Working to raise awareness of Klinefelter's Syndrome

and to provide help and advice to all those affected

Consult TOM

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These pages provide information on Treatment, Options and Maintenance (TOM) for males diagnosed with KS. The information presented in these pages relating to testosterone replacement include extracts taken from part of the KSA Member's Binder written by Pierre-Marc Bouloux MD, FRCP, Professor of Neuroendocrinology at the Royal Free Hospital, University College, London.

For those diagnosed with KS who believe they are female or of both male and female genders there is sparse information available - something the KSA is working on to rectify. However, for the time being the KSA highly recommend checking out: www.gendertrust.org.uk/faq/hormones-mtf/, which provides some very useful information. Another newly created website (2013) is the XXY Brain Trust which is geared to understanding intersex.

The KSA gratefully acknowledges the award of a grant from the British Society of Endocrinology which enabled the creation of the Consult TOM pages.

Treatment

Not everyone diagnosed with KS needs testosterone replacement therapy. Some individuals may be producing enough testosterone for their own personal perception of how they feel or they may have some other health issues that would not benefit from treatment with testosterone. Any decision to start using testosterone replacement therapy should be made in full consultation with your endocrinologist, your GP and any other medical professionals that you are working with/consulting at the time.

Before treatment is started, the male patient's suitability is assessed, as there are some conditions which testosterone replacement may be contraindicated. These include:

  • A history of prostate cancer (as the cancer 'feeds'; off testosterone and grows)
  • An abnormal prostate test - the cause must be determined
  • A history of male breast cancer (as the cancer 'feeds'; off testosterone and grows)
  • A history of raised red cell count which increases the risk of getting blood clots

Patients who have benign prostate hypertrophy (BPH: a non-cancerous enlarged prostate) can have testosterone treatment but need to be monitored closely as the testosterone can cause the prostate to get even larger (the prostate gland depends on testosterone for its growth and 'maintenance'), which can lead to urine flow problems. There is no evidence that this causes it to develop into cancer.

Prostate Tests

Two of the commonest tests include:

PSA (Prostate Specific Antigen) Test
The PSA test is not specifically for prostate cancer alone but an abnormal (high) result can indicate a prostate problem, including cancer. PSA is a protein made by the prostate and small amounts are found in the blood. If there is a problem with the prostate, the blood levels can go up.

Digital Rectal Examination
This is a quick examination and usually done in outpatient clinics or a GP surgery. It involves the doctor feeling the prostate through the back passage by passing a finger into the rectum. The back of the prostate is the only accessible part , but it is still useful to examine it as it is the commonest site for prostate cancer and abnormalities in size and texture can be detected this way in the early stages. Some men find the examination a little uncomfortable or embarrassing but it should not be painful. Further investigations may be performed and referral to a Urologist may be made if abnormalities are found. The prostate can be removed if symptoms are troublesome.

Testosterone treatment and cancer of the prostate and breast (male)

Prostate: There is no specific literature that suggests testosterone replacement therapy increases the risk of prostate cancer in men, as the testosterone is being used to substitute for what the body would normally make. However, if cancer is in place or is suspected, treatment should be sopped immediately as cancer 'feasts' on the testosterone (men who do not require testosterone replacement may be given medication that stops them producing testosterone. In rare cases both the testes need to be removed to cut off the testosterone supply).

Breast: About 3-5% of men with KS develop breast cancer and the risk increases if there is a family history of the disease in female relatives. The reason for this is unclear. Normally a small proportion of testosterone is converted into oestrogen in certain tissues, including the breast. Men with KS have been found to have a higher conversion of testosterone to oestrogen and it is not known whether this increases the risk of breast cancer in this group of patients. Oestrogen blood levels are not always elevated however. Therefore, testosterone can indirectly aggravate male breast cancer. Men with KS are urged to check their breasts regularly for and changes in appearance or texture.

Red blood cell production

Testosterone stimulates the production of red blood cells. Red blood cells are responsible for carrying oxygen round the body. Low levels of red blood cells therefore cause extreme tiredness, lack of energy and in extreme cases breathlessness. The blood test for measuring the red blood cells is called the haemoglobin (Hb) and haematocrit test. A low haemoglobin level is also called anaemia. Testosterone replacement usually improves the anaemia as it stimulates red blood cell production, resulting in less tiredness and more energy. Testosterone, if given in excessive amounts can cause overstimulation of red cell production, resulting in a high red blood cell count in the blood (polycythemia) in certain patients, which causes the blood to get thicker, which increases the risk of blood clots. It can also cause numbness or tingling in the hands or feet if the blood cannot easily get into these areas.

A full blood count should be performed periodically to monitor for this condition. Treatment may include blood 'letting' (removing a few pints of blood from the patient), temporarily stopping treatment, reducing dose or lengthening the interval between treatments. Some patients are more disposed to developing polycythemia than others.

Once the 'all clear' to start treating the KS is given, testosterone replacement is commenced and in order to maintain the benefits, will need to be continued for life otherwise the signs and symptoms will recur. 

In the 'Options' section various testosterone replacement methods are described. Which option is a matter of personal choice and the patient should not feel 'forced' into making any decision.

Side Effects of Testosterone Replacement Therapy

Testosterone is generally very safe with few side effects, providing the therapeutic dose is not exceeded. However, some patients do complain of the following:

Acne - it is not uncommon to develop 'spots' or acne at the start of treatment as testosterone stimulates sebum production. This is usually transient, requiring no treatment.

Male pattern baldness - as testosterone works on the hair follicles of the head, some patients will find their hair starts receding in the male pattern, or go 'thinner on top'. This will occur in genetically susceptible individuals.

Joint and muscle pain/stiffness - the reason for this is unclear but is thought to be due to the increased salt and water retention that can occur.

Less Common Side Effects:

Breast enlargement - usually subsides as treatment continues.

Abnormal liver function - is thought to occur in men with a pre-existing liver disease. Severe liver damage and liver cancer has not been found to be associated to testosterone treatment but rather to certain groups of 'anabolic steroids' (as used illegally in body building/sports).

Increased red blood cell count - thickening of the blood causing blood clots (see above)

Sleep apnea (snoring and stop breathing during sleep) - unclear if this is related to testosterone, as testosterone can enlarge the voice box and surrounding muscular structures. It is thought more likely to occur in men with a predisposition to the condition, e.g. obese or overweight men and men with obstructive lung disease.

Painful and sustained erections (priaprism) - It is unclear if this is related to testosterone and only isolate cases have occurred.

Aggression - some patients have commented on feeling more aggressive and short tempered after starting testosterone replacement. However, it is thought that patients who have never been exposed to testosterone or who produce little testosterone, may not be used to the 'manly' side of their personality and interpret this as aggression when treatment starts.

Prostate gland - enlargement may occur in susceptible individuals.

Options

The majority of testosterone preparations now available offer steady levels throughout treatment and the patient does not suffer from major disadvantages, though testosterone injections, for example Sustanon (given every 7-28 days) and capsules (testosterone undecoanate, taken 1-3 times a day) still have the disadvantage of fluctuating levels.

With the choices now available, the 'up and down times' should well and truly be a thing of the past. If a preparation proves unsuitable for an individual, another can be tried until a suitable one is found. This also takes into consideration convenience and effect on lifestyle.

PreparationDuration of ActionAdvantagesDisadvantages
Testosterone implants
Pellets implanted under the skin in subcutaneous (fatty) tissue
3 to 6 months (depending on dose)- Steady testosterone levels throughout
- Convenience- Pure testosterone crystals
- no additives
- Minor surgical procedure (needs local anaesthetic, small cut, and stitches
- removed after 7 days
- Infection (small risk)
- Small scar
- Extrusion
- implant works its way out or body rejects it
Long acting testosterone injection
e.g. Nebido
Deep intramuscular injection, single dose preparation. Intervals rather than dose adjusted according to response
10 to 14 weeks- Steady levels of testosterone
- Does not contain peanut oil or other nut oils
 - Convenience- May take a few months to reach normal levels in some individuals
- Larger and thicker volume of liquid than conventional testo injections and hence needs to be given in buttocks (slowly)  and should be warm
- Not suitable for self injection
- Can have pain from injection site/leg lasting 1-2 days
Shorter acting testosterone injections
e.g. Sustanon, Virormone, testosterone enanthate
Deep intramuscular injection
Every 4 to 28 days- can be self-administered
- 'old' and well established preparation
- Contains nut oil (peanut)
- large fluctuations between 'peak' and 'trough' testosterone levels
- some people feel the 'ups and down' effects between injections
- Frequent injections
Testosterone gels
e.g. Testogel (sachet), Testim (tube), Tostran (pump pot)
Daily application- Self administered
- Apparently steady levels- One dose (though this is not always the case)
- Can use part tube as Testim comes with screw top
- Convenience
- Pump pot delivery method with measured dose (Tostrum)
- Can be a little messy
- May leave white residue on skinRisk of testosterone transfer to other people, in particular women and children
- avoid contact for 6 hours after application or alternatively wear long sleeved tops. Hands should be washed immediately after application
- Available in one dose only hence need to work out right amount to apply if less than one sachet/tube is required. Discard partial unused sachet- Some patients have been known to get abnormally high or low levels necessitating dose increase or reduction
- Skin irritation can occur
Testosterone patches
e.g. Androderm, Andropatch
Applied to shoulders, back, upper arms. thighs, or abdomen
Daily- Self applied
- Convenient- Steady levels
- Localised skin irritation common
- Do not use same site within a seven day period
Testosterone capsules
e.g. Testosterone undecoanate
1 to 3 times daily- Easy to take
- Suitable where low doses required, e.g. in children where puberty is gradually being induced
- Poor blood levels and not as potent as other preparations
- Compliance and remembering to take preparation
- Inconvenience- May need to take several
Oral preparationcapsules a day

Maintenance

Main test
Blood tests, as well as assessment of general well being, are used to monitor effectiveness and tolerance of treatment. The KSA offer a Testosterone Diary (available in the Free Downloads section), which can help patients record the effect of testosterone treatment. The ideal preparation generates a steady flow of testosterone levels throughout, avoiding large fluctuations in blood levels and has positive effects on the symptoms with which the patient originally presented, such as, sexual function, mood and physical capacity.

Whilst taking testosterone it is not necessary to have blood tests taken at 8.00am, in contrast to someone who is not taking treatment testosterone levels are checked once treatment is established and the dose or interval of treatment is adjusted accordingly. The levels are usually measured mid-treatment ('peak' levels) and prior to the next treatment ('trough' levels).

The peak levels are when the testosterone levels are supposed to be at their highest, midday through treatment whilst the trough levels are when they are at their lowest. The ideal of treatment is for both peak and trough to remain within the normal range.

Other preparations, such as, gel preparations are best measured about six hours following application.

Other tests
Cholesterol checks; blood count (haemoglobin and haematocrit); sodium levels; liver function tests; PSA tests; bone density (DEXA) scan and a physical examination to check for body hair, size of testes and gynaecomastia (male breasts) are useful in monitoring progress.

 

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KSA Activity Weekend 2014

You are too late for the April 2014 Activity Weekend which was a huge success so book soon for the next one!

Next 2014 Activity Weekend - 19th to 21st September.

Special 'Earlybird' price IF BOOKED BEFORE 8th August:

Cost: £80 for those with KS or under 19, £135 for other Members, £310 for Non-Members.

If booked later the the costs rise to £90, £145 and £350 respectively.

Please book early as the weekends fill up fast!!

 There is more information about Activity Weekends under the Read more.... link below.
 Contact me as soon as possible if you want further information or wish to book a place by phone on 01629 534402 or activityweekends@ksa-uk.net

Read more...

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2015 Annual Conference & AGM

When: June 2015
Venue: Derby
Cost:   £ t b a

 

The 2014 Conference in Brighton was a huge success with delegates travelling from Ireland, Wales, Scotland and across England.

Nearly 50 attended the Friday Night Get-together!

 

 

  


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