As a person with chronic illness, it is vital for you to stand up for yourself and ensure the level of care you deserve. Know your rights:
SICKLE CELL DISEASE (SCD) BILL OF RIGHTS:
- TO RECEIVE THE HIGHEST STANDARD OF MEDICAL CARE
- HAVE ACCESS TO INFORMATION, RESOURCES, AND SERVICES FOR SICKLE CELL DISEASE
- TO BE TREATED WITH RESPECT
- TO HAVE RESOURCE COMMITMENT FOR RESEARCH AND CURES THAT IS NOT INFERIOR TO ANY OTHER DISEASE CONDITION
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SCD requires lots of regular screening and preventative action. Using the list below, make sure that you and other SCD patients are getting the necessary care:
A) SCREENING- Prevention is the BEST treatment!!
- TRANS-CRANIAL DOPPLER – This is a simple non-invasive ultrasound test that can prevent strokes. All kids with SCD should get this test ANNUALLY from age 2 to 16 years old. Click here to see more information about TRANS-CRANIAL DOPPLER
- In children with SCD, annual screen for high blood pressure, asthma, kidney disease and if short of breath for pulmonary hypertension (high pressures in the lungs).
- Annual eye exam starting at age 10 to evaluate for retinopathy.
B) REGULAR AND COMPREHENSIVE MEDICAL FOLLOW UP:
People who have Sickle Cell Disease should be seen by a health care provider regularly and be a part of a comprehensive care program.
Medications such as hydroxyurea are very important from a prevention standpoint and can reduce the likelihood of painful sickle cell crises. See below for all the medications that can play a role in treating sickle cell disease.
Blood transfusion may be necessary and can really help to treat sickle cell disease crises and to prevent complications because giving normal blood cells allows the body to avoid clogging up small vessels with sickled red blood cells and allows the body to oxygenate tissues.
Newer but possibly completely curative therapies may be available and you should inquire about this. This includes hematopoietic stem cell transplantation and some gene therapy treatments (although these are mostly investigational at this point).
Please see this inspiring story of a successful Sickle Cell cure from the New York Times (September 14th, 2021):
C) IMMUNIZATION AND INFECTION CONTROL
It is super important to understand that sickle cell patients are extremely vulnerable to both viral and bacterial infections. People with sickle cell disease do much worse with these infections than those who don’t have sickle cell disease. Sickle cell patients should be considered immunosuppressed!
Immunizations are most important for preventing infection!! Prevention is the best Medicine!
All sickle patients should be vaccinated and boosted with COVID-19 shots.
All sickle cell patients should get the flu shot.
All sickle cell patients should get all the regular childhood vaccinations. This includes: streptococcus pneumoniae, meningitis, hepatitis B, measles/mumps/rubella (MMR), tetanus, diphtheria, pertussis, and HIB.
Remember that all these vaccinations have to be kept up to date all the way into adulthood.
All patients with sickle cell disease should be getting (or at least offered) antibiotics to prevent infections particularly under the age of 5, but sometimes longer. Part of this reason is that the spleen in sickle cell disease does not work properly and so leaves you more vulnerable to bacterial infections. Usually people are given Penicillin but if there is a Penicillin allergy then Erythromycin or other antibiotic is used.
D) NEVER IGNORE SYMPTOMS:
Are you experiencing changes in your vision? This could be due to problems with your retina. At least annual eye and retina exams should be done.
Asthma? This is of course more prevalent already in urban settings like Bridgeport. Asthma is very common in children with sickle cell disease and they do much worse if not treated.
Vitamin D deficiency is so common in Bridgeport. Calcium and vitamin D are important.
Mental health is as important as any physical condition. People with sickle cell disease have many challenges and depression is VERY common. This can be and must be treated! There is no shame or stigma with this.
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If you have SCD, there are also many medications and treatments available to you to help manage your symptoms and prevent complications. Ask your doctor about these options below to see if they would be beneficial to you:
- Hydroxyurea.
- helps to prevent formation of sickle-shaped red blood cells
- 1 pill taken every day
- Learn more here
- Blood transfusion
- introduces healthy red blood cells into your body
- aims to lessen anemia, make it easier for blood to flow, ease disease symptoms and prevent complications
- Learn more here
- L-glutamine (ENDARI)
- “reduce the acute complications of sickle cell disease”
- available for adult and pediatric patients 5 years of age and older
- Learn more here
- Visit ENDARI’s official website
- Voxelator (Oxbryta)
- interferes with the part of the process that causes red blood cells to sickle in the first place
- available for patients ages 12 and up
- Learn more here
- Crizanlizumab (Adakveo)
- stops blood vessels and cells from being sticky, preventing some blockages
- reduce frequency of pain crises
- available for patients ages 16 and up
- Learn more here
- Pain control medications.
Join the SCD community in Connecticut!
Sickle Cell Disease Association of America
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