Erectile Dysfunction in Men with Sickle Cell Anemia

Erectile dysfunction is common in post-priapism clients with sickle cell disease. Evidence has shown that sickle cell disease has been linked to several reproductive issues, such as low sperm count and decreased testosterone levels. This can create challenges between the client and nurse. Sickle cell clients are already dealing with a chronic debilitating disease. The nurse must be knowledgeable and provide culturally competent care when dealing with these clients. Sickle cell disease can cause a severe hemolytic anemia that results from inheritance of the sickle hemoglobin (HbS) gene, which causes the hemoglobin molecule to be defective. HbS acquires a crystal-like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change; consequently, the erythrocyte containing HbS loses its round, pliable, biconcave disc shape and becomes dehydrated, rigid, and sickle shaped. These long, rigid erythrocytes can adhere to the endothelium of small vessels; when they adhere to each other, blood flow to a region or an organ may be reduced. If ischemia or infarction results, the patient may have pain, swelling, and fever.

Sickle cell disease is characterized by painful vaso-occlusive crisis when sickle-shaped erythrocytes block blood flow to tissues, bones, cells, and organs. Thus, causing ischemia, infarction, necrosis, and inflammation. Sickle cell disease is a debilitating disease. When perfusion is resumed, substances are released (e.g., free radicals, free plasma hemoglobin) that cause oxidative damage to the vessel. In turn, the endothelium of the vessel becomes dysfunctional and vasculopathy develops. Sickling of the blood cell causes reduced blood flow to the penis. The reduced blood flow will lead to vascular thrombosis and priapism may develop. Priapism is a prolonged erection. This condition can lead to erectile dysfunction by causing edema, necrosis, and fibrosis if left untreated. Frequent episodes of priapism can also contribute to significant pain, decreased libido, and impotence. These problems can be a source of extreme embarrassment and depression. Male patients may develop sudden painful episodes of priapism. The patient is taught to empty his bladder at the onset of the attack, exercise, and take a warm bath. If an episode persists longer than 3 hours, medical attention, which consists of IV hydration, administration of analgesic agents, and possible penile intracavernosal aspiration, is recommended. If this condition continues, extensive vascular thrombosis and scarring will develop. The client will become impotent, also called erectile dysfunction.

Erectile dysfunction is the inability of a man to maintain an erect penis. Other characteristics include soft erection, rapid subsiding of the penis, reduced sex drive, and frequency in erections. The physiology of erection and ejaculation is complex and involves parasympathetic and sympathetic components. Erection involves the release of nitric oxide into the corpus cavernosum during sexual stimulation. Its release activates cyclic guanosine monophosphate (cGMP), causing smooth muscle relaxation. This allows flow of blood into the corpus cavernosum, resulting in erection. Current treatment for erectile dysfunction includes pharmacologic therapy, surgical implants, penile injections, and vacuum devices. However, since the causes of erectile dysfunction in these clients are attributed to fibrosis and scarring, injections and implants are the treatment of choice. Penile injections include the injection of alprostadil, phentolamine, and papaverine into the shaft of the penis. These drugs cause vasodilation of the blood vessels, increasing flow of the blood to the penis. Erection can last for an hour, and it’s suggested to inject medication 20 minutes before intercourse. Penile implants involve surgical implantation of a prosthesis into the penis. The implantation will result in a permanent semi-erection.

Conclusion

Practice nurses can play a key part in offering informed education, guidance and emotional support. Sickle cell clients will look to the nurse for understanding and assistance during this process. Be knowledgeable about the client social activities and activities of daily living. Nurses can refer clients to support groups or therapy to help with this issue. It’s very important to include the partner in on teaching about this disorder. Ongoing counseling session can help adapt them to new lifestyle changes.

References

1. Graves, J. K. (2016). Continuing Nursing Education. Depression, Anxiety, and Quality of Life In Children and Adolescents With Sickle Cell Disease. Pediatric Nursing, 42(3), 113–144. Retrieved from https://hindscc.idm.oclc.org/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=tfh&AN=116249507&site=eds-live.
2. Massey, M. T. (2015). Managing infertility in primary care. Practice Nurse, 45(3), 32–37. Retrieved from https://hindscc.idm.oclc.org/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=bth&AN=101673934&site=eds-live
3. Hinkle, J. L., & Cheever, K. H. (2018). Brunner & Suddarths Textbook Of Medical-Surgical Nursing (14th ed.). S.l.: Wolters Kluwer.