Severe Combined Immunodeficiency (SCID) - My Child Has

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Severe Combined Immunodeficiency (SCID)

Severe Combined Immunodeficiency (SCID)

Programs that treat this condition

Immunology Program

Stem Cell Transplantation Program

What is severe combined immunodeficiency (SCID)?

SCID is a group of very rare, life-threatening diseases that are present at birth. The disease causes the child to have very little or no immune system. As a result, the child's body is unable to fight off infections. This disease process is also known as the "boy in the bubble" syndrome, because living in the normal environment can prove fatal to these children.

Children with this rare inherited disease become very ill with illnesses such as pneumonia, meningitis, and chickenpox and can die within the first year of their life. With new advances in treatments for these children, however, children with SCID can be successfully treated.

What causes SCID?

SCID is a group of inherited disorders. Many genetic defects can cause SCID. These defects reduce the ability of white cells in the blood to fight infection.

What are the symptoms of SCID?

Symptoms of SCID usually become apparent within the first year. The following are the most common symptoms of SCID. However, each child may experience symptoms differently. Symptoms may include:

numerous, serious, and/or life-threatening infections that are not easily treated and do not respond to medications (as they would in children without SCID), including the following:

pneumonia - infection of the lungs.

meningitis - infection of the brain.

sepsis - infection in the bloodstream.

other infections, including the following:

chronic skin infections

yeast infections in the mouth and diaper area

diarrhea

infection of the liver

How is SCID diagnosed?

A diagnosis of SCID is usually made based on a complete medical history and physical examination of your child. In addition, multiple blood tests may be ordered to help confirm the diagnosis.

Treatment for SCID:

Specific treatment for SCID will be determined by your child's physician based on:

your child's age, overall health, and medical history
extent of the disease
your child's tolerance for specific medications, procedures, or therapies
expectations for the course of the disease
your opinion or preference

The best treatment for this disease is a stem cell transplant which, in most cases, would cure the problem. A stem cell transplant is a treatment involving stem cells, a specific type of cell from which all blood cells develop. Stem cells develop into red blood cells to carry oxygen, white blood cells to fight disease and infection, and platelets to aid in blood clotting.

Transplantation of normal stem cells from another person is used to help restore normal blood production in patients whose own ability to make any or all of these blood cells has been compromised by cancer, intensive cancer treatment, or other types of damage or abnormality. The use of cells from another individual is called allogeneic transplantation. Stem cells collected from patients themselves prior to intensive treatment can also be used to supplement the recovery of the patient's own cells after particularly aggressive course of chemotherapy or radiation therapy. The use of a patient's own cells is referred to as autologous transplantation.

Stem cell transplantation and the treatment needed to manage its effects are complex. Your physician will give you more detailed information on what to expect.

Other treatment options include giving the child injections to help boost the child's immune system, and treating each infection very promptly and accurately.

What is the latest research on stem cell transplantation for SCID?

In the area of stem cell transplantation, the Dana-Farber/Children's Transplant Program is one of only eight institutions around the country that are investigating the use of umbilical cord cell transplantation. Also under study is a novel method for preventing graft versus host disease, a serious complication that occurs when transplanted cells do not recognize the tissues and organs of the recipient's body and react against the recipient's tissue. The result of this treatment approach, if it continues to be as successful, will be that the degree of match between donor and the recipient will not need to be particularly close, greatly increasing the pool of potential donors for each patient. This could also eliminate the need for long-term drug therapy traditionally needed to treat graft versus host disease.


					Children's Hospital Boston is the primary pediatric teaching hospital of Harvard Medical School

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