Aging and Sickle Cell Disease

When I was 10 years old a well meaning doctor bent down to my 3 foot height and told me very gently, “You know, you’re only going to live to be 30 years old.” I blinked and thought, “That’s a long time from now.”

Fast forward thirty-eight years and I’m 48 years old and still alive.

That doctor didn’t realize that he had given a 10 year-old child a death sentence. He could not have imagined that his prognosis would sit in the back of my mind as I turned 20, then 30 and then 40 years old. As I aged, I laughed at that doctor’s prediction, but secretly I prayed for God to give me more time.

As time passes, and I age, I discovered that Sickle Cell Disease would take its place as the most important thing in my life. The effects on my body have been gradual, yet undeniable. Regardless of how well I “take care of myself”, I have to pay attention to everything I do as it relates to my health.

Recently, I heard a young man with Sickle Cell Disease say “Sickle Cell gets better as you learn how to live with it.” Sorry baby, that ain’t true. Sickle Cell Disease doesn’t “get better.” The lack of oxygen, low red blood count and trapped sickling cells eventually takes its toll on your joints, internal organs and your life. This is not bad news, it’s the truth.

There is good news, however. There are things we can do to help ourselves live and age well. I call them “the five mandatory things.” They are; drink lots of water (to keep your blood fluid), take vitamins (to get extra nutrients), rest, rest, rest (to rejuvenate your aging body), eat healthy foods (anything that assist in blood development {I know I hate liver too}), and exercise (mild movement to keep the aging body mobile and to increase oxygen). These five things have helped me live past my doctor’s dooms day prognosis.

Does Sickle Cell Disease get better? No. Do people living with Sickle Cell Disease get smarter? Yes.

The smarter you are the longer and healthier you’ll live. Will you still have episodes of pain? Yes. But, the better you get at managing your disease (doing those five things) the better you will manage the “triggers” of Sickle Cell “crisis”. The more clearly you are about accepting your condition, AND your responsibility to live your best life in spite of it, the better life will be. There are some uncontrollable issues like stroke, and spleen infarctions that can’t be managed, but so what.

We can hope for a “complete” cure, and in the meantime, also live full happy lives. I’ve even heard of people with Sickle Cell Disease living to be 70 & 80 years old. (I haven’t met any of them yet, but I hope to join their club of survivors.)

Do I know a thing or two about living this life with Sickle Cell Disease? Yes, I do. All 48 years of it (and past that darn number 30!)

BCL11A

Researchers have finally identified the genetic address of Sickle Cell Disease. It’s BCL11A, that wonderful alphanumeric.

In a report from the National Institute of Health on Friday, December 19, 2008, they report, “Researchers have identified a gene that directly affects the production of a form of hemoglobin that is instrumental in modifying the severity of the inherited blood disorders sickle cell disease and thalassemia. The discovery could lead to breakthrough therapies for sickle cell disease and thalassemia, which could potentially eliminate the devastating and life-threatening complications of these diseases, such as severe pain, damage to the eyes and other organs, infections, and stroke."

This is only the beginning.

Researchers report that by suppressing the gene BCL11A, fetal hemoglobin (HbF) production could improve dramatically. They say, “New therapies targeting BCL11A would be the first to directly affect the natural processes involved in increasing HbF.”

They’re not there yet.

So, what does this mean? I don’t know, but what I do know is that if researchers can “target” the gene, then help is on the way.

I hope I live to see the day.

Source:
http://public.nhlbi.nih.gov/newsroom/home/GetPressRelease.aspx?id=2607

This Ain't No Pity Party

I was reviewing my blog and I kinda felt the “woe is me” vibe coming from my words. That is the farthest from what I feel, think or believe. I have Sickle Cell Disease, like millions of others. We all suffer in silence because no one can feel our pain for us. We all live past this pain. We all work, play, sing, and dance despite our condition. Heck, a few of us even do great things with our lives (shout out to T-Boz from TLC). So, this blog is not a pity party, a cry to the blues about my sad story, or even an indirect way of getting compassion. I live on anyway!

What this blog is is a light shining in the dark. The darkness of ignorance and suffering. This blog is a way to document the plight of people living with Sickle Cell Disease and offer an “inside look” at our suffering.

Do we want compassion? Yes. A warm hand on our throbbing legs is greatly appreciated. Do we want pity? Hell no! We lay in our hospital beds with IVs of glucose and blood flowing and all we want is a smile, our hands held, and your love. Thank you very much.

Work and SC

Most people are defined by the work they do. A fireman will boast about how many fires he puts out, a doctor will describe a perfect surgical procedure she has performed, or a singer will sing songs that people love to hear. Work is good, and a job well done always feels good too.

People living with Sickle Cell Disease, if they can work, press past the pain and limitation of their bodies and try to earn a living like everyone else. We take our medications and pray for painfree days. We get up eachday and give life the best we have. When Sickle Cell Disease, however, takes a more prominent place in our lives, work seems impossible. Simple tasks, like getting up and getting dressed can take all our energy. When this dreadful day comes, a person's sense of worth can come into question. They might ask, “Do I still matter?”, and “Am I of value to anyone or anything?”

I asked myself these questions when Sickle Cell Disease came and took my career away from me. No longer could I answer the question, “What do you do for a living?” I was sick and in pain, that’s all. The career I worked so hard to achieve for over twenty years was gone. Even though my mind was willing, my body and Sickle Cell Disease said “enough!” Since that time, I have made peace with Sickle Cell Disease. And I have concluded, I do matter, despite my illness. I have value, even if I don’t have a career to boast about.

The complications of Sickle Cell Disease are: (pneumococcal pneumonia,gallstones, enlarged heart, heart murmurs, myelofibrosis, anemia, enlarged spleen/destroyed spleen, enlarged liver, oxygen deprivation, high Blood pressure in the lungs, pulmonary hypertension, avascular necrosis, stroke and pain) should also include the loss of work, career, value, and worth.

Despite these losses, people with Sickle Cell Disease have to fight each one of these complications, and we must live on….anyway.

To Those Who Care About Us

I was going to write a separate blog for each wonderful Sickle Cell Center, Hospital, or Clinic that stood out as a beacon of hope for patients with Sickle Cell Disease. What I found was way too many of them to mention separately.

There are so many doctors, advocates, universities, clinics and organizations who are fighting in-behalf of patients with Sickle Cell Disease. They are working to touch the lives of hurting people, researching new drugs to give comfort for their pains, and fighting the uphill battle to find a cure. The more I researched the more I was moved to tears.

There is an army out there who cares about me and all the other patients suffering with Sickle Cell Disease. To this army of health care professionals who see pain and suffering everyday, I say thank you for your compassion. To the advocates who organize walks, conferences and seminars, I say God bless you. And, to those researchers who never get tired of looking into a microscope at sickle cells, looking for a cure, I say DON’T GIVE UP.

There is an army of Sickle Cell patients cheering you on, petitioning God in your behalf, and silently hoping for your success. I tip my hat, I bow my head in respect, I yell “YIPPY!!!”, and I pass on their love for all your hard work.