Infertility Issues in Men with Sickle Cell Disease

Abstract

Patients with sickle cell disease (SCD), especially males, face a key problem in the form of infertility. Apart from low serum testosterone, abnormalities involving accessory sex organs are also observed in these patients. Infertility is a known complication among males with SCD, and it comes about due to reasons such as impotence, relative primary gonadal failure, priapism, and delayed or impaired sexual development. Infertility manifests due to multiple causes, including primary gonadal failure (hypogonadism), erectile dysfunction (ED) as a result of priapism, sperm abnormalities, and delayed or impaired sexual development. The ejaculate volume, sperm density, sperm motility, and normal sperm morphology were significantly reduced in these patients as compared with normal patients. Most importantly, primary testicular failure is marked by low levels of testosterone and infertility results mainly from diseases or conditions that affect and destroy the testis. The low levels of testosterone lead to fertility reduction, which is aggravated by impotence, secondary to earlier priapism. Studies among mice have shown that increased doses of HU increase testicular germ cell apoptosis, decrease sperm count, induce testicular atrophy, increase abnormal sperm morphology, and decrease sperm motility. A multifactorial etiology is responsible for impaired male fertility and it includes sperm abnormalities, ED, hypogonadism, and effect of therapy on sperm function. This paper summarizes some clinical manifestations which play an important role in the development of infertility among men with SCD. The author suggests that prospective studies among males with SCD should be carried out using various end points to determine cellular and functional impairment in fertility.

Testosterone Deficiency in Sickle Cell Disease: Recognition and Remediation

“…Furthermore, compensated hypogonadism (characterized by increased gonadotropins and normal testosterone levels) has also been identified in men with SCD ( 22 ). Smaller testis size in SCD men ( 6 , 23 ) and reduced testis weight in SCD mice ( 24 ) is further evidence of hypogonadism related to this disease.…”

“…Testosterone plays a critical role in muscle physiology, body development, bone density, sexual function, fertility, as well as social, emotional, and neurocognitive functioning in males ( 32 ). Patients with SCD exhibit reduced height and weight, decreased physical strength, and delayed sexual maturation ( 23 ). Low levels of testosterone have been associated with very low bone mass density in SCD patients compared with those having normal bone mass density ( 33 ).…”

“…Although poorly studied in SCD, male infertility is recognized to be a common complication of this disease ( 23 , 37 – 39 ). Impaired male fertility in SCD is due to multiple causes, including hypogonadism, gonadal failure and sperm abnormalities (such as oligospermia, reduced sperm motility and density, and abnormal sperm morphology), decreased ejaculate volume, and delayed or impaired sexual development.…”

“…Prevalence rate of at least one abnormal sperm parameter in male patients with SCD is 91% ( 40 ). Erectile dysfunction, largely as a result of penile damage from recurrent or prolonged priapism, further contributes to reduced fertility in SCD men ( 23 ).…”

Haemoglobinopathy: considerations for reproductive health

“…In contrast, the prevalence of hypogonadism in men with homozygous beta thalassaemia, has been quoted to be in the region of 75% . This may account for the finding of abnormal semen parameters, with some studies suggesting that up to 90% of men with sickling disorders are affected . Erectile dysfunction is also a common finding in men with sickling disorders, at an estimated 21–35%.…”