Sickle Cell and Menopause

If you suspect that you’re perimenopausal or menopausal, and your symptoms have started to interfere with your quality of life, it’s a good idea to make an appointment to see your doctor or a healthcare professional who specialises in the menopause.

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/sickle-cell/about/?CDC_AAref_Val=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.htmlhttps://www.cdc.gov/sickle-cell/about/?CDC_AAref_Val=https://www.cdc.gov/ncbddd/sicklecell/facts.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

Preparing for your appointment

If your local surgery offers double appointments it’s a good idea to book one of these so that you’ll have enough time discuss your symptoms and concerns. Before your appointment, take a look at the symptoms list to record the menopausal symptoms that you’re experiencing.

Make a note of any changes to your periods, and bring a list of any medications that you’re taking, including herbal supplements. It’s also wise to jot down any questions or concerns, so that you don’t forget to mention them during your appointment. If you’re feeling anxious, you can bring along a friend or family member for moral support.

Your first appointment

According to the 2015 NICE menopause guidelines, your doctor should discuss the following:

  • the stages of menopause
  • common symptoms of the menopause
  • how the menopause is diagnosed
  • lifestyle changes that can help your health and wellbeing
  • benefits and risks of treatment
  • how the menopause can affect your future health

 

Your doctor may want to carry out blood tests to check your hormone levels, but this isn’t usually necessary as these can vary by the hour. Most women can start HRT without needing any investigations or blood tests.

Sometimes your doctor may want to carry out blood tests to rule out other underlying issues, such as an underactive thyroid, and they will also check your blood pressure.

If you would like to take HRT and you feel you would benefit from taking it then you should ask at your first appointment for a prescription of HRT. The majority of women benefit from taking HRT and women can start taking HRT during their perimenopause. No women is usually too old to start taking HRT, even if it is many years since your menopause then you should still be able to take HRT.

Try to leave the room with as many questions answered as possible, ask if your doctor has any leaflets or information they can share with you, and check if you need a follow-up appointment. NICE guidelines recommend a review three months after your first appointment, but you should ask for an earlier review if you are experiencing side effects or the treatment doesn’t seem to be working.

A second opinion

Unfortunately, some doctors and healthcare professionals still believe outdated reports that HRT is linked to cancer, blood clots and heart problems, so they may be reluctant to prescribe HRT. If there’s no medical reason why HRT is unsuitable for you, then it’s important to speak up and, if necessary, ask for a second opinion.

You could ask to speak to another healthcare professional at your surgery, or ask to be referred to a specialist. Alternatively, you could refer yourself to a private menopause clinic. In the UK, the British Menopause Society has a register of recognised menopause specialists at thebms.org.uk. If you live outside the UK, you can contact the International Menopause Society.

What happens if my doctor / nurse won’t give me HRT?

  • Informing your doctor about what you are wanting to discuss prior to the appointment or at the start of your consultation will help to ensure you get the most out of your consultation.
  • Know your rights as a patient. Doctors will be more likely to consider your views if you can show you are fully informed and understand what any risks are and explain clearly why you still wish to have that treatment option because of the benefits to your life and health you believe it would bring.
  • Be persistent but polite. If you do not get the desired outcome at the first appointment, try again another time. You can ask to see another doctor within your practice.

In general, your best approach when talking to your doctor about your menopause is to clearly state your reasons for what you would like, explain what information has led you to this decision, and that you know what the associated risks might be but that it is still what you choose to do. This information may need repeating on several occasions, to several doctors or nurses, but persistence often pays off when you can give a clear and rational argument that shows careful consideration of the evidence of the benefits to your health.