Dear G.P
My child has been diagnosed with sickle cell anemia. He suffers terible pain and I’m so helpless.Is this something my child will eventually outgrow? What is the best possible way to treat this.
Worried parents
Dear Worried parents,
My heart goes out to you and your little one.Unfortunately sickle cell anemia is not something your child will outgrow but will have to live with for the rest of his life.The good news is by taking precautions and aggressively managing problems that occur, sickle cell patients now have with a better quality of life than they did years ago.
What is sickle cell disease
Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape. (Red blood cells are normally shaped like a disc.This stops your blood carrying oxygen as well as it should. And sickle cells can’t travel in your bloodstream as easily as ordinary red blood cells. They sometimes get stuck in your small blood vessels. This can stop blood getting to some parts of the body.Any part of the body not getting a sufficient blood supply will become painful and damage to that tissue could occur if circulation is not restored.
What are the symptoms?
-The hallmark of sickle cell disease is Pain. Almost everyone with sickle cell disease gets pain at some point, although for some people it doesn’t happen very
often.Pain in commonly in the abdomen, legs and arms and chest.
-Because sickle cell disease stops blood carrying oxygen properly, your child may get tired or breathless easily.
-He may be more likely to catch infections as children with sickle cell anemia are more vulnerable because of damage to their immune systems from the disease.
-He may get swollen hands or feet.
-He may have yellow color of the eyes and skin
What can you do to help?
Fluids are number one in importance. Your child should drink as much water as possible each day to prevent dehydration.( 8-10 cups of water)
Avoid stressful situations and make sure your child gets enough rest and warmth.
It’s best not to expose the child to smoke. Both active and passive smoking may promote acute chest syndrome in sickle cell patients.
Some people find very hot or cold temperatures bring on attacks of sickle cell pain.
Moderate exercise can be good but very strenuous exercise might bring on an attack of sickle cell pain.
Avoid conditions, such as crowds, that increase risk for infections.
Avoid high altitudes if possible. If flying is necessary, be sure that the airline can provide oxygen.
Foods- Good nutrition is essential for anyone and critical for patients with sickle-cell disease.
Feed your child lots of green,red and yellow vegetables, fruits, or juices(9 servings daily) as these are rich in antioxidants and other important nutrients. Some research suggests that antioxidant foods or supplements (such as vitamins E or C) may help inhibit the formation of the dense cells that trigger a sickle-cell crisis.
-Protein is important for sickle-cell patients.
-Omega-three fatty acids
Some minerals that are of benefit include
-Zinc
-Magnesium
-Arginine
-L-glutamine
Patients should take daily folic acid and vitamin B12 and B6 supplements.
Note on Iron. Although sickle-cell disease is often referred to as anemia, iron supplements or iron rich foods should be avoided in patients receiving multiple transfusions, which increase the risk for iron-overload.
What treatments work?
Your child may need to take medicine by the mouth for up to 10 years to prevent life-threatening infections. Later in life, care focuses more on managing pain.Thus he will require regular doses of penicillin. Penicillin doesn’t normally cause serious side effects. Some children are allergic to it, but they can take a similar drug called erythromycin instead.
Vaccines can also help prevent infections.Your doctor may suggest a vaccine to protect your child from illnesses such as pneumonia and meningitis. These vaccines can cause a mild fever, but they don’t usually have serious side effects.
Being in Zambia you will require anti-malarial drugs because malaria can trigger sickle cell pain.
folic acid (to help prevent severe anemia)
Treatments for pain
If your child gets mild pain, an over-the-counter painkiller, such as panadol or ibuprofen will suffice. Aspirin is another common painkiller, but you shouldn’t give it to children under 16.
For moderate pain, your doctor may give you or your child a weak opioid drug, such as codeine. Or you may be prescribed a strong nonsteroidal anti-inflammatory drug (NSAID).
If you or your child gets severe pain, your doctor may recommend a strong opioid painkiller, such as morphine.You’ll have to go to hospital.You may also be given extra fluids from a drip and oxygen to breathe in through a mask.
Severe sickle cell anemia can be treated with a medicine called hydroxyurea. This medicine prompts your body to make fetal hemoglobin. In people who have sickle cell anemia, fetal hemoglobin helps prevent red blood cells from sickling and improves anemia.
Given daily, hydroxyurea reduces how often painful sickle cell crises and acute chest syndrome occur. Many people taking hydroxyurea also need fewer blood transfusions and have fewer hospital visits.However scientists ares still studying the long-term effects of hydroxyurea on people who have sickle cell anemia. Early studies in children suggest that the medicine may help improve growth and preserve organ function, but this has not been proven.A side effect of concern is Hydroxyurea can reduce the number of white blood cells in your blood. This can lead to an increased risk of infections.
Other treatments
Bone marrow transplant offers the only potential cure for sickle cell anemia. But, finding a donor is difficult and the procedure has serious risks associated with it, including death.
If you become seriously ill because of sickle cell disease, you may need a blood transfusion. A transfusion can help with severe anaemia, lung problems, or a stroke. Most people with sickle cell disease will need a blood transfusion at some point.
Prognosis
Many people cope well with sickle cell disease and live successful lives. However, it is possible to get severe pain and a lot of health problems. It’s important to know about the problems your child could get, and act straight away if your child gets ill.
When to seek medical attention
-fever
-pain that doesn’t go away after taking ordinary painkillers
-pain in the abdomen that lasts a long time
– chest pain
– difficulty breathing
-trouble speaking
– changes in vision
– weakness in the hands or feet
-headaches that don’t go away
-in boys an erection that doesn’t go away (priapism)
What is the future of Sickle Cell Anemia treatment?
Scientists have learned a great deal about sickle cell anemia during the past 30 years – what causes it, how it affects the patient, and how to treat some of the complications.
The ultimate cure for sickle cell anemia may be gene therapy. In sickle cell anemia, the gene which switches on production of adult hemoglobin shortly before birth is defective. Two approaches to gene therapy are being explored. Some scientists are looking into whether correcting this gene and inserting it into the bone marrow of people with sickle cell anemia will result in the production of normal adult hemoglobin.
Others are looking at the possibility of turning off the defective gene and simultaneously reactivating another gene that turns on production of fetal hemoglobin. In both cases, the research is at a very early stage. Progress is being made, however, and there is a real possibility of an eventual clinical cure for sickle cell anemia.
Although the genetic defect that causes sickling was identified more than 40 years ago, until very recently, research into the development of treatments for the disease was hampered by the lack of an animal model that could be used to test experimental drugs and gene therapy. Recently, however, scientists were able to genetically engineer a line of mice that exhibit some of the characteristics of sickle cell disease in much the same way humans do. This is an important advance in the search for an effective treatment and eventual cure for sickle cell disease.
Some researchers are focusing on identifying drugs that will increase the level of fetal hemoglobin in the blood. Fetal hemoglobin is a form of hemoglobin that all humans produce before birth, but most stop making shortly after birth. Most humans have little fetal hemoglobin left in their bloodstream by the time they reach the age of 6 months. However, some people with sickle cell anemia continue to produce large amounts of fetal hemoglobin after birth, and studies have shown that these people have less severe cases of the disease. Fetal hemoglobin seems to prevent sickling of red cells, and cells containing fetal hemoglobin tend to survive longer in the bloodstream.
Hydroxyurea appears to work primarily by stimulating production of fetal hemoglobin. There is some evidence that administering hydroxyurea with erythropoietin, a genetically engineered hormone that stimulates red cell production, may make hydroxyurea work better. This combination approach offers the possibility that lower doses of hydroxyurea can be used to achieve the needed level of fetal hemoglobin. However, both of these drugs may produce serious side effects, so researchers continue to search for safer agents that are just as effective.
‘Butyrate, a simple fatty acid that is widely used as a food additive, is also being investigated as an agent that may increase fetal hemoglobin production.
Clotrimazole, an over-the-counter medication commonly used to treat fungal infections, is under investigation as a treatment to prevent the loss of water from the red blood cells that contributes to sickling. It is hoped that this medication, used alone or in conjunction with other anti-sickling agents, may eventually offer an effective long-term therapy for sickle cell anemia patients.
Bone marrow transplantation has been shown to provide a cure for severely affected children with sickle cell disease. Although many of the risks of this procedure have been reduced, it still is not entirely without risk. In addition, the marrow must come from a healthy matched sibling’ donor, and only about 18 percent of children with sickle cell anemia are likely to have a matched sibling. Researchers are working on techniques to further reduce some of the risks of bone marrow transplantation for patients with sickle cell disease.
Disclaimer:The information in this article is provided for educational and entertainment purposes only. It is not intended as a substitute for professional advice of any kind.If you have any health concerns please see your health care provider
