Sickle cell anemia is a genetic disease that deforms red blood cells and reduces their ability to carry oxygen to cells. In addition, due to their sickle or crescent shape they get trapped in smaller blood vessels, slowing or blocking blood circulation and causing severe pain. With the exception of bone marrow transplantation, sickle cell anemia cannot be cured, although there are some treatments that can help control symptoms and reduce pain attacks.
Steps
Part 1 of 2: Treating Sickle Cell Anemia
Step 1. Give antibiotics, especially to young children
Sickle cell anemia is hereditary, therefore it is present from birth and can be life threatening in infants and young children due to hyposplenism which, resulting in reduced spleen function, increases the risk of contracting severe forms of infections. Usually, in these cases, antibiotics, including penicillin, are prescribed to prevent bacterial infections in both younger people and adults.
- Infants with sickle cell anemia can start taking antibiotics at around 2 months of age and continue for the first 5 years of life.
- Infants need to take penicillin in liquid form, while older children and adults can take it in pill form, usually twice a day.
- The most dangerous infection associated with sickle cell anemia is bacterial pneumonia.
Step 2. Take pain relievers
In addition to the constant feeling of tiredness and exhaustion due to the lack of oxygen in the blood, there are frequent manifestations of severe pain in subjects suffering from sickle cell anemia. To manage these episodes of chronic pain, often referred to as sickle cell crises, take an over-the-counter medication, such as acetaminophen (Tachipirina) or ibuprofen (Momento or Brufen) for a day or two until they pass. They can last a few hours as well as a couple of weeks.
- Pain of moderate or severe intensity occurs when sickle-shaped red blood cells decrease or block blood circulation within the smaller blood vessels in the chest, abdomen, and limbs.
- Since the pain is mostly located in the joints and bones, it is deeper than that felt on the surface.
- For fairly severe episodes that last for days, your doctor may prescribe a stronger medication, such as an opioid pain reliever.
Step 3. Apply heat to the painful parts of the body during sickle cell crises
During these episodes, it may be useful to put a heat pad or a natural pillow on the body that releases warm and humid because the high temperature tends to dilate the blood vessels and allows the sickled red blood cells to move better in the bloodstream. Moist heat is a better choice than heat produced by electrical devices because it doesn't dry out the skin. Choose a pillow to put in the microwave, containing natural materials (such as bulgur or rice), herbs and essential oils.
- Heat the pillow in the microwave for about 2-3 minutes and apply it where you feel pain (joints, bones or abdomen) for at least 15 minutes, three to five times a day.
- By pouring a few drops of lavender or other relaxing essential oils on the pillow, you can also reduce the discomfort and anxiety caused by sickle-cell crises.
- The bathroom is also another great way to reap the benefits of humid heat. For best results, add 550g of Epsom salts - the magnesium contained within can further relieve pain.
- Avoid ice packs and cold heat pads, as they can promote sickle cell disease (or deformation) of the red blood cells.
Step 4. Increase your folic acid intake
Red blood cells, produced by the bone marrow contained in the canals of long bones, need certain nutrients to reform regularly. One of the most important nutrients for the production and replacement of red blood cells is folate (vitamin B9), also called folic acid when it is present in vitamin formulations and in so-called fortified foods. So, if you have sickle cell anemia, take folic acid supplements every day and / or eat folate-rich foods regularly.
- Vitamins B6 and B12 are also important for red blood cell production and can even activate chemical processes that inhibit red blood cell sickle cell disease.
- Excellent sources of these B vitamins are: red meat, high-fat fish, white meat, most whole grains, enriched cereals, soy, avocado, baked potatoes (with peel), watermelon, bananas, peanuts and brewer's yeast.
- The recommended daily requirement of folic acid ranges from 400 to 1000 mcg (micrograms).
- Iron-free multivitamins are also recommended.
Step 5. Use hydroxyurea
When taken regularly, hydroxyurea (Oncocarbide) is a drug that helps relieve pain attacks caused by sickle cell anemia, but also reduces the need for blood transfusions in moderate or severe cases. Hydroxyurea tends to stimulate fetal hemoglobin production in children and adults, preventing the formation of sickle-shaped red blood cells.
- As announced by the National Heart Lung and Blood Institute (NHLBI), the clinical trial investigating the efficacy of the drug hydroxyurea in the treatment of sickle cell anemia was prematurely closed because the conclusive data obtained were deemed sufficient to establish that the drug it is safe and effective.
- Fetal hemoglobin occurs naturally in infants who, however, quickly lose the ability to produce it within a few weeks or months.
- Hydroxyurea was initially administered only to adults with severe sickle cell disease, but today many doctors prescribe it to children with excellent results.
- Look out for any side effects, including the increased risk of infections and a possible relationship to leukemia (cancer of the blood cells). Ask your doctor if taking hydroxyurea is safe for you or your child.
Step 6. Undergo routine checkups and tests
If you suffer from sickle cell anemia, know that there are a series of checks and tests that are very important in the treatment of this condition and in reducing complications.
- Undergo a fundus examination from the age of 10 to rule out sickle cell retinopathy. If your results are okay, check out every one or two years. If any abnormality is found, consult a retinal disease specialist.
- Get diagnostic tests for kidney disease from the age of 10. If they are negative, you should repeat them once a year. If they are positive, undergo more thorough tests.
- Check your blood pressure regularly. Even a slight increase in blood pressure can increase the risk of stroke in patients with sickle cell disease.
Step 7. Fight fatigue with oxygen therapy
Lack of oxygen in the blood causes fatigue, lack of strength and chronic fatigue. Sometimes, even just getting out of bed in the morning can feel tiring. Supplemental oxygen delivery, through a mask connected to a pressurized oxygen cylinder, can help you overcome a crisis or face your day, as happens to those with severe emphysema. Ask your doctor about the pros and cons of using supplemental oxygen for sickle cell anemia.
- Oxygen therapy does not put sickle red blood cells in a position to carry the delivered oxygen: this task is performed by healthy red blood cells which, saturated with oxygen, transmit it to the various tissues of the body.
- Typically, supplemental oxygen contains more oxygen than is present in the air at sea level. If you travel to higher altitudes, taking it with you can prevent sickle-cell crises until the body is able to adapt to the new conditions.
Step 8. Discuss the need for a blood transfusion with your doctor
Another type of treatment that works directly by replacing sickled red blood cells with healthy ones is blood transfusion. Blood transfusions introduce a large number of red blood cells into the bloodstream and, as a result, help relieve the symptoms caused by this disease. Healthy red blood cells live longer than sickle cells, up to 120 days, while the latter last no longer than 20 days.
- Children and adults with severe sickle cell anemia and at a higher risk of stroke due to blocked arteries can greatly reduce the dangers they face by having regular blood transfusions.
- Blood transfusions are not without risk. They can increase the risk of infections and increase the body's iron stores, consequently damaging the health of internal organs, such as the heart and liver.
- If you have sickle cell anemia and have regular blood transfusions, ask your doctor about deferasirox (Exjade), a drug that lowers blood iron.
Step 9. Ask your doctor about nitric oxide therapy
Patients with sickle cell anemia have a rather low level of nitric oxide in the blood. It is a molecule that promotes vasodilation and reduces the "viscosity" of red blood cells. Ask your doctor for nitric oxide treatment, as it can prevent sickle cells from clumping together and blocking smaller arteries (studies have had mixed results on how effective this therapy is).
- Treatment involves inhaling nitric oxide. However, it is difficult to administer and the doctor may not necessarily feel able to handle this procedure.
- It is possible to increase blood levels of nitric oxide by taking supplements based on arginine, an amino acid. It carries no risks and has no known side effects.
Step 10. Consider a bone marrow transplant
Bone marrow (or stem cell) transplantation consists in replacing the bone marrow that produces sickled red blood cells with another healthy one from a donor with which there is histocompatibility. This is a long and risky surgical procedure, involving the destruction of the entire bone marrow of the anemic patient with radiation or chemotherapy and, subsequently, intravenous infusion of the donor stem cells. It is the only solution that allows you to cure sickle cell anemia. Ask your doctor about the pros and cons and if you can have this surgery.
- Not all people with sickle cell anemia can undergo a bone marrow transplant. Furthermore, it is not easy to find donors with whom there is histocompatibility.
- Only about 10% of children with sickle cell disease have a family with healthy stem cell donors with whom there is histocompatibility.
- The risks of bone marrow transplantation are numerous and include life-threatening infections due to a destroyed immune system.
- Because of the risks, transplantation is generally only recommended for those with severe and chronic symptoms related to sickle cell anemia.
Part 2 of 2: Preventing Sickle Crises
Step 1. Focus on infection prevention
This is a very important precaution in patients with sickle cell anemia, as they are exposed to viral and bacterial infections due to impaired function of the spleen, which often occurs from early childhood. Therefore, in addition to antibiotic prophylaxis from an early age, it would also be advisable to immunize oneself against certain diseases, for example, to carry out compulsory childhood vaccinations, but also to those against influenza, bacterial meningitis and some types of pneumonia.
Step 2. Avoid high altitudes if your body isn't used to it
At higher altitudes there is less oxygen and this phenomenon could quickly trigger a sickle cell crisis if the body is not used to such conditions. Therefore, be cautious if you are traveling to high-altitude places (such as mountainous regions) and consider using supplemental oxygen if you decide to go.
- Consult your doctor before traveling to high altitudes and weigh the health benefits against the risks.
- If you have to fly, choose only those with pressurized cabins (found on the largest aircraft of all commercial flights) and avoid going high in smaller, non-pressurized aircraft.
Step 3. Stay hydrated
It is important to keep your blood volume high, especially if you have sickle cell anemia. If its values are low (common phenomenon when dehydrated), the blood becomes more viscous and tends to clump, triggering a sickle cell crisis. Prevent dehydration by drinking at least eight 240ml glasses (about 2 liters) of purified water per day.
- Avoid consuming caffeinated drinks, otherwise they will have a diuretic action (make you urinate more often) and can reduce blood volume.
- Caffeine is found in coffee, black tea, chocolate, soda, and virtually all energy drinks.
- Increase the amount of fluids you ingest each day if you exercise a lot or live in a warmer climate.
Step 4. Don't get too hot or too cold
Another possible trigger for sickle cell crises are sudden changes in temperature: excessive heat or cold. The heat increases sweating resulting in dehydration and lowering of blood volume. The cold, on the other hand, favors the narrowing of blood vessels (in practice, they become smaller), hindering blood circulation.
- If you are in a hot and / or humid environment, try to stay in places and vehicles that have air conditioning. Wear clothes made of natural fibers (cotton), which promote perspiration.
- Stay warm in cold weather by wearing multiple layers of clothing made of insulating fabrics, such as wool. It is especially important for people with sickle cell disease to keep their hands warm with a pair of gloves.
Step 5. Avoid engaging in physical activities that are too heavy
Sports that involve a high expenditure of physical energy increase oxygen demands and trigger sickle cell crises, because the body does not have enough hemoglobin to carry oxygen to the cells that need it. Exercising regularly is good for your health and circulation, but avoid fatigue by running, cycling, and swimming over long distances.
- Instead, focus on low-impact exercises - for example, you might walk, do aerobic activity, practice yoga, and do less strenuous garden work.
- If it is light or medium intensity, weight lifting is good for strengthening and maintaining muscle tone, but heavy lifting is not recommended for people with sickle cell disease.
Advice
- In the 70s of the last century, the average life expectancy of patients suffering from sickle cell anemia was around 14 years, but today with the advances made in modern medicine, people affected by this disease can even exceed 50 years.
- Usually, women with sickle cell anemia experience less severe symptoms and live longer than men.
- Do not smoke and avoid exposing yourself to secondhand smoke, especially if you have sickle cell anemia, as it impairs circulation and increases blood viscosity.
