Human Reproduction, Vol. 15, No. 10, 2152-2153,
October 2000
© 2000 European Society of Human Reproduction and Embryology
Case Report |
Successful pregnancy and birth after IVF in a woman with cystic fibrosis: Case Report
1 Respiratory Medicine Unit, City Hospital, Hucknall Road, Nottingham NG5 1PB, 2 Obstetrics and Gynaecology Unit, Frimley Park Hospital, Camberley, Surrey GU16 5UJ and 3 Centres for Assisted Reproduction, The Park Hospital, Arnold, Nottingham NG5 8RX, UK
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Abstract |
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As survival increases, patients with cystic fibrosis (CF) are often confronted with reproductive issues. Initial reports gave conflicting advice regarding the outcome of pregnancy in CF. However a recent large longitudinal study of pregnancies in CF women suggested that pregnancy has little impact on morbidity or mortality. Reduced fertility in CF women has been described, possibly due to thickened cervical mucus, and intrauterine insemination (IUI) has been used to overcome this. We report the first woman with CF, to our knowledge, to be successfully treated with IVF after repeated failed attempts at IUI.
Key words: cystic fibrosis/fertility/IVF
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Introduction |
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The first successful pregnancy in a woman with cystic fibrosis (CF) was reported in 1960 (Siegal and Siegal, 1960
) but initial reports gave conflicting advice regarding the outcome of pregnancy in CF women with mild to moderate lung disease (Cohen et al., 1980; Corkey et al., 1981; Canny et al., 1991; Edenborough et al., 1995). However a recent large North American longitudinal study of 258 pregnancies in CF women suggested that pregnancy has little impact on morbidity or mortality (FitzSimmons et al., 1996).
Several sources suggest that CF women have reduced fertility (Stern, 1993; Johannesson et al., 1998; Robbins and Ontjes,1999) and possible mechanisms include dehydrated, thickened cervical mucus and cervical polyps (Oppenheimer et al., 1970; Kopito et al., 1973). Intrauterine insemination (IUI) has been used to overcome thickened cervical mucus (Kredentser et al., 1986) but little work has been published in this area. We report the first woman with CF, to our knowledge, to be successfully treated with IVF after eight failed attempts at IUI.
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Case report |
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A 25 year old woman, homozygous for the CF mutation
F508, had a 3 year history of infertility. She was diagnosed as having CF at birth following meconium ileus, had pancreatic insufficiency and was chronically colonised with Pseudomonas aeruginosa. Nutritionally she did not have CF-related diabetes and maintained a pre-pregnancy weight of 54 kg (body mass index 20) by regular nasogastric feeding. Her husband was not a carrier of the 12 commonest CF mutations and his sperm parameters revealed a normal count and motility. The cause of the couple's infertility was thought to be secondary to abnormal cervical mucus as post-coital tests had been abnormal. The couple was counselled with regard to the patient's reduced life expectancy and the impact this would have on the welfare of the child. The implications of the child being a CF carrier, or having CF if the husband carried a rare CF mutation (risk ~0.25%) or even having congenital bilateral absence of the vas deferens (CBAVD) if male (possible if the husband carried a rare CF mutation unidentified at screening) were also discussed. At the time of referral for IUI the patient's lung function was FEV1 (forced expiratory volume in 1 second) 1.71 (53% predicted) and FVC (forced vital capacity) 2.24 (61%). She was treated with 8 cycles of IUI over 18 months. She became pregnant on the third attempt but miscarried in early pregnancy. The couple were referred for IVF treatment in 1998. At the initial infertility consultation, issues relating to the patient's CF were discussed with the couple and the patient's CF consultant, including the effect of the possible risk of multiple pregnancy on the patient's health. The couple decided to proceed to treatment. Five oocytes were recovered by the transvaginal ultrasound-guided route under sedation anaesthesia. Four of these fertilized and two embryos were replaced. Following her first cycle of IVF a rising titre of ßHCG was demonstrated. An early pregnancy scan revealed a singleton pregnancy. The first and second trimester were uncomplicated but she required several courses of i.v. antibiotics in the last trimester. Weight gain during the pregnancy was also difficult, and despite regular nasogastric feeding she lost 2 kg by 27 weeks gestation. She was delivered by elective Caesarean section under spinal analgesia at 35 weeks gestation due to maternal respiratory distress. A baby boy, birth weight 2.43 kg, was delivered with Apgar scores of 9 and normal cord bloods. Mother and baby are well 11 months postpartum.
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Discussion |
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This report shows that IVF as a treatment for female infertility in CF is possible. A successful outcome can be achieved for both CF mother and child with careful patient assessment, combined with liaison between the CF physician, fertility specialist and obstetrician. Careful monitoring of ventilation, immunology and nutrition is important as all these parameters may be adversely affected in a CF pregnancy. Although this outcome was successful, achieving pregnancy through IVF poses additional hazards for the CF patient. Ovarian hyperstimulation syndrome can result in severe respiratory compromise. Avoidance strategies include using a gentle ovarian stimulation regime, close monitoring of the cycle and a low threshold for cycle cancellation. Likewise, multiple pregnancy is associated with increased maternal complications and increased uterine volume can lead to respiratory compromise. By replacing one or two embryos rather than three (the maximum permitted in the UK), the multiple pregnancy risk is reduced but the overall pregnancy rate will be lower. Surplus embryos may, however, be frozen for potential future use. If a multiple pregnancy did arise, selective reduction (termination) to a singleton pregnancy may need serious consideration. Liaison between fertility specialist and CF physician, in conjunction with the couple, is particularly important to ensure that these issues have been addressed.
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Notes |
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4 To whom correspondence should be addressed at: Centres for Assisted Reproduction, The Park Hospital, Arnold, Nottingham NG5 8RX, UK. E-mail: info{at}care-ivf.com
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References |
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Canny, G.J., Corey, M., Livingstone, R.A. et al. (1991) Pregnancy and cystic fibrosis. Obstet. Gynecol., 77, 850–853.
Cohen, L.F., Saint `Agnese, D.I., Friedlander, J. et al. (1980) Cystic fibrosis and pregnancy – A national survey. Lancet, ii, 842–844.
Corkey, C.W.B., Newth, C.J.L., Corey, M. et al. (1981) Pregnancy in cystic fibrosis: A better prognosis in patients with pancreatic function. Am. J. Obstet. Gynecol., 140, 737–742.[ISI][Medline]
Edenborough, F.P., Stableforth, D.E., Webb, A.K. et al. (1995) Outcome of pregnancy in women with cystic fibrosis. Thorax, 50, 170–174.[Abstract]
FitzSimmons, S., Fitzpatrick, S., Thompson, B. et al. (1996) A longitudinal study of the effects of pregnancy on 325 women with cystic fibrosis. Ped. Pulmonol., 13, 99–101.
Johannesson, M., Landgren, B.M., Csemiczy, G. et al. (1998) Female patients with cystic fibrosis suffer from reproductive and endrinological disorders despite good clinical status. Hum. Reprod., 13, 2092–2097.
Kopito, L.E., Kosasky, H.J. and Schwachman, H. (1973) Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertil. Steril., 24, 512–516.[ISI][Medline]
Kredentser, J.V., Pokrant, C. and McCoshen, J.A. (1986) Intrauterine insemination for infertility due to cystic fibrosis. Fertil. Steril., 45, 425–426.[Medline]
Oppenheimer, E.A., Case, A.L., Esterley, J.R. et al. (1970) Cervical mucus in cystic fibrosis – a possible cause of infertility. Am. J. Obstet. Gynecol., 108, 673–674.[ISI][Medline]
Robbins, M.K. and Ontjes, D.A. (1999) Endocrine and renal disorders in cystic fibrosis. In Yankaskas, J.R. and Knowles, M.R. (eds) Cystic fibrosis in adults. Lippincott-Raven, New York, pp 383–418.
Siegal, B. and Siegal, S. (1960) Pregnancy and delivery in a patient with CF of the pancreas. Obstet. Gynecol., 16, 439–440.
Stern, R.C. (1993) Cystic fibrosis and the reproductive system. In Davis, P. (ed). Cystic Fibrosis. Marcel Dekker Inc., New York, pp 381–399.
Submitted on April 27, 2000; accepted on June 22, 2000.
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