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Sickle cell disease is an inheritable blood disorder that affects Red Blood Cells (RBCs). The main purpose of RBCs is to deliver oxygen to the body and the part of the blood that carries oxygen is called haemoglobin. Sickle cell disease affects the haemoglobin. The normal RBCs appear as disc shaped and resemble doughnuts without holes in the centre and they move through blood vessels carrying oxygen to all parts of the body. The sickle cell on the other hand has a crescent shape and it is stiff and sticky. The condition is characterised by anaemia due to rapid destruction of sickle red blood cells, pain due to the cells becoming stiff and rigid, thus have trouble passing through small blood vessels, limiting blood from reaching all part of the body and eventually resulting in the crisis episodes. As a result, parents and loved ones are always worried about their children and relatives with sickle cell disease. There is no special diet regime for people with sickle cell disease; however a nutritious and well-balanced diet will aid in the rapid synthesis of new RBCs to prevent anaemia, possibly preventing or reducing the crisis episodes experienced by most sickle cell persons. Nutritional recommendations There is no special diet regime for sickle cell disease, however, a nutritious and well-balanced diet plus adequate hydration is the key to preventing complications associated with the disease. The following nutrition and nutritional recommendations are important to achieve good health. Fluids are number one in importance. Sickle cell persons should drink as much water as possible each day at least eight–10 glasses to prevent dehydration because less water in blood may cause the blood cells to sickle, blocking blood vessels, hence causing pain. Due to this, caffeine-containing drinks, such as energy drinks, cola or coffee and alcohol should be avoided because they cause frequent urination, making one dehydrated. Ice water and icy products should also be avoided because both caffeine and ice cause blood vessels to narrow, causing difficulty in blood flow through vessels, hence pain. Children with sickle cell Children with sickle cell disease need more calories than others and this is because they often delay in growth and maturation. Eating fruits, vegetables, grain products (breads, pasta, cereals and rice) and low fat yogurt without too much fat can help children get the calories needed and prevent obesity. Protein, a macronutrient, is needed by everyone to promote growth and people with sickle cell need adequate protein to grow and make haemoglobin, thus diet must be protein-dense with emphasis on plant-sources of proteins such as beans, peas, pulses, lentils, melon seeds (agushie) soybeans, groundnuts, cashew, mushroom and milk. Food sources such as the oily fish (fresh salmon, fresh tuna, tilapia and fresh sardine) and soybean oil are rich in omega- three fatty acids which scientific studies have found to make red blood membranes less fragile and possibly less likely to sickle. Some micronutrients Some micronutrients are vital in the production of new RBCs and these nutrients must always be readily available to help prevent anaemia. These include folate, vitamin B12, vitamin B6, and zinc. The human body cannot make folate, hence it must be obtained strictly from the diet. Sources of folate include all green leafy vegetables such as kontomire, ayoyo’, ‘alefu, bokorbokor and dandelion and other vegetables such as beetroots, broccoli; sweet corn, beans and legumes, fortified cereals, beef liver, orange and pineapple juices. Vegetables should be partially cooked to prevent leaching of nutrients. Some food sources of vitamin B12 and zinc are meat, milk products (such as cheese), liver, shellfish, and fortified cereals. Food sources for vitamin B6 include meat, oily fish, vegetables, whole grains, beans, groundnuts, potatoes, banana, plantain, and mushrooms. Since red blood cells are rapidly destroyed, iron is stored in the liver, hence all animal sources of protein which are very rich sources of iron, as well as vitamin B12 and zinc should be eaten cautiously to prevent iron overdose. Dietary thiocyanate is also important, since it acts as an oxygen carrier and increases capacity of blood to transport oxygen to every cell in the body and helps reduce sickling of red blood cells. It is a must-have substance in dealing with challenges of sickle cell. Food sources of thiocynate, include yam, cassava, millet, sorghum, plantain, banana, almonds, broccoli cabbage, carrots, cauliflower, lentils, lima beans, beans, peanuts, beetroots and plums. Blood vessels relaxation Another important nutrient is citrulline, which is a quick absorbing amino acid that promotes blood vessels relaxation, improving oxygen and blood circulation in all parts of the body. Rich food source of citrulline is watermelon, including the rind (the white portion before the green back of the watermelon) and seeds. Since it might be difficult chewing the seeds, watermelon fruit juice could be prepared by blending the flesh with the rind and seeds. Other sources include milk, legumes and cucumber. Current research has also found that drinking unsweetened pure cocoa powder every day helps improve on complications associated with sickle cell disease because cocoa is enriched with most of the nutrients needed by sickle cell individuals. In conclusion, good nutrition is the key to preventing the vascular disorders in sickle cell and people with sickle cell must practise optimum personal hygiene and food safety practices to avoid infections and promote good health. • The writers are with the University of Ghana College of Health Science Department of Dietetics, Korle Bu, Accra. Daily Graphic / Ghana
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Srpasta anemija – uzroci, simptomi i liječenje Anemija srpastih stanica je nasljedni oblik anemije – stanje u kojem ne postoji dovoljno zdravih crvenih krvnih stanica za nošenje adekvatne količine kisika u vašem tijelu. Normalno, crvene krvne stanice su fleksibilne i okrugle te se kreću lako kroz krvne žile. U anemije srpastih stanica, crvene krvne stanice postaju krute i ljepljive te su u obliku srpa ili polumjeseca. One mogu zapeti u male krvne žile, što može usporiti ili blokirati protok krvi i kisika u dijelovima tijela. Ne postoji lijek za većinu ljudi s anemijom srpastih stanica. Međutim, tretmani mogu ublažiti bol i spriječiti daljnje probleme povezane s anemijom srpastih stanica. Uzroci srpaste anemije Anemija srpastih stanica je uzrokovana mutacijom u genu koji je odgovoran za proizvodnju hemoglobina, spoj koji daje krvi svoju crvenu boju. Hemoglobin omogućuje crvenim krvnim zrncima da prenose kisik iz pluća u sve dijelove tijela. U anemije srpastih stanica, abnormalni hemoglobin uzrokuje da crvene krvne stanice postanu krute i ljepljive. Prenosi se s generacije na generaciju, autosomnim recesivnim nasljeđivanjem . To znači da i majka i otac moraju imati neispravan oblik gena da bi i dijete naslijedilo srpastu anemiju. Ako dvije osobe sa srpastim stanicama očekuju dijete šanse za oboljenje njihovog djeteta izgledaju ovako: - 25 posto šanse da se rodi dijete s normalnim hemoglobinom - 50 posto šanse da se rodi dijete koje je također nositelj - 25 posto šanse da se rodi dijete s anemijom srpastih stanica Simptomi srpaste anemije Znakovi i simptomi anemije srpastih stanica obično se pojavljuju kod djeteta starog četiri mjeseca i mogu uključivati anemiju koja uzrokuje umor, bolove u tijelu koji mogu varirati u intenzitetu i trajanju, a mogu se pojaviti i u kostima. Kao simptom može se pojaviti i oticanje ruku i nogu. Nadalje, mogu se pojaviti česte infekcije, odgođen rast te problemi s vidom. Svi ti simptomi pojavljuju se zbog premale opskrbe tijela kisikom. Liječenje srpaste anemije Presađivanje koštane srži je jedini potencijalni lijek za anemiju srpastih stanica. No, pronalaženje donora, teško je i postupak ima ozbiljne rizike, uključujući i smrt. Liječenje je obično usmjereno na ublažavanje simptoma i sprječavanje komplikacija koje uključuje redovito posjećivanje liječnika koji će provjeravati broj i stanje crvenih krvnih stanica. Tretmani mogu uključivati lijekove za smanjenje bolova, ali i transfuziju krvi te dodatan kisik. Lijekovi koji se koriste za lječenje srpaste anemije kod djece su antibiotici i to najčešće penicilin koji pomaže u sprječavanju infekcija kao što je upala pluća koja može biti opasna po život. Znanstvenici proučavaju nove tretmane za anemiju srpastih stanica, a jedna od njih je i genska terpija, korištenje dušikovog oksida (osobe sa srpastom anemijom imaju nisku razinu dušikovog oksida u krvi) koji pomaže u održanju otvorenosti krvnih žila i smanjuje ljepljivost crvenih krvnih stanica. Znanstvenici također proučavaju i razne lijekove kako bi smislili način da povećaju proizvodnju fetalnog hemoglobina koji zaustavlja formiranje srpastih stanica. Sensifer Plus Djelotvoran kod anemije, bez nuspojava. bioENER Mix Smanjuje umor i iscrpljenost.