By Babatunde Jose
Nobody knows the trouble I’ve seen; Nobody knows my sorrow; Nobody knows the trouble I’ve seen; Glory, Hallelujah – Song by Louis Armstrong
Sickle cell disease (SCD) is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a “sickle.” The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications (health problems) such as infection, acute chest syndrome and stroke.
People with sickle cell disease start to have signs of the disease during the first few months of life, usually around 5 months of age. …And that is when the nightmare starts for parents. Particularly those that have no idea of genotype or blood related disease.
It’s a most harrowing experience, even for knowledgeable parents as they are helpless. Parents can only empathize but cannot feel the excruciating pain of the patient. In the infant stage of the affliction, parents keep sleepless nights depending on the frequency of the crisis. Crying, wailing, and weeping rent the air all night and they eventually end up in the emergency department of the hospital, where they ultimately become familiar faces.
Yet, that is the beginning of their life challenge as SCD is a disease that worsens over time. No two cases are the same in terms of severity; though, treatments are available that can prevent complications and lengthen the lives of those who have this condition; their life usually hangs on the will of God. Sometimes a simple crisis might not only be life threatening but life taking. My daughter and an unknown lady were both on admission, same evening at Redington hospital for the same Vaso-occlusive crisis. The lady dropped dead in the morning just as they were about to be discharged.
Sickle cell disease is a lifelong illness. A bone marrow transplant is currently the only cure for sickle cell disease. Gene therapy is also being explored as another potential cure.
As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms, and preventing complications. People with sickle cell disease face many challenges, including severe pain episodes, stroke, and organ damage, including adverse side effects of drugs, like hydroxyurea.
The drug Hydroxyurea, when taken daily reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations.
Hydroxyurea, however, exposes the patient to increased infection as it lowers the number of white blood cells in the blood. It can also lower the number of platelets which are necessary for proper blood clotting. And there is some concern that long-term use of this drug might cause problems later in life for people who take it for many years.
According to Mayo Clinic there are certain precautions you can take, especially when the blood count is low, to reduce the risk of infection or bleeding:
· Avoid people with infections. Check with your doctor immediately if you think you are getting an infection or if you get a fever or chills, cough or hoarseness, lower back, or side pain, or painful or difficult urination.
· Check with your doctor immediately if you notice any unusual bleeding or bruising, black, tarry stools, blood in the urine or stools, or pinpoint red spots on your skin.
· Be careful when using a regular toothbrush, dental floss, or toothpick. Your medical doctor, dentist, or nurse may recommend other ways to clean your teeth and gums. Check with your medical doctor before having any dental work done.
· Do not touch your eyes or the inside of your nose unless you have just washed your hands and have not touched anything else in the meantime.
· Be careful not to cut yourself when you are using sharp objects such as a safety razor or fingernail or toenail cutters.
· Avoid contact-sports or other situations where bruising or injury could occur.
“Using this medicine for a long time may increase your risk of developing leukemia (cancer of the blood) or skin cancer.
“While you are being treated with hydroxyurea, do not have any immunizations (vaccinations) without your doctor’s approval. Live virus vaccinations (eg, nasal flu virus vaccine) should not be given while you are using hydroxyurea.
“This medicine may increase your risk of having lung or breathing problems (e.g., interstitial lung disease). Check with your doctor right away if you develop a fever, cough, or trouble breathing while using this medicine.
“The results of some tests (e.g., continuous glucose monitor) may be affected by this medicine.
“Talk with your doctor before using this medicine if you plan to have children. Some men who use this medicine have become infertile.
“Do not take other medicines unless they have been discussed with your doctor. This includes prescription or nonprescription (over-the-counter [OTC]) medicines and herbal or vitamin supplements.
“Using a special ultrasound machine (transcranial), doctors can learn which children have a higher risk of stroke. This painless test can be used on children as young as 2 years. Regular blood transfusions can decrease stroke risk.
“Childhood vaccinations are important for preventing disease in all children. They’re even more important for children with sickle cell anemia because their infections can be severe.” These are some of the directives of Mayo Clinic on use of Hydroxyuear.
Blood transfusions carry some risk, including infection and excess iron build-up in the body. Because excess iron can damage the heart, liver and other organs, people who undergo regular transfusions might need treatment to reduce iron levels.
The two gene therapy treatments for sickle cell disease recently approved by the FDA in the US, called Casgevy and Lyfgenia, cost $2.2 million and $3.1 million per patient, respectively. Definitely beyond the reach of the average sufferer.
Sickle cell anemia can make life more difficult, particularly for a child, who will need to deal with delayed sexual maturity and stunted growth. You will need to avoid things that can cause a crisis, such as certain medication that restricts blood vessels, high altitudes, and strenuous exercise.
As people with sickle cell anemia grow older, they may develop different and more serious medical problems that happen when organ tissues don’t receive enough oxygen. People with sickle cell anemia are at increased risk for stroke and lung, kidney, spleen, and liver damage.
The leading causes of death in sickle cell diseases are infection, pain episodes, acute chest syndrome and stroke. Death can be sudden and unexpected in sickle cell anemia. Vaso-occlusive crisis is one of the commonest presentations and a leading cause of death.
The pain of a Sickler child is the agony of parents who are helplessly left to witness their child writhing in pain during a crisis, spending endless hours with them in the hospital during frequent bouts of admission and sometimes having the ill-luck of watching them die.
The pain of having a Sickler is well known and cannot be overemphasized. It is this threshold of unbearable bouts of pain that made my daughter to pen the following preamble to the NGO she is currently setting up: THE CRESCENT INITIATIVE for Sickle Cell, to empower those living with Sickle Cell Anemia:
“Two years ago, I sat down on the floor in my bathroom crying and thinking about the quickest method to end my life. I had had a painful episode that went on for several days and I was feeling hopeless.
“My pain score felt like 100/10 and my body felt like I had been hit by a bus. The pain got so bad that I could feel it in my fingers, and I could feel the floor under my feet. Due to the severity of my pain and my low blood level, I had to have a blood exchange. Managing the pain was too much for me and it ate away my faith, my confidence, and my will to keep on surviving. I was tired, my life seemed hopeless, and it was unfair that this kept happening to me.
“I cried for several hours recalling all I had gone through…several needle pricks just so that they could find a vein; my foggy memory, a side effect of the medications; and my long absence from work which had started to become “an inconvenience” to my manager and a sign that I might be losing my job.
“It was too much….it was unfair…. I did not want to be here anymore. I cried for several hours and decided to call my therapist whom I had just started seeing.
“Just hearing his voice made me break down again and in between sobs, I explained what I was feeling. He calmed me, listened to what I was going through and tested me for depression which the test confirmed. Since then, I’ve been taking antidepressants and seeing a therapist regularly.
“During my sessions, I began to notice an improvement in how I felt and discovered that some of the issues I thought were unique to me were actually common for individuals with sickle cell. For example, I became aware that my emotions and stress levels could trigger my pain episodes, which, coupled with anxiety, attacks became more frequent.
“Learning coping strategies and positive affirmations helped me find balance. Through opening up to my therapist and later my hematologist, I realized that many individuals with sickle cell disease could and should benefit from mental health therapy, though it is often not included in their treatment plans.
“Therapy was transformative for me, and I knew other warriors would also benefit. After thorough research and consultations with healthcare professionals, I decided to establish The Crescent Initiative for Sickle Cell.”
Fortunately, in everything, we always have cause to thank God. (Quran 16:53) and also (Quran 16:78)
We thank Almighty Allah for preserving the life of our daughter Asia Atinuke Jose (AJ) as she clocks 45 next week, May 8 and still counting, In Sha Allah.
Allah has found it pleasant to preserve her despite the Sickle Cell challenges. We also pray for other afflicted souls. May it please Allah to make it easy for them. For all those who have walked this path with us this far, I say a very big thank you.
Barka Juma’at and a happy weekend.
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