We're Leaders in
World Class Medical Research
You can help
July 28, 2016 – The recent death of an adult with thalassemia, apparently from sepsis (overwhelming bacterial infection) has led to some vigorous discussions in the thalassemia community. The discussions reveal some of the clear advantages, but also disadvantages, of social media. An advantage is that word about important events spreads rapidly. Unfortunately, news spreads faster than facts are collected or ascertained (not just in the area of thalassemia!)
As a result of this tragic and unfortunate passing, CAF contacted Dr. Ellis Neufeld, Chair of CAF’s Medical Advisory Board, and asked him to provide us with some important reminders based in medical evidence that people with thalassemia should keep in mind. These include:
a. Fortunately, there are vaccines for these germs. Every splenectomized thalassemia patient at all ages should have up to date vaccines including PCV-13 (Prevnar 13), PPV 23 (Pneumovax), HIB vaccine (hemophilus), and Menactra. The rules about “up to date” are changed by CDC every few years. If you haven’t had updates in a few years, please check with your physician immediately to catch up.
b. Unfortunately, some forms of pneumococcus are NOT covered by the vaccines.
c. Therefore, every fever over 101.5F (38.5C) in splenectomized patients should be considered an emergency. Different physicians have slightly different approaches for this problem, but no high fever can be safely ignored with no spleen.
i. Even if other household contacts have a nonbacterial illness (like influenza virus), the splenectomized patient should be seen emergently for high fever. This is true even if the circumstances are inconvenient (e.g., Thanksgiving weekend, traveling on vacation, etc.)
ii. In general, this emergency visit should include a blood culture, CBC/diff, and a dose of strong antibiotics awaiting the blood culture results.
iii. If a patient seems or feels particularly ill and had no spleen, he or she should also be seen urgently, even without a very high fever. But if the fever is very high, he or she should be seen even if s/he feels otherwise ok.
2. The spleen also is the primary filter for a class of protozoal germs that includes malaria (all forms) and babesiosis. In the US, babesiosis is now famous for being possible to aquire from transfusions, but it is endemic to the coastal Northeast US from Cape May through Massachusetts, and to areas around Maine, New Hampshire, the lakes of Wisconsin and Minnesota, among other places. It is carried by the same deer ticks that carry Lyme disease. Precautions at the beaches (such as ,don’t go in the dune grass with shorts on, use insect repellent wisely, etc.) can reduce the risk.
3. Some germs (not the big three noted above for the spleen) grow best when they can get iron from their environment. Paradoxically, chelators, especially deferoxamine (Desferal), help get iron INTO these germs, and this is why we strongly suggest being off chelator at least at the beginning of illness with fever until evaluation is completed, and these germs are ruled out. Particularly bad actors among these germs can include Listeria, Vibrio, and Klebsiella. For these kinds of germs, warnings that come about food borne infections make sense to heed. Probably everyone should get pasteurized milk products, but raw milk particularly carries Listeria, and thefore raw milk (at farms or in some cheeses) should be avoided. Raw seafood, but not cooked seafood, can carry Vibrio. Raw beef (such as steak Tartare) can carry toxoplasmosis. If a person on chelators has unexplained fevers of feels particulary ill, it is very important to tell your physician if you had any of these exposures, and in general any travel.
If you have specific questions about areas not addressed here, please ask your physician. We welcome comments and queries to CAF as well (firstname.lastname@example.org).