Model essay.
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There is probably more in this answer than you would be expected to produce in the exam.
Somewhat to my surprise, I was able to hand-write the following in the permitted time.
But we had discussed the topic in the tutorial group a few days earlier, so it was fresh in my mind.
You might think that this is a bit arcane for the MRCOG.
Few of us will ever see a case: there are only 30 - 40 annually in the UK out of a total of ~ 700,000.
But the same applies to topics like pregnancy after kidney transplant, which has appeared a couple of times.
A question on asthma and pneumonia featured in 2009.
So maybe it will be a few years before we get another on a respiratory problem in pregnancy.
There have been articles in TOG in 2009 and OGRM in 2010, so it is a topic that is going to come.
Fortunately it is not that difficult.
When we discussed it in the tutorial group, we came up with more or less all the key points although no one had read either of the above articles recently.
The exam committee likes the sort of question that that generates a wide spectrum of marks and this one would.
The supposition is that the ones with the highest marks are the best candidates and the ones who score badly are the dumbos.
There is no point in them putting on essays for which everybody gets about the same mark.
Of course, with a topic as esoteric as this, it is more a question of whether or not you have had the good fortune to have read the topic recently.
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A nulliparous 26-year-old woman with cystic fibrosis presents for pre-pregnancy counselling.
1. Critically evaluate the factors that would lead you to conclude that pregnancy is contraindicated. 6 marks.
2. Critically evaluate the advice you would give about the effect of pregnancy on progression of the disease. 2 marks.
3. Critically evaluate the advice you would give about the effect of the disease on pregnancy. 4 marks.
4. Critically evaluate the advice you would give re the management of the pregnancy, labour and delivery. 6 marks.
5. Justify the advice you would give re the baby and inheritance of cystic fibrosis. 2 marks.
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You need to consider the template and scoring system carefully.
1. Critically evaluate the factors that would lead you to conclude that pregnancy is contraindicated. 6 marks.
The severity of the cystic fibrosis (CF) and its complications are the main determinants of the risks to the woman and her fetus.
Pulmonary hypertension and cor pulmonale are often lethal in pregnancy, whatever the aetiology, and are major contra-indications to pregnancy.
Severe lung disease (forced expiratory volume in 1 second, FEV1 , < 30 - 40%), greatly increases maternal and fetal risk.
The best outcomes are for women with FEV1 > 70%, but there are no universally agreed figures for a safe threshold.
Malnutrition adversely affects maternal and fetal outcomes and is a relative contraindication.
Expert advice from a physician and an obstetrician specialising in CF should be obtained to give the best prediction of risk.
Pancreatic damage greatly increases the risk of diabetes, but this is not usually a contraindication.
There is some evidence that infection with Burkholderia cepacia increases the risk.
20% of mothers with CF die before the child is ten years old, the figure being 40% for women with severe lung damage.
If her prognosis is poor, this will be a major contraindication for most women.
Particularly as it will be accompanied by an increase risk of maternal death and adverse fetal outcomes.
A few cases of pregnancy after heart-lung transplant have been reported.
There may be an increased risk of graft rejection.
Pregnancy should not take place for 2-3 years after the transplant and expert review.
She may have co-morbidities with poor prognosis, which should be elicited.
If pregnancy is contraindicated, contraceptive advice is essential.
2. Critically evaluate the advice you would give about the effect of pregnancy on progression of the disease. 2 marks.
One might expect the relative immuno-compromise that comes with pregnancy to increase the risk of infection-induced lung damage.
And worsen the prognosis for women with CF.
The evidence is that in most cases pregnancy has no effect on disease progression.
3. Critically evaluate the advice you would give about the effect of the disease on pregnancy. 4 marks.
If the mother has complications of CF such as pulmonary hypertension, there is a substantial risk of death.
In most cases, CF’s main effect on the mother is an increased risk of gestational diabetes mellitus (GDM) if she does not already have diabetes.
This needs to be looked for and managed in conjunction with an expert physician.
The extra nutritional demands of pregnancy may be problematic, particularly in the woman who is malnourished before conception.
Miscarriage and congenital anomaly risks are not increased.
Most of the drugs used are safe in pregnancy, but this needs to be checked on an individual basis.
If the woman has diabetes or develops it in pregnancy, the risks associated with diabetes will be present and need appropriate care.
Premature delivery and IUGR are more common.
Osteoporosis risk is increased in both pregnancy and CF.
4. Critically evaluate the advice you would give re the management of the pregnancy, labour and delivery. 8 marks.
The management must be by an expert multidisciplinary team:
obstetrician, anaesthetist and specialist CF team: physician, bacteriologist, nurse, dietician, physiotherapist.
The paediatric team need to be aware that there is a risk of the baby having CF.
Preconception counselling is essential to identify the risks for her and her baby.
She needs to be checked for severe lung damage, pulmonary hypertension, cor pulmonale and malnutrition.
She needs to be fully informed about the risks of pregnancy and the possibility of the child having CF.
And of any child being left motherless before maturity.
Diabetes needs to be excluded.
If she has diabetes she should have optimum control before conception and be under joint care with a specialist diabetic team.
She should be informed of the extra risks of diabetes and the need for a fetal cardiac scan at 24 weeks.
She needs the standard advice about diet, alcohol, smoking, drugs, healthy lifestyle etc.
She should have her rubella and chickenpox immunity checked and be immunised if need be.
Obesity is unlikely to be a problem, but she should be counselled if she is overweight.
She needs expert advice from the dietician about the extra demands of pregnancy and how she can cope.
Adequate food intake may be further imperilled by vomiting in early pregnancy, which should be treated early.
She may need nutritional supplementation via naso-gastric tube or even jejunostomy.
She should take folic acid 5 mg. daily.
The risk of the baby having CF can be reduced but not eliminated.
The partner’s CF status must be checked.
If he is a carrier, the risk of the baby having CF is 50% and PIGD should be considered.
Alternatively, CVS can be done with a view to TOP of an affected pregnancy.
The woman must be fully counselled about the risk.
Cell-free fetal DNA has been used to screen for CF, but is not available for routine use.
Antenatal care involves the above multidisciplinary team and hospital booking.
Screening for GDM must be done, usually an oral GTT in the first trimester and again at 24 – 28 weeks.
Pre-existing DM and GDM necessitate:
management by a specialist multi-disciplinary team:
physician, obstetrician and diabetic nurse, in a joint clinic
and a fetal cardiac scan at about 24 weeks.
Lung function must be checked frequently.
Infection must be identified early and treated aggressively, often in hospital, with drug therapy paying heed to the possibility of adverse fetal effects.
If the only option to treat the mother is a drug with possible adverse fetal effects, treatment must go ahead with informed consent.
Serial scans are needed from 28 weeks to look for IUGR.
If found, the local protocol for monitoring fetal welfare and deciding optimum time and mode of delivery should be employed.
With measurements of amniotic fluid volume, Doppler studies of umbilical artery blood flow, fetal heart rate monitoring etc.
The timing of delivery should be decided in conjunction with the paediatricians.
Steroids to promote fetal lung maturation may be needed.
Normal labour and delivery should be anticipated.
Regional anaesthesia is not contra-indicated and is preferred to general anaesthesia.
Prolongation of the second stage should be avoided because of the risk of pneumothorax and the mother may not be fit to push for long.
Breast feeding is not contraindicated unless there are drug contra-indications or nutritional problems.
5. Justify the advice you would give re the baby and inheritance of cystic fibrosis. 2 marks.
The mother only has CF genes, so the fetus must get a CF gene from her.
At best, it will be a carrier.
If the partner is Caucasian, he has a 1 in 25 chance of being a carrier.
With a risk of the fetus having CF of 1 in 100.
The partner must be screened.
If he has a negative screen, the risk to the fetus is reduced to < 1 in 250, but not to zero.
This is complex and difficult information to digest.
The woman should be provided with information leaflets, data about useful websites and the opportunity for further discussion.
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The above was written in 18 minutes.
As mentioned above, I had the distinct advantage of having discussed the topic in the tutorial group a few days before.
This highlights the value of going over the “hot topics” and stuff that is difficult to remember in the days before the exam.
The bits in blue are facts that I didn’t know and got from the TOG article:
the % of women who do not survive until their child has reached maturity
and how much screening reduces the risk of an affected fetus..
In the original version I just said “significant numbers”.
In the tutorial we forgot about heart-lung transplant, the sort of stuff that is likely to be on the score sheet and get a mark.
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