What is polycythemia vera?
Polycythemia vera is a type of health condition that results from an increased level of circulating RBCs or red blood cells in the bloodstream. A person known to suffer this kind of health problem exhibits increased levels of hematocrit, hemoglobin and/ or red blood cell count which is more than the maximum normal limit. A condition is usually classified as polycythemia vera basing on the level of the hematocrit and the hemoglobin of the person.
Once an individual has polycythemia vera, the hematocrit level is said to be more than or greater than 48% in women and 52% in men. The hemoglobin level, on the other hand, in polycythemia vera patients have a measurement that is more than 16.5 g/dL in women and more than 18.5 g/dL in men.
In addition to these, polycythemia vera is also divided into two categories namely the primary and the secondary. In the primary type of polycythemia vera, the increase of red blood cells is caused by the inherent problems in the process of RBC or red blood cell production in the body. However, the secondary kind of polysythemia level generally occurs as a reaction or a response to other factors or causative conditions that enhances the production of the red blood cells in the blood. Normally, red blood cell production or otherwise known as erythropoiesis takes place in the bone marrow through a complex process or regulated steps. The main regulator that plays a huge role in the RBC production is a hormone called erythropoietin or EPO. This hormone is mainly secreted or produced by the kidneys; nevertheless, approximately 10% of the total amount of EPO in the body is also created by the liver.
The body normally regulated the secretion of this hormone in order to maintain the normal levels of RBC in the body. This is usually increased only as a response to certain health problems such as low oxygen levels in the blood or medically known as hypoxia. This response is the body’s way to compensate with the low oxygen levels since O2 can be carried to the different tissues of the body once the RBC production is stimulated by the EPO.
Although polycythemia vera can happen to anyone, most cases are seen in children as well as in newborns. In fact, neonatal polycythemia can happen to 1 to 5 percent of all newborn. This is commonly a result from transfusion of blood, the transfer of placental blood to the infant post delivery and/or the chronic and inadequate oxygenation of the fetus (medically known as intrauterine hypoxia), which is usually a result from placental insufficiency.
How to prevent polycythemia?
Polycythemia can be prevented depending on the type as well as the factors that have caused the problem to occur. Polcythemia vera that occurred as a result from long standing smoking and/or exposure to hazardous gases such as carbon monoxide can definitely be prevented once these risk factors are alleviated. Aside from this, reducing also the risk factors that can cause heart failure such as high blood pressure and DM or diabetes mellitus can also be effective in reducing the chances of having polycythemia. Nevertheless, those conditions that are congenital in origin and are classified as primary polycythemia disorder are already considered not preventable.
What is the polycythemia vera life expectancy?
The prognosis or the outlook of a disease actually depends on a lot of factors. In case of polycythemia vera, the prognosis basically depends on the underlying cause of the problem. In general, the outlook is considered favorable for people who are experiencing secondary types of polycythemia vera cases. On the other hand, the prognosis of primary polycythemia vera is considered fair even though it is considered incurable and long standing for some cases. This is due to it being a controllable and treatable condition.
If untreated, the condition has a poor prognosis with a life expectancy rate of only a few years from the time of diagnosis. However, with the advancement of research and breakthroughs of medical technology, the life expectancy of polycythemia vera sufferers has greatly improved, with most patients now enjoying a normal life span. Typically, this is achieved with continuous phlebotomy treatment, the use of medications such as Hydroxyurea to reduce the RBC, Aspirin to thin the blood and reduce the risk of blood clots, as well as other interventions for this condition. Also, some of the debilitating symptoms and associated conditions (e.g., erythromelalgia) can be relieved by suitable medication/treatment, e.g., Aspirin can relieve the bone pain and burning feelings that some patients experience, and for those with excessive itching, doctors may prescribe antihistamines, or recommend ultraviolet light treatment to relieve the discomfort.
These are just some of the important facts and details with regards to the condition known as polycythemia vera. Rare and complex diseases are still possible to manage and treat as long as the right and proper interventions and treatment are given immediately. For those who wish to share their story and support with others, the MPN website has lists of peer-to-peer, online and in-person support groups.