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Renee Wittenmyer, PA-CRenee Wittenmyer practices at the Indiana Hemophilia and Thrombosis Center in Indianapolis. She has indicated no relationships to disclose relating to the content of this article. The article was written by Laura Howard, MPAS, PA-C, who is Ms. Wittenmyers colleague.The Indiana Hemophilia and Thrombosis Center (IHTC) was started 10 years ago by two hematologists who dreamed of a comprehensive clinic that could meet the needs of patients with benign hematologic conditions. They began hiring PAs in the spring of 2004 to help them manage the complex medical care their patients required. IHTC is staffed by seven physicians, two NPs, and five PAs, who manage patients with disorders such as hemophilia, von Willebrand disease, thrombophilias, and sickle cell disease. PAs and NPs at IHTC have unique roles requiring expertise in their subset of patients. I follow adult patients with hemophilia who have acquired HIV infection, hepatitis B, or hepatitis C from tainted blood products. 7:30 AM![]() I like to get to work at least an hour before my first patient comes to clinic, which allows me to get caught up on Universal Data Collection reports. These reports are an ongoing collection of individualized patient medical data allowing for documentation for ongoing research and to assure continued funding for IHTC from government grants and the CDC. My pager goes off as I am entering data. Its a call from a 19-year-old Amish man with severe factor VIII deficiency. A deficiency in clotting factor VIII can cause severe bleeding in soft tissues, muscles, and joints, with significant consequences such as compartment syndrome, arthropathies, or joint deformities. Hemophilia is an X-linked inherited deficiency of either factor VIII (hemophilia A) or factor IX (hemophilia B). Acquired hemophilia can occur but is very rare. To treat bleeding in patients with hemophilia is theoretically simple; give them the factor they are deficient in to bring their levels back to normal. Until the early 1990s, replacement factor came from the plasma of people without the disease. The blood was cleaned, but the cleaning process was less than perfect and allowed for the transmission of unwanted viruses, including HIV, hepatitis B, and hepatitis C. At IHTC, we follow more than 300 patients with hemophilia who have acquired one of the aforementioned viruses from blood products received before 1993. Fortunately, plasma is now treated with viral inactivation processes and recombinant factor is available, both of which have significantly reduced the risk of virally transmitted diseases. IHTC manages the care of approximately 150 Amish patients with bleeding disorders. Because of their beliefs, the Amish do not carry medical insurance, and the cost of factor replacement can add up to millions of dollars over a patients lifetime. Many are in a disease management program that provides free treatment; this government program has been shown to save the state millions of dollars when patients are treated promptly after an acute bleed. 8:00 AMI am in an early morning educational meeting when I get paged about one of our patients. He is a 21-year-old Amish man who was kicked by his horse in his left shoulder this morning at 5 AM. He lives 3 hours away and will require factor replacement at 100% concentration to minimize the risk of soft tissue bleeding into his shoulder joint. He is able to catch a ride in a car with a non-Amish friend to a local emergency department (ED) to have his shoulder x-rayed and scanned if needed. I call the ED before he arrives, giving instructions to the charge nurse about how to mix factor replacement before infusion. The Amish man will need to continue infusing himself with factor VIII replacement at 100% correction for the next 5 to 7 days. Patients with hemophilia learn how to self-infuse at a young age. Luckily, IHTCs Outreach Program is coming to the town where this patient lives next week, so we will be able to assess his shoulder in person. 9:30 AMBack in my office, I try to catch up on dictations and reports. I am able to get a couple dictations done before my pager goes off again. I gulp some coffee and dash down the hall to clinic. The patient is a young man with acute intermittent porphyria, along with a seizure disorder. He comes in today for a routine exam. Late last year, he had a rather complicated hospital course after an acute porphyric attack, with subsequent respiratory failure and a catatonic state. As I am examining him, he suffers a grand mal seizure. I run out of the room to alert the physician and have the clinic nurse call 911. He is brought over to the hospital and admitted for supraventricular tachycardia. 10:30 AMAfter calling our hospitalist to give him a heads up on the man with the seizure and v-tach, I am paged by Craig, our data coordinator, who reminds me that I have a 10:30 meeting with him and our medical director to review forms and specific links being created for the new electronic medical record (EMR) system. By the time I get out of the meeting, I only have a few minutes to eat a quick lunch. 12 NOON![]() The front office manager is waiting for me in my office when I return from lunch and asks if I can see a walk-in with a knife wound. The man is a 45-year-old with severe factor VIII deficiency and transfusion-acquired HIV infection. To date, he has had an undetectable viral load and a CD4 cell count of 600/μL. He has HIV-associated renal failure requiring hemodialysis 3 days a week. When he comes in, I quickly assess the knife wound, which he says he got while defending himself from an attack. He was stabbed on the left posterior aspect of his forearm just below the elbow. I learn that he went to a local ED and received factor VIII replacement and wound care including cleaning and stitches. This man is currently homeless and moves from motel to motel. Two weeks before the assault, he was given a tetanus booster in our clinic during a routine exam. During todays visit, he is given more factor replacement and is scheduled to come in for the next 5 consecutive days for more factor replacement to correct his levels to at least 80% to prevent further bleeding and allow for adequate wound healing. 1:00 PMMy next patient is an 85-year-old man who is coming in to go over the results of his labs and abdominal CT scan. He was at the clinic last week for evaluation of a chronically elevated WBC count. He recently moved to a nursing home because he has Alzheimers disease, and he has a history of alcohol dependence. The mans son and daughter-in-law come with him to the appointment today. I discuss the laboratory test results, which show anemia and a low platelet count. The CT scan of his abdomen reveals lesions suspicious for carcinoma. I explain that his liver is probably the site of the primary cancer, with likely metastasis, and provide a referral to an oncologist. 1:30 PMI run back to my office to dictate more notes and get a few more reports done before seeing my next patient. This one is a 42-year-old man here for a routine follow-up visit for anemia from myelofibrosis. Today, he presents with lower extremity edema and a rash located on his abdomen and groin. The rash appears as a small cluster of papules without drainage. This appears to be a side effect of increasing doses of thalidomide. The patient receives a topical cream for the rash and furosemide for the edema. 2:00 PMI meet with a 65-year-old woman who had come in 6 weeks ago for evaluation of an enlarged spleen with borderline low normal platelet count. CT showed an enlarged spleen with displacement of the left kidney. A follow up cervical lymph node biopsy revealed B cell lymphoma. I discuss her prognosis and treatment options, and I refer her to an oncologist. 2:30 PMAs I am waiting in clinic to review a patients case with my supervising physician, the triage nurse comes up to tell me that a hepatologist from Indiana University Medical Center wants to talk to me about a patient I sent over for a liver biopsy. After I get off the phone, I go into another clinic exam room to see another patient. He is a 30-year-old man with severe factor VIII-deficient hemophilia with a high responding inhibitor requiring a bypassing agent. Patients with hemophilia can develop inhibitors that block the activity of the recombinant factor VIII product. This makes treating the patient more difficult and more expensive in that they require much more recombinant factor to overwhelm the inhibitor. The patient is on the schedule but has shown up 3 hours early with nausea, vomiting, diarrhea, and stomach pain. After a history and physical exam, a severe GI bleed is diagnosed and the patient is admitted to the ICU. 4:30 PMAt this point, I try to keep an eye on the time and wrap up my day. I return phone calls and call patients with lab results, respond to e-mails, double check the EMR desktop for any messages, and dictate the days office notes. Working at IHTC offers me a rewarding experience as a PA, allowing me to provide multifaceted care to patients with varying hemoglobinopathies in the acute and chronic setting. JAAPA |