grandmother of Caitlyn (6) and Julia (2). [2007]
I am and have always been very active and constantly on the go. The photo is  Bill and I.

ann&billcloseI have osteoarthritis in both knees and was taking diclofen sodium for pain relief.  In a regular blood test in the spring of 2005, the lab reported a low hemoglobin count to my orthopedic doctor.  They called me to stop the anti-inflammatory medicine immediately and contact my general practitioner to find the cause of the problem.  After lots of tests, I was sent to Dr. Jerome Bart, hematologist/oncologist for Kelsey-Seybold in Houston.  He was the one who diagnosed Cold Agglutinin Hemolytic Anemia.  He said my red blood cells clump causing the anemia.  He  said cold  aggravates the condition  and for me to wear a hat and gloves in the winter and take folic acid every day He also said to come in every 3 months for blood tests.  I have done well since my diagnosis and continue to do my normal home, church, and baby-sitting activities.  I feel our warm climate in Houston, Texas, has really helped me avoid the extreme low hemoglobin counts.

My knees have gotten to the point that I am going in for surgery for a complete knee replacement. (September 2007)  My hematologist is cautioning the orthopedic surgeon that I must be kept warm.  The hematologist has ordered two units of warmed blood for me the day before surgery.

I really can say that I didn’t realize how serious cold agglutinin disease can be.  I also did not realize that it is an autoimmune response that kills red blood cells.  I am glad for this web-site so I can become better educated on this condition.

Ann

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Janice’s story

My name is Janice and I am 60 years old. I live in Belmont Massachusetts (a suburb of Boston) with my husband Larry, and our Shetland Sheepdog, Heschy. I am a piano teacher, an artist, and I love cooking and gardening. I have 2 children, 3 step children, and 1 grandson, born this past July (Solly – the piano player).

Janice and Solly at piano - largerI was diagnosed with Cold Agglutinin Disease in April of 2004. The months leading up to this were very difficult, involving the sickness and death of my father, and moving my mother (with dementia) up to Boston from New York. That autumn, my neighbor saw me leaving the house and told me that my cheeks and lips were dark purple. At the time, I was so busy with my mother’s move into an assisted living home that I didn’t pay much attention to it. As the weather got colder, I noticed that my hands were really starting to hurt, turning black, and were sometimes so cold that I couldn’t move them.

I thought that I had Raynaud’s syndrome and made an appointment with a rheumatologist at Massachusetts General Hospital in Boston. He told me that I probably had Raynaud’s but he needed to do blood tests to rule out anything else. That was when my nightmare began. My blood tests came back with reports of high levels of IgM antibodies, and my blood was coagulating before some of the tests could be completed. It was obvious after the 4th set of tests that I needed to see a hematologist. I am twice a breast cancer survivor, so the natural person for me to see was my oncology hematologist at MGH. In the next few months, I had scans which came up with nothing. I also had a bone marrow biopsy, showing no other cancer involvement. My diagnosis was then confirmed. My doctor called it ideopathic Cold Autoimmune Hemolytic Anemia. She told me that if I wasn’t going to move to a warmer climate, I needed to figure out how to keep warm. My symptom was only cold sensitivity, not fatigue from loss of red blood cells. The treatment offered to me was Rituxan. Having experienced chemotherapy drugs, I was not going to take it. I really wanted a different doctor to handle this disease, so my oncologist had me see another hematologist at MGH. He was very impatient and not very helpful in answering my questions, telling me that Rituxan is the treatment and if I wasn’t going to do it, he didn’t want to see me again. He also told me that this could possibly develop into lymphoma.

I then went to the anemia unit at Dana Farber Hospital. The doctor I met with was much kinder, gave me more time, but the treatment offered was the same. I came home feeling very discouraged. I then decided to see a homeopathic doctor. It was somewhat helpful mentally to do this, but after going for around four months, I stopped..

These are what my counts are now. They have not changed very much since 2004. The titer is sometimes lower than this.

Heschy cRed Blood Cells 3.04
Hemoglobin 11.4
Hematocrit 30.6
Cold Agglutinin titer, greater than 2048.

Last year I finally found a hematologist at Massachusetts General Hospital who gives me the time I need and is very understanding and supportive. I see him twice a year for blood tests. And to talk.

For me, the worse part of this disease is the limitation that it has placed on my life concerning my mobility and many of my interests. I am legally blind and am unable to drive. In the past, I walked to many places and also took public transportation. I can no longer stand outside to wait for the trolley in cold weather (now below about 55F), and it is very difficult to walk far in all of my layers of clothing, not to mention the comments I sometimes get. My husband and many friends help me, but I feel even more limited than I did before. Cooking and gardening have become quite challenging. My hands are always turning black and hurting but I will not give up these activities. On the positive side, I have made progress with my artwork, and last January I worked with a friend to design my first website.

I would like to end by thanking everyone who has contributed to this site. Reading all of your stories has been extremely helpful to me. The isolation I have felt over the last few years has increased, but reading your stories has made me feel less alone.

Janice

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