# data[].attributes.body
People with Sickle cell are expect to live around 40 to 60 years old but may increase due to advance technology. But living with it can cause your lifestyle to be a bit different. They would have to get enough sleep because they can get tired pretty easily. People with sickle cell disease have to stay away from smoking and try not to drink alcohol so much. Stay away from extreme warm and cold temperatures. It is always important to take your prescribed medication from your doctor and to get any lab tests he/she orders. Pain is very common for people with sickle cell so they can help sooth it by taking a warm bath, a heating pad, a message, physical therapy, or doing a relaxing activity.

# data[].attributes.body
Sickle cell disease is when a group of disorders affect the molecule in a cell that transfers oxygen to the cells throughout the body. This could change the cells into a sickle like shape.

Symptoms:
Sickle Cell symptoms can be seen in the early childhood and can result in shortness of breath, fatigue, and delayed growth and development in children. Early symptoms could be the child will have yellowish skin and painful swelling of the hands and feet. Yet these vary for everyone and will change over time.

# data[].attributes.body
If a person does not know whether or not they have Sickle Cell Disease they could take blood test that shows if they carry the trait. A child should be diagnosed early so it could prevent any symptoms.
Babies can also get diagnosed before they are born.

# data[].attributes.attachment_link.readable_text
An Alberta woman is the first adult in Canada to be cured of sickle cell anemia with the help of a sister's love - and a stem cell transplant.

Revée Agyepong, 26, of Edmonton was diagnosed with the disease as a toddler and has never known life without it.

"I remember as far back as elementary feeling sickle cell complications and not knowing what it was," she said.

"I thought that everyone would go out for recess and play, then come back with a terrible headache and body pain, couldn't breathe ... eventually I realized it was just me."

Sickle cell anemia is a genetic disease in which red blood cells change into a semi-circular shape and block blood vessels. Symptoms vary from patient to patient but it puts every organ in the body at risk.

Agyepong's disease manifested as chronic bone and joint pain, irregular heartbeat, kidney stones and shortness of breath.

Successfully treated in kids

Two years ago, the Alberta Children's Hospital announced it was successfully treating sickle cell disease in pediatric patients using stem cell transplants.

Agyepong said when she found out about the success of the treatment in pediatric patients, she was at a point where her health was declining and she was running out of treatment options, but doctors told her she was too old to undergo the procedure.

She was devastated.

According to Alberta Health Services, the older the patient and donor in bone marrow transplants, the greater the risk of graft versus host disease, which is a type of immune rejection that can have serious health repercussions and can even result in death.

But Stephanie Amoah, Agyepong's older sister, said she could no longer watch her sister struggle in constant pain and started to research which other centres were testing the treatment on adult patients.

"My sister has always been my protector, my guardian, she found a way," Agyepong said.

Sister was a 100% match

The sisters approached Agyepong's doctors and told them of the treatment going on in the east coast of the United States. Dr. Andrew Daly, who leads Alberta's bone marrow transplant program and oversaw Agyepong's stem cell transplant, said they had coincidentally been thinking about conducting adult stem cell transplants for sickle cell disease.

"My persistence, my sister's persistence, and they said my dedication to wanting a cure, was enough for them to just say, you know what, she's going to be the person, let's do this," Agyepong said.

Amoah was tested and was found to be a 100 per cent match for her sister. Daly said the odds of siblings being a perfect match is one in four.

The transplant, which took place at Calgary's Tom Baker Cancer Centre, involved Agyepong taking drugs to suppress her immune system and doing a course of low-dose radiation. Amoah underwent a process called apheresis that collected her sickle-cell-free stem cells, which were then given to Agyepong.

'Do the things she's always wanted'

"Over the past few months, what we've seen is that Revée's sister's bone marrow has taken over the production of Revée's red blood cells," Daly said. "The amount of sickle cell hemoglobin in her bloodstream has decreased almost to zero."

Today, Agyepong's blood tests don't show any signs of the disease, which makes her essentially cured. Amoah said the change she sees in her sister is amazing.

"It's phenomenal. I'm just so happy to have been part of a process that will give her a new life, a new hope and be able to do the things she's always wanted to do," Amoah said.

Agyepong said she is still recovering from the transplant but when she is one year out, she plans to dedicate her time to advocating for the disease, and just live her life like a normal person.

"I want to travel somewhere exotic and somewhere warm and feel dehydration like anyone else would, because I couldn't with sickle cell, I would be dehydrated and end up in the hospital," she said.

"It sounds so insignificant but it's really exciting just to be able to be more spontaneous."

# data[].attributes.attachment_link.title
Alberta woman 1st adult in Canada to be 'cured' of sickle cell anemia through stem cell transplant | CBC News

# data[].attributes.attachment_link.description
An Alberta woman is the first adult in Canada to be cured of sickle cell anemia with the help of a sister's love - and a stem cell transplant. Revée Agyepong, 26, of Edmonton was diagnosed with the disease as a toddler and has never known life without it.

# data[].attributes.body
Some treatments for Sickle Cell could be blood transfusion so they could replace the blood that have the sickle cells with RBC's. Surgery could help you to remove part of your spleen. Antibiotics like penicillin may be given from 2 months of age to 5 years. They help prevent pneumonia in sickle cell patients. Giving the patient oxygen through a mask helps so they could breath easier.

A doctor will take all sorts of lab tests like height, blood, weight to see if the child is growing like it should be. Blood and urine tests show if they are having problems like kidney problems

# data[].attributes.headline
More information on Sickle Cell Disease:

# data[].attributes.subject
More information on Sickle Cell Disease:

# data[].attributes.attachment_link.description
Sickle cell disease is an inherited disease. People with sickle cell disease have red blood cells that are sickle- or crescent-shaped, which block blood flow through the body and can cause serious problems such as pain, infections, and stroke.

# data[].attributes.attachment_link.readable_text
To use the sharing features on this page, please enable JavaScript.

Description

Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells ( anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.

The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs, such as the lungs, kidneys, spleen, and brain, of oxygen-rich blood and can lead to organ damage. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension), which can lead to heart failure. Pulmonary hypertension occurs in about 10 percent of adults with sickle cell disease.

Frequency

Sickle cell disease affects millions of people worldwide. It is most common among people whose ancestors come from Africa; Mediterranean countries such as Greece, Turkey, and Italy; the Arabian Peninsula; India; and Spanish-speaking regions in South America, Central America, and parts of the Caribbean.

Sickle cell disease is the most common inherited blood disorder in the United States, affecting an estimated 100,000 Americans. The disease is estimated to occur in 1 in 500 African Americans and 1 in 1,000 to 1,400 Hispanic Americans.

Causes

Mutations in the HBB gene cause sickle cell disease. The HBB gene provides instructions for making one part of hemoglobin. Hemoglobin consists of four protein subunits, typically, two subunits called alpha-globin and two subunits called beta-globin. The HBB gene provides instructions for making beta-globin. Various versions of beta-globin result from different mutations in the HBB gene. One particular HBB gene mutation produces an abnormal version of beta-globin known as hemoglobin S (HbS). Other mutations in the HBB gene lead to additional abnormal versions of beta-globin such as hemoglobin C (HbC) and hemoglobin E (HbE). HBB gene mutations can also result in an unusually low level of beta-globin; this abnormality is called beta thalassemia.

In people with sickle cell disease, at least one of the beta-globin subunits in hemoglobin is replaced with hemoglobin S. In sickle cell anemia (also called homozygous sickle cell disease), which is the most common form of sickle cell disease, hemoglobin S replaces both beta-globin subunits in hemoglobin. In other types of sickle cell disease, just one beta-globin subunit in hemoglobin is replaced with hemoglobin S. The other beta-globin subunit is replaced with a different abnormal variant, such as hemoglobin C. For example, people with sickle-hemoglobin C (HbSC) disease have hemoglobin molecules with hemoglobin S and hemoglobin C instead of beta-globin. If mutations that produce hemoglobin S and beta thalassemia occur together, individuals have hemoglobin S-beta thalassemia (HbSBetaThal) disease.

Abnormal versions of beta-globin can distort red blood cells into a sickle shape. The sickle-shaped red blood cells die prematurely, which can lead to anemia. Sometimes the inflexible, sickle-shaped cells get stuck in small blood vessels and can cause serious medical complications.

Inheritance

This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

Other Names for This Condition

HbS disease

hemoglobin S disease

SCD

sickle cell disorders

sickling disorder due to hemoglobin S

Additional Information & Resources

Patient Support and Advocacy Resources

Catalog of Genes and Diseases from OMIM

References

Ashley-Koch A, Yang Q, Olney RS. Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review. Am J Epidemiol. 2000 May 1;151(9):839-45. Review. Citation on PubMed

Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004 Feb 26;350(9):886-95. Citation on PubMed

Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore). 2005 Nov;84(6):363-76. Citation on PubMed

Schnog JB, Duits AJ, Muskiet FA, ten Cate H, Rojer RA, Brandjes DP. Sickle cell disease; a general overview. Neth J Med. 2004 Nov;62(10):364-74. Review. Citation on PubMed

Serjeant GR. The emerging understanding of sickle cell disease. Br J Haematol. 2001 Jan;112(1):3-18. Review. Citation on PubMed

Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004 Oct 9-15;364(9442):1343-60. Review. Citation on PubMed

Vichinsky E. New therapies in sickle cell disease. Lancet. 2002 Aug 24;360(9333):629-31. Review. Citation on PubMed

Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med. 2004 Feb 26;350(9):857-9. Citation on PubMed

MedlinePlus links to health information from the National Institutes of Health and other federal government agencies. MedlinePlus also links to health information from non-government Web sites. See our disclaimer about external links and our quality guidelines.

Genetics Home Reference has merged with MedlinePlus. Genetics Home Reference content now can be found in the "Genetics" section of MedlinePlus. Learn more

The resources on this site should not be used as a substitute for professional medical care or advice. Users with questions about a personal health condition should consult with a qualified healthcare professional.

# data[].attributes.attachment_link.title
Sickle cell disease: MedlinePlus Genetics

# data[].attributes.attachment_link.description
To use the sharing features on this page, please enable JavaScript. Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

# data[].attributes.body
https://www.webmd.com/a-to-z-guides/tc/sickle-cell-disease-topic-overview#1

https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease

http://www.hematology.org/About/History/50-Years/1534.aspx

https://www.drugs.com/cg/sickle-cell-crisis-ambulatory-care.html

https://www.cdc.gov/ncbddd/sicklecell/treatments.html

# data[].attributes.body
Sickle Cell was first discovered in the 1870s and was referred by many names, it was officially named Sickle Cell in the early 1900s. It first originated from Africa and India and spread from there. Anyone's ancestors that came from those 2 inherit the disease and will pass it on to their children.