Treatment

Treatment for proportionate short stature disorder requires a course of various treatments rather than simply one single course.

In children, a human growth hormone is usually administered to help stimulate development. Doctors tend to recommend one called Somatropin, though it will only be administered to children with an officially diagnosed growth hormone deficiency.

Somatropin is administered in a single dose injection, given on a daily basis and has been proven to increase height by up to 10cm. For girls in the early stages of the Turner Syndrome based version of the disorder, Somatropin can be safely administered to help prevent short stature, alongside estrogen and other hormone related therapies.

Adults have also been treated using Somatropin as it can improve bone mineral density, reduce the risk of cardiovascular problems and arthritis, though growth developments are not expected.

Prior to administration, a doctor must ensure the adult patient fills the criteria set out for Somatropin use.

Somatropin criteria

Firstly, an insulin tolerance test is taken to determine the presence of a growth hormone deficiency. Insulin is injected into the blood, which causes sugar levels to fall. This tests the efficiency of the pituitary gland, which should release growth hormones. If it fails to, or produces a lower amount than expected, the condition can be confirmed.

Secondly, the patient must complete a questionnaire that will indicate whether their life is currently being impaired by the condition. Finally, the doctor must check their medical records to ensure they are not receiving another treatment for a pituitary gland hormone deficiency. Once these tests are complete, the patient can begin Somatropin treatment.

Around 1500-2000 adults are treated with growth hormones in the UK alone and while the effectiveness of such treatment in adults remains difficult to prove, chronic conditions can be improved.

Disproportionate short stature treatments focus on either preventing the complications that can occur as a result of the disorder or treating them.

Children

Children with the condition should make regular visits to a paediatrician, who will look at the individuals’ case and recommend various treatments and therapies based on their personal specifications. These treatments include physiotherapgy, speech therapy and sessions with an audiologist who will analyse any hearing problems and work towards adequate solutions. The patient may also consult with neurologists and orthopaedic specialists to identify any nervous system or spinal/joint disorders respectively. Additionally, the patient can meet with a geneticist in order to look closer at any genetic disorder that may be present, any available treatments for it and to discuss its implications for their future plans.

Riskier treatments

Due to the irregular growth of bones and limbs that occur in cases of disproportionate short stature, a number of riskier treatments are available to help lengthen bones – particularly in the legs. The most common of these is a treatment called distraction, whereby the leg bone is broken and placed within a specially designed frame. This frame is then adjusted on a daily basis to create a longer, stronger bone in the leg. Prior to treatment, it is important to understand that the procedure does not come with any form of guarantee. It does not always work and can often cause side effects such as pain, infection, irregular bone lengthening, irregular bone formation and even deep vein thrombosis.

Less popular treatment

A number of similar, less popular treatments are also available. Patients can have metal staples inserts into the ends of any long bones, so that as the bones grow they maintain a correct direction. Limb bones can be divided, re-straightened and then reinforced with metal plates. Rods and staples can also be inserted around the spine to help correct its shape. Additionally, one procedure increases the size of the openings in the bones of the spine in an effort to reduce pressure placed on the spinal cord by the condition.

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