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Tutorial on Neutropenia from Dr. Dale

“White blood cells (WBCs) in our body help fight infections. There are various types of WBCs and each has its own way of fighting microbial infections. The most important WBC is called the neutrophil.

“The number of neutrophils in the blood is expressed as absolute neutrophil count (ANC) and it measures the percentage of neutrophils in the differential WBC count. The ANC of a healthy individual falls in the range of 2,500 to 6,000 per microliter.

“A total blood count gives the levels of the three types of blood cells – white blood cells, red blood cells, and platelets. Neutrophils are present in two forms – polys and bands. Polys are also known as segmented neutrophils and are the most abundant of the WBCs. Bands are also called stabs and are immature polys. Both polys and bands destroy invaders (i.e. microbes) in the body.”

Learn more:

Absolute Neutrophil Count (ANC)

Twin Peaks Sheryl Lee, Also Struggles with Neutropenia

Twin Peaks Sheryl Lee, Struggles with Neutropenia

Twin Peaks Sheryl Lee, Struggles with Neutropenia

Sheryl Lee rose to fame playing murder victim Laura Palmer in David Lynch’s cult series Twin Peaks. But seven years ago the American actress, now 47, fell victim to a mysterious sickness — neutropenia — that was to plague her for years and make it impossible for her to work.

She had always been very healthy and had a strong immune system, so it was strange that she got a cold and couldn’t easily get rid of it.  Then when she did get rid of it something else would come right back. She kept getting sick for about six months. She couldn’t figure out what was going on.   Her doctor suggested doing some blood tests and immediately discovered that her white blood cell count was low. So then she went through many more tests including bone marrow biopsies until they figured out that she had neutropenia.

After six months of unexplained illness Sheryl, who has an impressive string of film, TV and theatre credits to her name, was diagnosed with neutropenia, a rare blood disorder which makes her much more susceptible to bacterial infections.  As a result of her illness Sheryl says she is still struggling with her health and finances.  From the age of 40, she went through illness for four and a half years. She tried to keep working through it as much as she could but she was physically not able to do as much and if you look sick it’s hard to get a job.

Neutropenia occurs when the bone marrow fails to make enough of a specific type of white blood cells called neutrophils. They are an important part of the body’s immune system and attack bugs that enter the body, particularly bacteria. It usually appears later in life as the result of another condition such as leukaemia, auto-immune disorder lupus, rheumatoid arthritis or infections such as HIV and hepatitis.

It can also result from chemotherapy, a bone marrow transplant or certain medications such as those for an overactive thyroid gland. However it can also sometimes be genetic.   In Sheryl’s case, the cause is something of a mystery. “Nobody knows how I got it. No one in my family that we know of has it,” she explains.   Sheryl, who was treated in a hospital in Los Angeles, had to learn how to give herself injections to stimulate her bone marrow to increase production of neutrophils.    If she developed an infection such as pneumonia she would have to go into hospital and have her medication administered via an intravenous drip. “But being in a hospital is not ideal for someone with a really low immune system so fortunately I was able to get my treatment during the day and then go home and sleep at night.   “But there’s a huge list of side effects, the biggest being intense bone pain. It also causes weakness, nausea and sensitivity to everything – taste, smell, light and sound.  “For years the mental, physical and emotional exhaustion were relentless,” she says. “You face the big questions when you go through that kind of illness. There were definitely times when I didn’t know if I was going to get better. There were a lot of nights spent on my knees in prayer.”

Sheryl, who has a son Elijah, 14, from her brief marriage to Neil Diamond’s eldest son Jesse, searched for her own solutions to her illness, changing everything from where she was living to her diet and cosmetics. She also tried alternative therapies such as acupuncture.   She now lives in a tiny Californian guest house and is taking acting work when she can, recently appearing with Katherine Heigl in Your Right Mind about a country singer fighting for custody of her daughter, which premiered at the Venice Film Festival in August.

“I have to be very aware of how I manage my health. I still have relapses if I push myself too hard and my immune system can’t handle it.”

 

Novartis Now Has Version of Amgen Neupogen Drug

Sandoz announced that its version of Amgen’s NEUPOGEN proved to be highly similar to the original in preventing severe neutropenia in individuals with breast cancer being treated with neoadjuvant myelosuppressive chemotherapy. Neutropenia is a condition where the body has an excessively low number of white blood cells.

The U.S. Food and Drug Administration had accepted Sandoz’s filgrastim application in July, making the company the first to have an accepted filing under the Biologics Price Competition and Innovation Act of 2009.

According to Professor Kimberly Blackwell from the Duke University School of Medicine, biosimilars have the potential to play important roles in widening access to top-quality biologics in the U.S. Since biosimilars are more affordable than the originals they are copying, savings will be generated which can then be used to finance other medical needs.

The biosimilar that Sandoz intends to distribute in the U.S. is already available in over 40 other countries. By the end of the decade, it is expected that biosimilars will account for around a quarter of the $100-billion sales generated by off-patent biological drugs.

Read More: Tech Times

Blood drive to offer support for children with neutropenia

Neutropenia is not a well-known disease and there’s only one network that conducts research for a cure.

‘There’s no awareness month, there’s no ribbon, there’s nothing. It’s just us moms trying to get the story out there and say please can you help us,” said Arnold (the mother of a Neutropenia child)   While most of the people who are diagnosed with neutropenia are young, it’s a disease that can go un-noticed, even by the doctors, or its risks can be ignored.

Luckily Arnold found a doctor who took the condition seriously and, in turn, saved her child from unnecessary risk to its life.   ‘I don’t really want to imagine what it would’ve been like without that diagnosis, because it could’ve been life or death for him.”

A blood drive to offer support for children with neutropenia will be held October 21st Ottumwa, Iowa in honor of Ryder and others affected by the disease.  The Neutro Heroes blood drive and Halloween Bash will be held at the Ottumwa Knights of Columbus hall from four to seven pm next Tuesday, October 21, 2013.

Learn more at KTVO

If you cannot make the blood drive, please sign the petition to help Ariana-Leilani, who also suffers from Severe Chronic Neutropenia.

GoPetition

 

Congratulations to Ariana-Leilani on Graduating from Elementary School

Congratulations to Ariana-Leilani from her mother Dr. Ariel King.   Although Ariana-Leilani has made it this far, she still needs your help in urging that she get the free medicine and an second medical opinion she needs to finally rid herself of her Severe Chronic Neutropenia.

Sign her petition today:http://www.ariana-leilani.com

Understanding the biology of sepsis in neutropenic patients

Sepsis comes with serious consequences. The blood infection, most commonly caused by bacteria, can lead to septic shock, organ failure, loss of limbs, and even death. Patients whose immune systems are already compromised are at even greater risk.  Neutropenia is a condition that Ariana-Leilani suffers is one in which patients have a low number of infection-fighting , can occur as a result of anemia, certain cancers or chemotherapy treatment, and other hereditary disorders.

Nuala J. Meyer, MD, an assistant professor of Pulmonary, Allergy and Critical Care in the Perelman School of Medicine at the University of Pennsylvania examined the effects of sepsis on neutropenic .  Meyer looked at a single-center cohort of patients who presented to the emergency department or medical ICU with . Of the 1,269 patients studied, nine percent were neutropenic; 75 percent had hematalogic malignancy; 15 percent had metastatic solid organ malignancy, and 15 percent were . Neutropenic patients had a higher incidence of shock and were more likely to die within 60 days, a retrospective review of their electronic health record found. Meyer and her team set out to understand why this occurs.

“We hypothesized that the neutropenic patients would have higher levels of the growth factor angiopoietin 2, a vascular growth factor and marker of injured  vessels, and that these would serve as biomarkers for the most severe cases of neutropenic sepsis,” says Meyer. Increased levels of angiopoietin is known to lead to an increase of permeability of the blood vessel, which allows the contaminated blood to travel more easily from the vessel to the rest of the body and vice versa.

Contrary to this, the study showed that the neutropenic patients not only had lower than expected levels of angiopoietin 2, but they also had high levels of inflammatory cytokines, specifically interleukin-8, a protein important to the body’s immune response. “The neutropenic patients, despite a low white blood cell count, surprised us with the increased number of cytokines compared to the immunocompetant patients,” Meyer said.

This led the team to believe that mortality risk for sepsis patients might be associated with high cytokine levels independent of severity of illness, and that anti-inflammatory medications targeting interleukin-8 specifically, might be helpful, even among neutropenic patients, as interleukin-8 is highly associated with mortality. “In the future, testing for interleukin-8 in septic patients could lead to early intervention with inflammatory medication, and possibly, better outcomes for patients,” says Meyer

Neutropenia Family Conference 2014 July 11- 13th Ann Arbor, MI

IMG_0464Participants in the 2014 Neutropenia Family Conference will have a chance to speak with the experts, hear presentations covering the latest treatment and research, and to participate in programs geared toward managing life with neutropenia in it. It is a weekend of learning and fun for the children. There will be many opportunities for participants to get to know each other in a comfortable and nurturing setting.

This year sessions will start on Friday July 11th at 1:00 PM with a choice of workshops and an opening session for all.  The popular Friday Meet and Greet Reception has not gone away. As in the past families and presenters will have a chance to mingle and get to know each other while enjoying a filling assortment of appetizers.

Private one-on-one sessions with the doctors will be offered towards the end of the conference on Saturday afternoon instead of before the opening session on Friday.This should make them more productive than ever as patients will have the benefit of knowledge gained at workshops.

This year with the first ever Friday Kids Kamp parents will have the opportunity to attend the educational sessions on Friday afternoon from 1:00 to 5:00 knowing their children are having fun in a safe environment with their peers. Children will be welcomed back to Kids Kamp on Saturday from 8:30 to 4:30 where another day of learning and fun activities will be presented by the Child Life staff from the University of Michigan.

Kids Kamp is now divided into two age groups. One for ages 3-5 and one for ages 6 and older.

On Saturday evening there will be a family dinner where participants can relax and enjoy time with new friends.

The 2014 Neutropenia Family Conference comes to a close on Sunday, July 13 after breakfast and a final session.

Friday, July 11, 2014 at 1:00 PM Sunday, July 13, 2014 at 11:00 AM (EDT), Ann Arbor, MI

To learn more and/or to register go to Neutropenia Family Conference. www.neutropenianet.org.