Special delivery



Special delivery
--Fabienne Sikron

Life with CPT deficiency is a kind of combat, and birth-giving is only one of the battles, giving us more strength than it consumes.

My first pregnancy was not particularly difficult, I just had to eat enough. When I went to the perinatal high-risk clinic, the doctors did not want to take responsibility for a natural delivery and suggested a cesarean operation. The recovery from the operation was very hard and painful. I was really weak, but that was not the responsibility of the hospital anymore.

After a few months I got pregnant again. (Someone told me breast-feeding prevents pregnancy!) I went to Professor Zakut, who was the director of the department and of the hospital, and implored him to find a way to let me deliver my baby without an operation.
He decided to give me a massive supply of glucose. I didn’t suffer from muscle weakness during the second delivery at all. The day I left the hospital I was already active.

Five years later I became pregnant again. I went to another hospital during my pregnancy and proudly showed them my case study paper. At the time of delivery, they checked for blood glucose levels and gave me glucose all the time. It was a piece of cake.

Now I have three girls at the ages of nine, eight and two.

I think it is preferable not to automatically perform a cesarean operation, but it is very important to inform the doctors about our problem during the pregnancy and not at the last minute. The best way perhaps is to show the Dreval paper and give the team enough time to learn about the subject. Our problem can be nothing if doctors know what to do, but it can be dangerous if they don’t.

I wish the best for all the young women with CPT deficiency who will become mothers--and for us all.

Biographical note: Fabienne Sikron grew up in Belgium and lives in Israel. She and her brother both have CPT deficiency. In 1994 Fabienne’s case history was reported in the American Journal of Obstetrics and Gynecology by Dreval et al. It was the first published account of pregnancy and childbirth in CPT deficiency. The case is also notable in that uterine muscle was biopsied and found deficient in CPT. Previously, it was thought that the deficiency affected skeletal muscle but not smooth muscle. To read about Fabienne’s childhood experiences with CPT deficiency, visit the Mailbox.

Related links:

Anesthetic management of obstetrical labor in a parturient with muscular carnitine palmitoyl transferase deficiency

CPT II deficiency and pregnancy
Note: Scroll down to CPT header on this "Ask the Experts" MDA page. A 26-year-old woman with CPT II deficiency asks about labor and delivery. Answers are provided by two physicians from MDA clinics. Neither mention the Dreval paper or suggest glucose IV during labor. Posted February 2000.

A Baby? Maybe
Four women with neuromuscular disorders tell their pregnancy stories.

What worries doctors most about pregnancy (for women with neuromuscular disorders)
Companion Quest article by Margaret Wahl.

To read about the effects of hormonal changes in CPT deficiency, see Probable cause.

With regard to the risks of pregnancy, you should consult with a "high-risk" obstetrician if you become pregnant. I do not believe that pregnancy is contraindicated, since several of my patients who have CPT deficiency have successfully delivered children. It would be prudent, however, to be cared for by an obstetrician who can anticipate and deal with problems, particularly during actual labor and delivery.

--Lawrence Philips, M.D.