DO YOU KNOW...

Hereditary persistence of fetal hemoglobin

 

Hereditary Persistence of Fetal Hemoglobin (HPFH) is an unusual condition in which red blood cells contain greater than normal amounts of hemoglobin F (fetal hemoglobin). About one in a thousand African-Americans have the HPFH carrier (trait) condition, compared with about 1 in 12 who have sickle cell trait. The gene for HPFH is passed down from parent to child just like hair color and eye color. HPFH cannot be identified with a blood test done at birth because the newborn baby normally has a large amount of hemoglobin F.

In most children, hemoglobin F decreases and is replaced with hemoglobin A during the first few months of life. Children with HPFH continue to make high levels of hemoglobin F throughout their lives.

Having HPFH trait is harmless to the carrier. However, a few newborns inherit a gene for HPFH from one parent and a sickle cell gene from the other. This causes a condition called hemoglobin S/HPFH (HbS/HPFH), which looks similar to sickle cell anemia on laboratory testing at birth.

Special tests are usually needed at about 1 year of age to determine if a child has HbS/HPFH instead of other more serious types of sickle cell disease.

The St. Jude Hematology Clinic follows patients with this condition, but it is rare for these patients to have any health problems related to HbS/HPFH. The symptoms vary in the small percentage of patients who do have problems. Children with HbS/HPFH usually do not have the common symptoms of sickle cell disease, such as pain, acute chest syndrome (pneumonia), blood stream infection, and anemia. For this reason, patients with HbS/HPFH usually come to the Hematology Clinic only one time each year. During this visit, the fetal hemoglobin level is measured.

Important things to know about HbS/HPFH

  • It is important to maintain current recommended vaccinations. Vaccines help fight infection. These may be completed with your child’s primary care provider (doctor) or the health department.
  • Extra vaccines given to children with sickle cell disease to protect against pneumococcal infection (Pneumovax) are not required for children with HbS/HPFH.
  • Your child does not need to take daily penicillin, unless prescribed by your primary care provider to treat an infection.
  • No special fever precautions are required for children with HbS/HPFH. If your child has a fever, you should contact your primary care provider (doctor).
  • Remember to follow up each year with your child’s hematologist.

Does it hurt to get medicines through a nerve block infusion?

No, most children cannot feel the catheter or the medicines at all.

What medicines would my child receive in a nerve block injection or infusion?

Local anesthetics (numbing agents), among other medicines, are used for both nerve block injections and infusions.

Questions?

If you have questions or concerns about your child having a nerve block injection or infusion for pain relief, please talk to the doctor or nurse. If your child is an outpatient, call 901-595-3300 and ask for the anesthesiologist on call. If you are outside the Memphis area, dial toll-free 1-866-2STJUDE (1-866-278- 5833), and press 0 when the call connects.


 

This document is not intended to take the place of the care and attention of your personal physician or other professional medical services. Our aim is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specific treatment options should be discussed with your physician.

St. Jude complies with health care-related federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex.

ATTENTION: If you speak another language, assistance services, free of charge, are available to you. Call 1-866-278-5833 (TTY: 1-901-595-1040).

ATENCIÓN: si habla español, tiene a su disposición servicios gratuitos de asistencia lingüística. Llame al 1-866-278-5833 (TTY: 1-901-595-1040).

تنبيه: إذا كنت تتحدث باللغة العربية فيمكنك الاستعانة بخدمات المساعدة اللغوية المتوفرة لك مجانا. .يرجى الاتصال بالرقم. 5833-278-866-1  (الهاتف النصي: 1040-595-901-1).