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Hydroxyurea: Did you Know? 
Author: Kara Martina, Biopsychology B. Sc.
       
        The blood Journal describes Hydroxyurea as both a feared and an overused drug. It's praised one moment, shot down the next. What's the truth about hydroxyurea and its potential treatment for those with Sickle Cell disease? The answers aren't so simple. Given its both lauded and loathed reputation, hydroxyurea is worth knowing more about......

Did you know hydroxyurea reduces the amount of sickle cell crises?

According to WebMD, Hydroxyurea increases fetal hemoglobin production (HbF). Fetal hemoglobin is the main oxygen transport for unborn babies. HbF continues to develop until the babies are roughly six months old. But small amounts also persist throughout adult hood, especially in those with the sickle cell trait, who have a genetic predisposition to produce more of it. Fetal hemoglobin reduces the chance that red blood cells will sickle by interfering with the polymerization of the sickle cell hemoglobin and then reducing the occurrence of sickling-related complications (1). Which is why hydroxyurea is prescribed widely to adults and children alike with Sickle Cell Anemia.

Did you know hydroxyurea also kills your healthy cells?

            Hydroxyurea is classified as a chemotherapy drug. Once absorbed into the cells, hydroxyurea prevents those cells from dividing, resulting in their death. According to the American Cancer Society, chemotherapy drugs kill cells that are rapidly dividing.  But unfortunately, chemotherapy does not know the difference between the cancerous cells and the normal cells. Hydroxyurea isn’t different; it sees Sickle blood cells and normal body cells equally. It also attacks the cells in the mouth, stomach and bowel, and the hair follicles. The result can be low blood counts, mouth sores, nausea, diarrhea, and even hair loss. The side effects of Hydroxyurea and their degree depend on how much of the drug is taken, varying from person to person.

Did you know Leukemia has been linked as a potential side effect of taking hydroxurea? 

         Although not considered leukemogenic, according to the US National Library of Medicine, several studies have questioned the link between prior use of hydroxyurea and later leukemia on-set (2). According to WebMD, more research is needed on the long term effects of hydroxyurea (3).

Did you know alternatives are being developed?

         Despite its use for over 30 years, hydroxyurea’s method of action is uncertain. However, recent studies have demonstrated that hydroxyurea generates the nitric oxide (NO) radical, a chemical involved in vasodilatation. It’s been hypothesized that NO-donor properties might determine the hemoglobin phenotype expressed in the body (4). Strong evidence for a NO-derived mechanism of HbF induction by hydroxyurea suggest possibilities for therapies based on NO-releasing or NO-inducing agents. Perhaps in the future, alternatives to chemotherapy will be available to Sickle Cell patients.  

References 

1.         Alkinsheye I, Alsultan A, Solovieff N, DuyenNgo, Baldwin C, Sebastiani P, Chui D, Steinberg M (2011). Fetal hemoglobin in sickle cell anemia. BloodJournal, 118(1):19-27.

2.         Baz W, Forte F, Najfeld V, Yotsuya M, Talwar J, Terjanian T (2012). Development of Myelodysplastic Syndrome and Acute Myeloid Leukemia 15 Years after Hydroxyurea Use in a Patient with Sickle Cell Anemia. Clin Med Insights Oncol, 2012; 6: 149–152. 


 3.         Strouse JJ, et al. (2008). Hydroxyurea for sickle cell disease: A systematic review for efficacy and toxicity in children. Pediatrics, 122(6): 1332–1342.

4.         Beleslin-Cokic B, Cokic V, Gladwin M, Miller J, Njoroge J, Schechter A, Smith R (2003).  Hydroxyurea induces fetal hemoglobin by the nitric oxide–dependent activation  of soluble guanylyl cyclase. Journal of Clinical Investigation. 111(2): 231–239
Watch the Signs.
Author: Kara Martina, B. Sc. Biopsychology  
You know you need a transplant when....
Bone marrow transplants are the only known successful cure for sickle cell patients. Not without risks, this procedure can save Sickle Cell patients from reoccurring crises. Potentially able to survive for most of their lives without a BMT, several signs indicate one is necessary....

1) More and more transfusions are needed, due to the increase number of crisis moments. Too many transfusions lead to iron overload and may indicate that a better, more long-term solution is needed.  

2) Kidney failure. Because the blood is chronically low on oxygen, many organs may not receive adequate blood supply. Such organs include the kidney, liver and spleen, among others. Organ damage can be fatal, indicating that perhaps a Bone Marrow Transplant is needed to relieve stress from the oxygen-deprivation of the sickle cells (1).

3) Pulmonary hypertension. Shortness of breath and fatigue indicate a high blood pressure in the lungs. This hypertension can be fatal, and if persistent treatments fail, a Bone Marrow Transplant may help.

4) Loss of vision. Tiny blood vessels that supply your eyes can get blocked by sickle cells. Check with an ophthalmologist if your vision blurs. A Bone Marrow Transplant has the potential to prevent further damage to the eyes.

5) Priapism. Because of the nature of sickle cell blood, the penis can be stuck in a long-lasting erection, eventually damaging the vital organ, leading to impotence.  

6) An Acute chest syndrome episode. Acute chest syndrome occurs when sickle cells clump in the tiny vessels that supply the lungs. Often resembling pneumonia, ACS is accompanied by a violent cough, pain and a fever. Multiple episodes can cause permanent damage to the lungs and the vessels that supply blood, leading to pulmonary hypertension (see 3). If you've had one episode, a Bone Marrow Transplant can prevent more.  

7)Strokes. Having one stroke, a person is more likely to have a second or a third. Strokes occur when the blood sickles in the vessels that supply blood to the brain. Again, if you've had one, action needs to taken to prevent a likely second (2).

Bone Marrow transplants require adequate matches, the better the match the less potential complications. How can I donate/become a Bone marrow donor?
Simply register! To become a donor it only takes a small vial of blood or swab of cheek cells.  Individuals wishing to register can:

· Call 1-888-236-6283 (1-888-2-DONATE)
· Create an account and receive a cheek swab kit with blood.ca (create username and password)
· Contact Kyshah Powell at: [email protected]
·   Outside of North America visit here. 

 References
1. Mayo Clinic. (2014). Sickle Cell Anemia, Complications. Diseases and Conditions. Retrieved from http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/complications/con-20019348

2. John Hopkins Medicine. (2014). Sickle Cell Disease. Health Library. Retrieved from http://www.hopkinsmedicine.org/healthlibrary/conditions/hematology_and_blood_disorders/sickle_cell_disease_85,P00101/

HELP MAKE IT LEGISLATION

Support Bill C-221: An Act respecting a comprehensive National strategy for Sickle Cell Disease and Thalassemic Disorders
PETITION to the House of Commons in Parliament assembled.


The Most Important Part of the Struggle: Bill C-221/Bill 175 & Sickle Cell Disease
Author: Deleone W.J. Downes, B.A. Hons.

As readers may or may not be aware, September is Sickle Cell Awareness Month.  On September 19 & 20, 2014, the Sickle Cell Disease Association of Canada (SCDAC) together with the Canadian Haemoglobinopathy  Association (CanHaem), hosted the second annual Canadian Conference on Sickle Cell Disease, the slogan/mantra of the event being, "Learning Together, Working together toward a better future for sickle cell disease."

Much of the discussion at the conference circulated around the current private member's bill introduced by Kirsty Duncan (L-Etobicoke North (ON)) and later re-introduced by Mike Colle, MPP (Eglinton-Lawrence, Toronto (ON)).  The purpose of the bill, BIll C-221---the long form being An Act Respecting a Comprehensive National Strategy for Sickle Cell Disease and Thalassemic Disorders, and the short form being, the National Strategy for Sickle Cell Disease and Thalassemic Disorders Act---is to develop "a comprehensive national strategy for the health care of persons afflicted with sickle cell disease and thalassemia disorders," which Ms. Duncan and Mr. Colle along with supporters of the bill hope the Minister of Health will discuss with provincial and territorial health ministers.  Hopefully, this will allow for sickle cell disease information and re-education to be disseminated to health care professionals from coast to coast to coast.

At present, in Ontario, Bill 175, The Sickle Cell and Thalassemia Care Ontario Act, 2014, seeks to establish Sickle Cell and Thalassemia Care Ontario -- an organization which would come into effect if Bill C-221 is passed -- to effect the following objections / goals:
  • Seeks to establish June 19, of each year, as Sickle Cell and Thalassemia Awareness Day.
  • Redefine Sickle Cell as being a cancer of the blood, thus re-educating the Canadian medical community and public at large, allowing sickle cell to have its own medical category and subsequent treatment void of cancer related medication and/or pharmaceutical maintenance prescriptions.
  • Have Sickle Cell recognized as a Canadian disease AND a Canadian problem (Western problem).

As of March 19, 2014, the Bill's status read: First Reading Carried.

Help us support this great cause by putting heavy pressure on your local, municipal, provincial and national politicians (councillors and MPs).  Let us make this private member's bill, the law!



Works Cited

[1] http://www.sicklecelldisease.ca/advocacy/get-involved/ Web. Accessed 20 September 2014.

[2] http://openparliament.ca/bills/41-2/C-221/ Web. Accessed 20 September 2014.

[3] http://www.ontla.on.ca/web/bills/bills_detail.do?locale=en&Intranet=&BillID
=2958. Web. Accessed 20 September 2014.

More In-depth Analysis of the Relationship between Nutrition and Sickle Cell Disease 

Author: Deleone W.J. Downes, B.A. Hons.

According to NutritionMD.org, SCD sufferers require higher levels of calories and micro-nutrients, being vitamins and minerals.  As corroborated by a plethora of sources, "a diet emphasizing fruits, vegetables, whole grains, and legumes will provide a great proportion of essential vitamins and minerals absent from Western diets, coupled with supplements (one to three times the recommended intake for most essential nutrients)," thus preventing potential deficiencies in caloric intake and required minerals, vitamins and nutrients.

Moreover, NutritionMD.org states, with regards to blood levels of the above-noted vitamins and minerals in SCD sufferers such as vitamin A and carotenoids, vitamin B6, vitamin C, vitamin D, vitamin E, magnesium and zinc, can more often than not register quite low, resulting in antioxidant deficiencies, increasing the chance of SCCs or EPOCs.

However, the article goes on to state that studies have shown that vitamin-mineral supplementation (being vitamins C, E and, zinc and magnesium) and/or high-dosage antioxidant treatment, may reduce the level of sickled blood cells in an SCD sufferer's body.

Corroborated again by a plethora of sources, the article argues omega-3 fatty acids found primarily in fish oils may "improve the membranes of red blood cells," thus decreasing the advent of EPOCs requiring hospitalization.  Though the article admits this conclusion has "not yet  been confirmed in controlled trials."


Education-Osmosis: Information Takeaway
  • Drink plenty of fluids (water or juice) to reduce the advent of EPOCs and maintain healthy kidneys 
  1. avoid caffeine and soda
  • Children with SCD need more calories  than other children
  1. "may use about 20% more energy at rest than other children";
  2. maintain normal caloric intake before, during and after illness; 
  3. avoid eating too much (whether adult or adolescent).
  • Some SCD medications can cause constipation
  1. eat vegetables and grains containing fiber (beans, peas, oats and bran products).
  • Children with SCD need folate to create new blood cells.  Good sources of folate include:
  1. (alternatives) dry beans: red beans, navy beans, soybeans, lentils, chickpeas, cow peas, peanuts;
  2. Veggies being: leafy greens (spinach, cabbage, Brussel sprouts, romaine, loose leaf lettuces), peas, okra, sweet corn, beets, broccoli;
  3. Fruits being: blackberries, boysenberries, kiwifruit, oranges, plantains, strawberries, orange juice, pineapple juice.

Remember: Some of these fruits are great sources for antioxidants.

Works Cited

"Sickle Cell Disease: Nutritional
Considerations." NutritionMD.org.  Web.  20 September 2014.

University of Cincinnati, The Ohio State University, Case Western Reserve University-Consumer Health Information.  "Sickle Cell Anemia: Treatment for Sickle Cell."  10 July 2009.  Web.  20 September 2014.

"Diet & Nutrition in Sickle Cell Disease."  Web.  20 September 2014.


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Did you Know? Cold water Chlorine filled pools hurt us!!!!

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So everybody loves to jump in a pool on a hot summer day, but it might not be the best thing for you Sickle child. The Chlorine that is put in pools and hot tubs are known to start crisis' in Sickle Cell patients. Chlorine in a hot tub doesn't do as much damage because of the temperature of the water. Nonetheless your child breathing in the hot chlorine fumes may not be to entertaining. Chlorine in cold water pools cause problematic for sickle cell patient. The crisis' can range from just a normal ache and pain in the joint to full fledged chest crisis. I have witnessed this first hand on several different occasions before noticing when I put Jaiden in natural water or warm salt water from the Caribbean he has no issues. As soon as I brought him to a recreation center own pool swim no later 2 minutes Jaiden was cold and complaining of pain. By the time we got home only 15 mins Jaiden had went into a full crisis' and we had to go to the hospital where he stayed for 2 weeks. All this just because he wanted to swim. So please my fellow Sickles take head when going swimming in chlorine filled pool.
Kicking the Sick out of Sickle Cell
Author: Deleone W.J. Downes, B.A. Hons.

Misconceptions of Sickle Cell Anemia or Sickle Cell Disease
When it comes to Sickle Cell Anemia or Sickle Cell Disease (hereinafter referred to as "SCA" for the former and "SCD" for the latter, and used interchangeably), there are many misconceptions.  One, for example, is SCA only, or rather, for the most part, affects individuals of African/Caribbean descent.  This simply is not true.  According to University of Iowa Health Care (hereinafter referred to as "UIHC"), SCD 
"is the most common worldwide disease...passed through families...[and] is most commonly seen among individuals of African, Mediterranean, Caribbean and South American descent..." [1].  More specifically, "the sickle cell trait  is most common in people whose ancestors lived in malarial regions - not only Africa and South America, but the Mediterranean, the Middle East, and Southeast Asia." [2]

Another significant and common misconception is SCA must only or can only be treated (illness management) with pharmaceutical drugs.

Nutrition-Based Illness Management
SCA sufferers, as part of illness management, can increase their quality of life through reducing episodes of crises (also known as "sickle cell crises" hereinafter referred to as "SCC" or "EPOCs") through healthy living - specifically, a healthy nutrition.  Before we discuss the and end on a good note with the positive symptoms of SCD, let us discuss the negative symptoms of the same.

Factors Which Increase the Probability of EPOCs
According to "Keeping Well with Sickle Cell Disease.," [3] the following can increase your chances of experiencing an SCC, quote:
  1. Not having enough water in the body (dehydration).  Drink plenty of fluids (3-4 liters of water a day); avoid caffeine as it constricts blood vessels.
  2. Not eating enough food. Maintain a balanced diet [3].  Your nutrition should include "adequate calories, protein, fats, and vitamins and minerals (perhaps supplements of the latter two). Existence of studies suggesting omega-three fatty acids (fish oils and other types of healthy oils, and some found in dietary supplements) "make red blood cell membranes less fragile," thus potentially decreasing the advent/chance of sickling red blood cells; however; there is no conclusive evidence to support this stance. [4]
  3. Not taking recommended medications and vaccinations.  There is some disagreement regarding the former, however, the latter is discussed below.
  4. Being too cold or too hot.  Extreme or sudden/drastic changes in the weather/in temperature.
  5. Excessive physical exertion.  According to "Sickle Cell Anemia Treatment & Management," "patients may not be able to tolerate vigorous exercise," further suggesting restrictions for certain SCA suffers depending on their current physical condition(s); and/or, as above, drinking plenty of fluids. [5]
  6. Emotional Stress and Anxiety.  Light exercise coupled with healthy, nutritious food-intake can often play a significant role in the lives of individuals suffering from mental illness.  Exercise, however light, releases "feel good factor" chemicals throughout the body and the brain, which compliments a healthy food intake thus potentially turning that frown upside down.
  7. Infection(s).  Take precautions (i.e. Vaccinations) if you are travelling. [3]

Just Maintain a Healthy Food Intake: Could it Really be that Simple?
Despite the fact that there is no known cure for SCD, a healthy, balanced food intake based on excellent food choice can significantly reduce or prevent EPOCs. [1]  This is corroborated by various sources and studies which one can locate online.  Many sources suggest speaking to either a dietitian or nutritionist in order for the same to recommend a food-plan of sorts. The National Heart, Lung, and Blood Institute (hereinafter referred to as "NHLBI") suggests the following:


"A healthy diet that is low in saturated and trans-fats, sodium, sugar and dietary cholesterol.  Some healthy food options include various fruits, vegetables and whole grains, fresh fruits and vegetables daily for individuals with sickle cell anemia.  Fruits and vegetables such as berries, citrus fruits, melons and green leafy vegetables are significant sources of essential vitamins, minerals, antioxidants and dietary fiber.  Low-fat dairy products, legumes and lean meats such as poultry, beef and fish are some other healthy food options that provide protein necessary for good health.  In addition to healthy foods, individuals with sickle cell anemia should consume at least eight (8) glasses of water daily to prevent dehydration." [1]
Works Cited

[1] Palacios, Anthony I.  “Sickle Cell Diet.”  Livestrong.com. 24 October 2013.  Web.  16 September 2014.

[2] Charles, Katie.  “Daily Checkup: Sickle Cell Disease is a hereditary, life-shortening ailment.”  New York Daily News.  16 September 2014.

[3] Brent Sickle Cell & Thalassaemia Center.  “Keeping Well with Sickle Cell Disease.”  Web.  16 September 2014.

[4] University of Maryland Medical Center.  “Sickle Cell Disease.”  27 June 2013. Web. 16 September 2014.

[5] Maakaron, Joseph E.  “Sickle Cell Anemia Treatment & Management.” Medscape.  2 September 2014. Web. 16 September 2014.
The Lymph System
~How the Lymph System is Affected by Sickle Cell Disease~

Author: Kara Martina, B. Sc. Biopsychology

            Responsible for the drainage of the body, the Lymph system provides a pathway for fats and fat soluble vitamins. It returns proteins and tissue fluids back to the blood, also helping to defend the body by transporting white blood cells.   

            The word Lymphatic derives from the Latin word lymphaticus, meaning "connected to water," named so because of its clear appearance. It consists of lymph vessels, lymph nodes, lymph fluid, the tonsils, adenoids, spleen and thymus. Incorporating 600 to 700 lymph nodes in the human body, the lymph system filters the lymph before it returns to the circulatory system (1).
Although many diseases and complications can affect and arise in the Lymph system, those with Sickle Cell Disease typically suffer from those associated with the spleen.

The Spleen:  Acts as a filter where old red blood cells are recycled. Also the spleen produces white blood cells. Although having a spleen helps fight infections, the production of lymphocytes and platelets can be overtaken by the bone marrow and the recycling function can be overtaken by the liver. 

Sickle Cell Complications Include:

Acute splenic sequestration (ASSC):  Sickle cells clump in the spleen causing enlargement and resulting in a reduction of hemoglobin circulation throughout the body (2).

Oxygen deprivation of the Spleen: Sickling of red blood cells can keep the spleen from receiving adequate blood supply, eventually leading to its dysfunction.
Treatments:

Transfusions: Remove the build up in the spleen, increase hemoglobin circulation 

Splenectomy: Removal of the spleen or partial removal of the spleen. The University of Maryland Medical Center notes that most children with sickle cell anemia undergo surgical removal of the spleen or do not have a functioning spleen by the age of eight years (3).

Education: Early detection and sign of an enlarged spleen help coordinate proper treatments. Parents are encouraged to self-examine the spleen and bring their child in for evaluation if the spleen enlarges (4).

Antibiotics: After a spleen removal, infection is common and easier to catch. For this reason, prior immunization and vaccinations are needed as well as extra antibiotics to keep the body up to date. Common threats include: Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenza, bacteria that cause severe pneumonia, and meningitis (5).

References

1.         Kim Ann Zimmermann. (2013). Lymphatic System: Facts, Functions & Diseases. Live Science. Retrieved August 22, 2014, from http://www.livescience.com/26983-lymphatic-system.html

2.         A.D.A.M. (2014). Sickle Cell Disease. Penn State Hershey. Retrieved August 23, 2014 from http://pennstatehershey.adam.com/content.aspx?productId=10&pid=10&gid=000058

3.         Noreen Kassem. (2011). Systems of the Body Affected by Sickle Cell Anemia. Live Strong.com. Retrieved August 23, 2014 from http://www.livestrong.com/article/128264-systems-body-affected-sickle-cell/

4.         Mayo Clinic Staff. (2013). Enlarged Spleen. Mayo Clinic. Retrieved August 23, 2014 from http://www.mayoclinic.org/diseases-conditions/enlarged-spleen/basics/tests-diagnosis/con-20029324

5.          David T. Derrer, MD. (2014). Splenectomy. WebMD. Retrieved August 23, 2014 from http://www.webmd.com/digestive-disorders/splenectomy?page=3