A Case Of Griscelli Syndrome: The Silver Baby
NATIONAL JOURNAL OF HOMOEOPATHY 1999 May / Jun VOL VIII NO 3.
Dr Mirza Anwar Baig
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Editor: This unique and rare case has had tremendous coverage in the newspapers in Mumbai. It is also something of a pride for the Homoeopathic science. That the baby has benefited, is beyond doubt and borne out by the series of photographs. Therefore reporting the case was mandatory for NJH.
The treatment and reasoning leaves much to be desired, yet we spent a lot of time with the doctor to try to understand his thought processes and to get accurate follow-ups.

Introduction :
'Griscelli Disease' is a congenital immune deficiency syndrome [NOT acquired immune deficiency syndrome -AIDS] due to genetic mutations, maps to chromosome 15q21. Worldwide, only twelve cases have been reported so far. This 13th, case in India (India's first silver baby), came under my treatment. Though it is a little premature, I would like to share the case with you. She is today 6 mths old. She came to me at 24 days and then at 4mths. She suffered from recurrent attacks of pneumonia, bronchitis, fever with enlarged spleen and liver and jaundice since birth. Her only brother died of the same disease in 1998 at age 3 mths. Actually their skin pigmentary dilution differs from other humans, so they look unique- with silvery gray hair.

For the same reason, they show abnormal antibody response to antigens. This is a peculiar kind of immune deficiency disease and one most likely to be misdiagnosed as Chediak - Higashi Syndrome (common among partial albinism), Thallasemia (due to enlarged spleen) or AIDS (because of recurrent infections). Dr C Griscelli, MD of France was the first to identify this syndrome. There is little literature or treatment available anywhere in the world; only internet provided some information, with respect to their congenital anomaly, partial albinism, immunodeficiency and association with skin cancers, but its association to tuberculosis has, to our knowledge, not been reported..

It is an autosomal disorder. The pigmentary dilution is characteristic; they show large clumps of pigments in the hair shafts and an accumulation of melanosomes in melanocytes. They are incapable of showing normal skin hypersensitivity. According to Dr Mirza Anwer Baig, who is treating her in Mumbai, the cause could be due to Inherited Tuberculosis.

He says ill-treated sexually transmitted diseases have given rise to AIDS; similarly suppressed TB can present with still more mysterious diseases like Griscelli. In the final analysis, HIV or TB Bacilli are not the cause, but just the trigger; for the soil has been well prepared by our miasmatic heritage through our genes, which has to be cleansed and treated first. Nosodes play a major role in this.

Griscelli, as a clinical diagnosis may be new, but such altered mysterious behavior of a cell due to these pigments, has been described centuries ago by the Persian Arab Physician, Bu-Ali Sina [Abisina]. He called this group of diseases: Saudavi- Amraz. In Arabic, sauda = a kind of craze and amraz = diseases. Abisina [980-1037] has written in his book Al-Qanoon that, when black pigments become abnormal, the humour [blood] becomes thick and forces cells to behave abnormally.

The American Journal of Medicine, Vol. 65, Oct 1978 has published the research work of Dr Griscelli et al along with few case reports of the same disease.

The First case was a 11-year-old North African girl, born on Aug 30, 1965; her parents were first cousins. Her birth weight was 3.6 kg, and her hair looked silvery gray.
BCG at birth. At 2 mths-diarrhoea. At 7 mths- hospitalized for pneumonia and peritonitis. Treatment with intravenous antibiotics resulted in diminution of lung abnormalities and hepatosplenomegaly. Yet died ? of recurrent respiratory infections, hepatosplenomegaly with generalized lymphadenopathy. At the time of death, her hair, eyebrows and eyelashes were silvery grey. Blood chemistry showed no abnormalities, except low level of gamma globulin and a pronounced hyposidermia.

The Second case: also a girl, fifth child of Caucasian parents, born on May 21, 1975. Her sister and a brother died at 2½ and 1½yrs respectively. Her hair looked silvery blonde, and skin was abnormal colour with depigmentation. Liver, spleen and nodes were enlarged. Died of septicaemia, due to recurrent infections.

Our Case
Introduction: Our purpose here is to describe the association of suppressed tuberculosis to the most peculiar defect in these two children born of the same parents. The father, who is mother's distant cousin and 20 yrs older, was treated for tuberculosis in his childhood. The mother, 24, was treated for recurrent ear discharge of tuberculosis type in her early childhood. So from the parents, genetically this child had a double load of the tubercular miasm. A person, who has one copy of the same gene, is a carrier of a genetic condition. A recessive condition results when a child inherits two copies of an altered gene, one from each parent. Both parents must be carriers of the same recessive disease gene in order for any of their children to be affected. Carriers themselves do not get the disease, and a carrier status does not affect the person physically or mentally; they only transmit the particular genetic trait to their child.

Case 1: Mrs N, a intelligent young lady, married to her distant cousin, delivered a FTND- male child with low birth weight in a public hospital. Soon after receiving BCG, the child passed a big stool and developed severe respiratory distress. The grandmother described this. The child was taken to ICU, where he was misdiagnosed as albinism. Thereafter he developed various complications, one after the other and was treated symptomatically for each. His condition went from bad to worse, the parents took discharge against medical advice and consulted Dr Baig.

The child was in state of shock, with platelet count markedly low, Ammonia content very high. Blood culture showed Pseudomonas. History of 4 to 6 blood transfusion. F/H Jaundice-Mo, seven days before his birth. 3rd day of his life developed a benign growth on the forehead, which was tested.
Final Diagnosis: pre- maturity, neonatal sepsis, CCF, chronic lung disease with necrotising enterocolitis.

When we saw the child, he had already turned into ashes with distended abdomen from marked hepatosplenomegaly; and was fighting for his breath. We found his remedy in the ashes of vegetable charcoal, on similar lines. His hair, eyebrows and eyelashes were also silvery grey. On 24/2/97 few doses of potentized vegetable charcoal were given to boost his vital force. On 25/2/97 Thuja 200, single dose on basis of AF BCG. Thuja, in dynamic form boosts the immunity which is affected due to sycotic cause. The other remedies used were Phosphorous and Syphilinum as an inter-current.

It helped the fight. One month's treatment helped in diminution of the lung abnormalities and hepatosplenomegaly. No antibiotic or other life saving drugs used.
At 2½ mths, the child received triple polio. This time his abnormal antibody response to the antigen was so severe that he was immediately admitted to Wadia hospital, where he was misdiagnosed as 'Thallasemia'. Though the parents consulted us, it was too late, we were helpless and the child died.

Second Case
After a yr the mother delivered Baby F, the silver baby. FTND in a private nursing home. At discharge given oral polio. On the very same day she developed fever with hepatosplenomegaly, and was sent to Wadia hospital and finally diagnosed correctly.
The Serum Immunoglobulin levels in these two patients (brother and sister) are shown below in the Table. This shows their strong susceptibility to various infections.

Table: Serum Immunoglobulin Levelsof both cases: Torch ( Elisa) Test reports

The child was discharged from the hospital on 24/12/98 and was brought to us on 25/12/98. She was only 24 days old.

The First Prescription: The child slept with one eye half open. Frequent hiccoughs in sleep. Trauma of medical procedures, and investigations eg bone marrow examination at 6 days age. Sleep with one eye half-open was interpreted as the relaxed posture of Arnica. Arnica 30 single dose was given.

Silicea, selected on her totality followed this. Phosphorous and Syphilinum were given intermittently. The child improved, her liver and spleen reduced almost to normal. A relapse after a month was treated on the same lines. The weekly blood transfusions are now down to monthly, but the fight is still on.

Later Follow Up: When brought at 4mths

Some Possible Conclusions

1. Griscelli Syndrome shows a deep seated patho-physiology: Psoric1 Tubercular2 Syphillitic3. Miasmatic heritage.
2. The first symptom in the form of allergic respiratory distress or benign growth shows deficient response in the antibody production. According to Dr Baig such infants can be prevented through homo immunization after studying their intra-uterine life, which can correct at the level of sick genes.
3. These children are very susceptible to any kind of antigen: even a booster dose of conventional vaccines causes severe reaction, let alone skin cancer screening tests, micrographic surgery, or excision biopsy.
4. Whether it is silver baby or other mysterious disease, like Tay-Sachs, Down syndrome, Maple Syrup disease, Metabolic block or metabolic disorders etc. the cause is a Genetic defect.
5. One important observation: 'Syphilinum' prescribed as intercurrent acted well but Tuberculinum aggravated in 2nd case and the child's condition deteriorated. That shows Syphilinum acted on line of similars.
6. These cases are also of acquired deficiency syndrome but not HIV, but a tubercular taint acquired from parents. It is an alarming situation if ill treated sexually transmitted disease has given rise to HIV, suppressed conventional anti tubercular treatment might result into complex tuberculosis, falling under the Tubercular Miasm.
   
   Phosphorous was discovered in 1673 by Brandt, an alchemist of Hamburg and was used in medicine as a tonic, but Hahnemann's proving showed us many other indications and the silver baby could be one such. Phosphorus alone could win half the battle against TB. Syphilinum can protect these delicate children, born with some congenital defects due to suppressed tuberculosis."
7. These two cases illustrate that modern medicines have failed to describe the actual causation and the treatment. Even bone marrow transplant could not have saved these children but only palliate the Thallassemic- like syndromes for some time. Dr Griscelli says that it is a caprice of nature, which curtails the lives of such children.
8. Homoeopathic explanation in terms of miasms also offers a basis for treatment. The specific characteristics of these patients are, that despite their deficiency in immune response and abnormal reaction to antigen during infection; the humour also shows other signs of recession; it pools down in venous side resulting in pseudo hepatosplenomegaly, pseudo CCF, pseudo chronic lung disease; acute cor pulmonale and their recession of RBC s gives a picture of false anemia
   It is because when the fever goes away all functions of these organs become normal.
9. Explanation For The Remedy Selection: Phos proved as effective remedy to check their respiratory infections, while Syphilinum, after clearing its tubercular association (Syphilitic miasm) built up her immunity.
   
   Iodum prescribed on 23-4-99 has helped her to come out of her thalassemia like syndromes (Tubercular miasm).
   
   The child is now 6 months old and has not received any blood transfusion during the last 2 months. Fever and umbilical hernia responded well to Lach, which also attacked the sycotic miasm. The Lach prescription also got confirmation from mother's mental state. Mo was 24, Fa 44. In that period he had had a special girl friend. Mo was very jealous when she found out. (A little far-fetched !) Lach was given.
   
   The child's Hb did not fall this time. Her weight has increased from 2.3 kg to 6 kg- 3.7kg/ 6 months; she eats well and plays with every one. Photos taken before and after are conclusive evidence of the efficacy of Homoeopathic treatment.

Editorial Comments: 
Dr Baig has been presenting many such difficult, abnormal cases, autosomal cases in the NJH. From the Homoeopathic Standpoint, these cases fall under the syphilitic miasm.
An important social point arises from this. Could these cases be predominant in the Muslim community due to consanguineous marriages? Most probably, yes.
If this is so, then we as Homoeopathic doctors have more than a medical commitment, we have a social commitment. Fortunately we have a large no of Muslim patients; more importantly, we have a large number of Muslim Homoeopaths. So the avenues to advise and bring about changes in the community's social norms seems to a distinct need and efforts, if made, will pay off. Let us apply our mind to this problem, and start working on it, in both our individual capacity and as a group.