A Heartfelt Sickle Cell Tale by Gary Grist RN CCP Emeritus

I am sure that many of you have your own stories about sicklers. But please indulge me while I convey my story – “mea culpa, mea culpa, mea máxima culpa.” Many, many years ago the hospital I worked for had a large sickle cell disease (SCD) clinic. They wanted to start an exchange transfusion program. Because I had a lot of previous experience with venipuncture in hemodialysis patients and my experience with blood transfusions as a perfusionist, I was ‘volunteered’ for the project. I would be the hospital’s first hematology nurse, albeit only part time. My duties were to be performed only after my daily duties in the OR were completed. The hospital did not even have a real hematologist to care for the sicklers. Their care was provided by Stanley Hellerstein MD. Stan was a nephrologist who, in the absence of a hematologist at the hospital, had been caring for all the sickle cell children.  He had asked me to help him with exchange transfusions on children who had strokes due to their illness.  There was no way I could refuse since my experience and training made me the best candidate for the job.

The exchange program was set-up for SCD kids who had a prior crisis that resulted in a stroke.  Most of these kids were from about 8 y/o to 14 y/o and had hemiparesis in varying degrees. Most could still walk, but usually with foot dragging or with the aid of a cane or walker. Lowering the hemoglobin S levels by exchange transfusion was a priority to reduce the potential for another, possibly fatal crisis and stroke.

Initially I gave each child a BP cuff and told them to place it on each arm twice a day and pump it up for 30 minutes to 30 mmHg.  This was to stretch the veins in their arms to make them easier to find and take a large needle. This is a trick I learned long ago to help hemodialysis patients with peripheraal AV fistulas in their arms to help them “mature” so better blood flow could be obtained from them. I got 2 units of PRBC from the blood bank and spiked them to a blood warming IV set.  Then I started the IV on one arm with as large a cath as I could get in to administer the blood. In the other arm I started a blood donation bag which flowed down to a scale so I could measure the amount of blood being removed. I drew labs to assess the HgbS before starting. Hopefully if the child had been getting chronic transfusions, this value would be below a critical value set by Dr. Hellerstein,  I can no longer remember what that value was. As the “bad blood” flowed out of one arm, I pumped the “good blood” into the other one. Was there mixing?  Of course, that was impossible to avoid.  But the system seemed to work well enough to get their “S” values down.  How often did it need to be done?  That depended on the drop in HgbS values as reviewed by Dr. Hellerstein.  Initially, the procedure might need to be done weekly.  But later, with good results it might only need to be done bi-weekly or even monthly. The actual procedure was the easy part.

The hard part was talking to the kids during the procedure.  Most of the kids were exuberant about life.  They talked about school and their teachers and what they wanted to be when they grew up.  One patient wanted to play baseball professionally. Another wanted to join the Marines since his dad was a Viet Nam veteran.  Others wanted to be in the Olympics or be firemen.

Perfusionists and many ECMO Specialists do not usually commune in an intimate one-on-one way with their patients. This can be both a blessing and a curse.  As a hematology nurse, I met with each patient about every two to three weeks for a couple of hours. I got to know their faces, their smiles, their personalities and worst of all, their hopes and fears.  I say “worst of all” because those faces and hopes and fears are still with me today, 40 years later.

I realized that they did not realize that they would never achieve those dreams due to their disease.  Back then (40 years ago) , as still today, sick children are often told that they can overcome their disability and be whatever they want to be.  No one had ever told these SCD kids that since they had already suffered a cerebral vascular accident in the form of a stroke with hemiparesis, many of them would not live past young adulthood, let alone become a soldier or an Olympian. Those truths would become obvious as they grew older. But I was not going to be the one who spoiled their dreams. So I encouraged them as well, crying on the inside as I spoke to them.  Even now, as I write this, I still become emotional at the thought.

Today, sicklers have better treatment and more options than they did back then, at least I hope so.  But I am still plagued to know that I was performing multiple exchange transfusions before the hazards of transfusion acquired disease were as well understood as they are today.  This was before AIDS and before the risks of hepatitis B and C were well known.  Many of the children I transfused probably suffered consequences later in life from those transfusions.  I will never know for sure.

The method I used for sicklers on CPB up until the day I retired still involved a lot of transfused blood.  Is it better not to transfuse or make any special accommodation for sicklers and risk a severe post-CPB crisis? Or is it better to perform exchange transfusion at the initiation of CPB to reduce the risk of crisis but run the transfusion related risks? I don’t know! My CPB method for SCD did include exchange transfusion, simply because I was too much of a coward to recommend anything else, given my experience with sickle cell stroke patients. Fix their hearts but damage their brains; what kind of tradeoff is that?

There are certainly some perfusionists who pump SCD patients without any special management.  As I said earlier, which is riskier; not transfusing and not using hyperoxia and risking a post-CPB SCD complication or doing an exchange transfusion with hyperoxia and risking a transfusion complication?  I still don’t know the answer to that one, particularly when published studies support both positions 1,2. Hopefully in the years since I retired the “sickle cell patient on CPB” conundrum has been solved.  But I doubt it.

1. Hypothermic cardiopulmonary bypass without exchange transfusion in sickle-cell patients: a matched-pair analysis. Edwin F et al. Interact Cardiovasc Thorac Surg. 2014 Nov;19(5):771-6.
2. Perioperative considerations in a sickle cell patient undergoing cardiopulmonary bypass. Raut M et al. Indian J Anaesth. 2014 May;58(3):319-22.