Diagnosis

Blood Transfusion Therapy

One type of treatment that might be recommended for someone with DBA is blood transfusions. Blood transfusions might be recommended just as needed when the hemoglobin is lower than normal, or as a chronic blood transfusion program. Chronic blood transfusions consists of scheduled blood transfusion every 3-6 weeks to maintain the hemoglobin level in a safe range. A DBA patient normally makes his or her own white blood cells and platelets, and therefore would only require transfusion of red blood cells.


Blood transfusions are typically given in a hospital setting. Before a transfusion, a small amount of blood will be drawn from the patient for “typing and screening.” Typing refers to the type of blood the patient has (A, B, AB, or O) and screening refers to identifying whether certain antibodies are present. Generally, people must receive blood of their same type to avoid severe transfusion reactions. These tests are followed by the compatibility testing (cross-match). This test insures that no antibodies are detected in the recipient’s serum that will react with the donor’s red blood cells.


Generally, when patients receive a blood transfusion, they receive blood that has been donated from the general population. In the United States, the blood supply is considered to be safe. The federal Food and Drug Administration (“FDA”) is responsible for ensuring the safety of the blood industry in the United States.


The FDA has established five levels of overlapping safeguards for the industry:


  1. Donor screening: Potential blood donors must answer questions about their health history and lifestyle. Donors whose blood may pose a health hazard are encouraged to exclude themselves. If a donor’s history suggests that he/she might pose a risk to the blood supply, blood donation will be denied for that individual Furthermore, potential donors can be temporarily deferred for a number of reasons, including having a temperature, cold, cough, or sore throat, or if they are taking certain medications, or if they have traveled outside the United States. Additionally, potential donors can be permanently excluded from donating blood if there is evidence of HIV infection, male homosexual activity since 1977, a history of intravenous drug abuse, or a history of viral hepatitis, as well as for a variety of other reasons. For a complete list of reasons go to http://www.fda.gov/cber/blood.htm.
  2. Blood testing: After donation, the blood is tested for blood-borne agents, including HIV, Hepatitis B, Hepatitis C, Syphilis, and Human T-cell Lymphotropic Virus. Any blood that tests positive for any of the these illness, or is suspicious for any reason will be destroyed and not given to anyone requiring a blood transufusion.
  3. Donor lists: Blood donation establishments must keep a current list of deferred donors and cross check donor names against the list, not accepting blood from anyone on that list. In addition, each donor goes through the same rigorous process each time they donate. If their history or health conditions change, each blood bank has policies for tracking these donations.
  4. Quarantine of untested blood: Blood products are not available for general use until the products have been thoroughly tested.
  5. Investigation of problems: Blood establishments must investigate any breaches of safeguards and correct deficiencies. Any manufacturing problems, errors or accidents that may affect the safety, purity or potency of blood products must be reported to the FDA. Furthermore, establishments are required to maintain accurate records for the FDA to review during an annual inspection.

Directed Donors

Some patients and their families elect to use “directed donors.” Directed donors are typically family members and friends with the same blood type as the patient who donate their blood specifically for use by the patient. Some patients and their families feel better about receiving blood from a smaller, known group of individuals, rather than from the general population.


Although directed donation programs are available in most areas, there are some disadvantages to using directed donor blood. Most importantly, blood donated by a very close family member may result in immunologic complications for the recipient such as transfusion associated graft-versus-host disease or transfusion related acute lung injury. Additionally, in the event of a stem cell transplant, complications caused by the development of antibodies against the family member’s blood could prevent that familly member from acting as a stem cell donor. Because of these possible complications, immediate family members should never donate blood to the patient.


Directed donor units of blood must go through all of the screening processes that volunteer donations do. Therefore, medical research has shown that blood from directed donors is not safer than blood from volunteer blood donors. As donors may only donate one unit of blood about every 2- 3 months, multiple directed donors might be needed to provide blood for one patient. There may be additional medical charges for this service, which are often not covered by insurance, so the patient may incur these extra charges.


Anyone interested in directed donors should discuss this option with their hematologist and/or local blood bank.


IV access problems and solutions

It can be very difficult to start an IV in an infant or in a patient who receives chronic transfusions. Some potential solutions include:


IV Transport Team:

Many hospitals have a team of nurses who are especially skilled at starting challenging IVs. In some hospitals this team is referred to as the transport team. In other hospitals, a neonatal nurse may be used. It may be helpful to ask the hematologist whether the hospital has any such skilled nurses. Sometimes the wait is long to get one of these nurses, but the reward of not having to do multiple needle sticks makes it worth it. Do not be afraid to ask for this service if it will benefit your child.


Surgical Intervention:

Some patients who receive chronic transfusions may get a device to make this process easier. Intravenous access devices can be implanted beneath the skin (port) or external.


Intravenous access devices include:

Port: Ports are small medical devices that are installed completely beneath the skin and have a tube that is connected directly to a large vein. A port has a septum which is accessible through the skin, usually with less pain than a typical needle stick. The septum is used for drawing blood samples, giving blood transfusions, and/or for giving medications.


Some common brand names of ports include: Port-a-Cath, Microport, Bardport, PowerPort, Passport, Infuse-a-Port, and Medi-Port. Ports are surgically installed in the patient’s upper arm or chest and typically require general anestheia. DBA patients should ask their physician if they recommend a port made without any metal components. This will allow the patient to be able to have full access to any tests for iron levels in the body.


External Catheter: Another type of intravenous access device is an external catheter. This is a temporary IV line that is placed into a vein through a child’s chest. Unlike a port, part of the catheter remains outside of the child’s body. Like a port, however, the catheter is used for drawing blood samples, giving blood transfusions, and/or for giving medications.


Some common brand names of catheters include: Broviac and Hickman. Catheters are also surgically placed in the patient’s chest and typically require general anesthesia.


Iron Overload

One of the risks of chronic blood transfusion therapy is getting too much iron in the body. Every cubic centimeter of blood contains 1 milligram of iron. A person who does not have DBA uses iron from destroyed red blood cells to create new red blood cells. Since a person with DBA does not make many of his/her own red blood cells, this iron is not needed for the production of red blood cells, and consequently remains in the body unused. When a patient receives transfusions, the iron contained in the donated red blood cells goes into the body. The human body does not have a mechanism for removing any excess iron. This excess iron gets stored in the body’s organs, and if it is not removed, it will slowly destroy those tissues. Iron overload and organ damage can begin with as few as eighteen transfusions. Therefore, it is critical for the health of a DBA patient receiving blood transfusions to monitor iron levels in the body. Medications, known as chelation drugs, are available to help remove the excess iron from the body. You will find more information about these medications in the section titled chelation.


Blood Transfusion Therapy