Acute And Chronic Transfusion Therapy
Family physicians should request consultation with a hematologist and a blood bank physician when considering transfusions for patients with sickle cell disease. In general, transfusions should not be used for uncomplicated, acute, painful episodes minor surgical procedures chronic anemia without symptoms or uncomplicated pregnancies. As described earlier, transfusion therapy often is of great benefit. The risks of transfusion therapy, whether acute or chronic, need to be managed carefully to avoid adverse events, because the end points for transfusion in patients with sickle cell disease differ from the end points in other patients.
Exchange transfusion may be necessary as an alternative to simple transfusion to avoid hyperviscosity and volume overload. The blood bank should be notified immediately of the possible need for transfusion because patients with sickle cell disease have special needs for matching red blood cell antigens, and they are at increased risk for transfusion reactions because of sensitization by previous transfusions. Delayed hemolytic transfusion reactions may be difficult to recognize because the symptoms may overlap those of a severe painful episode. Finally, patients who are receiving chronic transfusions must be monitored for iron overload that ultimately will require chelation therapy. Table 418,28,29,32,37â45 provides some major indications for transfusion therapy.
|Prevention of recurrent splenic sequestration|
Strengths And Limitations Of This Study
This systematic review will include both published and unpublished literature, hence reducing the risk of publication bias.
Duplicate and independent screening and data extraction will minimise the risk of error when identifying eligible studies and extracting relevant data.
This review will include non-randomised studies which tend to overestimate the efficacy of an intervention and are prone to selection bias.
Plans For Future Shielding
Given the improved protections against the virus , and the knownharms to physical and mental wellbeing that shielding can cause, the current clinical advice is that itis not appropriate to reintroduce shielding.
Those in the clinically extremely vulnerable group are advised to follow the national restrictions and guidance.
You can find more details about the national restrictions here: www.gov.uk/government/publications/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19
Some of this guidance is specific to England, you can find more specific guidance for Wales, Scotland and Northern Ireland here:
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Stem Cell Or Bone Marrow Transplants
Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they’re not done very often because of the significant risks involved.
Stem cells are special cells produced by bone marrow, a spongy tissue found in the centre of some bones. They can turn into different types of blood cells.
For a stem cell transplant, stem cells from a healthy donor are given through a drip into a vein.
These cells then start to produce healthy red blood cells to replace the sickle cells.
A stem cell transplant is an intensive treatment that carries a number of risks.
The main risk is graft versus host disease, a life-threatening problem where the transplanted cells start to attack the other cells in your body.
Stem cell transplants are generally only considered in children with sickle cell disease who have severe symptoms that have not responded to other treatments, when the long-term benefits of a transplant are thought to outweigh the possible risks.
How Can I Help My Child
To help your child manage sickle cell disease:
- Go to all doctor’s visits and share any concerns or new symptoms.
- Make sure your child takes all prescribed medicines.
- Follow up with any recommended specialists to check for complications.
- Help your child avoid pain crisis triggers, such as extreme temperatures or stress.
- Talk to the doctor about which activities are OK for your child and which to avoid.
- Make it clear that your child should not smoke, drink alcohol, or use drugs because these can cause pain and other problems.
- Encourage your child to drink lots of liquids and get enough rest.
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Why Are Immunizations So Important For Children With Sickle Cell Disease
Childhood survival rates have greatly increased with universal use of pneumococcal and other routine vaccines. The first pneumococcal vaccine decreased the rate of pneumococcus infection in children under 3 years old by more than 90 percent.2,3
Vaccinations work by helping the immune system develop protection from a specific disease. They are safe and effective ways to prevent many infectious diseases for everyone. They are even more important for people with increased vulnerability to infections, such as people with SCD.
Vaccination schedules are also easier to follow than twice-daily penicillin, but are not a replacement for penicillin. Children with SCD should receive all routine immunizations, as well as special schedules for additional immunizations.3,4
The Centers for Disease Control and Prevention recommends an immunization schedule based on how childrens immune systems respond to vaccines and when they may be exposed to diseases. It also adapts the schedule for people with chronic conditions. Continued education helps parents and caregivers get children with SCD their vaccinations on time.5,6
Medicine For Sickle Cell Pain
If you continue to have episodes of pain, a medicine called hydroxycarbamide may be recommended. You usually take it as a capsule once a day.
Hydroxycarbamide can lower the amount of other blood cells, such as white blood cells and platelets , so you’ll usually have regular blood tests to monitor your health.
If you have regular episodes of pain, you may also be able to have a medicine called crizanlizumab. Crizanlizumab can be taken on its own or alongside hydroxycarbamide. You will usually be given crizanlizumab by injection into a vein every 4 weeks.
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Taking Medicines During The Covid
If you are taking hydroxycarbamide or iron chelators it is important these are continued. Your centre of care will make arrangements to monitor this treatment through the hospital or your GP. There is no evidence these drugs affect the risk of COVID-19 one way or the other. However there is some concern that anti-inflammation pain killers like ibuprofen might make coronavirus infection worse. During the COVID-19 outbreak it is recommended that you take paracetamol which is safe instead.
Where Do You Stand Right Now In Terms Of Getting The Covid
When the pandemic first started, I strictly quarantined with my immediate family. Professionally, I am a dancer and model so I am used to being around different groups of people often but that quickly changed. From March 2020 to February 2021, I only spent time at my house and my parents home, therefore, I view myself as low risk for COVID-19. More recently, my life has resumed normal activity, I recently started booking gigs again and socializing with friends. However, strict safety measures are in place whenever interacting with others.
As of right now, I am still not interested in getting the vaccine. Personally, I want to wait for more information, mainly due to my personal history with triggered pain crisis due to vaccine symptoms. I am also hesitant due to the time frame of knowledge since the vaccine release. I have always been a reserved and patient person, so I tend to wait a few years even when a new phone comes out before getting it. I remember when the PS4 came out and I wanted to buy one right away, my dad told me to wait three or four years, because by then there would be a refined version out with way more games. I think thats where I received that approach to trying new things from, and why I would rather wait to have information that can only be received through an observation of time.
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For Clinically Extremely Vulnerable From Covid
For people who fall into the clinically extremely vulnerable category , you should first look at the general guidance found above and any specific guidance for your area.
Information on advice for the clinically extremely vulnerable group can be found here: www.gov.uk/government/publications/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19
Get Medical Advice From A Genetic Expert
We’ve partnered with Genome Medical to provide you with access to trained and licensed genetic experts in all 50 states. These experts are ready to meet with you one-on-one and answer any questions you might have. Genome Medical is a nationwide medical practice focused on genetics and genomics. Their goal is to make it easier for people to access genetic experts and get the information they need to make informed decisions about their genetic health. To find out more about our partnership, . If you’re located outside of the United States, .
Consultations are available anywhere in the U.S. by phone or video. During a consultation, the genetic counselor will answer your questions and take you through a comprehensive discussion to determine what steps and/or genetic tests would be appropriate for you. The cost of the consultation will vary, depending on whether an insurance claim is submitted for the service. You can make an appointment over the phone, or through an online process.
If you’d prefer, you can also submit questions to a Genetic Counselor by email. This service is provided by ThinkGenetic and is free.
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Dr Wally Smith Addresses The Impact Of Covid
1/21/2022 12:00:00 AM
Sickle cell disease is a chronic blood disorder that in the United States mainly affects the Black community. If you have sickle cell disease, your immune system is weaker than those of other people. Does this mean its easier for you to get COVID-19? Will your symptoms will be worse?
Dr. Wally R. Smithisthe Florence Neal Cooper Smith Professor of Sickle Cell Disease at VCU and medical director of the adult sickle cell program at VCU Health. Here he addresses concerns you may have about COVID-19 if you or your child has sickle cell disease.
Whats It Like To Live With Sickle Cell Anemia
New treatments are helping people with sickle cell anemia live longer and with better quality of life. The U.S. Centers for Disease Control and Prevention has the following suggestions for living well with sickle cell anemia:
Living with medical complications
Sickle cell anemia is a chronic illness with symptoms that change over time. For example, children and adults have to cope with pain from acute chest syndrome or VOCs. Theyre at increased risk for stroke. As people grow older, theyre likely to develop new complications such as chronic lung disease and painful leg ulcers.
If you or your child has sickle cell anemia, ask your healthcare provider what you can do now and what you should expect. Theyll likely plan regular tests to monitor signs and symptoms so they can diagnose and treat complications as early as possible.
Studies show people with sickle cell anemia are constantly trying to manage pain that many times affects their quality of life. Sometimes, they have acute pain that happens when sickled cells block blood flow. This is VOC and is a medical emergency. Other people have chronic pain pain that lasts for more than three to six months. If you have chronic pain, consider working with a pain management specialist. They can recommend different ways you can manage pain.
When should I go to the emergency room?
Sickle cell anemia may cause serious medical conditions. Go to the emergency room if you have the following symptoms:
- Extreme fatigue.
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Who Is Affected By Sickle Cell Anemia
Sickle cell anemia is rare in the United States, affecting about around 100,000 people. It mostly affects people whose ancestry links back to parts of the world where many people have malaria and carry a gene that provides partial protection against anemia. This gene also causes sickle cell anemia. In the United States, sickle cell anemia affects many people who are Black. It may also affect people from southern European, Middle Eastern or Asian Indian ancestry.
Immunization Of Persons New To Canada: Canadian Immunization Guide
For health professionals
Last partial content update
: This chapter was updated to include a new section on COVID-19 vaccine with recommendations for individuals planning to live, work or study in Canada who have received 1 or 2 doses of a non-Health Canada authorized vaccine.
Last complete chapter revision: July 2015
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Multiple Organ Failure Syndrome
This life-threatening complication involves the acute development of severe dysfunction in at least two of three major organs during a painful episode. Fever, a drop in hemoglobin level, decreasing platelet count, rhabdomyolysis, and mental status changes are all associated with the onset of multi-organ failure. This rare complication is one of the most challenging for even the most experienced sickle cell disease expert. Prompt consultation with a hematologist is advised because emergent exchange transfusion can be lifesaving.32
Health Assessment Of Persons New To Canada
Assessment before arrival to Canada
Immigration, Refugee and Citizenship Canada generally conduct Immigration Medical Examinations before foreign nationals arrive in Canada. IME is required for:
- most people seeking permanent residence in Canada
- foreign nationals seeking to work in Canada in an occupation in which the protection of public health is essential
- foreign nationals seeking temporary residence in Canada for 6 months or more and who have been residing in a designated country for 6 months or more
- convention refugees selected for resettlement in Canada and
- refugee claimants in Canada.
If the IME is not conducted prior to arrival it is done as soon as possible after arrival.
Assessment after arrival in Canada
Health care providers in Canada who see persons newly arrived in the country should prioritize assessing and updating immunizations for persons new to Canada because the IME does not include a review of immunization status. In addition, health care providers should perform a complete health assessment, including comprehensive testing for a variety of chronic and non-vaccine preventable diseases.As part of the health assessment, the following tests should be completed to determine the need for vaccines or contraindications to vaccination:
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Are The Side Effects Of The Vaccine Any Different For People With Sickle Cell Disease Is There Anything Specific To Sickle Cell Disease I Should Look Out For
What people are observing mostly is a good effect. Your body is supposed to react when you introduce a foreign body into it. You’re supposed to get a little fever. You’re supposed to have a little immune response. That’s what people call side effects. My observation is that patients with sickle cell disease have side effects just like everybody else. That’s a good thing.
What Routine Immunizations Are Necessary
Children with SCD should receive all routine immunizations. This includes vaccines for:7
- Hepatitis B
- Hepatitis A
- Human papillomavirus
Children and adults with SCD should also receive the annual flu vaccine, starting after 6 months old. People with SCD should usually take a version called the inactivated influenza vaccine .7
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Covid Mrna Vaccine Technology Could Be Used To Target Hiv Sickle Cell And Cancer
Combined with the genome editing tool which won the 2020 Nobel Prize in Chemistry, mRNA vaccines could reshape medicine.
Monday 8 February 2021 14:29, UK
The coronavirus pandemic has spurred on the development of a range of new medical technologies, some of which could have a lasting impact on humanity’s ability to treat difficult diseases.
Both COVID-19 vaccines Pfizer/BioNTech and Moderna use something called messenger RNA to send genetic instructions to the body’s cells that result in them producing antibodies for the coronavirus without the virus actually being introduced to their body.
Speaking to MIT Technology Review magazine, Professor Drew Weissman and Dr Katalin Kariko – whose research underpins the technology – said that mRNA could in the future be used to treat HIV, sickle cell disease and potentially even cancer.
It isn’t the first time hopes have been raised about the medical uses for mRNA.
Speaking on the Sky News Daily podcast with Dermot Murnaghan, World Health Organisation epidemiologist Dr Larry Brilliant described the technology as a “breakthrough” that “has the potential for phenomenal knock-on effects”.
Messenger RNA is the molecule that contains essential information for cells to produce particular proteins.
Addressing The High Incidence Of Stroke
Neurologic complications are relatively common in children and adults with sickle cell disease.16 In fact, 17 percent of asymptomatic children with hemoglobin SS disease have structural changes on magnetic resonance imaging consistent with âsilentâ infarctions.17 Occlusive strokes are attributed to persistent sickling and probable endothelial proliferation, leading to narrowing of cerebral blood vessels. Fortunately, primary prevention of childhood stroke is now possible because effective screening and treatment exist.
Screening with specialized transcranial Doppler ultrasonography is recommended for asymptomatic children with sickle cell disease beginning at two years of age to identify those with an abnormal transcranial blood-flow velocity .18,19 Children with TBV of 200 cm per second or more are at a particularly high risk of stroke.20 In a multicenter clinical trial18 using this TBV criterion for enrollment, 16.4 percent of children receiving standard care had a stroke during a mean follow-up of 18 months chronic transfusions reduced the incidence of stroke to 1.6 percent. Furthermore, the most recent data indicate that patients revert back to high-risk status if the transfusions are discontinued.21 Transfusion therapy should be considered for children at increased risk of stroke.19
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Critical Gaps And Future Directions
Although flu shot coverage was higher among Medicaid enrollees with SCD than those without SCD in each of the six flu seasons studied, flu shot coverage was low overall. More than 2 in 3 Medicaid enrollees with SCD had not been vaccinated. Programs and campaigns focused on increasing the number of people with SCD receiving annual flu shots might reduce flu-related hospitalization and death in this high-risk population.