Metabolic Mayhem: Living with Lipodystrophy

The story of Sonia

The two people in my life that lived with lipodystrophy, died before the age of 35; my mother and sister. The journey of living and coming to terms with my disease has been an arduous struggle on all aspects. My mutation caused my sister and I to develop symptoms rather early, starting with the development of acanthosis nigricans (darkened skin patches associated with insulin resistance) in the creases of our skin, around the age of 5. My mother would try to clean it away by rubbing it the skin with steel wool, to no avail. Things got only worse. My mother, a non-compliant heart patient, who never took her anticoagulants, suffered a massive stroke that caused her death at 32. The heart problems were from the lipodystrophy, but we didn’t know that at that time. She was skinny, muscular, with a partial fat loss profile, but in those days, she was considered lucky to be so slim, and we didn’t know that it was a sign of metabolic disease now known as familial partial lipodystrophy.

My sister, again following in my mother’s footsteps followed the “If I don’t see it, It doesn’t exist” philosophy, suffered diabetic complications starting in her 20s. Complications including diabetic neuropathy, retinopathy, nephropathy and cardiac disease saw my sister slow deteriorate and die from sudden death at the age of 35. She was my rock, my everything, and she was gone.

I turned 35 this year, and I’m going strong (knock on wood, people!). I try to be the best patient I can be, working with my doctors and medicines, but everyone falls occasionally, as do I. 9 prescriptions and 5 supplements as of today, and the number will increase as I get older. I’ve had a major heart attack and stroke due to my lipodystrophy affecting my heart rhythm, causing atrial fibrillation. Apart from the serious medical complications of my disease (heart disease, diabetes, hyperlipidemia…), I suffer from the physical attributes of the disease. My build is muscular and hard, not soft like a typical woman. It hurts to sit due to no padding on my buttock and I gain fat on all the wrong places, like my face and abdomen. Most importantly, the insulin resistance has lead to me having androgen excess and facial hair, which has been the most difficult thing I struggle with. Needless to say, this has affected my overall mental health, with diagnosis of major depression, with which I cope with regular psychotherapy and antidepressant medications.

I hope to see new therapies for lipid and insulin resistance disorders such as lipodystrophy. I anticipate increased interest from stakeholders and the medical community, as our global community is seeing greater metabolic disease prevalence due to increased obesity and diet alterations.