Sexual Health in Women With Sickle Cell Disease

What Is Sickle Cell Disease?

Sickle cell diseases are a group of inherited red blood cell RBC disorders. Hemoglobin, a protein in red blood cells, carries oxygen to different body parts. These round-shaped red blood cells circulate through the blood vessels carrying oxygen. In sickle cell disease, the RBC is hard, sticky, and C-shaped like a sickle, which is called sickle cell. Due to the change in the shape of the red blood cells, blood flows through the blood vessels is obstructed. This results in proper oxygen supply to various body parts.

How Does Sickle Cell Disease Cause Health Issues?

The sickle-shaped blood cells stop the transport of oxygen to various body organs. The lack of oxygen supply can damage organs and cause pain. The absence of healthy red blood cells causes anemia, making the patient feel tired and weak. The lack of oxygen supply can lead to serious complications like organ damage or inflammation.

What Are the Types of Sickle Cell Diseases?

• Hb SS Diseases - A common type of sickle cell disease, also called sickle cell anemia. It occurs when two copies of Hb S genes get inherited from both parents.

• Hb SC Disease - Second most common type of sickle cell disease. It occurs when one copy of Hb S from one parent and one copy of Hb C get inherited from the parents.

• Hb SB + Beta (Thalassemia) - Sickle cell beta thalassemia occurred when one Hb S gene and a gene for beta thalassemia got inherited from parents.

What Are the Clinical Features of Sickle Cell Disease?

• Anemia (deficiency of red blood cells and hemoglobin).

• Episodes of pain.

• Weakness.

• Feeling tired.

• Swelling of hands and feet.

• Frequent infections.

• Delayed growth.

• Problem with eyesight.

How Is the Sexual Health of Women Affected by Sickle Cell Disease?

The clinical manifestations of sickle cell disease occur within the first year of life.

• Delayed Development and Puberty - In children with SCD, the sickle cells are unstable and die, which leads to low red blood cell count and anemia. Anemia can cause developmental delays. Children with SCD are shorter in height and underweight. Disrupted oxygen uptake by the pituitary gland delays pubertal development. A girl child with SCD attains puberty later than those in the same age group. Lack of pubic hair and delayed breast development are seen in girls. Sexual development follows the bone age. In SCD, delayed maturation of bone also causes delayed sexual development.

• Delayed Menarche (First Menstruation) - Menarche occurs late by two to three years; the possible reasons could be anemia and lower body weight.

• Menstruation Problems - The menstrual cycles are shorter for women with sickle cell disease. Longer days of menstrual blood flow, which is heavy blood flow, severe pain, abdominal cramp

• Pain Crisis - Episodes of extreme pain are an important clinical symptom of sickle cell anemia. The sickle-shaped blood cells block blood flow in tiny blood vessels to the chest and abdomen, resulting in extreme pain. The intensity of pain varies, and it can last up to a few days.

• Pain During Sex - Women with sickle cell disease experience pain during sex. It is more commonly seen in women experiencing pain crises. Certain drugs used to manage pain crises can affect hormone levels, making sex a painful experience.

• Fertility - Irregularities in the menstrual cycle can result in early menopause and ovarian dysfunction and can, in turn, lead to infertility.

• Pregnancy - In a pregnant woman with SCD, the risk is higher for:

1. Premature birth.

2. Low-birthweight child.

3. Extreme pain episodes.

4. Inheritance of sickle cell disease to the child.

5. Poor fetal growth.

6. Severe anemia.

How Is Pregnancy With Sickle Cell Disease Managed?

• Early and regular prenatal care is given.

• Close monitoring of sickle cell disease and the health of the developing fetus.

• Prenatal tests are done to determine whether the child has inherited sickle cell disease.

• Avoiding medications that cause hormonal imbalance and complications to the fetus.

• Blood transfusions are carried out to replace sickle-shaped cells.

• IV (intravenous fluids) are given during labor to prevent fluid loss and blood transfusion is done if needed, ensuring the availability of oxygen through a mask.

What Are the Complications Associated With Sickle Cell Disease?

• Stroke - Obstruction of blood flow due to sickle-shaped cells, causing poor blood supply to the brain resulting in a stroke.

• Acute Chest Syndrome - Poor blood to the lungs can raise blood pressure in the lungs, resulting in acute chest syndrome.

• Organ Damage - Lack of proper blood supply, resulting in inflammation and damage to various organs like the kidney, liver, or spleen.

• Blindness - Blockage of tiny blood vessels results in blindness.

• Deep Vein Thrombosis - Sickle-shaped cells can cause clogging of blood cells.

How Is the Sexual Health of Women Managed With Sickle Cell Disease?

Sexual health can be maintained well with proper medication and medical advice.

• Birth Control Methods - In a woman with SCD, it is advisable to use normal birth control methods. Low-estrogen oral contraceptives are found to be safe and effective.

• Consultation of the Doctor When Needed - Talk to a doctor when facing sexual problems. Various tests are done to find the reason. Seeking medical advice helps reduce complications during and after sex.

• Communication with Partner - Talking with the partner about sexual health helps to understand the problems and can improve intimacy during sex. The partner needs to be supportive to have a good sex life.

• Talk to a Therapist - Various problems associated with sickle cell disease can lead to mental trauma. Stress and anxiety can be reduced by taking therapy sessions. Proper communication and implementing strategies can improve mental health and sexual health.

• Proper Education - In women with SCD, proper knowledge about the disease, sexual health, and probable complications is important. In addition, the management of an emergency and a proper understanding of health issues can help them.

Conclusion

In women with sickle cell disease, sexual life is not that easy. Episodes of intense pain, painful menstruation and sex, complications associated with pregnancy, and other health issues can adversely affect sexual health. Treatments are given to reduce complications and lengthen life. With proper medical care and management, women can have safe sex and a safe pregnancy.