What the HHT?

A Voice for the HHT Community

Jacob’s Song


By Desiree Clark

Jacob's Song“What’s wrong with his hands?” asked our agent. “His fingertips are round and purple. I’ll have to make a note – no hands in shot.”

That was the last straw! Jacob was three years old when he started modeling, and it took a total stranger to tip me over the edge of my frustration about what I had been telling doctors for years, SOMETHING IS WRONG WITH THIS KID!

It’s called clubbing – when the fingertips begin to form an abnormal roundness indicates an issue with circulation. No one ever put a pulse oximeter on his little clubbed finger at medical appointments, and this is something VERY important in detecting possible signs of danger for HHT patients.

So, I prayed, “Lord, I know there is something wrong with Jacob. No one is listening, so please show me what it is so I can take care of him.”

Ten days after my prayer, Jacob plopped on to my bed to watch a movie. Moments later, I heard an unusual noise, so I ran to my room and discovered he stuck his head through a decorative wrought iron circle in the middle of the footboard for better viewing. I quickly lifted his head to open his airway and gently tried to wiggle him out to no avail.

By the time 911 arrived he was out of the bed frame, but the EMTs checked him over anyway and took a pulse oximeter reading. It was very low. After 20 minutes it wasn’t improving so they strapped him to the gurney and took him to the hospital.

pulse oximeter

A Pulse Oximeter

The ER physician took one look at his hands and told us there was a problem with his lungs. Jacob was admitted for several days while his oxygen levels continued to drop. Tests reveled he had to have a lobectomy. His right lower lobe was completely useless due to pulmonary arteriovenous malformations. PAVM is an acronym our family was soon to know well.

When the surgery was scheduled for three weeks out, the voice inside my head exploded: “Three weeks are you kidding me?!!!!! This poor kid keeps crashing and they’re going to wait three weeks!!! Dear Lord, what do we do?”

While I was screaming at the surgeon in my head, there was a knock at the door. He seemed annoyed to be interrupted, but after some whispering with a nurse he turned back to us and said there was a surgical opening the next morning at 7 a.m. We took it.

I never heard a child scream as loud as my little boy when he came out of that surgery. The surgeon told us it was a very difficult operation, and that my little guy would not have survived three more weeks. We could only see him from behind a window because they do not want a suffering child to associate the pain with their parents.

Through that one-way window I watched the same tiny hands that foretold his medical future, reach out for me to find comfort in his only known security. I could only reach my hand out and place it on the cold glass as my heart broke in two.

Jacob 17

Jacob Now

But there was another hand that rested on this little hurting soul – the hand of a Father who sent an angel/nurse to Jacob’s room. On her guitar she played his favorite songs for hours as his screams of pain melted to soft whimpers. To this day, Twinkle Twinkle Little Star brings a wave of peace to me that could only be drawn from a moment like this.
This is only the beginning of Jacob’s story and our family’s path through the ups and downs of HHT, but I tell it to emphasize how important it is to be screened. A simple pulse oximeter reading could have made this situation a manageable one.

Knowledge of this disease has come far since my now 17-year-old son lost part of an organ, but there are still many in the medical profession who do not have HHT on their radar. There is power in your hands to “click” on Cure HHT, to forward information to your doctors so they may save lives. There is power in your hands by putting them together in prayer for friends and family who may be suffering from nosebleeds, migraine headaches and embolisms. The cure will come. We’re so close…..

2 thoughts on “Jacob’s Song

  1. Thank you for sharing Jacob’s Story, it is one I know first hand. I was 5 months when I went blue and my nails clubed they took a third of my right lung out due to Pulmanary AVM’s. Did not anticipate I would survive the surgery. I did and was anemic all my life but HHT was not diagnosed until my 30’s. Finally the random parts of the puzzle fit together.


    • How are you doing? Did your doctors recommend screening every year? Have you had any other AVM’s? It’s so frustrating not knowing the future. One interesting thing that a doctor asked us back then when they were trying to figure out what was wrong, was if Jacob had any unusual birth marks. He did. One tiny circle on the bottom of his foot. He told us that they did not know what the connection was but that most folks he had seen with AVM’s had a birthmark type thing somewhere else on their body. Has anyone ever heard of this?


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