Treatment - Sickle Cell Anemia
Pain with SCA
My Experience as a RN
When I was in college studying to be a RN, one semester I was training in the pediatric area of a hospital. One child I was assigned to care for was an African-American boy, who was about 7 or 8 years old, and he had Sickle Cell Anemia (SCA). He was sitting on the floor playing with some little cars and trucks in the children’s playroom.
I was watching him and noticed some tears running down his face while playing, but he didn’t make any noise. This little fellow wanted to play, but he was in so much pain he really could not play at all. It broke my heart, as I am the mother of 3 boys, and I started to cry.
I learned two things that day:
- Sickle cell anemia is a horrible, painful disease
- I could not be a pediatric nurse.
Crying as a nurse when taking care of children is unacceptable!
Facts About Sickle Cell Anemia
In the United State there are 70,000-100,000 people with sickle cell anemia, which is an inherited disease. One out of every 365 African-Americans have SCA, 1 out of 16,300 Hispanic-Americans has this disease.
If each parent has a sickle cell gene, then a child will likely have this disease. If only one parent has only one sickle cell gene, the child will not get the disease. This disease can be easily diagnosed with a blood test. The earlier the diagnosis, the easier it is to avoid complications. The blood test is done on newborns in every state.
Red blood cells are round and normally flow easily through the blood vessels carrying oxygen, but with sickle cell anemia the red blood cells have abnormal hemoglobin. This causes the red blood cells to become stickier, and they are formed into a crescent shape.
These abnormal red blood cells can stick to the blood vessel walls, which can damage the lining. This situation can attract defensive cells that may enhance the stickiness and lead to actual slowing of normal blood flow and partially blocked blood vessels. This is painful, and it reduces oxygen delivery to organs and tissues.
Normal red blood cells live an average of 120 days, and sickle cells die after 10-20 days. This causes the anemia. Bone marrow cannot produce blood cells quickly enough to replace those that are dying.
Sickle Cell Disease
Who Gets Sickle Cell Anemia?
This genetic disease is most common in families that come from the following areas:
- South or Central America (especially Panama)
- Families with ancestors from sub-Saharan African area
- Caribbean islands
- Mediterranean countries (Greece, Turkey, Italy)
- Saudi Arabia
Sickle Blood Cells
Symptoms of Sickle Cell Disease
he symptoms are mild in some patients and serious in others, requiring hospitalization in others. Anemia is the most common symptom, and constant pain is located in the bones.
Some of the other common symptoms include:
- Shortness of breath
- Cold feet and hands
- Chest pain
- Leg ulcers that do not heal
- Jaundice, eyes and skin yellowed
- Pale skin due to the anemia
The pain is termed “sickle cell disease.” When anemia exists over a long period of time it will cause damage to organ;, including the heart, liver, spleen, brain and kidneys. Other serious complications include: stroke, blindness, gallstones, bacterial infections and priapism.
Gene Therapy and Sickle Cell Disease
Medication treatments for sickle cell anemia include:
- Children begin taking penicillin at 2 months until 5 years of age to prevent pneumonia.
- Children’s vaccinations are essential to avoid infections.
- Adults who had their spleen removed or have had pneumonia take antibiotics throughout their lives.
- Pain medications are given during a sickle cell crisis.
- Hydroxyurea (Droxia, Hydrea) taken daily reduces the number of crisis, but may result in more infections.
Endari is a new medication, and the first one to treat this disease in twenty years. This drug is given to people 5 years and older to treat the serious complications of this disease.
The only cure for sickle cell anemia is a bone marrow transplant, used for the most serious cases on children under 16 years of age. This procedure has serious risks. It requires a sibling on someone else that can donate stem cells for the patient.
Ways to Help Prevent SCA Crisis
There are some ways to help prevent the crisis, which include:
- Drink plenty of water
- Avoid exposure to high altitudes, or area with lower oxygen levels
- Do not get too hot or to cold
- Get enough rest
Change Me - Ruben Studdard - Supporter of SCA Research
One study of 2764 patients between birth to 66 years of age found that patients with SCA died by 60 years for men and 68 years for females. An organ failure accounted for 18% of the deaths, which was typically renal failure. An acute SCA crisis accounted for another 33% without organ failure. Another 22% had a stroke.
The Sickle Cell Disease Association of America (SCDAA) is a non-profit organization that supports research, education and funding for individuals and even families.
This genetic disease is very difficult to treat as the only way to eliminate the disease is stem cell replacement, which is a risky, difficult procedure. There is more success now, and hopefully the future will result in more ways to treat this very difficult disease.
This content is for informational purposes only and does not substitute for formal and individualized diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed medical professional. Do not stop or alter your current course of treatment. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.
© 2019 Pamela Oglesby