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Treatment - Sickle Cell Anemia

Updated on May 22, 2019
Pamela99 profile image

After 22 years as an RN, I now write about medical issues and new medical advances. Diet, exercise, treatment, and lifestyle are important.

Pain with SCA

Source

My Experience as a RN

When I was in college studying to be a RN, one semester I was training in the pediatric area of a hospital. One child I was assigned to care for was an African-American boy, who was about 7 or 8 years old, and he had Sickle Cell Anemia (SCA). He was sitting on the floor playing with some little cars and trucks in the children’s playroom.

I was watching him and noticed some tears running down his face while playing, but he didn’t make any noise. This little fellow wanted to play, but he was in so much pain he really could not play at all. It broke my heart, as I am the mother of 3 boys, and I started to cry.

I learned two things that day:

  1. Sickle cell anemia is a horrible, painful disease
  2. I could not be a pediatric nurse.

Crying as a nurse when taking care of children is unacceptable!

Facts About Sickle Cell Anemia

In the United State there are 70,000-100,000 people with sickle cell anemia, which is an inherited disease. One out of every 365 African-Americans have SCA, 1 out of 16,300 Hispanic-Americans has this disease.

If each parent has a sickle cell gene, then a child will likely have this disease. If only one parent has only one sickle cell gene, the child will not get the disease. This disease can be easily diagnosed with a blood test. The earlier the diagnosis, the easier it is to avoid complications. The blood test is done on newborns in every state.

Red blood cells are round and normally flow easily through the blood vessels carrying oxygen, but with sickle cell anemia the red blood cells have abnormal hemoglobin. This causes the red blood cells to become stickier, and they are formed into a crescent shape.

These abnormal red blood cells can stick to the blood vessel walls, which can damage the lining. This situation can attract defensive cells that may enhance the stickiness and lead to actual slowing of normal blood flow and partially blocked blood vessels. This is painful, and it reduces oxygen delivery to organs and tissues.

Normal red blood cells live an average of 120 days, and sickle cells die after 10-20 days. This causes the anemia. Bone marrow cannot produce blood cells quickly enough to replace those that are dying.

Understanding Sickle Cell Anemia

Who Gets Sickle Cell Anemia?

This genetic disease is most common in families that come from the following areas:

  • South or Central America (especially Panama)
  • Families with ancestors from sub-Saharan African area
  • Caribbean islands
  • Mediterranean countries (Greece, Turkey, Italy)
  • India
  • Saudi Arabia

Sickle Blood Cells

Source

Symptoms of Sickle Cell Disease

he symptoms are mild in some patients and serious in others, requiring hospitalization in others. Anemia is the most common symptom, and constant pain is located in the bones.

Some of the other common symptoms include:

  • Fatigue
  • Shortness of breath
  • Cold feet and hands
  • Headache
  • Chest pain
  • Leg ulcers that do not heal
  • Jaundice, eyes and skin yellowed
  • Pale skin due to the anemia

The pain is termed “sickle cell disease.” When anemia exists over a long period of time it will cause damage to organ;, including the heart, liver, spleen, brain and kidneys. Other serious complications include: stroke, blindness, gallstones, bacterial infections and priapism.

Treating Sickle Cell Disease

Treatments

Medication treatments for sickle cell anemia include:

  • Children begin taking penicillin at 2 months until 5 years of age to prevent pneumonia.
  • Children’s vaccinations are essential to avoid infections.
  • Adults who had their spleen removed or have had pneumonia take antibiotics throughout their lives.
  • Pain medications are given during a sickle cell crisis.
  • Hydroxyurea (Droxia, Hydrea) taken daily reduces the number of crisis, but may result in more infections.

Endari is a new medication, and the first one to treat this disease in twenty years. This drug is given to people 5 years and older to treat the serious complications of this disease.

The only cure for sickle cell anemia is a bone marrow transplant, used for the most serious cases on children under 16 years of age. This procedure has serious risks. It requires a sibling on someone else that can donate stem cells for the patient.

Pain

Source

Ways to Help Prevent SCA Crisis

There are some ways to help prevent the crisis, which include:

  • Drink plenty of water
  • Avoid exposure to high altitudes, or area with lower oxygen levels
  • Do not get too hot or to cold
  • Get enough rest
  • Medications

Change Me - Ruben Studdard - Supporter of SCA Research

Medical Research

One study of 2764 patients between birth to 66 years of age found that patients with SCA died by 60 years for men and 68 years for females. An organ failure accounted for 18% of the deaths, which was typically renal failure. An acute SCA crisis accounted for another 33% without organ failure. Another 22% had a stroke.

The Sickle Cell Disease Association of America (SCDAA) is a non-profit organization that supports research, education and funding for individuals and even families.

In Summary

This genetic disease is very difficult to treat as the only way to eliminate the disease is stem cell replacement, which is a risky, difficult procedure. There is more success now, and hopefully the future will result in more ways to treat this very difficult disease.

This content is for informational purposes only and does not substitute for formal and individualized diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed medical professional. Do not stop or alter your current course of treatment. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.

© 2019 Pamela Oglesby

Comments

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  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Maria, I will always remember that little boy, and he would now be a man. I wonder if his man's health improved, but from what I know it probably didnot. It is sad. I admire nurses who work in pediatrics.

    Thank you for commenting. I hope you have a peaceul day too.

    Love, Pam

  • marcoujor profile image

    Maria Jordan 

    4 weeks ago from Jeffersonville PA

    Like you, I knew early on in my career that I would be a basket case in pediatric nursing, dear Pam. I commend those nurses who are able to give heart and soul to this nursing division.

    This post is great in raising awareness - reminding me of the emotional toll that the pain of this disease can have on the child / family.

    Have a peaceful day. Love, Maria

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Linda, It really is a horrible and doning a stem cell transplant is a big procedure. I am hoping for new cures too. I appreciate of your comments.

  • AliciaC profile image

    Linda Crampton 

    4 weeks ago from British Columbia, Canada

    This can be a horrible illness. Your description of the little boy who was trying to play while he was in pain is so sad. I hope improved treatments are created soon. Thank you for raising awareness of the disease, Pamela.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Clive, I think there is a God, but life has numerous problems that we try to overcomes. This disease is awful for sure, but I am not sure that means there is no God. Thank you for your comments.

    Hi Eric, I suppose that is true for Native Ameriare Americans as well. I did not see statistics for them, but I would think they would fall into similar statistics for the Latinos. This disease is heartbreaking for anyone, that is for sure. Thank you for commenting my friend.

    Hi Ruby, Thank you so much for your kind comments, and I hope this does help some people. This is an awful disease and early intervention is very helpful for longterm health. Thanks for commenting.

  • always exploring profile image

    Ruby Jean Richert 

    4 weeks ago from Southern Illinois

    This is a terrible disease. Your info will help many. It is wonderful that you share. Blessings...

  • Ericdierker profile image

    Eric Dierker 

    4 weeks ago from Spring Valley, CA. U.S.A.

    Pamela I think the numbers must be a bit higher for Native Americans. Don't know but it seemed like it when we all got together to do chemo typed stuff together.

  • clivewilliams profile image

    Clive Williams 

    4 weeks ago from Jamaica

    sigh...I wish sometimes there was a God.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Liz, I am glad you found this to be a good explanation of SCA. Thank you for commenting.

  • Eurofile profile image

    Liz Westwood 

    4 weeks ago from UK

    I had heard of sickle cell anaemia before but did not know what the symptoms were. This is a good explanation.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Linda, Since the prcentage is higher in the black population, I think most people don't know it can happen in other populations. It is a horrendous disease. Thank you for commenting.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Bill, Yes, I hope there are more advances too. I appreciate your comments.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Eric, I am sorry to hear you lost two buddies, and high elevation with less oxygen is sure not good. I hope there are more advances in treating this horrid disease. Thank you for your kind commments.

  • Carb Diva profile image

    Linda Lum 

    4 weeks ago from Washington State, USA

    Pamela I had heard of this disease but did not have an understanding of what it is--and I was surprised to learn that it is not just black people who can have it. I hope that more research can be done for this dreadful illness. Thank you for sharing.

  • billybuc profile image

    Bill Holland 

    4 weeks ago from Olympia, WA

    You filled in gaps in my knowledge about this, so thank you! I hope modern medicine finds a way to stop this hideous disease soon.

  • Ericdierker profile image

    Eric Dierker 

    4 weeks ago from Spring Valley, CA. U.S.A.

    Pamela you have done a wonderful job here. I lost two buddies to this disease. Growing up. Super high elevation and not such good testing back then.

    They were never alone in our small town.

    I am pleased to hear of advances in care. I hope the palliative care is getting better also.

    I really appreciate the time you take to share.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Flourish, I think many people don't know much about this painful disease, and I hope new progress is made if the treatment. Thanks for your comments.

  • Pamela99 profile imageAUTHOR

    Pamela Oglesby 

    4 weeks ago from Sunny Florida

    Hi Lorna, I am glad your found the article informative, and I think many people would have been sad watching that little boy. I appreciate your comments.

  • FlourishAnyway profile image

    FlourishAnyway 

    4 weeks ago from USA

    This is really sad. I hope that more progress is made in treating, modifying the course or even curing the disease.

  • Lorna Lamon profile image

    Lorna Lamon 

    4 weeks ago

    Hi Pamela, This is a very informative article and filled in my sketchy understanding of it. I don't believe I could have kept my emotions in check when watching the little boy play - it's heartbreaking.

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