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Sickle Cell Anemia News

Apr. 11, 2024 — A drug approved to treat pulmonary arterial hypertension may be effective at managing hypertension and end-organ damage in patients with sickle cell disease, according to a new study. An early phase ...

Jan. 30, 2023 — Researchers have designed a microfluidic device, or 'spleen-on-a-chip,' that can model how the spleen's filters become clogged by sickled red blood cells, leading to a life-threatening ...

Feb. 16, 2022 — Sickle cell anemia is an inherited blood disorder where red blood cells become sickle/crescent shaped. It causes frequent infections, swelling in the hands and legs, pain, severe tiredness and ...

Jan. 14, 2022 — A world-first study has revealed how space travel can cause lower red blood cell counts, known as space anemia. Analysis of 14 astronauts showed their bodies destroyed 54 percent more red blood cells ...

Dec. 13, 2021 — An experimental gene therapy for sickle cell disease restored blood cells to their normal shape and eliminated severe pain crises for years after treatment, a multicenter study has ...

Dec. 10, 2021 — Organ-on-a-chip technology has provided a push to discover new drugs for a variety of rare and ignored diseases for which current models either don't exist or lack precision. In particular, ...

Nov. 11, 2021 — Studying mice, researchers have developed a method of stem cell transplantation that does not require radiation or chemotherapy. Instead, the strategy takes an immunotherapeutic approach, combining ...

Sep. 21, 2021 — A multifaceted microfluidic in vitro assay is helping to identify the role of hypoxia on red blood cell aging via the biomechanical pathways. It holds promise for investigating hypoxic effects on the ...

Aug. 6, 2021 — Pooled results from phase 3 clinical trials demonstrate that roxadustat is efficacious and may be comparable to placebo in studies done to date for treating anemia in patients with chronic kidney ...

Sickle Cell Anemia And Acute Chest Syndrome

Sickle cell anemia causes sickle-shaped cells to block blood flow to the lungs, leading to acute chest syndrome. Common treatment methods involve oxygen supplementation and pain management.

Sickle cell anemia is a genetic blood disorder that changes the shape of red blood cells, making them sickle-shaped (like a "C") instead of round.

These sickle-shaped cells can block blood flow, leading to various complications, such as acute chest syndrome (ACS), a severe condition that can occur in people with sickle cell anemia and cause significant respiratory distress.

Learn more about the relationship between sickle cell anemia and acute chest syndrome, including symptoms, underlying causes, and possible treatment options.

Sickle cell anemia can often lead to complications such as acute chest syndrome.

The signs and symptoms of acute chest syndrome can vary, but it often presents with pneumonia-like symptoms, which may include:

sudden and sharp pain in the chest.

dyspnea (shortness of breath) or tachypnea (rapid shallow breathing)

wheezing

hypoxemia (low oxygen level) in the blood, leading to cyanosis (a bluish coloration to the skin or fingernails)

fever

cough

vaso-occlusive pain (severe pain episodes due to lack of blood circulation)

These symptoms require immediate medical attention as acute chest syndrome can progress rapidly and become life threatening.

While the exact cause of acute chest syndrome is unknown, researchers believe it occurs when sickle-shaped red blood cells obstruct blood flow in the lungs, leading to inflammation and reduced oxygen levels.

Several factors are linked to acute chest syndrome, including:

Infection: Bacterial or viral infections can trigger acute chest syndrome. Common pathogens include Mycoplasma pneumoniae and Chlamydia pneumoniae.

Fat embolism: When sickled red blood cells block small blood vessels in the lungs, this can lead to a fat embolism (fat entering the bloodstream and blocking blood vessels).

Pulmonary infarction: This health event involves blockage of blood flow to lung tissue, causing tissue death.

Bone marrow fat embolism: This health event occurs when bone marrow releases fat particles into the bloodstream due to a sickle cell crisis. The particles lodge in the lungs.

There are several risk factors that can increase the chance of sickle cell anemia developing into acute chest syndrome.

These include:

Recent trauma or surgery: Pain and medications postsurgery can lead to shallow breathing and low oxygen levels, resulting in sickling in the lungs that causes acute chest syndrome.

Infections: Respiratory infections like asthma and pneumonia are common triggers.

Hypoxemia: Low oxygen in the blood and lungs promotes the formation of sickled cells, often due to lung infections.

Vaso-occlusive crisis (VOC): This occurs as a complication of sickle cell disease (SCD). VOC leads to sudden or gradual excruciating pain in the chest, back, or extremities and may lead to acute chest syndrome.

Smoking: Smoking increases the risk and severity of acute chest syndrome, especially in children.

COVID-19: A 2020 case study suggests that people with COVID-19 are more likely to develop acute chest syndrome.

Young age: Acute chest syndrome can occur at any age, with peak onset in children with SCD between ages 2 and 4 years.

Acute chest syndrome can lead to severe complications if not treated promptly. These include:

A healthcare professional will begin by taking a detailed medical history, focusing on any previous episodes of acute chest syndrome, sickle cell crises, recent infections, or surgeries.

They will then perform a physical exam to check for signs of respiratory distress, such as rapid breathing, wheezing, decreased breath sounds, and other symptoms.

To confirm the diagnosis, a healthcare professional will order some tests. These may include:

Chest X-ray: A chest X-ray can help detect lung infiltrates and areas where fluid, inflammatory cells, or both accumulate in the lungs. These are common indicators of acute chest syndrome.

Blood tests: A complete blood count (CBC) helps identify anemia and an increased white blood cell count. Additional blood tests, including C-reactive protein (CRP) and erythrocyte sedimentation rate test (ESR test), are done to assess inflammation or infection.

Arterial blood gas (ABG): This test involves taking a blood sample from an artery, usually in the wrist, to assess oxygen and carbon dioxide levels in the blood and blood pH.

Bronchoscopy: In certain situations, a healthcare professional may perform a bronchoscopy. During this procedure, a flexible tube with a camera is inserted through the nose or mouth into the lungs to visualize the airways and collect samples for further testing.

Computed tomographic angiogram (CTA): This test involves a special type of X-ray that uses contrast to assess blood vessels. It's commonly used to diagnose pulmonary embolism (blood clot in the lung).

Treatment for acute chest syndrome addresses the underlying causes and relieves symptoms. Possible treatment options may include:

Oxygen supplementation: This treatment can help correct low oxygen saturation levels and maintain adequate oxygen levels in the blood.

Pain management: A healthcare professional may prescribe pain medications like ketorolac (Toradol), acetaminophen (Tylenol), or opioids for severe pain.

Rehydration: Fluid administration can help correct dehydration since hypovolemia (low blood volume) can worsen sickling.

Antibiotics: If a bacterial infection is suspected or confirmed, a healthcare professional may prescribe antibiotics such as azithromycin (Zithromax) or ceftriaxone (Rocephin).

Blood transfusions: When hemoglobin levels fall below 7 grams/deciliter, healthcare professionals recommend blood transfusions to reduce the number of sickled cells and improve oxygen supply.

Incentive spirometry: This treatment can help promote deep breathing and lung expansion by encouraging you to use incentive spirometry every 2 hours while awake to help prevent atelectasis (collapse of part of the lung).

How do you treat sickle cell pneumonia?

Healthcare professionals typically prescribe antibiotics such as ceftriaxone (Rocephin) and cefuroxime (Zinacef) to treat sickle cell pneumonia.

Why do people with sickle cell anemia have difficulty breathing?

People with sickle cell anemia can have difficulty breathing as the condition can cause pressure to build up in the lungs.

Does sickle cell anemia affect the heart?

Sickle cell anemia can affect multiple organs, including the heart, lungs, kidneys, and liver.

Acute chest syndrome is a serious and life threatening complication of sickle cell anemia. Early recognition of symptoms, prompt diagnosis, and effective treatment are important in managing this condition.

If you have sickle cell anemia, consider working closely with your healthcare team to monitor your condition and to learn preventive measures to help reduce the risk of acute chest syndrome.

Iron Loss, Deficiency, And Anemia: Signs To Watch For

When your body doesn't have enough iron, you typically feel it. Most commonly, low iron makes you tired, cold, and short of breath. Your skin may be pale and clammy, too.

Here's why low iron saps your energy and takes your breath away. Your body needs iron to make hemoglobin, a substance in your red blood cells that helps them carry oxygen to your organs. Without enough hemoglobin, your tissues can't get enough oxygen to work like they should. When they struggle, you struggle.

When your hemoglobin drops below normal levels because of low iron, you have iron-deficiency anemia, the most common type.

Causes of Low Iron and Anemia

You can have low iron that leads to anemia for a number of reasons. Here are a few:

You don't get enough iron from food. Iron-rich foods include meats, poultry, and fish, as well as cereal and bread with added iron, legumes, tofu, spinach, dried fruit, broccoli, and nuts. When your regular menu lacks iron, your body will run low on it.

Your body is changing. If you're pregnant, breastfeeding, or going through a growth spurt, your body needs more iron -- and more red blood cells -- than usual. If you don't get more, your iron levels will dip.

You have problems with your GI tract. Your body takes in most of its iron through the upper small intestine. Issues with your gastrointestinal (GI) tract, such as celiac disease or a problem after GI surgery, can keep you from absorbing iron through your gut. Some medications for reducing stomach acid can also lower iron absorption.

You have a condition that prevents your gut from absorbing iron. Chronic kidney disease is often to blame.

You've lost blood. When a lot of blood leaves your body, your iron levels plummet. This might result from bleeding in your gut, your period, or an injury.

Symptoms

Low iron affects everyone a little differently. Some people notice no symptoms at all. The most common effects of low iron include:

Extreme fatigue and weakness

Cold hands and feet

Fast heartbeat (tachycardia)

Chest pain

Shortness of breath

Headache

Dizziness

Irritability

Pounding in your ears

You might also see physical changes that point to low iron. Common ones include:

Sore or swollen tongue

Brittle nails

Hair loss

Loss of color in your skin or yellow skin

You could also crave non-food items such as ice, starch, clay, or dirt.

Getting Diagnosed

Doctors have several tools to help them find out if you have low iron or iron-deficiency anemia. First, they'll do a physical exam to check for symptoms.

Next, your doctor will order blood tests, including one called a complete blood count (CBC). This shows how many red blood cells you have, how big they are, and what shape they are. You may also get further tests specific to iron.

When you have low iron or iron-deficiency anemia, these test results usually show:

Low hemoglobin (Hg). Remember, iron helps make hemoglobin. So when iron is low, hemoglobin can run low. Normal is 13.2 to 16.6 grams per deciliter (g/dL) for men and 11.6 to 15 g/dL for women.

Low hematocrit (Hct). That's the volume of red blood cells in your blood. Normal is 35.5 to 44.9 percent for adult women and 38.3 to 48.6 percent for adult men.

Low mean corpuscular volume (MCV). This test shows the size of your red blood cells. Normal is 80 to 95 femtoliters (fL).

Low ferritin. Ferritin is a protein in your blood that stores iron. When iron runs low, so does ferritin. Normal ferritin is 24 to 336 micrograms per liter (mcg/L) for men and 11 to 307 mcg/L for women.

Low serum iron (FE). This is a measure of the iron in your serum -- fluid in blood that isn't part of the clotting process. A normal result is 60 to 170 micrograms per deciliter (mcg/dL).

High transferrin or total iron-binding capacity (TIBC). This is a measure of your blood's ability to attach itself to iron and move it around. A normal test result is 204 to 260 milligrams per deciliter (mg/dL).

You may also get other tests to help your doctor figure out why your iron is low, such as:

Endoscopy or colonoscopy. In these tests, a doctor looks at your GI tract through a tiny camera to see if problems there might be causing bleeding or interfering with iron absorption.

Pelvic ultrasound or uterine biopsy. If heavy periods are causing your iron loss, your doctor might order one of these tests to understand what's to blame.

The treatment your doctor recommends for low iron or iron-deficiency anemia will depend on the cause.

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