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.

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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.

Kathy and Nathan’s story

November 12, 2013 at 11:52 am

Kathy’s son Nathan has received treatment for hemophilia at Puget Sound Blood Center since he was an infant. She shares her experiences with this bleeding disorder. 

Can you tell me a little about Nathan?

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Nathan and his wife pose with their dog

My son is a miracle child! I was a single mom from about the time he was born, and he’s made me grow up. He’s now 29 years old.

He went to Central in Ellensburg and loves to be physical. He wrestled in high school and played baseball. He works out, drinks protein drinks, and is very health conscious – actually, he’s about 80% health conscious. He eats his ice cream and stuff too.

Nathan’s a huge (and I mean HUGE) Seahawks fan! Our family knows not to bother Nathan on game day; Nathan is really funny about his Seahawks.

A lot of people don’t know he’s a hemophiliac; he doesn’t want them to think he’s weak and ask questions like, “Aren’t you afraid of AIDS?” He gets tired of questions. People think he’s going to get cut and blood is just going to rush out of him. People ask him why he doesn’t go on disability, but he wants to work [for the Des Moines water sewer district].

How was he diagnosed?

At birth, I didn’t know he was a hemophiliac. He was circumcised and rushed that night to the hospital.

In those days, he was the youngest hemophiliac to have a Port-A-Cath. He used to tell the kids at school that he had a gunshot wound and they couldn’t get the bullet out, that the Port-A-Cath lump was a bullet.

How did you hear about Puget Sound Blood Center?

[PSBC] picked up on me when Nathan was circumcised. They came and started talking to me about what hemophilia was. I was so devastated that I even went to the 700 club asking what I could do! Many times, the Puget Sound Blood ladies came to our house after hours, and many times I cried on their shoulders, them standing around and finding a vein on him in the bathtub and hanging a “drip drip” bag from the curtain rod.

What obstacles has PSBC helped you through?

It’s been the training and guidance. They trained me on flushing his Port-A-Cath. They gathered all my supplies when I was doing it at home – made sure that I had the heparin, the needles, and cleaning kit. They took care of me. Otherwise I wouldn’t have known what to order.

I’ve learned a lot – I have no medical background. When I was young, I even gave him pepto bismol and learned the hard way that pepto bismol has aspirin [which prevents blood from clotting] in it!

Can you tell me about any memorable bleeds?

One time I went to a baby shower and I left Nathan with a girlfriend who had two boys and she called me hysterically crying because Nathan was bleeding hard and didn’t know what to do because he was hurting so bad. She had him in the hot tub to soothe him, and hot tubs make your blood rush faster.

He’d be going to baseball, and we’d hide in the parking lot and he’d infuse in the parking lot before the game. He’s got a wonderful group of friends as far as support. I remember one time going to a baseball game when he was 14 and having to carry him because he had a bleed and he couldn’t walk. I used to carry an ice chest with dry ice for blood products and one time I went to lift it out and half the seat came with it.

What has Nate been able to do because of treatment?

He really, really wanted to play football. Every single one of his friends were football players, and that hurt him. In his last year, he did make the football team, but he never actually played a game because he knew he couldn’t. But he did it just to know that he could do it, that he could make the team and he was satisfied with that.

What advice do you have for moms who might be new to living with their child’s hemophilia?

It was really hard for me to accept, but – I hate to say just let him go and be – but it works itself out. It was harder on me than it was for him; I learned a lot from him in just taking it as it comes. It hurts, but seeing him interact with his friends and stuff – it just fell into place. Let him be a kid. I always told myself I wouldn’t hold him back from anything (except football) but I tried to do it nonchalantly. I treat him like I treat my other child.

It’s all about “Twinning:” Dr. Barbara Konkle shares her experience with World Federation of Hemophilia in Botswana

October 28, 2013 at 9:28 am

wfhAs Puget Sound Blood Center’s Hemophilia Care Program congratulates the World Federation of Hemophilia (WFH) on 50 years of “advancing treatment for all,” our clinical team recognizes the amazing contributions that WFH has done through their Twinning Program.

WFH established this innovative framework 15 years ago: hemophilia organizations or treatment centers engage in a two-way partnership to share information, expertise and experience. This program has made it possible for governments in less economically developed countries to purchase factor concentrates and increase access to hemophilia care for their patients.

Our Hemophilia Care Program’s Dr. Barbara Konkle participated in WFH’s Twinning program and shares her experience below. Dr. Konkle joined Puget Sound Blood Center in 2009 and was previously a Professor of Medicine and of Pathology and Laboratory Medicine at the University of Pennsylvania.  She directed the hemophilia care programs at Thomas Jefferson University and the University of Pennsylvania.

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Dr. Barbara Konkle with Factor VIII at Princess Marina Hospital in Botswana

Where did you go?

At the University of Pennsylvania the twinning experience was different from others because they had an expanding program that not only served Hemophilia patients, but also patients with HIV living in Botswana. I contacted WFH expressing my interest, as well as a contact from Botswana, reached out to WHF and that’s how we got paired. The experience was also unique because they had a really good team that had a universal education and were fluent in English.

How many people from your team participated in this program?

We had a pediatric hemophilia nurse, a physical therapist, a social worker and a physician.

What was your Center’s commitment?

We supplied the personnel to educate the clinical staff from Botswana. South Africa had a more developed hemophilia program compared to other countries. That’s why our focus was to help them develop the right structure and bring recognition to hemophilia care through their centralized healthcare system that could provide treatment for all.

Did you bring supplies?

Our team didn’t bring supplies because they were provided by the government of Botswana. The Republic of Botswana is a relatively wealthy country that grows their own natural resources. The government was able to purchase clotting factor for the country.

Did you go more than once?

We visited Botswana three times. Our team had the opportunity to go to Francistown, which is the second largest city and to Princess Marina. Our team supported their outreach clinics.

Was this a worthwhile experience?

Yes it was. We had a wonderful time and appreciated the help from Dr. Haruna Jibril who was the pediatric hematologist at Princess Marina Hospital, Botswana Baylor Children’s Clinical Centre. He supported us starting the children on prophylaxis. Our team made a difference with their outreach program and collaborated on HIV care to pregnant women. We trained the nursing staff to infuse patients as well as conducted home infusion classes for patients. Previously, only the physicians could infuse patients.

Another great accomplishment was to have the hospital agree that children that have bleeds could go see their hematologist instead of going to the ER. Also, the patients who were already diagnosed could obtain their factor when needed instead of waiting to get their factor levels tested every time they had a bleed. Our twinning activities in 2008 were recognized by the World Federation of Hemophilia (WFH) and we won Twin of the Year Award in July 2009.

Would you recommend this program to other HTC’s? What preparation is needed?

Definitely! The challenges are trying to find the funds and donations that can help the team establish a time to go, and identify the resources that are needed to serve a less developed country. You need to start with an assessment to see if it will work. Our situation was unique because the staff in Botswana had an amazing team of experts that were fluent in English. We also had an existing partnership with the Government of Botswana and the University of Botswana. Their government supplied clotting factor for the patients.

September is Sickle Cell Awareness Month

September 9, 2013 at 12:45 pm

September is Sickle Cell Awareness Month, and we’ve put together an infographic with a few facts about this genetic disease that affects 70,000 to 80,000 Americans.

Sept-sickle-cell

Puget Sound Blood Center helps sickle cell patients by providing needed blood transfusions — these transfusions reduce the number of abnormal cells in a patient’s bloodstream, making them feel better and preventing certain complications.

Your blood donations improve the lives of patients like Kendra.

Kendra Hogenson blog

Kendra was in and out of the hospital because of her sickle cell disease, suffering three bouts of meningitis, a stroke, and a lot of pain before the age of 13.

Her life changed after receiving her fist blood transfusion. Today, more than two decades and many transfusions later, thousands of blood donors have given Kendra health and hope.

Schedule a blood donation today!

Living a normal life, thanks to regular donors

December 13, 2010 at 3:18 pm

At least 15 blood donors are needed each week to keep Reid Morgan healthy.

By Reid Morgan

At 5 days old, Reid was diagnosed with hemophilia and referred to Puget Sound Blood Center. From infancy through adulthood, he has lived with the treatments, the physical limitations, the slow-clotting cuts and the more dangerous bruising and internal bleeding that all sever hemophiliacs endure. He’s also lived with pain. A slightly sprained ankle can bleed unchecked inside the joint capsule; it not only hurts, but it can lead to deformity and arthritis.

The Blood Center has helped Reid deal with it all. For over three decades, he and other Northwest patients have received both medical care and education about coping with bleeding disorders through the Blood Center’s hemophilia clinic and its annual camp retreat, Camp I-VY.

“Camp I-VY is invaluable,” he says. “It allows patient families to get together: parents talk to one another; kids talk to other hemophiliacs and find out that, despite all the blood products we receive, we’re really not that different.

“Without the Blood Center’s hemophilia program, I don’t know how my life would have turned out,” Reid says. Not as well, he reasons, without the care he received from hemophilia clinic co-founders Dr. Richard Counts and Dr. Art Thompson, who still runs the program.

“For me to have a ‘normal life,’ I need the protein from 45 pints of blood every week,” Reid explains. “That’s a lot of people donating to Puget Sound Blood Center and I’m really very appreciative. I really do have a normal life because of them.”

Schedule your own blood donation at a donor center.

Edited by Sean DeButts, Social Media Coordinator

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