Sickle cell anaemia* is most common in West and Central Africa where approximately 25% of the population have sickle cell trait and 1-2% of all babies are born with a form of the disease. In the United States with an estimated population of over 270 million, about 1000 babies are born with sickle cell each year.
I decided to highlight this debilitating condition by conducting an in-depth interview with Dr A, a remarkable woman in her late 40’s, who has lived successfully with the disease through childhood, marriage, motherhood, a demanding medical career, and relocation across three continents.
Her story, told in her own words, will encourage other sufferers to fight and survive, and to resist any attempt to pigeonhole or categorise them as disabled by their inherited condition. Thankfully, with medically advances and increased awareness, sickle cell anaemia is no longer a death sentence from birth.
INTERVIEW: LIVING SUCCESSFULLY WITH SICKLE CELL ANAEMIA
Q: At what age were you diagnosed with sickle cell anaemia, and what was your earliest memory of realising you had the condition?
A: I have had sickle cell disease all my life but didn’t really understand what it was or what it meant till I went to medical school. We were doing a physiology practical which required drawing blood and the instructor noted that my blood was thinner and paler than some of my colleague’s specimens. I eventually learned the specifics of sickle cell disease. I do remember as a young child going to the hospital and always having blood tests but I did not know what it meant.
Q: How did members of your family, and friends treat you as a result of their awareness that you had the condition? Were there others you could relate to in your environment?
A: I doubt my family really understood the implications of my disease fully. I don’t remember being treated any differently. I do remember being unable to keep up with my peers in activities that required some sort of physical effort.
Q: What impact did having sickle cell anaemia have on your education, ability to participate in sports, travel, independence from parents, etc?
A: As I mentioned, I doubt my family really understood the full implication of my disease. It had no impact on my education, but I could not keep up with my peers in sports. I also distinctly remember that even though I travelled a lot because I left secondary school in Nigeria midway to continue my education in England, sometimes I would develop pain after flying which impacted the first few days of wherever I happened to be.. I eventually learned about the changes associated with flying that can cause a propensity to have a crisis after traveling and I have learned some tricks to prevent or at least mitigate the effects.
Q: As a young woman, probably beginning to consider marriage and childbearing, how were your expectations shaped by the condition? Did you have any negative experiences specifically as a result?
A:I was aware that I needed to be careful choosing a partner to avoid having children with the disease but I was a normal teenager/young woman and did not ask dates for their status before considering them as friends/partners. I just consider myself very lucky that the person I fell in love with and decided to marry did not have any S genes. I don’t know if I would have been strong enough to look for someone else if he did. We did discuss the implications of having the disease on my life and the need for close medical care. He has always been by my side during the difficult times. I would have liked to have more children but we decided to limit it to 2 at his urging (I am so very fortunate) because of the difficulties we experienced during my pregnancies.
Q: Do you believe that your choice of profession was impacted by your having sickle cell anaemia? Were any professions barred to you by reason of the condition?
A: It is funny, but my disease had absolutely no impact on my choice of profession. As I mentioned I didn’t even really realise my medical condition and understand it until I got into medical school. I just wanted to be a doctor and never entertained the thought of following another profession. Maybe it was ignorance but I can’t remember why I was so attracted to the profession. Maybe if I had realised how much work was involved, I might have considered something else! LOL. I discovered later in life that I do like the arts . I pursue my love of the arts in my free time. I do love being a doctor though so I have the best of both worlds.
Q: How have you coped with times when you have had to be hospitalised due to sickle crisis, and what effect has that had on your relationships and work? What systems have you put in place to minimise the negative effects?
A: I have a rare combination of sickle genes which has afforded me the blessing of not having too many crises. I have had my fair share believe me but I don’t have them as frequently as some others. It has a little impact on my relationships but they go about their lives and just spend a bit more time than others by my side at the hospital after work and school if necessary. I know some of the triggers and try and avoid them at all costs; I stay out of the cold or wrap up really warm when I go out (since I live in a cold climate! LOL) I drink plenty of fluids to make sure I stay hydrated and I try to avoid stress in any way I can. I used to love to swim as a young girl but stay away from water now which is one of my big regrets. My work colleagues have had to pick up some slack when I have to take time off work but as I mentioned, it doesn’t occur very often so they tend to forget in-between episodes!
Q: Pain management is a vital part of the treatment of sickle cell disease. What have you found works for you personally, for optimum relief during sickle crisis?
A: I usually end up in the hospital for pain management when I have a crisis because they are so infrequent, they tend to be severe requiring IV medication.
Q: Are you apart of any support groups, and how does that help you psychologically? Are you aware of any sickle cell disease patients who are virtually crisis-free, and how do they manage the condition?
A: Where I live I don’t have easy access to support groups. I have several friends who suffer from the same condition but they all try to live their lives to the fullest. I have lost one relative and a very good friend to the disease, which makes me very, very sad.
Q: Can you name 2 or 3 factors which exacerbate the condition, to your knowledge?
A: Cold weather, stress, dehydration.
Q: Over the last 20-30 years, great strides have been made in the management of sickle cell disease, leading to a better quality of life and increased life expectancy for sufferers. Do you have any comment on these?
A: I am in awe of the strides that have been made, which is very useful for sufferers. Hopefully with the advance of technology, a cure will be found somewhere in the near future. It is a devastating disease.
Q: What special privileges do you think you may have enjoyed from family, friends, employers, the government or society in general as a result of the condition?
A: None whatsoever. I think it is because I don’t have too many crises. Most of my friends and family forget until they hear I am in the hospital.
Q: Can you name any celebrities who have come forward to admit that they have the condition, and perhaps spearheaded a campaign to find a cure?
A: I am not aware of any.
Q: What is the worst thing about having sickle cell disease?
A: One develops an intimate relationship with pain which is unfortunate. It can rule your life and limit one’s ability to do many activities. However I don’t let pain rule my life and I am aware of my limitations. I try to do whatever i can within those limitations. I would so love to go scuba diving in the Great Barrier Reef but I know that will never happen.
Q: What advice would you have for anyone who finds either from pre-natal tests or on giving birth that their baby newborn has sickle cell disease?
A: Education is absolutely vital. Educate yourself about the disease and get to know all the triggers to avoid and what treatment modalities are available . However within the constraints of the disease, just bring up your child as a normal individual. I consider it a blessing that my parents did not fully understand the implication of my disease; they did not impose any limits because they did not know. Otherwise they may have stopped me from doing things like getting in the pool, which I loved as a young girl.
Q: What impact has your faith had on your attitude towards having sickle cell disease?
A: My faith is strong and I do believe that even on my darkest days, there is always something to be thankful for. It may be that I haven’t been in hospital that year. It may be that I know I will be ok in a couple of days. I always have amazing support from family and friends, and always have a quiet assurance from my faith that at the end of the day all will be well. I understand that my life expectancy is statistically shorter than that of my peers but I believe no matter what, one must live one’s best life every minute of the day; breathe in that air, celebrate each day, live life to the fullest, do what you enjoy, appreciate every minute of every day.
Thank you for sharing your story with us. I wish you continued good health, and a long and happy life.
Copyright Angel Works, March 2013.
*In her article entitled “How to Live with Sickle Cell anemia” on www.livestrong.com, Rose Kvi gives a useful outline of Sickle cell anaemia, also known as Sickle Cell disease:
“Sickle cell anemia is a genetic disease that affects the red blood cells. A mutation in hemoglobin causes the red blood cells to be malformed. Instead of the circle shape of normal red blood cells, patients with sickle cell anemia have red blood cells shaped like a sickle. The sickle shape prevents the cells from carrying optimum amounts of oxygen, inhibits the ability of the cells to effectively travel through the blood vessels, and the malformed cells die early. A painful and potentially life threatening condition called a sickle cell crises develops when the cells get stuck in the blood vessels. There is no cure for sickle cell anemia. Treatment for sickle cell anemia focuses on keeping the immune system strong, preventing a sickle cell crisis and controlling pain.”
by Angel Works.
Wow! Interesting!
ReplyDeleteI wish you hadvspoken a bit more on those with AS and Thalassemia!
Great stuff!