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Top Health Tips on Living with Sickle Cell Disease

World Sickle Cell Day - What Should You Know?

Fellowship In PCCM, Fellow-Pediatric Flexible Bronchoscopy, Fellowship In Pediatric Cardiac Critical Care, D.C.H., M.B.B.S
Pediatrician, Ahmedabad
World Sickle Cell Day - What Should You Know?
World Sickle Cell Day is observed each year on June 19th to raise the public awareness regarding this disease and its treatment methods. Sickle Cell disease is an inherited transmitted deformity/abnormality of haemoglobin. It is an inherited form of anaemia. This abnormality generates changes in the shape of red blood cells from round to crescent, which reduces the oxygen carrying capacity and slows down the flow of blood to various parts of the body.

Generally, red blood vessels are round and flexible, passing easily through the blood vessels. The red blood cells become sticky and rigid in sickle cell anaemia. Sickle cells last 10 to 20 days, which causes anaemia because of the disorder of red blood cells, while healthy red blood cells could live up to 120 days.

Millions of people around the world are affected by Sickle Cell Anemia. There is no specific cure for this disease as Sickle cell anaemia is not contagious, it is hereditary. Sickle cell anaemia is named so for the reason that red blood cells are crescent-shaped or sickle-shaped, and anaemia signifies lack of blood. There is a difference between iron-deficiency anaemia and sickle cell anaemia.

Symptoms

Sickle Cell Disease (SCD) has many symptoms which includes: serious anemia, episodes of suffering, additionally a range of complications, heart failure, dizziness, chronic kidney disease (CKD), acute chest syndrome (ACS), chest pain, gallstones, pulmonary hypertension, immune deficiency, paralysis or stroke, splenomegaly, joint damage mainly in the hip joint, foot ulcers, delayed puberty, retinopathy, and endocrine disorders.

Diagnosis

Sickle Cell Disease is diagnosed by blood tests.

Treatment

Bone marrow transplant or stem cell transplant in patients usually below the age of sixteen is the only available cure for the disease.

Aim of the World Sickle Cell Day

The aim of the World Sickle Cell Day is to support and promote research for the patients in order to help them upgrade their overall quality of life to the maximum possible extent. It also aspires to increase awareness for sickle cell disease disorder in the general public so that they know how to deal with patients suffering from the disease. Moreover, the World Sickle Cell Day has the objective of raising awareness of the importance among generations of pre-marital screening to lessen the transmission of Sickle Cell Disease.

Target Groups for Awareness Programs

Though it is desirable that everybody knows about Sickle Cell Disease, the target groups for the awareness programs include health decision-makers, sickle cell anaemia sufferers and their families, health organizations and associations, people who are planning to get married, and the health workers including health educators, physicians, and nurses.

On the World Sickle Cell Day, different societies provide advocacy, information, and advice for raising awareness. They also provide educational and welfare grants, support services, annual children s holiday, home help, hospital visits, mentoring and befriending through holding talks, assisting into research and training, lobbying for better service provision, patient education seminars, and campaigning.
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सिकल सेल के बचाव - Sickle Cell Ke Bachaw!

Bachelor of Ayurveda, Medicine and Surgery (BAMS)
Ayurvedic Doctor, Lakhimpur Kheri
सिकल सेल के बचाव - Sickle Cell Ke Bachaw!
सिकल सेल रोग असामान्य जीन से उत्पन्न एक आनुवांशिक रोग है जो माता-पिता से प्राप्त होते हैं. शरीर के रक्त मे लाल रक्त कण का आकार सामान्यतः उभयातल डिस्क के तरह होता है जो रक्तवाहिकाओं में आसानी से गमन करते हैं. पर सिकल सेल की स्थिति में ये लाल रक्त कण उभयातल डिस्क के तरह न होकर अर्धचंद्राकार हंसिया (सिकल) के तरह हो जाता है. जिससे रक्तवाहिकाओं में इसका संचरण सही ढंग से नहीं हो पाता है. जिससे शरीर के विभिन्न अंगों में रक्त पहुँचने में अवरुद्ध होता है. ये असामान्य लाल रक्त कण जो हंसिया या सिकल के तरह होता है इसे ही सिकल सेल कहते है तथा जब इस स्थिति से शरीर में रोग हो जाती है तो उसे सिकल सेल रोग कहते हैं. ये सिकल सेल कठोर व चिपचिपा होता है. इसका आकार हंसिया (सिकल) के तरह होने के कारण रक्त प्रवाह अवरुद्ध हो जाता है जिस कारण दर्द होता है और विभिन्न अंग तो क्षति भी पहुँचती है. सामान्य लाल रक्त कण की आयु 120 दिन होती है. जबकि सिकल सेल लाल रक्त कण की आयु 10 से 20 दिन होती है. इस प्रकार सिकल सेल के जल्द नष्ट हो जाने व इसके श्हरीर के विभिन्न भाग में पहुँचने में दिक्कत होने से शरीर में खून की कमी एनीमिया रोग हो जाती है.

क्या है सिकल सेल?
सिकल सेल की बीमारी खान-पान, छुआछूत या अन्य तरह से होने वाले बीमारी न होकर यह एक जेनेटिक बीमारी है जो जीन में हुये परिवर्तन के कारण होती है. चिकित्सा इतिहास के अध्ययन से पता चलता है कि हजारों वर्ष पूर्व कुछ स्थानों पर हमारे हिमोग्लोबीन के जीन्स में परिवर्तन हुये जिस कारण लाल रक्त कण का आकार गोलाकार से बदलकर अर्द्ध चंद्राकार हँसिये (सिकल) के रूप में हो गया. यह परिवर्तन उन क्षेत्रों में ज्यादा हुआ जहाँ मलेरिया बहुतायत में पाया जाता था. परिणामस्वरूप यह रोग अविकसित आदिवासी दुरूह क्षेत्र के जनजातियों में ज्यादा पाया गया. यह बीमारी अफ्रीका, बहरीन, तुर्की, ग्रीस, सऊदी, अरेबिया के साथ-साथ भारत में बहुतायत में पाया जाता है. महत्वपूर्ण बात है कि विश्व में समस्त सिकल सेल मरीजों में से आधे से ज्यादा भारत में हैं.

यदि बच्चे को माता व पिता दोनों से सिकल सेल के जीन मिले हों तो बच्चे सिकल सेल का रोगी होता है. पर यदि बच्चे को माता या पिता में से किसी एक से ही सिकल सेल के जीन मिले हों तो इन बच्चे में रोग के कोई लक्षण दिखाई नहीं पड़ते हैं व उन्हें कोई इलाज की जरूरत नहीं होती है.

सिकल सेल रोग से बचाव-
सिकल सेल का रोग एक बहुत ही गंभीर बीमारी है. और इसकी रोकथाम जरूरी है. पर इसके इतनी बड़ी समस्या होने के बावजूद भी इस मामले में खामोशी होने का वजह है कि लोग इसे आनुवांशिक रोग मानकर इसलिए इसपर ध्यान नहीं देते हैं क्योंकि उनका मानना होता है कि आनुवांशिक रोग का कोई इलाज नहीं है. यह सत्य है कि आनुवांशिक रोग को जड़ से नष्ट करने का कोई उपाय नहीं है पर रोकथाम द्वारा इसे बढ़ने से रोका जा सकता है. शादी से पहले सिकल कुंडली मिला ली जाये तो 70 प्रतिशत तक इस रोग को कंट्रोल किया जा सकता है. इसके लिए शादी से पहले लड़का व लड़की दोनों का रक्त जाँच कर यह देख लेना चाहिए कि इनमें सिकल सेल के जीन तो नहीं है. यदि दोनों में सिकल सेल पाये जाते हैं या दोनों सिकल सेल रोगी हैं तो उन्हें शादी नहीं करनी चाहिए. इस प्रकार ऐसे लोगों को आपस में शादी न करके सिकलग्रस्त बच्चे की उत्पत्ति रोकी जा सकती है.

सिकल कुंडली का मिलान करके ही शादी करने के लिए सामाजिक संगठन को आगे आना चाहिए. क्योंकि जनजागृति ही सिकल सेल रोग से बचने का एकमात्र उपाय है. साइप्रस व बहरीन जैसे देशों में शादी से पहले सिकल की जाँच हेतु खून जाँच अनिवार्य कर दिया गया है. इस प्रकार के व्यवस्था से वहाँ सिकलग्रस्त बच्चे के जन्म में काफी कमी आयी है.

सिकल रोग ग्रस्त बच्चे में खून की कमी होती है जिस कारण बच्चे कमजोर होते हैं व इन्हें संक्रमण का खतरा बढ़ जाता है. ऐसे बच्चों का टीकाकरण व अन्य जरूरी दवाएँ देकर उन्हें दीर्घायु बनाया जा सकता है. सिकल रोग के कारण होने वाले प्रभाव व विकारों का उचित इलाज से सिकल रोगी को लंबा जीवन मिल सकता है. जमैका जैसे देशों में सिकल रोगियों का उचित इलाज व पुनर्वास की सुविधा है जिस कारण वहाँ सिकल ग्रस्त लोग भी लंबे जीवन जी रहे हैं. पर भारत में व्यवस्था के अभाव में सिकल ग्रस्त लोगों की आयु कम है.
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World Sickle Cell Day - All You Need To Know About The Sickle Cell Disease!

Bachelor of Ayurveda, Medicine and Surgery (BAMS)
Ayurvedic Doctor, Lakhimpur Kheri
World Sickle Cell Day - All You Need To Know About The Sickle Cell Disease!
Sickle Cell Diseases or Sickle Cell Anaemia is considered to be the foremost genetic disease that today s world is grappling with. W.H.O. has been fighting to spread awareness regarding the same since 2006 and the U.N. joined it in 2008. On the 19th of June, 2009 the first World Sickle Cell Day was celebrated. While it is not a public holiday, it allows organizations and institutions, celebrating it, to make people aware of the existence of this disease.

So what is Sickle Cell Disease? Here is all you need to know about it.

Primary cause: It is a genetic disorder that a child gets from its parents. It is important to note that one can only get sickle cell anaemia if both of one s parents are carriers. According to a W.H.O. estimate, close to 5% of the entire population carry the mutated genes causing this disorder.

How it works: This disorder affects one's RBCs or red blood cells. We ve all seen images of red blood cells in biology textbooks. Thus, by its name one automatically imagines round or circular red cells. Due to the mutation of haemoglobin, these cells in the affected patient change their shape. From circular and flexible they become crescent-shaped and stiff. The name of the disease comes from the sickle-like shape that the mutated blood cells acquire.

Key effects: The changing of the shape of our red blood cells may not sound too severe. However, its effects range from bouts of pain caused by lack of oxygen to strokes, blindness, and even erectile dysfunction! The changed shape of the blood cells restricts their movement. Since oxygen is sent to different parts of our body via these red blood cells, their restricted movement results in a reduced supply of the same. This leads to excessive pain in certain body parts. In extreme cases, lack of oxygen becomes the cause of other problems such as blindness, heart attack or stroke, and even bone damage! Continuous lack of oxygen to certain organs can lead to their failure. Thus, without treatment, this disease leads to a very slow and painful death.

Cure: Treatment of this disease refers to the treatment of its symptoms or complications to prevent death and ease the pain of the patient. This is why more research is required to find a way to prevent it altogether or cure the mutation.
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What Is the Importance of World Sickle Cell Awareness Day?

Bachelor of Ayurveda, Medicine and Surgery (BAMS)
Ayurvedic Doctor, Lakhimpur Kheri
What Is the Importance of World Sickle Cell Awareness Day?
Sickle cell Anaemia is a hereditary blood disease that is passed on to the child of often healthy parents via genes that get mutated. WHO estimates put the number of patients at 300k babies and the number of mutated gene carriers at 5% of the world s population. Despite this large number, most people are unaware of the several ways in which one can manage and prevent this disease. To rectify this error, W.H.O. has been attempting to improve people s knowledge regarding the disease since 2006. The primary purpose is to create awareness about the different methods of prevention and cure of Sickle Cell Anaemia. One can say there are 4 key significances of the day,

Spread Awareness - Sickle Cell Anaemia is considered to be one of the most dangerous genetic diseases in today s world. Yet, maximum people are unaware of not just the methods of combating it, but also its presence. The worldwide recognition of 19th June as World Sickle Cell day makes people aware that such a disease exists. Activities undertaken by various organisations and government institutions further educate the people about its symptoms and methods of cure. There are also several myths associated with it that need to be removed. Like any other disease, if people are just told that it s dangerous and has been declared to be a worldwide problem by W.H.O., they are bound to panic. Hence, campaigns are required to not just make people aware of this disease but also educate them about its different elements.

Make healthcare services more accessible - activities organized to celebrate the day include the screening of patients at health camps. If one becomes aware of the fact that they are a gene carrier, they ll be aware of the risk of diseases their children can have and can take appropriate prevention measures or at least be aware of the situation before they engage in a relationship with another carrier. Moreover, there are several methods of cure and prevention ranging from immunisation to care packages varying by country. New parents should be aware of this.

Provide support to countries struggling with this disease - W.H.O. and the U.N. themselves conduct several activities that help equip certain countries better for combating this disease.

Encourage research into the treatment of the disease - while there are already several forms of treatment available for this disease, there is always scope for improving our understanding of the same. While prevention and immunisation are the easiest ways to fight this worldwide problem, it is only through research into the root causes of the problem that we can completely eradicate it.

What Is The Sickle Cell Test?

Bachelor of Ayurveda, Medicine and Surgery (BAMS)
Ayurvedic Doctor, Lakhimpur Kheri
What Is The Sickle Cell Test?
Sickle cell Anemia or Sickle cell disease is a part of a group of genetic blood cell disorders that is inherited from parents who are carriers of this particular gene mutation. This disease causes a transformation of red blood cells from a circular shape to a sickle-like shape. Hence the name. While it may not sound too serious, the change in shape changes the way these blood cells move within the body. Restricted movement of blood cells leads to a restricted supply of blood and ultimately, a restricted supply of oxygen. This can lead to major health issues and depending on the severity, even death. Some of these include excessive pain, organ failure, stroke, and even blindness.

A sickle cell test is a blood test which identifies the shape of the patient s red blood cells, thus, identifying whether or not they have this disease. It has now been included as a part of the list of tests conducted on newborn babies in the U.S. However, this does not mean that this test is functional only on babies. Adults who may not have been screened at birth can also get this test done to identify whether or not they have this disease.

This test is extremely important as it helps your doctor diagnose your problems and give better solutions. Sickle Cell Disease patients are susceptible to various infections, especially when they are babies. Hence, knowing that someone has this disease makes it easier for doctors to treat it.

There are no pre-conditions or preparations required for this test. However, if you are an adult getting tested then you may not get the same within ninety days of receiving blood externally. Blood transfusion can change the composition of haemoglobin you have and hence, make it difficult for the test to identify whether or not you have the disease.

The test is similar to a simple blood test. The nurse or doctor ties a band on your arm and then removes blood via a puncture. For a baby, they use a special tool instead of an injection to make a puncture in its heel and extract the blood.

The test is an extremely simple procedure. There are no risks involved. One can drive themselves home after the test as well. In rare cases, there may be bouts of lightheadedness. But this is extremely rare and is normally caused when the patient is already weak or undergoing some other issue.

The test results are simple to understand. It helps the doctor identify if you have one or both abnormal sickle cell genes. If you just have one of them, then you shall be diagnosed as having the sickle cell trait. If you have both, then you ll mostly be diagnosed as having sickle cell disease. The doctor will check on the symptoms and order follow up tests to determine the severity of the disease.

What Are The Harmful Complications Of Sickle Cell Anaemia?

Bachelor of Ayurveda, Medicine and Surgery (BAMS)
Ayurvedic Doctor, Lakhimpur Kheri
What Are The Harmful Complications Of Sickle Cell Anaemia?
Sickle Cell Anaemia or Sickle Cell Disease is a congenital disorder that causes the red blood cells to contort into a sickle shape. The sticky rigid cells may get stuck in the smaller blood vessels and in turn, prevent or slow down the process of oxygen and blood flow to other body parts. This painful condition referred to as a sickle cell crisis may lead to a wide range of complications over time.

Look out for the following complications

Here is a look at the various and most common complications of sickle cell anaemia

Damage to the organs - Although sickle cell anaemia does cause blood to have less oxygen, the condition is not serious enough to damage your organs. However, the risk of organ damage develops when a sickle cell is caught in the blood vessel and restricts the flow of blood and eventually causing permanent damage to organs, such as the liver, spleen, and kidneys.

Even though damage to the organs is irreversible, it is possible to delay the process if the condition is detected at an early stage. Therefore, regular health checkups and blood tests are strongly recommended for people with sickle cell anaemia.

Acute chest syndrome - This may occur if a sickle cell ends up blocking the blood vessels in your lungs. Acute chest syndrome is a potentially fatal condition characterized by chest pain, shortness of breath, coughing and fever. This requires immediate medical intervention.

Hand-foot syndrome - This condition is also known as dactylitis. It occurs due to the blockage of the blood vessels of the feet or hands. For some people, this could be the initial symptoms of sickle cell anaemia characterized by fever and painful swelling in the feet or hands. The treatment of hand-foot syndrome primarily involves a combination of certain pain medications and drinking fluids.

Stunted or delayed growth and development - Red blood cells provide your body with oxygen and other nutrients essential for healthy growth and development. However, due to poor or lack of oxygen flow, a majority of children and teenagers may experience delayed growth and later onset of puberty. It may also cause infertility in men.

Loss of vision - In some cases, the smaller blood vessels supplying blood to your eyes may become blocked with sickle cells over time, thus causing severe damage to the retina. Some people even develop excess blood vessels due to the reduced flow of oxygen. Both the conditions man contribute to the loss of vision. People with sickle cell anaemia are hence advised to undergo eye testing at least once a year.

Gallstones - The breakdown of red blood cells results in the formation of bilirubin your body. For a person with sickle cell anaemia, the breakdown happens at a faster rate resulting in excess production of bilirubin. A high amount of bilirubin in the body may lead to the formation of gallstones in the gallbladder.

The most common symptoms of gallstone include nausea, vomiting, as well as pain in the back, shoulders, and abdomen. While medications are often prescribed to remove gallstones, surgery may be required in severe cases.

Splenic sequestration - This occurs when a cluster of sickle cells block the splenic vessels. Common symptoms include rapid breathing, pale lips, extreme thirst, sudden weakness, and abdominal pain. Blood transfusion may be required urgently to treat the condition.

Infections - Damage to the spleen caused by sickle cells may make you prone to a range of infections, including pneumonia, common flu, and meningitis. These types of infections are usually characterized by signs, such as high fever, coughing, body aches, and fatigue, and can affect your immunity before you know it.

Leg ulcers - Leg ulcers are common in people with sickle cell anaemia. A person who develops leg ulcers (open sores in the skin of the legs) is likely to notice swelling, heaviness, or an aching sensation in the legs.

Topical ointments, antibiotics, and compression bandages may be used to manage the condition.

Stroke - A blockage in the blood vessels in the brain may lead to a stroke. Immediate medical attention is required if you experience symptoms, such as slurred speech, headaches, difficulty memorizing, walking, or moving the arms, drooping or numbness on one side of the body etc.

Even though complications of sickle cell anaemia are not always preventable, there are certain ways to lower their risk and severity. Here is what you can do

Get at least 150 minutes of moderate exercise every week
Eat a healthy, balanced diet comprising of sufficient amounts of fruits, vegetables, and whole grains
Drink enough water and fluids
Reduce stress levels
Be aware of the altitude and temperature
Take precautions to avoid infections
Quit smoking
Most importantly, follow up with your doctor if you have sickle cell anaemia to get tested for any of the above conditions and to reduce the risk of harmful complications.

How To Prevent Sickle Cell Crisis?

Bachelor of Ayurveda, Medicine and Surgery (BAMS)
Ayurvedic Doctor, Lakhimpur Kheri
How To Prevent Sickle Cell Crisis?
Sickle Cell Anaemia or Sickle Cell Disease is a genetic disorder where the haemoglobin of the affected individual gets mutated, causing their red blood cells to change shape and become crescent-shaped. As a result of this change in shape, the movement of these blood cells becomes restricted. Since haemoglobin carries oxygen, its restricted movement results in a lack of oxygen in various body parts and organs. This causes the patient extreme pain and in some cases leads to organ failure and death!

One can receive the mutated gene from one of the parents and become a carrier. If both of one s parents are carriers, then they ll most certainly get the disease! With the world s exploding population the number of Sickle cell disease patients has been on the rise. Following are the best ways to prevent this crisis,

Blood tests - blood tests help screen babies and adults and identify those with the disease. Recently, this test has been included in the screening of babies in the U.S. However, adults who wish to check for this disease can also get it done. Once you test positive, your doctor will decide if you need further tests to check the severity of your disease.

Ultrasound test - this test helps identify patients with a risk of getting a stroke. Since doctors still haven t found a way to remove this disease completely, the treatment involves removing or reducing its symptoms. Hence, if you know you are at risk of a stroke, your doctor can take preventive measures such as increased blood transfusions, etc.

Pre-birth screening - parents who don t want to deal with children having this disease often leave them to fend for themselves. To prevent this outcome, doctors can conduct tests on an unborn baby and confirm whether or not they ll have sickle cell anaemia. This allows couples to decide whether or not to undertake the responsibility for caring for such a patient.

Treatment - other than medication to relieve its symptoms there are two key forms of surgeries that can be performed. The first is blood transfusion. In this procedure, healthy red blood cells from a donor s blood are taken and infused into the patient. This increases the presence of red blood cells in their body and reduces the risks of death by a stroke. The second is a bone marrow transplant, where they replace the affected bone marrow with that of a donor. Bone marrow is that part of the bone which is responsible for the production of red blood cells. The problem with this procedure is that one must have an exact donor which normally can only be a sibling. What s more, your sibling must not have the disease themselves to be able to help.