For as long as possible the fight to find a cure for sickle cell disease has been ongoing. And as the veritable search continues, it is those who suffer continuous sickle cell crisis as a causative effect of the genetic disorder that bear the brunt of the long wait.
Sickle Cell Disease (SCD) affects millions of people throughout the world. It is a rare, but genetic condition that is particularly common among those whose ancestors came from sub-Saharan Africa; Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries.
Some statistics reveals that almost 300,000 children are born with a form of SCD every year. In 2013, it resulted in about 176,000 deaths. The figures and statistics vary across geographical divides. And in comparison to the millions that the condition affects, the rate of mortality can be said to be fairly dwindling.
Nevertheless, it in no way stands as a numeric euphemism to downplay the painful crisis that a lot of those with the disorder face daily.
Sickle Cell Crisis | What Is It?
Sickle cell crisis comes often as a direct and painful consequence of the SCD. Sickle cell crisis is also called vaso-occlusive crisis and it is pain that can begin suddenly and last as long as hours and even days.
According to an article on the American Family Physician official website, aaafp.org, sickle cell crisis “…happens when sickle red blood cells block small blood vessels that carry blood to (patients) bones.” The article further explains that sometimes, the pain can either be throbbing, sharp, dull or stabbing.
In general, the risk for a sickle cell crisis is increased by any activity that boosts the body’s requirement for oxygen such as illness, physical stress, or being at high altitudes. Admittedly, it is explained that the severity of a sickle cell crisis varies from person to person and from crisis to crisis.
And although the causes of sickle cell crisis are definitely and directly related to the abnormal blood condition itself, there are a few things that have been discovered to accentuate the crisis spells. Some of these include:
- Alcohol consumption
- Smoking
- Moodiness
- Insomnia, etc
Being the eerie hallmark of SCD, the sickle cell crisis is by far the most prominent complication of the disease. Other additional medical complications may include:
- Infections
- Pulmonary hypertension (increased pressure in the arteries of the lungs)
- Stroke
- Anaemia
- Kidney problems
- Priapism (prolonged and painful erections)
- Liver problems
- Gallbladder disease
- Spleen damage
- Bone and joint problems
- Leg sores and ulcers
- Eye damage in the retina, etc
Conquering The Deathly SCD | What The Viable Sickle Cell Crisis Treatments Are?
‘Deathly’ is a word often used to describe the wanton effects of this genetic blood disorder. And the ordeals of those who suffer from the rare blood condition remains to be completely alleviated.
This very much explains the haste of medical researchers and expert clinical specialist at finding a befitting cure to the disease. However, till date, only two possible avenues have arisen that heralds the possibility of hope for those plagued by sickle cell disease and its crisis.
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Hydroxyurea Therapy
Although several pharmacological agents have been studied for the treatment of Sickle Cell Disease (SDC), the only drug currently approved by the US Food and Drug Administration (FDA) for Sickle cell crisis treatment is Hydroxyurea. Long-term hydroxyurea is currently the accepted treatment for severe and frequent pain.
According to an article on MedCape.com, Hydroxyurea increases total and foetal haemoglobin in children with SCD. In 2008, a report given in a National Institutes of Health Consensus Development Conference concluded that “strong evidence supports the efficacy of hydroxyurea in adults to decrease severe painful episodes, hospitalizations, number of blood transfusions, and the acute chest syndrome. For children however, the efficacy of the therapy is not as strong but emerging data are revealed to be encouraging.
Hydroxyurea is usually prescribed by a haematologist. And unlike any other treatment therapy, the selection criteria for the administration of the drug are quite rigorous. Patients receiving hydroxyurea require frequent blood testing and monitoring, with special attention that culminates into a doctor-patient relationship and rapport.
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Stem Cell or Bone Marrow Transplant
Stem cell transplantation is a potential cure for SCD. Stem cells can be found in bone marrow, hence the alternative name. Bone marrow is a soft, fatty tissue in the centre of the bones that produces red blood cells. A person with SCD has bone marrow with haemoglobin S defective red blood cells.
The treatment is considered one of the foremost breaks at a cure for those with SCD as replacing a sickle patient’s bone marrow with healthy bone marrow will ensure the production of normal haemoglobin over time.
However, the major limitation to this is the search for a befitting (healthy) donor. Bone marrow or stem cell transplants are very risky, and can have serious side effects, including death.
This option of treatment for SCD is often used only in cases of severe SCD for children that have minimal organ damage from the disease. Although stem cell transplant is a very risky procedure, the treatment has been reportedly successful in about 85 out of 100 children according to webMD.com.
However, the success rate of the procedure is often hindered by factors like the availability of matching donors, the cost of the procedure, unforeseen complication before and after the procedure, and so much more. Also there are very few hospitals that offer this procedure.
One can only hope that with the rise in technological advancement and the growing awareness of more people to their health status, in no time, a less risky procedure or less complex drug would have been made available to keep those with sickle cell crisis in check.
However, till that happens, round the clock pain management procedures would have to suffice as a viable and quick-safe method to helping the embattled through their sickle cell crisis.
dr. Puan