I am the mother of three, including a set of twins, one of which has sickle cell. In honor of September’s sickle cell awareness month, we share our story.
The disorder runs on my father’s side of the family and I lost two older brothers from sickle cell complications. I learned that my youngest daughter had sickle cell from her newborn screen. After the initial shock and confusion (My ex husband and I were unaware that he carried the trait although we were aware that I had it) I did everything I could to become educated on the disease.
Sickle Cell is a blood disorder that causes the red blood cells to change from their normal pliable circular shape to a sickle shape. These cells are sticky and can become stuck in blood vessels, leading to numerous complications, one being severe pain in the area of the sickling.
There are different forms of Sickle Cell:
Hemoglobin SS Disease:
Hemoglobin SS disease is the most common type of sickle cell disease.
Hemoglobin SC Disease
Hemoglobin SC disease is the second most common type of sickle cell disease.
Hemoglobin SB+ (Beta) Thalassemia
Hemoglobin SB+ (beta) thalassemia affects beta globin gene production. Symptoms are not as severe.
Beta-zero thalassemia is the second type of beta thalassemia. It is associated with a poorer prognosis.
People who only inherit a mutated gene from only one parent are said to have sickle cell trait. They may have no symptoms or reduced symptoms. To learn more about the different types please click here.
My daughter has a combination of SC and Thallasemia. We had our first real experience with the disorder when my daughter spiked a fever of 103 degrees. Anything over 101 degrees is an automatic emergency room visit. This is because people with sickle cell have a compromised spleen and cannot effectively fight off bacterial infections. If left untreated it can be fatal. She was 7 months old. Luckily it was just a virus, but it took five days in the hospital to make sure. This began a series of emergency room visits and hospital stays due to fevers, thankfully all viral, but extremely stressful nonetheless.
She had her first pain crisis at the age of three, a couple of days before she started preschool and a few days after divorce fillings. Although not understanding exactly what was going on, I immediately focused all of my energy on doing everything within my power to make sure that my child was okay and well taken care. The differences her father and I had were irrelevant. Our daughters health superseded that and we behaved as such. After an emergency room visit, blood draws and an I.V. of morphine, we were released and began home-care. Here ensued a month of around the clock pain med distribution: codeine, then ibuprofen, every three hours, 24/7. Family members had to relieve me so I could go to work. But the hardest thing was seeing my child in pain and feeling helpless, hearing my child cry out every time she changed positions, watching her unable to walk as the pain was centered in her lower back, taking her to the doctor to see if she would need physical therapy in order to walk again, being strong for her. It broke my heart. And through the intense, non-stop blur, while somehow meeting the increased needs of my other two children; we made it through.
Things were quiet for a couple of years, a few flair ups here and there but all manageable at home. Then another pain crisis hit, a bad one, we were trying our best to manage at home when the the night before her 5th birthday we found ourselves in the emergency room: another 103 degree fever. I packed up my three girls and off we went. With the support of her sisters my little one endured another round of pokes while she had blood drawn and an I.V. inserted. We waited. My mother picked up my other two girls and we continued to wait. The blood cultures came back clean but she was still in pain. With instructions to keep doing what I was doing and to immediately return if anything changed, we were released around midnight. YAYAYAY she would not have to spend her entire birthday in the Hospital! We went home!
And we did our best to make it a good day! We went to Build A Bear, her and her twin got their ears pierced (BIG GIRLS) and there were moments when she laughed and smiled but I could tell that my baby was not feeling well. So we skipped the Cheese Cake Factory, grabbed food and went home.
But she was getting worse. Lethargic and pale she got home and immediately laid down. She complained that her stomach hurt. Concerned, I checked her tummy. It was hard and tender to the touch, I was pretty sure that her spleen was enlarged. In people with sickle cell, the spleen can become enlarged when cells become sickled and trapped inside the spleen causing it to get bigger and push on other organs causing pain. If left untreated it can burst and be fatal. This condition is called splenic sequestration. The best defense when dealing with sickle cell or any illness is to be as knowledgeable as possible. My awareness put me on high alert and I reacted immediately.
Back to the emergency room we went and she was admitted. I was right, her spleen had enlarged. Three days and a blood transfusion later, she was released. A blood transfusion is a routine procedure done for Sickle Cell patients to push out the sickling cells and replace them with normal cells. The transfusion in this case played two roles, it helped to push the sickling cells out of the spleen so that it could go back down and it replaced the sickling cells in her back to help ease her pain crisis. This was her first transfusion and I was apprehensive but it worked and I am Thankful. However, if her spleen enlarges again they will recommend removing it because the more often it enlarges the higher the risk of it erupting.
We left the hospital three days after her fifth birthday, a little disappointed in how things went but very grateful at how they turned out. It wasn’t the best birthday but it was definitely blessed.
For those of us taking care of someone with a chronic illness or perhaps dealing with one ourselves, it is of the utmost importance that we take care of ourselves physically and mentally. Care taking is the number one role susceptible to burnout.
During this time I never stopped and honored myself, I was constantly running on empty and after months and months and months of neglecting my own needs I hit a wall. I was depleted, there was just nothing left to give, to anyone. I knew I had to do something, so I implemented a few things. I began walking the hospital grounds when my child was admitted. Once she was comfortable and asleep I would notify the nurses’ station and go for a 30 minute walk. I talked to my therapist regularly. Having someone to talk to, an outlet, was indispensable. I also began dedicating one day a week to myself (as much as possible as a single mom of three children) where I didn’t go sit with my dad who has dementia, where I didn’t take too many calls, where I didn’t obligate myself to be available to others: I catered to me. This is an extremely important and often overlooked task of most caregivers: self-care.
In order to continue to pour into others we must regularly and consistently replenish our reservoir so we can continue to do good work. We are all worthy of being holistically well.
Feeling ALL better!!!