What IS

Sickle cell anemia?

Care and Treatments

Pain in a person with sickle cell disease, because it will recur more often than not, must be treated quickly and effectively.

Phenylketonuria (PKU), Hemophilia, Tay-Sachs, and Muscular Dystrophy. Repeated pain, by the way it is experienced, will have an impact on the psychological and physical development of the child and on the way he feels the painful crises that will occur again and again.

AN INCURABLE DISEASE

Currently sickle cell anemia is incurable, but proper medical care can prevent its effects.

Drink lots of fluids
Tylenol (acetaminophen)
Oral and intravenous drugs
Daily penicillin in children two months to five years of age
Blood transfusions
Hydroxyurea treatment
Daily folic acid intake
Bone marrow transplants, etc.

DETECTION OF FALCIFORM ANEMIA

Most states in the United States and some hospitals in Canada now have a simple blood test automatically performed on all at-risk newborns. This test is done simultaneously and with the same blood samples as other routine newborn tests. The electrophoresis of hemoglobin “hemoglobin electrophoresis” is the most used. Routine prenatal screening is already done in Ontario. If the results show the presence of sickle hemoglobin, a second blood test will be administered to confirm the diagnosis. These tests can also show whether the child carries the gene for the disease.

NEW HYDROXYUREA DRUGS

Hydroxyurea (capsule) is a medicine that was originally used to treat certain forms of blood cancer (leukemia) and blood disorders that can develop into leukemia. For over 15 years, hydroxyurea has now been used to treat sickle cell anemia.

Hydroxyurea works in a number of ways, the main one being by increasing the production of fetal (baby) hemoglobin in the body. We know that this then provides protection against sickle cell anemia. This medication is safe and there is no reason to believe that its use could increase the risk of cancer in patients with sickle cell anemia. Like any other medicine, it can cause some side effects and the patient should monitor their blood pressure closely. In addition, unlike pain relievers that the patient must take as needed, hydroxyurea must be taken every day for it to be effective.

Hydroxyurea is the only drug available to treat sickle cell anemia and has been shown to be effective in prolonging survival, decreasing pain episodes and reducing certain complications. Unless you have been confirmed by a specialist that your sickle cell anemia is very mild, you should consider switching to this medicine.

WHEN IS HYDROXYUREA AVAILABLE TO THE PATIENT?

The patient is usually offered hydroxyurea when they have frequent episodes of pain or acute chest syndrome. This medication may also be offered if there is concern that sickle cell anemia could damage any organ, or as an alternative treatment to blood transfusions.

BLOOD TRANSFUSION

Blood transfusions for the treatment of sickle cell anemia can be done in 3 ways. The easiest method is to simply transfuse units of blood or what is called a “top-up” transfusion. This improves the supply of oxygen in the body. The more complex method is to receive blood by “automated exchange of red blood cells” (erythrocytopheresis). This requires connecting the patient to a machine that automatically filters the sickle red blood cells and replaces them with new normal red blood cells. The third method is “partially manual exchange transfusion” whereby the nurse withdraws 2 units of blood and gives a 2-unit transfusion.

Each of these 3 methods offer advantages and disadvantages and are suitable for different reasons.

Transfusions are most often given to patients who have had or are at high risk for a stroke. They can also be given to patients in severe crisis, such as acute chest syndrome, or if the patient is very ill and needs to be admitted to the intensive care department. The patient may need a transfusion if they are very anemic (aplastic crisis), which is usually related to a viral infection (cough or cold). It may be appropriate to give a transfusion if the patient is pregnant and has complications, or if the patient needs major surgery.

PHENOTYPIC BLOOD

Phenotypic compatible blood means that the blood has been matched as closely as possible to the patient’s blood group. This reduces the risk of a reaction to the transfusion and the development of an antibody. However, since most blood donors have a different ethnic background from sickle cell anemia patients, the compatible blood supply is limited.

HOW CAN WE HELP TO INCREASE THE PHENOTYPIC BLOOD RESERVE?

Canadian Blood Services (CBS / CBS) and Héma-Québec would like to be able to count more donors from ethnic minorities. If your friends or family want to help people with sickle cell anemia, becoming a blood donor is a very practical, very useful and very rewarding way to help. If you would like to organize a blood drive event or session, Héma-Québec and the CBS / CBS can help you do it. However, you cannot donate blood if you have sickle cell anemia.

COMPREHENSIVE CARE

Sickle cell anemia is a lifelong condition with symptoms and complications that vary greatly from one person to another, and from one period of life to another in the same person who has it. Currently, hematopoietic stem cell transplantation is the only effective treatment method, but it is available to a small minority of people, due in part to the lack of suitable donors.

In babies with sickle cell anemia, serious health complications can occur as little as a few months after birth, such as life-threatening infections, vaso-occlusive crises, etc., for which it is essential to obtain the corrective information. care from specialized medical teams. Parents of these babies must be educated to be able to anticipate these complications, so that appropriate measures can be taken quickly to ensure the safety and optimal development of their children. The onset of adolescence is a great time for these patients to learn in detail the nature of their own health condition, so they can learn how to stay healthy and how to deal with it themselves. The transmission of this knowledge is crucial, in addition to respecting their regular clinical appointments. Adolescents and young adults face many challenges as the person with Sickle Cell Disease must demonstrate greater independence under the pressures of both school and work. It is essential for these people to have a healthy self-esteem and to be motivated to stay healthy. Aging inevitably leads to concern since, in addition to the process of normal wear and tear, sickle cell anemia can cause premature damage to organs. To respond to these various concerns, recent research carried out in this sector must be supported by the services of specialized teams. During these crucial stages of life, people with sickle cell disease have distinct physical and psychosocial needs from those without sickle cell disease. These patients require comprehensive care from specialized medical centers that are truly able to meet their specific needs.

WHAT IS COMPREHENSIVE CARE?

Comprehensive care is not only required for the management of sickle cell anemia, but it greatly relates to the importance of preventing its complications and creating a healthy lifestyle. This care affects both physical and emotional health at various stages of life and is provided to people with sickle cell anemia according to their family context, life events and their environment.

The quintessential comprehensive care team should include hematologists and specialist nurses with the expertise and interest in caring for patients with sickle cell anemia. The services of medico-social workers and psychologists are also needed to help patients cope with their psychosocial challenges. Support from specialist medical personnel, such as emergency department physicians, pain management physicians, intensive care personnel, pediatricians, interns, laboratory and blood transfusion physicians, medical geneticists and obstetricians, is absolutely essential at different times.

The specialist medical center is expected to be on duty 24 hours a day, 7 days a week, to treat patients with serious problems or to remotely offer real-time medical advice to patients presenting to their local hospital. In addition, by providing services to patients and their families, such as information, counseling, advocacy, support groups and other special programs, this will help improve the quality of life for patients and their families. families.

Until a way is found to cure all sickle cell anemia patients, providing the best comprehensive care to these people will allow them to achieve the best clinical results and a good quality of life.

Acknowledgment: Certain information on this page is courtesy of: John K. Wu, MBBS, MSc, FRCP (C)
British Columbia Children’s Hospital and British Columbia Women’s Hospital & Health Center