Definition of envy according to the Merriam-Webster

: painful or resentful awareness of an advantage enjoyed by another joined with a desire to possess the same advantage.

The great Aristotle defined envy as pain at the sight of another’s good fortune, stirred by “those who have what we ought to have”. Bertrand Russell the great British philosopher and polymath said that “envy was one of the most potent causes of unhappiness. Not only is the envious person rendered unhappy by their envy, but that person may also wish to inflict misfortune on others to reduce their status.”

I recently read an interview titled “I Envy HIV/ AIDS Patients In Nigeria – Maureen Aisha Edward, A Sickler” and I was so moved by the title and the article that I decided to explore envy and it’s historical significance/relevance in my life.

Envy and Sickle Cell often come as a package deal thanks to our life experiences and interactions with basically anyone and everyone. Throughout my life I have envied classmates, friends, acquaintances, random people passing by on the street, athletes I see on tv playing the sports I love and will never get to play and, I’m even ashamed to say, I have envied people with other medical conditions not as serious as Sickle Cell or worse than Sickle Cell as long as they didn’t have what I have. I heard Stephen Fry say on a podcast about envy recently, and I paraphrase here, ‘envy is a slow burn that seeps into us as we sleep, envy can make you hate others or hate yourself. Envy is the outward projections of the deepest depths of our self hate, our jealousy of things we can’t attain because of whatever reason.’ That hit me hard.

The Nature of Envy is one of the more intriguing articles I have read recently and it also spurred on my curiosity about the mental health effects of envy, something all Sicklers struggle with like I mentioned earlier. “Envy can be a destructive emotion both mentally and physically. From my experience envious people tend to feel hostile, resentful, angry and irritable. Such individuals are also less likely to feel grateful about their positive traits and their circumstances. Envy is also related to depression, anxiety, the development of prejudice, and personal unhappiness.” Obviously such a negative emotion and mental state can impact physical health. And what happens when we feel stressed and overwhelmed, we get a Sickle Cell crisis.

Envy is generally considered a socially unacceptable emotion, it appears in two of the Ten Commandments of the Old Testament and is even listed as one of the seven deadly sins for gods sake. Many of us deny having these feelings both publicly and privately but we all suffer from envy occasionally.

Recently after a long discussion with a therapist, I just started seeing, about my medical history and its contribution to my sometimes overwhelming social anxiety, she hit me with this line; “You have always hidden your condition as a way of trying to project outwardly that you are the same as your peers. You have always wanted to fit in and by not accepting yourself as you truly are you will never feel like you actually fit in. You have been running away from the thing you are chasing the most.” The cogs in my brain just shut down and I was speechless for at least a minute. At the root of my social anxiety that led to repetitive self destructive behaviors like overindulging in drinking and some drugs was ENVY. I still think about this line at least 16 times a day, once every hour I am awake.

Personally I feel I have grown a lot and I am still growing, I am much much less envious than I ever used to be and I’ll never be completely rid of it as long as I have this condition. In my lowest most painful moments envy and pity will always rear their ugly heads. But I have learned different tips and tricks on how to deal with envy, some of the best ones are from that article The Nature of Envy

Dr. Richard Smith and Dr. Sun Hee Kim, from the University of Kentucky had great suggestions that were mentioned in the article. Below is their advice on how to try dismantle thoughts of envy with a variety of cognitive therapy techniques and strategies, including but not limited to:

1. Self-Reliance and Perseverance. To “perseverate” is to repeat an action over and over. In this instance, the term is used to suggest that you repeatedly examine your thoughts to determine whether they are envious. If you find that they are envious at any given moment, remind yourself of how these thoughts don’t help your life and can actually harm it. The more you can manage to catch and correct your thinking, the easier it will be to remain envy-free.
   
2. Selective Ignoring and Distraction. When you find yourself thinking envious thoughts, quickly remind yourself that the other person’s advantage isn’t important in the grand scheme of things, and then focus on other thoughts (a pleasant memory, things that need to be done, etc) or engage in another activity. By distracting yourself with another absorbing thought or activity, you can stop your envious thoughts in their tracks. 
   
3. Self-Bolstering involves reminding yourself of your own positive qualities and advantages. This strategy doesn’t seem to reduce envy itself, but can make you feel less angry and depressed in the face of your envy.

If none of these suggestions help I personally recommend and can attest to to seeking help from a trained mental health therapist.

I hope this article interests or helps you, or even if it didn’t, thank you for taking your time to read this. No one is ever alone dealing with their struggles in this world. This blog is my way of processing that and I hope others can realize this too.

Note: This is a personal blog and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.

It has been exactly a year this week since I moved back from California to Kampala abruptly and unplanned, in the midst of a global pandemic. It is something I did not even want at the time, but looking back it is something I desperately needed. In that way this pandemic served as the end to an amazing chapter and the beginning of an exciting new one. The peace of mind brought about just knowing I am a few miles away from friends and my whole family compared to the thousands of miles that separated us before is an indescribable mental weight off my shoulders.

Although it has been great being back the transition has definitely come with its own challenges. Undergoing and recovering from a hip replacement only a couple of weeks after just having come back. Having to readjust to a new (but old) environment for the 3rd time in 4 years (4th in 8 years). Having to depend on my parents again when I was so used to an independent and fairly reserved life. Getting a new job and finding a new apartment, getting used to different healthcare standards and getting used to a more active social life in a city that never sleeps even during a lockdown. Let’s just say that learning to live in Kampala at this age, in a city I have spent less than a quarter of my life living in and visiting over 30 years, is like learning to ride a bike without training wheels. Most people have to deal with regular work-life balance. People with sickle cell who work have to deal with work-life-health balance. Finding your work-life-health balance can be challenging to say the least and even more so when you have sickle shaped blood cells running through your veins causing a whole heap of additional stress, fatigue and pain.

Uganda, like many other countries, has a very career and status driven culture. The career ladder is always an ever present topic over meals at family gatherings between parents, grandparents, aunties, uncles, neighbors, friends and even those village elders you see once a year on Christmas. Since I got back I have had issues with the way terms like ‘career ladder’ are used. Mary Shaniqua addressed it better than I could in her blog post called ‘How to Cultivate a Successful Career While Living With Sickle Cell Disease’. She said “We often speak about career ladders, but I think that term is one-dimensional and linear. I prefer to liken careers to a mountain climb. Unlike climbing a ladder, climbing a mountain is not as simple as step up, step up, and so on. Instead, a mountain climb may require you to take lateral steps, or even steps backward, to eventually get to the top.”

Recently I feel like I have been making a lot of backwards and lateral steps. The unnecessary attachment of age to life goals has messed with me. The pressure of trying to stay healthy and sane while attempting to climb the “career ladder” to the point where I could consider myself financially secure and independent without having to work 10+ hours, 7 days a week is intense. I have realized that it doesn’t help that I am constantly comparing myself to my friends and colleagues like we are on a level playing field in this game of life.

I don’t want to limit my capability using my health as an excuse. I need to learn how to prioritize what is important, balance my decision-making, be able, flexible and willing to alter my long-term career plans and general expectations from life due to my sickle cell-related complications, and I have to get comfortable in knowing that it is OK to do so.

“We need to do a better job of putting ourselves higher on our own ‘to do’ list.” —Michelle Obama, former First Lady

We are called Sickle Cell Warriors for a reason. We are tough, resilient fighters. But because we are so often seen as such strong people, it’s easy to overlook our inner struggles, which can lead to devastating outcomes such as depression or suicide. Chronic pain is associated with depression and other psychological disorders. According to the American Foundation for Suicide Prevention “This is primarily due to psychological states such as hopelessness, helplessness, and desire for control over death.” 

Sickle Cell and depression are major health concerns as separate entities. Thousands of people are hospitalized from these diseases or complications. There are racial and ethnic disparities in healthcare where minorities are sometimes under-treated and symptoms of depression are sometimes overlooked. But what happens when these two illnesses are coupled together?

It’s not simply just having a chronic illness like Sickle Cell that causes someone to be depressed or think of ending their life, it’s the quality of life that that person is actually living. For people who live with Sickle Cell that are generally able to maintain good health and have infrequent pain episodes, the risk of depression and suicide is definitely lower. On the other hand, patients who experience higher occurrences of crises are at increased risk. Depression increases as the severity of the disease increases.

As stated in this Journal of the National Medical Association article, some studies estimated that Sickle Cell patients experienced a lifetime rate of depression at 50%. Other studies estimate between 35% – 50%. Depression is a major factor in considering suicide. As one may assume, education, socioeconomic status, access to medical treatment, and access to weapons or drugs can also play a part in whether or not someone may consider suicide.

Patients, especially Black patients with chronic illnesses tend to “suffer in silence”. This may be due to the social stigma in the Black community that if you admit that you are depressed, that people or society would think you are “crazy” and shun you, so therefore a significant amount do not report symptoms to their physician. These patients need to be made aware that people with chronic illnesses do experience signs and symptoms of depression, and that it is acceptable to talk about it with their healthcare provider, close relatives and friends. Religion should also be looked at as important factors as to why these patients do not report depressive symptoms or think they are depressed. 

Psychological therapies should be offered as a standard of care, and Cognitive Behavioral Therapy (CBT) and/or antidepressants should be started immediately when necessary. It is shown that having a good support system improved one’s quality of life. More support groups should be developed for those patients who do not have the support of family or friends.

On a more positive note, quotes from @sicklecell101 on Twitter.

Sickle Cell has taught me…

“Sickle Cell has taught me to believe that suffering has its unique benefits. My life with Sickle Cell has made me a person more capable of enduring. Making it through lets you know that you can make it through. My endurance allows me to continue the fight.”

“To put myself first and also the importance of priority. Also letting go whatever doesn’t deserve my time and energy.”

“To live today to the fullest because I do not know what tomorrow will bring.”

We are called Sickle Cell Warriors for a reason. We are tough, resilient fighters.

September is National Sickle Cell Awareness Month designated to help focus attention on the need for research and treatment of Sickle Cell disease.

In 1975, the National Association for Sickle Cell disease, Inc. created a series of awareness campaigns. In 1976, they launched a national awareness month in September amid growing concerns about the amount of misinformation available about Sickle Cell. The foundation aimed to dispel myths and provide educations about Sickle Cell. Along with its co-founder, Dr. Charles F. Whitten, who pioneered advancements in understanding the condition and improving treatment, the organization made great strides in bringing information to the general public. Together, they also increased opportunities for research and more accessible screening methods.

Since then, the organization continues to pursue research, treatments, and a cure under the name Sickle Cell Disease Association of America.

The SCDAA tradition of selecting a national poster child (now called the Child Ambassador Contest) from local candidates began in 1976 with President Gerald Ford greeting the first winner (8-year-old Bridgete Earby of Oakland, California) at the White House. Presidents Carter, Reagan, Bush, Clinton and Obama have also greeted the national poster child.

Then in 1983, the Congressional Black Caucus of the House of Representatives passed a resolution. It asked President Ronald Reagan to proclaim September National Sickle Cell Anemia Awareness Month. The President signed Proclamation 5102 in September of 1983.

The annual observance provides an opportunity to increase public knowledge and an understanding of sickle cell disease and traits. Also, the month addresses the challenges experienced by patients, their families, and caregivers. The World Health Organization (WHO) estimates sickle-cell disease affects nearly 100 million people worldwide, and over 300 000 children are born every year with the condition.

10 Things You Should Know About Sickle Cell

• Sickle Cell disease is the most common genetic blood disorder in the country.
• It affects almost all races, not just African Americans.
• You can’t “catch it”- you’re born with it.
• Sickle Cell Anemia is the most common form of sickle cell disease.
• Blood tests called “Hemoglobin Electrophoresis and CBC” are needed to determine whether you have Sickle Cell trait or a related red blood cell disorder.
• Sickle Cell disease causes red blood cells to become hard and “sickle” or banana shaped which makes them block the flow of blood.
• Blocked blood vessels cause pain, damage to organs and tissue, or even a stroke.
• Some people with Sickle Cell Anemia need frequent blood transfusions.
• You can help by becoming a blood or bone marrow donor.
• The only cure for Sickle Cell disease is a bone marrow transplant.

HOW TO OBSERVE #SickleCellAwarenessMonth

Be a Sickle Cell fighter by making an appointment to give blood. Donating blood can save a life, somebody like me with Sickle Cell Anemia. I would encourage anybody healthy to donate blood. You never know who you’re saving.

Spread the Word on Social Media. Please tag SCDAA in your social media posts so we can share your events and happenings during the month of September!

#SickleCellMatters
#SickleCellAwarenessMonth
#SCDAA2020AwarenessMonth
#SCDSCTMatters

I wish there was more information about the benefits of supplements out there especially in relation to Sickle Cell warriors. Dietary supplements are products designed to augment your daily intake of nutrients, including vitamins and minerals. Many are safe and offer significant health benefits, but there are some that pose health risks, especially if overused. Dietary supplements include amino acids, fatty acids, enzymes, probiotics, herbals, botanicals, and animal extracts.

Normally, you should be able to get all the nutrients you need from a balanced diet. However, supplements can provide you with extra nutrients when your diet is lacking or certain health conditions trigger a deficiency.

While most dietary supplements are safe as long as you follow the product instructions, large doses of certain nutrients can have adverse effects. You can even overdose on certain supplements, risking serious harm and death.7

Among some the harmful interactions or dosing concerns:

• Vitamin K can reduce the effectiveness of blood thinners like Coumadin (warfarin).8
• Vitamin E can increase the action of blood thinners, leading to easy bruising and nosebleeds.
• St. John’s wort can accelerate the breakdown of many drugs, including antidepressants and birth control pills, thereby reducing their effectiveness.
• Antioxidants like vitamins C and vitamin E can reduce the effectiveness of some types of cancer chemotherapy.8
• High-dose vitamin B6 (pyridoxine), when used for a year or more, can cause severe nerve damage. Vitamin B6 can also reduce the effectiveness of the anti-seizure drug Dilantin (phenytoin) and levodopa (used to treat Parkinson’s disease).
• Vitamin A used with retinoid acne medications such as Accutane (isotretinoin) and Soriatane (acitretin) can causes vitamin A toxicity.8
• Vitamin B3 (niacin), when used with statins, may increase the risk of rhabdomyolysis (the abnormal breakdown of muscle tissue).
• Iron and calcium supplements can reduce the effectiveness of antibiotics, namely tetracyclines and fluoroquinolones, by as much as 40 percent.
• Vitamin C, when taken in doses exceeding 2,000 milligrams, can cause nausea and severe diarrhea.

Advise your healthcare provider about any supplements you plan to take as well as any medications you are currently taking, whether they be pharmaceutical, over-the-counter, herbal, traditional, or homeopathic.

Now for supplements that are really benefit Sicklers.

Arginine 100 mg per 2.2 lbs (1 kg) of body weight, three times per day.

Something I’ve personally had a scare from. People with pulmonary hypertension (a life-threatening complication of Sickle Cell) who received L-arginine had significant improvement in one study. In a preliminary study, individuals with pulmonary hypertension received L-arginine in the amount of 100 mg per 2.2 pounds of body weight, three times per day for five days. L-arginine treatment resulted in a significant improvement in pulmonary hypertension, as determined by a 15% decline in the pulmonary artery systolic pressure. Longer-term studies are needed to confirm these preliminary results.

Fish Oil 250 mg of oil per 2.2 lbs (1 kg) of body weight daily.

Supplementing with fish oil appears to reduce the frequency of severe pain episodes. Fish oil has been studied. In a double-blind trial, supplementation with menhaden oil, in the amount of 250 mg per 2.2 pounds of body weight per day for one year, reduced the frequency of severe pain episodes by approximately 45%, compared with placebo. This treatment may work by correcting an imbalance between omega-3 and omega-6 fatty acids that occurs in people with Sickle Cell. The beneficial effect of the omega-3 fatty acids present in fish oil was confirmed in another double-blind study.

Folic Acid.

In one trial, patients with Sickle Cell who were given folic acid plus aged garlic extract, vitamin C, and vitamin E saw significant improvement and less painful crises.

In a preliminary trial, 20 patients with Sickle Cell were given either 1 mg of folic acid per day or folic acid plus 6 grams of aged garlic extract, 6 grams of vitamin C, and 1,200 mg of vitamin E per day for six months. Patients taking the combination had a significant improvement in their hematocrit (an index of anemia) and less painful crises than those taking just folic acid.

Preliminary research has found that patients with Sickle Cell are more likely to have elevated blood levels of homocysteine compared to healthy people. Elevated homocysteine is recognized as a risk factor for cardiovascular disease. In particular, high levels of homocysteine in Sickle Cell patients have been associated with a higher incidence of stroke. Deficiencies of vitamin B6, vitamin B12, and folic acid occur more frequently in people with Sickle Cell than in others and are a cause of high homocysteine levels. A controlled trial found homocysteine levels were reduced 53% in children with Sickle Cell receiving a 2–4 mg supplement of folic acid per day, depending on age, but vitamin B6 or B12 had no effect on homocysteine levels. A double-blind trial of children with Sickle Cell found that children given 5 mg of folic acid per day had less painful swelling of the hands and feet compared with those receiving placebo, but blood abnormalities and impaired growth rate associated with Sickle Cell were not improved. In the treatment of Sickle Cell, folic acid is typically supplemented in amounts of 1,000 mcg daily. Anyone taking this amount of folic acid should have vitamin B12 status assessed by a healthcare professional.

L-Carnitine 50 mg per 2.2 lbs (1 kg) of body weight daily

In a study of children with Sickle Cell, supplementing with L-carnitine reduced the number of painful crises and improved abnormal heart function and pulmonary hypertension. In a preliminary study of children with Sickle Cell, supplementing with L-carnitine (50 mg per 2.2 pounds of body weight per day for six months) significantly reduced the number of painful crises and significantly improved the abnormal heart function (diastolic dysfunction) that was present in most of the children. L-carnitine supplementation also significantly improved pulmonary hypertension in the children who had this abnormality prior to treatment.

Zinc. Take under medical supervision: 100 mg of zinc (plus 2 mg of copper daily to protect against depletion)

Supplementing with zinc appears to help prevent cell damage and speed healing of leg ulcers associated with Sickle Cell.

Antioxidant nutrients protect the body’s cells from oxygen-related damage. Many studies show that Sickle Cell patients tend to have low blood levels of antioxidants, including carotenoids, vitamin A, vitamin E, and vitamin C, despite adequate intake. Low blood levels of vitamin E in particular have been associated with higher numbers of diseased cells in children and with greater frequency of symptoms in adults. A small, preliminary trial reported a 44% decrease in the average number of diseased cells in six Sickle Cell patients given 450 IU vitamin E per day for up to 35 weeks. This effect was maintained as long as supplementation continued.

The zinc deficiency associated with Sickle Cell appears to play a role in various aspects of the illness. For example, preliminary research has correlated low zinc levels with poor growth in children with Sickle Cell. In a preliminary trial, 12 people with Sickle Cell received 25 mg of zinc every four hours for 3 to 18 months. The number of damaged red blood cells fell from 28% to 18.6%. Addition of 2 mg of copper per day did not inhibit the effect of zinc. (Zinc supplementation in the absence of copper supplementation induces a copper deficiency.) Patients with the highest number of damaged red blood cells had a marked response to zinc, but those with lower levels of damaged cells (less than 20% irreversibly sickled cells) had little or no response.

Chronic leg ulcers occur in about 75% of adults with Sickle Cell. In a controlled trial, Sickle Cell patients with low blood levels of zinc received 88 mg of zinc three times per day for 12 weeks. Ulcer healing rate was more than three times faster in the zinc group than in the placebo group. Zinc supplementation (25 mg 3 times per day for 3 months) also decreased the number of infections in adults with Sickle Cell.

L-glutamine (Endari). Refer to label instructions

Endari is an oral L-glutamine therapy for Sickle Cell Anemia and Sickle Cell Thalassemia developed by Emmaus Life Sciences.

It was approved for use by patients age 5 and older by the U.S. Food and Drug Administration (FDA) on July 7, 2017, making Endari the first approved pediatric treatment for Sickle Cell, and the first new treatment for adults in almost 20 years.

Glutamine, an amino acid, plays various and fundamental roles in cell metabolism, including protein synthesis, chemical energy production, and anti-oxidant formation. The most abundant amino acid in the body, glutamine is naturally produced (largely in the lungs), and usually in sufficient amounts for our needs. But is often used as a supplement to counter infection, injury, stress, and to treat certain diseases.

Patients with Sickle Cell have an altered form of the hemoglobin protein (which carries oxygen, via the blood, to tissues) that renders red blood cells susceptible to oxidation damage.

High oxidative stress has been associated with an increased stiffness in these red blood cells, leading to their sickle-like shape. Less flexible than healthy (and round) red blood cells, these abnormal cells often stick to vessel walls, blocking or slowing blood flow and oxygen transport.

Endari increases the amount of free glutamine circulating in the blood, which is taken up by the sickle cells and used to generate anti-oxidant molecules as a product of glutamine degradation.

These new antioxidants help neutralize the oxidative stress in sickle red blood cells, allowing them to regain the flexibility needed to travel through blood vessels and capillaries, carrying oxygen to tissues throughout the body.

Note: This is a personal blog and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.

As far back as I can remember I have always been a huge video game nerd, capable of playing games from sunrise to sunset or sunset to sunrise. I remember my very first console was the Sega Mega Drive and this was the first game I was addicted to. This began my obsession.

I wish I could add up the time I’ve spent lost in many worlds behind my tv or computer screen. It would be months at this point, close to a whole year I’m sure. And I have no regrets about it.

Up until recently I did not even question why I was so into gaming. So I sat down and thought about it for a couple of hours yesterday as I took a break from the 8 hours I had just put into Star Wars Jedi: Fallen Order. I came to the conclusion that it is and has been a coping mechanism for me, it has been my therapy and my distraction from pain. Curious to know what other SC warriors thought I asked about a dozen or so about how they feel about gaming, and they all had the same answers. “I’ve been gaming since I was very young too.”, “It helps distract from the pain.”

You can’t focus on pain if you are focused on killing a boss in some RPG for 4 hours straight. Anyway I began looking into how games can be a form of pain management and to my surprise there have been dozens of studies on this already, even specifically regarding Sickle Cell patients. In fact, over the past decade or so, video games have also been shown to help reduce pain in children with chronic diseases, such as SCD.

I found a study by done in 2014 by Talal Ali, a Ph.D student of nursing at Wayne State University in Detroit, Michigan. She wrote a dissertation called, Using Video Games For Decreasing Pain Caused by Acute Painful Crisis In Adolescents With Sickle Cell Pain. To collect results for this research study, Talal enrolled 30 participants, ranging in age from 12 to 21, to play video games during painful crises and then complete a self assessment of the pain before the games and the pain after the games. The findings? “The use of video games as a distraction modality has proven to show positive and significant results in the treatment of acute pain.”

In an interview with Everyday Health, Dr. Sarah Rebstock, a pediatric anesthesiologist at Children’s National Medical Center in Washington, D.C., she said, “It’s all due to the concept of neuroplasticity … the notion that experience causes the brain to wire itself in certain ways and that rehabilitative therapy can essentially rewire the brain … You’re increasing range of motion, increasing blood flow, and retraining your body to decrease the pain so it’s not as intense.”

After talking to other Sicklers about it I have realized that what games work for me might not work for them. A lot of people are really into Animal Crossing right now, which I have not had the opportunity to play yet, but that’s their go to drug for a few hours of pain relief. Personally online shooters, for example, are a no go when I’m in pain. It doesn’t matter how much I love playing games like Call of Duty: Warzone, the fast-paced nature and inability to pause tends to be too much. I find RPG’s, like the Star Wars series of games, to be helpful but only for lower levels of pain. These tend to be less reflex based, and I can pause when I need to. If I’m having a particularly bad flare up, sometimes I’m still not up for this level of activity. The game that may work for you can depend largely on the level of pain you’re in.

I was glad to find out VR is already used for acute pain management, cancer treatments, and routine medical procedures.

The Children’s Hospital Los Angeles published details about how patients can generally tolerate more pain, and also be more willing to participate in procedures using VR. This also led to less stressful procedures, especially with children. When they were using VR they were more distracted, and also more compliant. Compliance means calmer, stiller children, which means better procedures like inserting a needle once instead of multiple attempts on a wriggling kid.

But is virtual reality a legitimate form of pain relief? Based on its ability to keep patients mentally and emotionally happy, as well as distracted, researchers believe that might just be the case. And I am onboard, I can’t wait for VR to become more mainstream and affordable for my fellow warriors and I. With more research underway every day, maybe we’ll see a day where doctors prescribe gaming sessions to ease pain, alongside medication.

The other day a friend of mine from Washington called me while he was completely wasted and it was quite hilarious and weirdly touching. My friend, called me to express how worried he was about me and how seriously I should take my health during this pandemic (only one other friend has expressed concern like this, my other friends attempted to get me to go to the beach).

So yeah I was genuinely surprised that someone was checking up on me. And that got me thinking about how rare a connection like that is and how much I despise how rare it is.

Since the start of this pandemic I have had time to kill, I have spent my time trying to connect with more people with SC that I can relate to. After hours of searching I found what I was looking for on Reddit; an invite only Discord group specifically for people with Sickle Cell 18+. This is very new to me, never have I ever been part of a group (besides my sibling group), with anyone else who has the same condition I do, never have I been part of a group chat where advice and answers to my specific questions are given. I’ve never been part of a group where people wake up in the morning and ask how I slept and it’s not a general question but has a more specific underlying question; they mean ‘how long did you sleep tonight with those aches and pains’, ‘how are your oxygen levels this morning’ ‘any joints feel like they’re on fire?’. A question answerable by all in this group. Questions I’m so used to presenting in regular group chats that get completely ignored or glanced over because no one can relate.

With this new group of like-minded Sickle Cell Warriors I can finally talk about how my sleep cycle is greatly affected by my hip pain and someone else who is going through the same thing offers advice that works. I can moan and complain and get replies not silence… never before have I felt okay moaning and complaining about this drug and that drug, this pain and that pain, this hospitalization and that hospitalization and having someone honestly reply “saaame” and totally mean it.

These individuals I’ve met are like me even though we have very different lives and past experiences, from the 18 years old to the 40 year olds, growing up in dozens of different countries. A complicated group of anime, art, design, photography loving, gaming nerds, medical professionals and so much more, people who deal with the invisible problems I struggle with daily. I am not normal or average and it feels amazing having a group of people where I don’t have to pretend to be normal to feel normal.

From day one it was like society was this violent, complicated dance and everybody had taken lessons but me. Knocked to the floor again, climbing to my feet each time, painfully humiliated. Always met with disapproving faces, waiting for me to leave so I’d stop fucking up the party. I always felt like I was on the outside, where the freaks huddle in the cold. Out there with the misfits, the broken, the glazed-eye types who can only watch as the normals enjoy their shiny new cars and careers and marriages and vacations with the kids. The freaks spend their lives shambling around, wondering why and how they got left out. Their encounters with the world are marked by awkward conversations and stifled laughter, hidden smirks and rolled eyes. And worst of all, pity.

You can be a drunk. You can be a survivor of abuse. You can be an ex-con. You can be a homeless person. You can lose all your money or your job or a husband or a wife, or the worst thing imaginable, a child. You can lose your marbles. You can be standing inside your own failure, a small sad stone in your throat, and still you are beautiful, your story is worth hearing, because you–you rare and phenomenal misfit–are the only one in the world who can tell the story the way that only you can.

I feel like I’ve always known this but I’ve never realized it. I’m not sad it took me 27/28 years to realize this, I’m happy because it took me only 28 years to realize this and not a lifetime. Because I have a long way to go and lot of lives to help and connect with, a community to start, a community I was unable to find until I was an adult.

Sickle Cell in Uganda is a major public health problem with poor visibility and low, awareness, a situation that will persist if patients continue to be hidden away in families.

Uganda was among the first countries in Africa with a documented large burden of Sickle Cell Anemia. In 1949, substantial differences in the prevalence of Sickle Cell trait were reported between different tribes, ranging from less than 5% for Hamites in the southwest to more than 20% for the northern Nilotices (Lango and Acholi). Some Bantu tribes had even higher rates, including 45% among Bamba living in the western region.

Yet we have no high functioning nonprofit organizations that can raise awareness and spread the message and draw attention to the extent of homozygous Sickle Cell (SS) disease as a public health problem in Uganda. Data showed an enormous burden, with sickle cell trait being present in all 112 districts assessed. Prevalence was highest in the Mid Northern and East Central regions. Overall, the prevalence of sickle cell trait was 13·3%, but it was more than 20% in eight districts. Among babies aged 6 months or younger, the overall prevalence of sickle cell trait was 13·2% and of disease was 0·8%, which suggests that at least 15 000 babies per year are born with Sickle Cell in Uganda. 

Worldwide, sickle haemoglobinopathies lead to a substantial burden of disease that is not adequately addressed. Accurate data is lacking, but the worldwide estimate for neonates born with Sickle Cell each year is 400 000, including 300 000 with Sickle Cell anaemia. The greatest burden is seen in sub-Saharan Africa, where more than 75% of all sickle cell disease occurs, with this proportion projected to increase by 2050. In Africa, sickle cell disease contributes substantially to mortality in children younger than 5 years.

A later study, however, has suggested lower values. Of note, though, both studies were based on small samples and were not representative of the whole country

So, hopefully within the next year I plan on starting a foundation in my late brothers name specifically for this cause that afflicted him, my sister, me and thousands/millions in Uganda and globally.

You’re awesome they say.

So strong they say.

It’s laughable how people can say this straight faced.

You don’t know how I fear the hours coming ‘til I awake.

You don’t know what a struggle it is for me to make it through the day.

Such a fighter they say.

Tap my gloves at dawn with the sunlight.

First round begins before the bright rays pierce my shades to touch my eyes.

Well, what if I don’t want to fight?

Why should it be a constant battle to get through life?

Two left jabs and a right hook just to try to stay alive.

Maybe I can’t live up to Darwin’s theory, I’m just not fit to survive.

They say keep stirring up the gift.

You’re destined to uplift.

Do you even lift?

Strong because my soul has bench pressed my body weight in pain.

Cried so many tears, sadness drenched my life like a torrential rain.

Agony scars my very being like a bloodstain.

Riding out my dreams on the winds of change.

Work never ceases, trying to locate the keys to this life steady racking my brain.

Clinging to a thread of hope just trying to maintain.

Doing my best to try to remain sane.

Constantly praying.

Still I spiral, my aspirations fading, disappearing down a drain.

Always wondering if all my efforts are in vain.

– March 3, 2012

Excerpt from: “My Book of Tears” by Candis St. John.

I got lucky today stumbling across this amazing poet who is also a Sickler like me. Her book can be found on Amazon and Scribd as far as I’m aware.

Sickle Cell was the first disease understood at the molecular level, but we’ll get to that awesome story later. The story of Sickle Cell is a study in how a good thing can come with bad consequences.

Once upon a time in the Green Sahara (this part of Africa was wet and rainy and covered with forest 7,300 years ago) a child was born with heightened immunity to malaria.

It was a great habitat for mosquitoes, which carry malaria, a disease that these days kills one child every two minutes.

This child would have been born with a genetic mutation in one chromosome, affecting the shape of the blood’s hemoglobin. The child wouldn’t have suffered because the other gene would have been perfectly normal.

With a better chance against an illness that was a major killer, then as now, this child with the genetic mutation lived and had children, and those children spread out. The mutation would have endured, passing on for hundreds of years before one unfortunate child would have been born with two copies of the mutation and would have developed Sickle Cell Anemia.

The first recorded cases in history occurred in Egypt during the predynastic period (3200 BCE)

During the Bantu migrations and, later, the slave trade, Sickle Cell would have travelled to other parts of Africa and on to other parts of the world, including the Near East, India, and Southern Europe, developing into different sub-types along the way.

The first modern report of sickle cell disease may have been in 1846 where the autopsy of an executed runaway slave was discussed; the key finding was the absence of the spleen. Reportedly, African slaves in the United States exhibited resistance to malaria, but were prone to leg ulcers.

The abnormal characteristics of the red blood cells, which later lent their name to the condition, were first described by Ernest E. Irons who was an intern to Chicago cardiologist and professor of medicine James B. Herrick in 1910. Irons saw “peculiar elongated and sickle-shaped” cells in the blood of a man named Walter Clement Noel, a 20-year-old first-year dental student from Grenada. Noel had been admitted to the Chicago Presbyterian Hospital in December 1904 suffering from anaemia.

Shortly after the report by Herrick, another case appeared in the Virginia Medical Semi-Monthly with the same title, “Peculiar Elongated and Sickle-Shaped Red Blood Corpuscles in a Case of Severe Anemia.” This article is based on a patient admitted to the University of Virginia Hospital on November 15, 1910.

In 1922, the name “Sickle Cell Anemia” is first used in a description by Verne Mason.

In 1927, scientists Hahn and Gillespie showed that sickling of the red cells was related to low oxygen.

In 1940, Sherman (a student at Johns Hopkins Medical School) noticed a difference between the way light passed through sickled blood cells compared to normal cells.

In 1948 Janet Watson, a pediatric hematologist in New York, suggested that the scarcity of sickle cells in the peripheral blood of newborns was due to the presence of fetal hemoglobin in the red cells, which consequently did not have the abnormal sickle hemoglobin seen in adults.

In 1949, chemist Linus Pauling, who would go on to win two Nobel Prizes, co-authored a paper in Science offering the first-ever proof that a human disease was caused by an abnormal protein. A genetic mutation inherited from both parents caused defects in the hemoglobin protein found in red blood cells. Using the new technique of protein electrophoresis, Linus Pauling and Harvey Itano showed that the hemoglobin from patients with sickle cell disease is different than that of regular people. This made Sickle Cell the first disorder in which an abnormality in a protein was known to be at fault.

In 1956 Vernon Ingram and J.A. Hunt sequenced sickle hemoglobin and showed that a glutamic acid at position 6 was replaced by a valine in sickle cell disease. Using the known information about amino acids and the codons that coded for them, he was able to predict the mutation in sickle cell disease. This made Sickle Cell the first genetic disorder whose molecular basis was known.

In 1977 the development of DNA sequencing by Walter Gilbert and Frederick Sanger allowed the mapping of the Sickle Cell gene.

In 1984 a bone marrow transplantion in a child with Sickle Cell produced the first reported cure of the disease. The transplantation was done to treat acute leukemia. The child’s Sickle Cell was cured as a side-event. The procedure nonetheless set the precedence for later transplanting efforts directed specifically at Sickle Cell.

In 1995 Hydroxyurea became the first drug proven to help prevent complications of Sickle Cell.

In July 2019, a team edited the DNA of an American woman with Sickle Cell. Doctors first removed stem cells from the woman’s bone marrow. Then, they genetically modified them using CRISPR to make them produce a protein that makes healthy red blood cells but whose production is typically shut down shortly after birth. The procedure was successful, but researchers need time—at least two years—to know how it will hold up in the long run.

In November 2019 the U.S. Food and Drug Administration approved Adakveo, a treatment to reduce the frequency of vaso-occlusive crisis for patients age 16 years and older.

This month a trial investigating the safety, feasibility and efficacy of a form of gene therapy (ARU-1801) for children and adults with Sickle Cell is underway in the U.S. and Jamaica.