Today, sickle cell anaemia is found mainly in equatorial Africa, North anaemia was reported in early 1995 when a study in the US showedAmerica (where about 80,000 people have the disease), southern
that daily doses of the decades-old antineoplastic agent hydroxyurea
Italy, northern Greece, southern Turkey, the Middle East, Saudi
(Droxia - Bristol-Myers Squibb) reduced the frequency of painful crises
Arabia and the eastern province of central India. The disease
and lessened the need for blood transfusions. Approved by the FDA in
occurs in approximately one in every 500 African-American births
1998, it appears to work by stimulating production of protective foetal
and one in every 1,000-1,400 Hispanic-American births. Roughly
haemoglobin within the red blood cells.
9% of African-Americans have the trait.
Apart from hydroxyurea, there have been several other advances in
Early diagnosis of sickle cell anaemia is vital to ensure that children
treatment over the past 5 years. But gene therapy surely holds the key
who have the disease receive proper treatment, and in the US more to a major breakthrough and a number of approaches are being
than 40 states now perform a simple blood test on all new-born
explored. One line of research is looking at whether correcting the
Coughing could save
babies. States where screening is carried out report survival rates in defective HbS gene and inserting it into the bone marrow of people withchildren with sickle cell disease that are equal to those of African-
sickle cell anaemia will result in the production of normal haemoglobin. lives
Americans without the disease. It is also now possible to check for
Another investigation is exploring the feasibility of turning off the
genetic problems before the child is born, either through
defective gene whilst simultaneously reactivating another gene that
page 1
amniocentesis or chorionic villus sampling. You'll find more
turns on the production of foetal haemoglobin. Study reveals ACE
information at Testing Your Baby for Genetic Problems During
inhibitor can have
The disease known in some African languages as 'a state of suffering'
The pre-SCRIPTION is a complementary newsletter lifesaving benefits
now appears to be at the mercy of human ingenuity and endeavour; the
that provides Nigerian healthcare professionals with a
Only about 50% of people with sickle cell anaemia live beyond 50
possibility of an eventual cure for sickle cell anaemia, although many
page 2 convenient preview of the news, views and latest
years and whilst there's no cure for the disorder, other than
developments within the industry that can be found HIV “treatment
experimental transplantation procedures, treatment is continually
Links on the pre-SCRIBE.com web site.
improving, and increasing numbers of patients are now living into
holiday” risk
adulthood. Basic treatment of sickle cell crises relies mainly on
page 3
1. http://sickle.bwh.harvard.edu/scd_history.html
analgesics to alleviate pain and administration of fluids for
2. http://www-medlib.med.utah.edu/WebPath/HEMEHTML/HEME062.html
rehydration. Blood transfusions are used to correct anaemia and
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3. http://www.aafp.org/afp/20000301/1349.html
Athletes’ gene
treat spleen enlargement in children, and regular transfusions can
4. http://www.sicklecellsociety.org/inherit.htm
identified
also help to prevent recurring strokes in children at risk of
5. http://www.emedicine.com/emerg/topic26.htm
developing crippling nervous system complications. Click on Sickle-
Walk proves as good as a workout for
patients used the technique, the symptoms
page 3
6. http://www.sicklecellsociety.org/prenatal.htm
women
and scroll down to Treatment of Sickle-Cell
7. http://my.webmd.com/webmd_today/home/404
You don't need to go for the burn to shed
pounds. A walk to the bus stop is as good as an
proven and so couldn't be recommended 'as a
The first effective drug treatment for adults with severe sickle cell
weight, American researchers say. The study
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University of Pittsburgh, published in the
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Three portions of fruit and vegetables a day
Cell Anaemia
may give the same health benefits as eating
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healthy women aged 21 to 45 who were put on
the recommended five. In contradiction to
a diet and assigned to different exercise
Government advice, Greek researchers found
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regimes. The women lost an average of 25lb
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problems by 70 per cent. Eating extra portions
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physical activity. There was no significant
'Three servings a day was the cut-off point. all registered healthcare
difference in the amount of weight lost or in the
Youreach a plateau after that.' Dr Panagiotakos
level of fitness gained between the moderate
and his team asked 850 cardiac patients and
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group who walked normally and the vigorous
1000 healthy volunteers how much fruit and
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study in the same issue of the journal suggests
that exercise also has a protective effect
sub-SCRIBER Benefits Neck treatments “do not work” OCCUPATION: 2003 Independent Newspapers (UK) Limited
Researchers in Finland say two treatmentsused to alleviate neck pain are a waste of
INSTITUTION: Diabetes risk with Atkins
Doctors say slimmers who follow the Atkins diet
training and relaxation therapy, both of which
may be at an increased risk of developing
involve learning how to strengthen or relax key
ADDRESS:
diabetes. They say following the high-protein
muscles, do not work. Dr Matti Viljanen and
regime for a prolonged period could lead to
colleagues at the Institute of Occupational
raised cholesterol levels, which in turn might
Health in Tampere base their findings on a
trigger diabetes. Diabetes specialist Dr Jim
study of female office workers with chronic
Mann of the University of Otago, New Zealand,
neck pain. After a year they found that the
PHONE:
also claims the Atkins diet creates a resistance
women who had done dynamic muscle training
to insulin - another condition that can lead to
and relaxation therapy had the same level of
diabetes. He said of the diet: 'I tell my patients
neck pain as those who had no treatment at all. EMAIL:
under no circumstances do I recommend it.'
Dental care cuts birth risks PROFESSIONAL REGISTRATION Coughing Could Save Lives TYPE (SPECIFY):
Heart attack deaths can be prevented by deep
woman’s risk of giving birth prematurely,
coughing, according to Polish doctors. They
say regular coughing at the first signs of severe
treating severe gum disease with scaling and
NUMBER/REF:
chest pains pushes blood through the body to
root care cut premature births by 84 per cent.
the brain. This could help patients survive until
Professor Marjorie Jeffcoat, of the University of
Alabama at Birmingham school of dentistry,
a copy of The pre-SCRIPTION to be sent to me each month
Cardiological Foundation in Katowice and his
who led the research, said, “I recommend that
team trained 115 patients at high-risk of heart
attack to cough when they experienced sudden
pregnant or who are pregnant receive a full
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periodontal exam and diagnosis.” Doctors have generic medicines could be imported to cure
New drug offers hope to heart patients
already established that severe gum infections
any life-threatening disease, so long as it was a
A new drug could help as many as five million
focus-ON: A tour around Sickle Cell Anaemia
public health emergency." Assurances that
people in the UK with a condition that doubles
by Geoff Barnett, Freelance Medical Writer and Editor
prostaglandin and tumour necrosis factor,
countries would not take advantage of the
the risk of dying from heart disease. Results of
a trial of rosuvastatin show that it has a broad
Sir Archibald Edward Garrod (1857-1936) was an English physician ahead of his time. Combining a perceptive understanding of
copies of patented drugs to reap commercial
range of effects against metabolic syndrome,
biochemistry with a thorough knowledge of Mendelian laws, he was the first person to suggest that some diseases were caused by
profits rather than public health needs, was key
Study reveals ACE inhibitor can have
to getting the U.S. to sign on, a news source
inborn errors of metabolism. Although well
symptoms are obesity, high triglycerides,
documented, his work was largely ignored, and not
YOUR DIABETES CENTRE lifesaving benefits
reports. This assurance also won the backing
cholesterol problems, high blood pressure and
A large four-year study of over 12,000 adults
of the drug industry."The final piece of the
until the middle of the twentieth century was the
www.yourdiabetescentre.com
with coronary artery disease finds that adding
jigsaw has fallen into place. It proves once and
symptoms is suffered by one in five men and
link between many diseases and heredity proven.
an ACE inhibitor to their daily medication
one in four women. It is linked to an increased
Among those disorders that paved the way for this
The Diabetes Association of Nigeria and NGC
lowered the risk of dying from a heart attack or
humanitarian as well as trade concerns,"
risk of heart attack, stroke, type 2 diabetes, and
acceptance was sickle cell anaemia; and among
Healthcare have teamed up to bring you the essential
stroke by 20 percent compared to those not
Supachai Panitchpakdi, director general of the
those who showed that the haemoglobin from
resource for information about diabetes management
WTO is quoted by a news source as saying.
(26/08/03) showed that rosuvastatin, sold by
patients with the disease was fundamentally
AstraZeneca as Crestor, cut bad cholesterol
different from that of normal people was a famous Nobel prizewinner. Spot his name at A Brief History of Sickle Cell Disease ( .
levels by 46 per cent. According to Professor
Society of Cardiology in Vienna, also found that Dental care cuts birth risks
Steen Stender of Gentofte Hospital in Denmark,
Sickle cell anaemia is an inherited blood disorder characterised
it in your back, your legs, your chest. It's constant.' HbS is caused
patients had a greater chance of surviving a
Simple dental treatment may reduce a woman’s
who led the study, it also had other effects
primarily by anaemia and episodes of pain, and is caused by a
by a defective gene that instructs the body to manufacture the
heart attack if they were taking the drug. ACE
risk of giving birth prematurely, research
which make it particularly suitable for treating
genetic defect in the haemoglobin (Hb) molecule. A normal red
abnormal haemoglobin. The error in the gene - the most common
blood cell contains a molecule known as haemoglobin A (HbA);
inherited blood condition in the US - came about as the result of a
coronary artery patients over 55 and others
severe gum disease with scaling and root care
in a sickle red blood cell, a variant of this molecule exists and is
genetic mutation that occurred several thousand years ago in
who are considered at a high risk of dying from
cut premature births by 84 per cent. Professor
called haemoglobin S (HbS). The difference between HbA and
people in parts of Africa, the Mediterranean basin, the Middle East
complications from diabetes or hypertension.
Marjorie Jeffcoat, of the University of Alabama
Progress on tenacity of H. pylori
HbS lies in only one amino acid out of about three hundred that
Dr. Kim Fox, from the Royal Brompton Hospital
at Birmingham school of dentistry, who led the
and India. Involving a deadly form of malaria, this is a fascinating
Scientists in Germany may have found out why
are common to both, but this tiny abnormality has devastating
in London, who was the director of the study,
research, said, “I recommend that all women
the body cannot eradicate Helicobacter pylori,
said that the study is a milestone in that it
who are thinking of becoming pregnant or who
a potentially deadly bacterium found in the guts
"proved for the first time that an ACE inhibitor
are pregnant receive a full periodontal exam
of half of all middle-aged men. The bug, which
Fortunately the majority of people with a high genetic risk of sickle
can have lifesaving benefits even in younger
causes stomach ulcers and is linked to gastric
The sickle cell disease process is triggered when HbS
cell anaemia inherit only one copy of the HbS gene from their
coronary patients and those considered to be
established that severe gum infections cause
cancer, seems to be able to evade the immune
molecules yield their oxygen to the tissues. Some of them
parents; the other Hb gene is normal and able to override HbS,
at low risk of complications," a news source
an increase in the production of prostaglandin
aggregate to form long rods (polymers) that distort the normal,
blocking the development of the disease. Such people have the
and tumour necrosis factor, chemicals which
flexible, doughnut-shaped cells into rigid, sickle-shaped cells.
sickle cell trait and lead largely normal lives, but run the risk of
perindopril. "Perindopril added to standard
Medizinische Mikrobiologie, in Munich, say a
Haematopathology(2) is a photomicrograph of a blood smear
passing on the abnormal gene to their offspring. Children who
optimal therapy over a four year period would
toxin released by H. pylori is able to target a
that clearly shows the cells' distinctive crescent shape.
inherit copies of the defective gene from both parents will develop
stop 100,000 heart attacks or cardiovascular
full-blown sickle cell anaemia. All of this is explained
Fats “help body fight TB”
Because of their shape, the sickled cells move less smoothly
diagrammatically at How are Sickle Cell Anaemia and Sickle Cell
60 million," Fox is quoted by a news source as
Dietary fats could help the body fight against
mechanism which produces inflammation - a
than normal red blood cells through small blood vessels, reduce
saying. The study's results may increase the
tuberculosis (TB), researchers have suggested.
the blood's rate of flow, stack up like a log jam and cause
number of patients prescribed ACE inhibitors,
However, it is not yet clear whether eating the
experts say, according to the news sources.
fats could be used as a treatment for TB, and
blockages that deprive the tissues and organs of oxygenated
Symptoms of sickle cell anaemia usually don't occur until late in the
researchers, writing in Nature Cell Biology, say
New benefit to blood pressure drug
blood. In turn, this process produces the periodic episodes of
baby's first year and typically include fever, swelling of the hands
further work should be carried out to find out
A drug used to treat high blood pressure could
pain, or 'crises', that may start suddenly and last for many hours
and feet, pain in the chest, abdomen, limbs and joints, and
WTO reaches an agreement on poor
more about how their properties could be used.
or extend to several days. There is wide variation from person
enlargement of the heart, liver and spleen. Frequent upper
nations' access to generic drugs
The body’s macrophages go through certain
diabetes. Losartan has already been shown to
to person and crisis to crisis, ranging from mild discomfort that
respiratory tract infections are common. After infancy, patients
significantly cut the risks of having a stroke,
is treatable at home, to excruciating pain that requires
develop symptoms of anaemia, and pain is the most frequent
agreement that poor nations would be allowed
destroy the bacteria. TB prevents a key stage
and now research in Sweden indicates it may
hospitalisation. The subject is well covered in a paper published
complaint. At Anaemia, Sickle Cell, (5) under the headings Vaso-
to import generic versions of patented drugs
of this process, disabling the cells. This latest
reduce by half the risk of diabetics dying from a
earlier this year in the American Family Physician entitled
occlusive, Haematologic and Infectious Crises, there's a list of
and could purchase them from countries like
research suggests certain fatty acids, such as
Approach to the Vaso-occlusive Crisis in Adults with Sickle Cell
clinical manifestations that reflect their diversity and wide organ
India and Brazil, news sources report. The
arachidonic acid, found in meat, can prevent
Disease.(3) One patient vividly describes the pain thus: '. like
this disabling taking place, allowing the body's
the worst toothache you can imagine. Now take that pain and put
defence system to work efficiently. But they
Pfizer's Lipitor proves highly effective for
that it rejected last December when it said that
also said omega-3 fats, found in oily fish, had a
cutting heart attack risk in diabetes patients
the plan should be restricted to a few diseases
slightly negative effect on the body's ability to
heart attacks and strokes in patients with diabetes, news sources
The Collaborative Atorvastatin Diabetes Study,
appears that the gene exists in two versions, one of which delivers a
report. The study was halted so that all of the approximately 2,800
and limited to certain countries. However, this
slated to run four years, has ended two years
protein called actinin-3 which gives extra power to muscle fibres. In a
enrolled patients could benefit from Pfizer's cholesterol fighter. The type
early after Lipitor proved significantly effective
study of the genetic profiles of 300 athletes, 95 per cent of the best
proposal, "but included the demand that such
2 patients enrolled in the study had no previous history of heart disease
in reducing major coronary events such as
sprinters had at least one copy of the gene variation, and 50 per cent
or stroke; however, the disease is considered one of the biggest risk
had two copies. Only 5 per cent of sprinters had two copies of the non-
factors for those conditions. Patients in the study received 10
milligrams of the drug each day. Pfizer's Lipitor is the world's top-selling
Fluorquinolones use associated with reduced CHD risk in Type II diabetes
drug. In the first quarter of 2003, it raked in sales of $2.1 billion.
Results of a study published in the October issue of the European Heart Journal (2002;23:1575-1579) suggest that
Merck drug “reduces risk of prostate cancer”
treatment with fluorquinolones in doses commonly prescribed in routine clinical practice is associated with a reduction in
Merck’s drug to treat enlarged prostates, Proscar, has been found to
the risk of coronary heart diseases (CHD) among Type II diabetes patients. HIV “treatment holiday” risk
reduce the risk of prostate cancer in men by 25 per cent, according to a
Patients who take a break from their HIV drugs on the instructions of
government study published yesterday (24/06/03). The Prostate
Data were obtained from the PHARMO Record Linkage System comprising
their doctors may risk the condition getting worse, say experts.
Cancer Prevention Trial said finasteride, the generic name for Proscar,
pharmacy records and hospitalisations for all 450,000 residents of 8 Dutch
Although some studies have suggested that so-called "treatment
helped prevent prostate cancer over a seven-year period. The data
cities. In a nested case-control study among Type II diabetes patients, 244
holidays" could actually improve their health, researchers at the
could allow Merck to market the drug for the prevention of the disease.
cases with a first hospitalisation for CHD and 686 controls without CHD
University of California say that it could be harmful for a few patients.
matched on age, sex, calendar time and registration date in PHARMO RLS
Tests during a four-month trial suggested that the break may speed up
were selected. Use of antibiotic drugs among cases and controls was
the attack on the immune system. Their results, published in the NewEngland Journal of Medicine, will be a disappointment to those experts
HEALTH INSURANCE
determined over 3 years prior to the event. Use of fluorquinolones was
who hoped that treatment holidays could be a way of tackling HIV
associated with a lower risk of CHD. No association between tetracycline,
which has grown resistant to modern drug cocktails. The theory is that
Would you like to protect your
macrolide and lincosamide treatment, or other antibiotic drugs (penicillins,
when the treatment is resumed, it would have a far greater impact,
cephalosporines, sulphonamides and trimethoprim), and the risk of CHD
health with one of the worlds leading healthcare providers. Espicom Business Intelligence, 21/10/02Athletes’ gene identified
Australian scientists believe they have identified a gene that gives
PLEASE FEEL FREE TO PASS THIS NEWSLETTER TO YOUR COLLEAGUES
sprinters the sudden speed and power they need to succeed. It
The RESONATETM-2 (PCYC-1115-CA) Clinical Trial A resource guide for patients and their families The purpose of this study is to test whether or not the investigational (experimental) study drug (ibrutinib, also known as "PCI-32765") works and is safe in patients with CLL / SLL. It compares ibrutinib to the FDA-approved drug chlorambucil. This study is being conducted in patients who
F e a t u r e d P o e t Joel E. Chace Selected Poems: 3 4 6 7 9 14 18 19 29 31 34 44 47 ______________________________________________“Pork” is from the electronic chapbook The City on the Hill , from 2River View “Cheers: Freaking in Tongues” is from the electronic chapbook The Reason Why I Cannot “Ma