Letter to my nephew – managing that sickle cell
Visiting you in the hospital brought back memories of my being struck down with a painful sickle cell crisis during a short training course in Tehran in the mid-1970s. Tehran is supposed to be the fount of modern medicine, but because the doctor spoke no English and I did not speak Farsi, you can imagine the difficulty I had in communicating through an interpreter who did not know sickle cell.
In your case, you have been rather more fortunate. You were admitted to a hospital where all the nurses and doctors spoke English and where there was an established protocol for treating sickle cell patients, thanks to the campaigns by the Sickle Cell Society, sickle cell support groups, many forums and individuals’ the across the UK.
My view when I visited was that the underlying problem of your crisis was excitement and exhaustion. I also felt that you needed more rest after you were discharged from the hospital. I knew you had several things to do but walking in the inclement autumn weather was bound to bring on the relapse that occurred shortly before you went back home. Now that you are recovered and back in Ghana enjoying warmer weather, I thought that the advice you asked for should be better expressed in this letter.
But what advice could I possibly give to a young ambitious and talented young man in his prime who is eager to work hard to make a name for himself in his chosen profession?
In this piece, I will attempt to describe my personal experience of the life-threatening condition of the sickle cell disease to assure you that though you cannot prevent the incidence of a crisis, the key to effectively managing the condition is through understanding a bit of what is happening to you.
The genes – it is in the blood
Science was not my best subject so I will provide my layman’s understanding here and it is important to be patient with my ‘non-carbon’ or non-scientific explanation here. My understanding is that Sickle Cell Disease is a group of blood disorders caused by genes inherited from parents. You inherit one gene from each parent. The normal genes are Haemoglobin AA but I am told that the Haemoglobin S and Haemoglobin C genes were mutations that were supposed to provide protection against malaria; these abnormal genes reduced the content of oxygen in the blood to starve the malaria parasite as it flows through the bloodstream.
- When you inherit a Haemoglobin A gene from each parent, you are Haemoglobin AA and are prone to malaria.
- When you inherit a Haemoglobin A gene from one parent and a Haemoglobin C from another parent, you are designated as Haemoglobin AC you may have that protection against malaria, but you are essentially a Haemoglobin C carrier or have the Haemoglobin C trait.
- When you inherit Haemoglobin C from both parents. you have Haemoglobin CC disorder, this, however, is not the sickle cell disease.
- When you inherit a Haemoglobin A from one parent and a Haemoglobin S from the other parent, you are designated as are Haemoglobin AS, you are a carrier of Sickle Cell and have the Sickle Cell Trait but do not have the disease.
But when you inherit an abnormal gene from each parent, then you have the sickle cell disease, designated as Haemoglobin SC or Haemoglobin SS, a double dose that provides you with no protection against malaria but a life sentence.
I have a sister who inherited the normal genes AA and a brother who has the trait AS but do not know which one of my parents transferred the C or the S gene. Though to his dying day my father refused to be tested claiming that this condition must have come from my mother’s side of the family, I am however certain that they were both carriers because, not only do I have cousins on both sides of my family with the sickle cell disease, but my late sister also had the SC condition.
My illness is not due to some witch sucking my blood because it looks juicy, or some demons who have attacked me, or because I have poked my poisonous finger in someone’s delicious pie, or stolen someone’s sweet water to drink, or eaten someone’s sumptuous food in secret or chased after someone’s wife or girlfriend.
Sickle cell is not something that I have done, it is because I inherited my precious genes from my parents who loved and cherished me.
Crisis of pain
The effect of the sickle cell disease is that under certain conditions when your blood is starved of the oxygen, you go into crisis, the doughnut-shaped red blood cells turn into a sickle shape and stick together around the small arteries and joints restricting the blood flow which creates the excruciating pain.
Sickle cell crisis affects different patients differently. The crisis can be set off by a variety of things, temperature changes, changes in the weather, exhaustion or dehydration, and because the immune system in our bodies is seriously affected making us more susceptible to infection and most importantly the rapid spread of
infection. It is unexpected, comes on at the most inconvenient of times causing pain to different parts of the body indiscriminately.
There is pain and then there is excruciating pain which starts in one part of the body and then spreads till it becomes unbearable. It is unending and sometimes so intense that you end up crying and wailing but it does not cease. You just want to fall asleep, but the sleep does not come, you pray for relief, but it does not come till it takes its course. A sickle cell crisis can last for a week with no let-up.
Describing the pain is not easy. As Bob Marley sang – ‘He who feels it knows it more’! Whatever the cause of these episodic bouts of pain experienced by the patient the condition is best described by the Ga onomatopoeia – twetweetwe.
Genetic counselling may not help in the treatment of your pain but it is important that when choosing a lifelong partner who will have your children, you just do not ask about her sexual history which is quite normal these days, but you also ask about the history of her genes and whether she has the sickle cell trait. If she does and you still love her to the extent that she is the only one you want, then at least you will know that you may have children who may have the sickle cell disease.
There was no genetic testing during the days when my parents got married; if there had been it would perhaps have prevented the anguish, of nursing my sister and me through the frequent bouts of crisis that we endured during our infant and childhood days and the considerable amount of money they spent consulting all manner of traditional healers to diagnose and provide relief for us.
Diagnosis and school life
Though I lost a lot of school days to the illness, it never really dulled my thirst for knowledge and my capacity for learning. During my primary school days, there were times when my teachers were worried about the extent of care my parents were providing me with. I spent my 10th birthday in secondary school but that first year was a disaster. I struggled academically, my teachers were not sure whether I was too young and homesick, was malingering or was just plagued by a mysterious illness. Even my class and housemates did not understand why I would be happily enjoying myself in the evening and be in unbearable pain the next morning.
Fortunately, Dr Kuta-Dankwa and the nurse, Miss Teye at Achimota Hospital, agreed that Mr Peter Renner should take me to Korlebu Hospital in Accra. It was there that I met Dr Portuphy-Lamptey and Dr Konotey-Ahulu who would later become my doctor through my adult years. He was the person who made the diagnosis of sickle cell disease and talked me through issues relating to the illness including genetic counselling! I was too young to be admitted to the Medical Ward and ended up at the Children’s Ward under the care of an Indian lady doctor, Dr Kuma who was kind enough to set me some English essays and Maths textbook so that I could still study – my mother supplied the Bible which was a sort of comfort.
I was too ill for my exams at the end of the first year, so with my poor academic record, a decision had to be made as to whether I would continue in the school. My older sister who had suffered pretty much the same problems at Aburi Secondary School was asked to be withdrawn from the school and ended up in a school at Accra. My father, however, resisted all the pressure and insisted that I stay at Achimota School. I repeated the first year. My illnesses and frequent absences continued during the rest of my stay in Achimota to the extent that during the month of my ‘O’ levels, I sat some of the exams even though I was in crisis.
Sixth Form at Ghana Secondary School was great, but it rained every day in Koforidua, the dew-laden atmosphere in the morning was not the best for me, cold in the evening was also a problem. So, the illnesses continued complicated I think by new habits of drinking and smoking that I picked up to make me look cool and hip as a senior.
In university, my long-suffering roommate Ekow Sam had to put up with my crises as I added on some partying to the list of things that my doctor felt triggered a crisis.
Types of illnesses
What are these illnesses that I have been talking about? I will limit the litany of diseases that I have been afflicted with since most of these are prevalent in Ghana, the problem was that I was more susceptible to these illnesses because my sickle cell anaemia reduces my resistance.
Malaria is an ever-present danger though I always slept in a mosquito net even when our windows had mosquito netting. Everyone goes out to have a meal, I have a mouthful and must rush to the loo; diarrhoea hit because some little bacteria found its way into my stomach. I go to Scout Camp and by the time I finished climbing to the top of one of the Shai Hills, I am down with a painful crisis and must be carried down. Camp at Prampram with the Cadets brings a disastrous result. We all swim in the pond and I must be treated with bilharzia. I go drink odoka, a gin-based drink and I find myself in hospital with typhoid fever, I eat chichinga – kebabs or domedo, roasted pork, and next day it is salmonella poisoning, I eat shito loo, – fried fish or Circle Rice with ‘ruffians’ and get struck down with jaundice and end up in isolation ward.
The retinopathy that produces floaters in my eyes, the avascular necrosis of my hip bone that laid me out for over 6 months, gout that is hampering me now, the peripheral neuropathy that has started affecting my gait, are all results of my sickle cell disease.
When you have a rampant, raging erection that will not go down, what is happening is priapism. It will not be cured by intercourse, just rush to the nearest hospital and seek immediate medical attention.
I suppose that by the time that I finished university, I should have gotten to know the signs of when I crisis was coming on: – the weather, the way the wind blows, the way that I am sweating, when I have not slept for a long period, when I was under stress, when I was under pressure, when it was raining, when it got damp, if I knelt for long periods.
Disruption to life
I started work before I turned 21 years and would say that the worst periods of crisis were in my 20s when I was working; it was kind of difficult to explain to an employer that I could not turn up for work because I had been taken to hospital and the more frequently this happened the more frustrating it got. With hindsight, I would say that the jobs probably contributed to my illnesses. Working as a Systems Engineer meant that I spent a lot of time with mainframe computers that had to be in a very cold airconditioned room. Constant entry and exit from that room into the hot sun wearing a suit did not help my condition. As a travel agent, I had to engage in a lot of air travel and there was always this worry about whether I will survive if the plane was not fully pressurised.
As much as I tried to not let the condition disrupt my life, it did! Sometimes at crucial times, like when I was billed to have an interview for a scholarship, and I did not show up or having to do an exam in pain or being laid up with a crisis just a day before I was supposed to travel to do some work. It did disrupt both my working and planned leisure activity like not showing up for a date or some sports event or some party.
But when you are young, it can be quite difficult to accept that you should let the sickle cell disease control your life, I was in my element or so I thought. I intended to prove to everyone and myself that despite the illness, I could live my life to the fullest, doing things that all young people would like to do without a care in the world. I am sure that I would have saved myself a lot of pain if I had listened to the free advice given by my doctors and parents.
I have come to reflect that a lot of the late nights I spent though pleasurable provided no benefits to my health and that I should have sought a more balanced regime of life then, than the free abandon that characterised the period of my youth.
Management of the condition
I left Ghana for the UK just before I turned 30 years, first to cold and wet Manchester to study for my master’s degree. I stayed for two and a half years and then to London where I have lived and worked for the past 35 odd years; I have been fortunate not to have been hospitalised because of a sickle cell crisis. The work I do as a management consultant is high pressured and my social life has been rather full, so I can only conclude that the environment of the UK has been kinder to me. I, however, think that I may have adopted a more matured and measured way to live. The precautions I take may have helped me to reduce the number of crisis and kept me away from the hospital.
There is not much one can do to reduce stress in this rat race with all its pressures, I now know that the stress of studying for exams which coincided with the rainy season must have been a source of worry for me. I can deal with exhaustion – I just rest and rest and rest when I feel I am getting tired. I make sure that I do not get dehydrated, so I drink a lot of fluids, I mean a lot and I love fizzy water. I do not do saunas and places where the oxygen intake may be low, and I dress for the weather, I always have a hat on. I just make sure I do not get in the rain and always have an overcoat or sweater and scarf in the boot of my car as well as walking stick.
I stopped drinking beer in 1976, around the same time as I gave up cigarettes. Alcoholic spirits I could not stand anyway since any gin-based drink just triggers a crisis and I finally gave up even social wine when my bouts of gout started about five years ago.
Medication and painkillers
I do not play with my daily medication, it is like a pharmacy at home – Folic Acid and Penicillin V as a preventative antibiotic, though these days my doctors say that there is a change in protocol and I no longer need them. But I also take other medications which are supposed to be preventative – one for keeping the balance of the protein that could damage my kidneys, a known problem for sickle cell patients, compression socks or ‘tights’ to prevent the leg ulcers and blisters that could develop during the hot summer months, and now my doctor and I are trying to keep gout at bay. We discovered that the dose of the medication I have been taking was low and could have is triggering gout, so I have an increased dosage for the preventative medication and taking the one to cure it as well. I do not ask about side effects when I take medication! One fortunate thing is that I can have long discussions with my doctors which makes me feel like an expert patient of sorts and because I have lived with this condition for so long I have them listening sometimes.
My problem, however, is that I hate painkillers! and try not to take them though I have a stock of dihydrocodeine. I take this to assure my wife that I do not need to be taken to hospital where I may be given opioid-based painkillers that could create more problems for me.
When I visited you in hospital and morphine had been administered as the protocol, my anxiety was because you were complaining of chest pain which I am quite scared off. This was why I spoke to the doctors and asked them to reconsider their protocol because you were a visitor to this country and your medical history was very important in administering the painkillers.
Thankfully they started hydrating you provided you with oxygen which I believe set you on the road to recovery. I am sure that a manual blood exchange would have been useful if those procedures had not worked.
My attitude to painkillers is that I grew up in an environment when most of my crises were caused by infections and exhaustion and I was more interested in the source of the crisis being resolved rather than just the pain. I also remember that at age 28 when I suffered from avascular necrosis of the hip, it took a long time for me to wean myself off the medication. It gave me a high and felt that I was getting addicted to it, I could not drive and had to get back to working instead of lazing at home feeling like someone who was on pot all day.
Cure for sickle cell
But of course, we all look forward to a cure for sickle cell disease. There is progress but I hope that there will be more rigorous testing of ‘incidental’ drugs discovered when finding cures for other ailments before they are used on young patients. I also hope that the assessment of the side effects will be thoroughly investigated.
The reports I read about bone marrow transplant also seem promising, but here again, the stories do not fully describe the haemoglobin type of the donor or recipient. I am still sceptical about gene therapy because I always wonder whether the genes given me by my parents, who I so cherish because of the way they nurtured me, will be replaced by something that I do not recognise and turn me into something else
I can assure you that I am happy with the medical profession and I can live with their explanations and I am happy about the advances that they have made in this very politico-medical sphere of the sickle cell disease. There are still a lot of battles to be fought around the non-medical issues and effects of the condition and the recognition of the inequalities of health since this illness mainly affects people from areas where malaria is prevalent like places of our of African heritage.
Well, this is a long story of pain and misery and the death sentence dealt to me at birth has never been lifted. The stigma never resolved by the constant reminder that you will have an unexpected death, just like you get an unpredicted crisis. In the mind of some, having the disease means that you are constantly discriminated against. Some say that you cannot fly often because your spleen could be enlarged when the real issue is pressurisation of airplanes to provide enough oxygen at high levels. Others think that because you lose some much time in the school system you will not be useful to anyone and condemn you to a life of hopelessness and helplessness where others will have to support you all your life.
Every patient is different therefore this shared experience must be a guide we can only look forward and do our utmost to work and encourage more and more research to enable a better understanding of sickle cell disease and fight for improved health services. In the meantime, we should make a conscious effort to better our selves on how sickle cell disease affects us individually by having a written diary and learn for yourself about yourself as my experiences may be different to yours.
Some people wonder that you are still alive and are surprised when you tell them that you have sickle cell disease, that you look so well; I do not know what they will say when they see you at your most vulnerable and in pain,
Lead your life in moderation, no excesses and find yourself a good wife.
Your loving uncle