Tag Archives: sickle cell disease

PRIAPISM.

Priapism, Corticosteroids, Opisthotonus and LMP (which for all intents and purposes should be Last Menstrual Period, but is apparently not) are some names of teams that make up the (medliga) football league of the University of Ghana Medical School. It left me wondering how team names are chosen and what, if I ever ventured into that business of football club ownership, I would call mine.

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Now, Priapism FC. They go by the mantra, STAND STRONG, HARD and FIRM. Interesting isn’t it? Team priapism, new entrants into the medliga last year, actually ended up league champions. In the words of their coach “It is proof that with hard work and determination you can turn the most dire situation into success” The guys actually stood long, hard and firm. Taking a cue from them, I guess WE ALL NEED SOME PRIAPISM IN OUR LIVES. Eeermm actually, you really do not want to have the medical condition though. U can take my word for it.

I think at this point, a disclaimer is in order. MEDICAL INFORMATION. BRACE YOURSELF!

WHAT IS PRIAPISM?

Priapism is a medical condition in which there is an abnormal, persistent, involuntary, prolonged erection unrelated to sexual stimulation and unrelieved by ejaculation. Simply, the erect penis does not return to the flaccid state despite the absence of both physical and psychological stimulation within 4 hours. Priapism is a MEDICAL EMERGENCY and should be treated as such,

It is categorized as either high-flow or low-flow (which accounts for about 80% of cases). Management is based on this categorization, as both are managed differently. There is a myriad of causes of this condition such as spinal cord trauma ( neurological), enzyme deficiency (G6PD), drugs, malignancies and vascular (haematological-Sickle cell disease and trait, thalassaemia and leukaemia).

Priapism in sickle cell disease is categorised as low-flow priapism. Sickle cell disease accounts for 89% of adult cases of priapism with studies indicating that 42% of adults with sickle disease will develop priapism at some point in their life. In the paediatric age group, 2/3 of cases of priapism are in children with sickle cell disease. Evidently, priapism affects people of all age groups with its peak between the ages of 19 and 21 in patients with sickle cell disease. It obviously affects only men, usually African or African America (even though my reading yielded mention of a female equivalent, clitorism, which is not widely accepted as a true  equivalent).

Without going into much detail, what basically happens is that blood in the penis becomes trapped and unable to drain, leading to, ischaemia, scarring and permanent erectile dysfunction (if not promptly managed). In a person having a sickle cell crisis, just as occurs anywhere else in the body, there is occlusion of vessels, increased blood viscosity, stickiness of sickle cells to the blood vessel walls, depletion of nitric oxide ( a potent vasodilator), hypercoagulabilty of the blood, chronic inflammation and a lot more mechanisms by which  vaso-occlusive crises cause damage. It is thus no wonder that sickle cell disease accounts for a large proportion of cases of priapism and that so many patients suffer this complication.

The key feature of priapism in sickle cell disease is pain, apart from of course an erection lasting more than 4 hours. Immediate medical attention should be sought to prevent occurrence of complications. On reporting to the hospital, labs will be done to determine the levels of all blood cells. This is important in determining the actual cause of the crisis. Blood samples will also have to go through checks to ensure that you get the right blood group because management actually involves a blood transfusion. The doctor will also perform other tests, including angiography if necessary.

General management of priapism involves both medical and surgical interventions. Every patient who suffers priapism will need to see a genitourinary system specialist. In sickle cell disease however specific protocols need to be observed. For one, priapism is a complication of a vaso-occlusive crisis. Thus, the crisis needs to be managed effectively. The doctor will take steps you are probably already familiar with such as adequate hydration, analgesia, alkanization and oxygenation as appropriate.

Next step is an exchange blood transfusion. This would seek to increase your red blood cell level and exchange the sickle cells in your blood for normal cells from the donor blood. After which oral medication can be given. Then the blood needs to come out.  Under local anaesthesia the blood will be aspirated from the penis. This is crucial in reducing the swelling and built up pressure. If these medical options fail to bring relief, medication will have to be injected directly into the penis and if it still proves unsuccessful, a surgical shunt will have to be inserted.

As you have read above, this is complex stuff. Staying at home with priapism, even for persons without sickle cell disease, who may have it as a side effect of medication or due to recreational drug use, really is not a good idea.  The complications as I already mentioned are ischaemia, due to the blood in the penis which can’t drain, thus oxygen cannot be supplied to the tissues as well as impairment to the removal of metabolic waste. Damage to blood vessels, resulting in long term reduction in erectile function (impotence) and in severe cases, gangrene which will result in the penis being surgically removed.

What can we do?

  1. Don’t have sickle cell disease.  Radical as this may sound it has a simple explanation. Since you are more likely to have priapism as a complication of the disease, primary prevention, which I am very passionate about, is simply that you get tested and know your genotype and that of your partner and ensure that your child does not have sickle cell disease. Alternatively, IVF with genetic selection and bone marrow transplants and all the financial and heath implications that come with them need to be considered realistically and appropriate measures taken

2. For the rest of us who happen to already be born with sickle cell disease:

*stay healthy ie. anything to prevent a vaso-occlusive crises

*take your daily meds

*stay hydrated

*stay warm

*go for routine medical checks

*eat right

*avoid stress

*avoid use of recreational drugs

*pay attention to danger signs from your body and take appropriate measures…

Live each day to the fullest, make it count.

Love,

Sefakor-Enam.

(I hope this has been educational. Please like this post, comment and share. Thank you.)

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LITTLE PEOPLE!!!

Children! I love them. Really, what is there not to love? They generally love me too. The reason for the mutual ‘amor’? Well, I guess the kids can just sense that am not like the other big people. You know, putting a cap on all things fun, and such.

ImageWant to talk about imaginary friends?  Well, my imaginary twin was the best. I could go on and on about her. Let’s! If you want to play, please, let’s! I love playing. Children are very interesting people. They have so much to say, somehow the ‘big people’ think they don’t usually make sense, but they do. You just have to patiently follow their train of thought to realize how profound some of their statements are. Anyways, I had such a great time at the department of paediatrics I am seriously considering a carrier in paediatric haematology. I think I’ll have the most job satisfaction spending my days around children.

Let me just say, y’all need to start paying attention to the children. For one, they are not simply miniature adults. They are people. Actual humans, with the entire spectrum of emotions you and I feel. We can start by showing them a little respect and not considering them as ‘little people’ that are not smart enough to have a say in anything. What is that we say about life beginning at 40? Hell, by this, I ain’t even born yet, I guess that makes me one of the little people then. We the children would like to be heard, if that is not asking too much.

Anyways, while at the department of medicine I really thought there were too many sickle cell patients on the wards. Within the period, we saw sickle cell patients from about 20 to 65 years with conditions from pneumonia to infective endocarditis to HIVAN, etc. I also heard about sickle cell patients on other wards  in the department. Their current admissions may or may not have been directly related to sickle cell, but I was just uncomfortable with how many there were at any given time. I felt like sickle cell was following me everywhere I went.

Oh, but nothing could prepare me for my experience at the department of paediatrics. There were just too many children in crisis on the wards, it was simply traumatic. There were those who came in vaso-occlusive crises, VOC. They would spend a few days on the ward and be out, feeling absolutely well.  Almost always there was the characteristic ‘gi-normous’ well-used folder. Been there, done that! It really is not a pleasant feeling, spending a lot of time on admission and being jabbed constantly to get your pain meds and IV fluids.

At a point, one of the kids, absolutely terrified of needles threw such a tantrum when it came time to receive his pain meds, I really felt sorry for him. The child was screaming, on top of his lungs. He just kept shouting, “I’ll bite you, I’ll bite you, leave me alone.” I guess that was the most violent thing his young mind could think up to harm his ‘assailant’. Suffice it to say, he got his meds anyway and slept like a baby after. If only that was the most violent thing in the world, won’t it be such a peaceful place? Ok, some smart a** will probably produce a biological weapon transmissible through saliva, but still. Just think of the US sending a group of marines to Iraq to bite suspected enemy combatants. Won’t that be something?

Staying on topic. The constant admissions and how much school we miss really takes it toll. One of the kids I clerked presented with severe back pain. Another VOC. The child was in pain, yet would not cry. Brave kid! He kind of reminded me of…me! He was wincing, doing stuff I’ll generally do when I am trying to act all macho. Well, I noticed and checked his folder.  He was two hours behind on one of his pain meds. I prompted the nurse who attended to him immediately and he was pain-free and asleep in no time. That made two happy kids, yes, I was happy too.

So I returned to the ward the next morning and my friend was crying. Had been for quite a while. First thought, these people have delayed his meds again. So I asked, turns out he was fine. Very fine, actually too fine. He wanted to go home. That was the reason for the tears. You see, my new friend ranked 10th out of 34 in his class in the previous term and was two weeks away from exams. Simply he did not have time to be sick and on admission. He wanted to go back to school. Now, that is my kind of kid. You know the whole drink loads of water advice for persons living with sickle cell, I asked if he drinks his water and in the cutest voice ever he replies, “I drink plenty water so that my sickness will go away”. Tell me, this is not the best kid ever. Very determined, again, like myself. I hope he came out tops in class this term. He simply ain’t got time to be sick.

The second most common presentation of the children with sickle cell disease I saw was the jaundice and dark coloured urine combination. This one kid had very remarkable jaundice. I saw those eyes, and just had to find out more. After a few questions I found out he was SS. Thing is, he was 11 years. I don’t think he had any biceps or triceps to speak off. So I decided to measure his mid upper arm circumference, which we generally do not do for children of his age. It was less than 11. That is just to give you an idea of the severity of his malnutrition. Weirdest thing though, he had the most beautiful silky black hair. Malnutrition causes silky hair, (think kwashiorkor) but his was black. Whatever happened to the characteristic browning of hair?  Damn! I’ll pay to have his hair. There was no way this was due to malnutrition, but actually, it was!  By the way he was in class 1. Which is a class for 6 year old children, he is actually 5 years behind in his education. Turns out, he spends most of his time in the hospital instead of school.

Another child who had been on the ward for quite a while, presented with what was thought to be a vaso-occlusive crises. Later, treated for septicaemia, he spent about a month on the ward.  He was sick, he really wasn’t going anywhere. The whole time, his mom was at his bed side daily. Lord only knows the effect this had on her employment. X-ray findings later showed osteomyelitis (bone infection) which by the way is very common in children with sickle cell disease. Oh this kid was absolutely terrified of needles too. I think you would too after about a month of constant jabs. The plan, surgical management of the affected limb, which entails more hospitalization, more absence from school for him and work for mom, more health costs and so much more.

Stroke. Oh yeah, you read right. This child was about 10 years and had already suffered his second stroke. Young person! Stroke! Think sickle cell, you won’t be too far off.  ( Throw in other hypervicosity states into the mix for differentials). Thing with this child, cheerful boy, I must say, he had suffered a complication. Expressive aphasia. Simply, he couldn’t speak. He could hear and understand us but had lost the ability to communicate with us. I really have no idea what the long term plan for management is with this child, maybe some form of speech therapy would come in handy.

These are but a few of the wonderful children I met during my paediatrics rotation. I always made time to play, even once pushing one of the kids on his little car thingy along the corridors. When you have been on admission so often you really appreciate the little gestures.

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It was also sad, seeing so many sick children daily. Anywhere but the hospital! Children should be out playing, running around in school, yet, these children spend so much of their childhood in hospitals and my question is why? Simply, why?

Why are children still being born with a preventable disease? Why are children still being subjected to so much pain and illness? Is it lack of information or do we just not care about what the children we bear go through?

I keep harping on this point. It starts with you. Know your Haemoglobin genotype, and then that of your partner. Then you need to get us much information as you possibly can to help you make an informed decision.

What are the odds of you two having a child with sickle cell disease? Is that a risk you are willing to take? Can you afford to prevent it, maybe IVF with genetic selection. Is a bone marrow transplant affordable and accessible to you?

Or maybe you will have that child, because missing several days of work sitting by your sick child on admission will not adversely affect your finances. Maybe you can afford extra tutors so they don’t fall behind in school. You can afford all the medications, test and procedures to keep your child healthy. You can give that child a good life. Well, fine by me. As I always say it is a personal decision.

Ensure that you have considered everything before you make a decision. I am glad to be alive. I have a pretty good life, I can’t say same for others. Really, can you afford this? More importantly, can you live with seeing your child in distress?

In making that informed decision, remember, there are always variables that we cannot control such as a child with sickle cell disease having two strokes before the age of 10. You can afford it, but should you? Just consider this carefully and make an informed life choice.

Love,

Sefakor_Enam.

PERSONALITY PROFILE- PROF. KONOTEY-AHULU.

“…the patient is most important. There is a lot of focus on the disease. I focus on the patient… Do u know about my achiever’s foundation? Persons living with sickle cell disease like you. Medical students, doctors, even consultants meet up and share their experiences living with the disease…”-Prof. Konotey-Ahulu.
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Professor Konotey-Ahulu and yours truly. If you can tell me the biography of the Kardashian clan from before Kim got famous, and you don’t know of this man, it is really unfortunate. As he says, HTGT-Here today gone tomorrow. The passing of Prof Kofi Awoonor (RIP) reminded him again of HTGT. We have living African legends to celebrate. to inspire our youth to greater heights. Get ready to be blown away!

Professor Felix Israel Domeno Konotey-Ahulu, is one of the world’s foremost experts on sickle cell disease. He, in collaboration with some colleagues, undertook research that led to the discovery of Haemoglobin Korle-Bu and Haemoglobin Osu-Christiansborg. (Exactly, it goes beyond HbSS and HBSC). Oh, he actually coined the term gnatopathy. (Yeah, that’s who I’m talking about)

I heard about the good professor many years ago and I knew I had to meet him. There simply, in my opinion, is no one, who has as much knowledge on this condition as he. It was a pleasure  meeting and interacting with him.

Professor Konotey-Ahulu was one of the recipients of the Dr. Martin Luther King Jr. Foundation Award “for outstanding research in Sickle Cell Anaemia” in 1972, and in 1976, received the Guinness Award for Scientific Achievement in the Commonwealth “in recognition of his work in applying science to the service of the community”. He also received the 1999, Third World Academy of Sciences Award for outstanding work in the Basic Medical Sciences. In the citation to this award “his outstanding contributions to haemoglobinopathy” were mentioned, but also his “first description of some 5 new physical signs in clinical medicine” and  invention of the ‘Male Procreative Superiority Index’  (MPSI) which shed light on aspects of  African anthropogentics, among others. In 2008, he received the Ghanaian state award, The Order of the Volta-Officer for “distinguishing himself in medicine/research into sickle cells”These are but a few of the many awards he has won over the years.

In 2004, Professor Konotey-Ahulu was named one of the 100 greatest Africans of all time, in a publication by, New African, a popular monthly based in London. The list included greats like Nelson Mandela, Kwame Nkrumah, Kofi Annan, Martin Luther King, W E B Du Bois among others. Prof Konotey-Ahulu requested that he be replaced by Dr. J E Kwegyir Aggrey, about whom he wrote an article in the same publication later that year. (link below)

Professor Konotey-Ahulu, is well travelled, holding lectures in many countries all over the world. He was Annual Visiting Professor to Howard University College of medicine, and Honorary Consultant Physician to Howard University’s Centre for Sickle cell disease, Washington DC. In 1998, He was appointed ‘Chief visiting clinician/scientist, Kenya Medical Research Institute (KEMRI)’ where he is on the editorial board of the African journal of Health Sciences. Between 1965 and 2001 he published more than 185 articles, editorials, book reviews and comments. (261 with 10 yet to be published at last count)

A prolific writer, he was at one time, the Editor of the Ghana Medical Journal. He was also a member of the WHO Expert Advisory Panel on human genetics and a temporary consultant to the Commonwealth Secretariat’s Medical department in London. Among his published works, are a 643 page book titled THE SICKLE CELL DISEASE PATIENT and a 32 page booklet titled, SICKLE CELL DISEASE-THE CASE FOR FAMILY PLANNING. Having visited many sub-Saharan African countries, studying AIDS, he published a 227 page book titled, WHAT IS AIDS?

He is a visiting consultant physician at Cromwell Hospital, London and Valco Trust Hospital Ghana, where he is involved in community work and adult education in the local languages. He was an external examiner in the University of Ghana Medical School’s department of Medicine and Therapeutics. Professor Konotey Ahulu was appointed Kwegyir Aggrey Distinguished Professor of Human Genetics by the University of Cape Coast, Ghana in April 2000.

Professor Konotey-Ahulu is very active on new media. His website www.sicklecell.md is a very credible source of information for persons living with sickle cell disease and everyone interested in the subject.  There is a blog associated with the website. Please check it out sometime. He is also quite active on Facebook and Twitter, please like or follow his pages.

Prof. Konotey-Ahulu  is Ghanaian, born in Odumase-krobo on 12TH JULY 1930. He is married to Mrs Rosemary Konotey-Ahulu with 3 adult children and several grand kids. His early education was in the Gold Coast (Ghana). He proceeded to the United Kingdom for his premedical and medical training. After which he returned to Ghana. Most of his graduate work was  in the United Kingdom, but he always returned.  Professor Konotey-Ahulu is a devout Christian and has even written a hymn. He was also one of the founding fathers of Korle-Bu Community Chapel (Accra Chapel).

I am really very grateful for the opportunity to meet Prof Konotey-Ahulu and for all the work that he has done and is still doing in the area of sickle cell disease. I read his work, and I am impressed with the passion with which he educates people and corrects the misinformation out there. I wish him the very best, good health and  many more years of life.

Sources:

[pdf] Felix I D Konotey-Ahulu FGA Key achievements.

[pdf] DR FELIX I D KONOTEY-AHULU, CURRICULUM VITAE. September 2002.AD

http://www.modernghana.com/newsthread/132669/52/8289

BROKEN??? (Part 1)

Sickle Cell Disease is no excuse! I went to the university of Michigan for my electives! The head of the neurosurgery department is a woman who has never walked! She is in a wheel chair! She has spina bifida…

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SURGERY! Important info on those pink sheets. BAJA textbook and of course, tea

Strong words from a colleague, one I respect a lot. She is helping me with preparations for my end of surgery rotation exams, which by the way, are just next week.  She couldn’t quite understand why I seemed so unconcerned about my upcoming exams. Considering, I’ve missed a lot of school due to ill health and I still am, due to ‘aggressive physiotherapy’ totally messing up my schedule.

I’m broken. The drive is lost. A lot has happened in the past, but nothing has ever gotten to me so much. I am even surprised at myself. Everyone says, let go of this already, but I just can’t. Sickle cell has never been, and is still not an excuse for me. I am the kind of person who goes all out. Gives a-hundred-and-ten-percent when  I am healthy, just so, if I happen to be unwell, I don’t bother about missing school and such. That’s me!

In recent times however, there has been a change. The genesis? End of medicine rotation exams. Yeah! That was one terrible time for me. Of the 12 week medicine rotation, you could say, I was only fully present for 4 weeks. My body started acting up. I knew I was unwell, just couldn’t put a finger on it. Frustrating, that’s what it was. Started seeing my doctors. Diagnosis? Relapse of pneumonia, right through exacerbation of bronchitis secondary to asthma to rib infarcts. Everything and anything.

Every diagnosis came with a new set of drugs. Went through the normal antimalarial, analgesics, antibiotics (from co-amoxiclav to levofloxacin), steroids( both prednisolone and symbicort pump and oh, I don’t even have asthma, did all the tests to prove that). Did xrays, lab tests, everything, I was still sick and in the end, my body just gave up. We had had enough.

One Friday, while doing what I usually do, stay on the wards after-hours to clerk patients, the ‘doctor’ became the patient. Excruciating back pain and there, I had my very own bed with my patients on my floor. Monday morning, my colleagues, consultants, literally my whole class knew I was on admission (I wasn’t amused). Was there for about a week in excruciating pain, and for the first time pethidine was not touching my pain.

Exams were approaching, and I needed to be out of there already. The pain meds were not working, my lab results looked good, there was no point keeping me there, so I was out. Exams in two weeks with an excruciating pain in my right shoulder (I am right handed), we resorted to an intra-articular steroid injection. Worked like magic. My joy was however short-lived.

Few days later, what do you know? Hip pain. Worst ever. On a scale of 1 to 10, I’ll say, we need a bigger scale.No kidding! Good thing, I did pharmacology, I know how to work my meds (no am not a drug addict, I take drugs because I need them). 100mg diclofenac was not touching my pain, and I wasn’t about to get re-admitted in under a week of discharge especially so, with exams staring me in the face.

So, I was limping around school. those were hard times. Now, we know it was the avascular necrosis of the femoral head. I did not know then. I would have probably ‘made reservations for a bed’ in the emergency room. Being the driven student that I am/was I went to the wards in my worst pain ever to examine patients and be the doctor. Though I more than anyone needed doctors at that time.

Then the day of the exam came…

WHY DE-BOARDINISE ME? STRUGGLES OF THE SICKLE LIFE.

De-boardinisation! Isn’t that punishment, reserved for the most disobedient, disrespectful, trouble-causing, simply, deviant students?  Punishment meted out after others have failed?  Is this not a decision resorted to only after a person has had several ‘visits’ to the disciplinary committee? Why would a straight A student be lumped up with this group?

This is how it plays out in most senior high schools in Ghana. Somehow, without doing anything wrong, I found myself being asked to leave the boarding house. All it took, the headmistress asking that I invite my parents to her office.

My offense you wonder? I happen to fall sick once too often. Like there was a stipulated number of times each student was allowed to fall sick. Sorry, I didn’t get the memo! Basically, all it took was, me being a person living with sickle cell disease, and like the students who did everything, from stealing to leaving school without permission, I got thrown out of the boarding house.

Apparently, there were too many students and the school was not willing to take ‘the burden of caring for a sick student.’ Notice that I am quoting. That would be my headmistress. She then proceeded to go through a list of students with ‘special needs.’ Turns out, I was just a nuisance she would rather not bother about. Suffice it to say, my mom was in tears at the end of that meeting.

She took her precious little nuisance out of their care. Oh I was incensed. Was ready to fight them but she would rather not,she was really hurting. I was still under 18. Could not have done it on my own, thus, we let it go, and I continued high school as a day student.

I believe determination and focus rather than the school you attend or the course you read,or the challenges you encounter along the way are what determine if you will make something of yourself or not.Image

Having said that, Sickle cell placed limitations on my choice of high school because my mom wanted me close to ensure that I was healthy, even though I could probably have gone anywhere with my grades. I find it  laughable then, that after settling, they found it within themselves to de-boardinise me. Oh well…

Having gone through all the challenges, missing school and exams. Sickness and hospitalization. After all that, I make it to senior high school, and what do you know? I am not even going to be given a fair playing-field  to make it to university? Seriously?

Give me a reason why they were right to de-boardinise me and I will give you 10, why their reasoning was flawed.

Medicine and science have progressed so much, it is even astonishing what we can achieve now. Yet, someone happens to have sickle cell disease and you treat them so unfairly. We might as well go back to being hunters and gatherers, live in caves. then we can justify our atrocious behavior!

Dedicated to the high school student currently fighting her de-boardinisation for having sickle cell disease. I did not fight  when it was done to me, and several others have suffered the same ordeal. Keep fighting. It starts with you, be the change you want to see in the world.

Constantly Adapting! This is my sickle life. (Part 2)

What I like most! sportsAggressive physical therapy! Whatever that means!!! For a sports enthusiast, that is music to my ears. Except, am not allowed to jog or play basketball anymore, because, that will be putting too much weight on my hips. Bummer! So exactly how aggressive can my physiotherapy be?

Seeing the glass half-full tho,I have access to a gymnasium, hopefully at a subsidized cost. All am gonna say is, watch out for my toned abs and butt. Oh, and am allowed to swim. Remember your basic physics? Archimedes’ principle? Upthrust? Yeah, my hip joint stays mobile without having to deal with my body weight. Guess I have a new favorite thing. Except, doesn’t cold water precipitate crises in some people? Isn’t that too much of a risk?

Oh, and still reducing weight load on my hip, no more of those high heels. Well, supposedly. Cut a girl some slack. I ain’t gonna wear heels as often as I do now, obviously. But never say never, right?

Dancing! Yes! Azonto, Amanda, all that, just turned serious health hazards for me. I ain’t even trying to be funny. The point is, am taking steps to reduce the rate of degeneration at my hip joint so that my femoral heads don’t collapse anytime soon, if ever. Its serious business.

The sickle life, is a life of constant adaptation. I don’t know if I can manage this whole reduced physical activity lifestyle, but I am all for doing everything to stay healthy, Because, life really is a lot of fun, and I cannot enjoy it if I am constantly sick. Any suggestions of indoor activities a ‘hyperactive’ person may find interesting?

Lets not make this a pity party. I am fine, can walk okay. Except when I get the pain episodes. I am fortunate enough to have access to best medical personnel and equipment, and blessed with the finances to do the required tests as needed. Others, however are not. I have been diagnosed quite early, steps are being taken to limit the effects. The same cannot be said for most people.

Hopefuly, you know more about avascular necrosis of the femoral head (osteonecrosis) now than when you started reading. Remember, sickle cell disease affects every part of the body. Get tested. Know your status. Is this a disease you will risk passing on to your child? Make an informed decision.

Constantly Adapting! This is my sickle life. (Part 1)

Dats me in the middle. Ilove basketball. Love all things sports!You have sickle cell, you are weak, fragile! You should not partake in any kind of sports. You have probably heard that notion passed around, maybe even agree, I however, strongly disagree.

Being proudly non-conformist, sports is my favorite thing to do. Am the kind of girl who likes to play basketball with guys, jog, because I think it is fun. Weird, right? Who thinks working out is fun? Well, you know those ‘happy substances’ released when you workout, endorphins? I need bucket loads of those. Considering all that I have to deal with, having sickle cell disease compounded by the crazy medical school schedule. Its all about the endorphins. It is a good feeling, you should all try working out.

Then sickle cell strikes again! What do you know? Bilateral avascular necrosis of my femoral heads (osteonecrosis). And am like, really? seriously? bilateral? oh no! basketball! babies! kill me now!                                                                                                                                           In all of us old bone is constantly being replaced by new bone at our joints, but  in sickle cell, because of inadequate blood supply to these joints, the new bone is not formed as quickly as the old is lost. Leading to death and possible breakdown of the bone. (osteonecrosis)

Apparently, jumping exerts at least twice your body weight on your joints when you land. That is a definite no no for me now, and the baby? I am absolutely crazy about those little people. Will cross that bridge when I get there. Now tho, in the orthopedic surgeon’s words, aggressive physiotherapy! Obviously, we weighed our options, we’ll leave surgery for later…