Sickle Cell Warrior #14

10003 Warrior Project Sickle Cell Warrior #14
Photo Credits : Paul Masamo

Hi, 

My name is Joy Risper Omondi, I am 6 years old and I live and study in Kisumu County. I am the first born in our family and I have one brother who is almost 2 and also a Sickle Cell Warrior. I live with my parents and due to having Sickle Cell Disease I always feel like my parents are discouraged by everything. They find it too much to take good care for me and my brother but they keep supporting and comforting me when in crises. I don't really understand what Sickle Cell is, all i know is I get sick a lot. The doctor has not explained what it is, my parents tell me it has no cure. I only can attend schools nearby my home for my parents’ fear of attacks while in school. I always live in fear of who will take care of me when I grow up like my parents do currently.  

As a young child I would like people living with Sickle Cell Disease to be treated equally and be given great medical care to enable us live a healthy normal life. 

Love Life!
Risper

Sickle Cell Symptom #2 - Infections

HI

My name is Lea and I have Sickle Cell Disease.

Ever since I was born i was handled with the utmost care so as ot to "break"

This meant, I never was allowed to do anything crazy like swimming, hiking, standing in the sun, eating outside home etc. While sometimes I may look back and think "wow! my parents were crazy paranoid" , I always feel a sence of gratiude for the best they did with the information they had at that particular time.

When you have Sickle Cell, your body is compromised from the get go. What do i mean? Okay, its quite simple. Knowing the nature of the disease; the sickling , destruction of red blood cells, anemia, reduced supply of oxygen and not to mention the extra amount of work the body has to do to ensure proper growth and development with its very little and stretched resources, the body is left defenseless. From the first doctors visit, a dose of penicillin is prescribed to up those defenses. However, this still doesnt hide the fact that Sickle Cell Warriors are quite suceptible to infections. 

From an article by Catherine Booth, Baba Inusa, Stephen K. Obaro on the International Journal Of Infectious Diseases , this is what I found out.

Infection is a significant contributor to morbidity and mortality in sickle cell disease (SCD). The sickle gene confers an increased susceptibility to infection, especially to certain bacterial pathogens, and at the same time infection provokes a cascade of SCD-specific pathophysiological changes. Historically, infection is a major cause of death in SCD, particularly in children, and it was implicated in 20–50% of deaths in prospective group studies over the last 20 years. Worldwide, it remains the leading cause of death, particularly in less developed nations. In developed countries, measures to prevent and effectively treat infection have made a substantial contribution to improvements in survival and quality of life, and are continually being developed and extended. However, progress continues to lag in less developed countries where the patterns of morbidity and mortality are less well defined and implementation of preventive care is poor.  SCD increases susceptibility to infections and the underlying mechanisms for susceptibility to specific pathogens.

Since you all know the clinical manifestations of SCD result from two key processes: vaso-occlusion (blood vessel obstruction by sickled red blood cells) and hemolysis (break down of red blood cells). Sickle cells, along with non-sickled RBCs, leukocytes, and platelets, form cell groups, which adhere to the vascular endothelium, causing obstruction of small blood vessels. This tiny circulatory obstructions lead to acute and chronic tissue damage and death, with multisystem effects, particularly in bone, lungs, brain, kidneys, and spleen.

Causes of Infection in SCD #10003 

Causes of Infection in Sickle Cell Disease

Impaired Spleen Function

The spleen has a key role in the increased susceptibility to certain bacterial infections seen in SCD. It functions as a phagocytic (protective cells i.e. white blood cells) filter, removing old and damaged cells and blood-borne microorganisms, and also produces antibodies. Individuals with SCD typically suffer from reduced and in some cases, lack of splenic function. The sluggish circulation through the spleen, high rates of O2 extraction, and local acidosis cause deoxygenation of HbS, promoting sickling, which leads to congestion and swelling of the sinusoids of the spleen with sickled cells. This can cause diversion of blood within the spleen, bypassing the normal filtering mechanisms. Macrophages (large stationary white blood cell) engulfing the abnormally shaped cells may become ‘blocked’, impairing their phagocytosis (ingestion of bacteria) of other particles.

Individuals with SCD hence they cannot mount a rapid specific response to captured organisms. Local infections can readily become systemic and this, in combination with the loss of the spleen's filtering function, can permit overwhelming sepsis (presence in tissues of harmful bacteria and their toxins, typically through infection of a wound) to develop. The main pathogen of concern is S. pneumoniae, though severe and systemic infections with H. influenzae, Neisseria meningitidis, and salmonellae also occur. Before preventive measures, children with SCD were 30–600 times more likely to develop invasive pneumococcal disease (IPD), including pneumonia, meningitis, and septicemia. Overwhelming sepsis can develop rapidly with no obvious primary source of infection, resulting in shock, disseminated intravascular coagulation, adrenal hemorrhage, and death within 24 to 48 hours. Mortality can reach 35–50% from septicemia and 10% in meningitis. The risk is confined almost exclusively to young children, with a reported incidence of 5.8 per 100 in children aged less than 3 years, 1.1 per 100 in those aged 5–9 years, and 0.6 per 100 in those aged over 10 years in the pre-treatment era.

While it is theoretically conceivable that children affected by SCD in malaria hyper-endemic settings like Kenya may be at increased risk of death from other pathogens such as malaria and invasive salmonellosis, poor diagnostic facilities and the high childhood mortality rates call this assertion into question. Well-planned longitudinal cohort studies to define the etiologic agents that predominate in SCD morbidity and mortality in this region are urgently needed.

Deficiencies in Micronutrients

Zinc is known to be important for immune function, so low levels in SCD have been suggested as a contributory factor in susceptibility to infection. Zinc deficiency is associated with lymphopenia (abnormally low levels of immune building white blood cells). Zinc deficiency may affect 60–70% of SCD patients. High protein turnover increases requirements, while hemolysis releases zinc, which is lost via the kidneys as renal tubular damage impairs reabsorption. At the same time poor diet and inadequate intestinal absorption could reduce intake. A study in 21 zinc deficient children suggested that giving supplements reduced the incidence of bacterial infections and cut hospital admissions.

Genetic Factors

Despite sharing the same underlying genetic mutation, the range of severity in SCD patients is striking, with some patients disabled by frequent crises and long-term complications while others live virtually normal lives. Individuals are also differently predisposed to particular pathological manifestations of the disease. This suggests that the SCD phenotype is multi-genic: since many unlinked genes are involved in the underlying extreme processes in SCD (such as destruction of sickled cells or endothelial adhesion), variation in genes at multiple locus points may modify outcome. Complexities in a number of genes involved in the immune response have been suggested as contributing to increased susceptibility to infection in SCD.

Mechanical Factors

The pathological effects of SCD can themselves create an environment supporting infection. Children with SCD are predisposed to osteomyelitis (inflammation of bone/bone marrow). The bone marrow space is expanded to accommodate the increased hematopoiesis (formation of blood/blood cells) needed to compensate for chronic hemolysis (red blood cell destruction), and oxygen demand is high. At the same time circulation is sluggish. Together these factors render bone vulnerable to vaso-occlusive (obstruction of blood vessel) episodes and infarction (tissue death). Areas of necrotic (dead) bone act as focus for infection, which becomes established via hematogenous (bloodstream) spread.

Edwardsiella tarda is another enterobacterium that has been reported with increased incidence in SCD. Increased gut permeability and biliary sludging in SCD is likely to be responsible for this association. It may be that patchy ischemia and infarction of bowel secondary to micro vascular occlusion permits gut bacteria to invade the intestinal wall and enter the bloodstream.

Another consequence of micro vascular disease is its association with acute chest syndrome. SCD carries an increased risk of prolonged and severe respiratory infections due to Mycoplasma, Chlamydia and other pathogens, particularly in children prone to pain or micro vascular sequestration, such as those with SCD.

Finally, SCD patients may be predisposed to certain iatrogenic (illness caused by medical examination or treatment) infections as a result of therapeutic interventions. Blood transfusion is commonly used to treat complications, particularly aplastic crisis or splenic sequestration (when Hb falls acutely) and acute chest syndrome, priapism, or strokes (when exchange transfusion is used to reduce the proportion of HbS). In general such programs are potentially associated with increased risk of blood-borne infections, particularly hepatitis B and C and HIV. Although all blood products in developed countries are screened for these viruses, standards in other countries may not be so exacting, so early hepatitis B immunization is recommended as a preventive measure. 

Now it is clear as day that my parents weren't crazy in being vigilant. As a sickle cell warrior speaking from experience, it is better to prevent than to treat. Sickle Cell is an expensive disease, however, it is way cheaper and less stressful to manage than to treat and bring the body back to balance when in crisis.

Next week we look at the effects of the infection on Sickle Cell Warriors and how we can prevent them. Until next time.

Love Life!

Lea

TRANSITION FROM PEDIATRIC TO ADULTHOOD IN SICKLE CELL PATIENTS

Before 1970s even in the industrialized world the life expectancy of a person with sickle cell was 14 years. Although most sickle cell warriors in Sub-Saharan Africa, still die before 5 years of age, but at least a reasonable number can transition to adulthood. Because most of the people with sickle cell disease died in their infancy the health-care was concentrated in caring for children and young adolescents and young adults.

Sickle cell has been considered a childhood disease. Most pediatric departments have well organized sickle cell medical services, but most adult hospitals lack the facilities for the adult patients.

The major challenge in sickle cell management is transitioning from pediatric to adulthood. Most sickle cell warriors find it extremely difficult to "accept life" as it is. According to my observation severe crises occurs when approaching adolescent/teen years. During this period the body wants to behave like other bodies at the same age yet its growth speed is slow. Girls want to see their breasts and hips grow. For boys they want to see their voice deepens. The slow response to growth and peer pressure from colleagues leads to stress and depression. Often some warriors find themselves excluded from their peers because they cannot keep up with the pace of body development. That's where the myth of people with sickle cell disease are anti social is born. I have been asked several times why sickle warriors are anti social but that will be a topic of another day. 

The major challenge for transition is lack of health care transition from pediatric to adulthood. Most of the doctors and nurses who handle people with sickle cell are pediatrics who are specialized in treating children. These are people who understand children not adult. If a person is blessed to survive childhood challenges you find yourself in a position where al most nobody "understands" you. In this case warriors find themselves out of specialized treatment centers. Most sickle cell warriors get lost in the transition from childcare to adult care.

Patients with sickle cell disease who transition from childhood to adulthood continue to develop physical complications. They also start to be affected by the disease psychologically.

While there are many advances in the treatment of sickle cell, there are still challenges in ensuring that the medical system and psychosocial system supports timely access to needed preventive and disease management protocols.

Due to lack of comprehensive care programs for adults and young adolescents with sickle cell, are forced to rely on emergency rooms to manage acute pains.

There is a need for comprehensive transition care programs to cater for young adolescents and adults with sickle cell disease. This is to make sure that transition programs are developed and education about transition can begin at an early age.

I understand the transition for most people is not smooth. Please share with us your transition journey.


Love Life!
Lea

SICKLE CELL: TO TELL OR NOT TO TELL YOUR PARTNER

Tina a friend of mine for a year now who is also a sickle cell warrior, has been grappling with a problem. She has been silently struggling with it for quite a long period of time.

I once asked her whether she is in a relationship and her answer was a NO. I went ahead to inquire whether she had dated before and Tina told me she has never dated before.

Tina told me that she wants to have her first date at 23. She is 22 years old now. I asked Tina whether it’s her wish to be single at her age against the norms. Many girls start dating as early as 12 years.

I dag deeper for more information because I believed telling me that she wanted to start dating at 22 was just a lame excuse. I found out that the reason she has for not dating is fear to disclose her sickle cell status to the partner.

This is the dilemma most warriors find themselves in on whether to tell or not to tell their partners. Many worry that they will lose their relationships when they tell their partners that they are sickle cell warriors. It is normal to feel nervous, embarrassed or even fearful of your partner’s reaction which may be verbal or even physical.

Disclosure is a process and has no written formula of when and how to tell. Some people prefer disclosing before the relationship, others want to disclose on the first date. Some want to do it after a certain period in a relationship. There are also those who will not disclose at all and prefer to have it “killing” them from inside.

My friend always wants to put it straight into the face of a man who wants to initiate a romantic relationship. She will tell from the start that she is a lady of ‘problems’ and whether you are ready to walk with her through the dark times.

It’s possible that your partner or spouse may change as time goes by. If you want to disclose you have to be aware that it may create new problems for you. There is still stigma attached to the whole subject of sickle cell disease and those who have it. Unsympathetic and prejudicial reactions are still common in some people.

There are important points you need to consider before disclosing;

• You need to ask yourself if the partner needs to know now or if it’s better to wait.
• Be prepared to talk about sickle cell in a clear way and provide basic information about what it means to have sickle cell disease.
• It may be helpful to have some information (printed material or websites) available to help with any questions your partner may have.

Do you have any experience you can share with us, where you had to disclose something private to a close friend, a lover or a spouse? How did it go and what was there response? Is there anything we need to keep in mind before approaching a sensitive subject.

Looking forward to hear your experiences.


Love Life!

Ssebandeke Ashiraf

Sickle Cell Warrior #13

10003 Sickle Cell Warrior #13

Lea
26 Warrior Years

"Sickle Cell is the best thing that ever happened to me.
It is not about the pain, the stigma and the depression. Its about what I have gained experiencing all of it. My ability to experience pain has increased my capacity to feel beyond that which many people can. And for me, stigma and depression are a constant test in my personal journey of self acceptance and self love. The daily physical , emotional and mental battles have made me the person I AM today, they prepare me to win this war I was born into. Everyday is an opportunity to live, love and learn. To those Sickle Cell Warriors who have yet to see their beauty, I urge you to trust the process, as messy as it is., and know that nothing happens by chance.

This title Sickle Cell Warrior, is a true priviledge."


Love Life!
Lea

SICKLE CELL MYTHS ABET STIGMA

In 2011 in my first semester of my final year at the university, I was seated in the lecture room holding a magazine with a photo of Mrs Ruth Nankanja Mukibi the founder and Executive Director of Sickle Cell Association of Uganda. By then she was 36 years old, my course mates asked me about the lady and I told them she was a sickler. Their uniform answer was that it she can’t be and I was just speaking ill about the lady. Their reasoning was that a person with sickle cell cannot live to celebrate their 9th birthday and if they live longer by God’s grace at least they do not make 12 years. I told them am also a sickler and am not dead. They were shocked. It was a difficult situation for me to confess because I did not know how I would be treated or what amount of stigmatization I was going receive.

I confessed being a sickler because I wanted to counter the myths and stereotype that comes with sickle cell disease, the people living with the disease and those who care for them. What I was waiting for was the harsh treatment I was going to receive from those people whom I had told that I have sickle cell. To my surprise their treatment was good. One of them who was just a colleague and a classmate from that time became my good friend and she always check on me to find out how am faring. Another classmate called a year after university and inquired about how to take care of her cousin who is also a sickler. What I had feared that my confession was going to haunt me has just turned out to be a blessing. I realized that these people were confined to the thinking that sicklers die early because they did not have anyone to counter and trash that myth.

Knowledge is power that is a universally accepted statement. A person who has been equipped with knowledge is better positioned to guide and also do the right thing. Myths, stereotypes, stigma and ignorance have all been erased by people who are well equipped with the right and useful knowledge

When I audit the last five years of my sickle cell advocacy, 8 out of 10 questions asked at every awareness session are about myths. It’s not surprising that sickle cell has remained one of the diseases that are poorly understood by the public. When it comes to our African society witchcraft and its association to the disease makes life of sicklers a menace.

I want to put the facts clear to counter the myths. Sickle cell awareness campaigns shall be smooth if the myths are countered and erased from the public domains.

Myth

Sickle cell is infectious and contagious.

Fact

This is one of the popular myths and has also greatly contributed to the stigma faced by people living with sickle cell disease Sickle cell is not contagious disease. It’s an inherited disease that passed from parents to the child. Just like skin colour which can not be changed when you sit, eat, play or sleep with someone with another colour also sickle cell can not be gotten by associating with someone with sickle cell disease.

Myth

Sickle cell patients die at 5, 9, 12, 16, 21, 25, 30

Fact

People living with sickle cell disease can live a normal life when taken care of well. Although their life expectancy may not be the same as other people but they can live longer. I remember Mzeei Bulasio Wamala a Ugandan who has been recorded as longest living sicklers who died in 2008 at the age of 78 years. It’s not true that people with sickle cell disease have an age ceiling which they can not live beyond. Most people are told that they will not live to celebrate their 5th birthdays, when they reach 5 they are told 9 after 9 its pushed to 12, 14, 16 19, 21, 30. Its only God who knows when a person will die even doctors don’t know. We should not believe the prophets of doom.

Myth

Sickle cell patients don’t bear children

Fact

This is a common “verse” for lack of better word being told to people with sickle cell. It’s common to find people with sickle cell confined in corners and have made to believe that they will never live to see the happiness of having their own babies. A person with sickle cell has the ability to conceive and bear children. There are a number of sicklers who have more than two children.

Every sickle cell awareness campaign should put a lot of effort on providing information that counters myths and sickle cell stereotypes.

Love Life!

Ssebandeke Ashiraf

Sickle Cell Warrior #11

10003 Sickle Cell Warrior #11

Etemesi
29 Warrior Years

I am a warrior born 29 years ago, and the journey has been full of pain and joy  at the same time. Like other warriors I have been in and out of hospital, and also like other warriors I have found unique gift that makes me special and significant.

Painful episodes and less frequent depression made me look at life as big mountain that I cannot climb. True, life is a  mountain I cant climb; especially if, my family and friends constantly look for hope in my situation. However, I can climb this mountain and bring hope; especially  when I look beyond the sickle cell  wound to the power of Christ that is in me. Power that works within me; that will enable the warrior in me, to reach the mountain of hope. 

I can now proudly say that I enjoy life as a warrior especially when I know that my significant role is to be a product of hope to my generation. As Paul puts it, that I can boast all the gladly about my weakness, so that Christ power may rest on me.. And in turn as a warrior, I will not only be a messenger of hope But a product of hope.

Catch up with me HERE as i continue through the journey that is life.

Love LIFE

Etemesi 

Where it all Began

From whence we came,
Find out how it all started and why we started the project.
This video was taken by Viionaries Aloud at the Pawa254 rooftop.

I still cant believe we made it this far, 5 months later. The greatest lesson learnt through this journey is that patience, persistence and courage go a very long way. Yo don't need to be superhuman to make change. As long as you understand there is no chnage greater than that made within yourself.

If you cant view the video above, watch it here 10003 Warrior Project

Let us know if you've interacted with the project or if you would like to do so.


Love Life!
Lea

Sickle Cell Warrior #9

10003 Sickle Cell Warrior #9

Mercy A

19 Warrior Years

I don't know what it is with girls and their fathers', perhaps a father can give us a bit of introspect into this subject.

When i first met Mercy, she like many other younger warriors were very keen to participate in the project. To speak out about a very sensitive issue that affects numerous young girls in Kenya takes a great deal of courage.

Courage is the one thing that i was reminded of when i talked to Mercy. She has faced great hardship in her daily battle with Sickle Cell disease. Many times she has had to stay in one class as her classmates advanced to the next level, as she tries to catch up on the missed lessons. In a system that doesn't quite cater for special needs students with SCD, many of them are shunned by teachers and deemed not good enough or just another non-performing cliche. 

Mercy however rose above those mental models society knowingly or unknowingly bestows on us. The biggest one being, Sickle Cell Warriors are lazy, dumb and sickly. 

 Here's how Mercy sees things

“Being a warrior does not make you different from others. Most of us have been told a lot about our lives but in all I have built my faith on hope and faith. I have ignored the voices of doctors and anyone who pulls my hope down, that’s why I’m still moving on. In all I've trusted in God.”

Love Life!

Lea

IS SICKLE CELL OVER 1003 YEARS OLD?!

Have you ever wondered where diseases originate from?

Why does disease arise and how do they come about?

How in the world did Sickle Cell Anaemia come about?

Sickle Cell Disease is a very old disease. It is older than you, me and the next two readers combined.  How do I know this you ask? Well, I connected the dots.   And as our good friend Steve Jobs put it, “You cannot connect the dots looking forward; you can only connect them looking backwards.”  So let’s go back in history.

The Discovery

In the annals of medical history, 1910 is regarded as the date of the discovery of Sickle Cell Disease, making 2010 the 100th anniversary of that discovery, but just what does it mean to say the disease was “discovered”? The disorder we call “Sickle Cell Disease” often abbreviated as SCD, had been present in Africa for at least five thousand years and has been known by many names in many tribal languages. What we call its “discovery” in 1910 occurred, not in Africa, but in the United States.

Enough about that, let’s go further back in time before 1910.

I’m sure we all know that Sickle Cell Disease comes about when two individuals with the Sickle Cell Trait/Gene come together; they pass down the gene and give birth to an individual with Sickle Cell Disease.  If this is new to you, go back and learn the basics HERE. The key word we need to note is Sickle Cell Trait. And since Sickle Cell Trait is the cause of Sickle Cell Disease, let’s look into the cause and origin of the Sickle Cell Trait.

Sickle cell trait occurred as a natural mutation of the haemoglobin gene. Sickle trait served and still serves as a protective mechanism against Malaria. Malaria is a deadly disease found in countries along the equator. People with Sickle Cell Trait are protected from Malaria while those with Sickle Cell Anaemia and normal haemoglobin are susceptible to it. Over the years people with Sickle Trait migrated to other continents to parts of the Middle East, Central India, and countries bordering the Mediterranean Sea, especially Italy and Greece.

So we now know the cause of the Sickle Cell Trait, Malaria resistance, what next? Yes, you got it; let’s have a look into the origins of Malaria.

King Tut's Golden Mask from Ancient Egypt

Malaria

The first evidence of malaria parasites was found in mosquitoes preserved in amber approximately 30 million years old. Human malaria likely originated in Africa and coevolved with its hosts, mosquitoes and non-human primates. About 10,000 years ago, Malaria started having a major impact on human survival, coinciding with the start of agriculture in the agricultural revolution. Consequences included natural selection for Sickle Cell Disease, Thalassaemias etc, because such blood disorders confer a selective advantage against Malaria infection (balancing selection). The major types of inherited genetic resistance (Sickle Cell Disease, Thalassaemias, etc) were present in the Mediterranean world by the time of the Roman Empire, about 2000 years ago.

To dilute it down, the information simply means that Sickle Cell Trait came about as a way to resist Malaria. Darwin's theory of 'survival of the fittest' is demonstrated well as individuals developed stronger genes to prevent from Malaria. The stronger gene is Sickle Cell Trait. However since there was no knowledge of this back then, people went about their business as usual. Intermarrying within gene families was not uncommon then which increased the chance of intermarriage between people who developed the Malaria resistant Sickle Trait, therefore bringing about Sickle Cell Disease.

King Tut of Egypt

Tutankhamun, is the world’s best known pharaoh. There are many speculations about the death of King Tutankhamun at the ripe age of 19 years. Why I bring this up is to show and demonstrate the age of Sickle Cell and how far back the disease had been existing.  Since the discovery of Tutankhamun's mummy, there has been a lot of speculation and theories on the exact cause of death, which until recent studies had been hard to prove with the evidence and data available.  One of the theories that caught my eye was one by Christian Timmann and Christian Meyer. They came to the conclusion that Tutankhamun did not die from a combination of bone disease and malaria, but instead had Sickle Cell Disease. Dr. Timmann and Dr. Meyer believed the Sickle Cell Disease turned fatal when Tutankhamun also contracted severe Malaria that was rampant in Ancient Egypt during his era. Tutankhamen's parents are thought to be related, boosting the chance that they both carried the Sickle Cell Gene. He is expected to have been homozygous recessive for the Sickle Cell Gene, thus making him not immune to severe malaria, which would have been fatal. So tell me, Is King Tut the oldest recently recorded Sickle Cell Warrior/patient?!

Wow isn't it all so surreal! The Sickle Cell Gene has survived millennial, centuries and decades of time up to this day and age. Sickle Cell Warriors such as myself can trace their gene back to the likes of King Tut.  Imagine a family tree that can be traced back in time to the beginning of ancient civilizations and evolution of mankind!

Coming back to the present moment, with the dots hopefully connected, do you feel you have a bit of understanding on the origins of Sickle Cell? I feel this brings everything into perspective and helps me understand why Sickle Cell Disease is. And not to mention the great need a couple thousand years and 115 years later, when humanity is still looking for sustainable solutions on Sickle Cell disease management.  

I say Knowledge is the Key! 

Love Life!

Lea

TO ADHERE TO TREATMENT SICKLE CELL PATIENTS NEED TO BE EDUCATED

In her debut memoir Living with sickle cell disease: The struggle to survive, Judy Gray Johnson 72, one of the longest living person with Sickle Cell Disease recollects that she was 16 years in 1959 when she had gone with her aunt to a doctor who diagnosed her with Sickle Cell Disease. She writes that the social mores of that time dictated that adults discussed nothing of substance with children. She was never told anything concerning Sickle Cell Disease.

It may seem that was for the 1950s or for the last century and never crossed into the 21st Century (2000s) or the information age. That has not been the case, in 2002 when I was first diagnosed with sickle cell 43 years after Judy was diagnosed it was the same story for me..

"That’s a universal situation. Most Sickle Cell patients lack knowledge about their condition"

I was 12 years then, when the doctor handed the results to my father nothing was taught to me about the disease I was going to live with for the rest of my life. It was only the cocktail of drugs prescribed after the diagnosis that was explained to me.

Were these people reading the same script? It’s the question I still ask myself.

That’s a universal situation. Most Sickle Cell patients lack knowledge about their condition. Some patients finds themselves in dilemma when they don’t know the symptoms, warning signs when a crisis is going to happen and what to do to arrest a crisis.

Patients with Sickle Cell need to be better educated about their health. Most are less likely to get accurate information about their disease. One mother to a Sickle Cell warrior daughter shared her experience with a nurse; she revealed that a nurse who even treats other Sickle Cell warriors told her that Sickle Cell is infectious and any person can get it even later in life. If a medical personnel can give unforgivable information like this, I don’t this this nurse can give correct information about treatment and how to avoid a crisis.

"The most difficult stage for people living with Sickle Cell Disease is transition from pediatric to adulthood. Because previously it was difficult to find many adults living with the disease,"

Still some of the patients lack a better understanding of many symptoms of Sickle Cell and there is a need for improved patient education. Improving patients understanding about why a particular treatment is important for an individual situation may lead to more informed decisions and better adherence to treatment.

A better comprehension of one’s symptoms and reasons for personalized treatment recommendations also could improve a patient’s trust, confidence and satisfaction with primary treatment provider.

The most difficult stage for people living with Sickle Cell Disease is transition from pediatric to adulthood. Because previously it was difficult to find many adults living with the disease, so all the efforts and resources were channeled to caring for children with Sickle Cell. The situation has now changed and sicklers progress to adulthood.

So now there is a need to have a special education programme for adolescent and adult patients. The programme needs to cover issues like the reasons for taking daily drugs, different pain coping methods and reasons for delayed growth. This can also reduce on the stress experienced by most teens.

A comprehensive patient education program can focus on connecting adult patients with sickle cell disease with primary care providers and support the relationship. Help older adolescents and young adults with Sickle Cell Disease to transition smoothly from pediatric to adult primary care doctors and finally primary care providers to care for people with sickle cell disease of all ages and provide necessary tools to deliver high quality care.

Love Life!

Ssebandeke Ashiraf

Catch up with me HERE where you can read more of my written pieces on Sickle Cell Disease. 

IMPORTANCE OF NUTRITION IN SICKLE CELL DISEASE

Nutrition! What has food got to do with my red blood cells? I’m sure you’re probably wondering what the big fuss is all about, but nutrition plays a very significant role in our lives. It is important for growth and development and a strong immunity against infections. In patients with Sickle Cell Disease, appetite and immunity are greatly affected which results in poor growth, poor sexual maturation and increased susceptibility to infections. This affects the quality of their lives and will often be sickly if basic proper care is not given.

Therefore dear warrior, keep in mind that you have extra requirements for nutrients such as energy, protein, water, folate & zinc. It is recommended that you take extra vitamins and minerals in order to replenish those being lost or those on higher demand by your body due to the nature of the disease. Extra protein and energy are required to promote growth & maturation especially if you are a child or adolescent with Sickle Cell Disease. Folic acid is needed in extra amounts so that it can aid in the increased production of red blood cells needed to replace the destroyed ones, while zinc is needed to increase oxygen affinity and perform its functions in skeletal & muscle growth and sexual maturation. While these are important we will also discuss other nutrients whose increased intake aid in management of Sickle Cell Disease. These include Vitamin A, Vitamin E and Vitamin C.

Hearty Nutrition for Sickle Cell

Food choices in Sickle Cell Disease have great health benefits to you and even go beyond providing nutrients only –they can affect how frequent your symptoms occur, imagine that! Most foods that have been implicated with triggering crises are animal protein sources due to iron overload and their complexity in digestion. Vegan diets have been associated with less frequent and severe episodes (yes contrast to common thought, being a VEGAN aids in much more than just weight loss). Your dietary intake may be low because of abdominal pain crises caused by some certain foods, but at the same time you need a high caloric intake because of hyper metabolism caused by the constant inflammation. Therefore, it is very essential for a healthy Sickle Cell diet to avoid trigger foods. Your diet should be low in:

• Sodium –a component of salt. If you’re one of those people who grab the salt shaker right away before tasting your food, keep note! Salt leads to dehydration by ‘sucking’ the water out of our body cells leading to painful crises episodes.

• Added sugars –such as those found in sodas, processed juices, sweets & candies cause dehydration in the same way as salt does.

• Solid fats – include saturated fat and trans fatty acids which adversely raise the levels of low-density lipoprotein (LDL)/ bad cholesterol and lower the levels of high-density lipoprotein (HDL)/ good cholesterol in the body. They cause abdominal obesity, inflammation and insulin resistance. In the long term, consumption of trans fats raises the risk of obesity, diabetes, heart disease, stroke, depression and many more diseases.

• Refined grains and products –such as white baked foods (bread, cake, mandazi and donuts), white rice and white refined flour are easily digestible & release glucose quickly. This causes a rapid spike in blood sugar, followed by a surge of insulin in the blood, typically followed by reactive hypoglycemia –a low energy feeling caused by over-secretion of insulin. This is why we feel so hungry & weak after eating such foods. Such foods also lack some nutrients present in their counterparts –whole unrefined grains.

Watch Importance of Nutrition Video , a short pictorial on cheap available foods that are easy to source within Kenya

Nutrition is irrefutably very important in managing Sickle Cell Disease. Don’t you agree? And this is just a tiny drop from an ocean of health benefits. Log on to see the nitty gritty details of how each nutrient we have talked about helps in managing Sickle Cell Disease & discover their richest sources. But before you do that remember that power is gained by sharing knowledge, feel free to comment below on how nutrition is so important to you. Until then, eat healthy & stay strong.

Love Life!

Sharlene Mule

WHAT IS SICKLE CELL DISEASE II?

Last week we started our discussions on Sickle Cell Disease. I gave you and introduction on the basics of Sickle Cell Disease. If you missed it, click HERE to read. It is a series of information that will be building up from where we left off last, hence it’s important to start from the beginning.

As many of you know, Sickle Cell Disease is a foreign term in Kenya. Not because we don’t have cases in Kenya but because there is not much awareness of the disease within our country. Awareness plays an importance of ending ignorance on a certain issue. And in this case creating awareness of Sickle Cell Disease ends the ignorance brought about by sheer oblivion of information on this social issue.

What I’m’ trying to say in very many words is this; we will not be able to drive change without identifying the need of that required change. Why do we need change?! And we will not identify the need without creating awareness of the many lives lost to Sickle Cell Disease and not to forget the ones who continue suffering due to lack of information and support systems/policies.

Sickle Cell awareness creation seeks to end the viscous cycle of misinformation and death of a community. This is by the simple fact of providing knowledge to a community to enable you to make informed decisions about yourself, your family and your community.

Last week I mentioned that few people in Kenya know about Sickle Cell and even fewer know about their Sickle Cell status. It is important to know your status as it helps you know whether you carry the Sickle Cell gene.  This will help you be aware of the risk of having children with Sickle Cell Disease.

It is most important so as to be screened to help in diagnosis and confirmation and also to monitor any treatment administered by doctors.

I need to know if I carry the gene. How can I be tested?

A simple blood test called the hemoglobin electrophoresis can be done by your doctor or hospital. This test will tell if you are a carrier of the sickle cell trait or if you have the disease. We certainly don’t like giving you half bake information that may be inaccurate; please exercise patience with us as we conduct our research. Stay tuned for a deeper discussion on where you and your family can get the test done in Kenya and at how much. We are still conducting research on where we can direct people and what are the methods and cost variability between the different health center labs.   We will also give you an overview of methods of evaluating the type and amounts of various normal and abnormal hemoglobin types. This will be discussed in a later post on April 1^st 2015. I promise :)

What will this test likely tell me? Are there different types of sickle cell disease?

This test will let you know of which gene/genes of Sickle Cell you carry. There are three common types of sickle cell disease

·         Hemoglobin SS or sickle cell anemia

·         Hemoglobin SC disease or Sickle Cell Carrier

·         Hemoglobin sickle Beta-Thalassemia

You now know what Sickle Cell Disease. Tell me why  it is so bad; What complications are associated with Sickle Cell Disease

Complications from the Sickle Cells blocking blood flow and early breaking apart may include but not limited to:

·         pain episodes

·         strokes

·         increased infections

·         leg ulcers

·         bone damage

·         yellow eyes or jaundice

·         early gallstones

·         lung blockage

·         kidney damage and loss of body water in urine

·         painful erections in men (priapism)

·         blood blockage in the spleen or liver (sequestration)

·         eye damage

·         low red blood cell counts (anemia)

·         delayed growth

Symptomatic Expressions of Sickle Cell Anaemia

What can be done to help prevent these complications?

Sickle cell patients should be under the care of a medical doctor that understands Sickle Cell Disease. All newborn babies detected with Sickle Cell Disease should be placed on daily penicillin to prevent serious infections. All of the childhood immunizations should be given plus the pneumococcal vaccine. Parents should know how to check for a fever because this signals the need for a quick medical check-up for serious infection. The following are general guidelines to keep the sickle cell patient healthy:

·         Taking the vitamin folic acid (folate) daily to help make new red cells

·         Daily penicillin until age six to prevent serious infection

·         Drinking plenty of water daily (8-10 glasses for adults)

·         Avoiding too hot or too cold temperatures

·         Avoiding over exertion and stress

·         Getting plenty of rest

·         Getting regular check-ups from knowledgeable health care providers

Patients and families should watch for the following conditions that need an urgent medical evaluation:

·         Fever

·         Chest pain

·         Shortness of Breath

·         Increasing tiredness

·         Abdominal swelling

·         Unusual headache

·         Any sudden weakness or loss of feeling

·         Pain that will not go away with home treatment

·         Priapism (painful erection that will not go down)

·         Sudden vision change

Well, there you go. I trust this explanation gives you a bit of introspect on what Sickle Cell Disease is how it manifests in the body’s of the Sickle Cell Warriors. Do you know anyone who has Sickle Cell Disease? Kindly comment below and tell us if this article has helped you to understand what they go through.

Are you a sickle cell warrior? Let us know what new thing you've learnt today about Sickle Cell.  Leave us a comment or a question that you may want us to cover on this series.

Stay tuned for the next exiting posts that will look into the origins of Sickle Cell. Find out the oldest record of Sickle Cell known to man. Until then  

Love Life!

Lea