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Sacral agenesis

Sacral agenesis (or hypoplasia of the sacrum) is a little known and rather infrequent congenital condition of spinal deformity affecting the sacrum - the caudal partion of the spine. It occurs at a rate of approximately 1 of 25,000 live births. more...

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Etiology

The condition arises from a set of conditions present during approximately the 3rd week to 7th week of fetal development. Formation of the sacrum/lower back and corresponding nervous system is usually nearing completion by the 4th week of development. While the exact etiology is unknown, the condition may be associated with certain dietary deficiencies including a lack or insuffient amounts of folic acid or other developmental aids. The condition may also be associated with or resultant of maternal diabetes.

Prognosis

There are four levels (or "types") of malformation. The least severe indicates partial formation (unilateral) of the sacrum. The second level indicates a billateral (uniform) deformation. And the most severe types involve a total absence of the sacrum.

Depending on the type of sacral agenesis - bowel or urinary bladder deficiencies may be present. A permanent colostomy may be necessary in the case of imperforate anus. Incontinence may also require some type of continence control system (e.g. self-catheterization) be utilized. Occasionally if deformities of the knees, legs or feet would prove unresponsive to corrective action - amputation at the knee may be proposed.

Before more comprehensive medical treatment was available, full amputation of the legs at the hip was often performed.

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Irish diabetes nurse specialist association - The Link
From Journal of Diabetes Nursing, 1/1/03 by Mary Coffey

The 2002 AGM was held at the Galway Bay Hotel, Salthill. As in previous years, an educational update on the Friday afternoon was followed by our AGM on the Saturday morning. This year the focus of the update was Diabetes and Pregnancy.

Pregnancy and type I diabetes

Dr Brendan Kinsley, Consultant Endocrinologist, Mater Hospital Dublin, who also attends antenatal clinics in three maternity hospitals in Dublin, spoke about Pregnancy and type I diabetes. He focused initially on the area of pre-conceptual counselling, noting that hyper-glycaemia is teretogenic. The congenital anomalies associated with poor glycaemic control are already determined by six weeks from conception, e.g. congenital heart defect; neural tube defects; and sacral agenesis. With a booking [HbA.sub.1c] of <8%, the risk of congenital anomaly is 0.7-4.2%. However, Brendan stated that an [HbA.sub.1c] of >9% gives a risk factor of 16-39%.

Reducing risks

Dr Kinsley recommended that women be seen in a combined Obstetric and Diabetology clinic with a dietician and a diabetes nurse/midwife specialist to reduce the risk of macrosomia, difficult deliveries, perinatal mortality and neonatal hypoglycaemia. In conclusion he proposed a heightened awareness and improvement in pre-pregnancy services, folic acid supplementation, early referral to specialist clinics and treatment aimed at reducing postprandial glucose peaks.

Lifestyle changes

Mrs Pauline Gibney, Senior Dietician, National Maternity Hospital (NMH) Dublin, spoke about the importance of good nutrition and healthy eating during pregnancy. She began with a quote from a patient who had type I diabetes for a number of years:

'Being pregnant with diabetes is like being diagnosed with diabetes all over again.'

The basis of her talk was that the changes brought about by pregnancy necessitate lifestyle changes. Mrs Gibney sees all patients with gestational or pre-existing diabetes individually. Her suggested eating plan is three main meals plus three small snacks daily.

Pauline stated the need for assessment of portion sizes, as well as education regarding healthy diet, in relation to activity and work routines. The importance of exercise (walking) on a daily basis was emphasised as an aid to improved glycaemic control.

A holistic approach

Mary Coffey, Clinical Midwife Specialist (Diabetes) in the NMH, Dublin spoke about gestational diabetes. This presentation took a holistic approach to patient care. She stated that screening was done using a glucose challenge test (50g CHO) and a I hour postprandial glucose level at 29 weeks gestation, in those with risk factors for developing diabetes. A GTT was performed if screening was positive. The importance of clear explanations of diagnosis and reassurance was stressed with an outline of expected sequence of events. Mary described the clinic routine, as a patient newly diagnosed with gestational diabetes would experience it. Of those who were diagnosed with gestational diabetes 29% required insulin therapy, with the need for education and support to facilitate self care. At postnatal check-up, a GTT would be performed and an advice sheet given to each patient, suggesting a long-term healthy lifestyle and to plan future pregnancies.

Committee changes

The AGM was well attended and productive. We said goodbye to Nicola Vizzard and Sandra Leeson of the executive committee and welcomed Patricia Keenan and Jackie McMahon to replace them for a 3 year term. Adrienne Brennan has taken over from Sheelagh Wickham as our representative 'on the Diabetes section of the Irish Endocrine Society. Our thanks to each member for her contribution over the past years and we wish them all a well-earned rest!

Pressing Issues

The most pressing issue was the proposed pilot scheme for nurse prescribing. A proposal was made that diabetes nurses/midwives attend their local seminars and focus groups. These meetings have taken place and we await further update.

For further information on the Irish Diabetes Nurse Specialist Association (IDNSA), please contact Mary Coffey, via email, at: Mcoffey@nmh.ie

COPYRIGHT 2003 S.B. Communications
COPYRIGHT 2003 Gale Group

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