Managing PK Deficiency | Know 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:

Hematologist

Family doctor, general practitioner, or pediatrician

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

Registered nurse

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

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.

Right now, there is no cure for PK deficiency.

Your doctor may recommend some of the following options to manage symptoms and complications:

Managing hemolytic anemia

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.

Splenectomy

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.

Managing complications

Cholecystectomy

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.

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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