Wednesday, August 17, 2016

MATERNAL MORTALITY IN INDIA IN 1960

These stories are from1960 when the maternal mortality rate in 
India was very high. Almost, 600 women died out of 100 000, live births. With the introduction of modern health, it has come down, one almost never sees what I am going to describe. These procedures were historically first done in the18th or 19th century.
(Warning)These stories are not to be read by the faint of heart. This day in the monsoon season was the hardest day of my 55 years of working life. The rain was nonstop, the roads? If we can call them that were flooded, Ram Devi came to our hospital in a bullock cart after being in labour for 2 days in this rain. She was 32 years of age had had 5 babies at home without any problems.  When she arrived her baby was lying across her stomach, the head was on the right side of her belly and a hand was prolapsing from the vagina. Above all this baby was dead and the mother was dying as well. She was septic, had a very high temperature and her haemoglobin was only 6. Our assessment was that her uterus was probably intact but very thin. We could not have done a Cesarean for a dead baby lying across the belly instead of lengthwise.
She was very weak; we had no trained anesthetist I was still very junior, I called my chief resident she decided to do what we called embryotomy. This means the dismembering of a dead fetus to remove it when normal birth and even Cesarean section is not safe. After this destructive procedure the woman can have a normal birth next time. My chief resident had witnessed an embryotomy but never done one herself. It required a lot of skill. Any way one of the residents went to pathology which was about half a kilometer from the hospital to cross match blood for her. I decided to help the patient to sleep after putting the woman in a comfortable operative position we started the antibiotic, gave her pethidine and Valium cleaned all the operative field put a catheter in her bladder, unfortunately the urine was blood stained. We were not sure if the uterus may have ruptured. The cervix was fully dilated, anyhow we proceeded with dismembering the baby. First the head, then the body; trying to protect the vagina all the time. At completion the uterus felt intact, we gave her the 2 units of blood. We all were happy as if we had saved the mother. We wrapped the baby parts and disposed of them. Since then I have never seen a case where this procedure has been required. I have seen a live baby with hand prolapsed on two occasions, and saved the baby and the mother both by Cesarean section.
On this occasion our mother died three hours later probably due to septicemia.
While we were still overcoming this disaster, Sitara a nineteen year primigravida came in with obstructed labour with a dead fetus. Her pelvis was very small and she had been in labour for many days. She was also septicemic and very ill. We had to do
a destructive operation for her called Craniotomy in which we crush  the head, compress it and remove it. We gave her some blood and antibiotics but could not save her.
Within 2 hours after this second maternal death, Bimla Devi aged 22 having had her second baby came in with a retained placenta. She had delivered about ten hours prior had been bleeding, but the placenta did not deliver naturally. Unfortunately
She was dead on arrival.
I was totally broken, three maternal deaths in one night.
The World Health Organisation has introduced a program called The Millennium Development Goals trying to improve maternal health and mortality rates around the world.

This includes nutrition, infectious diseases and they hope that things will improve by 2030. The most affected countries which need help are, India, Pakistan, Papua New Guinea and some African countries. 

Wednesday, August 3, 2016

TURNERS SYNDROME

Turner’s syndrome is a condition in which a human female is missing one of her chromosomes. As I have already discussed
Humans have 46 chromosomes. They appear in pairs of 22 which are anatomical chromosomes, and 2 are the sex chromosomes, XX in female, XY in male.  If a female child is born with only one X chromosome it does not develop in to a normal female. The general characteristics of these are very variable depending on as to how many cells are missing one x chromosome. This is called  Mosaicism
Even in utero this foetus does not develop normally its tissues swell and it develops thickening round the neck called cystic hygroma, lower than normal weight, swelling of hands and toes. This diagnosis can be made from the blood test from the foetus and pregnancy can be terminated. It is not an inherited condition, it happens during the process of reproduction. At birth a baby born with turners’ syndrome shows broad neck, small weight, hands that turn out, a high narrow palate and swollen hands and feet.

It may even look like a normal female baby depending on how many cells within the body are abnormal, this is called mosaicism.  A lot of turner syndrome pregnancies are lost as miscarriages, some are terminated and some are born normally.  They can appear normal up to 3 years of age, then their growth spurts stop, they have learning difficulties and puberty does not happen. Periods do not happen. The growth problem can be helped by female hormones. In present day with the help of IVF they can even have a baby. In my time I have terminated two pregnancies, looked after a woman who had a baby with the help of IVF. I must tell you this last story Tina had 7 miscarriages under my care, other experts and I could not help her when she was having her 8 pregnancy she was 41 years of age. Investigations into the wellbeing of the foetus showed it had turner syndrome but otherwise the baby appeared normal.She decided to have this baby. The baby was born in good condition and grew nicely up to 10 years of age unfortunately I lost contact with them. I hope she is growing up nicely and normally.


GENETIC OR CHROMOSOME ABNORMALITIES

Let me focus on chromosomes, these are thread like bodies in the living cells of each living being which give us our particular characteristics, these contain our DNA and GENES. As the cells divide they must transfer the exact number of cells into the new cells. We humans have 46 chromosomes and they live in pairs. 44 of these give us our characteristics e.g. our height, our eyes, that we get from our parents. Two of these chromosomes are sex chromosomes X and Y. A human female is XX and male is XY. When reproduction takes place these can be mixed up and produce children with abnormal sexual identity. When the chromosomes in other 44 pairs are mixed up they produce children with physical abnormalities named as different syndromes. Many of these have been identified and named but we are still working on many others when we cannot give a diagnosis to an abnormal child.
In this post I am going to describe two such cases with abnormalities of sex chromosomes.
Ronald and Teresa came to see me because they had been married for four years and unable to achieve a pregnancy. Teresa was 26 years of age well built like a normal female. She has never had any problems with her menstrual cycles. It seemed that she makes an egg regularly and in fact her test for ovulation was very good. Ronald’s who was 28 years of age was normal in appearance, normally men with Klinefelter  Syndrome are taller, but he was of normal height ,5ft 7inches he had no gynaecomastia(Breasts) which they often have, although the hair on his face and hands was scanty. This is another feature of Klinefelter Syndrome. Cardiac abnormalities are also noticed with this syndrome however not in this case.

A test for semen analysis showed azoospermia. The testicular hormone was low. When his chromosomes were done they were XXY which is a predominant feature of Klinefelter Syndrome. So obviously he had Klinefelter Syndrome. Sometimes the chromosome can go haywire they can be XXXY and so on. More number of X chromosomes, more obvious the condition. This was first described by Harry Klinefelter in1940 hence the name. It occurs in one child out of 590 births .Things can be improved as regard the appearance of the adolescent if diagnosis was made early and he had been given testosterone as an adolescent. In some modern cases infertility is treated by IVF by intracytoplasmic injection. I had referred this couple to IVF.