Nadya Siyam | 09-05-2019
In the heart of the Gaza Strip, in al-Shati Refugee Camp, live two children born with PKU (phenylketonuria), a genetic disease that wreaks havoc on their bodies. Jehad and Jana Abu Askar, 3 and 2 years old respectively, were diagnosed with PKU about a week after birth. It is a serious condition everywhere, but particularly in Gaza, where specialty medicines are in short supply. And the diagnoses changed the life of their family forever.
Children with PKU have difficulty metabolizing an amino acid found in protein called phenylalanine. The amino acid is central to the function of the body’s nervous system. Without proper treatment, phenylalanine accumulates, especially around the brain, causing profound damage. The consequences include developmental delays beginning at an early age, becoming more severe with time.
Testing and diagnosis easy out of Gaza; not so inside
Until recently, the test for PKU wasn’t available in Gaza. New parents often had no idea anything was wrong with their children until the symptoms, like IQ loss, were advanced. Sanabel, the mother of Jehad and Jana, told me, “I know a number of mothers whose kids suffered mental retardation or died at the age of 10 without being diagnosed.”
Now the test is available in Gaza and administered within the first week of a child’s birth. According to the Ministry of Health, the number of people with PKU in Gaza is approximately 232 and this number increases each year.
However, married couples are not routinely tested to identify whether they carry the gene for PKU. Jadad’s and Jana’s parents didn’t know they were at risk, since no one in either family had suffered PKU.
“The first time we heard the term PKU was when Jehad was born, and we received this news with shock,” Sanabel said.
Learning to live with PKU
There is no cure for PKU. However, it can be controlled with a protein-restricted diet, low in foods like meat, poultry, fish, eggs, legumes, bread, milk and cheese. They get the nutrients they need from protein by drinking a phenyl-free milk and other such custom foods. Regular blood tests determine whether phenyl levels in the blood are within a safe range.
This is when life for PKU children in Gaza becomes tenuous, since the Palestinian Authority in the West Bank has said since March 2017 that it cannot afford to send in phenyl-free foods.
Jana was 4 months old when imports stopped. At that age, she totally depended on custom milk. She could receive nutrients only via IV and her health deteriorated as she rapidly lost weight and suffered skin abrasions.
“It was as if her skin was consuming itself. She was slowly and painfully dying before our eyes,” Sanabel remembered. After a number of failed attempts, Jana finally received a permit to travel to Jerusalem, where she received care at al-Makassed Palestinian Hospital. “A miracle happened: My daughter was saved,” Sanabel said.
Jana has gradually regained her health. She travelled to al-Makassed Hospital another three times until she made a full recovery. But that didn’t change the fact that the milk was not available in Gaza.
Months later, several charities and private institutions began to work together to bring the phenyl-free food into Gaza. However, the amounts were small and the price was high.
“Each can of milk cost $78 and we need at least two cans a week for both children,” said Sanabel. The salary of the children’s father cannot cover the price of even half the needed amount every month, assuming the milk is available. “Sometimes I have to give my children smaller portions of the milk to make it stretch as long as possible.”
Sanabel’s father, Waleed, works at a medical relief organization and said, “The milk isn’t some luxury health product. No matter how old the children become they will never be able to live without it. In fact, as they grow older, they need to drink larger amounts of the milk to make up for the nutrients their diet lacks.”
Sanabel made the effort to educate herself about PKU. She found a number of PKU Facebook groups, which post the latest updates on the condition. Sanabel said the Facebook groups help her feel less alone; the members have become something like a family. They share their diets, blood tests and stories with each other, forming a small community of their own. Health organizations in Gaza also have organized sessions for parents of PKU children, introducing them to each other so they can form a support network.
Through those groups, Sanabel learned there are new foods tailored to people with PKU, ranging from pasta, to chicken soups, to eggs and biscuits. Scientists have developed a way of removing phenyl from these foods and make them similar in look and taste to unaltered variants. Unfortunately, those “custom foods” aren’t available in Gaza or the West Bank.
“I have contacted a Saudi company that is the main distributer of German free-phenyl products to Arab countries in the Middle East and it said they can’t export to Palestine,” said Sanabel. These foods have become standard for PKU patients worldwide, yet for Gazans they remain an elusive luxury.
According to Inji Shaheen, M.D., the physician who cares for Jehad and Janna, health organizations have issued several appeals to the Ministry of Health asking for more custom food for patients with PKU. However, the blockade, the lack of financial support from the Palestinian Authority and the rarity of the disorder have remained challenges.
Sanabel knows how much difference those foods will make in the lives of her children.
“They would feel less different from their peers if they could eat normal food,” she said. “I don’t have enough words to tell you how delighted I feel when I find free-phenyl biscuits in the market; I know for them this is like a unexpected gift.”
At home, Sanabel and her husband eat the same foods as their children. They avoid buying any food their children aren’t allowed to eat, so Jehad and Jana won’t feel deprived.
The challenge of 'fitting in'
Jehad and Jana live in a family building where they often see their cousins who are close to them in age.
“My children always want to visit their cousins and play with them, and they feel frustrated when I keep them home,” said Sanabel. “Whenever they see their cousins eating chips, deserts or even a cheese sandwich, they start crying, asking for some.”
When one of their cousins has a birthday party, Sanabel tries to distract her children from hearing the loud music mixed with laughter downstairs.
“Every kind of food served in any party or gathering is full of phenyl, and my children go crazy if I try to prevent them from trying a colorful candy, for example,” she sighed.
The family used to live on the ground floor, until it became unbearable to see passersby carrying plastic bags from the market with foods the children can’t eat. They switched apartments to be as far away from others as possible.
“It breaks my heart that they can’t live normally like other children, but I can’t risk letting them go out,” explained Sanabel.
What frustrates her most is the lack of awareness and understanding of the disease. Although she constantly talks to people about it, they surprise her by saying things like, “come on, let them try chocolate; one time won’t matter much.” As Dr. Shaheen added, the disease is too rare to expect hospitals to organize public-awareness sessions. So, it's up to the parents.
When it’s time for kindergarten, Sanabel will have to go with them every day. “I can’t keep them away from people forever, but I have to make sure they are safe until they are aware enough to depend on themselves. All I want for my children is for them to fit in with the others, to have a normal childhood--at least as much as they can in a place like Gaza.”
Sanabel’s family and others who must cope with PKU may soon receive relief. Our partner Rebuilding Alliance is raising funds to help purchase phenyl-free foods.
Posted: May 8, 2019
Mentor: Deborah Root