My daughter recently had her second Trans-Cranial Doppler (TCD) test.The TCD is an ultrasound used to monitor the blood flow in the blood vessels of the brain to check whether or not the patient is at risk for a stroke. The test is completed once a year in most sickle cell patients. Thankfully, the we received the results the same day which confirmed that our daughter is OK.
Why Test for a Stroke Risk?
According to the Sickle Cell Information Center
Strokes are very uncommon in childhood, but sickle cell disease is one of the few conditions associated with a higher possibility of strokes in children. Stokes occur in about 5 to 10% of children who have sickle cell disease (SS) or one type of sickle-beta thalessemia disease (Sb 0). In other words, we can expect that out of 100 children with sickle cell disease, about 5 to 10 may be at risk for having a stroke before the age of 15. This is 300 times more likely than in children without sickle cell disease.
In addition to receiving her TCD she had her yearly appointment with her hematologist as well. The appointment was very encouraging and I learned lots of new information as well. Two particular things stuck out in my mind.
1. Sleep Apnea. Children with Sickle Cell can be at a greater risk for having sleep apnea, so it is important to monitor their sleep. For example if your child is having trouble breathing and snores then wakes up to a gurgling sound like he or she is having trouble breathing. Take note and let your doctor know about it. Navid Mostofi, MD, a neurology fellow, at Temple University, Philadelphia, Pennsylvania, states “that sickle cell patients should be routinely screened for OSAS. [which is Obstructive sleep apnea syndrome and can put children at an increased risk for having a stroke] (Source). In order to confirm the sleep apnea diagnosis the patient will need to have a sleep study conducted and if the findings are positive for OSAS it may be necessary for him or her to receive a Adenotonsillectomy (a surgical removal of tonsils and adenoids), which could also require a blood transfusion when children with Sickle Cell undergo surgery. When children with Sickle Cell are scheduled to have surgery, they are placed in a vulnerable situation, so be aware of changes and systems in your child, but be very cautious about wanting a surgical procedure conducted, because they are best avoided.
2. How to detect a Sickle Cell Crisis. Recently, my daughter had a knee incident that worried me. She woke up early in the morning and screamed “My leg hurts!” I felt her leg and noticed her knee had a little bump and some fluid. Over the next couple of hours she preferred sliding across the floors instead of walking. She acted as if she didn’t want to walk at all. That night I gave her a warm bath and put her to sleep. When she continued acting like it was too painful to walk on the second day, I booked her an emergency appointment with her pediatrician. When we went to the doctor, her knee was no longer swollen. Of course, I was extremely concerned, but when the doctor asked her to walk on it, she hesitated, but continued walking to the door. Then, the doctor told me, she believes my daughter must have bumped her knee and forgotten about it. Then I remembered that she did wake up that night to use the rest room and possibly bumped it. Thankfully, her knee recovered well, but it led me to ask, “How would I know, if she was having a crisis?” The reply was the pain would be unbearable. My daughter’s hematologist also explained, if a child with SCD has unexplained swelling, joint pain, etc. it could be a crisis, just not a severe one. Some less severe crisis can be relieved by a warm bath, Motrin, and rest. The key is “unexplained pain.”
Over all the doctor reminded us to continue giving her plenty of fluids. The typical recommended amount for a person is the person’s body weight divided by half in ounces. So for us, I need to make sure that my daughter receives at least 12 ounces of water a day.