NBS-ParentsHbH Oct99 PDF
NBS-ParentsHbH Oct99 PDF
NBS-ParentsHbH Oct99 PDF
Hemoglobin H Disease
California Department of Health Services, Genetic Disease Branch, Newborn Screening Program, 10/99
California Department of Health Services Newborn Screening Program Genetic Disease Branch
To Parents:
California State Law requires that all babies have the newborn screening test before leaving the hospital. A few drops of blood were taken from your babys heel. One of the tests was for hemoglobin disorders. Your health care provider may have recently told you that tests show that your baby has a blood disorder called hemoglobin H disease or hemoglobin H-Constant Spring disease. This booklet was written to help parents learn more about hemoglobin H disease. People with hemoglobin H disease have varying symptoms. Use this booklet to discuss this disorder with the specialists at a hematology center and to help you learn more about how to care for your child. A list of state approved hematology centers is included at the back in this booklet.
Central California
Valley Childrens Hospital, Madera, 559/353-5460 Saint Agnes Medical Center, Fresno, 559/449-5378
Southern California
City of Hope National Medical Center, Duarte, 626/359-8111 ext. 2915 Orthopedic Hospital, Los Angeles, 213/742-1402 Los Angeles County-USC Medical Center, Los Angeles, 323/226-7622 UC Los Angeles Medical Center, Los Angeles, 213/825-6708 Childrens Hospital of Los Angeles, Los Angeles, 323/669-4151 Cedars-Sinai Medical Center, Los Angeles, 310/855-4423 Kaiser Permanente Med. Ctr., So. California, Los Angeles, 323/857-4462 Harbor-UCLA Medical Center, Torrance, 310/222-4157 Long Beach Memorial Medical Center, Long Beach, 562/492-1062 Loma Linda Univ. Medical Center, Loma Linda, 909/799-5283 UC Irvine Medical Center, Orange, 714/456-8411 Childrens Hospital of Orange County, Orange, 714/532-8636 UC San Diego Medical Center, San Diego, 619/543-5670 12
What is Hemoglobin?
Hemoglobin is found in the red blood cells. It gives the blood its red color and carries oxygen to all parts of the body. It is made up of iron and protein chains called globins. There are two kinds of globin in the usual adult hemoglobin called alpha globin and beta globin. The usual adult hemoglobin, called hemoglobin A, has 2 alpha chains and 2 beta chains.
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What Are the Chances of Having Another Child with Hemoglobin H Disease?
There are some combinations of alpha globin gene inheritance that can cause serious health problems. Special blood tests can tell you which combination you have and your chances of having another child with hemoglobin H disease in future pregnancies. Family testing should be discussed with the hematology center doctor. For example, if one parent has alpha thalassemia trait and the other parent is a silent carrier, with each pregnancy, they have a 25% chance of having a child with 4 working alpha globin genes (usual alpha globin), a 25% chance of having a child with 3 working alpha globin genes (silent carrier), a 25% chance of having a child with 2 working alpha globin genes (alpha thalassemia trait), and a 25% chance of having a child with one working alpha globin gene (hemoglobin H disease).
Sulfa Drugs Sulfacetamide (eye drops) Sulfapyridine Salfasalazine (Salicylazosulfapyridine) Sulfanilamide Dapsone Antimalarials Primaquine Chloroquine Hydroxychloroquine sulfate Other Antibacterials Nalidixic acid (Negram) Nitrofurantoins Furazolidone Chloramphenicol Beta-amniosalicylic acid Ciprofloxacin Doxycycline Unless laboratory proven iron deficiency.
Analgesics Aspirin (Acetaminophen is safe as alternative) Phenacetin Acetanilide Tuberculosis Drugs Isoniazid Rifampin Folic Acid Antagonists Pyrimethamine Other Items Iron Supplements Vitamin K analogues Quinidine Gluconate Phenazopyridine (pyridium) Toluidine Blue (a dye) Methylene Blue (a dye) Naphthalene (Mothballs)
Source: Bull WHO 1989, Beutler 1994, updated by Michael Irvin, School of Pharmacy, UCSF, 1998
*The medical literature suggests that these medications and products may cause problems for people with hemoglobin H disease; however no studies have been conducted to prove that these substances are actually harmful for people with Hemoglobin H Disease.
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Many people with hemoglobin H disease do not have serious health problems. However, since this disease affects a persons hemoglobin, they often have mild to moderate anemia which can cause them to be more tired. Sometimes the anemia can get worse and lead to other problems. However, these are rare except for children with hemoglobin H-Constant Spring disease which is a more severe form of this disorder.
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