Dr Abbie Laing

World Sickle Cell Day: 19th June 2021

Dr Abbie Laing, GP with a special interest in menopause and writer for The Menopause Charity answers these important questions:

What is Sickle Cell Disease?

Sickle cell disease is the name for a group of inherited conditions that affects red blood cells. Healthy red blood cells are round and can move freely through small blood vessels (1). However, for someone with sickle cell disease the red blood cells are harder, stickier and C-shaped like a sickle (1).

What is the problem with this?

C-shaped sickle cells can get stuck in blood vessels and clog the blood flow. Depending on the vessel involved this could cause many different problems, with some being serious. Here are some examples:

Pain Skin ulcers Chest pain
Infections Visual loss Blood clots in legs or lung (2)
Fever Problems with your spleen Stroke
Swelling of hands and feet Poor bone health (3)

Another problem with C-shaped sickle cells is that they break down easily. This can cause low red blood cells levels, known as an anaemia. Symptoms of an anaemia include tiredness, irritability, dizziness, palpitations, shortness of breath and looking pale. It is diagnosed following a blood test.

What are the types of Sickle Cell Disease?

There are different types of Sickle Cell Disease and this depends on the genes a person has inherited. The sick cell gene is termed Haemoglobin S (HbS). The abbreviation ‘HbSS’ means the individual has inherited two sickle cell genes (S), one from each parent. There are many other types.

What can help?

Simple steps can be taken to help prevent problems, these include drinking plenty of water, avoiding getting too hot or cold and staying away from high altitudes or places with exposure to low oxygen levels (4). Some adults with severe symptoms take a medication prescribed by their Specialist called Hydroxyurea (4). The only cure at the moment is a stem cell transplant.

What are some things to consider during the menopause?

  • Sickle Cell Disease can cause an earlier menopause in some women:

The average age of the menopause is 51 years. However, C-shaped sickle cells might clog small blood vessels that travel to the ovaries and this could, for some women, contribute to them turning off earlier (5,6). Hydroxyurea has also been linked with an earlier menopause (7).

  • It is easy to miss the symptoms of the menopause when living with Sickle Cell Disease:

Sickle Cell Disease causes many different symptoms so it can be tricky to work out if a symptom is being caused by the Sickle Cell Disease or something else. It is really important to have a thorough assessment of any symptom when living with Sickle Cell Disease. During this you can ask your Clinician ‘could this be the menopause?’.

  • The type of HRT needs to be chosen carefully:

Because women living with sickle cell disease have an increased risk of blood clots, heart attacks and stroke the choice of HRT needs careful consideration and you should take your time to discuss this with your Clinician. Unfortunately, there have been no studies that look specifically at the safety profile of HRT in women with Sickle Cell Disease. However other studies have shown that certain types of HRT do not increase the risk of stroke or blood clots above a woman’s baseline risk (8,9,10). These types of HRT include an oestrogen prescribed as a gel or a patch and Micronised Progesterone and should be considered the safest type for women with Sickle Cell Disease. There are some types of HRT that should not be used in women with Sickle Cell Disease. This includes HRT containing oestrogen in a tablet.

  • HRT started at the right time is protective for your heart:

Sickle Cell Disease can affect the heart, for example by sickle cells getting stuck in its small blood vessels. It is important to look after your heart and consider the effect a medication might have on it. If oestrogen is started within 10 years of your last menstrual period or under the age of 60 years it is considered to be beneficial for your heart (8,11).

  • HRT helps bones:

Another complication of Sickle Cell Disease is developing lighter, more fragile bones, a term called osteopenia or osteoporosis (3). The middle part of bones produce red blood cells and one theory is that in women with Sickle Cell Disease this part of the bone expands which leaves less room for the surrounding bone to provide strength (3). Oestrogen can help to protect against further bone loss associated with the menopause (12).

  • Hydroxyurea can be taken with HRT:

Hydroxyurea is a medication used in some women with Sickle Cell Disease. It is not known to interact with different types of HRT and can be taken together (13).

References

  1. Centres for disease control and prevention. What is Sickle Cell Disease? December 2020. https://www.cdc.gov/ncbddd/sicklecell/facts.html
  2. Naik RP, Streiff MB, Haywood C Jr, Nelson JA, Lanzkron S. Venous thromboembolism in adults with sickle cell disease: a serious and under-recognised complication. Am J Med.2013;126(5):443-449.
  3. Sarrai, H. Duroseau, J. D’Augustine, S. Moktan, R. Bellevue. Bone mass density in adults with sickle cell disease. Br J Haematol., 136 (2007), p. 666-672
  4. Centres for disease control and prevention. Complications and treatments of sickle cell disease. December 2020. https://www.cdc.gov/ncbddd/sicklecell/treatments.html
  5. Kopeika, A. Oyewo, S. Punnialingam, N. Reddy, Y. Khalaf, J. Howard, et al. Ovarian reserve in women with sickle cell disease. 2019
  6. Ghafuri D, Stimpson S, Day M, James A. Fertility challenges for women with sickle cell disease. 2017. Expert review of Haematology. 10:1-11
  7. V. Elchuri, R.S. Williamson, R. Clark Brown, et al. The effects of hydroxyurea and bone marrow transplant on Anti-Mullerian hormone (AMH) levels in females with sickle cell anaemia. Blood Cells Mol Dis., 55 (1) (2015), pp. 56-61
  8. Hamoda H, Panay N, Pedder H, Arya R, Savvas M. The British Menopause Society and Women’s Health Concern 2020 recommendations on hormone replacement therapy in menopausal women. 2020 Post Reproductive Health 26 (4) 181-208
  9. Canonico M, Oger E, Plu-Bureau G et al. Hormone therapy and venous thromboembolism among postmenopausal women. Circulation 2007;115(7) 840-845
  10. Scarabin et al 2018. Progestogens and venous thromboembolism in menopausal women: an update or oral vs transdermal oestrogen meta-analysis. Climacteric 4: 341-345
  11. Boardman HM, Hartley L, Eisinga A, et al. Hormone therapy for preventing cardiovascular disease in postmenopausal women. Cochrane Database Syst Rev 2015; 3: COCHRANE ANALYSIS
  12. Stevenson J. NICE guideline-Menopause: diagnosis and management. Long-term benefits and risks of HRT (section 11): Osteoporosis. Post Report Health 2016 22(2) 92-94.
  13. British National Formulary. Interactions. Last Accessed June 2021
This error message is only visible to WordPress admins

Error: No connected account.

Please go to the Instagram Feed settings page to connect an account.

Instagram