I am trying to raise enough money for a dear friend Mirjana Komljenovic so she can afford the medication for her Pulmonary Fibrosis which is a rare disorder, Hermansky Pudlak Syndome.HPS is a result of her condition Albinism. Her Expectancy is anywhere from 4-10 years of life after diagnosis. This medication Ofev that she has been approved for slows down the progression of the fibrosis. This medication is $4,000.00 a month, she has
50% covered so I am trying to raise enough money that will cover the remaining $2000.00 a month for 2 years of this medication she so needs. I will keep raising even after this amount
to make sure she is supplies for as long it is needed.
Mirjana has spent her life advocating for others, so I feel the need to advocate for her. She is a remarkable, amazing woman deserving the right to extend her life in any way fit.
THIS IS IT
I have Pulmonary Fibrosis.
There. I said it. The secret is out.
I am so nervous to let anyone know of my big secret. I am feeling very nervous and vulnerable right now. What’s the big deal? What is Pulmonary Fibrosis? It’s a disease without a cure. It is the means to the end of a life. It is progressive, cruel and it takes my breath away. In fact, it takes away the breath of anyone afflicted.
By definition, Pulmonary Fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. The thickened stiff tissue makes it more difficult for lungs to work properly. The scarring associated with pulmonary fibrosis can be caused by a multitude of factors, but in most cases, doctors cannot pinpoint what is causing the problem. When a cause cannot be found, the condition is termed Idiopathic Pulmonary Fibrosis or IPF. This is not the case with me. By the way,
I also have a rare genetic condition called Hermansky Pudlak Syndrome.
There. I said it. That secret is out too.
By definition, Hermansky Pudlak Syndrome abbreviated HPS) is an extremely rare genetic disorder which results in oculocutaneous albinsm, It is considered to affect around 1 in 500,000 people worldwide, There are eight classic forms of the disorder, based on the genetic mutation from which the disorder stem..
HPS-1 and HPS-4 present with features of HPS including oculocutaneous albinisim, reduced visual acuity, horizontal nystagmus, easy bruising of soft tissues, epistaxis, and prolonged bleeding after dental extraction, surgery or childbirth. Women may present with medically significant menstrual bleeding. Complications of HPS may include granulomatous colitis and pulmonary fibrosis. Pulmonary fibrosis is the most serious complication of HPS-1 and HPS-4 and usually presents in the fourth or fifth decade.
Management and treatment
Lung transplant is the only known treatment for pulmonary fibrosis in HPS-1 and HPS-4. Pirfenidone may slow progression but only in patients who have significant residual lung function. Steroid therapy is not effective.
Prognosis is poor as the pulmonary fibrosis is fatal.
If you’re keeping score, I have a condition, a disorder, and a disease at the same time.
I am little angry.
How did it get to this? One day, not long ago, I was in Toronto at the Royal York Hotel standing up at an elegant dinner, receiving recognition as one of the top 3% Women of Influence in the entire country. I was voted Citizen of the Year by my peers in my community. I received Employee of the Year in Vancouver for the entire province. I manage an award winning business and a non profit I manage was just awarded Non profit of the Year. I have a great family, happily married for 32 years, a close knit, fiercely loyal tribe of friends, an extensive network of colleges. Not of it was handed to me, I worked very hard for all of it. It was not easy growing up with albinism, my vision is very poor and I did not look like others. Many people were very cruel and judged me solely on my appearance and considered me not capable of much because of my disability. It was a matter of putting aside mistrust and anger and hurt and even the memories to fill my heart;s desire. I was clear on my purpose. I was not clear on how to get there. . I rose above so many of the obstacles placed before me. I will share more of my experiences another time, with the hope that my experiences will help another that may be going through difficulties.
So why am I doing this blog? I need a medium to express myself, one that works well for me, that medium is writing. A global pandemic that affects the respiratory system and facing my own mortality shifted my perspective. I feel an urgency to complete what has not been completed., not sure exactly what that is but I know I need to continue. I also need to release the burden I am carrying. I look great but I am not well. I cannot do the things I could before and I cannot do the things I want. I need to openly share that I need to be lifted by others, I need strength and support to move through my process.
Already I feel the love and support of all those I have shared this with To them, i am so very grateful. This is a burden on them as well. Thank you for carrying it with me. I want everyone to know that my will and abilities are larger and greater than any obstacle in my way.
Just watch me.
TAGSALBINISM, HPS, PULMONARY FIBROSIS
- Kim Ruttig-English
- Alexandra Fernandez
- Marilyn Burkett
- Linda Guernsey
- Colleen Power
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