Transcript UK THALASSAEMIA SOCIETY NATIONAL PATIENT & FAMILY

DBA UK 14th Anniversary Conference
17th-19th May 2013
Hormonal and fertility issues
caused by transfusion
Dr Scott Akker
Consultant Endocrinologist
St 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
LH drives hormone production
and stimulates ovulation
FSH drives follicle development
Hormones allow the womb
lining to develop
Ovary therefore has 2 separate but linked roles:
- make hormone (oestrogen & progesterone)
- make oocytes
Iron overload Damage
Damage can occur at level of:
1) Pituitary
2) Testes & Ovary
Testosterone
Oestrogen
Progesterone
Pituitary/Testis damage in men
Symptoms best treated by replacing
the missing testosterone:
1) Daily testosterone gel (Testogel,
Testim)
Testosterone
Oestrogen
Progesterone
2) Testosterone injections (Nebido
every 3 months)
BUT there will be no sperm production
Pituitary/Ovary damage in
women
Symptoms best treated by replacing
the missing oestrogen &
progesterone:
Testosterone
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)