Low red blood cells or hemoglobin called anemia is the most common blood disorder world-wide.  However, some patients have hemoglobin that is too high called polycythemia.  Polycythemia can be caused by a bone marrow disorder caused polycythemia vera or from other causes outside the bone marrow called secondary polycythemia.  Distinguishing between the two became much easier about ten years ago with the discovery and testing for Janus Kinase 2 (JAK-2) mutations.  Janus was the Roman god of gates and this protein regulates the production of red bloods cells.  When is mutated, red blood cells are continuously produced causing a high hemoglobin.  Treatment of polycythemia vera is removal of the extra blood in a process called phlebotomy.

Secondary polycythemia can be caused  by conditions that cause low oxygen levels in the blood.  Extra red blood cells are then produced as a compensation.  Common causes include tobacco smoking and sleep apnea.  Other causes can include congenital heart disease, carbon monoxide exposure and being born with an abnormal type of hemoglobin.  The best treatment for secondary polycythemia is treating the underlying condition such as smoking cessation or the treatment of the sleep apnea.

During World War II in 1943 as allied armies were gaining a foothold in Italy, the Southern Italian town of Bari was a major port to supply the growing armies. On December 2nd, 105 German bombers attacked the port catching the allies by complete surprise. 28 allied ships were sunk. One of them, the John Harvey, had a secret cargo of 2000 mustard gas bombs. They were there for retaliation in event of a German chemical warfare attack.

The destruction of the John Harvey released nitrogen mustard into the air and water. 628 service personnel and civilians developed mustard poisoning. 83 ultimately died and many underwent autopsy. At autopsy, changes to the bone marrow and destruction of lymph tissue were noted. This observation led to experiments and the ultimate development of the first chemotherapy, Mustine; and the class of chemotherapy called alkylating agents; many of which are still used today.

This past year has seen a long-awaited improvement in the treatment of hemophilia A and B. Regular use of prophylactic clotting factors has made vast improvements in the quality of life of boys and men diagnosed with hemophilia. With this use they can preserve joint health and participate in many sports and activities that were not previously possible. However to accomplish this requires patients to access their own veins and infuse themselves three or even more times a week. This has been improved in 2014.

In the last 12 months the FDA has now approved longer-acting Factor IX(used to treat hemophilia B) and Factor VIII(used to treat hemophilia A). This was accomplished by taking a protein found on antibodies and adding it to the clotting factors. This is much harder for the body to breakdown and the clotting factor now stays in the body for a longer period of time. In best case scenarios the new factor IX can be given as little as every 14 days.

Many other exciting products are still in development and further improvements are expected.

I wanted to use today’s blog to thank our employees. About 2 years ago they came to us with the idea of starting a Saturday clinic. They gave up their personal time on the weekend because they saw how it could give better care to our patients. Saturday clinic offers time for treatments for those that are unable to take off time during the work week. It also allows an extra day for patients not feeling well to be evaluated and to have symptoms managed, instead of long waits in the ER.  This often helps avoid admissions to the hospital. My thanks to each of them.