Profile of Reproductive Issues Associated with Different Sickle Cell Disease Genotypes

To describe the reproductive variables associated with different sickle cell disease (SCD) genotypes and the influence of contraceptive methods on acute painful episodes among the women with the homozygous hemoglobin S (HbSS) genotype.

Methods

A cross-sectional study was conducted between September of 2015 and April of 2016 on 158 women afflicted with SCD admitted to a hematology center in the Northeast of Brazil. The reproduction-associated variables of different SCD genotypes were assessed using the analysis of variance (ANOVA) test to compare means, and the Kruskal-Wallis test to compare medians. The association between the contraceptive method and the acute painful episodes was evaluated by the Chi-square test.

Results

Themean age of women with SCD was 28.3 years and 86.6% were mixed or of African-American ethnicity. With respect to the genotypes, 134 women (84.8%) had HbSS genotype, 12 women (7.6%) had hemoglobin SC (HbSC) disease genotype, and 12 (7.6%) were identified with hemoglobinopathy S-beta (S-β) thalassemia. The mean age of HbSS diagnosis was lower than that of HbSC disease, the less severe formof SCD (p < 0.001). The mean age ofmenarche was 14.8 ± 1.8 years for HbSS and 12.7 ± 1.5 years for HbSC (p < 0.001). Among women with HbSS who used progestin-only contraception, 16.6% had more than 4 acute painful episodes per year. There was no statistically significant difference when compared with other contraceptive methods.

Conclusion

With respect to reproduction-associated variables, only the age of the menarche showed delay in HbSS when compared with HbSC. The contraceptive method used was not associated with the frequency of acute painful episodes among the HbSS women.

Keywords:
sickle cell disease; sickle cell anemia; menarche; contraception

Variables	n = 158	%
Age (years) (mean = 28.3)
≤ 19	23	14.5
20–34	93	58.9
≥ 35	42	26.6
Location
Recife	50	31.6
Other cities in the RMA	51	32.3
Countryside	57	36.1
Ethnicity
Mixed	99	62.6
African-American	38	24.01.3
White	17	10.8
Indigenous	02	1.3
No information	02	1.3
Schooling
0 to 3 years	07	4.4
4 to 7 years	18	11.4
8 to 10 years	39	24.7
≥ 11 years	94	59.5
Occupation
No Job/Housewife	40	25.3
Self-employed/Other	27	17.1
Student	33	20.9
Retired	58	36.7
Family Income
≤ 1MW	86	54.4
1–2MW	36	22.8
> 2MW	29	18.4
No information	7	4.4

Variables	All the genotypes(n= 158)	HbSS(n= 134)	HbSC disease(n= 12)	Sβ-thalassemia (n= 12)	p
	Mean (SD)	Mean (SD)	Mean (SD)	Mean (SD)
Age of diagnosis	6.5 (7.4)	5.5 (6.2)	18.9 (9.2)^a The difference was in this group (Tukey test).	5.2 (6.8)	< 0.001*
Age of menarche	14.5 (1.9)	14.8 (1.8)^b The difference occurred only between these two groups (Tukey test).	12.7 (1.5)^b The difference occurred only between these two groups (Tukey test).	13.7 (2.0)	< 0.001*
Age of first intercourse	19.4 (4.2)	19.7 (4.4)	18.3 (2.6)	17.1 (2.2)	0.119*
Age of first pregnancy	22.0 (4.7)	22.2 (4.7)	22.3 (4.7)	19.1 (3.1)	0.248*
	Median (IQR)	Median (IQR)	Median (IQR)	Median (IQR)
Number of pregnancies	1 (1–2)	1 (1–2)	2 (1–2)	2 (1–2)	0.510**
Number of living children	1 (1–2)	1 (1–1.5)	1 (1–1.5)	1 (1–2)	0.427**

Variables	n	%
Sexual activity (n= 158)
Yes	130	82.3
No	28	17.3
Pregnancy history (n= 130)
Yes	89	68.5
No	41	31.5
Unplanned pregnancy (n= 89)
Yes	63	70.8
No	26	29.2
Contraceptive methods (n= 93)
Condom	32	34.4
Combined hormonal	31	33.3
Surgical methods (vasectomy and tubal ligation)	19	20.4
Progestin-only	6	6.5
Others	5	5.4

Contraceptive method	Frequency of acute painful episodes last year
	Up to 3 episodes/year		4 or more episodes/year		p*
	N	%	n	%
Progestin-only (n= 6)	5	83.4	1	16.6	0.093
Combined hormonal (n= 35)	10	40.0	15	60.0	0.072
Non-hormonal methods (n= 73)	36	49.3	37	50.7	0.118
Total	51	49.0	53	51.0

Federação Brasileira das Sociedades de Ginecologia e Obstetrícia Av. Brigadeiro Luís Antônio, 3421, sala 903 - Jardim Paulista, 01401-001 São Paulo SP - Brasil, Tel. (55 11) 5573-4919 - Rio de Janeiro - RJ - Brazil
E-mail: editorial.office@febrasgo.org.br

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[1] Address for correspondence Ariani Impieri Souza, PhD, Instituto de Medicina Integral Prof. Fernando Figueira (IMIP), Rua dos Coelhos, 300 Boa Vista, 50070-550-Recife, PE, Brazil (e-mail: ariani@imip.org.br).