Expecting Victory with Sickle Cell Disease

This past January I met an orthopedic surgeon for a second time. I met him 12 years before when he had replaced a recently retired seasoned orthopedic surgeon. The seasoned orthopedic surgeon had given me the diagnosis of Avascular Necrosis of both hips in 1992. If you do the math that’s a total 25 years of living with “bad hips”. 

The older “seasoned” surgeon told me at my last appointment with him, “Wait as long as you can before getting a hip replacement”. I held onto this “wait” idea until I met his replacement.

At my first appointment, he seemed to rush me into scheduling a hip replacement. Scared, but wanting relief, I agreed.  We talked about doing the surgery in the next few months and then I panicked. I kept hearing the older, and I thought wiser, doctor’s instruction to “wait.”  So, when months past and the surgery scheduler called I never returned her calls. I was going to wait.

Flash forward to January 2017 and I meet that young (a little too eager to cut my hips off) orthopedic surgeon for the second time.

Feeling embarrassed that I stood him up for our surgery date back in 2006; I was all prepared to offer my excuse. He lightly knocked on the door, and then entered. Before I could open my mouth he said, “I remember you”.  He was older now and still handsome, but worn. The fresh faced youngster, who was inching to cut my hips off, had lived some life. He had done some hip surgeries on other sickle cell patients and he had experience.  

We talked about the past, my not following through with surgery scheduling and the advancements in hip replacement techniques. We talked about my history of stroke and concerns about sickle cell complications. We were very transparent about what was going to happen. 

I told him “I’ve dealt with my fear because I’m in the hands of Jesus”.  And, I really have released this whole matter to my Savior and Healer, Jesus Christ. 

I have done all I could do from 1992 to 2017; with herbs, exercise, message therapy, etc.  All of these prolonged the issue but the issue remains. Avacular Necrosis in both hips, a complication of sickle cell disease could not be ignored any longer.  

The one thing that stuck me as God’s wisdom, was when he told me, “You’re in a fork on the road. One road leads to the same suffering, the other leads to a life of possibilities. Decide what kind of future you want.” 

I made my decision....surgery scheduled March 2017 and I’ll trust Jesus and leave the consequences to Him.

New Perspective- Living With Sickle Cell

This picture sums up my summer of 2015.

A trip to Utah to meet my newest granddaughter Kyndal, led to a health scare that I must take seriously. Utah, at 4400 feet elevation, is bad for me in the winter. Winter cold mixed with lower oxygen levels is not good. I know this. But it's June, my birthday month and I just HAD to see my baby be born supposedly on my birthday. Neither happened and everything went downhill quickly.



It's all better now, but it gave me a new perspective....I want to live. I MUST do EVERYTHING right, then put the rest in Gods hands. My granddaughter lives in Kansas now, elevation 1230 feet. So elevation problem solved.

I'm getting back to family, friends, and advocating for people with sickle cell disease......and staying the heck out of Utah!!!!!!!!! Finally!!!!!!!

Iron Overload

Did you know if you get 10 blood transfusions (in your lifetime) that you are at risk for iron overload?

No? I didn't either.

What is iron overload?

Healthy red blood cells usually live 120 days, damaged (sickled) cells live less. People with sickle cell or Thalassemia sometimes need to get a blood transfussion.

When we receive a blood transfusion we get more "hemoglobin", which helps our blood carry more oxygen. This is one reason we feel better after a getting a transfusion.

The blood in the transfusion also puts iron in our body. Over time, this iron builds up and our body has no way to get rid of it. It does not eliminate iron like other waste. This is Iron Overload (IO).

We can still have anemia AND have too much iron.

Too much iron can build up in our body (after 10 or more transfusions), even if a long time has passed between transfusions. (I DIDN'T KNOW THIS)

Iron Overload (IO) can lead to serious health problems, both now and later. Too much iron in your body can: (1) weaken your immune system, (2) cause sexual dysfunction or infertility, (3) heart failure (4) Liver damage (cancer), and (5) Diabetes.

Find out more information on Iron Overload (IO) and what actions you can take to be transfusion smart.

www.betransfusionsmart.com

RedCross.org

Airline Travel & Oxygen

I wrote a blog post “The Best Therapy,” about my love of travel. I received many favorable responses. I was excited about hearing from other people who love to travel, like me, despite dealing with a disability. I was excited until I received a letter written by Dr. Sota Omoigui, from the L.A. Pain Clinic in Hawthorne, CA.

Dr. Omoigui wrote a letter to The Open Hematology Journal, 2010, entitled, “Patients with Hemoglobinopathies Require Continuous Flow Supplemental Oxygen During Commercial Airline Flights.” In this letter Dr. Omoigui identified the results of a study he performed on people with sickle cell disease while they flew on commercial airlines.

The article is complex, but the conclusion is very clear. People with diseases of the hemoglobin (i.e. sickle cell disease) lose oxygen levels during airline flights. Less oxygen levels cause secondary issues like; avascular necrosis, splenic infarctions and bone pain.

Dr. Omoigui suggests, for flights longer than one hour, that commercial airlines should be mandated to provide supplemental oxygen (via nasal cannual). This is wishful thinking, asking airlines to give anything...for free. Maybe, they could be held accountable to comply with the ADA laws to provide "reasonable accomodation" for people with disabilities?

We patients with sickle cell disease could ask our doctors, when we consider flying longer than one hour, for an oxygen prescription. I know I will. I love to travel more than I am vain about carrying a portable oxygen tank with nasal cannula attached.

Check out Dr. Omoigui’s webpage for helpful pain care information:

L.A. Pain Clinic

True Sickle Cell Warrior - Adeja Johnson

In tribute to Adeja Johnson during Sickle Cell Awareness Month 2011.

A warrior is a person engaged or experienced in warfare or figuratively a person who shows or has shown great vigor, courage, or aggressiveness.

Adeja Johnson is one such person. She was a true sickle cell warrior.

Adeja fought sickle cell disease with the newly discovered procedure, Stem Cell Transplant. There are many complications of this treatment and the long-term effects are not fully known. Even with this procedure, there is still no "reliable" cure.

But Adeja and her family stood out in front of this battle with courage AND aggressiveness. As only a warrior would do, Adeja fought well and we are all better off because she did.

God bless you Adeja, I'll be looking for you in heaven.

See Video
Adeja Johnson Foundation

Kidney Infarctions

Complications of Sickle-cell disease related to the kidney are Sickle-cell nephropathy and chronic renal failure.

1. Nephropathy is caused by the long term usage of analgesics (pain medicines). These pain medicines include aspirin, acetaminophen, and nonsteroidal anti-inflammatory drugs, or NSAIDs.

Over use of pain medicines can cause a form of nephropathy called "chronic analgesic nephritis," which causes kidney function changes like:

a. loss and shriveling of tubules
b. thickening and scarring of tissue
c. inflammation.

Specifically, long-term use of the analgesic has been linked to death of kidney cells.

2. Renal Failure is the result of a kidney infarction. This is when a kidney artery is being blocked, compressed, ruptured by trauma, or interruption of blood supply which causes the cells to die.

The result of this damage is also known as kidney failure. Kidney failure is a medical condition in which the kidney’s fail to adequately filter toxins and waste products from the blood.

Problems frequently encountered in kidney malfunction include abnormal fluid levels in the body, increased acidity in the blood, abnormal levels of potassium, calcium, phosphate, and (in the longer term) anemia, as well as delayed healing in broken bones.

Depending on the cause, there may blood in the urine (people with sickle-cell should always lookout for this problem) and protein loss in the urine. Long-term kidney problems have significant repercussions on other diseases, such as cardiovascular disease.

Renal failure can be divided into two categories:

1. Acute kidney injury - acute kidney failure usually occurs when the blood supply to the kidneys is suddenly interrupted or when the kidney becomes overloaded with toxins.

Unlike in chronic kidney disease, the kidneys can often recover from acute failure, allowing the patient to resume a normal life.

2. Chronic kidney disease (CKD) can develop slowly and initially, show few symptoms. Renal function measurement is persistently poor. Sickle-cell complication falls into this category.

Where there is little renal function, dialysis and kidney transplantation may be treatment options.

How is chronic renal failure (kidney disease) found?

a. Measuring flow rate of filtered fluid through the kidney.
b. Kidney size.
c. Measuring levels of Creatinine (high).
d. Measuring acidity in the blood (increasing).
e. Measuring levels of Potassium, Calcium, Phosphate (abnormal).
f. Blood test for anemia - less than the normal quantity of hemoglobin (red blood cells)

BEWARE:

People with Sickle-cell disease have to be PRO-ACTIVE in their health maintenence. We should keep track of blood test results. Check to see if levels are too high or too low and notify your doctor if you notice blood in your urine.

Overuse of common drugs such as aspirin, ibuprofen, and acetaminophen can cause chronic kidney damage. Pace yourself when taking these medications, AND drink lot’s of water!!!!

Links:
www.wikipedia.org/wiki/Sickle_cell_nephropathy
www.wikipedia.org/wiki/Renal_failure

Sickle Cell "Crisis" Triggers

There are things in the lives of people with sickle cell disease that trigger a sickle cell “crisis”. Some things like simply living, working and surviving can causes triggers to push us over the edge into the pit of pain. Other things, in some cases, are preventable like lifestyle choices. Either way, once the trigger is set, a sickle cell crisis begins with full fury.

Some of the triggers are:
1. Excessive temperatures (too hot/too cold)
2. Infection (cold/flu/etc.)
3. Stress
4. Exhaustion (not enough rest)
5. Over-exertion (too much work/too much exercise).
6. De-hydration (not enough water)
7. Bad Nutrition (dieting/not eating good food)
8. Bad Lifestyle Habits (alcohol/smoking/drugs)

Once the crisis has begun, usually a hospital stay follows. God forbid a crisis leads to death, but usually a blood transfusion, oxygen, antibiotics, pain medication, and glucose (for hydration) are needed. After a week, maybe two, in the hospital you come out feeling thinner, slower, and hoping to never go back again. Wishful thinking.

Since we’re living in a stressful world full of every kind of trigger, it is important for people with Sickle Cell to really take care of themselves. What we can’t control, we should let go of. What we can control, we should. Good living and good habits will help us live.

Ps. I want to live as long as possible…so, I’m taking a nap right now! ;-)

Leg Ulcers

As a child I wondered why minor cuts to my legs and arms took a long time to heal. Everyone thought I picked or bothered my injuries but I hadn’t. They just wouldn’t heal. Sometimes, they would get infected and spread. As a child, I didn’t know what was wrong with me. Now I do. People with Sickle Cell Disease sometimes get leg ulcers that don’t heal. The reasons why are listed below. Live and learn.

What Causes Leg Ulcers?

Years of research have shown that the usual causes of leg ulcers are not a problem with the skin itself, but the lack of blood flow to the skin.
Because blood supply to the skin is crucial, ulcers can be a result of poor circulation. They can also be a result of chronic medical conditions such as diabetes, obesity, rheumatoid arthritis and hypertension (and sickle cell disease). Ulcers are also commonly associated with depressed or lowered immune systems and people who’ve had their spleen removed.

Treatment for leg ulcers
When treating leg ulcers, there are several areas that should be considered. Treatment can be a combination of: antibiotics, wound or ulcer care, anti-inflammation bandages, and elevation.
Once an ulcer develops, there is always the risk of infection due to bacteria getting into the ulcer. Antibiotics may need to be prescribed to control the bacteria growth and the spread of any infection.

Wound cleansing for the treatment of infection and increasing circulation are crucial. Many ulcers need weekly wound care to help them heal. This consists of having the ulcer regularly cleaned to remove any debris and rebandaged with clean bandages.

Anti-inflammatory treatment includes application of bandages wrapped with several layers of bandages over the dressing, and then a compression stocking is placed over the bandages. This helps to keep the pressure down in the veins, which is thought to be the most important aspect of ulcer healing. If any numbness or problems with circulation are discovered bandages should be removed immediately.

So long as there is no arterial disease, leg ulcers will also benefit from elevation. Elevating the leg higher than the hip helps to pull any fluid that has collected in the direction it should go, (towards the heart). This helps prevent excess swelling in the leg, which helps to relieve excess pressure in the veins of the leg as well, and promotes better healing.

If ulcers are neglected (because they don't cause pain) or if ulcers are not treated, they can lead to more serious problems.

Prevention
Once the causes of leg ulcers are under control the ulcer should heal by itself. Successful prevention for leg ulcers and successful treatment for leg ulcers must be directed at correcting the underlying cause, or what has prevented healing, not the ulcer itself. If an underlying disease is one of the causes of leg ulcers (diabetes, obesity, rheumatoid arthritis and hypertension (and sickle cell disease), it's important that it is treated.

Leg and foot ulcers have a tendency to recur in elderly people, and sometimes may require years of therapy. Prevention may include weight loss (if you are overweight) and regular exercise to promote good circulation. Body Detox can also help to purify the blood and cleanse the system. Good nutrition is also a good way to increase the body’s natural defenses to prevent ulcers.

Smoking is known to decrease the amount of oxygen that gets to the ulcer through the bloodstream, which prevents potential healing. If you suffer from a leg ulcer and smoke, you should stop smoking to allow better blood flow to the wound.

Nutrition
Nutrition plays a big role in healing leg ulcers. Getting the right mix of treatment and nutrition can help an ulcer heal. There are certain vitamins and minerals that are known to aid in the healing process: vitamin C is one of these vitamins. It has been found that vitamin C mixed with bioflavonoids increases the potential of better circulation.

Other vitamins known to help with circulation are vitamin E, B complex, zinc and amino acids. Vitamin K is also important; it is known to play an important role in the clotting of the blood. Getting the right nutritional balance with vitamins and minerals helps to fight infection, aids in blood clotting factors and properly oxygenates the blood for faster healing.

Your diet also plays a big role in curing a leg ulcer. A diet high in fiber and a lot of leafy, green vegetables, especially dark green vegetables, is best. You should avoid sweets and refined sugar and eat more garlic and onions, which help to increase circulation.

Surgery
Sometimes, it is necessary to perform surgery to help a leg ulcer to heal. It is a procedure that requires disconnecting malfunctioning perforator veins that are not allowing proper blood flow to get to the extremity. With the disconnection, the blood flow is redirected through the good veins and can get to the ulcer more efficiently to aid in healing it. After surgery, it is important to get plenty of rest while keeping your leg elevated. You will also need to continue wound care and to keep the ulcer free of bacteria.

For more information:
http://www.nativeremedies.com/ailment/natural-leg-ulcer-treatment.html
http://www.netdoctor.co.uk/diseases/facts/footandlegulcers.htmhttp://www.ehow.com/way_5339244_cure-leg-ulcers.html

*

Blood & Sickle Cell Disease

Blood is the life force within our bodies. Like oil in an engine, we can’t run without it. We can live with a transplanted heart and we can live without a kidney. If we lose a limb, we can order up a prostatic part and keep going.

Blood, however, cannot be replaced entirely. Sure, we can get transfusions, which will supplement what we already have, but we need a full supply to live.

People with sickle cell have red blood cells with a shorter life span. Usually, red blood cells live 120 days. For people with sickle cell the red blood cell lives only 10 -20 days. When those short-lived red blood cells die prematurely it also causes anemia. To top that off, in addition to having short-lived red blood cells, the ones we do have are sickled shaped and lacking oxygen.

Transfusions may be needed if red blood counts get too low to sustain life. Blood is made up of red blood cells, white blood cells, platelets, and plasma. Blood is transfused either as whole blood (with all its parts) or as individual parts. Every person has one blood type (A, B, AB, or O). Also, every person’s blood has an RH negative or RH positive. When getting a transfusion, the blood type received must work with your blood type.

*

Getting many blood transfusions can cause too much iron to build up (iron overload). People, who require multiple transfusions, are at risk for iron overload. Iron overload can damage the liver, heart, and other parts of your body. Blood transfusions can also damage lungs, making it hard to breathe. Most patients recover, however, 5 to 25 percent of patients (who are very ill to begin with) develop life threatening lung injuries.

When getting blood transfusions there is a (small) chance of contracting a virus or infection. In addition, some people experience allergic reactions. (I once grew hair on my chest after receiving a transfusion.) Hey, I’m a woman and this ain’t supposed to happen.

At some point, most people with sickle cell disease will need a blood transfusion, some more than others. Overall, this life flowing fluid is a blessing. Those of us who receive it, thankfully do. Those of you who give it, thank god for you.

For more information see:
National Heart, Lung and Blood Institute http://www.nhlbi.nih.gov/health/dci/Diseases/bt/bt_summary.html

NCAA Recommends Sickle Cell Trait Testing

My son plays football so this story is close to my heart.

The first time I heard about football players (with sickle cell) dying on the field was a story about a young player from Florida. I read, after he died from heat and over exertion, his family was blamed for "not telling anyone" that he had sickle cell trait. The problem was, he nor his family knew that he had sickle cell.

This terrible situation may be prevented forever, according to CBS Sports.com Senior Writer, Dennis Dodd. In his June 29, 2009 article, NCAA to recommend schools test for sickle cell trait he states, ... the NCAA and Rice University settled a lawsuit brought by the family of a Rice football player (Dale Lloyd) who died in 2006 due to complications resulting from sickle cell trait.

As a result of the settlement, the NCAA, regarding player safety, will now recommend testing for sickle cell trait of its players. In addition, Rice will help sponsor NCAA legislation to make sickle cell trait testing mandatory

Dale Lloyd did not die in vain. The legacy he leaves behind will benefit football players all over America. They will be tested prior to those HOT summer workouts and maybe now, they won't blame the players for not knowing.

Read Dennis' article:

http://www.cbssports.com/collegefootball/story/11903550

For more information on the subject:

Rice University -Dale Lloyd Bio Page - http://riceowls.cstv.com/sports/m-footbl/mtt/lloyd_dale00.html

Dale Lloyd Memorial Scholarship - http://riceowls.cstv.com/genrel/101106aae.html

Black Coaches and Administrators - http://bcasports.cstv.com/genrel/062807aaa.html

*

Sickle Cell and Stroke

I’ll start by saying I’ve had two non-serious strokes and many silent ones. It seemed odd to me that while I was having those strokes, doctors who treated me never wanted to say the word “stroke.”

I had all the symptoms: blurred vision, slurred speech, intense headache, and numbness on face and hands. Doctor’s seeing these symptoms treated me for pain, worked to bring my blood pressure down, and performed ultrasound scans to see if any clots existed, but they never said “You had a stroke.”

Thankfully, I’ve recovered. I also found out that people with blood disorders (like Sickle Cell) are high risk for having strokes. See more information below, get informed and live.

What is a Stroke?

Blood is circulating through your body all the time in arteries and veins. Usually, these blood vessels work fine and there's no problem. A stroke can happen if something keeps the blood from flowing as it should. A person might have a clogged blood vessel, or a blood vessel may have burst, flooding part of the brain with blood. Either way, with a stroke, brain cells die because they don't get the oxygen they need.

A "Mini stroke" or “silent stroke” is not full-blown stoke because the blood flow is only cut off for a short time and they don't cause the same kind of damage right away. These mini strokes are a warning sign that something is wrong and that a real stroke may be on its way.

What Happens During a Stroke?

A stroke usually happens suddenly, and a person having a stroke has several signs:

numbness or weakness on one side of the body
a very bad headache
dizziness
loss of balance or coordination
trouble talking or understanding what people are saying
trouble seeing

A stroke can cause learning disabilities and/or lasting brain damage, long-term disability, paralysis (an inability to move), or death. Anyone who has even one of these symptoms should get to the hospital right away.

What Are the Risk Factors?

Stroke occurs in all age groups, in both sexes, and in all races in every country. In African Americans, stroke is more common and more deadly--even in young and middle-aged adults. Conditions linked to stroke are:

High Blood Pressure - Treat it.
Cigarette Smoking - Quit.
Heart Disease - Manage it.
Diabetes - Control it.
Physical Inactivity - Just Move.
Poor Diet - Eat healthy foods.
High Cholesterol - Reduce it.
Certain blood disorders - "Sickled" red blood cells are less able to carry oxygen to the body's tissues and organs. They also tend to stick to blood vessel walls, which can block arteries to the brain and cause a stroke.
Alcohol Abuse - Limit it.
Illegal drugs - Don’t use them.

How Does a Person Get Better After Having a Stroke?

Recovering from a stroke can happen quickly or can take a long time. How soon someone gets better depends on how bad the stroke was and how healthy the person was before the stroke. People who have had a stroke may need medicine or surgery. Later, they may need re-habilitation. Treatment for a stroke will depend on what caused it.

Medicine - For a stroke caused by a clogged blood vessel (ischemic), the doctor might give the person medicine that thins the blood and keeps it from clotting too much.

Surgery - Doctors may do surgery to open up a clogged blood vessel to help prevent another stroke later on. If a person has had a hemorrhagic stroke, surgery may be needed to remove blood clots or fix weak blood vessels.

Rehabilitation - Rehabilitation, or rehab, means the person needs to relearn basic things, like walking, talking, writing, or taking care of themselves. They may need speech therapy, physical therapy, or occupational therapy.

How To Prevent Strokes?

Some strokes can be prevented in adults. Stroke-prevention tips are: Don't smoke, Don't drink too much alcohol, Eat healthy and be active. This can help lower cholesterol. Check blood pressure. High blood pressure is a major cause of stroke. Don't ignore problems like heart disease, high cholesterol, or diabetes.

For more information about stroke:

CDC's Heart Disease and Stroke Prevention (http://www.cdc.gov/cvh )

Department of Health http://www.omhrc.gov/templates/browse.aspx?lvl=3&lvlid=85

National Stroke Association http://www.stroke.org/site/PageNavigator/HOME

Kids info http://kidshealth.org/kid/grownup/conditions/stroke.html

Sickle Cell Information Center http://www.scinfo.org/nihchap9.htm
*

*

Altitude Sickness

When you go to the ocean the altitude level is equal to zero (0). When you travel up a mountain the altitude level increases and the air pressure decreases. At places like the Grand Canyon the altitude goes up to over 6,000 feet elevation, and in the Rocky Mountains of Colorado it can peak to over 14,000 feet elevation. As the elevation increases, the altitude is higher and the air pressure is lower. The lower the air pressure, the lower the number of oxygen molecules available to breathe. This is also known as thin air.

For people with Sickle Cell Disease, less oxygen means SICKNESS.

My altitude sickness symptoms begin around 3,000 feet. It starts with a headache, feeling tired, and dizziness. If I don’t get out of the mountains, then all of these symptoms get worse. At 4,000 – 5,000 feet, I begin to get confused and agitated. I have shortness of breath, my spleen, hands and feet begin to swell, and my head aches with no relief. At 6,000 feet, I descend into needing hospitalization. I throw-up, more headaches, dizziness, lips and palms turn white, and my body slowly collapses into a full Sickle Cell crisis.

How do I know so much? I found out about altitude sickness by accident. After taking a few vacations to the Grand Canyon and Utah I experienced altitude sickness first hand, the hard way. I travelled to these high places, got sick, and learned my lesson. I discovered that I can not go higher than 4,400 feet elevation EVER (without an oxygen tank). In winter, because oxygen is thinner by nature, I can not go above 3,000 feet elevation.

People with Sickle Cell disease have physical limitations, as well as geographic limitations. I know that I can’t visit the Swiss Alps, unless I have an oxygen tank strapped to my back and a mask flowing cool, clean oxygen into my lungs. That's ok with me. I am informed about my limitations and I can accept them. Information is power.

Now take a minute to inhale, now exhale. Enjoy each and every breath you have because it's very precious.

Why Do We Need Water?

In people with Sickle Cell Disease their red blood cells are dehydrated (they are thirsty for water). These cells are thirsty each and every day. Just imagine living in the Sahara desert and there is nothing but sand. Eventually, you will die of thirst, and you will die quickly. Well, that’s what happens to our red blood cells. They need water….everyday.

Dehydration in red blood cells, (according to American Society of Hematology) is due to increased loss of potassium, chloride, and water. (Yes, momma says “eat those bananas for their potassium too!!!”) By keeping hydration (water) in red blood cells they can survive longer. With longer surviving red blood cells in our body we will have more red blood cells (sickled or not) available for our beautiful bodies to use.

There has been research in trying to hydrate red blood cells with medication (US Institute of Health clinical trial), but this research has not fully been successful.
http://clinicaltrials.gov/ct2/results?term=ica+17043&cond= %22Anemia%2C+Hemolytic%22)

There is hope, however, that we can do our part by DRINKING MORE WATER, and we can wait for researchers to do their part. This is one more bit of information that our doctors don’t tell us. Why we need water? Now, I’m thirsty!

Retinal Detachment and Sickle Cell Disease

A complication of Sickle Cell Disease is a condition of the eye called Retinopathy. This condition occurs when the blood vessels in the retina become blocked, grow haphazardly or become leaky, and extra blood is trapped within the space between the eye lens and the internal membrane of the retina. This can damage the retina and cause retinal detachment.

Retinal detachment is a medical emergency. The detachment of the retina is painless, however, if not treated quickly, can cause permanent, partial, or total vision loss. Symptoms include: sudden appearance or increase in the number of “floaters,” (which are shapes that float in the eye and are seen in the field of vision), brief flashes of light in the eye, loss of the eye’s central or peripheral field of vision, a curtain appears to fall over part of the visual field, and sudden changes or blurring of vision.

To see, light must be able to pass through to the retina without anything getting in its way. You may not be aware of changes to the retina until your vision has been impaired. Distinguishing if blood is floating between the internal membrane and the retina's nerve fiber layer is not always possible, and this is why we must have our eyes screened every year.

My daughter suffers from this Sickle Cell complication and the suffering she endures is heartbreaking. Cataract surgery (at 14 years old), and vision loss is something she has tolerated. I watch her battle for healthy eyes with tears in my own.

The branch of medicine that is concerned with the diagnosis and treatment of eye diseases and conditions is ophthalmology.

Have you seen your ophthalmologist lately?

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!)

Sickle Cell Pain

####################
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.)

Why Is My Spleen Swollen?

After having a Sickle Cell crisis last year I experienced a new complication of Sickle Cell Disease. I experienced pain and swelling in the upper left part of my stomach. My doctors said that my spleen was swollen and that it should go down soon. Nine months later, it’s still swollen and tender to any pressure or touch. This problem isn’t going away.
I’d heard about spleen problems being associated with the many complications of Sickle Cell Disease, but I hadn’t got my dose of this problem, yet. Now that I’ve joined this happy group, I wanted to know more.

****** The problem I’m experiencing is called Hyposplenism (or reduced spleen function). And, they call it Asplenia (the absence of normal spleen function.)******

MY QUESTIONS ARE: What in the heck is the spleen and what is its function in the body?

1. The spleen is an organ in the upper left side of the abdomen that filters the blood by removing old or damaged blood cells and platelets.
2. It also helps the immune system by destroying bacteria and other foreign substances.
3. It also holds extra blood that can be released into the circulatory system if needed.
4. The spleen is a useful but is a nonessential organ. It is sometimes removed (splenectomy) in people who have blood disorders, such as thalassemia or anemia.
5. If the spleen is removed, a person’s of risk infection greatly increases.

WHAT DO I DO NOW?

Not much. As my spleen function decreases, I MUST prevent infections and get flu vaccines every winter. Eventually, my spleen may need to be removed as the “absence of normal function” causes more swelling, pain, or disfunction. I’ll hold my breath until that day comes…..just kidding, don’t want to turn blue too.

Oh well, life is hard, I'll get a helmet :-)

What is Avascular Necrosis?

Avascular Necrosis is one of many complications of Sickle Cell Disease. This condition is a result of temporary or permanent loss of blood supply to bones. Without blood, bone tissue dies and causes the bone to collapse. If dead bone tissue is near a joint (hip or knee), the joint may also collapse.

Is this painful? YES

I was diagnosed with Avascular Necrosis (also known as Osteonecrosis) in 1992. After having multiple Sickle Cell "crisis" in my legs, the lack of blood supply to my hip joints over-time caused Avascular Necrosis in both my hips. The cartilage has also decayed causing bone-on-bone exposure. When I walk, sometimes I hear a crunching sound from joint and bone rubbing against each other. The only long-term treatment for this condition is hip replacement.

A few years later, I was also diagnosed with Avascular Necrosis in my right knee which also causes joint pain and swelling. The eventual treatment for this condition is a knee replacement.

Am I afraid? NO

I have found herbs and vitamins that strengthen bones, help with pain, reduce swelling, and have given me some relief (and postponed the hip and knee replacement).

NOTE: I drink LOTS OF WATER everyday and I don't take any of these items everyday. I alternate them and sometimes I rest my system and don't take anything at all. Everything in moderation!!!!!!

Here's what I take:

Osteo Renew - for bone strengthening
Women's Ultra Mega - for bone health
any Multi-vitamin - any brand for vitamins A-zinc
any Antioxidant (with Green Tea, Grape Seed and Pine Bark) - to keep infection down.
Folic Acid - for blood
B-12 - for blood
Maxi-Minerals - for extra minerals needed for bone health
MSM - for joint and cartilage health
Garlic - for overall infection reduction
Iron - for blood
Cod Liver Oil - for vitamins A & D and joint lubrication
Flax Seed Oil - for overall health and joint lubrication
Arnica massage oil - to massage on knee and hip joints for pain
Yogi Joint Comfort Tea - drink tea to help relieve pain (It works!)
Echinacea/Golden Seal Tea - drink for immune health (helps cold/flu)

It's 2008, I'm not dancing anymore, but I'm still walking and still thankful for no titanuim replacements in my bones. Yeah!!!!!!!!!!!!!