I went searching to find out more about this disease and here are a few things to know about Sickle Cell Anemia.
Sickle cell anemia is a hereditary disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent.
Sickle cell anemia occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. Instead of being flexible and round, these cells are more rigid and curved in the shape of the farm tool known as a sickle - that's where the disease gets its name. The shape is similar to a crescent moon.
Sickle cell anemia is a blood disorder that affects hemoglobin (pronounced: hee-muh-glow-bin), a protein found in red blood cells that helps carry oxygen throughout the body.
Red blood cells with normal hemoglobin (HbA) move easily through the bloodstream, delivering oxygen to all of the cells of the body. Normal red blood cells are shaped like doughnuts with the centers partially scooped out and are soft and flexible.
Sickle cell anemia occurs when an abnormal form of hemoglobin (HbS) is produced. HbS molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape. Red blood cells containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently. Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body's tissues and organs of the oxygen they need to stay healthy.
Sickle cell anemia occurs when an abnormal form of hemoglobin (HbS) is produced. HbS molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape. Red blood cells containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently. Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body's tissues and organs of the oxygen they need to stay healthy.
Signs and Symptoms
Teens with sickle cell anemia may develop jaundice (pronounced: jon-dis), a condition that results from the high rate of red blood cell breakdown. Jaundice can cause the skin and the whites of a person's eyes to develop a yellowish tint.
People with sickle cell anemia also may have bouts of pain in the chest, stomach, arms, legs, or other parts of the body. This is caused by sickle cells blocking blood flow through the blood vessels. Feeling tired and having trouble fighting infections are also common among teens with sickle cell anemia, and they may grow more slowly and reach puberty later than other teens.What Do Doctors Do?
To diagnose sickle cell anemia, doctors use a blood test called hemoglobin electrophoresis (pronounced: hee-muh-glow-bin eh-lek-truh-fuh-ree-sis) to look for HbS in a person's blood.
There is no cure for sickle cell anemia, and it is possible for some people to die from the disease (although most young people with sickle cell anemia don't die). Doctors can provide treatments that help prevent complications from the disease, though.
Folic acid, a vitamin that helps the body produce new red blood cells, is often prescribed for teens with sickle cell anemia. Pain medications help relieve the symptoms of crises. And kids and teens who have sickle cell disease often take penicillin or other antibiotics to help fight infections.
Some crises can be managed at home with pain medicines, rest, and extra fluids. But if a crisis is especially intense, the person may need to go to the hospital for intravenous (IV) fluids and stronger pain medications.
People with sickle cell anemia may also use oxygen to help ease symptoms during a crisis or an episode of acute chest syndrome.What Can You Do to Stay Well?
With the right precautions, teens with sickle cell disease can do most of the stuff other teens do.
To stay as healthy as possible, people with sickle cell anemia should take these steps:
. Eat a balanced, healthy diet.
. Take vitamins, including folic acid supplements, as prescribed.
. Drink plenty of water to prevent dehydration.
. Avoid extreme cold or heat.
. Exercise regularly, but in moderation. Exercise is important for staying healthy, but overdoing it can trigger a crisis in some people, particularly if they become dehydrated, overheated, or exhausted.
. Get plenty of rest.
. Avoid alcohol, drugs, and smoking, which can aggravate sickle cell disease and its symptoms. Some people with sickle cell disease are prone to lung problems, so smoking is particularly risky.
Avoid places low in oxygen. (For example, it's not a good idea to go hiking at high altitudes or spend lots of time swimming under water.)
Prevent serious infections by contacting your doctor as soon as illness symptoms start. Be sure to get any immunizations (such as pneumonia and flu vaccines) that the doctor recommends.
Learn as much as you can about the disease and see your doctor regularly to help prevent complications.
There are some limits that people with sickle cell disease may need to put on their lives, but with the help of doctors, friends, and family, teens with sickle cell anemia can manage the disease and live their lives to the fullest.
Insist on a test before getting hitched
hi, feel bad about reading your blog everyday and never leaving a comment. i think its the satisfaction of fulfilling the amebo in me, i forget to drop a line. thanks for all the ifo, from political to entertainment and now health.
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ReplyDeleteMy hubby is a sickler too. With proper medication and God's grace, he is in good health most of the time. I am not a carrier so our kids will be safe.
ReplyDeleteGirl - you are working fast!! 90 posts in March alone and we've just gotten halfway thru the month. Ur posting speed is amazing. I wish I had ur kind of zeal. I try to post weekly right now or whenever I have anything to share.
this post is so spot on but all i can say is there is a God that does wonders am a living testimony, my kids, husby and i are all carriers- wetin remain make i tell you i serve a living God. tnx for the info i hope peeps get the point to this.
ReplyDeleteNICOSAN for the Treatment of Sickle Cell Disease
ReplyDeleteThere is a relatively new treatment for sickle cell being
produced in Nigeria by an American company called NICOSAN®,
it's proprietary name is NIPRISAN® . It was developed on
the premise of traditional Nigerian plant based medicinal
practices for the treatment of sickle cell disease.
It has been tested through phase IIb clinical trials and
found to be highly efficacious. Phase III trials have yet
to be completed however it was approved for sale in Nigeria
based on phase IIb trials and toxicity studies which showed
it to be safe and non-toxic.
Double-blind, placebo-controlled, randomised cross-over
clinical trial of NIPRISAN® in patients with Sickle Cell
Disorder
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B7GVW-4DS346T-1S&_user=10&_rdoc=1&_fmt=&_orig=search&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=211981d545303693affebb8c012d2cac
Efficacy of Niprisan in the prophylactic management of
patients with sickle cell disease
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6VS8-43DFJCH-G&_user=10&_rdoc=1&_fmt=&_orig=search&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=10528ecbab3ec7e977301fb9f2688ef6
NIPRISAN -- Nix-0699 Toxicity Studies
http://www.biospace.com/news_story.aspx?StoryID=15890720&full=1
Niprisan (Nix-0699) improves the survival rates of
transgenic sickle cell mice under acute severe hypoxic
conditions
http://www.blackwell-synergy.com/doi/abs/10.1046/j.1365-2141.2003.04536.x?journalCode=bjh
NIPRISAN Case, Nigeria
A Report for GenBenefit (2007)
http://www.theparliament.com/NR/rdonlyres/F46A1A12-0A1A-41DA-9F5D-A11486CA9BFA/0/Nigerian_Case.pdf
This drug is a major advancement in the treatment of sickle
cell disease unfortunately it is not available in the U.S..
Although the compound has been granted orphan drug status
by the FDA and the regulatory body of the European Union,
to date investigational drug applications for the approval
process have yet to be submitted. Getting a drug approved
in either area is extremely expensive. Until there is
funding available to proceed with the FDA and EU
applications it will be difficult for non-Nigerians to
obtain the drug.
I do say difficult but it is not impossible. If you have a
hematologist or hemoncologist who is willing to put fourth
the effort there are special dispensations available
through the FDA for the importation of unapproved drugs on
a compassionate use basis.
"Expanded access program (EAP). EAPs are typically designed
to provide widespread access to a drug that has proven
efficacy in clinical trials but is still awaiting FDA
approval. They’re similar to standard clinical trials with
a specific treatment plan and certain FDA requirements, but
they have looser patient eligibility criteria. More than
23,000 U.S. cancer patients enrolled in an EAP for Iressa
before it was FDA-approved, for example."
"Single patient use. This program offers an experimental
drug to an individual patient, rather than a group. The FDA
approves these uses on a case-by-case basis. Decisions are
based on other treatments already available and information
about the drug’s efficacy and potential toxicities."
http://www.curetoday.com/backissues/v3n3/departments/specialreport/index.html
To date I have no knowledge that anyone has sought any
single use or expanded access from the FDA for Nicosan.
Unfortunately regardless of the dissemination of this
information thus far no one has put forth the effort to
obtain the drug for use.
If just one person would start the ball rolling with a
caring and concerned medical practitioner it could open up
the drug for wide spread use by tens of thousands of
patients across the U.S. Unfortunately thus far the general
response I receive is that people don't believe that their
physician would be interested in going to this sort of
effort nor do they themselves seem to be inclined to seek
the use of a treatment that could potentially end their
crises.
There has to be at least one physician out there who has
enough care and concern for his patients to be willing to
put forth the effort necessary to obtain this medication
legally. I urge anyone who is effected by sickle cell to
approach their physicians with this information and attempt
to obtain this treatment not only for themselves but for
all patients who could potentially benefit from it's use.
We already know the benefits of the treatments available in
the U.S. and the E.U.. In many cases they are only
marginally effective or in the case of hydroxyurea cause
side effects so serious that many choose not to use it as
treatment. Here we have an opportunity to use a treatment
that has been shown to be highly effective, eradicating
crises in the majority of patients and reducing crises by
50% in the most refractory cases.
Although the clinical trial group was what the casual
reader might interpret as quite small it is common for
drugs which fall into the orphan drug category to use small
sample groups. Many orphan drugs have been approved based
on very small phase II and phase IIb clinical trials in the
U.S. In the case of FDA fast track status, a drug may be
approved during phase II trials if the drug shows
significant advantage over current approved therapies for
life threatening illness.
Fast Track Designation is a program that, if granted, is
designed to facilitate the development and expedite the
review of new drugs, thereby allowing the FDA to approve
drugs used to treat a serious condition or a
life-threatening disease with less safety data following
the conclusion of phase II studies, rather than phase III,
the normal practice.
The main criterion for a Fast Track Designated drug is the
potential to treat a life-threatening illness or fill a
major unmet medical need. Fast Track may be submitted with
the IND or at any time during the clinical development of
the drug. The Fast Track designation may allow a company's
application to follow Priority Review, Standard Review, or
a Rolling Review of the application.
http://www.fda.gov/CbER/gdlns/fsttrk.pdf
Nicosan by Western standards is an extremely inexpensive
drug. It is available in Nigeria without prescription at
$23/month for adults and child doses at $18/month.
Here is a link to the company and product website.
http://xechemnigeria.com/products.htm
I sincerely hope that you find this information helpful. I
would encourage you to to forward and post this information
to any person, blog or website where persons effected by
sickle cell anemia can have access to this information.
Feel free to write me with any questions or you may have.
NicosanForSickleCell@yahoo.com
United Nations Economic Commission For Africa
ReplyDeleteBook Of Abstracts
Science With Africa Conference
March 3-7, 2008
page 30
Evaluation of Niprisan (Herbal Medicine) for the Management of Sickle Cell
Anaemia
Charles Wambebe and Hadiza Khamofu, International Biomedical Research in Africa, Abuja,
Nigeria, wambebe@yahoo.com, Joseph Okogun, Nathan Nasipuri and Karynius Gamaniel,
National Institute for Pharmaceutical Research and Development, Abuja, Nigeria.
About 70% of all sickle cell anemia (SCA) subjects reside in Africa, estimated at over 12 million. The prevalence of SCA is estimated at over 2% while infant mortality is about 8% and survival rate of SCA babies in rural areas by five years of age is about 20%. These statistics indicate that SCA is probably the most neglected (and sometimes forgotten by health authorities) serious public health disorder with serious mortality and morbidity rates in Africa. The objective was to undertake pre-clinical and clinical assessments of a herbal extract vis-à-vis management of sickle cell anemia using Good Laboratory Practice and Good Clinical Practice principles respectively. In Africa, there is no standard treatment for sickle cell anemia, only palliative management is generally available. In view of this situation, most SCA subjects use herbal medicines. NIPRISAN is a standardized extract from four medicinal/food plants: Piper guineenses seeds, Pterocarpus osun stem, Eugenia caryophyllum fruit and Sorghum bicolor leaves. Short term toxicity study indicated that NIPRISAN was safe in laboratory animals. Bio-activity guided fractionation show that vanillin and aromatic aldehydes may be the bioactive moieties. NIPRISAN reversed sickled red blood cells and
protected them from being sickled when exposed to low oxygen tension. NIPRISAN dose-dependently delayed polymer formation of haemoglobin S. NIPRISAN induced 85% increased solubility of deoxy haemoglobin S. The in vivo efficacy study was undertaken at Children Hospital of Philadelphia, USA. Histological examination of lungs of control Tg transgenic mice carrying human sickle haemoglobin showed entrapment of massive numbers of sickled cells in alveolar capillaries. NIPRISAN significantly cleared the lungs of sickled cells. Furthermore, NIPRISAN induced profound effect on the survival time of Tg mice under hypoxic conditions (p<0.0001). The phase II clinical data indicated that all the subjects benefited from NIPRISAN with no serious adverse effect. About 80% of the subjects did not experience any crisis during the study (12 months). The subjects experienced significant reduction in hospital admission while attendance at school profoundly increased. Furthermore, there was no evidence of kidney or liver damage. NIPRISAN has been patented, licensed to an American company, registered and being manufactured at Abuja for global market.
http://www.uneca.org/sciencewithafrica/content/swa_book_of_abstacts-en.pdf
NICOSAN / NIPRISAN
I read you blog and am very upset about what you said, I have sickle cell, both my parents have the trait and I am never upset at both of them for loving each other and deciding to have children, they had four children, 2 of us have the disease and the other 2 don't, we the sicklers live normal happy lives, we just don't overwork ourselves or overtire ourselves, you are on the outside judging people's love for each other, how dare you? We do have crises but I never once blamed them as sometimes I think it pains them more to see me in pain. You don't know what it is like so don't go around spouting crap,
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