ToniV 2.0: The Orkambi Odyssey

Yesterday, I began taking the highly anticipated Cystic Fibrosis drug, Orkambi.

My box of hopes!

My box of hopes!

The medication, a combination of two drugs, aims to correct the defect that causes the characteristic thick mucus in CF patients with two copies of the most common gene mutation. The mutation, Delta f508, causes both lung and digestive involvement, in addition the sinuses, liver, and other organs.

The mutated gene exists in every single cell in my body. So taking this medication will affect the way that every single cell in my body works. Without the Orkambi, the gene mutation doesn’t allow salt and water to go through the cell properly, creating the thick, sticky mucus that harbors bacteria in the respiratory system and clogs the digestive system.

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Taking those 2 pink pills twice a day will tell every cell in my body to work properly.

Every. Single. Cell.

If Orkambi works as designed in my body, imagine the potential positive effects of no longer producing the thick mucus! Oh, the possibilities!

  • Without the mucus clogging every organ, Superbugs will no longer find my lungs a warm, moist place to build a colony.
  • I might not have a deep, croupy, constant cough. My husband would have to find a different way to find me in Target.
  • My pancreas – what is left of it (as it is very damaged), might still product some enzymes and insulin, which could increase my nutritional status, and everything else that goes along with eating a nutritionally balanced diet. My diabetes could become more manageable. I might be able to eat more than a handful of tried and true foods that I know don’t make me sick.
  • My sinuses won’t be clogged to the point I needed surgery every few years.
  • My belly wouldn’t clog up and need medical “roto rooter” or heavy medications to prevent bowel obstruction.
  • My liver may start functioning properly.
  • I may no longer lose tremendous amounts of sodium through my sweat, lowering my risk of electrolyte imbalance and heat stroke.
  • Women with historically too much mucus in their reproductive system might now become better able to conceive.

IMAGINE!

Imagine – not getting sick every 4-6 weeks. Not going into the hospital every few months. Not necessarily having to lose thousands of dollars in cancelled trips and events due to suddenly being sick. Being able to actually PLAN something, follow through, and build a memory.

But all of this is not without cost.

First, there is availability. It is only patients over 12 years old with copies of the mutation Delta f508 and lung function of a certain level are cleared to take it now.

Financial – Personal finances, health insurance, public health coverage, lack of coverage, and even where you live will play a role in if and how you get Orkambi. AndWithout the help of the manufacturer, the CF Foundation, my insurance, and the help of some very dedicated people, I would not have access to Orkambi, I won’t spend any space here talking about the financial impact, because every person and every situation is different.

Next, there is a the “cost” of immediate health when starting Orkambi. Many/nost patients experience some combination of side effects, such as shortness of breath, fever, chest pain, low Oxygen levels, active infections, and basically whatever usually happens when you “get sick”. Many patients end up feeling much sicker while in the beginning stages of taking Orkambi, and their lung function tests show a lower level of functioning.

This is because as the cells begin to work properly, and no more thick sticky mucus is being made, the body still has to get rid of what IS in there. So it’s often refered to as a PURGE. It’s like having pneumoina and the flu at the same time, as the mucus and colonies of bacteria move from the innermost crevices of the lungs up through the bronchial tree and out the esophagus and mouth.

Additionally, Some people experience bowel issues as well, but now, I am only experiencing the Lung Purge.

My Experiences in the Past Two Days – 4 doses

The first few weeks/months can be so difficult that my doctor is on standby should I need oxygen or antibiotics to get through it. It can make you feel terrible. Some patients have ceased treatment because the side effects were so unbearable and they were so sick.

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In addition, you must take each dose with 20 mg of fat.

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This is tough for someone who doesn’t eat for the first time until after noontime, and whose pancreatitis is not fond of fat or protein.

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Right now, I have experienced varying episodes of:

  • hacking coughing and coughing up liquid sputum every few hours

  • draining sinuses

  • followed by nausea from the gag reflex

  • shortness of breath

  • the feeling like I’m breathing in a very dusty room

  • chest pain all over on the inside of my lungs

  • pulled muscles in my abdomen from coughing too hard

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It’s tough because to get to the other side, you have to walk through hell. It could put you in a life-threatening situation – which is why it’s so important to realize that we are all so different, and that there are no assurances.

But at this moment, the potential impact on my life – on our lives is enough to motivate me through the experience. Who knows where I’ll be a week or month or year from now.

But if dreams mean anything, I might be able to be the wife I’ve always wanted for to my husband, who, like me, didn’t really know what “growing old with CF” entailed.

All I know now is that I can take these pink pills twice a day, and if my body responds to them favorably. I could potentially have a brand new life ahead of me.

This could be the start of ToniV 2.0!

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