BETTY USDAN - MY CAD JOURNEY
CAD PATIENT ADVOCATE
AMBASSADOR COLD AGGULTININ DISEASE FOUNDATION INC.
Following a bout of mycoplasma pneumonia in my early 30’s, I was mildly anemic with a low-normal hemoglobin trending between 11.3 and the low 12’s and was treated with iron supplementation. Since I was significantly uncomfortable from the cold, I left New York for Florida in 1975. Aged 37 and divorced, I thought I was going to my final resting place when I moved to Miami, then known for sun, surf and the aged, my parents among them. My anemia persisted even in the temperate climate so, finally in 2000, I was referred to a hematologist.
The doctor suspected internal bleeding, a likely cause for most anemias, and performed many sophisticated blood tests--the Coombs Direct and Indirect and Immunofixation Electrophoresis, GI testing–Colonoscopy & Endoscopy, a CT scan of my internal organs, and a Gallium X-Ray with radioisotopes of my Lymph nodes. PET scans are used instead today.
All tests were negative including a Bone Marrow Biopsy with the exception of the Coombs test which was positive. The diagnosis was chronic Primary Cold Agglutinin Disease, a form of AutoImmune Hemolytic Anemia. I was not iron deficient which had been the assumption in my younger years.
Following my initial CAD diagnosis, at age 62, I consulted with several other doctors who, unlike the first hematologist, were compassionate and reassured me that I could live a normal life-span with life style modifications—keeping warm at all times, no cold drinks or ice, being well rested, getting regular blood checks, and having only warmed transfusions and infusions.
In 2001, due to the dearth of information about Cold Agglutinin Disease and feeling quite isolated, I reached out to National Organization for Rare Disorders (NORD). I joined their networking group of ten CAD members and met my first friend who informed me about Rituxan. A gentleman from New Zealand contacted me through NORD and joined our CADdy Family, now three.
Primary CAD, is a low-grade clonal B-cell Lymphoproliferative disorder (LPD). Since I have Primary CAD, the possibility of transforming to active disease always weighs heavily on my mind as well as on the other patients with this diagnosis. Cold Agglutinin Disease must be considered a serious disease due to the possibility of thrombosis.
In 2002, I was started on Procrit, erythropoietin, a bone marrow stimulator for red cell production and continued for approximately seven years with weekly or bi-weekly self-injections to help raise my hemoglobin until my doctor stopped my usage due to the dangers listed in a Black Boxed warning.
As my disease symptoms progressed, and my energy was depleting in 2006 I was treated with two courses, eight infusions of Rituxan. I was infused again with eight in 2009-10 and six years later with one course of four in 2016. I have been very lucky to be a positive responder to Rituxan therapy, which is used off-label for Cold Agglutinin Disease.
I, like other CA patients, periodically experience a lowered hemoglobin when ill with a bacterial infection or virus and upon healing my hemoglobin improves. When my hemoglobin trends downward from mid-9’s and lower, my Cold Agglutinin symptoms are extreme fatigue, heartbeat whooshing sounds in my ears, breathlessness upon physical exertion, paleness and very rarely, hemoglobinuria-brown urine. I am happy that I can function well with a hemoglobin level of 10 or greater but do always have the fatigue factor.
In 2007, since there was a dearth of help for CAD patients, I recognized the need for an E-support website and founded coldagglutinindisease.org with the aid of the two CAD patients I had met through NORD. The goal was to provide information, support and comfort to those with this rare disease who felt as isolated as we did upon diagnosis.
The facebook.com group, CADdy CHATTER formed in 2009, is an outgrowth of the website, and has globally, nearly 1000 very supportive CAD patients and caregivers who share CAD information and concerns.
In 2019, Cold Agglutinin Disease Foundation Inc was created as a non-profit, 501(c)(3) corporation to foster and increase public awareness and education regarding the diagnosis, management and treatment of CAD.
I continue my efforts to help the global Cold Agglutinin Disease Community as a CAD Patient Advocate and an Ambassador to the CADFoundation. Considering how debilitating this rare hemolytic anemia can be, I am delighted that I am managing quite well, thus far.