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September is National Sickle Cell Awareness Month, a time to recognize the perseverance of patients living with sickle cell disease (SCD) and to recommit ourselves to improving the quality of life and health outcomes for all individuals living with SCD.

Mount Sinai Health System is reminding the community of the importance of newborn screening performed soon after birth with a blood test, education for families with this inherited condition and comprehensive care for children and adults including regular visits with a specialist can reduce complications of this illness.

SCD is the most commonly inherited blood disorder in the United States, affecting 100,000 people, and millions more worldwide. The disease primarily affects people of African, Hispanic, Mediterranean, Middle Eastern and South Asian ancestry.

What is sickle cell disease (SCD) actually?

The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells.

Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.

“Inherited” means that the disease is passed by genes from parents to their children. SCD is not contagious. A person cannot catch it, like a cold or infection, from someone else.

People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person’s body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD.

Hemoglobin SC disease and hemoglobin Sβ thalassemia (thal-uh-SEE-me-uh) are two other common forms of SCD.

Cells in tissues need a steady supply of oxygen to work well. Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it to all the tissues of the body.

Red blood cells that contain normal hemoglobin are disc shaped (like a doughnut without a hole). This shape allows the cells to be flexible so that they can move through large and small blood vessels to deliver oxygen.

Sickle hemoglobin is not like normal hemoglobin. It can form stiff rods within the red cell, changing it into a crescent, or sickle shape.

Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can’t reach nearby tissues.

The lack of tissue oxygen can cause attacks of sudden, severe pain, called pain crisis. These pain attacks can occur without warning, and a person often needs to go to the hospital for effective treatment.

Most children with SCD are pain free between painful crises, but adolescents and adults may also suffer with chronic ongoing pain.

The red cell sickling and poor oxygen delivery can also cause organ damage. Over a lifetime, SCD can harm a person’s spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

Sickle cells can’t change shape easily, so they tend to burst apart or hemolyze. Normal red blood cells live about 90 to 120 days, but sickle cells last only 10 to 20 days.

The body is always making new red blood cells to replace the old cells; however, in SCD the body may have trouble keeping up with how fast the cells are being destroyed. Because of this, the number of red blood cells is usually lower than normal. This condition, called anemia, can make a person have less energy.  Anemia ending line is lack of oxygen to the tissue body parts all over.

“Sickle cell disease is devastating for patients and their families,” said Jeffrey Glassberg, MD, MA, Assistant Professor of Emergency Medicine, Hematology and Medical Oncology, Icahn School of Medicine at Mount Sinai. “It’s a chronic disorder causing pain in the extremities and back, infections, organ failure and other tissue damage, skin infections, loss of eyesight, severe blood clots and strokes. Patients learn to function in a constant state of pain and when that pain becomes debilitating, they often end up in the emergency room,” said Dr. Glassberg, also Associate Director of the Comprehensive Sickle Cell Program at The Mount Sinai Hospital.

“Patients with SCD are more likely to live full lives if they undergo regular checkups, prevent infections and develop healthy habits,” said Jena Simon, MS, FNP-BC, RN, also of the Comprehensive Sickle Cell Program.

Tips to Staying Healthy for SCD patients:

• Get regular checkups. Regular health checkups can help prevent some serious problems.
• Prevent infections. Common illnesses, like influenza quickly can become dangerous for both children and adults with SCD. The best defense is to get a flu shot every fall and to stay up-to-date on other immunizations.
• People with SCD should drink 8 to 10 glasses of water every day and eat healthy food. They also should try not to get too hot, too cold, or too tired.
• Look for clinical studies. New clinical research studies are beginning all the time at Mount Sinai and elsewhere, with the goal of finding better treatments for SCD. Study participants gain early access to experimental medicines and treatments.
• Get support. People with SCD should find a patient support group or other organization in the community that can provide information, assistance, and support.

Sickle Cell Disease Facts & Figures:

• SCD is an inherited blood disorder that can cause severe pain and permanent damage to the brain, heart, lungs, kidneys, liver, bones and spleen.
• SCD is most common in Africans and African-Americans. It is also found in other ethnic and racial groups, including people from South and Central America, the Caribbean, Mediterranean countries, and India.
• More than 2 million people carry the sickle cell gene that allows them potentially to pass the disease on to their children. People of African, Hispanic, Mediterranean, Middle Eastern, and Indian descent may want to be tested for the gene before having children. You can carry the gene and not have any signs or symptoms of SCD. Both parents have to have the gene to have a child with SCD.

DIAGNOSIS & TREATMENT for SCD:

Minnesota Department of Health states the following about treatment:

“Sickle cell disease is usually diagnosed at birth through a blood spot test, which is part of the Newborn Screening Program. A blood test can also be performed at any time for those not screened at birth.

Treatment can help relieve symptoms and complications of SCD, which vary and can range from mild to severe. Treatment will be different for each person based on what symptoms they have. The goals of treating SCD are to prevent and relieve SCD complications, including pain, infections, organ damage, and strokes. Treatments can include:

• Receiving blood transfusions
• Drinking lots of water (8 to 10 glasses of water each day)
• Receiving IV (intravenous) therapy (fluids given into a vein)
• Taking pain relievers
• Taking an FDA-approved medication, such as hydroxyurea, crizalizumah, L-glutamine, or voxeletor

Currently, the only known cure for SCD is a blood or marrow transplant (BMT). A blood or marrow transplant is a procedure that takes healthy stem cells from a donor and puts them into someone else. These healthy stem cells cause the bone marrow to make new cells that are not in the sickle shape. Many people do well after a BMT and experience significant improvement in their quality of life. However, there are risks associated with transplants. Discuss eligibility for transplant and associated risks and benefits with a transplant doctor.”.

“Mpox, formerly called monkeypox, is a rare disease similar to smallpox caused by a virus. It’s found mostly in areas of Africa, but has been seen in other regions of the world. It causes flu-like symptoms such as fever and chills, and a rash that can take weeks to clear. There’s no proven treatment for mpox, but it usually goes away on its own.

For decades, mpox was mostly seen in Africa. But it’s occasionally found in other countries, including the United States.

In the summer of 2021, a case of mpox was found in a U.S. resident who had traveled from Nigeria to the United States. Then, 2022 brought outbreaks to regions outside of Africa, including Europe, the Americas and Australia.”

Cleveland Clinic (https://my.clevelandclinic.org/health/diseases/22371-monkeypox)

Monkeypox was first discovered in 1958 when two outbreaks of a pox-like disease occurred in colonies of monkeys kept for research, hence the name ‘monkeypox.’ The first human case of monkeypox was recorded in 1970 in the Democratic Republic of the Congo (DRC) during a period of intensified effort to eliminate smallpox. Since then, monkeypox has been reported in people in several other central and western African countries: Cameroon, Central African Republic, Cote d’Ivoire, Democratic Republic of the Congo, Gabon, Liberia, Nigeria, Republic of the Congo, and Sierra Leone. The majority of infections are in Democratic Republic of the Congo.

There are two distinct genetic clades of the monkeypox virus – the Central African (Congo Basin) clade and the West African clade. The Congo Basin clade has historically caused more severe disease and was thought to be more transmissible. The geographical division between the two clades has so far been in Cameroon – the only country where both virus clades have been found.

Monkeypox is a viral zoonosis (a virus transmitted to humans from animals) with symptoms very similar to those seen in the past in smallpox patients, although it is clinically less severe. Monkeypox virus is an enveloped double-stranded DNA virus that belongs to the Orthopoxvirus genus of the Poxviridae family.   With the eradication of smallpox in 1980 and subsequent cessation of smallpox vaccination, monkeypox has emerged as the most important orthopoxvirus for public health. Monkeypox primarily occurs in Central and West Africa, often in proximity to tropical rainforests and has been increasingly appearing in urban areas. Animal hosts include a range of rodents and non-human primates.

Various animal species have been identified as susceptible to monkeypox virus.. This includes rope squirrels, tree squirrels, Gambian pouched rats, dormice, non-human primates and other species. Uncertainty remains on the natural history of monkeypox virus and further studies are needed to identify the exact reservoir(s) and how virus circulation is maintained in nature.

Monkeypox cases in people have occurred outside of Africa linked to international travel or imported animals, including cases in the United States, as well as Israel, Singapore, and the United Kingdom.

The natural reservoir of monkeypox remains unknown. However, African rodents and non-human primates (like monkeys) may harbor the virus and infect people.

Symptoms of Monkey Pox:

People with monkeypox get a rash that may be located on or near the genitals (penis, testicles, labia, and vagina) or anus (butthole) and could be on other areas like the hands, feet, chest, face, or mouth.

• The rash will go through several stages, including scabs, before healing.
• The rash can initially look like pimples or blisters and may be painful or itchy.

Other symptoms of monkeypox can include:

• Fever
• Chills
• Swollen lymph nodes
• Exhaustion
• Muscle aches and backache
• Headache
• Respiratory symptoms (e.g. sore throat, nasal congestion, or cough)

Monkeypox symptoms usually start within 3 weeks of exposure to the virus. If someone has flu-like symptoms, they will usually develop a rash 1-4 days later.

“For Workplace safety, employers who show they care about the safety of their employees see improved morale, increased productivity, lower costs and fewer injuries.  Workplace safety culture begins with a well-trained workforce. Employees trained in NSC safety programs make their workplaces safer for everyone. NSC offers a variety of training options to fit your needs.”.

National Safety Council NSC (https://www.nsc.org/workplace/national-safety-month)

“June is National Migraine & Headache Awareness Month (MHAM), an opportunity to raise awareness about migraine and other headache diseases. Migraine impacts forty million people in the United States, one billion people across the globe, and is recognized as the #2 cause of disability worldwide. Currently, about 16 million people with migraines in the U.S. are undiagnosed. Approximately 400,000 Americans experience cluster headaches, recognized as one of the most painful diseases a person can have.”

National Headache Foundation (https://headaches.org)

June is National Migraine & Headache Awareness Month (MHAM), an opportunity to raise awareness about migraine and other headache diseases. Migraine impacts forty million people in the United States, one billion people across the globe, and is recognized as the #2 cause of disability worldwide. Currently, about 16 million people with migraines in the U.S. are undiagnosed. Approximately 400,000 Americans experience cluster headaches, recognized as one of the most painful diseases a person can have.

There is currently no cure for migraine, but we are in a renaissance of new treatments, therapies, and approaches to managing the disease.  If you have been struggling in silence, now is the time to speak up, educate yourself, and seek care. You might just change your life for the better.

A New Era of Care & COVID-19 Impact

During the pandemic, there was a significant rise in telemedicine. According to a survey by the Headache and Migraine Policy Forum and MigraineAgain, 78% of migraine and headache patients used telemedicine after the start of the pandemic, in comparison to just 22% before the pandemic. This trend was ushering in a new era of care, where patients could interact with health professionals from the convenience and comfort of their homes. The survey also revealed that there had been a nearly 70% rise in the number of migraine attacks during the pandemic and 84% of people had more stress managing their disease.

On the treatment front, there are new options for patients to explore. “Migraine and headache patients now have more options due to a wave of innovation in acute and preventive care,” noted Dr. William Young, Professor of Neurology, Thomas Jefferson University and Medical Advisor for the Coalition for Headache and Migraine Patients. “These include CGRP monoclonal antibodies, gepants, lasmiditan, and several neuromodulation devices.”

It is so much easier to for many humans to feel since they don’t see anything a migraine or general headache is not so bad but when you experience you may feel different when seeing those people with that symptom.  Headache specialists play a vital role in migraine advocacy and awareness efforts. The symptoms of migraine often cause patients living with the disease to withdraw from their daily lives.. Others may refrain from identifying themselves as a migraine patient, due to stigma surrounding the disease and a lack of compassion surrounding its symptoms. For these patients and so many more, we as healthcare professionals must continue to advocate on their behalf.

A disease awareness month plays a vital role to raise public knowledge, address stigma and build a stronger community of patient advocates.

“Posttraumatic stress disorder (PTSD) is a mental health problem. PTSD can only develop after you go through or see a life-threatening event. It’s normal to have stress reactions to these types of events, and most people start to feel better after a few weeks or months. Learn about PTSD symptoms and treatments to help you get better. There are currently about 8 million people in the United States with PTSD.”.

U.S. Dept of Veterans Affairs (https://www.ptsd.va.gov)

Can children have PTSD?

Children can have PTSD too. They may have symptoms described above or other symptoms depending on how old they are. As children get older, their symptoms are more like those of adults. Here are some examples of PTSD symptoms in children:

• Children under 6 may get upset if their parents are not close by, have trouble sleeping, or act out the trauma through play.
• Children age 7 to 11 may also act out the trauma through play, drawings, or stories. Some have nightmares or become more irritable or aggressive. They may also want to avoid school or have trouble with schoolwork or friends.
• Children age 12 to 18 have symptoms more similar to adults: depression, anxiety, withdrawal, or reckless behavior like substance abuse or running away.

People with PTSD may also have other problems. These may include:

• Feelings of hopelessness, shame, or despair
• Depression or anxiety
• Drinking or drug problems
• Physical symptoms or chronic pain
• Employment problems
• Relationship problems, including divorce

What helps? Research shows that high levels of social support after the war or event the person has gone through were important for those women  and men Veterans including those not veterans.

What can you do to find help?

If you are having a hard time dealing with your wartime memories, there are a number of things that you can do to help yourself. There are also ways you can seek help from others.

• Do things to feel strong and safe in other parts of your life, like exercising, eating well, and volunteering.
• Talk to a friend who has been through the war or other hard times. A good friend who understands and cares is often the best medicine.
• Join a support group. It can help to be a part of a group. Some groups focus on war memories. Others focus on the here and now. Still others focus on learning ways to relax.
• Talk to a professional. It may be helpful to talk to someone who is trained and experienced in dealing with aging and PTSD. There are proven, effective treatments for PTSD. Your doctor can refer you to a therapist. You can also find information on PTSD treatment within VA at: VA PTSD Treatment Programs.
• Tell your family and friends about LOSS and PTSD. It can be very helpful to talk to others as you try to place your long-ago wartime experiences into perspective. It may also be helpful for others to know what may be the source of your anger, nerves, sleep, or memory problems. Then they can provide more support.

Don’t be afraid to ask for help. Most of all, try not to feel bad or embarrassed to ask for help. Asking for help when you need it is a sign of wisdom and strength.

Don’t let PTSD get in the way of your life, hurt your relationships, or cause problems at work or school.

PTSD treatment can help.

Learn what treatment is like to help you make choices about what’s best for you.

If you suspect that you or a loved one has post-traumatic stress disorder (PTSD), it’s important to seek help right away. The sooner PTSD is confronted, the easier it is to overcome. If you’re reluctant to seek help, keep in mind that PTSD is not a sign of weakness, and the only way to overcome it is to confront what happened to you and learn to accept it as a part of your past.

TYPES OF TREATMENT:

• Trauma-focused cognitive-behavioral therapy. Cognitive-behavioral therapy for PTSD and trauma involves carefully and gradually “exposing” yourself to thoughts, feelings, and situations that remind you of the trauma. Therapy also involves identifying upsetting thoughts about the traumatic event–particularly thoughts that are distorted and irrational—and replacing them with more balanced picture.
• Family therapy. Since PTSD affects both you and those close to you, family therapy can be especially productive. Family therapy can help your loved ones understand what you’re going through. It can also help everyone in the family communicate better and work through relationship problems caused by PTSD symptoms.
• Medication is sometimes prescribed to people with PTSD to relieve secondary symptoms of depression or anxiety. Antidepressants such as Prozac and Zoloft are the medications most commonly used for PTSD. While antidepressants may help you feel less sad, worried, or on edge, they do not treat the causes of PTSD.
• EMDR (Eye Movement Desensitization and Reprocessing) incorporates elements of cognitive-behavioral therapy with eye movements or other forms of rhythmic, left-right stimulation, such as hand taps or sounds. Eye movements and other bilateral forms of stimulation are thought to work by “unfreezing” the brain’s information processing system, which is interrupted in times of extreme stress.

When looking for a therapist for post-traumatic stress disorder (PTSD), seek out mental health professionals who specialize in the treatment of trauma and PTSD. You can start by asking your doctor if he or she can provide a referral to therapists with experience treating trauma. You may also want to ask other trauma survivors for recommendations, or call a local mental health clinic, psychiatric hospital, or counseling center.

“Males experience more traumatic events on average than do females, yet females are more likely to meet diagnostic criteria for Posttraumatic Stress Disorder (PTSD), according to a review of 25 years of research reported in the November issue of Psychological Bulletin, published by the American Psychological Association (APA).

From the review, researchers David F. Tolin, PhD of the Institute of Living and Edna B. Foa, PhD, of the University of Pennsylvania School of Medicine found that female study participants were more likely than male study participants to have experienced sexual assault and child sexual abuse, but less likely to have experienced accidents, nonsexual assaults, witness death or injury, disaster or fire and combat or war. Sexual trauma, the authors conclude, may cause more emotional suffering and are more likely to contribute to a PTSD diagnosis than other types of trauma. Women’s higher PTSD rates were not solely attributable to their higher risk for adult sexual assault and child sexual abuse, explained Tolin. PTSD rates were still higher for women even when both sexes were compared on the same type of trauma. PTSD may be diagnosed more in women in part because of the criteria used to define it. Cognitive and emotional responses to traumatic events make a diagnosis of PTSD more likely.”.

American Psychological Association