stress. We all have stress in our lives, unexpected challenges happen, and we must adjust accordingly.
For sickle cell patients that could mean a visit to the emergency room. “When something happens, try to remain calm and try to reduce emotional stress,” says Dr. Hsu.
“A lot of the things that can change blood flow is the stress response when you get an adrenaline rush from excitement, fear or worry, that tightens up the blood vessels and tightened blood vessels don’t allow good blood flow. That helps to trigger sickle cell pain episodes.”
Dr. Hsu has known sickle cell students who have done well with their sickle cell pain during high school, but once they went off to college, the demands and stresses of college life created a crisis for students.
There was also a bride who was so tired and stressed on her wedding day, she had a crisis and had to go to the hospital on her wedding night because of sickle cell pain.
Even planning a birthday party can land the sickle cell patient in the bed with serious pain as it did with Bridget Reynolds, a Chicago area sickle cell patient. “I had two [crises] recently. I had one on my birthday and I think it was physical stress related because I had to do a lot of running around [preparing for my birthday dinner]. I had to order the food.
Then my car stopped on me. I had to wait in the parking lot in the hot car for my sister to pick me up. When I got home, I prepared the cake, did the cleaning, and at the end of the day when I was finishing dinner, my leg began to hurt. I knew it was going to be breakthrough pain. I immediately stopped what I was doing to take some pain medicine and went to bed.”
So, the importance of managing the sickle cell crisis cannot be understated. Trying to adjust your lifestyle so that it is at an even keel would help to reduce sickle cell pain, says Dr. Hsu. There is no specific data on what particular foods you eat that will minimize the pain but having a balanced diet with fruits and vegetables that have antioxidants will help.
Antioxidants in colorful vegetables will help reduce the oxidant damage of sickle cell. There is a small amount of data that fish oil, Omega3 fatty acids, that is found in fish and nuts helps. More protein, zinc, and vitamin D are helpful. The research on this is still a little fuzzy, admits Dr. Hsu.
Once, all you had was lifestyle changes, but in 1998 the Food and Drug Administration (FDA) approved Hydroxyurea (originally developed for cancer treatment) for sickle cell disease by reducing the severity of sickle cell pain.
It reduces the chances of admission to the hospital and pain by half. It reduces the chance of acute chest syndrome by half and cuts down on the need for blood transfusions. It improves your ability to avoid missing school and work and preserves IQ from diminishing with age.
In the last three years, there have been three more drugs approved. The aforementioned Adakveo and the following:
Endari (L-glutamine) is an oral L-glutamine is an oral therapy for sickle cell disease for adults and children over 5 years. It reduces antioxidants produced by glutamine that helps reduce the stress on cells caused by the disease and allows the cells to regain the flexibility needed to carry oxygen to the tissue throughout the body that was FDA approved in July 2017.
Oxbryta (Voxelotor) is an oral therapy also approved in 2019 by the FDA and helps to restore normal red blood cell function and oxygen delivery.
These new drugs affect sickle cell disease differently and show great promise for sickle cell patients to have a more improved life. Depending on the patient, these drugs are given with or without hydroxyurea.
Over the years, managing sickle cell disease and the pain associated with it was limited. Now, new drugs are here and showing tremendous promise in sickle cell patients having a life with minimal pain.
The way everyone can help sickle cell patients is through blood donations. Blood donations are always needed. Contact your local American Red Cross or donation center.