Hematological Disorders

Question of bone marrow transplant

06-09-2016:This is in response to the questions on the hematologic listings. Sickle cell disease is a heterogenous disease. There is basically, for simplicity reasons only sickle cell disease, homozygous, sickle cell trait, heterozygous and there is sickle thalassemia. Sickle cell homozygous is a condition of severe anemia and these persons have frequent bone infarcts, esp. if in higher altitudes. The only successful treatment is bone marrow transplant. Sickle cell trait is a non-severe condition and there is no hemolysis associated with it and the patients with this, the heterozygous trait, are not anemic. Evaluation of these should be on the basis of complications from the bony infarcts which can occur with going to elevated altitudes. However, these are usually not long lasting and do not prevent SGA for more than a few days at the most. Sickle-thal on the other hand, depending on whether it is alpha or beta thalassemia can be a severe anemic process and this would require bone marrow transplant.


The question of the transplant raises a different situation. The process is a complicated one in which the claimant undergoes a very thorough psychological process initially and is interviewed and given options and the possibility of complications. The process is one in which high does of chemotherapy drugs are given to ess. wipe out the bone marrow and then a donor’s marrow is infused. During this period a number, probably at present less than 5%, die from sepsis and bleeding. If they recover, then they are ess. normal with one exception. Immunosuppressive drugs are given to prevent GVH and HVG disease and the claimant is ess. at that time somewhat immunosuppressed. A successful bone marrow or stem cell transplant should result in normal functioning. However, there can be problems with the immunosuppression which would be addressed under 14.00 non-HIV. Otherwise, the situation is eventually non-severe. A period of time is needed to recover, probably less than a year but the 12 months diary is not unreasonable. Biggest complaint is fatigue but I have spoken to transplant doctors and the feeling that any problems from the transplant itself, including fatigue, should not last more than a year.


Bottom line, in my opinion the listings are not unreasonable and they are workable. One last comment, there is some research on sickle cell disease but it is not standard or approved as of yet. I hope this is not too lengthy and helps somewhat. If any questions then confer. Nick Mansour, M.D. Tucson DDSA


Historical comment:

05-13-2015: reviewed the new rules on the hematology section. Bear with me on a few comments. I realize what I say will do nothing to change the rules. First off, the main cells of the circulatory system re the RBCs and the platelets. Anemia can be defined anyway one wants to define it based on the normal for the lab. However, the normal guide line is that a hemoglobin of over 9,.5 does not require, usually, transfusion, whereas one below 7 does. Having said that I have seen one patient during my training with a hemoglobin of 7 gms. who lived in Albuquerque, elev. 5000 ft. who would go to Denver an play full basketball with no cardiac problems. The point is that a chronic anemia can become well compensated. The point is that a number does not mean much without a clinical assessment. I noted some of the comments. I believe a review every 5 years is not unreasonable as indicated. However, the practice of hematology, while becoming more technologically advance, it has not changed a great deal over the past several years. I.E. evaluations of anemias is clinically fairly unchanged. Secondary comment on disorders which do not fit criteria but the person is disabled: as I noted above, being anemic may not be disabling depending on many factors. I believe the commenters fail to realize there are other systems under which a person’s disability can be assessed. More on that below. I agree with noting in the preamble compliance with medication, esp. in persons with iron deficiency and B-12 anemia and persons with chronic anemia who are on erythropoietin. Note, the listing for chronic anemia defines it as of any cause and the preamble does not take into account that certain types of anemia are very treatable and reversible. I don’t agree with taking into account the cost of medications. Medications for most anemias is very inexpensive and there are available sources for those who have difficulty paying. In regards to hospitalizations, the rules of medicare are (check me out on this one but it used to be) were that a 48 hour admission was acceptable for observation in most cases. I believe prolonged hospitalization should be defined on the bases or the medical information and be longer than 48 hours. I also do not believe that 48 hours is an arbitrary number having been in that situation previously. However, for the purposes of assessment, my feeling is that it should be more than 48 hours from the stand point of assessing severity. As far as specific anemias is concerned, Sickle cell disease and trait can be assessed under 1.00 as particularly those with sickle cell anemia can develop joint bleeding and hemarthroses although they usually have bony infarcts. Note, also, because of their painful episodes they can become narcotic dependent. Hemophiliacs, those with A and B can and do develop painful hemarthroses and strictures which limit them and can be assessed under 1.00. Coagulation disorders cause, of course, hemorrhage and I believe they should be assessed on the frequency and severity of the hemorrhages. Persons with hereditary spherocytosis can be cured of their hemolysis by splenectomy. This may fall under the 14.00 listing of immunodeficient non-HIV. They can and sometimes do get serious, life threatening infections. I don’t agree with the commenters on the ulcers. I won’t belabor the discussion further although there were other comments with which I did not agree. However, there is 7.18 which is for repeated complications. As stated above, those complications can be assessed under the other systems; ulcers under skin, strokes under neuro, joint problems under 1.00, etc. I hope I haven’t bored you with this and I realize it changes nothing but I felt I had to reply to the new rules. Sorry for the long discourse. Nick Mansour, M.D. Tucson DDSA


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