Refractory Anemia with Ringer Sideroblasts (RARS)
If someone were to ask me what transfusions mean to me, I would say the short answer is that transfusions allow me to live a more normal life.
In 1990, at the age of 47 I was diagnosed with Refractory Anemia with Ringed Sideroblasts (RARS), a form of Myelodysplastic Syndrome (MDS). During the 80’s I had become increasingly tired, and my General Practitioner had checked me for all the more common causes of my anemia. A Hematologist performed a bone marrow biopsy, and concluded that my problem was definitely in my bone marrow, and not anything as easily cured as iron deficiency.
I asked my doctor what I could do, and he said, “There is nothing you can do.” He said, “You will learn to live at 2/3 speed, and you will find that anemia is a powerful sap to ambition.” At that time my hematocrit was around 25. A normal HCT for a woman is 35-40, and for a man is 40-45.
I decided immediately to take care of myself as much as I could, and to control the things that I can control. I exercised as much as possible, and worked on refining my diet. As a result, my “usual indicators” of blood pressure, cholesterol, pulse, and BMI are excellent. I do believe that diet and exercise contribute to stamina, strength, and a general sense of well-being.
My Hematologist suggested that my three brothers and I should be tissue-typed in the event that I might need a bone marrow transplant. Two of my brothers are potential donors.
In December, 2000, I went to Dana Farber in Boston for a consult. At that time my HCT was 23. The Hematologist at Dana Farber declared unequivocally that I was too old for a bone marrow transplant. He suggested a trial of a combination of Neupogen and Procrit, and also that I should consider receiving blood transfusions.
Since early 2001, I have been on four different drug trials, and none of them have significantly improved my blood counts. Some have had unpleasant or harmful side effects.
Blood transfusions are the only medical treatments that have benefited me by restoring my hematocrit and hemoglobin counts to more normal levels. I go into a transfusion with a HCT of 23 or even 19, and a few days later my HCT is between 29 and 33.
Recently a nurse asked me what it felt like to be so anemic. The short answer is that I feel lousy all the time. I have to compensate by concentrating on what I’m doing. Even after a good night’s sleep, I don’t feel like going back to bed, but I don’t feel like doing very much. I have to pace myself to get routine work done, and I have to plan and choose my activities. I get short of breath easily. I feel cold, especially in my face and hands. The ringing in my ears is constant. My eyes get puffy.
Worst of all, severe anemia is limiting; it forces some retreat from the world, because interaction takes energy. A friend of mine who was anemic for a short time said, “I thought I was depressed! It turns out my HCT was 18!” I find that it takes some thinking to sort out the possibility of depression from the effects of anemia. Most of the time I feel that some depression is a consequence of not being able to do all that I’d like to do.
Will power and enthusiasm can help, but only for a little while. I can try to live at a normal pace for only a short time. Then I begin to notice that I have to concentrate to do even simple things: standing up, or even carrying on a conversation. A few times when I have been exhausted, I’ve lost my balance and nearly collapsed.
During a transfusion, when the nurses connect the unit of blood to my IV, I say a “Thank-you” to the person who gave blood. I remind myself that this person took time out of their day to make this possible for me. I consider that when I’m out in public, any person I see could have been this wonderful donor, and I resolve to try to be more generous to others.
The first change I notice is that sensation comes back to my cheeks and lips. It’s like a blush. Then I notice that vision takes less effort; I can see more easily. I go home and take a nap, and then the next day I feel more energetic.
The biggest change is that I can go about my business without thinking about it so hard. I can include more activities, become more spontaneous, and all-round enjoy life. I can be myself in the world of normal people.
Until research comes up with a cure, transfusions are the gifts that make this possible.
South Burlington, VT
Red Cross blood donors help patients just like Jennifer in cities and towns throughout New England and across the United States. Please….give blood.