Managing PK Deficiency | Know PK Deficiency
See data on the long-term impact of PK deficiency

Managing PK deficiency

Building a management plan with the right team

Explore the key terms used on this page


Patients who communicate with their healthcare team are happier with their treatment and receive better care

Many different healthcare professionals will play an important role in managing PK deficiency:

Many different healthcare professionals will play an important role in managing PK deficiency:


Registered nurse

  • In addition to nurses at your doctors office, there are nurses who specialize in giving transfusions

Family doctor or general practitioner

  • The doctor you see for checkups and yearly flu shots or other wellness visits

Counselor or psychologist

  • Living with PK deficiency can cause stress and anxiety. It may help to find a support group or a mental health professional to talk to

Based on your symptoms, you may need to add other specialists on your care team such as a cardiologist, an endocrinologist, or a gastroenterologist.

There is no cure for PK deficiency

Your doctor may recommend some of the following options to manage symptoms and complications. Talk to your doctor to find out more.

Managing hemolytic anemia

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RBC transfusions

To boost RBC levels, donated blood cells can be added to the bloodstream. Some people with PK deficiency may never have transfusions, some may have them occasionally, and others may have them on a regular basis. The degree of anemia and symptoms that come with it are evaluated before adding transfusions to a management plan.

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The spleen may become enlarged due to RBCs breaking down. The spleen also sometimes removes RBCs that still work. A splenectomy may be considered to increase RBC counts or prevent further complications. But people who have their spleen removed may be at higher risk for certain bacterial infections. If you undergo a splenectomy, talk with your hematologist about ways to minimize your risk.

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An FDA-approved prescription medicine used to treat low red blood cell counts caused by the early breakdown of red blood cells (hemolytic anemia) is available for adults with pyruvate kinase deficiency (PK Deficiency).

Managing complications

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To prevent the ongoing risk of gallstones due to continued hemolysis, the removal of the gallbladder may be considered. Gallstones can cause nausea, stomach pain, or other forms of gallbladder disease.

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Chelation therapy

A type of medicine called a chelation agent binds with iron in the bloodstream to form a substance the body can remove more easily.

Cardiologist: A doctor that specializes in heart disease

Endocrinologist: A doctor that specializes in conditions caused by problems with glands and hormones (endocrine diseases)

Gastroenterologist: A doctor that specializes in diseases affecting the digestive system

Ophthalmologist: A doctor that specializes in vision issues and eye disease

Transfusion: The process of putting blood into the bloodstream by intravenous (IV, meaning through the veins) infusion into the arm

Splenectomy: Surgical removal of the spleen

Cholecystectomy: Surgical removal of the gallbladder

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