Tag Archive | White blood cell

How Plerixa Fights Neutropenia

English: Reference ranges for white blood cell...

Ariana-Leilani suffer from Severe Chronic Neutroepnai.   Neutropenia is a condition characterized by the lack of a type of white blood cells, also known as neutrophils, in one’s blood circulation. Patients with neutropenia who receive plerixafor become prone to oral, skin, genital infections and in worst cases, a fatal whole-body infection.

Previously, it was known that Plerixafor increases the concentration of these white blood cells in the blood by inhibiting a protein called CXCR4. This inhibition prevents neutrophils in the blood stream from returning to the bone marrow, which is the primary compartment where the white blood cells are stored and released. It is therefore commonly accepted that the efficacy of the drug arises only from the release of these white blood cells from the bone marrow.

However, scientists at SIgN found that the inhibition of CXCR4 by the drug actually plays a dual role – it increases the neutrophil count in the blood through their release from the lungs, while simultaneously promoting their retention in the blood stream. Results of the study were published in the Journal of Experimental Medicine (JEM).

Discovery of this additional mode of action not only provides a deeper understanding on the drug’s mechanism; it also contributes to a more effective use of the drug, which may help to reduce the risk of bacterial infections in neutropenic patients.

“We have identified the precise mechanisms of plerixafor treatment, which has important implications on its usage. We can understand through this study the effectiveness or limitations of the drug on certain patients and thereafter craft new clinical approaches to better treat them. Our study forms a conceptual framework to establish improved therapeutic strategies for neutropenia,” said team leader Dr. Ng Lai Guan from SIgN.

The article can be found at: Devi S et al. (2013) Neutrophil mobilization via plerixafor-mediated CXCR4 inhibition arises from lung demargination and blockade of neutrophil homing to the bone marrow.

Sign the petition to make sure that Ariana-Leilani gets the life saving medicine to fight her severe chronic neutropenia:

GoPetition for ariana-leilani

“Lanie’s sunshine Box” brings smiles to sick children

From Channel 7,  WDAM:

Very few people, much less children, can relate to the disorder that 4 year-old Lanie Sherman was born with. [Like Ariana-Leilani,] Lanie suffers from a rare condition called “neutropenia,” a blood disorder that drastically decreases the number of white blood cells.  Because of her disorder, Lanie is extremely susceptible to sickness since neutropenia deteriorates the body’s ability to fight off infections.  Due to the treatment that Lanie requires, she and her mother, Becky Sherman, have become regulars at the Blair E. Batson Hospital for Children in Jackson.

During one of their routine visits in August 2012, Becky couldn’t help but notice that the hospital’s toy supply was depleting.

“Usually the hospital’s kind staff, distributes these brand new toys to their highly sick children that seek treatment there regularly.  I felt like there was something Lanie and I could do, “said Becky.    She took it upon herself to begin placing boxes, righfully painted “Lanie’s Sunshine Box” at stores and offices in the Pine Belt.

The outcome was astounding.

“Just last month, we delivered over four hundred toys to Blair E. Batson! It’s God truly at work when you see the children opening these shiny new toys,” said Becky.

Their project to help bring smiles to the faces of sick children is far from done.  They plan to continue dropping off donations monthly from the Pine Belt to Blair E. Batson.

“I like helping kids like me smile,” said Lanie.

You can find “Lanie’s Sunshine Box” regularly set up at locations such as the Jones County Sheriff’s Department, Heritage Heights Baptist Church, and Ken Morgan Salon.  Becky and Lanie welcome any vendors/community centers that would like to participate in the “Lanie’s Sunshine Box” project to call (601)425-3064.

Please sign Ariana-Leilani’s petition so she can get the treatments Lanie and others receive to stay alive:

he Save Ariana-Leilani Coalition

Study Finds In Neutropenia the immune system is balanced on a knife-edge

Developed by Weizmann Institute mathematicians working with physicians at the Meir Medical Center in Israel, and Roche’s research center in Basel, Switzerland, a model demonstrates how the immune system functions under conditions of neutropenia resulting from chemotherapy or bone marrow transplant.

Corroborated by evaluation of healthy individuals, the model indicates that the ability of white blood cells, primarily neutrophils, to tackle bacterial infections doesn’t just depend on absolute cell number, or the bacteria-to-cell ratio, but is affected by factors including neutrophil function and the permeability of tissues to bacteria, which can increase as a result of chemotherapy. Effectively, in neutropenia the immune system is balanced on a knife-edge—described mathematically as bistability—which can go either way as a result of even minor disturbances to the equilibrium.

In healthy people, the fact that the effectiveness of neutrophils varies from one person to another usually has no significant consequences. In contrast, in patients with neutropenia, this individual variability can make a difference between life and death. 

Ariana-Leilani’s life is balanced on a knife-edge.   Sign the petition to get her life saving medicine and examination:


Read More: Mathematical Model of Neutropenia 

Ariana-Leilani King Pfeiffer

Ariana-Leilani King Pfeiffer

SCN Kids Camp Filled with Fun Activities

The National Neutropenia Network has announced that Kids Camp 2012 is set for Saturday July 14. “We are fortunate to have the enthusiasm and commitment of the terrific Child Life staff at Phoenix Children’s Hospital,” Lee Reeves said. “They understand the needs of our children and will plan a day of fun and learning in a safe and upbeat environment where children will have plenty to do.”

Hat-making and brick decorating were two of the most popular activities during SCN Camp for Kids 2011.

The group of about 18 children ranging in age from 4-12 kept busy throughout the morning and afternoon with a full schedule of activities that included making “bone marrow snacks,” drawing pictures of what having neutropenia means to them, and learning about laughter Yoga. Children also practiced proper hand washing and learned about how blood is manufactured in the body. Jessica Doletzky BS, CTRS and Marianna Loiselle, BS, CCLS, from CS Mott Children’s Hospital led the children in several activities along with a team of volunteers.

Comments from the children included: “Neutropenia affects me and my family because we have to go to the hospital a lot for my sister. My family has to take a lot of safety precautions more than others because of neutropenia.” 

“I have a lot of shots. I used to have to take one every other day, now I take one every day.” 

“We should always wash our hands when we start to eat and we should use a paper towel to open the doors. We should cover our mouth when we have a cough.” 

“If someone gets sick, we might get sick too. If you don’t have any white blood cells you might get sick and you might get dead, so we should wash our hands.” 

“Neutropenia affects me because we can’t spend as much time as a family.” 

Learn more at: http://www.neutropenianet.org