Children living with Sickle Cell disease face painful complications associated with the disorder; however, in no way is the children sufferings comparable to the likes of what the adult patients go through.
Adult patients face more problems far beyond the complications of the disease. Many experience difficulties in the area of job retention, finance, marriage, and housing. Over time, these snags may lead to low-self-esteem and frustrations around under-achievement. These life complications, coupled with perceived under-responsiveness of hospital staff to special needs, may trigger antagonism towards the staff members and result in increased negative life experiences.
Consensus Statement on SCD (CSS)
In an attempt to improve patient experiences around care and treatments, SCDAC/AAFC accepts the CSS document produced by the Canadian Hemoglobinopathy Association as the best practice information and the Canadian clinical guidelines for care and treatment of SCD. It is pleased to disseminate the CSS to all centers of excellence across the country.
For most patients, among other outcomes, the consensus statement would eliminate most feelings of mistrust towards hospital staff members. Most importantly, it would enhance the quality of care received, improve self-esteem, confidence and the quality of life of patients. It will also promote adherence to uniform standard of care across the country.
Hydroxyurea is a drug (capsule) that was originally used to treat some forms of blood cancer (Leukemia) and blood disorders that can later turn into Leukemia. It has been used for over 15 years to treat Sickle Cell Disease.
Hydroxyurea works in a few different ways, but the main way is to increase your body’s production of Fetal (baby) Hemoglobin. We know that this then protects you against Sickle Cell Disease. It is a safe drug and there is no evidence to suggest increases the risk of cancer in patients with Sickle Cell Disease. Like all drugs, it does have some side effects, and you need regular blood test monitoring. Also, unlike pain killers which you take when required, Hydroxyurea must be taken every day to be effective.
Hydroxyurea is the only drug therapy available for Sickle Cell Disease and has been shown to prolong survival, reduce pain episodes and reduce some of the complications. Unless you have been told by a specialist that you have very mild Sickle Cell Disease, you should consider going on the drug.
Why am I being offered Hydroxyurea?
You are most likely to be offered Hydroxyurea because you have lots of painful crisis or Acute Chest Syndrome. It may also be offered if there is concern about damage to any of your body organs from the Sickle Cell Disease, or as an alternative to blood transfusion treatment
Blood transfusions in Sickle Cell Disease can be carried out in 1 of 3 ways. The easiest way is to simply transfuse units of blood, or a “top-up transfusion”. This improves the supply of oxygen to your body. The most complex way of receiving blood is by “automated red cell exchange” (erythrocytopheresis). This involves hooking you up to a machine that automatically filters of the sickled Red Blood Cells and replaces it with new, normal Red Blood Cells. The alternative to these, is a “partial manual exchange transfusion”, whereby a nurse will remove 2 units of blood and then give you a 2 unit transfusion.
These 3 methods all have advantages and disadvantages and are suitable for different reasons.
Transfusions are most commonly given for patients who have had a stroke or are found to be at high risk of having a stroke. It can also be given for a severe crisis, such as Acute Chest Syndrome or if you are very sick and need to be cared for in Intensive Care. You may need a blood transfusion if you are very anemic (aplastic crisis), which is usually due to a viral infection (cough or cold). It may also be appropriate to give you a transfusion if you are pregnant and there are complications, or if you are planning on having major surgery.
Phenotypic matched blood means that the blood has been matched to your blood group as closely as possible. This reduces the chance of having a transfusion reaction and of developing an Antibody to the blood. However, because most blood donors are from ethnic backgrounds different to Sickle Cell Disease patients, there is a limited supply of suitable blood.
How can I help increase phenotypical blood reserve?
The Canadian Blood Service (CBS) and Hema-Quebec would like more blood donors from the ethnic minorities. If you have friends or family who want to help people with Sickle Cell Disease, becoming a blood donor, is a very practical, valuable and worthwhile way to help. If you would like to arrange a blood donor drive or session, the Hema- Quebec and the CBS can help with this. You cannot donate blood if you have Sickle Cell Disease
Blood Strategy: 2016-2018
Phenotypic Blood for all Canadians with SCD.
According to Canadian Blood Services, people who were born in or have lived in certain Central and West African countries since 1977, may have been exposed to a new strain of the virus that causes AIDS (HIV-I Group O), and are not eligible to donate blood. People who have received a blood transfusion while visiting there or who have had sex with someone that has lived there, are also not permitted to donate blood. This is not based on race or ethnicity but possible exposure to HIV-I Group O. Countries included are: Cameroon, Central African Republic, Chad, Congo, Equatorial Guinea, Gabon, Niger and Nigeria.
Majority of blood donors are Caucasians while many of the individuals living with Sickle Cell Disease in Canada include those from the African countries listed above. With Sickle cell disease, many patients are on continuous blood transfusion and need phenotype blood from their genetic community.
Phenotype Blood & Sickle Cell Disease
Due to the difference in the antigens of the Caucasians and the African-Canadians; there is a formation of alloantibodies making it impossible to locate compatible red cell units. When treating individuals living with Sickle Cell Disease, it is no longer sufficient to transfuse them with blood matched by blood groups alone as adequate research has shown that there is a great reduction in transfusion complications when phenotypically matched blood is given to these patients.
The permanent deferral policy continues to make blood donation drives for phenotype blood increasingly difficult.
The Good News:
We are pleased to submit that Health Canada has relaxed the deferral policy from permanent deferral to 12 months for those who have lived in or travelled to the African countries in question. SCDAC/AAFC continues to work on the clause around the spouses/sexual partners of these donors. Please read the response from Health Canada.
Communications with Blood Operators
Every time you donate blood in any part of the country, your donation can now be tracked and attributed to Sickle Cell Disease.
All you need to do is register online as PFL Member at: https://blood.ca/en/blood/join-your-organization
Once you register at Canadian Blood Services (CBS) website, also register below.
Sickle cell disease is a life-long condition whose symptoms and complications have considerable variability among the affected individuals – as well as during different times in the life of a particular affected individual. Currently, successful hematopoietic stem cell transplantation is the only cure but is only available for a small minority of individuals due to the lack of suitable donors and other reasons.
For infants with sickle cell disease, serious health complications can arise within a few months after birth such as life threatening infections, vasoocclusive crises, etc., which makes early involvement by expert medical teams essential. Their parents need to be educated to anticipate complications so that prompt and appropriate actions can be taken to ensure the safety and optimal development of these children. Early adolescence is the optimal time for the individuals to learn about the detail nature of their own condition, to be aware of how to stay healthy and to know how to advocate for themselves.
The nurturing of this knowledge is critical in addition to their regular scheduled clinic appointments. Adolescence and young adult period can be challenging times as the person with sickle cell disease achieve greater independence in the midst of peer pressure in schools and in their vocations. The building of a healthy self-image with motivation to maintain good health is paramount. Aging inevitably brings with it concerns with sickle cell related premature organ damage on top of the normal wear and tear processes. Recent research in this area together with the help of expert teams is needed to deal with the various concerns. Throughout these critical stages in life, persons with sickle cell disease have distinct physical and psychosocial needs that are different from the unaffected population at large. They will require comprehensive health care through expert medical centers that can address their diverse health needs.
So what is comprehensive care?
Comprehensive care deals not only with management of sickle cell disease, but also places a great deal of importance in the prevention of its complications and the creation of a healthy life outcome. It addresses both the physical and emotional health at all stages of life, and provides care to the persons with sickle cell disease in the context of his (her) family, life events and environment.
The optimal core comprehensive care team should include hematologists and specialty nurses with expertise and interest in sickle cell disease. Medical social workers and psychologists are needed to assist with psychosocial challenges. The support of medical experts such as Emergency room physicians, Pain management physicians, Intensivists, Pediatricians, Internists, Laboratory and Transfusion medicine physicians, Medical geneticists, Obstetricians are indispensible at different times. It will be importance for the core medical team to keep these supporting physicians updated with the newest knowledge in the field, and to ensure close communication when a patient is seen.
The expert medical center should have 24/7 coverage to attend to patients with acute medical problems, or be able to offer real-time medical advice at a distance should patients show up their local hospitals. In addition, the provision of services for patients and families, including education, counseling, support groups, advocacy, and other special programs will help to improve the quality of life of patients and their families.
The Cost of Health Care in Canada
Without comprehensive care plan for sickle cell disease; the cost of health care continues to rise especially with the increasing number of the affected across all provinces.
The cost may be divided into human and economic costs:
The human cost for sickle cell disease is unquantifiable as many lives are needlessly lost. The age group most vulnerable are the 16-35 year olds.
Many individuals with sickle cell disease are highly educated, skilled and willing to contribute to the economic growth of their community. While a few are able to, many more due to lack of comprehensive and proper treatments are not able to. This results, many a times in preventable complications and untimely deaths.
We have provided below a conservative economic cost based on non-ICU care Canadian estimates. ICU care would increase cost astronomically.
It is important to note that:
Until a cure is achieved for everyone affected with sickle cell disease, the offer of optimal comprehensive care to these individuals will enable them to have the best clinical outcome, and a good quality of life.
Acknowledgement: Some of the information on this page was provided by:
John K. Wu, MBBS, MSc, FRCP(C)
British Columbia Children's Hospital and British Columbia Women's Hospital & Health Centre
The Cost of Health Care in Canada
Long considered a rare disease within Canada, sickle cell disease is in fact the most common genetic disease in the world, with an estimated 100 million affected individuals world-wide and another 275,000 babies with SCD born annually1. The number of affected Canadians numbers in the thousands and additional 100 or more children with the condition are born in this country each year.
What is Sickle Cell Disease?
Sickle cell disease is an inherited condition that results in an abnormality of haemoglobin, the substance that carries oxygen within red blood cells. This abnormal haemoglobin can lead to red blood cell deformation and break down, increased blood viscosity or “sludginess”, and both acute and chronic organ dysfunction. Typical manifestations in patients with sickle cell disease include:
• Severe pain episodes due to bone marrow necrosis
• Susceptibility to infections
• Increased risk of stroke and vision loss
• Need for frequent blood transfusions
• Reduced life expectancy of approximately 30 years compared to normal population.2
Because sickle cell disease is considered the quintessential “multi-system disease”, patients often require comprehensive care to prevent and manage complications.3
Sickle Cell Disease in Canada
Because sickle cell gene mutation emerged in response to malaria, individuals with ethnic backgrounds associated with equatorial regions have a greater likelihood of carrying at lease one copy of the genetic mutation (a condition known as being sickle cell trait or being a sickle cell carrier), if not the disease itself. These individuals therefore tend to be of African, Middle Eastern origin or from the Indian subcontinent (India is second only to Nigeria), but due to centuries of inter-marriage and emigration, individuals of virtually any ethnic background may be affected. Universal newborn screening for children with sickle cell disease will soon be available in every province, but Canada continues to lag behind the United States in its lack of a national strategy for managing this condition.
According to Dr. Jacob Pendergrast, chair of the Sickle Cell Disease Association of Canada’s Medical Advisory Board, “A National Framework would help ensure that all patients in Canada with sickle cell disease receive evidence-based, comprehensive care. This would not only ensure that patients born with this condition live longer and healthier lives, but would also save the health care system substantial costs by shifting the medical focus from inpatient treatment of emergencies to outpatient preventative care.”
Furthermore, the president of the Association, Ms. Lanre Tunji-Ajayi notes that many patients have reported an apparent lack of familiarity with sickle cell disease amongst the physicians who treat them. In many instances this results in delayed and incomplete care, but occasionally patients experience life-threatening complications and even death due to failure to provide appropriate management. “In a country as well-resourced and diverse as Canada, this is simply unacceptable. Young adults with this disease are dying at a time when other countries with comparable populations are having fewer or no deaths due to the provision of comprehensive care programs. Over the last 10 years, repeated pleas for improved quality of care for patients with sickle cell disease have been sent to hospitals, regional health authorities and Ministries of Health by various sickle cell organizations across the country. While these pleas have been met with sympathetic responses, there has been little actual mobilization of resources.” Continued Ms. Tunji-Ajayi, ”It would be extremely beneficial to the patient community and save the health care system considerable expense long term if more comprehensive clinics for Sickle Cell Disease could be established in strategic locations across the country. This would allow more patients to attend regular clinics and stay healthier longer.”
Cost of Sickle Cell Disease in Canada:
The lifetime costs for a patient with sickle cell disease have been estimated at $9 million4. As there are approximately 5 000 patients with sickle cell disease in Canada, the total costs to their health care system may be as high as $4.5 billion6. Much of this cost reflects the frequent hospitalizations required by patients with sickle cell disease when they are denied comprehensive, preventative care: a one-week non-ICU hospitalization, for example, costs $20 000 on average, and patients with sickle cell disease may require hospitalizations several times per year if they are not treated with inexpensive medical interventions such as vaccinations and the medication hydroxyurea6. The human cost of sickle cell disease, on the other hand, both to the patient and their family, is unquantifiable, and includes missed school days and employment opportunities, social isolation and stigmatization, psychological strain and sometimes, preventable, untimely deaths.
It is worthy of note that in Canada:
About Sickle Cell Disease Association of Canada/Association d’Anémie Falciforme du Canada (SCDAC/AAFC):
Established in 2012; it is its mandate to ensure among other purposes that every Canadian living with Sickle Cell Disease has access to comprehensive care and treatment. To this end, SCDAC/AAFC is actively engaging different ministries of health across the country.
The Need for a National Strategy for Sickle Cell Disease (SCD) in Canada
A national strategy will reduce the health care cost associated with sickle cell disease and improve the quality of care received by the approximately 5 000 individuals living with the disease in Canada. As such, our Medical Advisory Board is willing to lend technical expertise to the federal, provincial and the territorial ministries of health in moving forward with the planning and implementation of a national strategy and has provided an extensive report on the need for this strategy based on SCDAC/AAFC’s three “Asks” identified in this document.
SCDAC/AAFC is seeking federal leadership and funding in cooperation with the provinces and territories for the three key asks to bring about:
1. Implementation of universal newborn screening program for haemoglobin disorder in all provinces and territories
2. Establishment of a Canadian network of Comprehensive treatment centres, and
3. Creation of a national patient registry.
Edmonton Adult program:
Centre for Rare Blood Disorders
3D.151, 11400 University Ave
Kaye Edmonton Clinic
EDMONTON, AB T6G 1Z1