What is Sickle Cell Anaemia?
The disorder affects the red blood cells which contain a
special protein called haemoglobin (Hb for short). The function
of haemoglobin is to carry oxygen from the lungs to all
parts of the body.
People with Sickle Cell Anaemia have Sickle haemoglobin
(HbS) which is different from the normal haemoglobin (HbA).
When sickle haemoglobin gives up its oxygen to the tissues,
it sticks together to form long rods inside the red blood
cells making these cells rigid and sickle-shaped. Normal
red blood cells can bend and flex easily. Because of their shape, sickled red blood
cells can't squeeze through small blood vessels as easily
as the almost donut-shaped normal cells. This can lead to
these small blood vessels getting blocked which then stops
the oxygen from getting through to where it is needed. This
in turn can lead to severe pain and damage to organs.
Everyone has two copies of the gene for
haemoglobin; one from their mother and one from their father.
If one of these genes carries the instructions to make sickle
haemoglobin (HbS) and the other carries the instructions
to make normal haemoglobin (HbA) then the person has Sickle
Cell Trait and is a carrier of the sickle haemoglobin gene.
This means that this person has enough normal haemoglobin
in their red blood cells to keep the cells flexible and
they don't have the symptoms of the sickle cell disorders.
They do however have to be careful when doing things where
there is less oxygen than normal such as scuba diving, activities
at high altitude and under general anaesthetics.
If both copies of the haemoglobin gene
carry instructions to make sickle haemoglobin then this
will be the only type of haemoglobin they can make and sickled
cells can occur. These people have Sickle Cell Anaemia and
can suffer from anaemia and severe pain. These severe attacks
are known as Crises. Over time Sickle Cell sufferers can
experience damage to organs such as liver, kidney, lungs,
heart and spleen. Death can be a result.
Another problem is that red blood cells
containing sickle haemoglobin do not live as long as the
normal 120 days and this results in a chronic state of anaemia.
In spite of this, a person with sickle cell disorder can
attend school, college and work. People with sickle cell
disorder need regular medical attention particularly before
and after operations, dental extraction and during pregnancy.
Many hospitals arrange follow-up appointments and it is
advisable to discuss with the doctors questions concerning
schooling, strenuous exercise, family planning, suitable
types of employment and air travel. When a person is found
to have a sickle cell disorder it is important that all
members of the family be tested. They will not necessarily
have sickle cell disorder but may be healthy carriers of
a sickle cell trait.
Copyright© (www.sicklecellsociety.com)
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Scientific Advisory Board
Mehboob Ali Hussain, MD:
Dr. Hussain is an Assistant
Professor, Department of Pediatrics and Medicine, John Hopkins Medical Institute, Baltimore.
He also serves on the Scientific Advisory Board for
Regenetech, Inc. Dr. Hussain received his MD from
the University of Zurich and completed a fellowship
from Harvard Medical School and Massachusetts General
Hospital, concentrating in Molecular Endocrinology.
He did his residency at New York Hospital.
EDUCATION AND DEGREES:
1989 MD University of Zürich,
Switzerland
Licensed New York State License in Medicine 2002 #225907
Licensed Illinois State
American Board Certification in Internal Medicine
(ABIM) 2002 #216942
POSTDOCTORAL MEDICAL AND SCIENTIFIC TRAINING:
12.1989-10.1995 Clinical and Research Training, University
Hospital in Zurich, Switzerland
11.1995-6.2000 Research Fellow, Laboratory of Molecular
Endocrinology with joint appointment in Department
of Medicine, Massachusetts General Hospital and Harvard
Medical School, Boston
7.2000-6.2002 Resident in Medicine, New York University
School of Medicine
6.2002-7.2003 Assistant Attending Physician Department
of Medicine, New York University School of Medicine
Present Visiting Scientist. Liver and Stem Cell Research
Laboratory, Beth Israel Medical Center, New York
AWARDS AND HONORS (partial list):
1997-1998 Juvenile Diabetes Research Foundation Postdoctoral
Fellowship
1998-2002 Juvenile Diabetes Research Foundation Development
Award
1998-present Highlights Editor, European Journal of
Endocrinology
2003-2004 American Diabetes Association Innovative
Grant
2003-2005 Juvenile Diabetes Research Foundation Regular
Grant
2003-2008 National Institutes of Health R01 Grant
DK 064646-01
2003 Selection to Readers Panel of Nature Magazine
Vincent F. LaRussa, Ph.D.:
Dr. Vincent F. LaRussa is currently a Research Associate Professor, Pharmacology, the Director BMT for Graft & Stem Cell Engineering, at Tulane Cancer Center, School of Medicine, New Orleans, Louisiana. He received his Ph.D. in 1981 from the Department of Physiology, Division of Roswell Park Memorial Institute (RPMI), State University of New York at Buffalo. He carried out doctoral studies on the regulatory functions of mature and immature T-cells on stem cell engraftment. He received the Special Fellow in Oncology award from the Johns Hopkins Oncology Center (Baltimore, MD) and trained under Drs. Lyle Sensenbrenner and Saul Sharkis, working on cell surface modification of sialic acid residues on regulatory T-cells and stem cell interactions. In 1985 he joined the Department of Hematology as a Senior Scientist at the Walter Reed Army Institute of Research (Washington DC) where he carried out studies on the biology of bone marrow stem cells and stromal cells and pathogenesis of HIV and Dengue viruses on purified populations of CD34+ hematopoietic stem cells and stromal cells of the microenvironment. Dr, La Russa's current work involves clinical stem cell and accessory cell graft engineering for transplantation and treatment of malignant and non-malignant diseases. Basic stem cell studies utilize in-vitro models to investigate the biology of novel cytokines that regulate both un-manipulated and genetically modified hematopoietic and mesenchymal progenitor cells, as well as in-vivo animal models for hematopoietic and genetically marked mesenchymal stem cell transplantation for hematopoietic and non-hematopoietic tissues.
RECENT PUBLICATIONS (partial list) :
- Jiang G, Yang F, Li M, Weissbecker K, Price S, Kim KC, LaRussa VF, Safah H, Ehrlich M. Imatinib (STI571) provides only limited selectivity for CML cells and treatment might be complicated by silent BCRABL genes. Cancer Biology and Therapy 2:1661-1667, 2003.
- Ramdas J, Warrier RP, Scher C, LaRussa VF. Effects of amifostine on clonogenic mesenchymal projenitors and hematopoietic progenitors exposed to radiation. J Ped Hem/Onc 25:19-26, 2003.
- LaRussa VF, Mondal D, Miller A, Safah H, Rozans MK, Curiel TJ, Agrawal K, Weiner RS. Neuronal Stem Cells Biology and Plasticity. Cancer Investigation 21:792-804, 2003 (Invited Review)
- Ramdas J, Warrier RP, Scher C, LaRussa VF. Effects of amifostine on clonogenic mesenchymal progenitors and hematopoietic progenitors exposed to radiation. J Pediatr Hematol Oncol. 2003 Jan;25(1):19-26.
- Zhang XY, LaRussa VF, Reiser J. Transduction of bone-marrow-derived mesenchymal stem cells by using lentivirus vectors pseudotyped with modified RD114 envelope glycoproteins. J Virol. 2004 Feb;78(3):1219-29.
- Srinivasan R, Marrero L, Erfurth F, LaRussa VF, Hemenway Cs. The synthetic peptide PFWT AF4-AF9 protein complexes and induces apoptosis in t(4;11) leukemia cells. Leukemia (in press)
- Hofer EL(1), Honegger AE(1), Bullorsky EO(2), Bordenave RH(3), LaRussa VF, Chasseing NA(1). Prognostic importance of studying the expression of IL-1, PDGF, TGF(, EGF and FGF receptors and c-Fos and c-Myc proteins in from bone marrow fibroblasts advanced untreated lung and breast cancer patients. Clinical Cancer Research (in review)
- Mondal A, Pradhan L, LaRussa VF. Signal transduction pathways involved in the lineage specific differentiation of NSCs: Can the knowledge gained from blood be used in the brain? Cancer Invest. (Invited review, In Press)
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