Bubble boy disease is a genetic disorder that may lead to immune deficiency, it is highly infectious one. Most of us don’t come across this disease. The possible treatment for this disorder is only gene therapy. Bubble boy disease is also known as SCID. This disease came to exist on 1970. It is found in birth itself, that’s why bubble boy disease is also said as birth disorder. It occurs very rarely, bone marrow transplantation is highly recommended in this disease. In this disease cells that safeguard from high infections are destroyed, so cell damage occurs. Our immune system is highly affected in this bubble boy disease. It is a hereditary disease. SCID full form is severe combined immune deficiency. Those people who are affected with this condition have a weak immune system or simply low immunity power, so the chances of getting infections on them are higher, so they have to safeguarding them by staying in environment which is free of germs.
The treatment given before for this condition, in that time bone marrow transplantation was failed. But now medical advancement pays way for successful bone marrow transplant. This bubble boy disease is found mostly among males. Out of fifty thousand one get affected by this condition. It is highly fatal disease, so immediate medical attention is advised. Treatment for this disease can be gene therapy, enzyme therapy or blood forming stem cell transplant.
When the non-functioning gene present on cell is transplanted with functioning gene, then the cell can turn in to normal condition. This is the simple concept of treatment on this bubble boy disease. Busulfan Conditioning combined along with gene therapy helps in treating this condition, the patient then has stronger immune power. Busulfan is nothing but a drug used during chemotherapy, in this gene therapy lentivirus is used, since it has incubation period at high rate. The right mutant gene is attained from re-engineered lentivirus. Gene therapy must be done at earlier stage of the disease; otherwise it could take over your life.
Various forms are found in bubble boy disease. Mostly found in chromosome- X. On these condition people without getting cold infections we have to see them, otherwise that might destroy them. Then see how it would be powerful and scarring disease. In our immune system, multiple parts were affected at the same moment, so early diagnosis of this bubble boy disease is suggested.
Symptoms of bubble boy disease
- Weight gain is lower.
- Retardation or growth failure.
- Thrush not get cured at all.
- Infection occurring rate higher.
- When given antibiotics for healing infections, it won’t work at all for even 3 months continuously.
The bubble boy disease is also called by other names like Alymphocytosis, and Glanzmann Rinker syndrome.
Tests for diagnosing Bubble boy disease
There are several test carried out to diagnosis this condition, let’s see it what are they.
- Immune function blood test
- IgM level
- IgA level
- IgM level
- T cell level
- B cell level
Causes of SCID
- Production ability of Il-2 receptor gamma is unavailable
- Adenosine de-aminase enzyme is absent
These are the possible causes for bubble boy disease.
Treatment methods for SCID
- Enzyme therapy
- Gene therapy
- Bone marrow transplant
- Intravenous administration of immunoglobulin
The antibodies on this bubble boy disease conditioned patients body will be lower; particularly IgA, IgM and IgG, so intravenous administration of antibodies like immunoglobulin IgG are highly advised in this condition. Initial diagnosis test may not find this condition, so your doctor advices further test repeatedly to diagnosis the disease on affected individual.
Complications of bubble boy disease
- Impairment of cell-mediated immunity power
- Impairment of antibody-mediated immunity power
This condition is very rare only, that’s why many of our lives are safeguarded. But once you note down any of the above said symptoms, please don’t deny consulting a well known physician, otherwise it would be life threatening one.
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