Sickle cell anemia is an inherited disorder that causes red blood cells (red blood cells) to become deformed, which reduces their ability to deliver oxygen to the cells of the body. In addition, deformed sickle-shaped red blood cells become lodged in small blood vessels, slowing or stopping blood flow and causing severe pain. Although sickle cell disease can only be treated with a bone marrow transplant, some methods can help alleviate symptoms and relieve painful crises.
Steps
Part 1 of 2: Treating Sickle Cell Disease
Step 1. Use antibiotics, especially for young children
Sickle cell anemia is a hereditary disease, which means it is passed from parent to child at birth. This condition is fatal to infants and young children due to hyposplenia (decreased spleen function), which increases the risk of serious infections. To prevent bacterial infections, babies and young children (and in some cases adults) are often given antibiotics, usually penicillin.
- Babies with sickle cell disease can be prescribed antibiotics from about 2 months of age, and often they take them until they are 5 years old.
- Babies need liquid penicillin, while older children and adults can take pills (usually twice a day).
- Bacterial pneumonia is the most common life-threatening infectious disease that occurs with sickle cell disease.
Step 2. Take pain relievers
In addition to increased fatigue due to low oxygen concentration in the blood, sickle cell patients often experience bouts of acute pain. To manage these attacks (also called crises), take over-the-counter pain relievers such as paracetamol (Panadol) or ibuprofen (Advil, Motrin) for one day or longer until the pain subsides. Individual crises can last from a couple of hours up to several weeks.
- Reduction or complete blockage of small blood vessels in the chest, abdomen, and extremities by sickle-shaped red blood cells can cause moderate to severe pain.
- The pain is felt more strongly in the joints and bones - in other words, it is felt inside the body.
- For acute crises that last longer than a few days, your doctor may prescribe a stronger remedy, such as opium medications.
Step 3. Apply warm compresses to problem areas during a crisis to reduce pain
A heating pad or wet bag of herbal remedy can help reduce pain as the heat dilates blood vessels and makes it easier for sickle-shaped red blood cells to pass. It is best to apply moist, warm compresses to avoid dry skin. Use microwaved bags of grains (crushed wheat or rice), herbs, and essential oils.
- Heat a bag of grains or herbs in the microwave for about two minutes and place it on aching joints, bones, or stomach for at least 15 minutes. Apply warm compresses 3-5 times a day.
- Add a little lavender or other soothing essential oil to the bag to help relieve the discomfort and anxiety of a sickle cell crisis.
- Another good way to warm up a sore spot is to take a bath. Add 2 cups (600 grams) of Epsom salt to the water - the magnesium it contains will help soothe pain.
- Ice and cold compresses should be avoided as they can promote sickle cell formation.
Step 4. Take a folic acid supplement
Red blood cells are produced in the bone marrow within the long bones, and the body requires certain nutrients to produce new red blood cells regularly. One of the most important nutrients involved in the production of new red blood cells is folate (vitamin B9), also called folic acid in supplements. Therefore, people with sickle cell disease are advised to take a daily folate supplement and / or eat foods rich in it on a regular basis.
- Vitamins B6 and B12 are also important for the normal production of new red blood cells, and they help to reduce the number of sickle-shaped red blood cells.
- Foods such as meat, fatty fish, poultry, many whole grains, vitamin-fortified cereals, soybeans, avocados, peeled potatoes, watermelons, bananas, peanuts, and brewer's yeast are high in B vitamins.
- The RDA for folate is 400 to 1,000 mcg (micrograms).
- It is also recommended to take a multivitamin without iron.
Step 5. Take hydroxycarbamide (hydroxyurea)
When taken regularly, hydroxycarbamide (Droxia, Hydrea) helps reduce pain during a sickle cell crisis, and can also reduce the need for blood transfusions in some acute cases. It is believed that hydroxycarbamide stimulates the production of hemoglobin F (fetal, or fetal hemoglobin) in children and adults and thereby reduces the number of sickle red blood cells.
- Hydroxycarbamide is the only drug approved by the US Food and Drug Administration (FDA) for the prevention of sickle cell crises.
- Fetal hemoglobin is present in newborns, but they quickly (over several weeks or months) lose the ability to produce it.
- At first, hydroxycarbamide was used for the treatment of acute crises of sickle cell anemia only in adults, but nowadays it is increasingly successfully used in the treatment of children.
- Be aware of potential side effects, which include an increased risk of infection and a possible link to leukemia (blood cancer). Ask your doctor if you should give your child hydroxycarbamide.
Step 6. Go through standard examinations and tests
There are many different medical techniques that can help assess the patient's condition and reduce the risk of complications.
- Get a dilated pupil examination (done starting at age 10) to help identify possible vascular retinopathy. If there are no abnormalities, take this test every 1-2 years. If you are found to be abnormal, consult an ophthalmologist.
- Check your kidneys from the age of 10. This should be done once a year. If violations are detected, undergo additional examination.
- Monitor your blood pressure regularly. Even a small increase in blood pressure in sickle cell patients increases the risk of stroke.
Step 7. Relieve fatigue with oxygen therapy
Lack of oxygen in the blood leads to increased fatigue and chronic fatigue - to the point that it can be difficult to get up in the morning. Breathing oxygen (a mask attached to a compressed oxygen cylinder) can help you cope with a crisis or even chronic fatigue, just like patients with severe emphysema do. Ask your doctor about the advantages and disadvantages of breathing oxygen for sickle cell disease.
- While breathing oxygen will not improve the ability of sickle-shaped red blood cells to carry it, it will help oxygenate healthy red blood cells and increase oxygen flow to various tissues in your body.
- Breathing oxygen generally has a higher concentration of this gas than normal air at sea level. If you are traveling in an upland area, bring oxygen with you for breathing to prevent crises until you get used to the new environment.
Step 8. Talk to your doctor about blood transfusion
Another treatment is to replace the sickle-shaped red blood cells with normal ones through blood transfusions. This allows large numbers of healthy red blood cells to enter the bloodstream, which can help relieve the symptoms of sickle cell disease. Healthy red blood cells live longer in the circulatory system than sickle cells - up to 120 days. At the same time, sickle-shaped red blood cells rarely live longer than 20 days.
- Regular blood transfusions can significantly reduce the risk of stroke in children and adults with severe sickle cell disease, when the risk of blockage in blood vessels is increased.
- Regular blood transfusions are also associated with certain risks. They increase the chance of infection and lead to iron buildup in the body, which can damage internal organs such as the heart or liver.
- If you have sickle cell disease and receive regular blood transfusions, ask your doctor about deferasirox (Exjade), which can help lower iron levels in your body that are too high.
Step 9. Consult your doctor about a relatively new nitric oxide therapy
Patients with sickle cell disease have low blood nitric oxide levels. This oxide causes blood vessels to dilate and reduces the "stickiness" of red blood cells. Ask your doctor about nitric oxide therapy to help reduce blood clotting and small vessel blockages. Studies of the effectiveness of this method have shown mixed results.
- This procedure involves inhaling nitric oxide. It is difficult to control, so your doctor may not recommend it to you.
- Nitric oxide is found in a dietary supplement such as arginine (one of the amino acids). It is a safe dietary supplement with no known side effects.
Step 10. Consider the possibility of a bone marrow transplant
A bone marrow (or stem cell) transplant involves removing the affected tissue and replacing it with healthy cells from a suitable donor. This is a long and risky operation, which involves the destruction of all the affected bone marrow from the patient with the help of radiation or chemical therapy and the subsequent introduction of stem cells taken from the donor. This operation is the only way to completely recover from sickle cell disease. Ask your doctor about the advantages and disadvantages of a bone marrow transplant and if it is right for you.
- Bone marrow transplant is not suitable for all sickle cell patients, and it can be difficult to find a suitable donor for this operation.
- Only about 10% of children with sickle cell disease have family members with healthy stem cells suitable for donation.
- Bone marrow transplant is associated with many dangers, including contracting a life-threatening infection with a compromised immune system.
- Due to the risks associated with the operation, it is only recommended for patients with severe chronic sickle cell disease.
Part 2 of 2: Preventing Sickle Cell Anemia Crises
Step 1. Pay special attention to the prevention of infectious diseases
This is very important in sickle cell disease. Due to the impaired function of the spleen, patients with sickle cell disease are particularly susceptible to various viral and bacterial infections that often occur in early childhood. In view of this, they should not only take antibiotics for prophylactic purposes in childhood, but also get the necessary vaccinations on time. This is the standard childhood vaccination, as well as vaccinations for influenza, bacterial meningitis, and some types of pneumonia.
Step 2. Don't go high above sea level if your body is not used to it
At higher altitudes, there is less oxygen, which in the absence of habit can quickly lead to a crisis of sickle cell anemia. Be careful when traveling to high altitude areas and take oxygen with you for breathing.
- Before going to the highlands, check with your doctor about whether it is worth risking your health in this way.
- Fly only aircrafts with a sealed cabin (this includes all fairly large passenger aircraft). Do not fly high in small airplanes with a leaky cockpit.
Step 3. Make sure that your body is not experiencing a lack of fluid
Blood volume should be maintained at the required level, especially in the case of sickle cell disease. Low blood pressure due to dehydration thickens the blood and increases blood clotting, which can trigger a crisis in sickle cell disease. Drink at least 8 glasses (about 2 liters) of clean water daily to prevent dehydration.
- Avoid drinking caffeinated drinks, which are diuretic and can lower blood counts.
- Caffeine is found in coffee, black tea, chocolate, many sugary sodas, and just about all energy drinks.
- Increase your daily fluid intake if you exercise vigorously enough or live in hot climates.
Step 4. Do not overheat or overcool
Temperatures that are too high or too cold can also trigger a sickle cell crisis. The heat increases sweating, which leads to dehydration and decreased blood flow. In turn, the cold causes the blood vessels to constrict, which makes it difficult for blood to circulate.
- If you live in an extremely hot and / or humid climate, try to stay in air-conditioned rooms and vehicles. Wear natural fabrics (cotton) to help your body breathe freely.
- In colder climates, wear warm clothing such as woolen clothing. Keeping your hands warm and wearing gloves is especially important for sickle cell patients.
Step 5. Observe the measure when engaging in physical exercise
Intense physical activity increases the body's need for oxygen, which, with an insufficient amount of hemoglobin (it is responsible for transporting oxygen to the tissues), can cause a crisis. Moderate exercise is good for your health and circulation, but be careful not to run, cycle, or swim too long distances.
- Engage in light to moderate exercise such as walking, light aerobics, yoga, and light-duty gardening.
- If you want to build muscle, lift light to medium weights. Heavy weight training is not recommended for people with sickle cell disease.
Advice
- Back in the 70s of the twentieth century, the average life expectancy of patients with sickle cell anemia was only 14 years. Nowadays, thanks to the advances in medicine, people with this diagnosis can live 50 or more years.
- On average, women have less sickle cell symptoms and live longer than men.
- Do not smoke and avoid secondhand smoke, especially for sickle cell disease. Smoking impairs blood circulation and increases blood clotting.