Kunle Tometi a Pharmacist, Entrepreneur and Public Health Advocate.
The World Sickle Cell Day is a United Nation’s recognized day to raise awareness about sickle cell disease (SCD) at a national and international level. On 22nd December 2008, the UN General Assembly adopted a resolution that recognizes sickle cell disease as a public health issue and “one of the world’s foremost genetic diseases.” The resolution calls for UN member states to raise awareness about sickle cell on June 19th of each year.
In this article, I would be creating awareness on sickle cell disease, the causes, symptoms, treatment and prevention.
What is sickle cell disease (SCD)
Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by inherited abnormal hemoglobin (the oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells.
SCD is more common in certain ethnic groups, including:
- People of African descent,
- Including African-Americans (among whom 1 in 12 carries a sickle cell gene)
- Hispanic-Americans from Central and South America
- People of Middle Eastern, Asian, Indian, and Mediterranean descent
- Approximately 2000 infants are born annually with the disease
- SCD affects approximately 200,000 Americans annually
- 1 in 365 African Americans
- 1 in 13 African Americans have the traits (carrying only 1 of the gene, S)
(CDC August 2017, Mayo Clinic)
Economics of SCD
10 years ago; Medical expenditure for children with SCD averaged $12,000 yearly for those with Medicaid and $15,000 yearly for those with commercial insurance.
There were also 113,000 hospitalizations costing over $500,000 paid by Medicare and Medicaid of which 75% of the visits were in adults and each with at least 3 Emergency Room visits per year. Children with SCD miss a minimum of 18 days per school year
Total healthcare costs nowadays for SCD is estimated at $2billion per year.
According to (David A.N et al 2018), ‘In Nigeria, the prevalence of SCD is 20–30/1000 live births. The burden of the disease has reached a level where it contributes 9–16% to under-five mortality in many West African countries. Hemoglobinopathies alone represent a health burden comparable to that of communicable and other major diseases’
Causes of SCD
Healthy red blood cells are round, and they move freely through small blood vessels to carry oxygen to all parts of the body. In SCD, the red blood cells become hard and sticky and look like a C-shaped called a “sickle” and they are not able to carry enough oxygen. When they travel through small blood vessels, they get stuck and clog the blood flow.
The sites most often affected by clogging or stacking of sickle cells are found in the lungs, liver, muscle, bone, spleen, eyes, and kidneys and other parts and tissues of the body: explains why patients complain of a lot of pain in these areas as the symptom of the disease.
Patients also have immunity suppression which leads to infections by bacteria, and viruses.
Symptoms of SCD includes;
- Excessive fatigue, irritability from anemia
- Jaundice (yellowing of eyes and skin), may also include retina damage
- Swelling and pain in hands, and feet, Pain in chest, back arms and legs, also damage of hip
- Frequent infections,
- Pain and problems in the spleen, (Nausea, vomiting, diarrhea)
- Delayed growth
- Stroke (20–30% of children stroke, 23% in African Americans)
- Genitalia (priapism, a constant erection, in which severe episodes may lead to impotency)
Treatment of Sickle Cell Anemia
Treatment of SCD pain or crisis is done in the following manner:
Rehydration: with IV fluids, helps Red blood cells return to normal shape
- Antibiotics: used to treat underlying infections. In some cases antibiotic prophylaxis, penicillins are recommended.
- Pain medications to treat acute pain
- Hydroxyurea: helps increase production of red blood cells
Immunization: Pneumococcal and Meningococcal vaccines have drastically reduced the rate of infections in SCD
Blood transfusion: improves oxygen and nutrients needed
Supplemental oxygen by mask makes breathing easier and improves oxygen levels in the blood
Bone marrow transplant: for severe complications and matching donors.
- Genetic counselling and testing (better before marriage and at pregnancy) can help prevent the likelihood of passing gene to your child
- Preventing infections can be achieved by practising simple hand washing techniques at every opportunity. Hand sanitiser gels and wipes are also available and affordable
- Immunisation is very important and one must assure shots and records are current to cut down on the rate of common infections.
- Re-hydration with fluids at all times is essential.
- Avoid staying in places with low concentration of oxygen, e.g. unpressurised air planes, or high altitudes
For more information about SCD, please speak to your Pharmacist or Doctor.
Article by Kunle Tometi a Pharmacist, Entrepreneur and Public Health Advocate.
- Mayo clinic https://www.gstatic.com/healthricherkp/pdf/sickle-cell-anemia.pdf
- CDC https://www.cdc.gov/ncbddd/sicklecell/data.html
- Sickle cell Disease: Public health agenda & Social, Economic and Health implications by CDR Althea M Grant, PhD September 2012
- Overview of the management & prognosis of sickle cell disease, Joseph Palermo, D.O.
- Economic impact of sickle cell Hospitalization. R Singh, Ryan Jordan and Charin Hanlon
- Prevalence and impact of sickle cell trait on the clinical and laboratory parameters of HIV infected children in Lagos, Nigeria
Prevalence and impact of sickle cell trait on the clinical and laboratory parameters of HIV infected children in Lagos, Nigeria.
Adaku Efuribe: COVID-19 treatment and the dangers of drug misuse in Nigeria
Adaku Efuribe (Image credit: Adaku Efuribe)
Drug misuse is defined as the use of a substance for a purpose not consistent with legal or medical guidelines (WHO, 2006). It has a negative impact on health or functioning and may take the form of drug dependence, or be part of a wider spectrum of problematic or harmful behaviour (Department of Health, 2006).
At the moment there seems to be an increased risk of self-medication and drug misuse especially in countries where prescription only medicines could be bought without prescription. For instance, countries like Nigeria where some patent medicines dealers who are meant to sell GSL medicines end up dispensing pharmacy only medicines and prescription only medicines.
As soon as a new drug for managing COVID-19 is announced by mainstream media, people run off to the shops to buy these drugs, even people who have not tested positive for the coronavirus, indulge in self-medication in a bid to prevent contracting the virus.
I am worried about the recent announcement for Dexamethasone as a new drug for treating COVID 19. Information reaching me shows, following hours of announcing this drug by the media, some Nigerian resident has started trooping to their pharmacy, ‘chemist’ and illegal drug dealers to buy dexamethasone tablets.
Dexamethasone is a corticosteroid, it has high glucocorticoid activity, and it should not be used without the guidance of a clinician. According to the Electronic Medicines Compendium (EMC), depending on the dose and duration of therapy, adrenocortical insufficiency caused by glucocorticoid therapy can continue for several months and in individual cases more than a year after cessation of therapy.
Through immunosuppression, treatment with Dexamethasone can lead to an increased risk of bacterial, viral, parasitic, opportunistic and fungal infections. It can mask the symptoms of an existing or developing infection, thereby making a diagnosis more difficult. Latent infections, like tuberculosis or hepatitis B, can be reactivated.
Dexamethasone also has some side effects; the following side effects are common (occurring in greater than 30%) for patients taking dexamethasone:
· Increased appetite.
· Difficulty sleeping (insomnia)
· Swelling in your ankles and feet (fluid retention)
· Muscle weakness.
· Impaired wound healing.
· Increased blood sugar levels
If only the media companies understood the fact that prescription only medicines could be bought without prescription in some countries, they would thread carefully and choose appropriate wording when announcing potential drugs for COVID-19 treatment.
It’s the duty of the Ministry of health and drug regulatory bodies of those countries where prescription medication could be bought in the market like sweets to continue to create awareness, educate the general public on the dangers of self-medication, drug misuse and drug abuse. They must not relent in their efforts of managing drug distribution/regulation.
My advice to people living in countries where you could buy prescription only medicines without prescription is this:
Do not run off to buy the latest drug announced for COVID-19 treatment.
This drug is a corticosteroid and should only be taken if prescribed by a clinician.
Please do not indulge in medication misuse and abuse. It could lead to adverse effects or even death
Author: Adaku Efuribe is a Clinical Pharmacist & Global consultant in Medicines Management
Sickle Cell Disease Educational Resources Initiative(SERI)- Our Story
Sickle Cell Disease Educational Resources Initiative(SERI)
Every year, nearly 300,000 children are born worldwide with this most painful disease and many of them will not survive beyond their fifth birthday. Discovered more than a century ago, Sickle Cell Disease (SCD) is an inherited genetic disease that is transmitted when both parents who carry hemoglobin S transmit it to their child.
SCD comes with many complications such as high blood pressure, kidney failure, kidney stones, growth delay, bone necrosis, stroke, retinopathy and increased risk of infection and sepsis. Treatment options include medications to manage the symptoms and blood transfusions to replace the sickled cells. A stem cell also known as bone marrow transplant might cure the disease. However, this procedure usually involves patients to have a matched donor, such as a sibling, who doesn’t have sickle cell anemia.
Both Agnes Nsofwa and Biba Tinga(Founders of The Sickle Cell Disease Educational Resources Initiative) gave birth to children with SCD type SS. Like all caregivers of children with a chronic illness, they had to face many challenges while managing their children’s health. Agnes, has a little girl who received a bone marrow transplant and was cured from SCD a year ago; She left a career in Business, trained to become a Registered Nurse in order to better understand the disease and care for her daughter. She fought hard for over 10 years to get her cured. Today her little girl is Sickle Cell free, but Agnes is still advocating for others who are still affected by SCD.
Biba has a young adult son living with SCD, but he is not a candidate for a bone marrow transplant to get cured. He will have to keep fighting every day of his life to stay healthy. As a treatment, he regularly receives Red Blood Cell exchanges or apheresis which allows him to avoid the terrible pain crisis. Their journey which started in Niger continues in Canada.
When they met for the first time in January 2020 in Amsterdam, they quickly realized they had been fighting the same battle. They have both been engaged in their respective communities, advocating on behalf of other families dealing with the same condition. Their combined years of experiences has taught them the need to unite. Because when life becomes a struggle, you engage with family. And sometimes family is someone who shares your life journey. After a short discussion, they knew they were going to join forces against this common cause and become friends.
Agnes had started the project translating sickle cell materials into her native language of Bemba from Zambia since 2018, in order to help others to better understand and care for their children since. When she shared this idea with Biba, she instantly agreed to come on board because she had also been sharing information in her native languages of Zarma and Hausa with parents of affected children.
Together they want parents, to have access to information so that they can make the best decisions to care for their children. To do this, they created SERI, Sickle Cell Disease Educational Resources Initiative, a platform of educational and informative resources on sickle cell disease in various languages.
As they present it, “we created SERI because without education we could not have looked after our children properly. We want all mothers to have what we did not have. SERI is more than a platform; it is also a movement for awareness and education. Whether you speak Bemba, Tonga, Hausa, English, French, Yoruba, Arabic, Hindi, Twi, Spanish or any other language, SERI will offer the information in the language you understand.
SCD requires long continuous care. When the parents or the patient does not understand the basic information, the consequences could be fatal. SERI will also provide audio recorded version in the local languages to ensure that those who cannot read and write can listen and still receive the education that will empower them to better care for themselves.
SERI will also share the stories and the experiences of those who are fighting SCD because their stories matter.
The stories will tell our journeys, the stories will say who we are. We are SERI!”
Digitisation, collaboration to help pharma supply chain weather Covid-19 impact
Pharmacist (Image credit: Reuters)
Sector must overcome capacity shortages to get supplies to patients worldwide
Stakeholders in the pharmaceutical supply chain are grappling with a growing gap between air cargo capacity and demand for lifesaving medical products, as flights are grounded and borders closed. Many African countries now report medicine shortages and challenges in securing costly imported pharmaceutical ingredients and medical equipment.
“Air freight capacity in Africa is reported to be down by 70% on what was available last year, even amid moves by regional airlines to repurpose passenger aircraft to close the supply–demand gap,” said Suzette Scheepers, CEO of Air Cargo Africa organisers Messe Muenchen South Africa.
“This echoes the situation around the world, where air cargo capacity has plummeted but demand for pharmaceuticals is spiking.”
The situation may be exacerbated in months to come as more non-pharmaceutical businesses return to production and compete for capacity, and airlines focus on the most lucrative lanes. Overcoming the disruptions of Covid-19 and the challenges of limited air cargo capacity will require collaboration, digitisation and innovation among all stakeholders in the pharmaceutical supply chain in the short to medium term.
This emerged during a Webinar entitled ‘The day after: how collaboration can prepare us for the impact after COVID-19’ presented by Pharma.Aero in partnership with STAT Times, who is a media partner to air cargo Africa.
Rita O’Sullivan, Head of Global Transportation at Zoetis, said during the Pharma.Aero webinar on Thursday: “The removal of 80% of passenger flights has had a significant impact on the pharma supply chain, with a knock on effect of increasing rates by between 200% and 500%. Reduced operations at sea and sea terminals have also increased lead time for customs clearance and handling, and non-pharma shipments not moving has added even more pressure.”
O’Sullivan noted that the pharma supply chain was a complex one, with thousands of nodes and lanes, and there was no one size fits all approach to cold chain and supply chain management.
“A concern for us is there may be a pause on new initiatives and development; and that not all airlines/freight forwarders will make it through this troubled time, so a return to ‘normality’ will not be straightforward,” O’Sullivan said.
New solutions for a new environment
Jaisey Yip, Vice Chairman of Pharma.Aero and Head of Cargo and Logistics at Changi Airport, said: “Covid-19 has exposed pharma supply chain companies to unprecedented challenges and risk. We should be taking a community approach and holding hands to ensure that life-saving medical supplies are safely and reliably transported from point to point.”
Digital technologies offer some solutions, said Ruud Van der Geer, Assistant Director Global Delivery Strategy Team at MSD. “Digitisation of visibility, detectability and control has never been more important,” he said, noting that MSD had last year embarked on a cloud-enabled digital logistics programme which had proved invaluable in managing distribution during the pandemic. “We incorporated data connectivity, real time shipping conditions sensors, end to end track and trace, proactive alerts and notifications, and predictive analytics and AI.
We also built in some insights to help us through this situation – tracking Covid-19 outbreaks, port and airport disruptions, average port dwell times, and Covid-19 trends per market. This enabled us to stop and reroute shipments, and support decisions on relocating critical products,” he said. “The next step is to move forward with the programme to move beyond a hypercare state to a more sustainable model.”
Innovative approaches can also help the sector address the challenges of reduced capacity and increasing cost, said Cihan Likogullari, Global Key Account Manager at Envirotainer. He cited examples of Envirotainer container optimisation which helped customers fit up to 35% more cargo onto each aircraft, and reduce losses due to temperature fluctuations in the cold chain. “We are all being pushed to do things in new ways, the only way to do it is through flexibility and collaboration,” he said.
“Air cargo is critical in the global fight against Covid-19. We need to maintain global supply chain quality standards in a reshaped landscape. Non normal operations require a robust safety risk assessment and implementation of mitigations; we need to reinforce globally harmonised standards and the consistent implementation of these,” said Andrea Gruber, Head Special Cargo at International Air Transport Association (IATA). “We need to accelerate digitisation and data sharing standards. We need to take a coordinated, constructive approach and introduce adaptive policies.”
Pharma supply chain challenges, solutions and opportunities will come under discussion at the 6th edition of air cargo Africa 2021, to be presented by Messe Muenchen South Africa at the Sandton Convention Centre from February 9 – 11, 2021. For more information, and to reserve exhibition space
Visit Air Cargo Africa