Post on 24-Dec-2015
DBA UK 14th Anniversary Conference17th-19th May 2013
Hormonal and fertility issues caused by transfusion
Dr Scott AkkerConsultant EndocrinologistSt Bartholomew’s Hospital
London
The Endocrine/Transfusion interface
• Growth and sexual development
• Diabetes
• Thyroid and Parathyroid
• Bone
• Gonad (Testis and Ovary)
• Adrenal
The pituitary gland – the control centre
The pituitary controls the function of the ovary and testis
GnRH – Gonadtrophin Releasing Hormone
LH – Luteinising Hormone
FSH – Follicle Stimulating Hormone
LH and FSH in men
1)LH drives Leydig cells to make testosterone
2)FSH drives Sertoli cells to make sperm
Testis therefore has 2 separate but linked roles:- make hormone (testosterone)- make spermatozoa
LH and FSH in women
Ovary therefore has 2 separate but linked roles:- make hormone (oestrogen & progesterone)- make oocytes
LH drives hormone production and stimulates ovulation
FSH drives follicle development
Hormones allow the womb lining to develop
Iron overload Damage
Testosterone OestrogenProgesterone
Damage can occur at level of:
1)Pituitary
2)Testes & Ovary
Pituitary/Testis damage in men
Testosterone OestrogenProgesterone
Symptoms best treated by replacing the missing testosterone:
1)Daily testosterone gel (Testogel, Testim)
2)Testosterone injections (Nebido every 3 months)
BUT there will be no sperm production
Pituitary/Ovary damage in women
Testosterone OestrogenProgesterone
Symptoms best treated by replacing the missing oestrogen & progesterone:
1)A combined oral contraceptive preparation
2)A preparation usually used for post-menopause
BUT there will be no egg production
Fertility/Men
• For men:– If requiring testosterone, unlikely to be
producing sperm– If there is damage to the testes, may be
unable to produce sperm– If there is pituitary damage, the testes may be
able to produce sperm by replacing the LH and FSH
LH & FSH replacement
• Intensive
• Expensive
• Takes at least 3 months to produce a sperm and on average 6 months for a couple to get pregnant so at least a year of treatment
• May require additional help (IVF, ICSI, etc.)
Fertility/Women
• Difficult decision and discussion– Overall health important– Heart will have to do 40% more work– Diabetes adds to risks
• If damage to ovaries, may not be able to ovulate
• Clomiphene can help ovulate with careful monitoring
• May need additional help (IUI, IVF, ICSI)
Other fertility treatments/ IUI
IUI – Intra-uterine insemination
Sperm are placed directly into the uterus (womb) with a small tube/catheter
Other fertility treatments/ IVF
IVF – In- Vitro Fertilisation
Eggs are collected after Ovary stimulation
The egg and sperm are mixed in a test tube
An embryo is then placed directly into the womb
Other fertility treatments / ICSI
ICSI – Intracytoplasmic Sperm Injection
Eggs are collected after Ovary stimulation
A single sperm is chosen and directly injected into a single egg
The embryo is then placed directly into the womb
Fertility summary
• Fertility is a complex area and treatment will be limited by the amount and type of damage and cost
• Keeping low iron levels throughout life gives the best chance of not having problems with fertility
• It is possible that, like the heart, some of the damage to the endocrine organs is reversible with good chelation.
Bone Growth & Strength
• Multiple factors (Anaemia, Iron, Chelators, Growth Hormone deficiency/resistance)
• Also reduced by steroids• Widespread Vitamin D deficiency
– From sunlight! Absorbs calcium from gut
• Vitamin D easily replaced – Adult dose 25mcg/1000 units once daily
• (less in pregnancy and children)
Underactive Thyroid
Thyroid gland makes thyroxine
Easily replaced with tablet or liquid thyroxine
Correct dose found by measuring free thyroxine level in the blood (fT4).
BUT TSH must be ignored (if iron)
Adrenal Failure/Steroids
• Very rarely due to iron overload
• If on steroid treatment or replacement– Steroid card & Medic Alert– Emergency access to steroids if vomiting– Understand when to increase dose (eg)