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Sickle Cell Crisis and Disease – Current Management (Final Part)

SCD Patient getting an injection

BY AZEEZ ADEWOLU POPOOLA ADEDUNTAN

B.SC (HONS), MBCHB**, MD, RVT, FACS, FICS

CONSULTANT VASCULAR SURGEON

CONSULTANT GENERAL SURGEON

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In this edition, we will conclude our series, on that disabling and hereditary disease – called Sickle cell disease. Notwithstanding the array of medications available in Year 2016 worldwide, there is no absolute cure for sickle cell disease, but relative cure, using bone marrow transplants - the success of which is modest, at best.

So don’t let anybody deceive, hoodwink or bamboozle you, either on Facebook postings, on Twitter handles and for that matter, a herbalist, Pastor or Alfas, that there is a cure for this disease at this material time. This is so because it is a genetic disease, inherited from your parents. In the future however, with the advent of molecular biology and genetic modulation in various laboratories, throughout the world, such a cure is not foreclosed.

Nigeria has the highest rate of sickle cell sufferers in the entire world. The specifics of this statistic are even more alarming and sobering. In fact, of the 200, 000 babies born yearly, with the disease on the African continent, 150,000 of them are Nigerian. Furthermore, about 100,000 Nigerian children are lost to the disease annually and 8% of the nation's child mortality deaths stem from sickle cell disease. Therefore, prevention is better than treatment or cure. So Get tested today and Know your Genotype before you marry anybody; no matter how strong the love.

Listed below are the current treatments (not exhaustive) that are available in the management of symptoms and signs of sickle cell disease discussed in our previous series.

Folic acid and penicillin

Children born with sickle-cell disease need close observation by the paediatrician to assure they remain healthy. These patients will take a 1 mg dose of folic acid daily for life. From birth to five years of age, they will also have to take penicillin daily due to the immature immune system that makes them more prone to early childhood illnesses, especially PNEUMONIA.

Malaria Prevention

While patients with sickle cell trait have only one bad gene (HBAS) - and tend to be protected from Malaria, this does not apply to people with sickle cell disease-two bad genes (HBSS); in fact, they are uniquely vulnerable to malaria. The most common cause of painful crises in malarial countries like Nigeria is infection with malaria. It has therefore been recommended that people with sickle cell disease living in malarial countries should receive anti-malarial chemoprophylaxis for life.eg PROGUANIL (Paludrine).

Vaso-occlusive (blockage of blood flow) or BONE PAIN crises

Most people with sickle-cell disease have intensely painful episodes called vaso-occlusive crises due to blockage of blood flow by the abnormal sickle cells. The frequency, severity, and duration of these crises, however, vary tremendously. Painful crises are treated symptomatically with pain killers, such as Panadol, diclofenac, Naproxen.and in severe cases with strong pain killers called opioids at regular intervals until the crisis has settled.

For more severe pain intravenous pain relievers like OPIOIDS may need to be administered.

Acute chest PAIN crisis

Management is similar to vaso-occlusive crisis described above, with the addition of antibiotics.

Hydroxyurea (HYDREA)

This is the first approved drug for the causative treatment of sickle-cell anaemia, and it has been, shown to decrease the number and severity of attacks and to possibly increase survival time. Side effects include infection and development of Leukemia.

NICOSAN (HEMOXIN IN USA)

Previously called (NIPRISAN), and its off shoot-Xickle RBC-Plus.

Nicosan/Niprisan was approved for the treatment of SCD in Nigeria in 2006 .It is an anti-sickling drug used for prevention of symptoms and pain management in patients with sickle cell disease (SCD). It is prepared from a mixture of four Nigerian plant materials: the seeds of Piper guineense, the stems of Pterocarpus osun, the fruits of Eugenia caryophyllum, and the leaves of Sorghum bicolor.Nicosan was developed at the Nigerian National Institute for Pharmaceutical Research and Development (NIPRD). It had been produced by Xechem International, Inc., an American-based company that has now filed for bankruptcy. NICOSAN     has been bought by another firm in the USA and now being marketed as a nutritional supplement under the trade name Xickle RBC-Plus.The latter has not been approved by the USA FDA.

Transfusion therapy

Blood transfusions are often used in the management of sickle cell disease in acute cases and to prevent complications by decreasing the number of red blood cells (RBC) that can sickle, by adding normal red blood cells.. In children prophylactic chronic red blood cell (RBC) transfusion therapy has been shown to be efficacious to a certain extent in reducing the risk of first stroke or silent stroke when non-invasive studies show abnormal increased brain blood flow velocities. In those who have sustained a prior stroke event, it also reduces the risk of recurrent stroke and additional silent strokes.

Bone marrow transplants

This is otherwise called Haematopoetic Stem cells transplantation. Big name –but simply means-a treatment procedure, where the bone marrow(remember our red blood cells are produced by the bone marrow) of the sickle cell patient is destroyed by radiation or chemotherapy, and repopulated by donor bone marrow cells from matched relatives, or from blood cells in the peripheral blood of new born or placenta cord blood. While cure is not absolute, it is relative and mainly available in the western world.

African Herbs, Medicinals and Concoction

While there are various African herbs and medicinal in the Nigeria markets - OSHODI, OJUELEGBA, IBADAN, BENIN, ABUJA, etc, that are being taunted as cure or treatment for this disease, the author does not wear the toga of superiority of Western Medicine over local herbs, but advises our readers to tread with caution, as the enlightened and educated public deserve to know the dosing regimen and side effects profiles of these herbs, as there is a rising incidence of liver and kidney failures, all over the world being reported by the unsuspecting and gullible public.

While the herbalists can vouch that they work, and they are not condemned, by this author, simply put, NIGERIAN ALERT cannot recommend them as part of the armamentarium of a well trained doctor, to prescribe these concoctions to patients without “proven” or proved efficacy.

We submit ONCE AGAIN, that it is inexcusable neglect for an educated couple to give birth to a sickler, in year 2016. So get tested today as Prevention is better than CURE, and there is no CURE for this disease at this present moment in time. Such a possibility, though a long shot, belongs to the future.

Thank you and best wishes for good health, from “NIGERIAN ALERT “to our readers, as Health is Wealth.