Journal Club Wilderness

Great post on Splenic Infarct at Altitude with Sickle Cell Trait

The crew has a very interesting post about a patient that suffered a splenic infarcture on a Peruvian trip.  Something to remember, as this patient had no known history of sickle cell disease or trait.


Thanks for the info!


  1. NICOSAN, FDA Orphan Drug for the Treatment of Sickle Cell Disease

    I would like to make you aware that there is a
    non-toxic viable treatment for sickle cell in the
    world and awaiting application for approval in the
    U.S. and E.U. In clinical trials 80% of patients had no
    crises and in the refractory 20% crises were reduced
    by half.

    Through awareness there may come availability for
    those who so desperately need it. All it is going to
    take is the effort of a few individuals, patients and
    doctors to make this drug a reality in the U.S. sooner
    than later. Although this drug was developed in
    Nigeria, the man behind it, Dr. Ramesh Pandey is a
    distinguished biochemist who has worked for the
    National Cancer Institute’s (NCI) Frederick Cancer
    Research Center as a Senior Scientist, Head of the
    Chemistry Section, Abbott Pharmaceuticals and
    produced the first commercially viable generic version
    of Vancomycin for Lyphomed Inc., a Visiting Professor
    at the Waksman Institute of Microbiology at Rutgers,
    the State University of New Jersey, holds patents for
    biotechnology analysis and rare drug production
    processes. He also holds several US and international
    patents for paclitaxel and its new analogs. He is a
    member of the Editorial Board of the International
    Journal of Antibiotics and of several professional
    societies. He has been awarded several grants from
    NASA, NCI and NIH. The drug NICOSAN has been granted
    Orphan Drug Status by both the FDA and E.U..

    This new treatment for sickle cell is being produced in Nigeria
    by an American pharmaceutical company, trade name NICOSAN®,
    and 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

    Efficacy of Niprisan in the prophylactic management of
    patients with sickle cell disease

    NIPRISAN — Nix-0699 Toxicity Studies

    Niprisan (Nix-0699) improves the survival rates of
    transgenic sickle cell mice under acute severe hypoxic


    NIPRISAN Case, Nigeria
    A Report for GenBenefit (2007)

    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.”

    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

    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.

    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.

    I sincerely hope that you find this information helpful. I
    would encourage you 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 you may have.

    Kristina Bruce RN

  2. 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

    Charles Wambebe and Hadiza Khamofu, International Biomedical Research in Africa, Abuja,
    Nigeria,, 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.

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