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.

NICOSAN vs. HYDROXYUREA

There has been a lot of talk about drugs that treat Sickle Cell Disease. (Not to mention cures that work for a few people but not all????) Currently, I’m not taking any drugs but I really wanted more information about the two drugs available, Nicosan and Hydroxyurea.

Here’s what I found:

The first drug I’ll discuss is the only US Food and Drug Administration (FDA) approved drug for the treatment of Sickle Cell Disease. It is Hydroxyurea, which is sold in the US under the trade name Hydrea(TM). It has also been used in the treatment of leukemia and certain other cancers.

Hydrea induces the synthesis of fetal hemoglobin, which inhibits the production of the abnormal sickle cells (what the heck does that mean?). I believe that means; this drug causes the production of healthy fetal hemoglobin without the sickle cell abnormality. In other words, it makes your body make good hemoglobin. Why do we care? Well, hemoglobin carries oxygen inside the red blood cell. One little change causes the hemoglobin to form long rods in the red cell (when it gives away oxygen). These rods change the red cell into the sickle shape…and we don’t want that.

The reports on this drug are mixed. I’ve read that not all patients respond to this treatment, and I’ve read of cases of leukemia being developed in patients…now, that’s scary.

Second, there’s Nicosan. Formally know as Niprisan, and in America it’s known as Hemoxin. This drug is not approved by the FDA. It is manufactured in Nigeria, Africa and is supposedly in clinical trials there, but none have begun in the United States. This drug is a plant base drug which means it’s made from organic plant material…that’s gotta be good, right? Some side affects reported are rash, headache, and weight gain.

XECHEM, the company that makes Nicosan in Nigeria, Africa says “Though NICOSAN/HEMOXIN does not cure Sickle Cell Disease (SCD), the medicine greatly reduce the degree of "sickling" of the affected red blood cells, which in turn eliminates or greatly reduces the devastating secondary consequences most patients succumb to, including strokes, kidney and liver failure, and extremely painful episodes known as "crises."

The problem with this drug is complicated.

It’s not FDA approved. Now, to get a drug tested and approved by the FDA takes loads of red tape (aka requirements). These red tape requirements could take a drug many, many years to get approved for use(in the US). This does not mean that a drug is not good or that it doesn’t work (look at Chinese medicines that are not FDA approved…where was the FDA in the first century anyway????)…..get it….the FDA is an American drug law/rule/red tape. But somehow, it makes me feel safer to take a drug that’s approved, and tested, with side affects known.

In addition, XECHEM (the company that produces Nicosan in Nigeria), said in a report to the SEC, “There is a lack of data to document the influence of raw materials (i.e. plant material quality, age, time of harvest, location, soil quality, preparation, handling, etc.) on the production of NICOSAN/ HEMOXIN.”
I have a problem with this lack of “data”.

Also, I’m not sure about the future of Nicosan because, according to a report to the Securities and Exchange Commission (SEC) by XECHEM, they are having severe financial troubles and may file bankruptcy.

So, in conclusion, I don’t know anything. All I know is that there are people suffering with Sickle Cell Disease, like me, and I would love to take a pill and send it far, far away.

Sickle Cell Pain

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Let’s finally talk about pain. I’ve avoided the subject because I wanted to stay upbeat and positive. But, the more I write and talk about Sickle Cell Disease, the more I see the need to discuss pain.

How does Sickle Cell pain feel you ask yourself? Well, I’ll tell you a few examples of the types of pain that I have personally felt during my life’s journey with Sickle Cell Disease.

1. Stabbing pain like a knife is pressing into my bones (sharp and thrusting).
2. Throbbing pain like a motor is running and it’s drilling pain through my body.
3. Quick burst of pain like a lightening bolt shocks electricity through me.
4. Slow, nagging pain that hangs around all day and night continuously tapping at my joints and bones.
5. Bloated, swollen pain like a ball is sitting on my spleen tender to any and all touch.
6. Headache/eyeache pain like a migraine that comes and goes whenever it pleases.
7. Sickle Cell “crisis” pain is like everything described above all at once.

Yellow Eyes

I always wondered why people who suffered with Sickle Cell Disease had yellow eyes. Not yellow contact lenses, but the white part of their eyes were yellow instead of white. I found the whites of my eyes would turn yellow periodically, and the only time they truly became white was when I was hooked up to an IV of glucose (sugar & water) in the hospital after a Sickle Cell crisis.
(picture is from internet...not me)

My curiosity peaked. I wanted an answer to this question, as well as all those people who asked me “Why are your eyes yellow?” Google came to my rescue as I searched key words “Yellow Eyes”, and here’s what I found.

1. Usually yellow eyes result from the build up of by-products from the increased destruction of red blood cells.

2. Yellow discoloration of the skin, and whites of the eyes are a result of an excess of the pigment Bilirubin in the bloodstream (also known as Jaundice) http://en.wikipedia.org/wiki/Jaundice

3. Bilirubin is the yellow breakdown product of normal hemoglobin. http://en.wikipedia.org/wiki/Bilirubin

4. Heme is formed from hemoglobin, a principal component of red blood cells.

5. Bilirubin is excreted in bile, and if its levels are elevated, it is responsible for the yellow color of bruises and the yellow discoloration in jaundice.

Conclusion,
People with Sickle Cell have an increased number of “damaged/dead” red blood cells. These red blood cells contain hemoglobin that’s damaged/dead too. This damaged/dead hemoglogin contains Bilirubin which is the breakdown product of hemoglobin. A larger than normal amount of Bilirubin in the body, accumulates in the blood causing jaundice. (yellow skin and eyes, and darker than normal urine.)

Shout Out To Clinics

This is the first shout out (say hello) to all those people/clinics who provide comprehensive services for both adults and children and the families of those with Sickle Cell Disease.

The University of Illinois Medical Center has a mission to "improve the quality of life for individuals with sickle cell disease and to reduce the morbidity and mortality of the disease through high quality medical care, patient community education, and research into new therapies.

And, they do just that!

Their encouragement, compassion and quality medical support is greatly appreciated. People with Sickle Cell Disease live better because of your tireless work.

THANK YOU!!!!!!!!!!