Comprehensive Care for the Whole Family

February 1, 2017 at 4:00 pm

The Estell familyDoctors knew something was drastically wrong when Henry Etsell, then one year old, lost two-thirds of his blood during routine surgery. He would spend the next five days at Seattle Children’s receiving multiple blood transfusions and clotting factor to control his bleeding.

Henry’s parents, Chad and Nicolette Etsell, were in for a shock—Henry had hemophilia. Henry’s younger brother, Graham, would later receive the same diagnosis.

Henry EstellFortunately, the Etsells could turn to the Hemophilia Care Program at Washington Center for Bleeding Disorders (WCBD) for help and support. Located at Bloodworks, the WCBD has been a resource for people with bleeding disorders for more than 40 years. The Etsells were among 450 patients treated by the WCBD last year.

It turns out Nicolette is a symptomatic carrier of the hemophilia gene and has mild hemophilia. She goes to the center for her annual physical and other checkups, and Henry and Graham both receive regular care from a team of doctors and nurses at Seattle Children’s who partner closely with WCBD to care for children with bleeding disorders. And thanks to the center’s 24-hour telephone helpline, the Etsells can get advice anytime from hemophilia nurse specialists.

“The WCBD has changed our whole outlook on living with hemophilia,” Nicolette says. “We now have the tools and resources to keep our family healthy and live our lives with the disease just in the background.”

Help families like the Estells—schedule your blood donation appointment today.

A platelet disorder leads to bleeding: Jen’s story

February 23, 2016 at 1:00 pm

Jen

Jen Schmitt is the type of person who always puts others before herself.

Her friend Erin Nissen says,

Jen is the most giving and caring person that I have ever known. If I’m having a difficult time, she’s the first friend that I call. She remembers little things. If you say there’s a type of wine you like, before you know it, she’s sent it to you in the mail. She’s so thoughtful.

The Harborview cardiac ICU nurse started having abnormal bleeding in her urinary tract at the end of November, 2015. It wasn’t profuse, but enough to be a concern.

Jen visited the doctor multiple times and had imaging performed to try to figure out where the bleeding was coming from. The week before Christmas, her doctors put her under anesthesia to investigate with an internal scope. Ironically, that’s when the real trouble started.

Jen has a rare genetic platelet disorder called Glanzmann Thrombasthenia. When she bleeds, her platelets adhere to the site of the injury, but don’t effectively clump together to stop the bleeding. Her tissues are easily irritated, so the ordinarily harmless scope used in the procedure caused an injury.

Big blood clots formed, and clung to her bladder wall. The clots would break off, causing bleeding, spasms, and excruciating pain, which then caused more bleeding, clotting, spasms, and pain – an ongoing cycle of misery.

She was admitted to UW Medical Center on Christmas Eve.

Jen’s parents and close friends have been by Jen’s side through the entire ordeal, and can’t even count the number of platelets and red blood cell transfusions she’s received – Jen gets three or four units of platelets with every infusion of NovoSeven, a drug that helps her blood clot.

Because of her bleeding disorder, Jen has needed periodic blood transfusions throughout her life, and as a result has built up antibodies: there are only eight people in Western Washington whose blood and platelets she can receive.

It’s upsetting for Erin to watch her hilarious, generous, and generally private friend accept help from others and face the unknown – no one can tell when this ordeal will stop or what the long-term effects will be.

One comfort is that the blood is there:

It makes her teary-eyed – she can’t believe complete strangers would come in and donate their time and themselves.

Jen’s coworkers have rallied, visiting her in the hospital, bringing her snacks, and sending messages of support.

Jen-Coworkers

Harborview already hosts regular blood drives, and several of Jen’s colleagues have come forward to champion extra drives in February.

Many of her coworkers at Harborview are stepping up to donate blood and platelets in her honor, either at a drive or at our Central Seattle and Federal Way donor centers. Even though they may not be a match for Jen, they know that countless other patients in our region depend on their gifts of blood components.

Jen was finally able to be discharged on February 11th and is recovering with her parents in Olympia. She’s able to find the positive in the whole experience:

She’s always been an amazing nurse and amazing patient advocate – always trying to see things through the eyes of the patient. This has shed more light on how the patient has it in the hospital. It’s giving her greater passion for her profession as a nurse.

Jen’s coworkers at Harborview are hosting another drive in her honor on Thursday, February 25, 2016. Patients like Jen depend on your donations every day.

Steven’s story: 40 years of hemophilia treatment

March 16, 2015 at 11:17 am

Hemo_awareSteven* was born with hemophilia in the late 1960s.

At that time, there was no treatment for joint bleeds, the most common complication of hemophilia. If he did have a bleed, his parents could only pack his joints in ice and wait for the pain and swelling to subside.

Steven recounts that his parents wrapped his knees in foam when he was learning to crawl.

Steven’s family moved to Washington State in 1976, and he began receiving care from Bloodworks Northwest (then known as Puget Sound Blood Center).  Back then, BloodworksNW was among the first in the U.S. to establish a comprehensive care center for people with bleeding disorders. Over the years that care has evolved to meet the changing needs of the bleeding disorder community.

[T]he wonderful people at the Hemophilia program are truly my go-to group for most medical concerns…from a stubbed toe to a bloody nose to a total knee replacement.

In addition to patient care, BloodworksNW developed the first practical means of producing cryoprecipitate on a large scale in order to treat patients with hemophilia, allowing them to begin to live more normal lives with the hope of better longevity as well. For Steven, this meant he could do things like play outside and go to school.

During Steven’s 40 years with BloodworksNW, the Hemophilia Care Program staff has become like a second family to him. They have prepared plans for surgeries, assisted with insurance gaps and glitches (critical for an expensive condition like hemophilia), coordinated travel (including to meet the woman who’s been his wife for the past 17 years), and provided emotional support—all in addition to tending to his bleeding disorder itself.

Today, Steven leads a happy life with his wife and their Chihuahua — he’s grown far beyond that baby with foam taped to his knees.

When asked how his life would be different without BloodworksNW, Steven says:

Plainly speaking, I would be lost… Without the confidence that I have through my medication via the Hemophilia Program at the Puget Sound Blood Center I would literally be crippled, bleeding, in pain, scared, and hopeless. Without exaggeration, I owe everything to them and the medications, resources, and compassion they provide. To answer the question bluntly – My life would be different in that I wouldn’t have one.

The road continues for BloodworksNW’s research, too. Our scientists are pursuing better, more effective treatments.  Short-term, our goals include developing personalized care plans for people with bleeding disorders to help them live free of pain, disfigurement, and disability.  Long-term, we’re working for a cure.

You are a critical part of this work.  Your support can help patients like Steven live longer, healthier lives.

*not his real name.

Bloody Fun Run on October 25

October 2, 2014 at 1:54 pm

BLOODY (2)Our Hemophilia Care Program is excited to be walking in the Bloody Fun Run, the Bleeding Disorder Foundation of Washington’s (BDFW) second annual hemophilia walk, on Saturday, October 25, 2014 at Greenlake park in Seattle. The morning promises to be fun for all ages!

The Bloody Fun Run’s goal is to raise awareness about bleeding disorders and raise funds for the BDFW for research — it not only helps children and adults in our state, but edges us closer and closer to a cure.

Bleeding disorders affect a small percentage of the population; our Hemophilia Care Program serves many kinds of bleeding disorders, including Hemophilia, von Willebrand Disease, and Platelet Disorders. People with bleeding disorders tend to bleed longer than the rest of the population, not harder or faster. Small cuts (like paper cuts) and bruises are generally not a problem, though traumatic injuries can result in life-threatening bleeds and crippling joint deformities.

We welcome you to join our team and walk with us or make a donation.

In addition to the walk, the event offers pumpkin painting, carnival games, trick-or-treating, a costume contest and more. Admission to the festival is free with a suggested donation of $25 per family.

Hemophilia care at Puget Sound Blood Center

March 11, 2014 at 11:03 am

konkleMarch is Hemophilia Awareness Month, as designated by President Reagan in 1986. Dr. Barbara Konkle is interim director of our Hemophilia Care Program and describes Puget Sound Blood Center’s work in treating hemophilia and other bleeding disorders. 

What is hemophilia?

Hemophilia is a bleeding disorder that results from either not having a protein that’s needed for clotting or having an abnormal clotting protein.

What misconceptions do many people have about hemophilia?

One thing with hemophilia, and it relates to other bleeding disorders, is the perception that women don’t have bleeding disorders. Hemophilia is an x-linked disorder, so men are  more severely affected, but women can also be affected with hemophilia, and they can have other bleeding disorders.

The other misconception is the is expectation that someone with hemophilia will look abnormal and they don’t. With current therapy, persons with hemophilia can lead normal lives – they can do basically what anyone else can do.

Can you tell me more about PSBC’s clinical program and research?

The clinical program provides comprehensive care to patients with bleeding disorders, including hemophilia; we have physicians and nurses, a social worker, physical therapy, and support staff that allows us to assess the needs of the patients and support them in their treatment. Many patients  treat themselves at home with factor replacement medicine that they have to put in their vein, so the patients and families have to learn how to do this.  The comprehensive care model allows us to support them in this treatment and other needs.

sheldon

Shawn poses with a machine that detects factor level in blood

It was shown some time ago that this model of comprehensive care allowed patients to be more independent and also allowed them to live longer. Research at PSBC is linked very closely to the clinical program.  We are testing better ways to diagnose and monitor hemophilia, von Willebrand disease and other bleeding disorders, and, in collaboration with industry, are assessing new products to treat bleeding disorders, including gene therapy for hemophilia B.  We are leading a national study to assess heart disease and blood clotting problems in older men with hemophilia.   We are collaborating with researchers at the University of Southern California and other institutions to help understand the challenges and expenses patients with hemophilia face.  Our physical therapist, Lorraine Flaherty, is studying how to decrease falls in persons with hemophilia and ways to improve physical therapy for elbow issues.

We are the central laboratory and a Steering Committee partner for a national project, called the MyLifeOurFuture program.  This project aims to determine the genetic cause of the hemophilia in all patients in the U.S., and eventually provide testing for females who may be carrier s of hemophilia.  With informed consent, we are collecting and storing blood samples and data in a repository which will be used nationwide for research in hemophilia in the future.

How did you become interested in bleeding disorders?

I got interested in bleeding disorders when I was a resident in internal medicine.  I found the medical problems the patients face and the close link to findings in laboratory testing fascinating. Hemophilia is a chronic disease where  our goal is to work  with the patient and family to minimize symptoms, so they can lead a normal life.  This is very rewarding.  I started in a hemophilia clinic as a fellow 30 years so, so this is my 30th year in hemophilia.

What are the most significant changes you’ve seen in the past 30 years?

The most significant changes – because I treat adults with hemophilia – we now have young men who are coming to our clinic and they have normal joints and no other signs of past bleeding. They’re just doing phenomenally well. In our young adult patients we are now seeing the benefits of the widespread use of prophylaxis therapy from a young age.

I entered hemophilia the year before the HIV virus was defined, and that was a terrible, terrible time in hemophilia with many patients dying of AIDS. It was before we had effective treatment for HIV infection, and we lost a lot of patients. No clotting factor products have transmitted the HIV virus since 1985 and none have transmitted hepatitis since around 1990.  Many older patients are still struggling with these infections, although treatment is much improved.  Fortunately our younger patients don’t have to deal with these infections.

What should parents of babies newly diagnosed with hemophilia know?

A 18_Kristan and Cole Prill White BkgdYou child should have a normal life expectancy and should be able to do what other children are doing.

When I see an 18, 19-year-old, we have to expect that he will live to 80 or longer, so I have to be sure that we do all the right things so that he can get down on the floor and play with his great-grandchildren. That’s our goal.

What should blood donors know?

Most of our treatment for hemophilia no longer comes from blood products. That was critical in the past, and that’s why hemophilia programs are often linked with blood centers.  While many treatment products are now produced by recombinant DNA technology, children and adults with bleeding disorders are still at risk of bleeding and needing blood, and so donations are still really important for the hemophilia population even though we use them less.  Also, some of our patients with rare bleeding disorders still rely on donated blood products for treatment.

Switch to our mobile site