Sickle Cell Crisis
Introduction
If certain conditions develop in a person with sickle cell disease, the person must contact a physician. If the physician is not quickly available or cannot see the person right away, the person with sickle cell disease may choose to go to a hospital's emergency department. Contact the physician in the following cases:
- Many people with sickle-cell disease have pain with enough frequency that they need to take pain
medications at home. If the pain is unrelieved by the medication, or the pain is significantly different from previous episodes, contact the health care provider.
- If experiencing nausea, vomiting, and diarrhea ; losing a lot of fluid; and having inability to drink and keep it down, the person with sickle cell disease is in danger of becoming
dehydrated. This is a serious concern with sickle cell disease. The physician or the hospital may provide IV fluids to replace the lost fluids.
- It is important to control infection. If it appears that a person with sickle cell disease is getting an infection, even if using antibiotics to prevent infection, contact the physician immediately.
It is important to control infection. If it appears that a person with sickle cell disease is getting an infection, even if using antibiotics to prevent infection, contact the physician immediately.
- Go to a hospital's emergency department if these conditions develop:
- Uncontrollable pain even with the use of narcotics
- Continued loss of fluid leading to dehydration (if vomiting)
- Uncontrollable fever
- Chest pain or
shortness of
breath
- Severe abdominal pain
Self-Care at Home
Even tiny changes in the red blood cells can begin a cascade of symptoms leading to a sickle cell crisis. Therefore, home care, even when a person is careful about drinking plenty of fluids and avoiding infection, is difficult. The best home care is understanding the illness and knowing when and where to seek immediate medical care.
Other Therapy
- Chronic therapy: New developments in regularly scheduled transfusion therapy have shown promise in decreasing the following:
- Bone marrow transplantation holds promise for a very small percentage of people with sickle cell disease. Discuss this with the physician.
Considering the many body systems involved and the likelihood that sickle cell crisis will occur time and time again, strong consideration should be given to follow-up with a hematologist (a physician with a specialty in treating blood disorders).
Most uncomplicated cases of sickle cell crisis can be treated in community emergency departments. People with this condition can be safely sent home when their pain is under control and their dehydration is eliminated. A short observational period in the emergency department helps to prevent acute relapse and admission for pain and rehydration.
Outpatient treatment centers on the control of infection, reduction of pain, and prevention of
dehydration. The use of narcotics is often necessary and should not be limited for fear of turning someone with sickle cell disease into a drug addict.
Prevention
Sickle cell disease is an inherited disorder that is common among African Americans. If you have sickle cell disease, you received the gene from each of your parents. If you received the gene from just one parent, you are a carrier. Genetic counseling and testing can give you information about the likelihood that you might pass on this gene to your children.
- Prevention of infection
- Infants and young children are at greatly increased risk of becoming infected with severe types of bacterial infections. Because of this increased risk, infants with sickle cell disease are placed on daily penicillin therapy until at least aged 5 years.
- It is also extremely important that infants receive all their immunizations on schedule to prevent these life-threatening infections.
- Subjecting yourself to conditions in which the oxygen concentration in the air is low may worsen sickling. This may include traveling in unpressurized airplanes or going to high altitudes.
Outlook
In spite of the tremendous improvements in diagnosis, genetic counseling, neonatal screening, and medical care, the ever-increasing body of information may have falsely raised expectations for a specific cure. Progress on genetic manipulation, which has the potential to cure the disease, is slow.
Developments in long-term transfusion therapy have given promise in decreasing the severity of pain crises and the incidence of
stroke; however, exposure to blood-borne diseases due to multiple transfusions is a risk.
Support and cure of infection are still the mainstays of treatment.