Q: My 32-year-old wife, mother of two children, is suffering from FSGS (Kidney problems - biopsy proven) and AIHA - severe anaemia- Low Hb - 4 to 5. Both the treatment and the opinion are very costly. Doctors advised gamma-globulin, which is temporary (life for 21 days) and they are not sure if it will work. This treatment will cost more than Rs 2.5 lakhs and all other treatments including anti-CD-30 are even more costly for which I have no money. Till last year, before steroids were given she was better, but after steroids, her life has become worse and she has experienced worse health. How can my wife be cured? Kindly advise.
A:I am sorry to learn of your wife's illness and can well imagine your distress.
Haemolysis is the premature breakdown of red blood cells (RBCs). This can lead to anaemia (haemolytic anaemia) when the bone marrow cannot compensate for the red cell loss. Clinical presentation of a patient depends on whether the onset of haemolysis is gradual (chronic) or abrupt (acute) and on the severity of red cell breakdown. Haemolytic anaemia may be due to a large number of hereditary or acquired causes, one of which is immune destruction of red cells. Though haemolytic anaemia may be seen at any age, hereditary disorders are the usual cause in children while autoimmune haemolytic anaemia (AIHA) is more likely to occur in middle-aged and older individuals. The direct antiglobulin test (DAT) is usually positive in AIHA but may be occasionally negative in some patients (5-10%). In such instances, the polybrene test can detect the disease.
In AIHA, antibodies (proteins that can react with an antigen on the surface of the red cell) usually of the IgG or IgM type, bind to the surface of RBCs. The red cells then stick together, rupture or are destroyed in the spleen. This may be due to several causes:
Idiopathic (no obvious cause) is seen in more than 50% cases.
Secondary to an underlying disease, usually an autoimmune disease (like systemic lupus erythematosus, rheumatoid arthritis, ulcerative colitis, a malignancy, etc.).
It may be drug induced.
It is important to investigate if the patient has been taking a drug that is known to cause haemolysis.
The treatment of AIHA varies depending on the cause of the haemolysis, the severity of the anaemia, and on the response of the patient to medication. If the antibody is an IgG antibody, cortisone and other drugs that suppress the immune system (immunosuppressives) may be very effective. Diseases associated with an IgM antibody most often do not respond to treatment with these drugs and cortisone. Folic acid supplements are given since the increased production of red cells by the bone marrow can lead to a deficiency of this vitamin.
However, in some cases, AIHA can be characterised by a chronic course and an unsatisfactory control of haemolysis, thus requiring prolonged immunosuppressive therapy. Sometimes when medical measures fail, it may be necessary to surgically remove the spleen (splenectomy). The clinical course of the disease may show either resistance to steroids or dependence on high-dose steroids with subsequent development of severe side effects on growth, bone mineralisation, and the endocrine system. Splenectomy is effective in about 50 to 60 percent of the time in IgG antibody diseases but is not usually effective in IgM antibody haemolysis. Splenectomy is of benefit in these people because the spleen behaves like a sieve and if it is removed, even though the RBCs are coated by antibodies, they are no longer caught and destroyed in the spleen.
There are some patients in whom splenectomy, use of immunosuppressives like azathioprine, cyclosporine A, or cyclophosphamide, or immunomodulating agents, such as intravenous immunoglobulin, have been used frequently, alone or in combinations, with the aim of reducing steroid dependence and controlling haemolysis. However, these therapies are not consistently effective and present a risk of infectious complications due to their profound immunosuppression. In such instances there has been a trial of monoclonal antibodies like rituximab and alemtuzumab and these have shown great promise.
You need to discuss this with your doctor who will be able to advise. You may also try and contact some local NGOs, self-help groups or the hospital social worker, who may be able to assist you financially.
