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Thursday, 15 March 2007

SICKLE CELL ANEMIA.

Some days ago, my neighbour lost his last child to Sickle Cell Anemia. His two other children died some years ago. The mistake he made was that he married a woman with Sickle Cell. He also had it. It's sad that at this day and age people still take little things like this for granted. Couples need to test themselves before getting married and bringing poor innocent souls into the world.

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

11 comments:

  1. 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.
    keep it up!

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  3. these are some hot bloggers.sto by their blogs and bring them over to yours.

    ReplyDelete
  4. Anonymous is right. If you stop on other people's pages and say hello, they'll defineltely come over to urs too and if they like what they read, will give you a shout out and even recommend ur blog on their page. London Buki does a lot of recommendations of other blogs. So do Bella Naija, Law Damsel, TaureanMinx.

    ReplyDelete
  5. My 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.

    Girl - 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.

    ReplyDelete
  6. 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.

    ReplyDelete
  7. NICOSAN for the Treatment of Sickle Cell Disease



    There 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

    ReplyDelete
  8. United Nations Economic Commission For Africa

    Book 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

    ReplyDelete
  9. 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,

    ReplyDelete
  10. I have removed this idea :)

    ReplyDelete
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Disclaimer: Opinions expressed in comments are those of the comment writers alone and does not reflect or represent the views of Linda Ikeji.

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