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Sickle Cell Anemia

Sickle cell anemia is a hereditary condition characterized by destruction of red blood cells in the body. It is noticed that sickle cell anemia affects people of African ancestry and those of Mediterranean and Middle Eastern descent. The soft round red blood cells develop an abnormal shape that resembles a sickle or crescent moon. Their shape stops them from flowing easily and they get clogged up inside smaller blood vessels. This stops the blood from flowing properly. Important organs like the brain, heart, or kidneys need constant blood flow to stay healthy. Although our body attacks and destroys these sickle cells, it can't make new blood cells fast enough to replace the older ones. This results in the decrease in number of red blood cells leading to anemia. Sickle cell anemia is generally caused by genetic defects or disease. A child with sickle cell anemia has inherited a defective hemoglobin gene.


Sickle shaped blood cells get stuck within the small blood vessels and obstruct blood flow to some parts of the body, thereby leading to pain. Symptoms of sickle cell anemia include fatigue, breathlessness, rapid heart rate, delayed growth and puberty, susceptibility to infections, excessive penis pain, chest pain and decreased fertility. Since the spleen is damaged by the sickle cells, the resistance to infections is reduced considerably. Hands and feet tend to swell (Hand-foot syndrome) and the body faces stunted growth. Retinal damage can occur due to the obstruction of blood flow to the eyes. In about 10% of the cases, a stroke can occur if the sickle cells block blood vessels in the brain. Acute chest syndrome is another complication of this form of anemia. In this condition, the patient suffers symptoms similar to pneumonia and will need to be treated in a hospital.


There is no cure for sickle cell anemia but treatment can be followed to prevent complications and improve the quality of life of the patient. The patient will need to be under the guidance and care of a hematologist or genetic counselor. Blood test for hemoglobin 5 gives an indication as to the presence of this disease. A microscopic analysis of a blood sample will exhibit the distinctly shaped sickle cells. Bone marrow transplant can offer potential cure for this form of anemia, provided the right donor is found. Red blood cells from a donor can be given intravenously to a patient. But it carries inherent risks. Penicillin is given to keep young affected kids from life-threatening infections. Painkillers like acetaminophen and ibuprofen can relieve the pain associated with sickle cell anemia. Children diagnosed with sickle cell anemia must be given regular childhood vaccinations as well as Hib (Hemophilus Influenzae B) vaccine and the pneumococcal vaccine.


Pulmonary Infarction

Infarction means 'tissue death' in medical terminology. It is caused by obstruction of blood supply to the tissue leading to lack of oxygen. Infarct, which refers to the resulting lesion is derived from Latin, 'infarctus' which means 'stuffed into'. Pulmonary infarction refers to the death of a small area of lung resulting from pulmonary embolism. It occurs in a small, dead end pulmonary artery.


How does pulmonary infarction occur ?

Pulmonary infarction results from free floating thrombus, when many material substances including fat, tumor, septic emboli, air, and amniotic fluid and injected foreign material may form an emboli and move to the pulmonary circulation. In other words, plugging of a branch of the pulmonary artery by a clot (thrombosis) or by a piece of clot carried by the blood stream to the lung from a thrombus located elsewhere can result in pulmonary infarction. The involved area of the lung ceases to function and complication of the thrombosed veins leads to heart disease.


Causes of pulmonary infarction

The most common cause of pulmonary infarction is pulmonary embolism, but there are other conditions which can cause pulmonary infarction including cancer, and autoimmune diseases such as lupus. Sometimes, this condition may occur following a surgery. Other underlying conditions especially in children with pulmonary emboli include sickle cell disease, nephrotic syndrome, chemotherapy and Inherited hyper coagulable state and Vasculitis.


Symptoms of pulmonary infarction

Symptoms associated with pulmonary infarction include shortness of breath, chest pain, and blood sputum or hemoptysis. Sudden piercing pain in the chest which often radiates to the shoulder is noticed. Difficulty in breathing, irritating cough and blood tinged sputum are other signs. Persistent hiccups are present. Most often the patient is anxious with a rapid pulse, sweats profusely and has an elevated body temperature. In some severe cases, the patient may be in a state of shock.


Diagnosis of pulmonary infarction

Reflex broncho constriction is often associated with pulmonary embolism. Increased breathing and decreased pulmonary compliance with diminished surfactant levels may occur due to pulmonary infarction. This contributes to increased work of breathing and diminished oxygen levels. In sickle cell disease, there is sickling of RBCs within the small blood vessels of the lungs due to dehydration as a result of fever, Tachypnea (rapid breathing) and decreased intake, which can precipitate in a cycle of relative de oxygenation that further exacerbates the sickling tendency. Many also suffer a component of reactive airway disease and oxygenation is further decreased due to this factor.


Morbidity may include pulmonary hypertension, right ventricular failure and Cor Pulmonale, paradoxical embolization in patients with intracardiac defects, and sometimes side effects of medications used to treat pulmonary embolism. If pulmonary embolism is large, there could develop right ventricular strain and right heart failure as there is sudden increase in pulmonary artery pressure leading to right heart failure. A sudden pressure in the right ventricle can cause a leftward shift of the intraventricular septum, which may result in a classic obstructive shock, thereby impairing left ventricular filling.


Treating pulmonary infarction

It is timely treatment that is vital. If symptoms of pulmonary infection develop while at home, consult a physician at once. In case, shock develops, it is essential to get first aid treatment. Many times, patients are already in the hospital when pulmonary infarction occurs. Administration of oxygen, use of anticoagulants and prevention of infection are some other suggested line of treatment. Surgery may also be indicated.



Ischemic bowel disease

Ischemic bowel disease is a medical condition that occurs due to lack of blood flow to the intestines. This disease is seen mostly in older people. If the condition is mild, the disease may improve without treatment. But Ischemic bowel disease can result in an emergency situation in some cases. Ischemic bowel disease results from blocked or narrowed arteries. Tumors or blood clots can be the cause for reduced blood supply. Atherosclerosis is another cause for narrowed arteries.


Patients suffering form Ischemic bowel disease notice abdominal pain, after eating. There might be rectal bleeding in some cases. Symptoms of ischemic bowel disease include abdominal cramps, nausea and vomiting. The abdomen appears distended and there might be episodes of diarrhea. Certain conditions such as colon cancer, diabetes, congestive heart failure, peripheral vascular disease and sickle cell cancer can increase a person's risk of developing Ischemic bowel disease.


Diagnosis can be done based on abdomen x-rays and colonoscopy. Angiography of the arteries supplying blood to the bowel helps in detecting the severity of the condition. Antibiotics help in minimizing the infection. Surgery is resorted to in severe cases.

Tags: #Sickle Cell Anemia #Pulmonary Infarction #Ischemic bowel disease
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Collection of Pages - Last revised Date: December 8, 2023